Chapter 1 :

UPPER RESPIRATORY TRACT INFECTIONS

a. Acute Coryza/Nasopharyngitis
b. Acute Otitis media
c. Chronic otitis media
d. Acute Tonsillopharnygitis

Anatomy
Nostrils → Nasal cavity → Pharynx → Epiglottis → Larynx → Tracheal →
Major bronchi

Definition
• Upper respiratory tract is the region above imaginary line drawn through the
laryngeal opening or epiglottis.

• Acute upper respiratory tract infections : are usually < 3weeks duration

Types of AURI
1. Acute coryza (Nasopharyngitis)
2. Acute Otitis Media
3. Chronic Otitis media
4. Acute Pharyngitis (Tonsillopharyngitis)

I.
ACUTE CORYZA (NASOPHARYNGITIS)

Definition

Aetiology
- Is usually viral (rpar)
i. Rhinovirus - most commonly
ii. Parainfluenza
iii. Adenovirus
iv. Respiratory syncytial virus

- Occasionally, it could be Group A B-hemolytic streptococcus

Clinical Presentation
- the frequency of attack is usually 6 – 8 episodes/year
- the incubation period is 1 – 6 days with an average of 2 days
- the duration of illness is usually 7 days

Symptoms
- Is usually the triad of “SRB”
i. Sneezing – is usually the first symptom
ii. Rhinorrhoea (nasal discharge)
iii. Blocked nostril
iv. Others
Cough
Headache
Low grade fever
Sore throat
Malaise
Loss of appetite

v. Progression
a. Thin rhinorrhoea – first stage
b. Mucoid rhinorrhoea
c. Mucopurulent (yellowish) – Is usually NOT an indication for giving
antibiotics.

Signs
i. Swollen nasopharyngeal mucosa (due to the inflammation)
ii. Cervical lymphadenitis

Complications :
- Spread via the anatomical spaces
1. Acute otitis media
2. Sinusitis
3. Tonsillitis
4. Pharyngitis
5. Epiglottitis
6. Laryngitis
7. Tracheitis
8. Acute Laryngotracheobronchitis
9. Bronchiolitis
10. Pneumonia

Diagnosis
Is usually clinical. Investigation is not necessarily needed.
Differential Diagnosis

1. Allergic rhinitis
- its sneezing is more frequent, there is no fever
- there is usually eye itching
- Nasal smear shows eosinophilia

2. Influenza
- has similar symptoms but are more severe
- diagnosis is by investigation of nasal secretions

3. Pertussis
- its catarrhal stage is very similar to coryza

4. Prodromal measles
- when there is nasal discharge
- Koplik’s spots are present

5. Foreign body in the nostril

- Is usually unilateral

6. Others
i. Choanal atresia
ii. Diptheria
iii. Tumour
iv. Congenital syphilis
v. Wegener granulomatosis
vi. CSF rhinorrhoea

Treatment
- is essentially symptomatic since the aetiology is commonly viral except in the rare
occasion where streptococcus may the cause.

a. Nasal toiletting
- Involves removal of nasal secretion using rolled tissue or gauze
(recommended by Primary Health Care)

b. Keep the infant warm

c. The next step depends if the child was exclusively breastfed or not.

d. If the child has exclusive breastfeeding, nothing else is given.

e. If not, and the child is coughing, recommend simple home remedy for cough
 • Infants : Honey licks
Honey and weak tea

• Older children : Palm oil & Sugar

N.B
Cough syrup is NOT recommended because many of such depress cough
which is actually a protective mechanism. Some may even cause respiratory
depression.

f. If a child has a distressing cough and you are highly suspicious of acute coryza,
give dextrometaphan.
g. The use of vitamin C is highly controversial. The reason for its usefulness is not
very strong. Orange fruit is recommended.
h. If the temperature is ≥ 38.5C, give paracetamol (acetaminophen or Ibuprofen). If
it is below, recommend tepid sponging. Note cold water cause vasoconstriction of
skin vessels, therefore causing hyperthermia.

Follow-up
- Is the most important step. Tell the mother to bring the child back in 2 days if she
thinks the condition is not improving or the child has diffilculty breathing (which
is a feature of development of pneumonia or brochiolitis) she should report
immediately.

Prevention of Common Cold

o The best method should have been vaccination, but this is not feasible because of
antigenic variability of viruses. However, the following can be done

1. Sauna bath – Studies have shown that hot temperature prevents the viruses from
surviving. High temperature also improves the ciliary beating
2. Taking yoghurt – also reduces acute coryza by 25% in adult. The bacteria it
contains may stimulate immunity.
3. Good and Adequate ventilation – to avoid back-to-back infection
4. Liberal fluid intake – you can determine by the colour of the urine
5. Good hygiene e.g handwashing because the transmission is by fomite and not
aerosols.

N.B
- Antihistamines are not helpful.
- The use of nose drops – xylometazoline or oxymetazoline.
 II.
ACUTE OTITIS MEDIA

Definition
o Acute inflammation of the middle ear mucosa.

Aetiology
- Depends on the age

a. Neonate
- is usually caused by
i. Staphylococcus aureus (Gram +ve)
ii. Pseudomonas species
iii. Escherichia coli (Gram-ve bacilli)

b. Older Children
i. Streptococcus pneumoniae
ii. Haemophilus influenzae
iii. Group A B-hemolytic streptococcus
iv. Others – Moraxella catarrhalis

Clinical Presentation

o Acute otitis media is very common in the paediatric age group because of the
following reasons :
a. They have high incidence of acute coryza
b. Their Eustachian tube is shorter & wider (therefore organisms can easily
reflux from nasopharynx into the middle ear).
c. The cartilaginous & osteous portions of the Eustachian tube forms a relatively
straight line whereas in older children or adult, the angle is more acute.

Symptoms
a. The classical symptoms are
i. otalgia
ii. Fever
iii. Otorrhoea (ear discharge) – is less than 14 days in acute otitis media
iv. Hearing loss

b. Non-specific symptoms are ( i.e occurring in other entities)

 i. Irritability
ii. Ear pulling
iii. Diarrhoea & vomiting
iv. Excessive crying
v. Feeding problems

N.B
If the otorrhoea is > 14 days, it is chronic otitis media, but you don’t need to
wait for otorrhoea to make the diagnosis, it should be made early because otorrhoea
shows that the ear drum has ruptured.

When associated with ear discharge, it is called acute Suppurative otitis

media.

Signs
- Carry out a proper examination paying attention to the fever

i. Immobile red (not pink) tympanic memebrane (done with pneumatic

otoscope)
ii. Serous or purulent fluid in the middle ear, this is seen as air-fluid level
behind the eardrum)
iii. If Advanced stage, there is perforation of the eardrum

Laboratory Investigations
- Is usually not necessarily if the patient presents early

1. Ear discharge swab for m/c/s

2. Sepsis screen in those who are toxic
3. Tympanocentesis – if the membrane has not ruptured

Complications :
“HMP LAM”
1. Hearing loss
2. Mastoiditis (Mastoid bone is tender & swollen)
3. Petrositis
4. Labyrinthitis
5. Meningitis
6. Febrile convulsion
7. Abscess
a. Eustachian abscess
b. Subdural abscess
c. Brain abscess
N.B
Standard Sepsis Neonatal Screen
 i. Full blood count
ii. Blood culture
iii. Urine culture
ii. Chest x-ray
iii. Lumbar puncture for CSF m/c/s

Other tests
i. Umbilical swab
ii. Rectal swab

Treatment

1. Specific
Antibiotic – Sspecific for the organism in each group
a. Neonate : Cefuroxime + Gentamicin (Gentamicin has now replaced
cloxacillin)

b. Older Children : Co-amoxiclav (Amoxicillin-clavulanate - Augmentin)

Co-trimoxazole (Trimethoprim-Sulphamethoxazole)
IM Procaine penicillin

2. Supportive Management
a. Antipyretic
b. Analgesic for the otalgia - paracetamol is used for both
c. Aural toilette – using rolled tissue paper or gauze
d. Counselling – If the AOM is frequent, advise against swimming or
preferably using ear plugs when swimming.
- Advise the mother to prevent water entering ear when
bathing the child.
 III.
CHRONIC OTITIS MEDIA

Definition
- Is perforated, painless ear discharge with almost always immobile tympanic
membrane.
- Note, the discharge must be > 14 days. If less, it is Acute Suppurative otitis media.

Investigation
1. Ear discharge swab for m/c/s
2. X-ray of the mastoid bone
3. Audiometry
4. Tympanometry
5. Mycology

Complications
1. Cholesteatoma – foul smelling whitish discharge seen through the perforation
2. Mastoiditis
3. Otogenic tetanus
4. Meningitis
5. Facial nerve palsy
6. Lateral sinus thrombois
7. Abscess – brain, subdural, & Eustachian

Treatment
1. Aural toilette
– very important
– the use of systemic antibiotic is controversial, but in case of acute
exacerbation of chronic otitis media, antibiotics will be needed.
– Another controversy is whether to use dry or wet dressing.
– Do not use gentamicin ear drop if wet dressing is chosen.

2. Flavine in spirit dressing

 IV.
ACUTE TONSILLOPHARYNGITIS

Definition
Is acute inflammation of tonsils & pharynx

Types
There are 3 main types differentiated by clinical appearance and causative organism

1. Exudative ATP /Follicular tonsillitis

2. ATP with membranous/Diphtheritic pharyngitis or tonsillopharyngitis
3. ATP with vesicles or ulcers - Vesicular or ulcerative pharyngitis

A. EXUDATIVE/FOLLICULAR TONSILLITIS

Aetiology

- Group A, B-hemolytic streptococcus

Symptoms

Younger children Older Children

. Fever . Fever
. Nausea & vomiting . Sorethroat
. Abdominal pain . Headache
. Malaise

Signs
• Pyrexia - usually > 38 C
• Exudative tonsillar enlargement – the exudates which are follicular in distribution
suggest the diagnosis

• The tonsils are reddened/erythematous & oedematous

• Lymphoid hyperplasia of the pharynx
• Anterior cervical lymphadenitis
Diagnosis
1. Throat swab for m/c/s
2. Rapid latex agglutination test – is very fast and is specific (i.e has few false
+ve result) but has low sensitivity.
3. Blood culture – is done if the patient is very ill or septicaemic
4. Full blood count – look for polymorphonuclear leucocytosis

Treatment

a. Specific
1. Antibiotic : The options are
a. Penicillin V (oral), 250mg for 10 days - drug compliance is very
difficult with it.

b. Single IM injection of Benzathine penicillin G or Procaine penicillin

– the complication to watch out for is allergy.

c. Erythromycin, 30 – 50mg/Kg/day in 4-divided doses for 10 days.

d. Amoxicillin or amoxiclav + potassium coagul - is used nowadays

b. Supportive
i. Analgesic
ii. Antipyretic
iii. Adequate fluid & calorie intake

Complications
- grouped into suppurative or delayed non-suppurative

a. Suppurative
i. Acute otitis media
ii. Acute Sinusitis
iii. Peritonsillar cellulitis or abscess
iv. Retropharyngeal abscess
v. Suppurative cervical lymphadenitis

b. Delayed Non-suppurative
i. Acute rheumatic fever
ii. Acute glomerulonephritis
B. ACUTE TONSILLOPHARYNGITIS WITH MEMBRANE

Aetiology
Corynebacterium diphtheriae

Symptoms
- As above – fever, sorethroat
- In addition, neck swelling which can be so big and is called Bull’s neck

Signs
• Enlarged tonsils with grayish white membrane covering it and is usually very
adherent to the tonsil. It extends to the fauces.

Diagnosis
- Is clinically made

Investigation
1. Throat swab
2. Full blood count – presence of polymorphonuclear leucocytosis
3. Shick test – is positive when the induration is > 10mm

Treatment

a. Specific
a. Penicillin – is the drug of choice
b. Diphtheritic antitoxins – to mop up the toxins of corynebacterium diphtheriae
c. Erythromycin – if penicillin is contra-indicated i.e allergy

b. Supportive
i. Bed rest
ii. Analgesic
iii. Antipyretic
iv. Adequate fluid and calorie intake

Complications
1. Myocarditis
2. Neuritis

C. ACUTE TONSILLOPHARYNGITIS WITH VESICLES or ULCERS

Aetiology
Coxsackie virus type A
Symptoms
- As above : Fever, sorethroat, dysphagia

Signs
• Enlarged inflammed tonsils
• Presence of ulcers or vesicles on the tonsil (instead of exudates or grayish white
membrane) is diagnostic.

Investigations
1. Throat swab for virus isolation, specifically for Group A coxsackie virus

2. Blood specimen for antibody titre measurement - the blood is centrifuged to

obtain the serum. After 14 days, another sample is taken to check for titre
elevation. The diagnosis is made if it is 4 times or above. (The 1st sample is taken
during the acute phase of the illness and 2nd sample during the convalescent phase,
by which the patient would have been well.

3. Blood culture – for secondary bacterial infection

Complications
1. Parotitis
2. Conjunctivitis
Treatment
a. Supportive - as above
b. Specific – appropriate antibiotics.
 Chapter 2 :
LOWER RESPIRATORY TRACT INFECTIONS

I. Acute Pneumonia
II. Pleural Effusion
III. Bronchiectasis
IV. Lung abscess

I.
ACUTE PNEUMONIA

Introduction
Community-acquired Pneumonia is the type acquired within the community
(different from Nosocomial Pneumonia which has different aetiological agent)

Definition
Acute pneumonia is the acute inflammation of lung parenchyma caused by micro-
organism (as different from aspiration pneumonia which is just acute pneumonitis, but if
there is secondary infection, it becomes acute pneumonia.)

Epidemiology
a. Incidence - the burden is on the under 5-year old children
b. Mortality – 1 in 5 deaths among the under 5-year old

c. Risk Factors – very many

1. Low birth weight
2. Infancy
3. Outdoor air pollution
4. Indoor air pollution – from combustion of biomass fuel e.g cooking gas,
kerosene, sawdust or wood. The best way to cook is to use electricity.
5. Cigarette smoke
6. Overcrowding
- due to poor housing
- large family size a family of ≥ 7including father & mother
7. Poor malnutrition
- Non breastfeeding (for the first 6 months)
- Vitamin deficiency
- Protein-Energy malnutririon
8. Human immunodeficiency virus
 - The pneumonia is usually unchanged in children with HIV
infection
- In symptomatic HIV-infected children, the incidence and severity
of bacterial infection is increased.

The important 4 risk factors are[LIMN]

1. Malnutrition
2. Indoor air pollution
3. Low birth weight
4. Non-breastfeeding

Aetiology

a. Bacteria
i. Streptococcus pneumoniae
ii. Haemophilus influenza (type B is more out of the A to F subtypes, it is
written as Hib)
iii. Staphylococcus aureus
iv. B-hemolytic streptococcus
v. Escherichia coli
vi. Klebsiella species
vii. Proteus mirabilis
viii. Pseudomonas aeruginosa
N.B
In developing countries, streptococcus pneumonia is the commonest, followed
by Hib, both are responsible for 57% cases in children. With staphylococcus aureus, it
increases to 71% cases.
In Ibadan, S. aureus & Kleb. Pneumoniae are the commonest causes from
available data.

b. Viruses
i. Measles virus –especially where vaccination is not done
ii. Respiratory syncytial virus
iii. Adenovirus
iv. Parainfleunza
v. Influenza A & B
vi. Herpes simplex type I – especially in malnourished children.

c. Non-viral, non-bacterial
i. Mycoplasma pneumoniae – in children of school age i.e ≥ 5 years (Pre-
school – S.pneumoniae & H. influenza)
ii. Ureaplasma urealyticum
iii. Chlamydia

d. Protozoal
 i. Pneumocystis carinii

e. Fungi
i. Candida
ii. Aspergillus
iii. Histoplasma

Clinical Presentation
- are diverse

Symptoms
General
i. Fever
ii. Chills
iii. Headache
iv. Irritability
v. Vomiting
vi. Diarrhoea

Specific
a. Pulmonary
- cough
- fast breathing
- diffilcult breathing
b. Pleural
- chest pain
c. Extrapulmonary
- pustules or abscess on the body

Signs
i. Pyrexia, usually ≥ 38 C
ii. Tachypnoea
iii. Indrawings of the chest wall
iv. Flaring of alai nasi
v. On auscultation
• crackles/crepitations - this suggests bronchopneumonia
• bronchial breath sound – this suggests lobar pneumonia

Investigation
1. Radiology
2. Blood culture
3. Full blood count
4. Rapid Antigen test
5. Lung aspirate – is mainly for research purposes and for severely ill patients
 1. Radiology
- Radiology helps to confirm the diagnosis
- Frontal projection which could be antero-posterior or postero-anterior
depending on
- Lateral projection – to check for effusion.
- Patchy infiltrate points to bronchopneumonia while lobar consolidation
suggests lobar pneumonia.
- There may be hilar opacity. Note, other causes of hilar opacity are tuberculosis,
Lymphoma.
- Pneumatocoele (overdistended alveoli) may be present. It indicates necrotizing
pneumonia.

Causes of Necrotising pneumonia

i. Staph. Aureus
ii. Escherichia coli
iii. Klebsiella
iv. Pseudomonas

N.B
• The presence of abscesses (in infant) and pneumatocoele suggest S. aureus until
proven otherwise.
• If there is no pneumatocoele, no abscess, but the pneumonia is associated with acute
otitis media, the common agents are Strep. Pneumoniae & Haemophilus influenzae
• If associated pleural effusion, assume it is S. aurues, though S. pneumoniae & H.
influenzae can also cause it.

2. Blood culture
- It is +ve in about 10 – 30% of pneumonia cases
- It is usually done in severe pneumoniae

3. Full blood count

- polymorphonuclear leucocytosis points to bacterial pneumonia

4. Rapid Antigen test

- Is done for S. pneumoniae
- Has a disadvantage of being false +ve in most cases, but any fluid can be used.

5. Lung Aspiration
- It is highly sensitive and specific
- The indications for it are
a. severly ill patient
b. immunocompromised who are likely to have unusual organism
c. for research purposes
- There must be expertise and good microbiology laboratory.
 - The lung juice is aspirated

Complications
a. Acute complications : “HEAR 4Ps”
1. Heart failure
2. Empyema
3. Atelectasis
4. Respiratory failure
5. Pneumatocoele
6. Pneumothorax
7. Pyopneumothorax
8. Pleural effusion
9. Septicaemia
10. Subcutaneous emphysema – is rare, occurs particularly in measles
pneumonia.

b. Chronic complications
1. Lung abscess
2. Bronchiectasis

N.B
- Pneumothorax ± Pneumatocoele usually points to Necrotising pneumonia
(including Proteus mirabilis)
- Pleural effusion is a radiological diagnosis while emphysema is made on
thoracocentesis.

Treatment

a. Specific
– involves the use of ANTIBIOTICS
– The choice of antibiotic depends on
a. aetiological agent
b. antibiotic sensitivity pattern
c. cost of antibiotics
d. nutritional status
 An Algorithm of Treatment of Pneumonia

Nutritonal status

Well-nourished Severe Malnutirtion

Non-severe Severe Cefuroxime + Gentamicin for 3 -5weeks

(Zinacet)
Ceftriaxone/Ceftotaxime – for Gram –ve
Organisms like Klebsiella sp., Proteus sp.
Cotrimoxazole (TMP/SMX) _________ ↕
Procaine penicillin or Ampicillin No Yes
Cephalosporin
+
Gentamicin

Yes No Crystalline
Penicillin X 48 hours

Continue for Yes No

5 days

Discharge on Chloramphenicol+
Amoxicllin Erythromycin
For 5 days

Yes No

Continue for 7 days

Information from the flow chart above

a. In Non-severe pneumonia,
- the features present are cough, tachpnoea, but no lower chest wall
indrawings.
- Other types of indrawings are
i. Intercostal
ii. Suprasternal
iii. Supraclavicular
iv. Subcostal - suggest lower airway obstruction first especially
bronchiolitis.

- It constitutes a large group

- There is no need to investigate or for admission

- Cut offs for Tachypnoea

Age Respiratory rate

< 1 week ≥ 65
1 week – 2 months ≥ 60
2 months – 1 year ≥ 50
1 year – 5 years ≥ 40

- Precautions when counting respiratory rate

i. count when the patient is awake and calm or asleep, but not when the
patient is agitated.

b. In Severe pneumonia,
- the features are
i. central cyanosis
ii. Feeding difficulty
iii. Lower chest wall indrawings
- Check if it is necrotizing pneumonia : pleural effusion is a feature. Staphylococcal
pneumonia is very roaring.
- In management, all cases must be admitted.
- The temperature falls by “lysis”

-
b. Supportive
i. Supplemental oxygen – to relieve hypoxia
The indications are
a. central cyanosis
b. severe lower chest wall indrawings
c. transcutaneous Hb oxygen concentration(SaO2) of <90%
In the absence of pulse oximeter, the following clinical signs of
hypoxaemia may be used:
• Tachypnoea of 20 breaths/min above the age specific cutt off point
• Restlessness – not due to CNS disease e.g meningitis
• Titubation
• Tachycardia
• Grunting

Methods of Giving Oxygen

i. Funnel
ii. Nasal prongs
iii. Nasal catheter - is convenient

ii. Adequate Calorie intake – important,if the patient is so ill as to have no appetite,
pass nasogastric tube and give small frequent feeds. If this not possible set up an
intravenous line to give dextrose saline.

iii. Fluid management

– Restrict fluid intake to 3/4th – 2/3rd of maintenance fluid because
pneumonia is one of the causes of Syndrome of Inappropiate ADH
secretion (SIADH)
– If there is dehydration, calculate the deficit and add to the 3/4th of
maintenance.
– If the dehydration is severe, calculate the deficit, add to 2/3rd of
maintenance

iv. Placement of chest tube – to drain effusion or pneumothorax

v. Digitalisation – if there is heart failure. Give diuretics & digoxin.
- It is very rare, occurs if acute pneumonia is not treated
especially if associated hypoxaemia.
Follow-up
o Inform the mother to bring the child back immediately if the clinical signs of
pneumonia reappear or if the sickness appears to be more.
o If neither of the above occurs, tell her to come back after 2 days.

Responses expected
i. Temperature – should fall within 24 – 48 hours
ii. Pulse rate – should fall as well
iii. Respiratory rate – should fall as well

II.
PLEURAL EFFUSION

Definition
- It is excessive fluid accumulation in the pleural cavity
- It is a radiological diagnosis

Classification

1. Pathological

Transudative Exudative

1. Total Protein < 3g/dl ≥ 3g/dl

2. Pleural protein/Serum < 0.5 ≥ 0.5

protein
3. Total Leucocytes < 2000/mm³, ≥ 2000/mm³, predominantly
predominantly mononuclear polymorphonuclear cells except in
Tuberculous effusion & malignancy
which have higher % of mononuclear
cells
4. Specific gravity < 1.012 > 1.012

2. Clinical types of effusion (thoracocentesis)

i. Empyema thoracis – purulent
ii. Haemothorax - bloody
iii. Serous - thin fluid
iv. Chylothorax - chylous or milky
Aetiology

1. Bacterial
o Staphylococcus aureus - is the commonest cause in our
environment
o Streptococcus pneumoniae
o Haemophilus influenzae
o Mycobacterium tuberculosis
o Streptococcus pyogenes etc.

2. Viral
3. Malignancies
4. Nephrotic Syndrome
5. Heart failure
6. Liver cirrhosis

Clinical Presentation
Symptoms
- Occurs if the effusion is large
- Cough
- Chest pain
- Difficult breathing

Signs
- tachypnoea
- Indrawings
- Cyanosis
- If large enough, it causes
o Contra-lateral shift of mediastinum
o Stony dull percussion note
o Reduced or absent breath sound

Investigation

1. Radiology (Chest X-ray)

- The feature is uniform dense opacity of part or whole lung field. It is also
seen in lobar pneumonia
- No lung markings
- Loss of costophrenic angle (except if the film is taken in decubitus/supine
position)
 2. Pleurocentesis (Diagnostic tap)
- the nature of the fluid may suggest the aetiological agent
a. Straw-coloured - Tuberculosis

b. Bloody - Malignancy
- Tuberculosis
- Trauma – from the aspirating needle

c. Strictly purulent – Staph. Aureus

Strep. Pneumoniae
H. influenzae

d. Thinly purulent (specks or spots) - Streptococcus pyogenes

e. Clear fluid (Transudate) - Nephrotic syndrome

Liver Cirrhosis

f. Milky - Lymphatic obstruction (which sometimes might be due to

tuberculosis )
3. Blood culture
- m/c/s for pyogenic organism
- ZN stain for acid fast bacilli
- Culture in Lowenstein-Jensen medium – acid fast bacilli

4. Cytology
- Mesothelial cells suggest tuberculosis
- Neutrophil predominance – suggests bacterial cause
- Lymphocytosis - Tuberculosis or malignancy

5. Mantoux test
If positive, it ONLY suggests tuberculosis

6. Pleural biopsy
- To detect tuberculosis or malignancy

Complications

a. Pulmonary complications
i. Emphysema necessitans – bulge formation on the skin surface. Its treatment
is drainage.
ii. Pockets of loculated pus – formed from the deposition of fibrin. It is
difficult to drain
iii. Bronchopleural fistula - the fluid dissects the lung tissue into bronchial
tree. Can cause coughing out pus.
 b. Cardiac
i. Purulent pericarditis

d. Skeletal
- Rib osteomyelitis

e. CNS
- Meningitis

f. Haematological
- Anaemia
- Septicaemia

Treatment

Supportive
- Depends on the amount of accumulated fluid. It can be

a. Small or lamella pleural effusion -- Requires no need of treatment

b. Large - Put CTTD

c. Thinly purulent - Aspirate it using syringe and a 3-way tap

d. Tuberculous & Malignant effusion - Aspirate it with CTTD only if it is

associated with dyspnoea

Note – If the drainage is rapid, it causes Re-expansion pulmonary oedema in which patient
becomes more dyspneoic, cyanosed and in shock

Definitive
a. For Staph. aureus - cefuroxime + gentamicin, if it fails, move to higher
cephalosporins
b. Other pyogenic organisms - Chloramphenicol + erythromycin

c. Tuberculous - see under pulmonary tuberculosis

d. Malignancy - Cytotoxic drugs

 III.
BRONCHIECTASIS

Definition
Is defined as irreversible abnormal dilatation of bronchial tree

Incidence
Is common in the developing countries

Pathogenesis
- I s multifactorial
- is classified into 4 groups of mechanistic theories

a. Pressure of secretion theory

b. Atelectasis theory
c. Traction thoery
d. Infections theory

- In most cases, it usually due to combinations of the above factors.

- In most instances, it follows this order

Bronchial obstruction → Retention of secretion→ Infection

Classifcation
- 3 groups are listed in order of increasing severity

I. Cylindrical bronchietasis
- The diameter of each part of the bronchial is increased, but the normal pattern (the
regular outline i.e the normal arrangement of decreasing diameters of the
bronchial tree) is still maintained.

II. Varicose bronchiectasis

- there are local constrictions
- irregularity of the structural outline, resembling varicose ceins

III. Cystic / Saccular bronchiectasis

- the bronchial dilatation increases progressively towards the periphery
Aetiology
- Very many

a. Infections
- is the most common
- is very common in the developing countries
i. Tuberculosis
ii. Pneumonia – especially the type complicating measles and pertussis
iii. Adenoviral infections
iv. Mycoplasma pneumoniae

b. Congenital & Genetic disorders

i. Cystic fibrosis – common in Europe & America
- Has not been reported among pure Negroes

ii. Marfan’s syndrome – is a connective tissue disease

iii. Alpha -1-antitrypsin deficiency

c. Ciliary Abnormalities
i. Congenital – as in Kartagener’s syndrome which consists of
o Bronchiectasis
o Situs invertus
o Chronic sinusitis

ii. Acquired – due to damage to cilia

d. Foreign body aspiration

e. Asthma
f. Others
i. Chronic sinusitis
ii. Recurrent aspiration e.g in Tracheo-oesophageal fistula, Cerebral palsy
iii. Racial predilection - Australian aborigenes,Eskimos, Polynesians

Clinical Presentation
Age – mainly preschool & early school age
Symptoms
i. Chronic cough
ii. Purulent foul smelling sputum – is not seen in all cases, there could be
dry bronchiectasis
iii. Halitosis
iv. Haemoptysis
v. Others
o Weight loss
 o Intermittent fever
o Wheezing
o Dyspnoea
o Chest pain

Signs
i. Harrison’s sulcus
- is sulcus along the insertion of diaphragm
- is due to chronic cough and dyspnoea
- there is indrawing because the ribs are not yet calcified i.e
rigid.
ii. Finger clubbing
iii. Central cyanosis
iv. Dullness to percussion
v. Crackles/crepitations
vi. Bronchial breath sounds

Note – the lower 3 features are usually localized except in diffuse cases

Diagnosis
1. Chest X-ray
- the presence of air-fluid levels suggests it, but not diagnostic
- Other features depend on severity of the disease
a. If mild – there would be segmental accentuation and loss of lung
markings (this is diffilcult to observe)
b. Severe - it would show honey-comb appearance of cystic changes

2. Flexible fibreoptic bronchoscopy – is useful in children and is diagnostic

3. Bronchography – is also diagnostic. It is done when if surgery is imminent

4. CT scan

To identify the cause

5. Mycobacterial analysis
6. Broncholavage fluid/ sputum for m/c/s
7. Skin tests for tuberculosis – using PPD (Mantoux test)
8. Skin test for fungal infection
 9. Serological studies
10. Viral culture
11. Sweat test – for cystic fibrosis
12. Barium swallow – to check for leakage of contrast into respiratory tract
13. Fungal culture

Complications
1. Brain abscess
2. Lung abscess
3. Empyema
4. Pyopneumothorax
5. Bronchopleural fistula
6. Severe atypical pneumonia
7. Haemopytsis
8. Amyloidosis
9. Cor pulmonale – occurs in advanced disease. It is the most terrible complication.
- Is defined as heart disease resulting from chronic lung
infections.

Treatment

a. Medical
i. Chest physiotherapy – involves percussion of the chest to encourage
expectoration of secretion
ii. Antibiotics
iii. Good nutrition
iv. Bronchoscopy (rigid) – to remove foreign body or secretions
v. Bronchodilator
b. Surgical
Is indicated when
a. there is failure of medical treatment
b. the disease is localized

Prognosis
- Depends on causative factor
- It is best in Group I type
 IV.
LUNG ABSCESS

Definition
Is localized area of suppuration due to destruction of lung parenchyma.

Pathogenesis
- Begins as inflammation, then to central necrosis
- Initially, the enclosing wall is poorly defined, but as it progresses, it becomes more
discrete

Classification
a. Primary – no preceding predisposing factors e.g unconciousness, cerebral palsy,
meningitis
b. Secondary – there is a predisposing factor

Aetiology
i. Staphylococcus aureus – is the commonest
ii. Anaerobes – occurs exclusively in secondary abscess
iii. Others
a. Tuberculosis
b. Mycoplasma pneumoniae
c. Aerobes

Clinical Presentation
- Onset is generally insidious

Symptoms
a. General
i. Fever – is often high grade (40C) and is almost always present
ii. Malaise
iii. Weight loss – is common
iv. Vomiting – is uncommon

b. Respiratory
i. Cough – usually chronic, productive of foul smelling sputum
ii. Chest pain
iii. Dyspnoea
iv. Haemoptysis
v. Putrid breath odour in secondary lung abscess
 Signs
Respiratory
i. may be absent
ii. tachypnoea
iii. chest wall indrawings
iv. reduced chest movement
v. dullness to percussion
vi. reduced breath sound
vii. bronchial breath sound
viii. fine crackles

Cardiovascular
i. Tachycardia

Investigations
1. Chest X-ray
- very useful and is diagnostic
- thick walled cavity [different from pneumatocoele (overdistension of
alveoli) which is thin walled]
- air-fluid level is often present except if the cavity is full

2. Full blood count - polymorphonuclear leucocytosis may be present

3. ESR – may be raised

4. Blood culture – occasionally +ve in secondary lung abscess

Note – In secondary lung abscess, the abscess cavity is usually multiple

Complications
1. Overexpansion of the abscess cavity
2. Spontaneous rupture with seeding to other parts of the lung
3. Tension pneumothorax
4. Empyema

Management
1. Antibiotics
Anti-staphylococcus agent
Metronidazole – for anaerobes
2. Physiotherapy – for postural drainage
3. Surgical
The options are
i. Minaldi’s procedure/Pneumonostmy
- Take X-ray to localize the position of the abscess
Anteroposterior – shows if it is high or low
Lateral – shows if it is anterior or posterior
Oblique (sometimes done) – localize the actual position

-Pass tube via the skin & pleura to the parenchyma to drain the
abscess out.
ii. Pneumonectomy or Lobectomy

Complication
1. Overexpansion of abscess cavity
2.Rupture to cause empyema and tension pnuemothorax
3.Rupture to cause seeding to other parts causin g multiple lung abscess
Differential diagnosis
1.Localized pyoneumothorax
2.hyatid cyst
3.neoplasm
4.plasma cell granuloma
5. infected congenital cysts and sequestrations
 Chapter 3 :
OBSTRUCTIVE AIRWAY DISORDERS

Anatomy
Nose → Nasopharynx → Oropharynx → Larynx → Trachea → Bronchi →
Bronchioles → Alveolar sacs

Introduction
Diseases that cause obstruction at various parts of the respiratory tract are divided
into two:

a. Upper Airway obstruction / Extrathoracic

• affect mainly the inspiratory phase
• its main feature is STRIDOR (Harsh sound)

b. Lower Airway obstruction / Intrathoracic

• affect mainly the expiratory phase
• its main feature is WHEEZE

Causes of Upper Airway Obstruction

1. Congenital – The common ones are

i. Laryngomalacia – is the commonest in this group
ii. Laryngeal web – is the 2nd commonest
iii. Subglottic stenosis
iv. Laryngocoele/Laryngeal cyst
v. Tracheo-oesophageal fistula
vi. Craniofacial anomalies

2. Acquired
a. Infections – most important
i. Laryngotracheobronchitis (LTB) – is the commonest
ii. Epiglottitis – not very common
iii. Retropharygeal abscess
iv. Peritonsillar abscess
v. Bacterial tracheitis
vi. Diphtheria

b. Burns
i. Thermal
ii. Chemical
c. Foreign body aspiration
 d. Trauma – to upper part of the face or chest
e. Allergy – spasmodic laryngitis
f. Neoplasm

Causes of Lower Airway Obstruction

i. Bronchiolitis
ii. Bronchial Asthma

LARYNGOMALCIA
(Congenital Laryngeal Stridor)
Introduction
• Is a relatively benign condition due to minor congenital abnormality of the
larynx.
• Is the commonest cause of congenital obstruction of upper airway

Clinical Presntation

• Age - Is usually at birth or during the first week of life

• Noisy breathing or stridor
• Weak cry
• Respiratory distress (tachypnoea & chest wall indrawings) especially during
feeding
• Symptoms may worsen when patient lies supine
• Chest retraction during inspiration (suprasternal & supraclavicular)
• Chest deformity

Note : all the signs are exaggerated during feeding

Diagnosis
1. Chest X-ray – to exclude the differential diagnosis
2. Lateral Neck X-ray – for the above reason
3. Direct Laryngoscopy – is diagnostic

Treatment
• Is self-limiting, therefore specific measure is usually not necessary.
• Reassure the parents
• Symptoms subside by the age of 12 – 18 months
• Keep the child more in prone position
• Advise to feed the child slowly
 LARYNGEAL WEB

Introduction
• is due to presence of a web in the anterior portion of the larynx
• Is less common than laryngomalacia

Pathology
- The obstruction is usually incomplete ( Obviously, if it is complete, the child would
not survive.
• Usually presents at birth

Clinical Presentation
• Similar to laryngomalacia
• Noisy breathing or stridor
• Weak hoarse cry
• Respiratory distress

Diagnosis
Direct laryngoscopy – is diagnostic

Treatment

The options are

i. Incision or excision of the web followed by repeated dilatation using a
laryngeal dilators
ii. Lysis of the web with CO2 laser

N.B
CROUP
- Is not synonymous with acute laryngotracheobronchitis
- Is a generic term encompassing a heterogenous group of relatively acute conditions
(mostly infections) characteristic by a brassy or barking cough accompanied by
stridor.

• ± hoarseness of voice
• Signs of respiratory distress
 LARYNGOTRACHEOBRONCHITIS
(Viral Croup)

Introduction
- Is the commonest form of croup

Incidence
• Occurs commonly in children aged 6 months – 3years

Aetiology
It is primarily caused by viruses
i. Measles virus – common and is dependent on location & seasons
ii. Parainfluenza
iii. Adenovirus
iv. Respiratory sycytial virus (RSV)

Clinical Presntation
• Is usually preceded by upper respiratory tract infection
Symptoms
• Fever
• Barking cough
• Stridor – is the hall mark
• Dyspnoea
• Tachypnoea
• Chest retraction
• Increased effort during inspiration
• Anxiety or restlessness which may progress to fatigue
• Reduced breath sounds
• Inspiratory rhonchi
• Scattered crepitations

Croup Score –“SC BRC”

0 1 2

1. Stridor None Inspiration Inspiration &

Expiration
2. Cough None Hoarse Barking

3. Breath sound Normal Reduced Markedly reduced

4. Evidence of Respiratory None - Nasal flaring, Suprasternal,

distress - suprasternal retraction Subcostal, &
Intercostals retractions
5. Colour Normal Cyanosis in room air Cyanosis in 40% O2

Grades
Total score = 10
0 – 6 = Moderate illness, the patient should be nursed in ward

7 – 10 = Severe illness, the patient should be admitted to intensive care unit.

Diagnosis
Is usually clinical

Investigations
1. Full blood count – leucocytosis with lymphocyte predominance (because it is a
viral infection)

2. Chest X-ray
3. Lateral radiograph of the neck
- its characteristic feature is subglottic narrowing

Differential diagnosis
1. Laryngeal diphtheria – there is presence of grayish-whitish membane

2. Epiglottis
- is the most important
- has sudden onset
- diagnosed by lateral radiograph of the neck

3. Foreign body in the larynx

- good history would point it out
- diagnosed by lateral radiograph of the neck: shows radio-opaque image. If
not, do bronchoscopy

4 Retropharyngeal abscess
- diagnosed by lateral radiograph of the neck : shows widening of
retropharyngeal space due to the abscess collection.

N.B
Q. Name the conditions that can be disgnosed by lateral radiograph of the neck.
Treatment
1. Humidified Oxygen
- is the mainstay
- is oxygen with water vapour
- Oxygen is passed through ice cubes
- The water vapour cools the respiratory tract and dissolves the thick
secretions

2. Adequate fluid & Calorie intake

3. Give mild sedative e. g chloral hydrate if the patient is restless

4. Antibiotics
- Is not routinely indicated, it is used if there is evidence of superimposed
bacterial infection (fever, toxaemia, leucocytosis with polymorphs
predominance)

5. Racemic epinephrine
• Given by inhalation
• it provides rapid but transient relief
6. Steroid – using dexamethasone or hydrocortisone

7. Tracheostomy – if respiratory obstruction progresses.

Complications

1. Pneumonia
2. Congestive heart failure
3. Pulmonary oedema

Ways of Oxygen delivery

1. Mask – not very effective especially if the child is restless
2. Nasal prong – fairly effective
3. Intranasal catheter
4. Pupette or Oxygen tent – it makes it difficult to observe patient
 EPIGLOTTITIS
(Bacterial Croup)

Introduction
o Is a severe, life-threatening, rapidly progressive infection of the epiglottis.

Aetiology
Haemophilus influenzae type B (Hib) - is the usual cause

Incidence
• Is less common here
• The age is between 3 to 7 years

Clinical Presentation
• The onset is very sudden
• High fever
• Aphonia
• Drooling saliva – because the patient is afraid to swallow
• Swollen inflammed epiglottis

N.B
Attempt to visualize the epiglottis by depressing the tongue may cause reflex
laryngeal spasm precipitating total airway obstruction, hence, when epiglottitis is
suspected, throat examination should not be done unless there are facilities for endotracheal
intubation.

Diagnosis
• It is usually clinical (like LTB)

Investigations
1. Full blood count – Leucocytosis with predominance of polymorphs
2. Lateral radiograph of the neck
• indicates supraglottic swelling i.e swelling of the epiglottis

Treatment

1. Antibiotics
• Ampicillin – not really effective in our environment now
• Chloramphenicol
• Cephalosporin – if there is resistance to the above drugs

2. Humidified oxygen
 3. Intravenous fluid
4. Tracheostomy – is indicated if there is increase in respiratory osbtruction

Differences between
Laryngotracheobronchitis Epiglottitis

1. Usually preceded by URTI 1. The onset is very abrupt

2. It is viral in origin 2. It is bacterial
3. Age incidence is 6 months -3 years 3. It is 3 years – 7 years

ACUTE BRONCHIOLITIS

Introduction
Is inflammation of the bronchioles, usually associated with obstruction

Epidemiology
• Occurs during the first 2years of life
• It is commoner in males, male: female = 2 : 1
• The incidence is higher during the rainy season & cold harmattan ( &
Winter months)
• Incidence is higher among
a. Non-breastfed babies
b. Babies living in overcrowded environment
c. Babies who attend day care centres
d. Babies exposed to cigarette smoke

Aetiology
Is mainly viral
1. Respiratory syncytial virus – accounts for 75% of cases
2. Parainfluenza virus types 1,2, & 3
3. Adenovirus
4. Mycoplasma pneumonia (is an atypical organism i.e non-viral, non-
bacterial)

Pathophysiology
The following changes are observed
i. Oedema of the bronchiolar mucosa
ii. Accumulation of mucus & cellular debris
iii. Constriction of the bronchiolar muscles
 These changes result in
o Reduction in the size of the lumen of the bronchioles
o Resistance to flow of air during inspiration & expiration, but more marked
at expiration.
o If the obstruction is complete, it results into atelectasis
o But if it is incomplete, there is airtrapping & hyperinflation
o All these result give rise to impairment of normal gaseous exchange at
alveolar level causing hypoxaemia at first and hypercapnia later.

Clinical Presentation
- It usually begins with URTI

Symptoms & Signs

• Cough
• Fast breathing
• Fever
• Refusal of feeds & vomiting
• Fretfulness
• Wheezing
• Chest retraction
• Cyanosis
• Hyper-resonant percussion note
• Prolonged expiration
• Reduced breath sounds
• Fine crepitation
• Liver & Spleen are displaced downwards due to hyperinflation of the
lungs.
N.B
Liver in Congestive cardiac failure is tender

Investigations
1. Full blood count – Leucocytosis with lymphocyte predominance (viral picture)
2. Chest X-ray – shows patchy atelectasis with hyperinflation in some areas.
3. Viral identification
4. Blood Gas Analysis

Differential diagnosis

1. Bronchial asthma
- is uncommon < 1 year
- there may be positive family history
- History of recurrent attack
- History of allergy e.g allergic rhinitis, vernal conjunctivitis, eczema in
 flexural surface
- Blood & Sputum eosinophilia

2. Bacterial pneumonia

3. Congestive heart failure

- presence of tachypnoea, cardiomegaly
- palpable enlarged liver
- no major cough
- note, it is also a complication of brochiolitis

Treatment
1. Humified oxygen
2. Adequate fluid & Calorie intake
3. Antibiotics – Is not indicated except if there is evidence of secondary bacterial
infection. Give Cloxacillin & Gentamicin.
4. Sedative – Is contra-indicated but chloral hydrate can be given if the patient is
restless
5. Trial of bronchodilator by inhalation
6. Digoxin – if there is heart failure
7. Ribavirin – antiviral agent
8. Mechanical ventilation – if respiratory failure occurs

Indications
Clinical /Subjective
a. Worsening respiratory distress - as evidence by
i. Increasing respiratory rate
ii. Increasing tachycardia
iii. Increasing restlessness
iv. Cyanosis

Objective
i. PaO2 < 60mmHg
ii. PaCO2 > 45 mmHg
iii. pH = 7.25

N.B
Bronchodilators & Steroids have no role.

Prognosis
o Is generally good (though the patient is acutely ill)
o Mortality is < 1%
o Most critical period is usually the first 48 – 72 hours, after this, recovery is
usually rapid & complete within a few days.
 Features of Poor Prognosis
i. Low birth weight
ii. Secondary bacterial infection
iii. Underlying congenital heart disease

Sequelae
o A proportion have hyper-reactive airways and wheezy episodes, and may
develop bronchial bronchial asthma later in life.
 Chapter 4
TUBERCULOSIS

Introduction
- Is an important infectious disease globally.

Epidemiology
- About 30% of the world’s population are infected
- 8 – 10 million people develop the disease annually, out of which 3 million are in
Sub-Saharan Africa.
- There was a recent resurgence in pulmonary tuberculosis and the reasons for
this are highlighted below

i. Worsening economic situations

ii. Multi-drug resistance
iii. HIV pandemic
iv. Decline of National Tuberculosis Control Programmes
v. Large number of displaced persons from conflicts and wars are
now living in poor conditions.

Predisposing Factors
1. Age – is common in children < 5 years, those without immunization are more
susceptible.

2. Sex - No sexual difference, except around the adolescent age, in which it is

commoner among girls because of the increased pressure of menstruation which
is associated with reduced immunity.

3. Malnutrition

4. Concurrent infections e.g measles, pneumonia

5. Overcrowding & Poor living conditions

Pathology & Pathogenesis

- It is a chronic infectious disease which has 2 very important characteristics:

• Association with vague symptoms especially in children
• Has a protracted course
- The infectious agents are
• Mycobaterium tuberculosis
• M . bovis
• M . Africanus
- The Special characteristics of the organisms are as follows
• They are non-motile, non-spore forming
• Are acid fast bacilli – i.e not decolourised by acid after taking up ZN
stain.
• Are slow-growing
• Can remain dormant and still be alive for years, they can manifest if
there is immune suppression
• About the atypical organisms
i. About 40 have been described
ii. cause similar symptoms like M. tuberculosis
iii. their treatment is usually different
iv. they are not of public health significance

- The Source of infection is SPUTUM of persons with open tuberculosis

- The Mode of Spread is by:

i. Inhalation – via the droplets nuclei.
ii. Ingestion – of contaminated cow’s or unpasteurised milk or
contaminated food.
iii. penetration of skin and mucous membranes.

PRIMARY INFECTION
- Is infection in those who have been exposed to the organisms before.
- 98% of it occurs in the lungs

Primary Focus + Regional lymph node/Lymphatics = PRIMARY

COMPLEX/GHON’s COMPLEX

Primary Focus
- is the small area of inflammation where the organism lies
after inhalation.
- the organism is carried to the regional lymph nodes by histiocytes to form
caseous necrosis.
- can be one or multiple
- its size varies from few mm to 2 cm in diameter.
- the usual site is sub-pleural region.

Symptoms & Signs

i. May be symptomless
ii. May be associatedwith minor symptoms like malaise, fever
(mimicking malaria)

iii. Mucocutaneous manifestations of primary tubeculosis:

- are due to allergic reaction in the presence of tuberculin
protein
• Erythema nodosum
- reddish nodules seen in the forehead, inner side of
forearm
- can be missed in Blacks, but easily picked up in
Caucasians
• Phlyctenular conjunctivitis
- numerous yellowish nodules usually in the lateral
aspect of the eye.
- it is engorged by vessels
- is commoner in this environment
Natural Course
- the primary complex heals in 85% of population
- the risk of primary infection developing into active disease is
about 5 – 15% in the first 10 years after infection.

Ways of Progression of Primary Infection to Active Disease

1. Primary focus can spread to the contiguous part of the lung
giving rise to tuberculous pneumonia.

2. Primary focus & the regional lymph nodes may merge and
give rise to an area of consolidation

3. Extensive caseation & liquefaction can develop giving rise to

cavity formation.

4. The inflamed nodes may compress the neighbouring bronchi

giving rise to atelectasis or emphysema if it is total or partial
obstruction respectively.

5. Node may erode through the bronchial wall, causing

endobronchial tuberculosis (Note, there is no rupture of the
bronchial walls here)

6. There may be discharge of the tubercle bacilli into the

bronchial lumen leading to bronchogenic dissemination to
other areas of the lungs
 7. Nodes may erode into the blood vessels giving rise to
haematogenous spread to other tissue

8. The affected nodes may develop fibrosis & encapsulation

with viable tubercle bacilli persisting within the node for
many years & may be the source of re-activation tuberculosis,
called Post-primary infection.

Periods Between the Primary Infection & Appearance of

Clinical Evidence of various Forms of Tuberculosis

Duration from Primary Infection

1. Pulmonary tuberculosis Few months

2. Miliary & Meningeal Tb 2 – 6 months

3. Tb Adenitis 3 – 9 months

4. Bones & Joints Several years except Tb spine which is

earlier
5. Renal & Genital Tb Decade

6. Post-primay infection (Reactivation) A number of years

Section I.

PULMONARY TUBERCULOSIS

Introduction
- Is the commonest form of Tb
- It constitutes about 70% when it occurs alone and in combination with other
forms
- In children, it consists mainly of primary complex and its direct progression.

Pathology
• Hilar enlargement may lead to bronchial compression with resulting hyperinflation
or atelectasis
• Consolidation, patchy or lobar with or without pneuomothorax & pleural effusion.
 • Cavitation – is now common in children, though the incidence is still higher in
adults.

Clinical Presentation

Symptoms
i. Chronic cough – is cough > 3 weeks therefore when clerking, ask the
following:
- Duration of the cough
- Which time of the day is it worse? Night points to asthma
- Does it prevent the child from sleeping?
- Relieving or worsening factors
- History of immunization

ii. Fever – is usually high grade

iii. Anorexia
iv. Vomiting
v. Weight loss or Not gaining weight
vi. Haemoptysis
vii. Night sweats
viii. Overcrowding

Signs
i. Chest examination may reveal no abnormality
ii. Dyspneoa
iii. Tachypnoea
iv. Localised wheezing
v. Reduced breath sounds
vi. Crepitations
vii. Bronchial sounds

N.B
Clinical features of re-activation tuberculosis in older children are similar to those of
the primary infection, but its cough is usually productive and there may be chest pain from
pleural effusion.

Differential diagnosis
1. Pneumonia – Bacterial, Viral, Mycoplasma
– especially if the pneumonia is not responding to usual treatment

2. Lung abscess - can also be a complication of Tb

3. Bronchiectasis - can also be a complication of Tb

 4. Pulmonary Fungal infection – Do bronchial washout to examine for hyphae

5. Pulmonary neoplasm - though primary ones are not common, but secondaries do
occur.

Diagnosis – Made from

• Detailed history of current illness – cough, precipitating factors
• Past Medical history
• Family and Social History
- for predisposing factors
- 24 – hour dietary report
• History of contact
- in the child’s immediate environment : nuclear family, type
of apartment, school.

Investigations

1. Tuberculin skin test

- there are many forms, but 2 are of importance

a. Heaf test
- is a multiple puncture test
- usually used for screening i.e for a large number of people
- is less sensitive
- Procedure
• Put the heaf gun on a slit lamp to sterilize.
• Put the old tuberculin, which is a clear fluid, on the
skin on the flexor aspect of the forearm
• Place the plunger (has 6 needles) on the fluid over
the skin and plunge into the area
• Mark the the area with a pen and inform the
person not to wash it

- It is read after 48 – 72 hours

Grades

0 6 Un-indurated separate needle parts

1 6 Indurated, but separate
2 An indurated circle with unraised centre
3 The center is raised
4 Ulceration of the area
 0 1 2 3

Reading
• Grade 3 and 4 are regarded as POSITIVE. Though this does
not indicate that the person has the disease, but has come in contact with
the organism

b. Mantoux test
- Is for clinical diagnosis. It is very useful
- The solution used is Purified Protein Derivative (PPD).
- It is given intradermally using small needle and syringe
- The dose is 0.1 ml of PPD, this contains 10 tuberculin units
- A transparent ruler is used to read the diameter of the
indurated area.

Grades

Diameter, mm

0–4 Negative
5–9 Doubtful positive, repeat the test with
more concentrated solution of PPD,
which contains 15-20 tuberculin units
≥ 10 Positive

Positive Mantoux test

- It only indicates that the person has actually come in contact
with Mycobacterial organisms and may not necessarily have
the disease.
- It is not an indication to start treatment
- But, it calls for further investigation

Causes of False Negative Result

1. Severe overwhelming tuberculosis – which is associated with a high
degree of immune depression
2. Severe malnutrition
3. Measles or Pertussis in the immediate past
4. Steroid therapy
 5. Cytotoxic therapy
6. HIV/AIDS
7. Poor technique
8. Impotent/Expired reagent
N.B
- the last 2 reasons are not medical

Causes of False Positive Result

1. Poor technique – if given intramuscularly, instead of intradermal
2. Cross reaction – with other atypical mycobacteria.

2. Chest radiograph
- Is indicated in all forms of Tb, but very important in chest Tb
- The features that may be seen are
• Hilar/Paratracheal adenopathy – shown by
widening of the mediastinum
• Parenchymal lesions :
i. Patchy infiltrates
ii. Consolidation
iii. Atelectasis
iv. Pleural effusion
v. Cavities – probably show more severe
disease
vi. Pneumothorax

3. Bacteriological investigation
i. Sputum – is difficult to obtain in younger children
ii. Gastric washout – done because the patients swallow sputum
especially in the morning before waking up.
The specimen is stained with ZN stain & cultured in
Lowenstein-Jensen medium. Note, the organism is not easy to culture,
even in good laboratories, it is only 50% sensitive in children ( 80% in
adult)

4. Erythrocyte Sedimentation Rate

- Is not diagnostic of Tb
- Is usually high ( seen also in other chronic conditions)
- It is very useful to monitor treatment.

5. Full blood count

- Leucocytosis with predominance of lymphocytes
 Section II.

PLEURAL EFFUSION IN TUBERCULOSIS

Introduction
Pleural effusion occurs when
i. sub-pleural primary focus ruptures into the pleural cavity
ii. caseous node ruptures into the pleural cavity
iii. there is haematogenous spread
iv. and as a result of allergic response to tuberculin protein (just like in
mucocutaneous reactions : erythema nodosum & phlyctenular
conjunctivitis).

Clinical Features

Symptoms
Fever
Cough
Chest pain on deep inspiration

Signs
Dullness to percussion
Diminished or absent breath sounds

Investigation
1. Pleural tap
- yields serofibrinous fluid, sometimes it is blood-stained
- the protein content is high, 2- 4g/dl.
- High white cell count with predominance of lymphocytes
2. Full blood count
- leucocytosis with predominance of lymphocytes

3. Culture of the specimen – yields tubercle bacilli in < 20%

 Section III.

MILIARY TUBERCULOSIS

Introduction
- Is the most severe form of disseminated tuberculosis because of the immature
immunity. (Disseminated Tb is when the disease is found in at least 2 distinct
parts/systems e.g Respiratory, GIT).

Epidemiology
- common in 6 months – 4 years

Pathogensis
- It occurs 2 – 6 months after primary infection
- It occurs when there is haematogenous spread to different parts of the lungs & the
body (i.e seeding)

Clinical Manifestations
- Is variable, it depends on
i. Load of organisms
ii. Organs affected
iii. Immune status of the child
- The onset of symptoms may be explosive or insidious

Symptoms
i. Fever
ii. Anorexia
iii. Weight loss
iv. Cough
v. Wheezing

Signs
- Depends on organs invloved
i. Generalised lymphadenopathy
ii. Hepato-splenomenogaly
iii. Respiratory distress
iv. Signs of meningitis – seen in 20 – 40% patients
v. Signs of peritonitis
vi. Choroidal tubercles on fundoscopy
Investigations
1. Tuberculin skin test
2. Chest x-ray
- done because at least 70% of cases involve the lung
- it is diagnostic
- it shows multiple small rounded white reticulo-nodular opacities
scattered all over the two lungs

3. Cerebrospinal fluid tap

4. Histological examination – should be considered last, you only do this if others
failed.
a. Lymph node biopsy
b. Liver biopsy
c. Bone Marrow Aspiration / biopsy

Differential diagnosis of Miliary Picture on Chest X-ray

1. Sarcoidosis – is also a chronic granulomatous disease which tends to occur in
adults
2. Eosinophilic pneumonia
3. Pulmonary fungal infection e.g Aspergillosis
4. Chicken pox pneumonia
5. Childhood histiocytosis syndrome

Section IV.

TUBERCULOSIS OF THE CNS

It comprises of
a. Tuberculous meningitis
b. Tuberculoma

a. TUBERCULOUS MENINGITIS

Incidence
- Most common in children aged 6 months - 4 years

Pathogenesis
- Occurs about 2 – 6 months after the primary infection
- Arises as a result of haematoganous spread of tubercle bacilli to the cerebral cortex
and meninges.The caseous lesions rupture into subarachnoid space to cause meningitis.
Clinical Manifestation

- Can be divided into 3 stages

Stage I : Non-specific symptoms like

o Fever
o Headache
o Weight loss
o Irritability
o Drowsiness

Stage II : Evidence of Meningeal Irritation

o Lethargy
o Vomiting
o Nuchal rigidity
o Seizures
o Positive Kernig’s sign
o Signs of brainstem involvement – i.e Palsies of cranial nerves II, III,
VI, VII, VIII
o Other focal neurological signs

Stage III
o Hemiplegia or paraplegia
o Coma
o Decerebrate rigidity
o Opisthotonus
o Papilloedema & presence of choroidal tubercles on fundoscopy

Investigation
1. Tuberculin skin test
2. Chest X-ray
3. Examination of CSF – most diagnostic

CSF Picture In Tuberculous Meningitis

i. The fluid is straw-coloured, or clear & colourless
ii. White blood cell count : 10 – 500cells/mm³ with
predominance of lymphocytes
iii. Protein is very high, may be over 1g/dl (Normal is 10 – 40
mg/dl). Note, in partially-treated bacterial meningitis,
there is combination of lymphocytes & polymorphs)
iv. The glucose level is low, i.e < 40 mg/dl
v. ZN staining may yield acid fast bacilli
vi. Culture may be positive.
Prognosis
- Depends on the stage of the disease at commencement of therapy
Stage I - Good
Stage III – Mortality is high, even a high percentage of those who survive
develop complications listed below

Complications
1. Blindness
2. Deafness
3. Paraplegia
4. mental retardation
5. Speech disturbance
6. Cranial Nerve Palsies
7. Seizures
8. Hydrocephalus

b. TUBERCULOMA of the CNS

Epidemiology
- Is less common than tuberculous meningitis.

Pathology & Pathogenesis

- Occurs from haematogenous spread
- Is usually infra-tentorial
- May be single or multiple

Clinical Manifestations
- It presents as an intracranial space-occupying lesion (it grows instead of
rupturing). It is difficult to differentiate from intracranial tumours.
- The features include
i. Headache
ii. Fever
iii. Convulsion
iv. Sutural diathesis
v. Lateralising signs

Investigations
1. Tuberculin skin test
2. Skull x-ray
3. Chest x-ray
4. CT scan – shows discrete masses with surrounding oedema
Diagnosis
- Is mostly made during surgical exploration for intracranial tumour. Note, excision
of tuberculoma is contra-indicated because it will result into fulminant meningitis.

Section V.

TUBERCULOSIS OF SUPERFICIAL LYMPH

NODES

Pathology & Pathogenesis

- Occurs within 3 – 9 months of primary infection
- Can affect any group of lymph nodes
- May be unilateral or bilateral
- The glands are initially discrete, mobile, firm and non-tender, but later become
matted together if there is periadentis (inflammation of the surrounding tissue).
- May form a discharging sinus

Locations
1. Neck – Anterior triangle
- Posterior triangle
- Supraclavicular nodes

Clinical Presentation
i. Constitutional symptoms – may or may not be present
ii. Swellings

Examination of Glandular Enlargement

iii. The site
iv. The size - ≥ 2cm, it is pathological except for supraclavicular
gland, in which even 0.5cm size is important. It is a pointer to lung &
abdominal pathology.

Investigations
1. Tuberculin skin test

2. Chest x-ray

3. Fine Needle Aspiration Biopsy (FNAB)

 - Using big bore needle
- it is sensitive & diagnostic. It is conclusive in about 80% cases

4. Excisional biopsy of nodes for histological examination.

- Used for the remaining 20%

Differential diagnosis

1. Pyogenic lymphadenitis
- Is common around the neck
- Usually develops from URTI (infections of tonsils, fauces) or
infections around the neck
- It is tender & warm

2. Hodgkin’s lymphoma
- The glands are firmer
- Clinically, it is difficult to differentiate from TB adenitis
- Is diagnosed by FNAC

3. Acute Lymphocytic Leukaemia

- The patient is toxic, febrile & pale
- There is leucocytosis
- Blood film shows abnormal white cells

4. Fungal infection of lymph node

- The nodes are usually hard (not firm)
- Diagnosed by FNAB

5. Infection with Atypical Mycobacteria

- Diagnosed by FNAB with special staining

6. HIV/AIDS
- In children, there is usually generalized lymphadenopathy
- Do retroviral screening
 Section VI.

ABDOMINAL TUBERCULOSIS

Introduction
It comprises of

1. Tb of the Intestine
2. Tb of the Abdominal Lymph nodes
3. Tb of the Peritoneum

Clinically, it is difficult to differentiate all these.

a. TUBERCULOUS ENTERITIS

Pathology & Pathogenesis

- Occurs as a result of haematogenous spread from a primary
focus or by swallowing tubercle bacilli coughed out.
- The most common sites are the jejunum & ileum, near the
patches
- They form shallow ulcers

Clinical Features
i. Abdominal pain
ii. Diarrhoea alternating with constipation
iii. Weight loss
iv. Fever

Mortality
Is very high

b. TUBERCULOUS MESENTERIC ADENITIS

Pathology & Pathogenesis

- arises from tuberculous enteritis (since the mesenteric nodes
drain the intestinal tract)
 - the lymph node, omentum & peritoneum may become
matted together, this is palpated as a firm mass, called
“Doughy Abdomen”

Clinical Features
i. Diarrhoea alternating with constipation
ii. Weight loss
iii. Abdominal mass – Subacute intestinal obstruction
iv. ± features of Subacute intestinal obstruction

c. TUBERCULOUS PERITONITIS

Pathogenesis
- May arise from haematogenous spread or direct extension
from an abdominal lymph node infection or intestinal focus

Clinical Features
i. Fever
ii. Abdominal swelling, due to ascites
iii. Mild abdominal tenderness (due to stretching of peritoneum), a
feature of subacute peritonitis

Investigation of Abdominal Tuberculosis

1. Tuberculin skin test

2. Chest x-ray
3. Plain abdominal x-ray : shows
- relative gaslessness in the GIT
- calcified glands
4. Abdominl ultrasound – preferred to abdominal x-ray
5. Ascitic tap – for bacteriological studies

Differential Diagnosis

1. Abdominal Malignancies
 Section VII.
TUBERCULOUS OF THE SPINE
(Tuberculous Spondylitis/Pott’s disease)

Introduction
- Commonest and most important bones affected by tuberculosis in children.

Pathology & Pathogenesis

- Occurs 2 years after primary infection
- The sites are
i. Thoracic vertebrae – is the commonest
ii. Lumbar – is the second commonest
iii. Cervical

- May arise from

a. Lymphatic spread from an adjacent area
b. Haematogenous spread from a primary focus
- The infection usually starts in the body of the vertebrae, then spread to other parts.
- It may follow this course

Infection → Collapse → Cold abscess → Cord compression

Clinical Features
i. Back pain
ii. Spinal rigidity & limitation of spinal movement
iii. Diffilculty in walking (short steps ) to avoid stretching the nerves
iv. Reduced muscle power which may progress to paralysis
v. Kyphosis (is exaggerated antero-posterior curvature of spine)
vi. Scoliosis (lateral flexion) ± gibbus
Gibbus – is sharp angulation
vii. Kyphoscoliosis
viii. Loss of voluntary bladder control
ix. Increased muscle tone (hypertonia)
x. Hyper-reflexia
 xi. Spastic quadriplegia – it the cervical vertebrae are involved
xii. Spastic paraplegia – if the other vertebrae are affected
xiii. Sustained ankle clonus

Investigation
1. Tuberculin skin test
2. Chest x-ray
3. X-ray of the Spine – Specify the region. To do this, Locate T12 or palpate anterior
superior iliac spine or end of scapula
Findings :
- Widening of intervertebral space
- Distortion of the body of vertebrae
- Vertebral collapse
- Paraspinal abscess

Complications

1. Paraspinal abscess

2. Psoas abscess
– Note that Psoas muscle arise from T12 – L4
– Patient presents with inability to walk or fixed flexion deformity while
walking

3. Retropharygeal abscess (from cervical spine Tb)

- There is hyperextension of the neck
- Is diagnosed by x-ray of neck

Differential Diagnosis

1. Idiopathic scoliosis or kyphosis

2. Acute non-tuberculous osteomyelitis of the spine

- Patient is acutely ill, febrile
- The site is tender & warm
- The duration is usually short

3. Rickets
- Look for stigmata of it

4. Secondary Malignancies Affecting the CNS

 - e.g Burkitt Lymphoma, the child presents with inability to walk. It is
diagnosed by CSF tap

5. Histoplasmosis duboisii of the spine

- Is difficult to differentiate
- CSF cytology for fungi (hyphae)
- Is not very common

Section VIII.

TREATMENT OF TUBERCULOSIS

• The mainstay of treatment is Combination chemotherapy.

• The duration of therapy is 8 months

• It is divided into 2 phases

a. Intensive phase – involves giving 4 drugs for the first 2 months

b. Continuation phase – 2 drugs are given for next 6 months

Currently Used Regimen

a. Intensive phase
i. Streptomycin or Ethambutol
ii. Pyrazinamide
iii. Isoniazid
iv. Rifamipcin

N.B
Streptomycin vs Ethambutol

Streptomycin Ethambutol
 • children < 3 years • children > 3 years
• • S/E – Optic neuritis
(blindness), therefore, do
visual acuity which is difficult
to do for those < 3 yrs

b. Continuation Phase
i. Isoniazid
ii. Thiacetazone – use rifampicin to replace it in patients with
HIV/AIDS because of the Stevens-Johnson’s
syndrome, though rifampicin is expensive.

Dosages & Side effects of Anti-Tuberculosis Drugs

Drugs Dosages Side effects

1. Streptomycin 20 – 40 mg/kg/day • Toxicity to Cranial nerve 8
• Rashes
( IM ) • Renal damage

2. Isoniazid 15 – 20mg/kg/day • Peripheral Neuritis

• Psychosis
( oral ) • Hepatotoxity

3. Rifampicin 1 0 – 20 mg/kg/day • Orange discolouration of urine &

tears
( oral ) • GI disturbance
• Thrombocytopaenia
• Hepatoxicity

4. Pyrazinamide 25 – 30 mg/kg/day • Hyperuricaemia

• Skin rash
( oral ) • Hepatoxicity

5. Thiacetazone 3 – 5 mg/kg/day • Skin rash

• Haemolytic anaemia
• Stevens-Johnson’s syndrome in
( oral ) HIV/AIDS patients
• Hepatotoxicity

6. Ethambutol 15 – 20 mg/kg/day • Optic neuritis – blindness

( oral ) • Hepatotoxicity
Note,
- Only streptomycin is not hepatotoxic.

- If the patient develops jaundice, to identify the drug that causes this, stop all the
drugs and start introducing them one by one.

- To improve drug compliance, the administration can be done in 2 ways:

a. Admit the patient
b. Use Directly Observed Therapy (DOT) – where patient comes to the
clinic to take the drugs. It is being given by the nurses.

Indications for Corticosteroid As an Adjunct

1. Large pleural effusion
- normally, you don’t drain the effusion in Tb.
2. Endobronchial tuberculosis
3. Pericardial effusion
4. Tuberculous meningitis

Supportive therapy
1. Improved nutrition
2. Screening of immediate family members
3. Surgical intervention where necessary e.g in Spine or Psoas or Retropharyngeal
abscess

Prevention of Tuberculosis in a Community

1. Case-finding and effective treatment
2. Contact tracing and INH prophylaxis
Patient → +ve tuberculin test → Chest x-ray
3. BCG vaccination
4. Improvement in the general standard of living.

Management of Newborn infant of a Mother with Tuberculosis

1. Mother’s treatment should be commenced or continued if she has already started
2. INH prophylaxis should be started in baby soon after delivery and continued till
mother’s sputum has been negative thrice.
3. After this, the infant should have Mantoux test
• If –ve, INH should be discontinued and the child vaccinated with BCG for
long term protection
• If +ve, the infant should have Chest x-ray. If it is normal, continue the INH
for 12 months, but if it is abnormal, treat as tuberculosis with Combination
therapy.

N.B
- BCG vaccination takes 6 weeks for it become effective.
- The use of INH-resistant BCG has been stopped since the company
manufacturing it stopped the production.
 Chapter 5
ASTHMA

Section I
INTRODUCTION, EPIDEMIOLOGY, RISK FACTORS & PATHOGENESIS

Introduction
- Is an heterogeneous disease
- Has 3 characteristics
i. variable airway obstruction (severe or minor) which is often (not
always) reversible
ii. chronic airway inflammation
iii. airway hyper-responsiveness

Three Clinical Patterns

1. Atopic / Extrinsic asthma – is the commonest in childhood
2. Non- atopic /Intrinsic asthma
3. Mixed

N.B Atopy is the propensity to produced IgE antibody to allergens commonly

encountered in the general environment e.g pollen, mites, moulds.

Definition of Asthma
“Occupational Definition supported by WHO”
- Is a chronic inflammatory disorder of the airways in which many cells including
mast cells & eosinophils play a role
- In susceptible individuals, this inflammation causes symptoms which are usually
widespread but variable airway obstruction that is often reversible either
spontaneously or with treatment and causes an associated increase in airway
responsiveness to a variety of stimuli.

Epidemiology of Asthma
- The distribution is worldwide
- The incidence is 10 – 20%
- In Paediatric age group of this environment., 60% of cases are seen before 3 years
& 80% before 5 years.
- The ratio male/female = 1.3/1
- At Puberty, the sex ratio changes and returns at adulthood.
- Commoner among Caucassians
- Commoner in industrialized countries
- Commoner among the affluents
- Commoner in the urban areas
Risk Factors

a. Hereditary – genes play an important role in pathogenesis of asthma

b. Environment - the presence of environmental factors is possibly mandatory in the

development of asthma.

Note, there is an interplay between both factors i.e it is a multifactorial condition

Three Environment Variables

1. Allergens
2. Air Pollution
3. Viral Respiration Infection

1. Allergens
- Is found indoors or outdoors or both
Pollens – from trees, grass
Moulds – Aspergillus fumigatus
Mites - They are different species
i. Dermatophygoides pteronyssinus – is the commonest in
this environment
ii. Dermatophygoides farinae – common in UK

Sources of allergens
i. Animals
ii. Dust
iii. Birds

2. Air Pollution
Outdoors : i. Ozone – it is increased where there are many cars
ii. Sulphur dioxide – it is high in industrialized areas because of
combustion of coal

Indoors : i. Tobacco smoke

ii. Contaminants from indoor gas combustion, the gas produced
is NO2

3. Viral Infection
Is usually those causing upper respiratory tract infection:
i. Respiratory syncytial virus – mainly
ii. Rhinovirus
iii. Parainfluenza
Inducers & Inciters of Asthma
• All these environmental factors can initiate asthma in an individual who is
genetically predisposed hence called Inducers of asthma.
E.g Formula feeds i.e non-breastfeeding in the first 6 months.
• After the first attack of bronchial asthma, certain factors are involved in the re-
occurrence of asthma. These are called Inciters, sometimes called Trigger
Factors.
• Many inducers are also inciters.

Examples of inciters are

i. Viral upper respiratory tract infections – is the most important in
childhood
ii. Aero-allergens
iii. Exercise (called Exercise Induced Asthma, though this now a
misnomer because it is not an inducer, but an inciter)
iv. Irritant fume
v. Emotion
vi. Cold air

Pathogenesis of Allergic Asthma

Pathogenesis of Allergic Asthma

T-cell (TH-2 Lymphocytes –Helper or CD4 +)

B – cell

Mast cell Eosinophil

Chymase LTC4 ] Epithelium Shedding

LTD4 ]
LTE4 ] Congestion
Histamine ] & Oedema

Glandular
Secretion

Airway Obstruction
ASTHMA

Airway Hyper-responsiveness

Tryptase Smooth Muscle

Loss of Epithelium
down regulation

TH -2 cells
- are the central cells in the orchestration of local eosinophilia, mast cell
replenishment & IgE production.
- Recognises and responds directly to allergens

First Stage : Sensitization

- Is the first process after the exposure to allergens.
- B – cells respond by producing allergen-specific IgE which is
attached to the surface of mast cells.
- Repeat contact with the same allergen causes cross-linkages of the
already attached IgE creating a signal for the mast cells to produce
 preformed or newly formed leukotrienes (shown above), histamine &
prostaglandins which then cause airway obstruction.

Second Stage : Immediate Asthmatic Response or Bronchospasm

- Is caused by Slow Reacting Substances of Anaphylaxis
- Leukotriene complex are the most powerful substance in causing
bronchospasm.
- If nothing is done, there is movement of cellular elements into
lumen of the cells worsening the obstruction of airways

Third Stage : Late Phase Response

- There is movement of other cellular elements into the airway.

Section II
CLINICAL PRESENTAION OF ASTHMA

Symptoms
i. Cough
ii. Attacks of wheeze
iii. Breathlessness

All the above features are also present in non-asthmatic conditions and what
gives clue to the the diagnosis of asthma are :
o Repeated wheeze i.e > 2 episodes
o Recurrent / Persistent cough
o Night time disturbance of wheeze or cough

These symptoms are usually precipitated by:

iv. Viral upper respiratory tract infections
v. Exercise
vi. Family & Emotional disturbance
vii. Potential allergens : Pests, pollen, dust, feathers, cigarette

Other features
viii. Presence of collateral conditions e.g allergic rhinitis, atopic dermatitis.
Therapeutic response to bronchodilator makes the diagnosis more
likely.
ix. Past nedical history of infantile eczema makes the diagnosis more likely
x. Family history of asthma
xi. What is seen during physical examination depends on whether the
patient is acutely symptomatic or not
Indices of pulmonary function

1. Vital capacity – is the largest volume a subject can expire after a single
maximal inspiration

Forced Vital Capacity (FVC) – is the vital capacity when the expiration is
performed as rapidly as possible.

2. Forced Expiratory Volume in One Second (FEV1) – is the volume expired

during the first second of FVC.

3. Ratio of FEV1/FVC
- The normal value ≥ 75%, though this depends on the age, sex and
height.
- The ratio is reduced in obstructive airway disorders e.g
o Asthma
o Emphysema
o Bronchitis
therefore, it is not pathognomonic for asthma.
4. Peak Flow Rate (PFR)
- Is maximum expiratory flow rate achieved during a forced expiration
(is different from FVC in that you don’t take in forced inspiration
before)
- Is measured in ml/min
- Is more convenient to measure than FEV1 ( as FEV1, FVC, FEV1/FVC
require spirometer)
- It only requires a Wright Peak Flow Meter (it measures more
accurately than RL1 usually given to the asthmatic patient.

Investigations
- Are divided into 3 main groups
i. Chest radiography
ii. Pulmonary Function Test (PFT)
iii. Others

1. Chest Radiography
- Chest x-ray is warranted in an acutely symptomatic patient if complications listed
are suspected.
o Pneumonia
o Pneumothorax
o Pneumomediastinum
- Otherwise, it’s not really necessary.
2. Pulmonary Function Tests
- Done to establish presence of bronchial obstruction, with the use of
spirometer (Vitalograph). The following are measured
o FEV 1
o FVC
- Their values are low if obstruction is present.
- Then, to confirm asthma, give a bronchodilator and wait for about 5 –
20minutes. An increase of ≥ 15 – 20% over the baseline suggests (not
diagnostic) asthma .

3. Others
a. Bronchial bronchodilation test
- Is done in selected cases i.e in those with unclear picture of
asthma.
- Any of the materials below can be used to incite or provoke
bronchoconstriction
o Hypertonic saline (3%, NaCl)
o Histamine
o Metacholine
o Cold air
o Exercise
- At the start of the procedure, take the baseline value, then
provoke bronchoconstriction and repeat the measurement.
- If FEV1 done after 5 minutes falls by ≥ 20%, it suggests asthma.

- Precautions
i. Ensure the patient is most likely not to be asthmatic
ii. Get bronchodilator ready to resuscitate.

b. Laboratory tests
1. Full blood count

2. Absolute Eosinophil count

- eosinophilia expected shows that the patient has atopic
constitution i.e prone to allergic dermatitis, eczema, asthma.

3. Cytological Analysis of Sputum

- Eosinophilia is higly suggestive of asthma, if pulmonary
eosinophilia (due to parasitic lung disease) can be excluded,
the diagnosis of asthma has almost reached 100%.

4. Measurement of specific IgE Antibody i.e the allergen specific IgE. It is

done in 2 ways
 i. In-vivo test
- Involves skin sensitive test in which specific allergens
e.g cat saliva, pollens, mites (faeces) are used

ii. In vitro test

- Using Radio Allergy Solvent Test (RAST) to measure
specific IgE antibody.

Differential diagnosis

- These are generally causes of wheezing (though cough may also be present)
- They are grouped into
i. Very common
ii. Common
iii. Uncommon
iv. Rare

A. Very Common
1. Viral Bronchiolitis
- Especially in those who are < 2 years of age.
- It is caused by Respiratory Syncytial Virus, especially 2
major serotypes A & B
- Do not have > 2 epispdes of wheezing because the child is
expected to develop immunity.

B. Common
2. Foreign body in trachea or bronchus

3. Endobronchial tuberculosis (causing narrowing of the airways)

4. Enlarged Tuberculous lymph nodes (causing extramural

compression)

C. Uncommon
5. Vascular rings (around the airway)
6. Bronchiectasis
7. Obliterative bronchiolitis
8. Laryngotracheomalacia
9. Chlamydial trachomatis infection
10. Bronchopulmonary dysplasia
11. Aspiration from Swallowing
 D. Rare
12. Tumour
13. Larygeal web
14. Alpha -1- antitrypsin deficiency
15. Cystic fibrosis

Note on Cardiac asthma

- It is seen in ventricular failure & pulmonary oedema.
- It responds to aminophylline, if adrenaline is used in bronchial
asthma, the patient will be dead.

Age Group Preferred Device Alternative Device

< 4 years Pressurized metered dose inhaler + Nebulizer with Face mask
Dedicated spacer +
Face mask
4 – 6 years Pressurized metered dose inhaler + As above
Dedicated spacer +
Mouth piece
> 6 years i. Dry Powder inhaler or Nebulizer with Mouth piece
ii. Breath-Actuated Pressurized Metered
dose inhaler or
iii. Pressurized Metered-dose Inhaler with
Spacer
Section III
TREATMENT OF BRONCHIAL ASTHMA

Choice of Inhaler Device for Children

Routes of Administration
The choice is based on the following factors
i. efficacy of the drug
ii. cost effectiveness
iii. safety and convenience

The routes are

1. Oral – is slowly-acting

2. Parenteral
a. Subcutaneous
b. Intramuscular
c. Intravenous
 3. Inhalation –for aerosols preparation. This is preferred for the follwing
reasons
i. It is easier to administer
ii. Has less side effects
iii. Is much more convenient

Salbutamol (Ventolin)
- is a β2 – agonist used in treatment of acute exacerbation
of acute asthma.
- Other routes of administration are oral, subcutaneous,
intravenous.
Terbutaline

N.B
OSCE :

I.
MANAGEMENT OF ACUTE EXACERBATION OF
ASTHMA

Introduction

- From the definition and its features, asthma can be described as acute-on-chronic
disorder, the acute exacerbation leads to obstruction.
- Its treatment can be divided into 2
a. Treatment of acute airway obstruction
b. Treatment of chronic inflammation (i.e day-to-day management)

Goals of Treatment
1. Relieve airflow obstruction & hypoxaemia as rapidly as possible
2. Plan prevention of future relapses
3. Close monitoring of patient’s condition & response to treatment are crucial for
successful treatment.

Steps
1. Brief History - to know the following
• Time of onset
• Cause of present exacerbation
• Severity of symptoms including exercise limitation and sleep
disturbance
• All current medications
- Doses usually taken
 - Dose taken in response to current deterioration
- Patient’s response to above medication
• Prior hospital/emergency room visits for asthma in the past year
• Prior episode of intubations / Intensive Care Unit care
• Use of oral glucocorticoids
• Use of inhaled glucocorticoids

Note, the last factors are high risk for asthma-related death

2. Physical examination
• To assess the severity of acute exacerbations
• Identify complications or possible precipitating factors (Pneumonia,
pneumothorax, atelectasis etc
• Do the following if possible : PEF or FEV1 or pulse oximetry.

Severity of Asthma Exacerbation

(Global Initiative /NA Recommendation)
Parameters Mild Moderate Severe Imminent
Respiartory
Arrest/Life-
threatening
1. Walking - Talking infant At rest, infant
Breathlessness - Softer cry stops feeding
- difficult feeding
2. Can lie down Prefers sitting Hunched forwards
3. Talks in Sentences Phrases Words
4. Alertness May be agitated Usually agitated Usually agitated Drowsy or
confused, close to
death
5. Respiratory Increased Increased Increased
rate
6. Acccessory Usually not Usually used Usually used Paradoxical
Muscles & thoraco-lumbar
Suprasternal movement
retractions
7. Wheeze Moderate, often Loud Usually loud Absence of
only end wheeze( it is
expiratory paradoxical,it is
due to total
airway
obstruction
8. Pulse rate Increased Increased Increased Bradycardia
9. Pulsus Absent May be present Often present Absence, suggest
Paradoxicus respiratory
muscle fatigue
10. PEF, after > 80% 60 – 80% < 60%
initial
bronchodilator,
% Predicted
% Personal best
11. PaO2 (on Normal > 60 mmHg < 60 mmHg,
air) possible cyanosis
12. PaCO2 < 45 mmHg < 45mmHg > 45 mmHg,
possible
respiratory failure
13. SaO2 (on > 95% 91 – 95% < 90%
air)

Note
 i. Pulsus paradoxus is not a good parameter in paediatric age group
ii. The presence of several parameters, but not necessarily all indicate the general
classification of the exacerbation.

Recognition of Acute Severe Asthma

i. Too breathless to talk
ii. Too breathless to feed
iii. Tachypnoea
iv. Tachycardia
v. PEF ≤ 50%, predicted of best

Life-threatening Features
i. PEF < 33% predicted or best (is difficult to measure)
ii. Cyanosis
iii. Silent chest or poor respiratory effort
iv. Fatigue or exhaustion
v. Agitation
vi. Reduced level of consciouness

Treatment Drugs
1. Oxygen – is given by nasal catheter or mask, SaO2 at > 95% (Percutaneous oxygen)

2. Rapid-acting inhaled B2-agonist e.g salbutamol (is used here), terbutaline.

If this is not available, use adrenaline or aminophyline

3. Corticosteroid – for resolution of the inflammation

4. Ipratropium bromide

5. Others :
• Antibiotics
• Rehydration
• NaHCO3

Salbutamol
Side effects
1. Tachycardia
2. Tremor
3. Hypokalemia
4. Hyperglycaemia

Adrenaline
 - is given subcutaneously, the dose 0.01mg/kg, maximal dose is 0.3mg
- if given on 3 occasions and there is no response, stop it.

Aminophyline
- be cautious with it
- the dose is 5mg/kg, give slowly over 20 minutes to prevent
cardiovascular collapse, GIT bleeding
- it has narrow therapeutic index ratio
- has little effect with Nebuliser

Corticosteroid
- e.g hydrocortisone Na succinate, prednisolone

a. Prednisolone : should be given orally if the patient can take orally

because oral administration has the same effect with intravenous.

b. Hydrocortisone Na Succinate

- when combined with B2-agonist, it is better bronchodilator

than using it alone. It also has greater effect on PEF & FEV1

Antibiotics
- Is not given routinely
- Is indicated if there is purulent sputum, x-ray evidence of pneumonia

Rehydration
- especially for infants and the young children
- Calculate
• deficit + ¾ maintenance

( because the patients are prone to SIADH)

(Pseudomorphine)

Sodium bicarbonate
- to correct acidosis (which can be metabolic or mixed)
- check electrolytes & urea
- 2 – 4mEq/kg empirically, dilute twice its volume and give slowly.
- After knowing the HCO3 result, use the formula below to calculate the
deficit

X (mEq/L) = (AHCO – OHCO3) X Weight X 0.3

3
 A – Actual value
O – Observed value
0.3 – is a distribution factor

- NaHCO3 used in UCH is usually 8.4%

Side effects
1. Cerebral acidosis : NaHCO3 dissolve in brain to Na+ + HCO3
which further dissociate to CO2 + OH-, the CO2 combines with water to
form carbonic acid.
2. Alkalosis
3. Hypokalaemia – because alkalosis causes potassium to move
into cells
4. Depression of respiratory rate
 Assessment of Severity

a. Mild b. Moderate c. Severe & Imminent Arrest

(Is discussed in the next chart)

Inhaled Salbutamol • Inhaled or nebulised salbutamol (3 times in 1 hour,

(8 – 10pops/1hour) and evey hour afterwards.
or • Oxygen to achieve SaO2 ≥ 95%
Nebulised Salbutamol • If there is no immediate response or patient recently
(3 times in 1 hour) took oral steroid, give oral prednisolne, 1 –
2mg/kg, 6 hourly

Response No response Response No response

Observe for 60 minutes Continue treatment Continue

( 1 – 3 hours) - O2
- Oral prednisolone, 6hrly
Stable Deterioration - Nebulised Salbutamol, q
Stable 30min or continously
- Add ipratropium to
Discharge home Neb. q 6 hour
- Continue salbutamol
every 3 -4 hours for 24 – 48 hours Discharge home
( 2 pops on each occasion) - Continue inhaled salbutamol
- & oral prednisolone ( in morning)
- Educate the patient

Response No response
N.B
Oral prednisolone should be taken in the morning
So as not to disturb the circadian rhythm of cortisol Aminophyline drip
Which is normally higher in the morning.

No response

Admit to ICU for

mechanical ventilation
Dose of Salbutamol (Rule of thumb)
Age < 2years - 2.5mg
Age > 2years - 5mg
- But it should not be above 0.15mg/kg

Dose of Aminophyline
0.9 – 1mg/kg/hour
 C. Severe & Imminent Arrest

Hospitalisation

Severe Imminent Arrest

• Humidified oxygen i. Humidified oxygen

• Nebulised Salbutamol ii. Salbutamol nebulisation (3 times in 1 hour)
( 3 times/hour) iii. IV Aminophyline, 5mg/kg stat, then drip,
• Oral prednisolone, 1 – 2mg/kg 1mg/kg
iv. IV Hydrocortisone
v. Add Ipratropium bromide,0.25mg to Neb
salbutamol

Response No response

No response
Continue Continue
- oxygen - oxygen
- oral prednisolone - oral prednisolone Admit ICU
- Neb. Salbutamol - Neb. salbuatmol, q30min
or continously
- add Ipratropium bromide Intubation & Mechanical
ventilation
Response sustained

Response No response
Discharge home
- continue on
• Salbutamol Aminophyline drip
• Inhaled steroid
• Oral prednisololone
• Patient’s education No response

Admit ICU
II.

DAY-TO-DAY MANAGEMENT OF ASTHMA

(Treatment of Chronic Inflammation)

Introduction

This is an essential aspect in the management of asthma since the acute exacerbation
occurs more frequently in patients with poor day-to-day management.

Concepts /Goals
i. To avoid allergens
ii. Improve bronchodilation
iii. Reduce inflammation

Components of day-to-day Management

1. Stepwise Approach
2. Asthma Management Zone System
3. Patient Education and Counselling

Classification of Asthma Severity for Long-term Management

Parameters Intermittent Mild Moderate Severe

Persistence Persistence Persistence
1. Symptoms <1 /week > 1 / week Daily Daily

2. Brief asymptomatic Affect activity Affect activity & Frequently

Exacerbations (between episodes) & Sleep Sleep
3. Night time ≤ 2 /month > 2 /month > Once /week Frequently
symptoms
4. Daily use of No No Yes
inhaled short B-
agonist
5. Physical ___ ___ ___ Limited by
Activity symptoms
6. PEF or FEV1
• % Predicted ≥ 80 ≥ 80 60 – 80 ≥ 60
• % Variability < 20 20 - 30 > 30 > 30
“Personal best” value - is taken when patient is not showing any symptoms

Calculation of % Variability
% Variability = Max. PEFor FEV1 -- Min. PEF or FEV1
of PEF or FEV1 Max. PEF or FEV1

Recommended Medications by Level of Severity

1. Concerning all steps : In addition to daily controller therapy, rapid acting inhaled
i.e salbutamol (B2-agonist) should be taken as it is needed to relieve symptoms,
but should not be taken for > 3 – 4 times a day.
2. Concerning all steps : Once control of asthma is achieved and maintained for at
least 3 months, a gradual reduction of the maintenance therapy should be tried in
order to identify the minimum therapy required to maintain control.
3. If control is not achieved, review patient’s inhaler technique, compliance and
environmental control.
4. Children with intermittent asthma but severe acute exacerbation should be
treated as having moderate persistent asthma.
5. The patient’s is the determined by the most severe feature
Level of Daily Controller Medications Other treatment Options
Severity
Step 1 : Not necessary
Intermittent
Step 2 : Inhaled glucocorticoid e.g
Mild Persistent • Budesonide, 100 – 400µg or Step 2
equivalent, using Tubb inhaler • Sustained-release theophyline or
• Fluticasone propionate (flixode), cromone (Na cromoglycate)
using Metered-dose, given bd, • Leukotriene modifier e.g
100 -200µg daily - Zafirlukast(Accolate)
Step 3 : Inhaled glucocorticoid e.g - Montelukast (Singulair)
Moderate Budesonide, 400 -800µg or equivalent • Inhaled glucocorticoids e.g
Persistent (< 800 µg Budesonide or its equiv.)
Step 4 : Inhaled glucocorticoid e.g +
Severe Persistent Budesonide, 800µg or equivalent Sustained-release theophyline
Asthma + 1 or more of the following • Inhaled glucoc. (800µg
- Sustained-release theophylline
Budesonide or equiv.)
- Long-acting inhaled B2-agonist
+
e.g seremine, salmeterol
Long-acting inhaled B2-agonist
- Leukotriene modifier
- Oral glucocorticoids e.g • Inhaled glucoc. At higher
prednisolone doses(>800µg Budesonide or
equiv.)
• Inhaled glucoc. (800µg
Budesonide or equiv.)
+
Leukotriene Modifier

N.B
Age Dose
Zafirlukast : ≥ 12 years 20mg twice daily
Montelukast : ≥ 6 years 5mg daily

Patient Education & Counselling

1. Partnership with patients and parents
2. Cause and prevention of asthma
3. Purposes and side effects of medications
4. Use of inhalers and volumatic spacers
5. Written daily plans.
 Chapter 6
EVALUATION OF A CARDIAC PATIENT

Section I
HISTORY OF A CARDIAC PATIENT

i. Onset /Duration
- For younger patient, think of congenital causes and vice-versa.
- Generally, for those < 5 years, think of congenital, and > 5 years,
think of acquired cause first.
- Note, there are exceptions, some congenital heart diseases are
asymptomatic at early age and show symptoms in 2nd or 3rd decade
e.g
• small VSD
• Coarctation of the aorta – commonly presents at
12years, with features of heart failure due to the
effects on left ventricle.
• Atrial Septal Defect – many are asymptomatic in
the first decade of life.
- Conversely, acquired heart disease e.g Rheumatic heart disease (most
common), infective heart disease & pericardial effusion do occur in infants,
though are commoner in older children.
ii. Cough - common

iii. Breathlessness - common

iv. Cyanosis - bluish, occurs especially in Cyanotic heart disease

v. Failure to thrive - results from compromised supply of nutrients in the

circulation

vi. Delayed developmental milestone – occurs in severe heart disease. It could be

a part of syndrome e.g

a. Down’s syndrome : (common among the Mongols) in which there is

mental retardation. The commonest heart disease associated with
Down’s syndrome is Atrial Septal Defect (ASD)

b. Congenital Rubella Syndrome : It consists of

• Mental retardation
• Deafness
 • Cataracts (Bilateral)
• Microcephaly
• Patent Ductus Arteiosus

vii. Convulsions /Fainting : Is seen in

a. Obstructive heart disease e.g aortic stenosis
b. Tetralogy of Fallot – usually has a lot fainting attacks

viii. Stroke : Occurs in patients with

a. polycythemia (hyperviscosity syndrome) as in TOF
b. Infective endocarditis

ix. Pedal /Abdominal / Facial swelling – indicates right sided heart failure
N.B
- In paediatric age group, both right & left sides are usually in heart failure

x. Past Medical history – is very important in Rheumatic heart disease

where you ask for previous history of fever.
- TGA is commonest in diabetic mothers
xi. Pregnancy – especially in congenital heart disease, ask concerning
• Features of intrauterine infections (fever or rashes during
pregnancy) e.g Rubella
• Duration of pregnancy – because of PDA
• Tetrogenic drugs
xii. Birth
- History of birth asphyxia – which predisposes to PDA, since reduced
oxygen tension is needed to close the duct.

xiii. Neonatal /Infancy : Ask for

• Cough
• Breathlessness
• Cyanosis
• Feeding difficulties
• Frequent chest infection – is a pointer to chronic heert
disease
xiv. Developmental milestones : rubella, chronic abnormalities

xv. Family & Social History

a. As low socio-economic status is a risk factor to
• Acute rheumatic heart fever
• Endomyocardial fibrosis
b. High predisposes to hypertension because of obesity
c. History of hereditary problems in the family
 Section II
PHYSICAL EXAMINATION OF A CARDIAC PATIENT

A. General

i. Feature of acute of chronic illness

ii. Nutritional status
iii. Dysmorphic features – these are anatomical defects which may(not) have
pathologic cause e.g Down facies in Down’s syndrome
o Slanting epicanthic flod
o Flat nasal bridge
o Flat occiput

Congenital rubella syndrome

o Features listed above
o Cleft lip or palate
o Polydactyly or syndactyly
o Talipes deformity
o

iv. Skin colour : pink /blue – as feature of congenital heart disease (Cyanotic &
Acyanotic), and evidence of central cyanosis
v. Digital clubbing
vi. Pedal oedema
vii. Dyspnoea

B. Specific : Cardiovascular System

I. Inspection
II. Palpation
III. Percussion
IV. Auscultation

I. Inspection
• As for general examination
• Praecordial bulge – is due to cardiomegaly, thought it is difficult to say if it
is LVE or RVE.
- It also shows the chronicity of the illness, i.e a long
standing illness that could be congenital
• Chest symmetry
• Abnormal pulsations in the neck
• Jugular venous pressure – Is not sensitive in children < 2years because of
the short neck, but important in older children.
II. Palpation

1.
Pulse

a. Rate : The cut off point for abnormalities is age-dependent

Neonate – 160 is the upper limit (as compared with adult
which is 100 )
- < 100 is Bradycardia

b. Rhythm – i.e the regularity

Types
i. Regularly Irregular
– Is not necessarily abnormal because many
children do have sinus arrhythmia (i.e the heart
rate changing with respiration)
- Is often difficult to pick, it is usually seen on ECG

ii. Irregularly irregular

- A common cause of it is Atrial fibrilation

c. Volume
Types
i. Bounding /Collapsing – seen in hyperdynamic
circulation. The causes are gropued below

Cardiac Non-cardiac
1. Patent ductus Arteiosus 1. Fever
2. Aortic incompetence 2. Fever
3. Chronic anaemia

ii. Small volume pulse – the causes are grouped below

Cardiac Non-cardiac
1. Aortic stenosis 1. Shock from any cause
2. Mitral stenosis
3. Pericardial effusion
N.B
Always remember to palpate the peripheral pulses – to be able to pick coarctation of
the aorta.

2. Apex beat

a. Location – the normal location is 4th or 5th left intercostals space medial to
mid-clavicular line.
- When displaced downwards & outwards, it suggests
enlargement either by hypertrophy or dilatation, but is usually
more of dilatation.
- Other causes are i. massive pleural effusion on the right side,
but check for the trachea displacement from midline.
ii. dextrocardia

b. Character
i. Heaving or hyperactive – suggests LVH (Note, it is possible to have
hypertrophy without dilatation in which the apex beat is not
displaced.

ii. Tapping : Suggest mitral stenosis

Palpation of left sternal edge – heaving there suggests RVH.

3. Thrills (palpable mumur)

- Is in phase with cardiac cycle
a. Timing
Systolic
At apex : suggests Mitral regurgitation
At Aortic area : suggests Aortic stenosis
Diastolic
At apex : suggests Mitral stenosis

b. Location
- as above
- At lower left parasternal : suggests VSD

4. Blood Pressure
- Is usually to the end because of the discomfort especially in children.
 III.
Percussion
- Has little value in children.
- Is done to check for enlargement.

IV.
Auscultation

1 - Aortic area (Right upper sternal adge)

2 - Pulmonary area (Left upper sternal edge)
3 - Tricuspid area (Left lower sternal edge)
4 - Apex /Mitral valve area

- Also, auscultate in intervening spaces between the valve areas

1. Types of Sounds

i. Normal heart sounds

I II I II
S1 S2 S1 S2

S1
- is due to closure of atrioventricular valves
- Mitral valve closes a split second before and also louder than
tricuspid valve (though, this is not very significant)

S2
- is due to closure of semilunar valves. Aortic valve closes
slighlt earlier than pulmonary valve. You will need to listen
carefully to get this.
- It has a normal split – aortic & pulmonary components
ii. Wide splitting of S2 (non-fixed)
I I I I I I
S1 S2 S1 S2
- It is wider during inspiration than expiration because the right
side of heart fills more during inspiration.
- Causes:
i. Aortic stenosis
ii. Pulmonary stenosis
iii. Right bundle branch block
iv. Left bundle branch block

iii. Wide & Fixed split S2

I I I I I I
S1 S2 S1 S2

Cause : Secondum ASD

iv. Single S2 - i.e A2 & P2 are one

I I
S1 S2
Causes
i. Severe aortic stenosis or atresia
ii. Severe pulmonary stenosis or atresia

v. Triple rhythm
I II I I II I
S1 S2 S3 S1 S2 S3
- When it is associated with tachycardia (i.e beating very fast), it is
called Gallop rhythm.
- It is heard at times in normal people
Causes
i. Myocardial heart disease
ii. Heart disease

vi. Ejection click

- It occurs just before S1
- Is louder on expiration
- May disappear on inspiration
- Is generated either from aortic or pulmonary valve
 Causes
i. Valval aortic stenosis
ii. Valval pulmonary stenosis
iii. Dilated pulmonary artery
iv. Dilated aorta

2. Intensity
a. High intensity (Loud heart sound)
- Is due to increased blood flow or large vessel
Causes
i. Loud P2 – Pulmonary hypertension
ii. Loud A2 - Systemic hypertension

b. Low Intensity (Soft muffled heart sound)

Causes
i. Aortic stenosis
ii. Pulmonary stenosis
iii. Myocardial disease
iv. Pericardial effusion

3. Murmurs
- Are abnormal sounds that are due to turbulent flow through a
narrowed or thickened channel.
Characteristics
i. Timing
ii. Intensity
iii. Location

i. Timing

- It may be systolic /diastolic / continous

I Systolic phase II Diastolic phase I II
S1 S2 S1 S2
Systole : is the phase between S1 and S2
Diastole : the phase between S2 and S1

A. Types of Systolic Murmurs

i. Pansystolic /Holosystolic - spans the time between S1
and S2

Causes
 a. Ventricular septal defect
b. Mitral incompetence
c. Tricuspid incompetence

ii. Ejection Systolic Murmur(ESM) – starts after S1 and

increases gradually in intensity, then decreases gradually
again, thus looks like a diamond i.e in crescendo &
decrescendo form.

Causes
i. Pulmonary stenosis
ii. Aortic stenosis
iii. Atrial septal defect

B. Diastolic Murmurs
- Generally, isolated diastolic murmurs are not as loud or
common as systolic murmur.
Causes
i. Aortic incompetence - it is usually high-
pitched
ii. Mitral stenosis – Is low-pitched or rumbling &
midiastolic
C. Continous Murmurs

- It usually starts anywhere in systole, then continue through S2

into diastole.
- It is also called To and Fro murmur / Machinery murmur
Causes
i. Patent ductus arteriosus – is loudest under the left
clavicle
ii. Arterio-venous malformations e.g coronary artery AV
fistula
iii. Combination of Aortic incompetence & VSD /ASD
/PDA
iv. Collateral circulations seen in coarctation of aorta

N.B
Venous hum
- is benign, i.e not pathological
- often heard at the base of the neck, from the large venous
system
 - Is not as loud as murmur in PDA.

ii. Intensity (Grading)

Grade Description
I Extremely soft(Softest)
II Louder, but still soft
III Loud enough to be heard by Medical students, but does
not produce a thrill
IV Produces a thrill
V Heard by putting the stethoscope at an angle to the chest
VI Heard with stethoscope away from the chest

- Grades III & IV are commonly found

iii. Location
- Is the place where a murmur is heard maximally (because a very loud
murmur can be heard all over the chest).

• Loudest at apex & systolic - Mitral incompetence

• Loudest at lower sternal edge - Tricuspid incompetence or
Ventricular septal defect, but VSD is 99 times more common.
• Right upper sternal edge - Aortic stenosis
• Left upper sternal edge - Pulmonary stenosis
• Left lower sternla edge - ASD is also heard there.

Evaluation a Patient With Heart Failure

Causes
1. TGA is often associated with heart failure
2. VSD
3. PDA
- Exclude Pulmonay stenosis & Tetralogy of Fallot
- If due to VSD, it shows that the abnormality is haemodynamically
significant i.e to prognosticate the lesion.
History
i. Cough
ii. Breathlessness
iii. Poor feeding
iv. Frequent chest infection
 Examination
i. Tachypnoea
ii. Tachycardia ( due to released catecholamines to compensate for the
failing heart)
iii. Tender hepatomegaly
iv. Peripheral oedema
v. Increased JVP
- The first 3 signs are very important while the last 2 signs are
seen in older children.

Section III

INVESTIGATION OF A CARDIAC PATIENT

1. Chest X-ray
2. Electrocardiogram (ECG)
3. Echocardiography (ECHO)
4. Cardiac Catheterisation
5. Angiography
6. Arterial Blood gases

1. Chest X-ray
- Is done for every suspected patient
- The following features are examined for
a. Heart size
b. Heart shape
c. Aortic arch
d. Pulmonary Artery
e. Lung fields

a. Heart Size
- In children > 5 years, it should not be enlarged beyond 50%
- In infants, 50 – 60% is allowed
- 50% should be the upper limit for < 2 years children

Causes of Enlarged heart

i. Heart failure
- chamber dilataion/hypertrophy
ii. Pericardial effusion
b. Heart Shape
- The normal heart is triangular in shape with left side having bumps

Abnormal Shapes of heart

1. Boot –shaped

- the apex is lifted

- pulmonary artery is small
- It suggests Tetralogy of Falot

2. Egg-on-side

- It suggests Transposition of Great Arteries (TGA)

3. Cottage loaf/Snowman /Figure-8 shape

- It suggests Total Anomalous Pulmonary Venous Drainage (TAPVD).

It is a condition in which all the pulmonary veins drins abnomally, i.e
anywhere beside the normal right atrium. They usually drain into
Superior venous cava.
 4. Globular heart shape

Causes
i. Pericardial effusion
ii. Endomyocardial fibrosis
iii. Dilatation of all the Chambers

c. Aortic Arch
Normal : The shadow of the arch is on the leht side

Abnormal :
i. Right Sided Aortic Arch
When it arches over the right side but the apex is pointing
to the left side i.e it is still laevocardia.
Causes
i. Tetralogy of Fallot – commonest ( it is
NOT seen in all cases, but about 25%)
ii. Pulmonary atresia
iii. Truncus Arteriosus

In Dextrocardia, where the apex is pointing to the right side and

the right sided aortic arch can be said to be normal in them.

d. Pulmonary Artery - May be large or absent

a. Large – when there is increased blood flow
Causes
i. Left-to-right shunt
ii. Pulmonary hypertension – from any cause
 b. Small or absent – when there is reduced blood supply to the lungs
Causes
i. Pulmonary atresia
ii. Tetralogy of Fallot

e. Lung Fields – Its feature tends to follow what happens to the pulmonary
artery
• Increased Pulmonary vascular markings (Pulmonary Plethora)
Causes
i. Left-to-right shunt
ii. Heart failure

• Decreased Pulmonary vascular marking (Pulmonary Oligaemia)

Causes – Sever obstruction as in
i. Tetralogy of Fallot
ii. Pulmonary atresia

2. Electrocardiogram
- Is done to confirm to confirm
• the heart rate
• rhythm
• axis – is important. It is the net direction of electrical forces

0 – 90 : Normal
RAD - Right Axis Deviation
LAD – Left Axis Deviation
ERAD – Extremely Right Axis Deviation
ELAD – Extremely Left Axis Deviation

Right Axis Deviation- is seen in i. TOF

ii. TGA
 In Paediatrics, the use of superior and inferior axis is preferred.
a. Superior axis (ERAD, ELAD, LAD)
Cause
i. With acyanosis, it alerts to Atrioventricular
Septal Defect (AVSD)

ii. With Cyanosis, it points to Univentricular

disorder e.g Tricuspid Atresia (is the commonest).
With Conduction defect, there is corroboration of
left or right ventricle.
- Atrial /Ventricular hypertrophy

3. Echocardiography
- Is ultrasound of the heart.
- Is advantageous because it can be repated many times
- there are 2 forms
a. M-Mode (Motion)
- Is older
- Is for measurement
- And to assess cardiac functions
- It is NOT good for structural defects

b. 2-Dimension Echocardiography
- Shows cross-section of the heart
- Is extremely useful for structural defects e.g detecting holes

4. Cardiac Catheterisation
- Is very useful, but highly invasive
Types
a. Right Sided Study – The catheter is passed via the below route
to the heart

• Femoral vein → Inferior vena cava→ Right atrium→

Right ventricle→ Pulmonary artery

b. Left Sided study

• Femoral artery→ Descending aorta→ Left ventricle
Bleeding is the major problem.
Uses
i. To confirm structural defects e.g ASD,VSD, PDA
ii. To measure pressures (which can now be calculated with M-
mode echo.
iii. For oxymetry (O2 saturation measurement)
5. Angiography

6. Arterial Blood Gases

- Is not routinel done here, but in advanced countries, any cardiac patient does it.
- It is to check for central cyanosis and determine its origin (Respiratory or Cardiac).
- When 100% oxygen is given, the cyanosis improves if the cause is a respiaratory
disorder, and does not in cardiac problems.
 Chapter 7
SEQENTIAL SEGMENTAL ANALYSIS

Introduction
- Is a system for describing congenital heart diseases
- It was adopted in 1971 by a consensus
- Heart is seen to develop from partitioning of a whole tube, thus failure of
septation (incomplete or complete) leads to abnormalities.
- This can affect any of the segments listed below

a. Venous segment : SVC, IVC & Coronary sinus

b. Atrial segment : Right & Left atria
c. Ventricular segment : Right & Left ventricles
d. Arterial segment : Aorta & Pulmonary

- It is known that there is connection with all these segments.

In determining the anatomy of the heart and its great vessels without cutting the body open,
sophisticated diagnostic tools are used e.g Plain Chest X-ray
It shows the
o cardiac silhouette
o trachea
o bifurcation of the trachea – left bronchus is usually longer than
th right. Thus, if there is a change, either that it is now shorter or
equal to the right bronchus, it is most likely that there is change
in structural patten of the heart.

A. Atrial Segment
1. Atrial Situs Solitus & Atrial Situs Invertus

- Morphologic right atrium occupies the ward location, and this is called
Atrial Situs

2. Situs Solitus & Situs Inversus

 Situs Solitus Situs Inversus

- When left bronchus is seen on the right in chest x-ray, it is 99% sure that right
atrium is now on the left side

2. Atrial Isomerism ( Right & Left)

- Can be said to occur if the bronchi are of the same length, and both
have the length of one bronchus. For this to occur, it means there will
be a replication of one atrium which could be right or left. If it is right
atrial isomerism, the two bronchi would have the same length right
bronchus, and vice-versa.

Application
To know the precise anatomy e.g a cardiothoracic surgeon who wants to
operate on the heart needs to know the normal morphology of the heart.
The reason is because there could be a change in structural pattern e.g
• Persistent Superior vena cava or IVC
• Right atrial isomerism – which means there are 2 sino-atrial
nodes.
• Left atrial Isomerism

B. Venous Segment

- This is consists of

Right Left
Superior vena cava Pulmonary veins
Inferior vena cava
Coronary sinus

- If there is transposition in the atria, each takes along the veins attached to it.
- E.g if it occurs on the right side, the pulmonary veins are taken along, therby
crossing the midline and can become obstructed.

C. Connection Between the Atria & Ventricle

- In this stage, only chest x-ray is not enough.

- This is described in terms of “type & mode “
a. Type of Connection
Some structural patterns in the heart used to differentiate the right & left
ventricles:

Right Left
Trabecular Pattern Coarse Fine
Valve Mitral (bicuspid) Tricuspid

- If it is a univentricular, the valve is used to identify the side present.

Terms Used
i. Concordance – Is when the right atrium is connected to the right
ventricle, and the entrance os tricuspid valve, and the left atrium is
connected to the left ventricle, and the entrance is guided by mitral valve.
This is type of atrioventricular connection is said to be concordant.

Therefore to have concordance, the following requirements must be met:

a. right atrium must be connected to the right ventricle
b. left atrium ust be connected to the left ventricle
c. situs solitus

ii. Discordance – Occurs when the ventricles translocate, with mitral valve
now on the right side and tricuspid valve on the left.
To have Atrioventricular discordance, there must be
o 2 distinct atria
o 2 distinct ventricles
o 2 atrio-ventricular ostia
But, the features are
a. right atrium is connected to left ventricle
b. left atrium is connected to right ventricle
c. situs solitus or inversus

iii. Ambiguous - Occurs when in either right or left atrial isomerism in

which the ventricles maintain their normal positions.

Here, the minimum requirements are not met i.e ther are no
2 distinct atria, therefore, it is ambiguous.

b. Mode of Connection

For example, in imperforate atrrioventricular valve e.g tricuspid or mitral

atresia, the type of connection is concordant, but the mode of connection is
such with an imperforate valve. The chordae tendinae & papillary muscles
(tension apparatus) are in the ventricle which has the valve.
 Normal Over-riding Straddling

In Over-riding valve,there is overlap but the tension apparatus is still in the

ipsilateral ventricle. It could occur on the right or left.

Stradling valve – there is overlap, and part of the tension apparatus of the
overlapping valve is in the contralateral side.

Both of these changes can be seen in Ventricular Spetal Defect and repairing it,
doctors should take of them.

D. Ventriculo-arterial Segment

Normal Discordant (Transposition)

For example, in Classical Transposition of Great Arteries (TGA), there are

i. Atrial situs solitus
ii. Concordant atriventricular connection with 2 valve mode
iii. Discordant ventriculo-arterial connection with mode

But in Congenitally Corrected TGA, there is atrioventricular discordance.

Persistent Truncus Arteriosus
Types
I : Common trunk, pulmonary is an off shoot

II : Right & Left pulmonary arteries have common trunk but in the
posterior aspect

III : They are side braches

IV : No pulmonary artery, the lungs are perfused by bronchial arteries.

Has the worst prognosis.
 Chapter 8
CONGENITAL HEART DISEASES -
OBSTRUCTIVE HEART LESIONS

General Information on Congenital Heart Diseases

Incidence of Congenital Heart Disorders In Ibadan (635 cases, 1965 – 1975)

Congenital defects %
1. Ventricular Septal defect (VSD) 35
- isolated is most common
2. Patent Ductuts Arteriosus (PDA) 22
3. Coartation of the aorta (CoA) 12
4. Tetralogy of Fallot (TOF) 10
5. Pulmonary Stenosis (Isolated) 9
6. Aortic Stenosis (AS) 7.5
7. Atrial Septal Defect (ASD) 7.o
8. Transposition of Great Arteries (TGA) 4.5

- The incidence of congenital heart disease is 4 – 9 /1000 of live briths

worldwide.

Classification
1. Left –to Right Shunt
- the essential pathology resolves on that oxygenated blood recirculates in
lungs, therefore cyanosis is not a problem unless heart failure ensues.

2. Right-to-Left Shunt
- Here, desaturated blood bypasses the lungs, therefore, associated with
cyanosis. It is Arterial desaturation.

3. Transposition
- Here, the circulations are in parallel i.e unless there is some points of
mixing (and to compensate), it is totally incompatible with life
 4. Obstruction
- Impede blod flow out of the heart
- It can be intra-cardiac or extra-cardiac, but whichever, there is increased
workload on the heart as a result of the impedance.

Example of
a. Right Ventricular Outlet Tract (RVOT) obstruction – Pulmonary stenosis
b. Left Ventricular Outlet Tract (LVOT) obstruction – Coarctation of aorta
c. Peripheral Obstruction – e.g involving any of the branches of pulmonary trunk

OBSTRUCTIVE HEART LESIONS

Section 1:
PULMONARY STENOSIS

Types
- Based on the parts of the pulmonary trunk
i. Valvar

ii. Infundibular – i.e the fibromuscular band

iii. Supravalvar – is rare. It is associated with hypercalcaemia. It usually

co-exists with similar lesion on the left side (aortic
stenosis)
- It is commonly found infants < 9 months.
- Double Arterial Stenosis – is a syndrome with
dysmorphic feature, it called Williams Syndrome.

iv. Peripheral – May be unilateral or bilateral stenosis of branche(s) of

pulmonary trunk.
- Is caused by i. drugs e.g thalidomide
ii. rubella infection
Incidence
As shown in the table above, the incidence in Ibadan over 10-year period was 9%

Mild to moderate lesions are common in children and adults, but hardly seen in
younger ones.

But, if there is critical stenosis e.g in Unicuspid pulmonary valve, the presentation
would be in the neonatal period.

Types
i. Isolated
ii. In combination with Tetralogy of Fallot.

N.B
o Sometimes, there is pulmonary atresia without VSD, a cardiac emergency. The
treatment is to keep the ductus arteriosus open.

o Pulmonary atresia + VSD

Clinical Features
i. It is more commonly asymptomatic (older children), this is because of the
high pressure on the right side, the foramen ovale remains open
ii. But, critical pulmonary stenosis/atresia with intact septum presents early in
the neonatal period
iii. The symptoms are
o Cyanosis (ASD or PFO)
o Dyspnoea on exertion – is severe
o Left parasternal haeve – due to right ventricular hyperterophy
o P2 is soft and delayed
o S2 may be single ( shows high severity)
o Ejection click – shows that the obstruction is less severe. It is due
to forced or pressureized flow of blood against wall of
pulmonary artery which produces a click.

Investigation

1. Chest X-ray
- Shows post-stenotic dilatation of pulmonary artery – seen as a
pulmonary knuckle (unlike in TOF, it is pulmonary bay)
- There is reduced pulmonary vascular markings.
- (Normal pulmonary vascular markings extend 2/3rd laterally from the
hilum
- Heart size is usually normal except the obstruction is severe.
 2. ECG
- Shows right ventricular hypertrophy which is an overwhelming
evidence.
- In very severe from, T-inversion is seen in V1 to V6 (RV-apex forming)

3. Cardiac Catheterisation – shows

i. Increased right ventricular pressure (gradient)
ii. Normal or reduced pulmonary artery pressure

- Right ventriculography
- Post-stenotic dilatation is seen on the lateral view after injecting the dye.

- demonstration of high pressure gradient between the pre- and post-stenotic areas

4. ECHO
- Has now replaced cardiac catheterization
- Doppler - Sample volume
- Transthoracic echo
- Is now the means of making diagnosis of this condition.
- Cardiac catheterization is still useful, if the pulmonary stenosis is in
combination with other complex abnormality.

Treatment
a. Conservative management : for mild cases i.e the right ventricular pressure is <
60mmHg
b. For severe form where the RV pressure is more than > 60mmHg.
i. there is need for urgent relieve of the obstruction to avoid
formation of hypertrophied muscle which outgrows its blood
supply, causing angina.
Procedures
o Ventriculotomy
o Pulmonary valvotomy/Infundibular resection
 o Balloon Valvuloplasty – the inflation is done with normal
saline (just in case the ballon ruptures)
- Is now an accepted standard procedure
- Can even be done as a case.

Section II:
AORTIC STENOSIS

Types
1. Valvar – bicuspid or unicuspid alone
2. Subvalvar – involves fibromuscular diaphragm and there is elongated stenotic
tunnel
3. Supravalvar – It is a component of William’s syndrome which consists of
o Mental retardation
o Elfin facies
o Hypercalcaemia
o Aortic stenosis
o Pulmonary stenosis

Incidence
It is rare in Ibadan, 0.6% of 635 cases of congenital heart diseases in 10 years

In middle age, Bicuspid aortic valve is quite common. It is usually asymptomatic

with or without calcification presenting in the middle age. (This is seen on lateral
chest radiograph)

In infancy, severe aortic stenosis ± coarctation of the aorta is the commonest cause of
heart failure in the first of life

Clinical Presentation
Mild or moderate - Asymptomatic
- it is an incidental finding of ejection systolic murmur on
right sternal edge of second intercostals space.

Severe : Usually in older patients, presenting with

o Dyspnoea on exertion
o Syncope on exercise
o Angina of effort

Presentation during infancy occurs because of severe obstruction

- It is usually associated with heart failure (hypoplastic LHS)
 Signs
- Pulse is of small volume and slow rising (anacrotic)
- Apical heaving impulse
- Systolic thrill on 2nd right intercostals space (aortic area)
- S2 may be single or there is reversed splitting of S2
- There is ejection systolic murmur on right intercostal space
- ± diastolic murmur
- ± 4th heart sound
- Aortic ejection click (apex)

- In supravalvar stenosis, right arm pulse is greater than the left arm pulse.

- In subvalvar stenosis, murmur is maximum in 3rd or 4th right intercostals space

In supravalvar stenosis, the blood flow produces a jet into right

brachiocephalic artery, thus to right radial artery, causing the pulse to be stronger.

Investigation
1. Chest X-ray
- It is normal initially, with time, post-stenotic dilatation develops. It is
caused by the jet flow.
- Cardiomegaly with prominence of ascending aorta
± caicification of aortic valve in older patient

2. ECG
- May be normal
- Or shows
o left ventricular hypertrophy
o inverted T-wave
 3. ECHO
- Shows the anatomic change
- Shoes thickened valve & left ventricular hypertrophy
- Doppler will demonstrate the turbulence across the obstruction

4. Cardiac catheterization
- To measure the pressure gradient and for left ventriculography

Treatment
a. Mild – Conservative
o Give antibody prophylaxis when goinf for denta or surgical
procedures for over 72 hours ( starting from 24 hours before,
then during the procedure and after)

b. Symptomatic Patient
o Aortic valvotomy
o Valve replacement – if there is calcific stenosis

Section III
COARCTATION OF AORTA

Introduction

Types
i. Concentric
ii. Cylindrical

Sites
i. Thoracic aorta
ii. Abdominal aorta
iii. More commonly, the entire arch can have a complete tubular
hypoplasia

Types
i. Pre-ductal coarctation
ii. Juxta –duatal “
iii. Post-ductal “

Incidence
 - Is the commonest primary cardiovascular lesion causing heart in acyanotic
neonate in 1st week of life.
- It is usually suspected with aortic stenosis
- In Ibadan, it is 2% of 635 cases of congenital heart disease in 10 years in UCH

Clinical Presentation

a. Older Children/Adults
- Is usually asymptomatic because it less severe
- The incidental findings are:
o Reduced femoral pulses
o Upper limb hypertension
o Disparity between upper & lower limb pulses – Normal difference
should be ≤ 20mmHg. If > 20mmHg, it suggests coarctation of the aorta

o Hypertensive encephalopathy
o Increased apical impulse – points to left ventricular hypertrophy
o Continous murmur – over the scapular, is due to collateral flow
o Ejection systolic murmur + Ejection click
o ± other associated anomalies

b. In infancy
- May present early with heart failure
- More commonly, there are
o Disparity between upper & lower limb pulses
o Blood pressure – 20mmHg difference in systolic pressure
in neonate ( normal – Isthmal narrowing)

Investigations

1. Chest X-ray : shows

- cardiomegaly, in heart failure
- ± incidental aortic knuckle
- ribnotching – if collateral circulation is established

2. ECG: shows
- left ventricular hypertrophy
- occasionally right ventricular hypertrophy – if interrupted aortic + VSD +
Pulmonary hypertension.

3. Cardiac catheterisation - to delineate the anatomy

Treatment
i. Urgent surgical relief – for symptomatic patients and those associated with
heart failure
ii. Surgical relief after 6 months of life – for asymptomatic, but has hypertension.
Give medical treatment for the hypertension and heart failure.
Procedures
• Classical resection & End-to-end anastomosis
• Sublaclavian flap repair
• Modified Subclavian flap repair with stent – Its complication is
re-coarctation.
• Percutaneous transluminal ballon aortoplasty
 Chapter 9
LEFT –TO-RIGHT SHUNT

1. Ventricular Septal Defect

2. Atrial Septal Defect
3. Patent Ductus Arteriosus

Section I :
VENTRICULAR SEPTAL DEFECT
(VSD)

Introduction
- Is the most common congenital heart disease

Incidence
- The worldwide incidence of congenital heart diseases is 4 – 9% of live births,
out of which, isolated VSD accounts 25%. In Ibadan, it is 35%. In some other
coutries, it is 40%.

Pathogenesis
- Is due to failure of complete of a portion of the intervening septum. It can be
of variable size depending on the stage in which the portioning was arrested.

Factors Predisposing to Congenital Heart Disease

- These factors exert their effect during the frist half of the first trimester, i.e
during morphogenesis – 12th day to 6 /8th week of intra-uterine life.
- Examples are
i. Irradiation
ii. Drugs – taken by the mother
iii. Specific chromosomal anomalies
iv. Specific gene defects

Pathophysiology

Flow Chart 1
If the defect is small, the haemodynamic change is not significant, but in a large
defect, a large amount of stroke volume is directed to the right.
 i. Left atrium
↓
ii. Left ventricle (initially, it is compliant) → Left ventricular outflow tract
(continues in the flow chart below)
↓
iii. Ventricular septal defect - a sizeable portion flows through it. The reason why
blood is flowing from the left to the right is because of higher pressure on the left (peak
systolic pressure is 130 -140mmHg) than on the left side (Peak systolic pressure in right
ventricle is 30mmHg)
↓
iv. Right Ventricle – Note, when this ventricle is receiving the shunted blood, it’s
also pumping its own blood received from the right atrium. Therefore, it is pumping larger
amount of blood to the lungs
↓
v. Pulmonary artery
↓
vi. Lungs – recieves large amount of blood that are not used to, thereby resulting into
reflex vasospasm in response to the high pressure.
↓
vii. Pulmonary veins
↓
viii. Increased venous return - causing high end-diastolic volume of the right
ventricle
↓
ix. Increased preload to the heart (Left atrium & ventricle)
↓
x. Increased tension of left ventricular wall
↓
xi Causing corresponding increase in ventricular pressure
↓
xii. Thus, more blood is pumped via septal defect
↓
xiii. This sets up a VICIOUS CYCLE.
 Flow Chart 2

Simultaneously, in systemic circulation, the following changes are taking place.

i. Left ventricle – the stroke volume is reduced since part of its blood is already
shunted
↓
ii. Aorta
↓
iii. Peripheral tissues - the reduced stroke volume causes hypoperfusion of tissues
↓
iv. Vasomotor centre – In an attempt to compensate, there is increased tonic
discharge to the heart to increase the heart rate.
↓
v. Increase in heart rate
↓
v. Leads to shortening of the systolic time interval, which results in further
↓
vi. Reduction in filling of left ventricle
↓
vii. Thus, increases the dampening of blood into the lungs – causing Wet lungs
↓
viii. Makes lung to be less compliant
↓
ix. Oxygen transfer is compromised, resulting hypoxia
↓
x. The hypoxia stimulates the apneustic centre in brain which now summons the
accessory muscle of respiration to help.
↓
xi. Indrawing of intercostals & subcostal spaces

- The vicious cycle set up above is made worse by the increase in heart rate by
stimulation of vasomotor centre.
- The dampened blood into the lung and reflex vasospasm cause thickening of
pulmonary vessels.
 - This causes increase in pulmonary vascular resistance, thus increasing the
afterload of right ventricle.

- So also, the hypoperfusion in the peripheral tissues is perceived by the kidney

as hypovolaemia, and thus. Initiate mechanism of fluid retention which
eventually worsens the heart function by increasing its preload. This effect
occurs much later. Concerning, the effect on the heart, dilatation occurs first,
then hypertrophy.

- Continuing increase in the preload of the left ventricle results in left

ventricular failure, which therefore occurs before right sided heart failure in
left –to-right shunt.

- Right-sided heart failure results from increasing preload (from fluid retention)
and afterload ( increased pulmonary vascular resistance)

- The whole system becomes decompensated and collectivel result into

congestive heart failure
N.B
What a frustrated child!

Clinical Presentation

Symptoms
i. Breathlessness
ii. Panting
iii. Recurrent chest infection – often has bouts of pneumonia
iv. Fever – from infections, if complicated by infective endocarditis, the fever may
linger on.
v. Failure to thrive
vi. Small for gestational age – both from hypoperfusion
vii. Unconsolable cry
viii. Excessive sweating – from increased adrenergic activity
ix. Cold clammy extremities
x. Paliptations – mother noticed when the child is carried to her chest
xi. Difficulty in feeding (sucking) – since the baby can’t hold its breath to suck

Examination
Inspection
i. Increased praecordial activity – It usually occurs at the onset. Seen at
the apex it is due to medial & anterior rotation while pumping in early
phase. Heaving occurs when there RVH & LVH occur later.
ii. Praecordial bulge
 iii. If in heart failure, the following would be seen
o Sweating – especially on the forhead & face
o Breathlessness
o Subcostal & intercostals recession
o Moist palm – from adrenergic activity, causing increase in
sweat gland activity
o Reduced urinary output – due to the effect of ADH.

Palpation
i. Cold clammy extremities
ii. If in heart failure, there would be poor pulse volume ( in radial,
femorals, dorsalis pedis)
iii. Evidence of systemic congestion
iv. Tender hepatomegaly – the acute liver congestion stretches the capsule,
thus makes it tender. It is the hallmark of heart congestive heart failure
v. Blood pressure – May be normal, but once the heart failure occurs,
hypotension ensues.

Ausculatation (Murmur of VSD)

i. Pansystolic/holosystolic murmur radiating to the back (not the axilla).
ii. Has variable pitch depending on the size of VSD)
iii. Is best heard on mid-left sternum
iv. ± diastolic rumble (gallop rhythm)

- Loud P2 - due to pulmonary hypertension.

- When the pressure on the right now rises above the pressure on the left side,
desaturation occurs on the left side. (Eisenmenger Complex/Syndrome)
- Pulmonary vascular disease oocurs later, it is due to thickening of the vessel
wall, which initially is reversible, but later becomes irreversible. This stage is a
contraindication to surgery, because of suprasystemic pressure on right side. It
causes Psot-cardiotomy syndrome because myocardium requires a lot oxygen
after surgery.

Investigation

1. Chest X-ray : Would show

- evidence of pulmonary plethora i.e increased vascular markings
- evidence of cardiomegaly ( if heart failure has set in)

2. ECG
- Initially, shows signs of LVH, then biventricular heart failure
 3. ECHO
- Shoes the defect
- Doppler colour flow - would demonstrate the left-to-right shunt

4. Cardiac catheterization
– No longer used routinely.
– Is needed only to determine pulmonary wedge vascular pressure
(measured in Woods )in preparation for surgery when pulmonary
vascular disease is suspected.

Treatment
Batch repair under profound hypothermia. Very rarely on bypass.

Section II:
ATRIAL SEPTAL DEFECT
(ASD)

Introduction

Pathogenesis
- The shunting is minimal because the pressure gradient is small, 3- 4mmHg
(Left atrium has 8 – 9mmHg while Right atrium has 5mmHg)

Clinical Presntation
- It is usually asymptomatic in early life.
- In pregnant female, it becomes problematic because of the hyperdynamic
circulation
- In males, it is often an accidental discovery e.g Kanu Nwankwo (Nigerian
Footballer)

Murmur
o Wide fixed splitting of S2
o Pulmonic flow murmur

The heart adapts to the volume overload, and can result in huge dilataion of
right atrium usually (left is usually normal)

Investigation
ECHO – it detects it
 Section III:
PATENT DUCTUS ARTERIOSUS
(PDA)

Introduction
o Ductus arteriosus normally closes 48 hours after delivery.

o It is classified as left-to-right shunt because the flow is from aorta (which has
higher pressure) to pulmonary pressure. Thus, the haemodynamic changes
are essentially similar to VSD. They are both involved with high pressure
system unlike ASD which is associated low pressure.

o It is persistent patency of a normal fetal structure between the pulmonary

artery and descending aorts.

Mechanism of Closure
- It is well established
- It is caused by prostaglandin effect (as indomethacin has a dilatatory effect)

Clinical Presentation
- Depnds on the size
- Small defect is insignificant while large defect may result into congestive heart
failure ( just as in VSD)

Features
i. Small for gestational age
ii. Tachypnoea
iii. Features of congestive heart failure
iv. Bounding Pulses
v. To and Fro / Continous murmur – very charactersistic in the infraclavicular
area. It is continous because it spans both systolic diastolic, with higher pitch
in systolic and lower in diastolic).
 Chapter 10
RIGHT-TO-LEFT SHUNT

1. Tetralogy of Fallot
2. Transposition of Great Arteries
3. Tricuspid Atresia
4. Others :
o Truncus arteriosus
o Total Anomalous Pulmonary
Venous Connection

General Infromation
This group is manifested by cyanosis, therefore, ir is called Cyanotic congenital heart
diseases.

Section I :
TETRALOGY OF FALLOT
(TOF)

Introduction
- It is the commonest cyanotic heart disease worldwide.

Incidence
• It forms about 10% of all congenital heart disease

Pathology & Pathogenesis

It consists of 4 major components
i. Pulmonary stenosis – is usually in the infundibular region
ii. Right ventricular hypertrophy
iii. Ventricular Septal Defect
iv. Over-riding aorta – dextropositioned i.e move to the right

From embryology, heart develops from a single heart tube which is divided by a
septum (ventricular & arterial).

In TOF, the arterial septum divides unequally in which the left half is much bigger
than the right side, and eventually results into pulmonary stenosis & over-riding
aorta. This unequal septation is also responsible for the formation of VSD.
 Haemodynamic Changes
The VSD causes increased pressure on the right side leading which eventually
leads to cyanosis

Clinical Presentation

1. Age – Many are > 1 year before presentation when the child can walk. Some may
even reach 5 – 7years before presenting.

2. Cyanosis
o the time of presentation varies and is determined by the severity of the
obstruction. The more severe the obstruction, the earlier the presentation
and vice-versa.
o Some may not even have any cyanosis initially, but as the patient grows
older, the cyanosis appears because the stenosis becomes more severe.
o The more the cyanosis, the more the symptomatology, therefore, a patient
at birth presents earlier.
o It hardly seen in dark-skinned people

3. Easy fatiguability

4. Effort intolerance

5. Squatting
o On exertion, the assumes this position or knee-chest position while
sleeping. This position suggests TOF until proven otherwise.
 Squatting position Knee-chest position

o This posture is a physiological adaptation to hypoxia. The mechanisms

involved are
a. Pressure effect of Shunting - When the femoral and popliteal vessels
are folded, there is increase in the resistance and consequently the
pressure in the systemic circulation, which also increase in the left
ventricular pressure (afterload), and finally reducing right-to-left
shunting of blood. More blood is then pumped to the lungs for
oxygenation. This is the major reason.

b. Reduction of venous return - Compression of the femoral & popliteal

veins causes reduction in venous return, thus less blood to right atrium
and reduced pressure on the right side.

6. Failure to thrive – The mother complains that the child is growing as the
children of the age.

7. Hypercyanotic / Hypoxic / Cyanotic blue spells

o The mechanism is that the infundibular region which is muscular goes into
spasm rapidly, thus causing worsened cyanosis.
o The precipitating factors are
i. Emotion /Excessive crying
ii. Excessive cold
iii. Dehydration
iv. Infections
v. Hypoglycaemia
vi. Metabolic acidosis
o The presentation might be like – the child starts to cry uncontrollably, then
the lips become blue, he may even have convulsion, fainting or syncopal
attacks from hypoxia.

8. Convulsion – from hypercyanotic spells or bleeding into the brain.

9. Cardiovascular accident/stroke – caused by polycythaemia (e.g PCV 88%) that

develops from the chronic hypoxia. The polycythaemia leads to hyperviscosity
syndrome in which the slowing of blood flow results into formation of clot which
can lead to thrombotic or embolic phenomenon.
 10. Bleeding diathesis – also resulting from polycythaemia

Physical Examination

Inspection
1. Small for gestational age – is seen often

2. Cyanosis – of varying degree depeneding on the severity, age of the patient. It

may be absent in the young patient.

3. Polycythaemia – is a physiological response to chronic hypoxia. It is recognized

by :
a. Conjunctival suffusion – the conjunctiva is reddened beause the vessels
become thick and tortous vessels.
b. Reddened bucal mucosa

4. Digital clubbing – depends on the degree of

5. ± Breathlessness – due to polycythaemia or sometimes metabolic acidosis

(compensatory hyperventilation), but is not due to heart failure or pulmonary
congestion).

Palpation
6. Left parasternal haeve – due to right ventricular hypertrophy

7. Systolic thrill along left upper sternal edge

Auscultation
8. Single and soft S2 - results from pulmonary stenosis

9. Murmur – it is either an ejection systolic murmur at the left upper sternal edge
(pulmonary area) or murmur of VSD – a pansystolic murmur at the left lower
sternal edge. Murmurs of VSD and pulmonary stenosis are not heard at the same
tim, one is usually heard to make the diagnosis.

Investigation
1. Full blood count – Polycythaemia
- Thrombocytopaenia – contributes to the bleeding diathesis

2. Blood film – It is paradoxical it often shows iron-deficiency (not iron-deficiency

anaemia). The reason is because of many red cells and inadequate iron. Such
patient must be given iron supplement.
3. Electrolytes & Urea – shows metabolic acidosis

4. Blood gases – The picture seen in cyanotic heart disease is below

o Reduced PO2 (hypoxia)
o Reduced pH
o Increased PCO2
The procedure involves taking arterial sample from right radial artery
after given 100% of oxygen for 10 minutes. It is done here routinely.

5. Chest X-ray - Shows the following

o The heart size is often normal
o Right ventricular hypertrophy
o Pulmonary bay – due to the small pulmonary artery
o In all, the heart is boot-shaped. This is only suggestive and not
diagnostic of TOF

o Evidence of right sided aortic arch. It is present in 25 – 30% of patients.

It is also seen in other conditions
o There is reduced pulmonary vascular marking (pulmonary oligaemia)

6. Electrocardiogram (ECG) - Shows

o Right atrial emlargement (RAE)
o Right ventricular enlargement (RVE)
o Right axis deviation (RAD)

Note – RVH and RAD occur together often, but not always, it depends
on the severity of the hypertrophy.
 7. Echocardiogeaphy - The 2-dimensional mode shows
o RVH
o VSD
o Over-riding aorta
o Pulmonary outflow tract stenosis

8. Cardiac catheterization – Right sided study shows

o Increased right ventricular pressure
o Reduced pulmonary artery pressure – because of
the obsturuction
o Reduced saturation in aorta (never above 95%)

9. Angiography – The dye flow shows right to left shunt via the VSD. The
pulmonary artery size

Complications

1. Complications due to chronic hypoxia

i. Growth retardation
ii. Metabolic acidosis

2. Complications due to polycythaemia

i. Cardiovascular accident
ii. Cerebral abscess
iii. Iron-deficiency
iv. Bleeding disorders

3. Hypercyanotic spells

4. Infective endocarditis – they are prone to

Mangement

Can be medical or surgical

A. Medical treatment – there are 2 gropus

a. Those who are relatively stable
b. Management of hypercyanotic spell – this is an emergency

a. For Relatively or Fairly Stable Patient

i. Propanolol (B-blocker) - to reduced the tendency of the infundibulum
of going into spasm.
Dose : 1mg/kg/dose, 2 - 4 times/day
ii. Plasma exchange / Erythropoiesis
• Is indicated when the PCV > 60% because of the risk of CVA or
cerebral abscess.
• The procedure involves removing the thick blood in aliquots and
replace it with plasma preferrably ( or normal saline which is
good enough). Plasma & normal saline are used because they
stay in the intravascular space. Do NOT use glucose.
• The formula to calculate the volume to be is below

Actual PCV – Desired PCV

Exchanged Volume = ________________________ X Blood volume
Actual PCV

Blood Volume = 80ml X Body weight

E.g Calculate the volume required to do plasma exchange for a

10kg-weighed patient with PCV of 80% and bring the PCV to 55%.

(Answer = 250mls)

Precautions
i. The PCV should be brought to about 45 – 55% , and not to
normal because of the present cyanosis.
ii. Do not take > 20 – 25% of patient blood volume at once (after
calculating) to prevent acute metabolic acidosis).

Note
Plasma exchange is only a temporary measure because the
compensatory production of red blood cells still continues. It only helps
to prepare the patient for surgery.

Complications
i. Transfusion complication e.g infections
ii. Air or fat embolism
iii. Anxiety – arises because the procedure is invasive, therefore
premdicate with morphia, before and after the procedure.
iv. Metabolic acidosis – therefore, premedicate with sodium
bicarbonate before and after as well.
b. Treatment of Hypercyanotic Spells
- Is an emeregency to avoid brain damage

Steps
i. Put the patient in knee-chest position – most important
ii. Give oxygen by face mask or nasal cathter
iii. Give intravenous propanolol

Dose : 0.1mg/kg
• Is different from oral drug
• Alternative is subcutaneous or intramuscular morphia
(has the dose).
• It relaxes the infundibulum
- Side-effects of morphia are
i. addiction
ii. has narrw therapeutic range
- Nalophine is the antidote for morphine toxicity

iv. Give Sodium bicarbonate

Dose : 1mEq/kg

B. Surgical treatment
Can be divided into
a. Palliative
b. Definitive

Palliative Surgery
1. Blalock-Taussig Shunt – It is a close heart surgery that involves shunting
blood from systemic into pulmonary circulation.
 Types
i. Classical – Subclavian artey is cut and anastomse with pulmonary
artery
-- Post-operatively, no radial pulse is felt. ( This is one for
checking equality of pulse on examination),collateral arteries
open up to supply the limb.

ii. Modified – Subclavian artery is not divided, a graft is interposed

between the 2 vessels. The graft is called Gore-tex graft.
Thus the radial artery is intact at the end of the surgey.
-- It is used for smaller patients

Disadvantage
i. Patient will outgrow the graft
ii. Clot formation

Indices of Succesful Shunt

i. Immediately, shunt murmur which is a continous like in Patent
ductus areriosus, is heard. But in practice, it is not often heard,
though the operation is successful.
ii. Cyanosis should start disappearing
iii. The symptomatolgy should be much better i.e patient becomes more
fit and reduced risk of hypercyanotic spells.
Objective Criteria
i. PCV should strat falling and stabilize at 45 to 55%
ii. Chest –x-ray : No more pulmonary oligaemia

Definitive Correction
Is open heart surgery to close the VSD. It requires cardiolplumanry
bypass(Heart-Lung Machine)
 Section II :
TRANSPOSITION OF GREAT ARTERIES
(TGA)

Introduction

Normal TGA

- Atrioventricular concordance - Atrio-ventricular concordance

- Ventriculo-arterial concordance - Ventriculo-arterial discordance

• The systemic and pulmonary circuits are separate making the condition to
incompatible with life unless there is a connection to compensate, which can
be at any lof this level:
o Atrial level (as atrial septal defect)
o Ventricular level ( as Ventricular septal defect)
o Arterial level ( as Fistula)
 For example, in those associated with VSD, there is right-to-left shunt,
but this is not why TGA is classified as Right-toleft shunt, it is because
SVC and IVC wnd as aorta. Don’t even call it right-to-left shunt, instead
blood flowing to the lungs is increased

Clinical Presentation
i. It most often presents at birth with
ii. Breathleness – because of the increased blood flow tot the lungs.
iii. Cough
iv. Hear failure
v. Failure to thrive

Physical Examiantion
i. Cyanosis
ii. Breathlessness/Dyspnoea
iii. Chest signs – for pulmonary oedema & infections
iv. Features of heart failure – as it is often associated with it (note, it is not often
associated with TOF particularly if the defect is large)
v. On auscultation
o Loud S2 which could be either loud A2 or P2.
A2 – becauses aorta is now anterior to pumaonary artery
P2 – Because of increased blood flow in pulmonary artery,
though it lies behind the aorta.

o VSD murmur – if VSD is present

o ASD murmur _ if ASD is present

Investigation

1. Chest X-ray : Shows

o Egg-on-side appearance of heart

o Right ventricular enlargement (RAE)

o Right atrial enlargement (RAE)
o Increased pulmonary vscular marking (PVM)
o Cardiomegaly
 2. Electrocardiogram (ECG) : Shows
o Right ventricular hypertrophy (RVH)
o Right atrial hypertrophy – occur sometimes
o Right Axis Deviation (RAD)

3. Echocardiography
o To define the structural anomaly using 2-dimensional mode

4. Cardiac catheterization
o Is diagnostic and therapeutic
o But echocardiography is preferred dor diagnosis.
o Therapeutic use is throught right-sided study passing a ballon
catheter inot the right atrium, crossing foramen ovale into the
left atrium, then inflate the balloon, and pull it back to make the
foramen ovale patent and creating atrial septal defect. This is
called Rashkind ‘s Atrial Septostomy

Management - Medical & Surgical

a. Medical
1. Treatment of heart failure – Diuretics & Digoxin

b. Surgical
• Is the mainstay of treatment

Options
1. Blalock – Hanlon Atrial Septectomy – Is not an open heart surgery. It is
done to create opening for mixing of blood.

2. Mustard Operation – Involves removing atrial septum, and divet all blood in
right atrium into left atrium & left ventricle using Atrial Baffle. The patient
becomes pink dramatically. The prognosis is good, but it is an open heart
surgery.
 3. Arterial Switch – Is open heart surgery like Mustard operation.

Congenitally Corrected Transposition of Great Arteries

There is
• atrioventricular discordance
• Ventriculo-arterial discordance

There are usually associated lesions like

i. VSD
ii. Pulmonart stenosis
iii. Bleeding disorders
iv. Valve incompetence

There should be no symptoms.

 Section III :
TRICUSPID ATRESIA
(TA)

Introduction
• It is much less common than the first 2 conditions.
• It is also called Univentricular (is the commonest example of univentricular
heart disease)

• Right ventricle is rudimentary

• The right connection is absent.

Clinical Presentation

• It varies, and depend on ventriculo-arterial connection.

a. If there is Ventriculo-arterial concordance i.e RV →PA, the

features would be due to reduced pulmonary blood flow,
therefore presents haemodynamically similar to TOF.

b. If it is Ventriculo-arterial discordance i.e RV→ Aorta & LV→

PA, the patient presents with cyanosis & heart failure.

• In summary, the presentation consists of cyanosis ± heart failure

Investigation

1. Chest X-ray
• the features seen are variable
• Odd-looking shaped heart because the right ventricle is absent
• There may be increased (as in TGA) or reduced pulmonary vascular markings
 2. ECG
• Is often suggestive (not diagnostic). It shows
o Right atrial hypertrophy – is large if ASD is small and vice-versa
o Superior axis deviation

o Left ventricular hypertrophy

These features together in a cyanosed child is suggestive of tricuspid
atresia
3. Echocardiography - is very diagnostic

4. Cardiac catheterization – shows no entrance into right ventricle

5. Angiography

Management

• Most patients usually give up in first year of life if no intervention is taken.

• Treat heart failure
• The definitive treatment is surgery :
Fontan’s operation – a classical operation for those ho have reduced
blood flow. The procedure involves connecting right atrium to pulmonary artery.
Valve conduit is used to allow blood to flow in one direction i.e to pulmonary
artery.
Differential Diagnosis of Tetralogy of Fallot

• In TOF, patient is usually older e.g 7years while in TGA & TA, they die earlier.

• A 6-month old patient in heart failure & cyanois

The differentials are : i. TGA
ii. Tricupid atresia
• A 6-month old with no heart failure
The differentials are
i. Tetralogy of Fallot – think of it first
ii. Tricuspid atresia
Note – TGA usually associates with heart failure. There are other
differentials e.g
iii. Pulmonary atresia ± VSD, with VSD, consider it as extreme
TOF, but it presents much earlier
iv. Tricuspid atresia or Univentricular heart disease.

• When you see heart failure with cyanosis in older children, think of Eisenmenger
syndrome.
 Chapter 11
ACQUIRED HEART DISEASES

I. Acute Rheumatic Fever

II. Rheumatic Heart Disease
III. Infective Endocarditis
IV. Others : Endomyocardial Fibrosis
Pericardial Effusion

Section I :
ACUTE RHEUMATIC FEVER
(ARF)

Introduction
o It is the commonest acquired heart disease in children in developing countries
(used to be common in developed many years ago, but it’s been controlled by
effective treatment of throat infection.
o It is a precursor of rheumatic heart disease

Incidence
- Sex : It is in slightly female (is about equal)
- The first attack is usually between 5 – 15 years(it is uncommon < 5 and >
15years). This suggests immune basis.

Risk factors
i. Overcrowding – since it originates from pharyngitis
ii. Low socio-economic status

Aetiology
Group A B-haemolytic streptococcus of lancefield

Pathogenesis
- It is only those causing pharyngitis that causes ARF (while skin sepsis,
cellulitis causes acute glomerulonephritis.

- The organism in the throat generates antibody or Antigen-antibody

complexes which get deposited in heart and joints. Note, it is not everyone
who has streptococcus in the throat that develops pharyngitis and not
everyone with pharygitis develops that develops ARF.
 - The cardiac damage is much more severe than joint damage.
It causes pancarditis (affect the pericradium myocardium, & endocardium)
It can be fatal in the first attack, as in this saying “ARF leaks the joint, but bites
the heart.

- a. Pericarditis – it forms a thin fibrinous sticky exudate, termed bread &

butter or small frank pericardial effusion.
b. Myocarditis – is usally direct
c. Endocardial surface
i. Mitral valve – most commonly
ii. Aortic valve
iii. Tricuspid valve – occasionally
iv. Pulmonay valve - rarely

- The histopathologic diagnosis is by demonstrating Aschoff Nodules which

are aggregates of inflammatory cells. The are pathognomonic of ARF.

N.B
The strain that cause pharyngitis can cause ARF & AGN
Also, both pharyngitis and skin sepsis can result into AGN.

Clinical Presentation

Because of non-specificity of the symptoms, Bence-Jones criteria was set, and it has
been reviewed.

Bence –Jones Criteria

Major : “CASES” Minor : 3clinical & 2 laboratory
1. Carditis 1. Fever
2. Arthritis 2. Arthralgia
3. Subcutaneous nodules 3. Previous history of fever
4. Erythema marginatum 4. Increase in Acute phase reactants
5. Syndenham’s chorea 5. Electrocardiogram

Major Criteria
1. Carditis
- is the commonest mode of presentation
- is the most important
- May come down with heart failure
- Patient may present with murmur (diastolic at the apex), due to the valve
involvement. Care-coombs murmur is heard in acute phase of ARF ( is
different from murmur of mitrals incompetence)
- Pericardial friction rub – if the fluid is small
 - Tachycardia – due to fever

2. Arthritis
- is defined as pain & swelling of joint (only pain is arthralgia). It is usually
reddened.
- It is self –limitng
- It is characterized by
o Tends to affect large joints e.g shoulder etc
o It is fleeting/migrating/flitting (most patient present with this)

3. Subcutaneous nodules
- small peanut nodules, usually over bony surfaces of the body e.g face, medial
side of leg
- it shows on-going carditis

4. Erythema marginatum
- A skin transient rash which often goes within few hours or days
- Is not seen commonly in Negroids
- Mainly attacks the trunk, but spares the face
- Comes and goes in a day or week

5. Syndenham’s chorea /St. Vitus’ dance

- A CNS manifestation due to involvement of basal ganglia
- Commoner among adolescent girls
- starting by droppingobjects – to involuntary movement

Minor Criteria

3 Clinical Criteria
1. Fever – may be mild/moderate/severe
- oftens fluctuating

2. Arthralgia – joint pain without swelling

3. Previous history of ARF

2 Laboratory Criteria
4. Increase in acute phase reactants
a. White cell count especially neutrophils
b. Erythrocyte sedimentation rate (ESR) – often raised in acute
inflammation – done in Ibadan
c. C-reactive protein – is not routinely done in Ibadan
 5. Electrocardiogram – shows prolonged PR interval
Normal Range
Children – 0.16s
Adolescents – 0.18s
Adult - 0.2s

Another Criterion : Evidence of Recent Streptococcal Infection

1. Positive throat culture – is not very good
2. Antistreptolysin O titre (ASO) – much more useful.
o If > 250 TODD unit/i.u is suggestive of recent infection
o If > 333Todd unit/i.u is suggestive of ARF

Diagnosis
o Is made clinically in UCH
o 2 major criteria or 1 major & 2 minors

Note, each attck predisposes to more attacks, and this is the reason for long term
prophylaxis.

Differential diagnosis of ARF

1. Malaria
2. Sickle cell disease – usually characterize with bone pains and not joint pains
septic if there is septic arthritis.
3. Leukaemoid reaction (Acute leukaemia)
4. Rheumatoid arthritis – joint pains tend to by symmetrical and affecting small
joints of hand and feet. It is not flitting.
5. Infective endocarditis

Treatment
1. Bed rest
2. Aspirin as as anti-inflammatory, antipyretic & analgesic agent e.g aspirin
(salicylate), it’s maximum dose is 100mg/kg/day in didvided doses

Side effects of Aspirin

i. Gastric erosion – must be 3 – 4 times a day with meals
ii. Metabolic acidosis – therefore, monitor electrolytes and urea

3. Corticosteroid e.g prednisolone, 2mg/kg/day

- Is used often for patients life threatening carditis

Side efefcts
i. Salt and water retention – due to mineralocortoid effect
ii. Glycosuria –therefore , monitor with urinalysis
 3. Treatment of heart failure – with digoxin & diuretics

4. Procaine Penicillin
– streptococcus are very sensitive to it
– Is taken for 7 – 10 days
– Is used to wipe the organism (antigen), thus preventing further
production on antibody.

5. Long term prophylaxis

– to prevent the second attack which is even more severe than the first.
– It is usually given for 3 years after the first attck, but should be
prolonged in those who have worse heart attack.
– Benzathine penicillin (Penadur) – is long-acting

Dose : 1.2 mega /month

(Adult dose is 2 – 4 mega)

- Alternatives are ( not as effective as benzathine)

o Oral penicillin (Penicillin V)

Dose : 125 – 250mg twice a day

o Oral erythromycin

Dose : 125 – 250mg.bd

Prognosis
Those with cardiac involvement should stay longer in the hospital.

Monitoring
o Check the well-being of the patient
o Rate of fall of temperature
o Check sleeping pulse rate
o ESR should be fallin.
 Section II :
CHRONIC RHEUMATIC HEART DISEASE
(RHD)

Introduction
- Implies permanent valvular damage with chronic cardiac decompensation

Pathogenesis
The falby valves healed by fibrosis.

Types
1. Mitral incompetence – is the commonest lesion in paediatrics
2. Mitral stenosis – Patient is usually older because time to develop.
3. Aortic incompetence
4. Aortic stenosis – not common in children. It is more likely to be congenital in
children and rheumatic in adult.

Clinical Presentation
± Previous history suggestive of acute rheumatic fever

Symptoms
i. Breathlessness
ii. Cough
iii. Palpitations
iv. Easy fatiguability
v. Pedal swelling
Note, “iv” and “v” are features of decompensation

Physical Examination
i. Active praecordium (at apex)
ii. Increased JVP
iii. ± Loud P2 - heard very often, it is due to backflow of blood from left atrium
into lungs.
iv. Apical pansystolic murmur – loudest at apex, radiating to the axilla. This is
murmur of mitral incompetence.
v. ± diastolic murmur at the apex – Mitral stenosis
vi. ± diastolic murmur in aortic area, along with collapsing pulse – Aortic
incompetence.
vii. Signs of heart failure – tender hepatomegaly
Investigation

1. Chest X-ray : shows

o Cardiomegaly
o Hump on left border/mitralised left heart border
o Splaying of the carina
o Double density right heart border
o Pulmonary congestion

2. Electrocardiogram : shows
o Evidence of left atrial hypertrophy/enlargement

a. Broad & flat b. P. mitrale/Notched c. Biphasic

- seen in mitral disease
o Increased PR interval
o Left ventricular hypertrophy
o ± Rgiht ventricular hypertrophy – if there has been long standing
hypertension

3. Echocardiogram – very useful in diagnosis especially the M-Mode

4. Cardiac catheterization – is invasive (not commonly used)

Treatment
Treatment of heart failure – Is the mainstay
o Digoxin & diuretics (ACEI is now being used, though not yet in
Paediatrics)

Surgery – Valve repair

Valve replacement

Prevention

Primary Prevention
i. Prevention of ARF – Recognise sore throat and treat it with procaine
penicillin (though most sore throat are usually viral)
ii. Health education

Secondary Prevention
i. Prevention of recurrence of ARF – using Benzathine
penicillin/Erythromycin
ii. Health education
Difenrential Diagnosis of Chronic Rheumatic Heart Disease

1. Endomycardial fibrosis – Left ventricular is not as common as Right ventricular

EMF whereas RHD commonly affects the left.
2. Other carddiomyopathies
3. Congenital Mitral incomptence
4. Congenital Mitral stenosis
5. Congenital Aortic incompetence – especially in those who are of low age.
6. Ventricular septal defect – murmur is usually loudest at the left upper sternal
edge.
7. Sickle cell disease

Section III :
INFECTIVE ENDOCARDITIS
(IE)

Introduction
o Is a septicaemic illness that causes damage to the endocardium & heart valves.
( Unlike in Acute rheumatic fever which is due to antibody formation)

Aetiological classification
a. Acute and Subacute
b. Bacterial and Non-baterial

Subacute
i. Is commoner in children with underlying heart disease.
ii. Is commoner in patient with pre-existing heart abnormality or
congenital heart disease.
iii. The organism are endogenous
Causes
i. Streptococcus viridans – (found in mouth) – is the commonest
ii. Streptococcus faecalis
iii. Other streptococcus
iv. Staphylococcus
v. Gram –ve organism – Pseudomonas, Klebsiella
vi. Fungi ( in immunosuppression)
Pathogenesis

Damaged Endothelium/Foreign material within the Heart

Vegetation

Sterile Infection

Sterile Infective Endocarditis

Progressesive enlargement

Infected Embolus Proliferation of valve leaflet

Epidemiology
Is commoner among older childrena & adults
Is less common in infancy
No sex predilection

Predisposing Factors
1. Pre-existing heart disorders
a. Congenital heart disease e.g as above
b. Acquired heart disease e.g rheumatic heart disease
c. Following palliative surgery
2. Septicaemic illness especially staph. aureus, Gram –ve organism.
3. Dental caries /dental manipulation
4. Cardiac catheterization
5. Prolonged IV cannulation
6. IV drug abuse
7. Urethral catheterization /bladder instrumentation
 8. Endoscopic procedure
9. Immunosuppression - Is usually associated with fungal infection.
o Malignancy
o Steroid therapy
o Chemotherapy
o HIV

Clinical Presntation

High index of suspicion is required.

i. History of predisposing factor
ii. Signs and symptoms of infection – fever, malaise
iii. Features of heart failure
iv. New or changing murmur
v. Signs of arteriolar embolisation – depends on the site
a. Systemic (mitral or aortic valve or right-to-left shunt)
i. CNS – Cardiovascular accident
ii. Renal – Acute renal failure
iii. Splenic arteries –
iv. Mesenteric arteries – acute abdomen
v. Coronary arteries – Angina, myocardial infarction

b. Pulmonary – Usually occurs in left-to right shunt e.g VSD, Tricuspid

atresia. It causes acute chest syndrome.
I
vi. Mycotic aneurysms – from microabscesses in vessels. It causes weakening of
the wall, causing a false aneurysm e.g brain, it is called Subarachnoihd
haemorrhage

vii. Autonomic manifestations - occurs late. It is common in subacute form

a. Petechiae
b. Splinter haemorrhages
c. Osler’s nodes – painless swelling found in the pulps of finger
d. Roth’s spot – exudates in the eye
e. Microscopic haematuria
f. Nephritis
g. Arthritis
h. Digital clubbing
i. Janeway lesions
Investigation

1. Full blood count

PCV – anaemia
WCC - Leucocytosis
2. Blood culture – take at least 3 samples
3. 2-Dimensional Echo – to outline the vegetation, though it’s not always seen
4. Chest X-ray
5. ECG - and chest x-ray are used to rule out underlying heart disease
6. Histology and Bacteriology of vegetation

Differential Diagnosis

1. Acute rheumatic fever

2. Sickle cell disease – because they are more prone to infections
3. Myocarditis
4. Pericardial effusion
5. Cardiomyopathies
6. Thyphoid & other septicaemia
7. Tuberculosis
8. Immunoproliferative disease e.g leukaemia
9. HIV
10. Collagen disease e.g SLE

Treatment

1. Antibiotics – Is the mainstay of treatment. The drugs should be bacteriocidal. It

is given parenterally.
Organisms Drugs
Strep. Viridans Penicillin (crystalline/IV) – is very good
Ampicillin – used in advanced countries
Amoxycillin – more extensive
± Gentamicin – for Gram –ve organism
± Probenecid – used in advanced countries to delay
the excretion of penicillin.
Staphylococcus aures Cloxacillin + Gentamicin
Unknown organism Penicillin + Cloxacillin + Gentamicin
Anaerobes Metronidazole
Fungi Amphotericin B
Others : Vancomycin, cephalosporins

These antibiotics should be given for a long time, for 6 weeks

 2. Treatment of heart failure – with digoxin & diuretics

3. Surgery
o Valve replacement
o Correction of underlying heart lesions
o Embolectomy
o Removal of mycotic aneurysm

Prohylaxis
1. Identify patient at risk
2. Good oral and dental hygiene
3. Health education
4. Antibiotics – once again, it is the mainstay

Aim
To give antibiotics before any procedure that may cause bacteraemia. The rationale is
to prevent further attacks.

Types
Oral prohylaxis Parenteral prophylaxis

Penicillin or Penicillin
Amoxycillin or Amoxicillin
Erythromycin Vancomycin
Gentamicin – for GUT surgery

It is given 1 – 2 hours before surgery and after.

 Chapter 12
RENAL EMBRYOGENESIS & CONGENITAL DISORDERS

RENAL EMBRYOGENESIS
Urinary system develops from
a. Pronephros
b. Mesonephros
c. Metanephric blastema (definite kidney)

a. PRONEPHROS

- is the earliest and simplest

- is vestigial system found in the cervical region

- it appears at 3weeks of intrauterine life

- It disappears at about the 30 days of gestation, except the caudal end of the
pronephric duct

- Its only function is to induce the appearance of the mesonephros.

b. MESONEPHROS
- Is the 2nd vestigial system which appears as pronephros regresses at about the
5th week of intrauterine life

- Is more advanced, and extends from the lower cervical to upper lumbar
segment

- Glomerular filtration begins aroud the 9th week of foetal life. The tubules
produce urine through to the 11th week.

- Before the tubules disappear at the about the 3rd month, the mesonephros
must contact the metanephric blastema to induce the formation of
metanephros.

- The persistent portion of the mesonephros forms the ureteric bud that
develops into the vas deferens, epididymis, ejaculatory duct.
 c. METANEPHROS

- It is the definitive kidney that starts to develop in 4th month of life.

- Kidney develops from 2 tissues : Ureteric bud & Mesenchyme

a. Ureteric bud : forms the collecting system – Pelvis, Calyces & Collecting
duct
b. Mesenchyme : forms the glomeruli, PCT, loops of Henle & DCT

- The whole nephrogenesis is complete by 34 – 36 weeks of foetal life.

- There is NO increase in the number of neurons after they have been formed,
the increase in size of kidney is due to elongation of the neurons, increase in
collecting tubules and blood vessels. Thus, disorder of embryogenesis may
result in renal agenesis.

- The initial location of the kidney is pelvis, it normally ascend to the lumbar
region, and during that, it rotates so that the ureters are placed medially.

CONGENITAL ABNORMALITIES OF URINARY SYSTEM

Types
a. Minor Aberrations
b. Major Malformations

Minor Aberrations

- The patients with can live normally, but can still develop little problems e.g
Vesico-ureteric reflux.

- Examples
1. Ectopic kidney
2. Double Ureters
3. Horse-shoe kidney

Horse-shoe Kidney
- The kidney are placed in the midline & ureters are anteriorly located leading
to increased risk of
o trauma
o urinary tract infection
o obstructive uropathy
 - It is commoner in Turner’s syndrome
- The risk of developing Wilm’s tumour is 2 – 8 times more common than in
the general population

Major Malformations

- These affecr the kidney functions

- Examples
i. Renal Agenesis
ii. Polycystic Kidney Disease
iii. Renal Cystic Dysplasia
iv. Segmental Renal Hypoplasia
v. Malformations of the Urinary Tract

1. RENAL AGENESIS

- It denotes a total absence of 1 or both kidneys

- It occurs because of the isolated failure of development of ureteric nud or as a
consequence of interference with pronephros –mesonephros-metanephros
system.
- It may be associated with partial or total absence of the genital duct system &
adrenals.

Types
a. Bilateral Renal agenesis
b. Unilateral Renal agenesis

A. Bilateral Renal Agenesis

- There is failure of urine production by the foetus, resulting into
oligohydramnious.
- Many affected infants demonstrate the typical Potter’s facies. i.e

Potter’s syndrome = Potter’s facies + Bilateral renal agenesis

Characteristic Features of Potter’s Syndrome

1. Low set & malformed ears
2. Prominent epicanthic folds
3. Beak-like nose with flattened tip
4. Micrognathia
5. Marked pulmonary hypoplasia – causes still birth and early neonatal death
( from respiratory distress)
6. Clubbed hands & feet
7. Abnormalities of joints
 8. Malformation of genitalia
9. Many are stillbirths and those born alive are frequently low birth weight.

Prognosis
Survival beyond a few hours after is uncommon, and death is usually
due to respiratory distress caused by pulmonary hypoplasia.

Note
Conditions associated Potter’s facies are
i. Bilateral renal agenesis
ii. Hypoplastic kidney
iii. Polycystic kidney

B. Unilateral Renal Agenesis

- Is more common
- The incidence is 1 in 1,500 births
- It is more frequently associated with congenital abnormalities of other
system. e.g
o Oesophageal atresia
o Anorectal anomalies
o Congenital heart disease

- It is aften asymptomatic
- It is seen as abdominal mass during physical examination or incidental
finding during investigation for other conditions.

2. POLYCYSTIC KIDNEY DISEASE

- It is usually bilateral
- The kidney is usually very big and consists largely of cysts in various sizes.

Types
- Based on the mode of inheritance

a. Autosomal Recessive Polycystic Kidney Disease (Infantile)

b. Autosomal Dominant Polycystic Kidney Disease (Adult)

A. Autosomal Recessive Polycystic Kidney Disease

- Is a rare condition, the incidence is estimated to be 1 in 40,000

- It can present at any time from neonatal period to adolescence, therefore
cannot really be called Infantile PKD
- It can be recognized at birth, because the kidneys are so large that they cause
obstructed labour.
- The mode of inheriatance is autosomal recessive.

Clinical Presentation
o It varies age, but it has one constant feature which is involvement of the liver.
o Early death is usually as a result of respiratory complications that may arise
any of the following:
i. Respiratory distress syndrome
ii. Pneumomediastinum
iii. Pulmonary hypoplasia

a. Newborn / Young Infants

o Severe hypertension – is an early feature. It may result into congestive
cardiac failure.
o Potter’s facies
o Failure to thrive (FTT)
o Renal impairment

b. Older Children
o Progressive renal failure
o Failure to thrive
o Sever hypertension
o Congestive cardiac failure
o Portal hyoertension – does not usually occur before the age of 5 years

Variants of ARPKD
1. Perinatal
2. Neonatal
3. Infantile
4. Juvenile – Usually has marked cystic liver in addition to the cystic
changes in the kidney. The patient develops liver cirrhosis
and die of liver failure.
B. Autosomal Dominant Polycystic Kidney Disease

- It is transmitted as autosomal dominance, with almost 100% penetrance by

the 8 – 9th decade. ( 100% Penetrance – means 100% of those carrying an abnormal
gene will express it)
- It accounts for 8% adults requiring End-stage Renal Failure (ESRF)
Mangement in Europe
- The gene is localized to chromosome 16

Clinical Presantation
o Initially, many patients present with symptomless abdominal mass.
o It used to be called Adut PKD, but can be present in childhood, even in
neonatal period.

o But, they usually present from about 40 years (therefore called Adult type) with
the following:
• Abdominal pain
• Haematuria
• Proteinuria
• Hypertension
• Chronic renal failure
• Liver involvement – is present in about 30% of the cases.

3. RENAL CYSTIC DYSPLASIA

- It may be bilateral or unilateral.

- Occurs in 2 major situations

a. In association with congenital obstruction of the urinary tract

– The renal cystic dsyplasia may be bilateral or unilateral

Examples
Bilateral Unilateral
1. Urethral atresia 1. Ectopic ureterocoele
2. Prune-Belly syndrome 2. Ureteric atresia
3. Post-urethral valves

b. In association with certain hereditary syndromes e.g

i. Beckwith-Weidmann syndrome
ii. Zellweger’s Cerebrohepatorenal syndrome

Diagnosis
 - Is histologic
- There is structural disorganization,cortical and medullary cysts are seen.
- There may presence of primitive ducts and cartilages.

Other Cystic Kidney Diseases

1. Medullary Sponge Kidney
2. Juvenile Nephronolithiasis

Beckwith-Wiedmann’s Syndrome Zellweger Cerebrohepatorenal Syndrome

1. Micrognathia 1. Mental retardation
2. Macroglossia 2. Sever hypotonia
3. Omphalocoele 3. Hepatomegaly
4. High birth weight 4. Failure to thrive
5. Microcephaly 5. Prolonged neonatal jaundice
6. Hepatomegaly 6. Facies characterized by
7. Enlarged kidney - High forehead
8. Ear lobe anomalies - brachycephaly
9. Hypoglcaemia - Hypoplastic suprorbital
- Micognathia
- Low ser ears
- Is a cause of neonatal gigantism 7. Eye Findings : Glaucoma, corneal
- Mode of inheritance is sporadic clouding, epicanthal folds, Brushfield spots,
- Is not associated with mental retardation Nystagmus, elevated liver enzymes

4. SEGMENTAL RENAL HYPOPLASIA

- Ask-Upmark kidney

- Transvers scars may be found from cortex to the medulla

- Unilateral hypoplasia, either involving the kidney or a portion is one of the

causes of hypertension in the first decade of life.

- Bilateral hypoplasia usually presents with the manifestations of chronic renal

failure.
 5. MALFORMATIONS OF THE URINARY TRACT

1. Pelvi-Ureteric junction (PUJ) Obstruction or UPJ Obstruction

Introduction
It is the most common obstructive lesion in childhood.

Aetiology
i. Intrinsic stenosis – is the usual cause
ii. Extrinsic cause e.gAccessory artery to the lower pole

Clinical Presentation
i. It is most commonly seen on maternal ultrasonography which reveals foetal
hydronephrosis
ii. Palpable renal mass – in newborn or infant
iii. Abdominal /flank / back pain
iv. Fever – secondary to urinary tract infection
v. Haematuria following minor trauma

Note
- About 60% of cases occur on the legt side
- About 10% are bilateral
- Male : Female – 2 : 1

Treatment
Pyeloplasty – done before the age of 6months of life, to prevent kidney
complications.

2. At Uretero-vesical axis
i. Ureterocoele
ii. Ureteric stenosis or atresia

3. At Bladder level
i. Urachal cyst – presents with urine discharge at umbilicus
ii. Ectopic Vesicae /Bladder Exstrophy
iii. Prune-Belly /Eagle Barret /Triad Syndrome
 Ectopic Vesicae /Bladder Exstrophy

Introduction
• Ectopic vesica is the classical form of bladder exstrophy in which
there is bladder protrusion from defective abdominal wall with
bladder mucosa exposed because of the defective anterior wall of the
bladder as well.

- Occurs in 1 in 35,000 – 40,000 births

- Male/Female – 2: 1
- It varies in severity
Clinical Presentation
i. Supra-pubic mass
ii. Downward displacement of umbilicus
iii. Wide separation of pubic rami in the midline – resulting into waddling
gait (therefore note it as one of the causes of waddling gait)
iv. Associated epispadias
v. Broad scrotum
vi. Urinary incontinence – occurs if untreated
vii. Increased risk of bladder carcinoma – usually adenocarcinoma
viii. Urinary tract infection – avoid this by treating the patient well or
transfer to tertiary hospital for proper treatment.

Treatment
o If the case is seen in areas where facilities are not available, cover it with
wrap to keep it moist and transfer promptly to appropaite centre for
surgery.

Prune-Belly /Eagle Barret /Triad Syndrome

It is characterized by
• Deficiency of abdominal muscles (is not due a defect as in Ectopic
vesicae)
• Undescended testes
• Hydronephrosis

It may be associated with other abnormalities

o Very large bladder & Ineffective bladder emptying
o Musculoskeletal
o Cardiac
o Mal-rotation of the bowel
o Disorder of urethra, uterus & vagina in females
 Incidence
• 1 in 40,000 births
• 95% of all cases occur in male

Aetiology
o It is thought to result from severe urethral obstruction in fetal life.

Prognosis
- Depends on dgree of pulmonary hypoplasia & renal dysplasia.
- 30% develop end-stage renal disease, requiring transplantation.

Physiological Differences Between Neonatal & Adult Kidney

1. There is reduced GFR in foetus & newborn because of high intrarenal pressure and
smaller size through which filtration occurs. Before 1 year of life, GFR is 75% of adult
value as opposed to 30% at birth.

2. Limited concentrating ability (Neonate : 1.006 – 1.012) – This is because infants have
more of anabolic phase, most of the protein intake is used for tissue formation and
little is broken down to form urea.
The tubules are said to respond appropriately to ADH

3. Infants have e relatively state of acidaemia – The reasons are

o The renal threshold for bicarbonate is lower in infants (Apprx. 20 21mEq/L)
than in adults (24 – 26mEq/L), thus leading to wastage of bicarbonate.
o Infants are unable to synthesize ammonia and excrete ammonium, thus
making more hydrogen ions to accumulate.
Extras
As a rule of thumb, if you see any congenital abnormalities, examine other systems
for associations.

What is the difference between association & syndrome

Causes of Antenatal Hydronephrosis ( diagnosed by Ultrasonography)

1. Anomalies of the pelvi-ureteric junction / Ureteric-pelvic junction obstruction #

2. Multicystic kidney #
3. Retrocaval ureter
4. Primary obstructive megaureter #
5. Non-refluxing non-obstructed megaureter # +
6. Vesicoureteral reflux #
7. stricture # +
8. Ectopic ureterocoele # +
9. Ectopic ureter #
10. Posterior urethral valve # +
11. Prune Belly syndrome # +
12. Urethral atresia +
13. Hydrocolpos # +
14. Pelvic tumour # +
15. Cloacal abnormalities # +

# - bilateral or unilateral
+ - bladder may be distended

Note,
Some may be transient during in-utero and become normal at birth. Therefore
the ideal thing to do is a repeat ultrasound after 3 days after even up to 1
month.
 Chapter 13
SIGNS, SYMPTOMS & INVESTIGATIONS
OF URINARY SYSTEM

SIGNS & SYMPTOMS OF URINARY SYSTEM

1. Oliguria
The normal urinary output in the newborn is 1 – 3ml/kg/hour

Oliguria :
Age Normal Urinary Output
Newborn < 0.6ml/kg/hour
Older children & < 300ml/m /day
Adults

Anuria :
Age Normal Urinary Output
Adult < 75ml/day
Children 1ml/kg/day

2. Haematuria – can be divided into gross & microscopic

Causes
1. Infections
i. Schistosomiasis (S.haematobium)
ii. Adenovirus
iii. Tuberculosis
iv. Pyogenic urinary tract infection
2. Trauma
3. Glomerulonephritides
4. Calculi
5. Congenital malformation - hydronephrosis, cysts
6. Vascular disorders
i. Arteritis
ii. Infarvtion
iii. Renal vein thrombosis
7. Tumours
i. Wilm’s
ii. Leukaemia
 8. Bleeding diathesis
i. Thrombocytopaenia
ii. Haemophilia
9. Chemical cystitis e.g cyclophosphamide
10. Sickle cell disease – is due to papillary necrosis
11. Sickle cell trait
12. Bacterial endocarditis

Other Causes of Red Urine

1. Food – especially beetroot & berries (anthocyanosis) and confectionary,
containing vegetable dyes.
2. Drugs e.g
Pyridium (phennopyridine) r’emindiane
Phenothiazines Rifampicin
Desferrioxamine Phenophthalein
Anthraquinine Laxatives e.g

3. Haemoglobinuria
4. Myoglobinuria
5. Urates – in high concentration, it may produce a pinkish tings.

Chemical Tests In Haematuria

o Most depend on peroxidase-like activity of free haemoglobin.

o It is positive in haematuria, haemoglobinuria, Myoglobinuria. But,

oxidizing agents of hypochlorite & microbial peroxidases associated with
UTI may cause fasle positive results.

o Rbc haemolyses fast in stnding urine especially if it alkaline.

3. Proteinuria
Types
i. Mild – e.g in urinary tract infection
ii. Mild to moderate – acute glomerulonephritis
iii. Massive – in Nephrotic syndrome

4. Frequent Dysuria

5. Loin pain
- Both dysuria & loin pain suggest pyelonephritis
6. Enuresis

Definition
- Is involuntary voiding of urine due ti lack of bladder control beyond an
appropriate age in childhood.
- Normally, at about 2years of age, a child controls urine in the day and at
4 years can control at night. Thus, by 5years most children are dry day and
night.
- Enuresis is a fairly common problem that is due to poor toilet rhythm & delay
in maturation of bladder.

Types
1. Primary enuresis – is when a child has achieved dryness for significant
period of time and it implies the absence of detectable underlying pathology.

2. Secondary Enuresis – is the recurrence of wetting in a child who has been

dry for at least 1 year. It is more likely to have a pathological basis.
Causes :
a. Organic
i. Urinary tract infection
ii. Diabetes mellitus
iii. Sickle cell anaemia

b. Psychological disturbances

Management
• Don’t scold the child
• Start achievement cards
• The use of enuretic buzzer alarms
• Drugs – Note, is not used first treatment. Examples are Imipramine ( a
tricyclic antidepressnt), desmopressin

7. Failure to thrive
 INVESTIGATIONS

1. Urinalysis
a. Macroscopic appearance – clear /turbid ?
- Colour?
- Blood-stained?

b. Routine chemical tests : Protein, sugar, bilirubin etc

2. Microscopy - Is done by spinning 10ml of urine and examining the sediment especially
in UTI. Is is abnormal when > 4 – 6 wbc /high power film is seen,
numerous rbc, bacteriuria, wbc/dbc casts.

3. Culture & Sensitivity

4. Renal Function Test

a. Serum Urea : Does not start to increase till half of the nephrons are knocked off,
therefore it is not a good indicator. It is also affected by nitrogen balance and state of
hydration.

b. Serum creatinine
- Is more useful
- It is derived from metabolism of muscles and its production is relatively
constant and its excretion is primarily though glomerular filtration.
- The cut off value is related to the age, but level > 1.2mg/dl is abnormal in
childhhod.

c. Creatinine clearance
- Is the most widely used
- Is a measure of GFR

Clearance = UV
P

- Other methods of GFR estimation using substances that are freely

filtered, but not secreted nor reabsorbed by the tubules.

d. Inulin clearance : is the gold standard against which other tests are judged,
though it is not done routinely.
 e. Radio-isotope methods – are available but expensive
i. 51 Cr – EDTA slop clearance
ii. 99m Tc –DPTA (diethlyene triamine penta-acetic acid
iii. 99m Tc – DMSA (Dimercaptosuccinic acid scintigraphy
iv. Iothalamate

5. Renal Scan
- Is non-invasive
- Shows the size, shape & number of kidneys
- Cannot tell about the excretory function

6. Intrvenous Urogram
- Demonstrate both structure and excretory function
- It is invasive
- Differential proteinclearance
- It is contra-indicated in patient allergic to dyes.

7. Others
a. Differential Protein Clearance
b. Renal Biopsy
c. Serum lipids : cholesterol, triacylglycerides
d. Water deprivation test – for diabetes insipidus
 Chapter 14
OBSTRUCTIVE UROPATHY

Introduction

• Normally, urine flow occurs in a stream & without effort.

• Obstruction results into stasis, dilatation and infection. If it is not diagnosed
early, renal failure ensues.
• Obstruction manifests as
i. dribbling
ii. straining
iii. dysuria
iv. ± reflux of urine
v. Distended bladder ± Urinary ascites
vi. Hydronephrosis – in long standing cases resulting into renal
impairment, failure to thrive, anorexia & vomiting.

Note, Lower tract urinary tract obstruction is the most common cause of neonatal ascites
which is due to rupture of dilated pelvis with extravsation of urine.

POSTERIOR URETHRAL VALVE

Introduction
• It is the most common cause of severe subvesical obstruction in the male
infant. (Note, females do not have posterior urethra)

• Valves appear as mucosal folds in the posterior urethra

• Early obstruction during renal development may result in renal dysplasia

Clinical Presentation

i. Dribbling
ii. Poor urinary streams in neonates & infants
iii. Bladder
All these form triads
iv. ± Symptoms of uraemia
v. ± Symptoms of infection
Complications
The more severe the obstruction, the more the degree of complications.

Later, in infancy
i. Vomiting
ii. Failure to thrive – secondary to chronic renal failure
iii. Dehydration – secondary to diabetes insipidus

Older children
i. Enuresis – may be the presenting complaint.

Diagnosis
o Can be made prenatally by ultrasound which reveals an enlarged bladder &
hydronephrosis.

Investigation

1. Micturating Cystourethrogram – is diagnostic. It shows

o Dilated elongated posterior urethral valve
o Trabeculated baldder
o There may be urinoma or urinary ascites

2. ± Intravenous Urogram – It is not routinely done. It may show extensive

hydronephrosis and hydroureters.

3. Sepsis screen

Treatment

Immediate
i. Fluid & Electrolyte correction i.e
o Metabolic acidosis
o Sodium Depletion
o Dehydration

ii. Establish bladder drainage – by urethral (preferred) or suprapubic

catheterization or vesicostomy(definitive treatment).

In very ill patient, do urinary diversion by bilateral, ureterostomy. It is

rarely done nowadays.

iii. Give antibiotics

Definitive
o Is surgery i.e excision of the valve
o In the past, obliteration with diathermy used to be done. But, recently,
what is done is Mohans valvotomy.

Follow –up
o Is done because of the complications, to monitor for continence,
stricture or development of chronic renal failure

Prognosis
o Depends on the severity of renal damage and dysplasia at the time of
diagnosis. Those who present late have poorer prognosis.
 Chapter 15
GLOMERULONEPHROPATHIES

Clinical Patterns Of Glomerular Diseases

1. Nephrotic syndrome
2. Acute Nephritic Syndrome
3. Mixed Nephritic-Nephrotic Syndrome
4. Acute Renal Failure
5. Chronic Renal insufficiency or Failure
6. Recurrent or Persistent Haematuria
7. Asmptomatic Proteinuria
8. Rapidly Progrssive Glomerulonephritis – the initial presentation is with a mixed
nephritic-nephrotic picture, which then takes a progressive course to renal
insufficiency within 6 weeks and a few months. It may be end result of many
conditions

Aetiology & Pathogenesis of Glomerular Injury

1. Hereditary or Familial factors e.g Nephritis in Alport’s syndrome

2. Immunological factors
i. Antigen-Antibody complex deposition : which activates complement,
system,the prinicipal mediator of injury.
ii. Formation of host antibody to glomerular basement membrane.
Antigen with linear endothelial deposit e.g Good Pasture syndrome =
Rapidly progressive nephritis & Pulmonary haemorrhage.
iii. Activation of alternative pathway by exogenous & endogenous factors
e.g Lymphomas
iv. Deposition of IgA aggregates in the mesangium e.g
o Berger disease (IgA Nephropathy)
o Nephritis of Anaphylactoid purpura
(Henoch Schönlein syndrome)

Complement participates in damage by

o Contributing to inflammatory reaction through adherence to
leukocytes and chemotaxis
o Damage to biological membrane
o Enhancement of blood coagulation
 3. Metabolic or Toxic factors

4. Coagulation disturbance

Minimal Change Disease

- is the commonest type of Nephrotic syndrome in Europe & America
- its aetiology is fully known
- But soluble toxins cause lossof polyanaions on the glomerular capillary & red cell
membrane, thus leading to widespread permeability.

ACUTE GLOMERULONEPHRITIS
(Acute Nephritic Syndrome)

Definition
o Refers to a specific renal disease in which inflammation and proliferation of
cells within the glomeruli are the major abnormalities.

Clinical Presentation
It is characterized by sudden, (often explosive) onset of symptoms of glomerular
injury. These include

i. Haematuria – grossly evidence by coca-cola colour(brownish)

urine. Or it may be microscopic.

ii. Hypertension – thought to be due to sodium and water retention

iii. Oedema - seldom as marked as in Nephrotic syndrome

iv. Oliguria – Urine volume < 300mls/m² /day. This is the amount
required for excretion of minimal solute load.

v. Circulatory congestion i.e pulmonary oedema, heart failure

vi. A varying degree of renal insufficiency

Other Findings
vii. Proteinuria – modest elevation of 30 – 100mg/dl ( i.e + or ++ with
dipstick)
viii. Findings due to reduced GFR
o Azotaemia : (The normal range or urea is 15 – 35mg/dl)

o Elevated creatinine (Normal is 0.5 – 1.2mg/dl)

 o Hyperphosphataemia

o Hyperuricaemia

o ± Hypocalcaemia – is due to hyperphosphataemia

POST – STREPTOCOCCAL ACUTE GLOMERULONEPHRITIS

(A Prototype of AGN)

Aetiology
The agent first noticed to cause it Lancefield group A B-haemolytic streptococcus.

Pathogenesis
o It usually follows a pharyngeal or cutaneous infection e.g pyoderma, scabies
(especially in this environment)
o Nephrogenic strains of strep – Serotype M

a. Pharyngitis- associated AGN : M type 12 - occurs frequently,

others are 1, 4, 6, 25

b. Pyoderma-related AGN : M type 49 and others are 53, 55, 56,

57, 58.
(Remember that there are rheumatogenic strains of streptococcus)

Epidemiology
o Features of post-strep. AGN vary from mild to severe, and mild cases evade
detection, therefore incidence is difficult to determine.
o Male : Female – 2 : 1
o It is commonly seen in early school age
o Peak age is 6 – 7years, it is not common < 2years (No difference with that
Nephrotic syndrome in the Tropics which is 6 – 8years)

Confirmatory Studies
1. Culture of streptococcus – taking from throat or skin swab
2. Serology – is indirect
a. Antistrepyolysin O (ASOtitre) – it starts to rise in the first 10 days,
reaches a peak in 4 weeks and tails off in 6 weeks.
Normal : < 166 TODD unit
Marked rise : > 333 “ .
-It is usually more raised in pharyhgitis-related AGN
 b. Antihyaluronidase : It is more in pyoderma-related than pharyngiti-
related AGN
c. Anti-deoxyribonuclease B – it is raised in both, but more in
pyoderma.

Note : If a to c are elevated, you are sure of the diagnosis.

3. Positive streptolysin test – is a slide agglutination test that incorporates the
above tests. It is a mixture of extracellular products of the streptococcus.

4. Serum C3 estimation – Complement system is activated in post-strep. AGN.

Serum C3 is low for 6 – 8 weeks and returns to normal. If it does not by 8 weeks,
review the diagnosis.

Clinical Presentation

i. The typical picture is previous infection of the skin or the pharynx

followed by a latent period of about 10 days for pharyngitis-associated
AGN and 3 weeks for pyoderma-associated AGN before the
appearance of features of AGN.
ii. Oedema – It is milder than in Nephrotic syndrome. It usually starts
from face, limbs, it is worse on waking up, improves later in the day or
with ambulation.
iii. Hypertension – It falls without recovery
iv. Haematuria – It is gross in 30 – 70% of cases. The brownish /coca-
colour / agbo coloured urine is due to degradation of haemoglobin to
acid haematin. Microscopic haematuria is seen in all cases.
v. Signs of circulatory overload
o Pulmonary oedema – leading to respiratory distress, crepitations
o Heart failure – Tachycardia, Tachypnoea, Gallop rhythm, Tender
hepatomegaly.
vi. Severe pallor – is due to dilutional anaemia, therefore blood
transfusion is not usually considered because as the blood volume falls,
the PCV is restored.
vii. Evidence of pharyngitis or pyoderma – Siblings may have the evidence
of AGN.

Complications
1. Hypertensive encephalopathy – causing convulsion, impaired consciousness,
paresis
2. Oliguric acute renal failure
3. Pulmonary oedema
4. Heart failure
 5. Nephrotic syndrome – presence of massive oedema & massive proteinuria. It is a
rare cause of Nephrotic syndrome in our environment.
6. Retinopathies
7. Rapidly Prgressive Glomerulonephropathies – leading to renal failure. It is
characterized by crescent formation.

Investigation
1. Urinalysis : checking the
o Colour
o Blood
o Protein (it is usully + or ++)

2. Urine micrscopy
o Red blood cells – it is usually +++
o White blood cell cast
o Red blood cast
Casts are proteins filtered but not reabsorbed which form moulds at the
tubules, rbcs attached – rbc casts, granular casts when rbc degenerate. Hyaline casts

3. Serum electrolyte
o Sodium – is usually normal or low
o Chloride – is usually normal
o Potassium – Increased
o Bicarbonate – Reduced
o Urea – Increased
o Creatinine - Reduced

4. Serum Protein
5. Serum cholesterol – is usually normal
6. 24-hour urine – is usually normal (unlike in Nephrotic syndrome)
7. Blood film – for malarial parasites especially for Plasmodium malariae which is
associated with Nephrotic syndrome.
8. Renal biopsy – is rarely indicated.

Indications
i. Low C3 for > 8weeks
ii. Hypertension, persisting for > 3 weeks
iii. Acute renal failure, persisting for > 3weeks
iv. Nephrotic syndrome
v. Proteinuria persisting for > 6months
vi. Microscopic Haematuria for > 1 year
vii. Gross haematuria for > 3 weeks
 Preparations
i. Renal scan
ii. Coagulation profile
iii. Blood grouping and crossmatching

9. Chest X-ray – indicated if heart failure or pulmonary oedema is present.

Differential Diagnosis

1. Post-infectious AGN (from other organisms)

Bacterial : other streptococci
Staphylococci
Mycoplasma

Viral : Echo virus

Cosackie
Varicella
Mumps
Influenza
Hepatitis

2. Berger’s disease (IgA Nephropathy)

- It is used to be called Benign Recurrent Haematuria
- The patient has episodes of haematuria often precipitated by non-
specific viral respiratory infection or febrile episodes.
- Males are more commonly affected
- Progressive disease develop in about 30% of patients, leading to poor
prognosis, with hypertension, reduced renal function & severe
proteinuria between episodes
- Histology predominantly shows mesangial deposit of IgA with or
without IgG, IgM, C3 & Properdin.

3. Causes of Haematuria
i. Alport’s syndrome : consists of Herediatry Nephritis &
deafness + cataract
ii. Nephritis of Henoch-Schonlein Purpura : which manisfests as
purpuric skin rashes abdominal symptoms like pain,
intussception+ GN.
iii. Benign Recurrent Familial Haematuria (BRFH) – is totally
benign unlike IgA Nephropathy. It is precipitated by febrile
illness.
iv. Haemolytic Uraemic syndrome (HUS) : It consists of
o Oligonuria
 o Acute renal failure
o Microangiopathic haemolytic anaemia
o Thrombocytopaenia
v. Polycystic kidney disease
vi. Systemic lupus erythematosus
vii. Polyarteritis nodosa

Treatment

Specific
1. Usually with Penicillin for 10 days – ?this would stop further antigen-
antibody complex formation. If patient is allergic to penicillin, give
erythromycin.
Procaine penicilline, given intramuscularly, once a day
Dose : 25 – 50,000 iu/kg body weight

2. Observe very closely by monitoring

• Blood pressure
• Input/Output of fluids
• Weighing the patient daily

3. Administer antihypertensive

Hydrallazine :
Dose: 0.5 – 1mg/kg IV, stat
0.15 – 0.3mg/kg IV 4 – 6 hourly

Reserpine
Dose : 0.02 – 0.07mg/kg/day

4. For hypertensive encephalopathy, use

IV Diazoxide :
Dose: 5mg/kg by push or IV drip

IV Lasix
Dose: 1 – 2mg/kg

Nifedipine – can be orally

 5. Diuretics – depends on
Using frusemide

6. Treatment of Acute renal failure

• Fluid restriction to
300 – 400ml/m²/day + preious day output

7. Treatment oh hyperkalaemia - using

• Calcium gluconate
• Sodium bicarbonate
• Glucose-Insulin infusion
• Salbutamol
• ± Ion-exchange resin
• ± Dialysis

Prognosis
Is not low. 80 – 90% recover fully without any sequlae, about 10% progress to chronic
renal failure.
It is better in children
 Chapter 16
NEPHROTIC SYNDROME

Introduction
• Is not a disease entity, but a feature of a number of kidney disease (like
neonatal jaundice), therefore, always look for its cause.
• It is the most common chronic renal disease of children worldwide
• It is characterized
i. Heavy proteinuria : > 50mg/kg/day in a 24 hour sample or
> 40mg/m²/hour or
> 1g/m²/day or
2g/m²/dsy
(Note, normal protein loss is < 150mg/dl (-200mg/dl)
ii. Hypoalbuminaemia : < 2.5g/dl

iii. Massive generalized oedema

iv. Hyperlipidaemia (cholesterol > 220mg/dl)

Physiology of the Glomerular Filtration

a. Molecular size: normally, substances with molecular weight > 60,000 Daltons are
excreted minimally.

b. Molecular charge (Charge Selectivity): There is negative charge (sialoproteins &

GAGs) on glomerular membrane capillary wall which retard polyanions in the
blood e.g. albumin, more than neutrally actively charged ones.

Other factors are

• Stereochemistry of the protein – deformability
• Hemodynamic factors – especially those producing alterations in GFR and
glomerular capillary flow.

Pathogenesis

1. Hyperproteinuria
• The dominant cause is the immunologic damage to the filtration pit which
causes excessive loss of protein. Remarkable changes occur in serum albumin
& plasma proteins of similar molecular characteristic e.g.
o Transferin
o Anti-thrombin III
o Factor B of complement
Thus, there is reduction in the serum level of all these.
 Examples of serum protein that are increased in their serum levels are:
o  2- globulin
o B-globulin
o Pre-B lipoprotein
o Fibrinogen etc
• There is increased catabolism of tubular albumin

2. Oedema
Excessive protein loss

Hypoalbuminaemia

Low Oncotic pressure

Loss of fluid into the interstitial space

Reduced intravascular volume

Compensations:
i. Increased ADH secretion – leads to increased water reabsorption
and thus worsen the oedema
ii. Activation of the Renin-Angiotensin_Aldosterone system – leads
to increased reabsoprtion of sodium & water, and loss of
potassium
iii. ? Stimulation of the sympathetic nervous system
iv. ? Suppression of atrial natriuretic factor – leading to Na & water
retention.

Note, all these reasons do not explain the symptom really e.g. hypovolaemia,
hypervolaemia & normovolaema can be seen, hypovolaemia is common in childhood.

3. Hyperlipidaemia
• In response to the hypoalbuminaemia, there is compensatory secretion of
albumin and concomitant production of lipoproteins. Whereas albumin is lost
in urine, the lipoproteins because of their large molecular weight remain in the
serum and bind lipids.
 LDL – Cholesterol
VLDL – Triacylglycerides
HDL –may be high or low
• There is also decreased lipid catabolism owing to reduced plasma lipoprotein
lipase, a major enzyme that removes lipids from plasma.

Incidence
• In temperate countries, the peak age is 2 – 3 years and male/female is 2 : 1

• In tropics, the peak age is 5 – 8 years and because of Quartan Malarial

Nephropathy, there is no sex predilection.

Aetiology
It is divided into
a. Primary – the cause is unknown
b. Secondary – is associated with some diseases

Primary Congenital Nephrotic Syndrome

Is a heterogeneous collection of primary or secondary disease that may share only
the fact that the onset occurs in the 3 months of life.

A notable example is Finish type of Primary congenital Nephrotic Syndrome

- Is rare in many parts of the world but has increased incidence in Finland
- It has autosomal-recessive inheritance
- The child is born with large placenta
- It presents in the first 3months of life
- It does NOT respond to steroid therapy.

Secondary Congenital Nephrotic Syndrome

Causes
1. Syphilis
2. Toxoplasmosis
3. Cytomegalovirus
4. Renal vein thrombosis etc
 PRIMARY NEPHROTIC SYNDROME

1. Minimally Change Nephrotic Syndrome

- is the commonest type in Europe & America
- Under light microscopy, there is no much abnormality, but on electron
microscopy, there is fusion (or better defacement) of the foot processes and
subendothelial deposits

2. Membranoproliferative Glomerulonephritis (MPGN)

- Is disease of the glomerular basement membrane with some proliferation of
the mesangial cells & endothelial cells
- There is deposit of immune complexes on the subendothelial aspect of the
capillary basement membrane.
- Most of the cases in UCH are of this histologic type, although they are
secondary. There is cellular proliferation. Plasmodium malariae is still known
as the commonest cause in Ibadan.

3. Membranous Nephropathy
- Immune complexes are deposited on the subepithelial aspect of the capillary
basement membrane.
- There are spikes of basement membrane-like material
- Some cases e.g. Plasmodium malariae nephropathy & SLE have this type of
histology.

4. Focal Segmental Glomerulosclerosis

- Can be classified into
i. Primary
ii. Secondary
iii. Familial
iv. Syndromic

Primary FSGS
o Is similar to minimally change nephrotic syndrome in
presentation, but has a poorer prognosis. Some scientist believed
it is a spectrum or continuum of minimally change disease,
while the others see it as a disease entity on its own.
 o It responds to steroid initially, but later becomes resistant
o Secondary FSGS is seen in
i. Diabetes mellitus
ii. HIV Nephropathy (seen in blacks)

o The primary FSGS has better prognosis than Secondary FSGS

5. Proliferative Glomerulonephritis
Types
i. Diffuse – as in post-streptococcal AGN
ii. Focal proliferation
iii. Mesangial – IgA deposits
iv. Mesangiocapillary (MPGN)
v. Crescentic – is usually severe and it is seen in rapidly progressive
glomerulonephritis. It progresses to end-stage renal insufficiency.

SCEONDARY NEPHROTIC SYNDROME

1. Post-infectious
a. Protozoal
i. Plasmodium malariae (Quartan Malarial Nephropathy)
- Is very important in this environment. In 1960s, it is responsible for 80% of
cases in Ibadan and 25% in Zaria.
- There is no typical histological picture that is currently accepted but more
show membranoproliferative glomerulonephritis.
- The characteristic lesions of Quartan Malarial Nephropathy are :
o Capillary wall thickening
o Segmental glomerulosclerosis
o Secondary tubular atrophy
o A unique feature of small lacunae scattered throught the
basement membrane containing island of material similar in
density as basement membrane.

Note, in QMN, cellular proliferation is unconspicous or absent

- QMN is an immulogically mediated disorder initiated by quartan malarial

infection which once established pursues a progressive course.
- It is graded into I, II & III depending on the severity of glomerular affectation.
I - < 30% of tubules are affected
II -
III - > 70%
 Treatment
Anti-Plasmodium malariae agent

ii. Palsmodium falciparum

iii. Toxoplasmosis

b. Parasitic
i. Schistosoma mansoni
ii. S. haematobium
iii. Filariasis

c. Viral
i. Hepatitis B
ii. Cytomegalovirus
iii. Varicella
iv. HIV

d. Bacterial
i. Post-strep. AGN – rarely
i. Syphilis
ii. Infective endocarditis
iii. Shunt Nephritis

2. Multisystemic & Connective Tissue Diseases

a. Systemic lupus erythematosus
b. Henoch-Schonlein Purpura (Anaphylactoid purpura) – Some people it
is a continuum of IgA Nephropathy.
c. Sarcoidosis
d. Amyloidosis

3. Allergic disorders
i. Bee sting
ii. Serum sickness
iii. Pollens
iv. Poison oak
v. Poison ivy

4. Drugs & Heavy metals

i. Mercury : in bleachin creams
ii. Lead
iii. Gold – in the treatment of rheumatoid arthritis
iv. Penicillamine – Wilson’s disease
v. Trimethadone – in the treatment of Petit mal epilepsy
 Note, it improves with drug withdrawal.

5. Neoplastic
i. Lyphomas
ii. Leukaemias
iii. Wilm’s tumor

6. Heredofamilial disorders
i. Sickle cell disease
i. Alport’s syndrome
ii. Nail-Patella syndrome

7. Metabolic disorders
i. Diabetes mellitus
ii. Hypothyroidism

8. Miscellaneous
i. Congestive cardiac failure
ii. Transplant rejection

Clinical Presentation
1. Oedema – Usually starts from the face (peri-orbital swelling), then involves the
abdomen, genitalia. The oedema subsides with ambulation.
o It may also be responsible for weight increase despite poor appetite
o Patient presents about 1 – 2 months after the onset of symptoms

2. ± reduced urinary output – depending on the oedema

3. Abdominal swelling – due to ascite
4. ± pleural effusion features
5. Features of infection
6. Blood pressure – is usually normal in the early stage, but in some types e.g.
Membranoproliferative GN, it could be raised.

Investigation

1. Urinalysis
Proteinuria : +++ or ++++
Haematuria
Glucosuria – due to transient tubular dysfunction or Diabetes mellitus

2. Urine microscopy
For rbc, wbc casts
 3. Stool microscopy - for S. mansoni ova

4. Serum electrolyte & urea

o Sodium – Low or normal
o Urea - Usually normal, unless in malnourished or complicated
o Chloride
o Bicarbonate
o Creatinine - normal

5. Serum calcium –the ionized calcium is normal, but total calcium is low because of
low albumin in blood

6. Blood film – for malarial parasite

7. 24-hour Urinary Protein

8. Creatinine clearance

9. Protein Selectivity Index

- Is done to detect the severity of damage
- The glomerulus is seen as a sieve. The lesion is classified as

a. Highly Selective Proteinuria - Renal lesions showing only minimally

change, albumin is lost mainly.

b. Poorly Selective Proteinuria - There is lesion with marked histologic

changes, and loss of larger molecules in addition to albumin.

Protein Selectivity Index = Clearance of Albumin X 100

Clearance of IgG

1. Highly Selective 1 – 15
2. Moderately 15 – 30
3. Poorly > 30%

Only Henoc-Schonlein purpura responds to therapy.

9. Renal biopsy – In the past, it was done for all patients because of steroid resistance.
In Europe, those between 1 – 10 years respond to steroids because the
common type in them is Minimally change Nephrotic syndrome.
 Steroid-resistance or steroid-dependence or frequent relapses are
considered for biopsy.

10. G6PD Assay – Because of the drug used to treat P. malariae whhch causes
haemolysis in G6PD deficient patient.

Treatment

A. Supportive therapy
i. Daily weighing
ii. Monitoring of blood pressure
iii. Urinalysis
iv. Monitoring of Input/Output of fluid

v. Diuretics – using mild and not fast-acting e.g. thiazides is preferred. For
secondary hyperaldosteronism, use spironolactone.

vi. Salt –poor albumin (expensive) + IV lasix or Fresh frozen plasma

10ml/kg + wait for 30 minutes, then give lasix (unlike in adults & other
conditions). Mecahnism of action : the salt-poor albumin or FFP draws
water into the vessels first while the lesix aids the excretion.
vii. Metolazone + Frusemide – require close monitoring

viii. Diet
o Protein of high biologic value (when broken down, gives essential
amino acids). Normal intake should be maintained if the renal
function is normal
o Cholesterol & saturated fatty acids should be curtailed.

ix. Modest exercise should be encouraged to prevent thromboembolic

phenomenon because Nephrotic patients are prone to hypercoagulable
state.

x. No femoral tap – to avoid thrombosis.

B. Specific Treatment
1. Adminstration of steroid : Prednisolone or prednisone

o Primary or idiopathic constitute about 90% of cases in Caucassians and

Minimal change disease is responsible for 85%. It was found out that
90% of these Minimal change disease respond best to steroid.
 o Generally, steroids are helpful in
i. Minimal change nephritic syndrome
ii. Focal segmental glomerulosclerosis
iii. Mesangial NS
iv. Proliferative GN

o In Membranoproliferative GN, steroid is not helpful, it causes

hypertension in good doses. More recently low dose alternate therapy
regimen for a long term
o In those who are steroid-dependent & steroid-nonresponsive, renal
biopsy is done.

2. Cyclophosphamide – is given for those with frequent relapses.

Dose: 2 – 3mg/kg for 2 – 3months

- Side effects : azoospermia in addition to many others

3. Chlorambucil – has been tried and found useful.

4. Azathioprine – is not useful in our environment

5. Levamisole –
6. Cyclosporine – is not used here because it has to be monitored.

For Minimal change disease

Favoured regimen
• 60mg/m/day till remission for 3 – 5days to 2 weeks
• Then reduce it to 60mg/m²/ alternate day for 6weeks to 3
months

Standard regimen
• 60mg/m²/day in divided doses for 4weeks
• Then tail off

Definition of Terms

1. Remission
a. Urinary remission – occurs when 3 consecutive days were with without
abnormal proteinuria (< 4mg/m²/hour which is equivalent to Nil to Trace ).
b. Compete remission – when serum albumin has reached the level of 3.5g/dl

2. Relapse
– denoted by reappearance of proteinuria of > 40mg/m²/hour (Albumin ++ or
greater) for 3 consecutive days.
 – Current definition is the reappearance of oedema (since proteinuria occurs in
proteinuria)
– Frequent relapses : is defined as 2 or more relapses in 6 months or 3 to 4 or more
relapses in 1 year while the patient is off prednisolone.

3. Steroid dependent – Occurs when there is a relapse as the corticosteroid therapy is

being tapered off.

4. Resistance to Prednisolone or prednisone

- Has persistent proteinuria & hypoalbuminaemia after a 4-week (now 6-week)
course of prednisolone at 2mg/kg/day.

Complications
1. Malnutrition – Occurs as a direct consequent of urinary protein loss and
aggravated by anorexia & vomiting.

2. Increased susceptibility to infections due to the following mechanisms

i. Reduced immunoglobulins
ii. Oedema fluid acting as culture medium
iii. Protein deficiency
iv. Reduced bactericidal activity of leucocytes
v. Immunosuppressive therapy
vi. Reduced perusion of the spleen due to hypovolaemia
vii. Loss in urine of factor B (alternative pathway of the complement
system is particularly significant in the opsonization of
encapsulated organism)
They can have Primary peritonitis which is usually due to Streptococcus
pneumoniae infection. Gram-ve organism can also cause it.

3. Hypercoagulable state
– Is due to imbalance between factors that promote coagulation e.g.
elevated fibrinogen, factors V & VIII, and factors that
normally inhibit coagulation. There is urinary loss of
antithrombin III.

4. Loss of transport protein : e.g. Vitamin D binding protein

5. Chronic renal insufficiency & failure

6. Hyperlipidaemia – predisposing to increased riak of ischaemic vascular

disease
Differential diagnosis of Nephrotic syndrome
1. Acute glomerulonephritis
2. Kwashiokor
3. Congestive cardiac failure
4. Chronic Liver disease
5. Beriberi (Wet)

Prognosis
• Is variable
• It is poor in Quartan Malarial Nephropathy, which develops hypertension and
end-stage renal disease within 5 to 7years
• In Minimal Change disease, the outcome is better. Patients may have relapses tii\ll
the 2nd decade.

Note the following on Quartan Malarial Nephropathy

o No response to antimalarials
o Steroid may cause hypertension
o No significant response to Azathioprine, but lead to sudden mortality and
increased infection
 Chapter 17
ACUTE RENAL FAILURE

Definition
o Acute renal failure is sudden reduction in renal function that is
accompanied by retention of nitrogenous waste and disturbance of water and
electrolyte balance.

o Oliguria : is reduction in urine output to < 300ml/m² or < 1ml/kg/hour

o Anuria : is defined as urine < 75ml in adults and 1 ml/lg/day in children.

o Polyuria : Urine output > 4ml/kg/hour

o Azotaemia : is presence of high nitrogenous waste as indicated by high urea.

Is a reflection of renal dysfunction, but may also ba due to non-renal causes
e.g
i. High protein diet
ii. Hormones e.g glucocorticoids
iii. High catabolic rate
iv. Surgery
v. Trauma
vi. Infection etc
o Uraemia – is the symptom complex reflecting organ dysfunction that occurs
when kidnay fails to regulate body composition.

Epidemiology
Incidence is lower in adults.

Note – Up to 50% of acute renal failure in chidren is non-oliguric.

Pathogenesis & Pathophysiology

There are 3 distinct phases in evolution of the disease

a. Initiation phase – due to damage to tubular epithelium

b. Maintenance phase – low GFR is maintained
c. Recovery phase
Events Leading to Reduced GFR are
i. Reduced glomerular permeability
ii. Renal tubular obstruction
iii. Filtrate back flow and vascular problems, damage to tubular epithelium will
allow filtrate toleak back into tubular circulation.

Important Factors in the Development of ARF

1. Haemodynamic Chnages During ARF

Tubular Injury

Reduced Cortical blood flow Altered reabsorbtion of solute & water

Increased vascular resistance Incrreased load that leads to stimulation of

Juxtaglomerular apparatus & release of vasoactive
susbstance e.g adenosine, rennin, prostagladins.

Reduced GFR/ARF
2. Nephronal changes

Proximal tubule injury

Epithelial cell necrosis

Loss of tubule integrity Impacted cell debris

Back leak of solute Tubular obstruction

Reduced GFR (ARF)

3. Metabolic changes
- There is release of oxygen free radicals
- Calcium influx

Aetiology

a. Pre-renal - can also lead to intrinsic renal damage

i. Hypovolaemia : Nephrotic syndrome
ii. Peripheral vasodilation e.g sepsis
iii. Reduced cardiac output e.g congestive cardiac output
iv. Renal vascular origin

b. Renal
i. Acute glomerulonephritis
ii. Haemolytic uremic syndrome
iii. Septicaemia
iv. Acute malaria
v. Pyelonephritis
vi. Haemoglobinuria in G6PD deficiency
vii. Myoglobinuria e.g Road Traffic Accident
 viii. Drugs
Aminoglycosides : gentamicin
Vasomotor : ACEI, Cyclosporin, NSAID
Interstitial nephritis : Penicillin NSAID, Diuretics

c. Post-renal
i. Posterior urethral valve
ii. Pelvi-ureteric junction obstruction
iii. Vesico-ureteric junction obstruction
iv. Neurogenic bladder
v. Calculi
vi. Ureterocoele
vii. Tumours e.g Burkitt’s lymphoma, leukaemia
viii. Trauma

Clinical Presentation

i. History suggesting the aetiology

e.g Road traffic accident - hypovolemia
Fever diarrhea – hypovolaemia
Viral infection - HUS
ii. Oliguria
iii. Features of fluid retention
iv. Dyspnoea
v. Cough
vi. Headache
vii. Convulsion
viii. Pallor
ix. Hypertension
x. Acidotic breathing
xi. Features of heart failure

Investigation

1. Urinalysis

2. Full blood count – Leucocytosis revelas septicaemia, thrombocytopaenia points to

haemolytic uremic syndrome.

3. Blood film for malarial parasite. Also red cell fragments points to
microangiopathic
haemolytic anaemia.
 4. Electrolyte, Urea & Creatinine - shows
o Metabolic acidosis
o Increased potassium
o Decreased or normal sodium - reduction is usually dilutional
o Increased urea
o Decreased calcium
o Increased phosphate
o Increased creatinine

5. Electrocardiogram – shows peaked tented T wave due to hyperkalaemia.

6. Fluid challenge – using 20 – 30ml/kg body weight of IV normal saline or Ringer’s

lactate over 30 minutes to 1 hour. If the child does make urine after, give lasix and
restrict fluid. If it is pre-renal, diuretic challenge should generate > 2 – 3ml/kg/hour
(50ml/m²/hour) in the subsequent few hours.

Parameter Pre-renal Renal

1. Urine Osmolality (mosm/kg water) > 500 < 500

2. Urine Na (mEq/L) < 20 > 40
3. Urine/Plasma Urea >8 <3
4. Urine/Plasma Creatinine > 40 > 20
5. Fractional flitered Na (%) <1 >1
6. Urine Specific Gravity Increased, Decreased,

>1.020 <1.020
RCF1 Na <1 >1

In pre-renal, the body tries to retain fluid, thus leading to increased urine osmolality,
increased S.G while in intrinsic damage, the tubules are damaged and can’t conserve any
longer.

Fractional Excretion of Filtered Na % = UNa x PCr

UCr x PNa

Renal Failure Index = UNa X PCr

UCr
Management

1. Fluid challenge test :

o If the child passes urine, it is likely to be pre-renal cause, therefore, replace the fluid
deficit and ensure adequate hydration.

o If there is no response to fluid challenge and diuretics, it is likely to be due to

intrinsic renal problem, therefore, restrict fluid to 300 – 400ml/m² + previous day
output.

2. Correction of electrolyte derangement :

a. Hyperkalaemia

Agent Dose Onset Mechanism

1. 10% Calcium gluconate 0.5 -1ml/kg over 10 Immediate Cardioprotective

minutes
2. 8.4% Sodium 2ml/kg, IV 5 minutes Drive potassium into
bicarbonate cell
3. Salbutamol 10mg via Nebuliser 5 minutes As above

4. 20% Glucose + Insulin 2 -5 ml /kg 30 minutes As above

5. Ion –exchange resin 1 g/kg 2 hours – oral Remove potassium

30minutes -
parenteral
6. Dialysis Peritoneal dialysis / Gradual Remove potassium
haemoflitration /
haemodialysis

Note
o Calcium gluconate shold be given in ICU to avoid hypercalcaemia which cause
cardiac arrest in systole.
o Calcium gluconate protects the effects of hyperkalaemia on the heart.
o Sodium bicarbonate is used commonly in UCH.
o Salbutamol is given via nebuliser and not intravenous

2. Hyponatraemia
- Is usually due to fluid retention, thus management is fluid restriction
 3. Hypocalcaemia
- Is usually asymptomatic, but if there are symptoms, treat with calcium
gluconate.

4. Hypertension
- Is usually due to salt and water retention, thus start with diuretic if the child is
not anuric.

5. Seizure
- Identify and tret the cause, symptom with anticonvulsant.

6. Nutrition
- Give adequate calorie at least 1,400kcal /m²

7. Infection
- Treat because it may worsen the outcome if left untreated.

8. Dialysis
- Early initiation and not when patient is moribund.

Indicatons
i. Symptomatic uraemia
o Anorexia, Nausea & Vomiting
o Itching
o Fatigue
o Drowsiness
o Headache
o Bleeding diathesis
o Chest pain

ii. Encephalopathy
iii. Pericarditis
iv. Bleeding
v. Pulmonary oedema
vi. Fluid overload
vii. To deliver calories
viii. Electrolyte imbalance not responding to conservative therapy.

Prognosis
Depends on aetiology

Moratlity is 10 – 60%.
 Chapter 18
CHRONIC RENAL FAILURE &
RENAL BIOPSY

CHRONIC RENAL FAILURE

Definition

o Chronic renal disease – is defined as either kidney damage or GFR <

60ml/min/1.73m² for ≥ 3 months.

o Kidney damage – is defined as pathological abnormalities or markers of

damage including abnormalities in the blood or urine test or imaging studies.

Chronic Renal Failure – is preferred now

Stages of Chronic Renal Disease – helps to prevent late progression t ESRD

Stage Description GFR

(ml/min1.73m²)
I Kidney damage with normal or Increased GFR ≥ 90

II Kidney damage with mildly reduced GFR 60 – 89

III Moderately with reduced GFR 30 – 59

IV Severely reduced GFR 15 – 29

V Kidney failure < 15

(or req. dialysis)

Chronic renal failure

o is a complex of clinical, chemical and metabolic disturbance that results form chronic
reduction in renal function. Renal failure is a continuum.

o Is also defined as a reduction of GFR to < 25% of normal that has been present for at
least 3 months.
Chronic Renal Insufficiency (CRI)
o Is defined as a decrease in GFR to between 25 – 50% of normal (of age & sex). It
almost invariably progress to chronic renal failure in spite of correction or arrest of
the cause.

Stages of Renal Failure

Stage GFR Feature
1. Impaired renal function 80 - 50 Asymptomatic

2. Chronic Renal insufficiency 50 - 30 - Metabolic abnormalities

- Impaired growth
- Progression
3. Chronic Renal Failure 30 - 10 As above

4. End-stage Renal Failure < 10 As above

- Requires renal replacement
therapy.

Uraemic Toxins
o Urea
o Creatinine
o Guanedine
o Parathyroid hormone – perturbation in the extracellular & intracellular calcium
levels resulting into toxicity.

Middle molecules (plasma protein), of molecular weight 500 – 500,000 D are thought to
cross the peritoneal membrane more effectively than using Cuprophan membrane in
haemodialysis.

Incidence & Prevalence

o In Europe, the acceptance rate to CRF programme is 3.7 – 6.6million of child
population.
o Figures for developing countries are unknown, but hospital-based study from
Portharcourt estimated the incidence to be 7.5million.

Aetiology (Incidence, %)

1. Chronic glomerulonephritis ( 25. 8% )

2. Chronic pyelonephritis
3. Renal dysplasia ( 13.5% )
4. Cystic disease
 5. Urologic abnormalities
i. Obstructive uropathy
ii. Reflux nephropathy (24.2% )
iii. Urinary tract malformation

6. Systemic disorders
i. SLE
ii. Henoch-Schonlein purpura

7. Vascular disorders – Haemolytic uraemic syndrome

8. Tumours – Bilateral Wilm’s tumour

9. Hereditary /Familial e.g Alport’s syndrome ( 15.6% )

10. Multisystemic disease ( 10.2% )

Note, majority of the cases in Nigeria are due to Steroid-resistant nephrotic syndrome
secondary to Quartan malarial infection.

Pathogenesis
Once the critical level of deterioration has reached, it progresses to end-stage. The
mechanism involves hyperfiltration which causes further damages and causes
glomerulosclerosis. There is also phosphate retention.

Clinical Feature
i. Antenatal detection of conditions that can cause CRF
ii. Failure to thrive
iii. Short stature
iv. History of recurrent UTI
v. Enuresis

Respiratory
vi. Acidotic breathing
vii. Uraemic breathing
viii. Pulmonary oedema
ix. Pleural effusion
x. Acute respiratory tract infection

Cardiovascular
xi. Hypertenison
xii. Congestive cardiac failure
xiii. Uraemic pericarditis – pleural rub is heard
xiv. Arrhythmia
Haematologic
xv. Pallor – the mecahanisms include
i. reduced erythropoietin production
ii. rapid haemolysis of red cells
iii. from bleeding
iv. reduced life span of red cells
v. iatrogenic
vi. nutritional – deficiency of iron, folate
xvi. Bleeding

Gastrointestinal
xvii. Anorexia
xviii. Vomiting
xix. GI bleeding

Central Nervous System

xx. Seizure
xxi. Uraemic encephalopathy

Musculosketetal
xxii. Renal osteodystrohy

Dermatologic
xxiii. Pruritus
xxiv. Uraemic frost
Pathophysiology of Renal Osteodystrophy

Loss of nephrons

Metabolic acidosis Defect in 1,25 D3 synthesis Increased phosphate

Reduced Calcium absorption Reduced Calcium in serum

Failure of mineralization of bones Increased Parathyroid

hormone

o Bone resorption
(osteosclerosis)
o Osteitis fibrosa
o Fractures

Clinical Features of Renal osteodystrophy

i. Growth impairment
ii. Marked bowing of lower extremities
iii. Slipped epiphyses & other skeletal deformities

Note
Stunted growth is due to
i. Malnutrition
ii. Chronic anaemia
iii. Metabolic acidosis
iv. Renal osteodystrophy
v. Insensitivity to growth hormone (due to impaired
somatomedin

Q. The common causes of renal failure infancy are congenital

Investigation

a. Group I : To assess the severity of the renal failure

1. Full blood count – usually normochromic normocytic anaemia

2. Biochemical Parameters (in comparison with Acute Renal Failure)

Parameters Chronic Renal Failure Acute Renal Failure

1. Sodium Decreased or normal Decreased or normal
2. Potassium Usually increased or low if Increased
the condition causes K+ loss
e.g Tubular acidosis
3. Bicarbonate Usually low Low
4. Urea High High
5. Serum creatinine High High
6. Calcium Usually decreased or normal Decreased
7. Phosphate High High
8. Alkaline phosphatase High especially in renal ---
osteodystrophy
9. Albumin Decreased -----

3. Glomerular filtratio rate (Creatinine clearance) - is decreased

4. Parathyroid hormone – Is increased if there is renal osteodystrophy
5. X-ray of the hand & wrist – to assess the bone age and features of osteodystrophy
which is superiosteal erosion especially in phalanges. It is one of the earliest feature.
6. Chest X-ray – for cardiomegaly that may develop from hypertension.
7. ECG – shows peaked or tented T wave due to hyperkalemia
8. ECHO

b. Group II : To find the underlying cause of CRF

9. Renal Ultrasound – shows shrunken kidney (though this is not always true
especially if the cause is one of the following:
i. Cystic kidney
ii. Diabetis mellitus
iii. HIV Nephropathy

10. Micturating Cystourethrogram (MCUG) – Shows reflux

11. Dimercaptosuccinic acid (DMSA) – it detects Acute pyelonephritis. It is good to
study the scarring of the kidney and differential function between the two kidneys.

12. Urinalysis – may show blood in urine

 13. Urine m/c/s – shows broad waxy cast due to tubular damage
14. Immunology
o Serum C3 & C4 assay
o Antinuclear factor (ANF0
o Double stranded DNA (DSDNA) – showing an active Lupus Nephritis
o ANCA

15. Renal biopsy - does really help because the kidney are shrunken and patient is prone
to have haemorrhage.

16. White cell cystine & Oxalate excretion – Nephrocalcinosis, urolithiasis

Management

For those in pre-terminal stage, the objectives are

i. To make the child feel normal and be seen to be normal by mates
ii. Slow progression to ESRF
iii. Maintain normal growth
iv. Preserve normal family milieu
v. Prepare family for management of ESRF

Teams : Nephrologist, Specialist, Nurse, Dietician, Social Worker, Psychologist,

Psychiatrist, Play specialist.

Protocol
1. Grwoth
- Majority usually have growth failure prior to presentation
- Find out the cause
- Give recombinant growth hormone 0.9 – 1g/week

2. Nutrition
- Ensure adequate calorie intake, 100 – 120kcal/kg
- Protein & phosphate restriction, if severe, give 0.6g/kg/day

3. Correction of Fluids & Electrolytes

- Give Sodium bicarbonate, 2mmol/kg to correct metabolic acidosis. This
ensures adequate growth and prevent bone deminralisation.

4. Bone – Renal Osteodystrophy

- Give phosphate binder calcium carbonate (Note – alludrops is no longer
used, it causes bone CNS toxicity)

- Give 1, 25 –dihyroxylcholecalciferol (Calcitriol), the dose is

0.02µg/kg/day, titrated with Parathyroid hormone.
 5. Treatment of hypertension
- Using diuretics (Note, ACE inhibitor is no longer used)
- Or Nephrectomy if the above failed.

6. Correction of anaemia
- Ensure adequate Iron & folate store
- Use erythropoietin, 50iu/kg for 3 times a week

Merits & Complications of Erythropoietin

Merits
i. It avoids blood transfusion
ii. It reduces sensitization to histocompatibility antigens
iii. Reduces exposure to infections
iv. Improves appetite
v. Enhances physical fitness
vi. Increase physical activity during the day
vii. Improves sleep
viii. Improves well-being

Complications
i. Iron deficiency
ii. Most require iron therapy
iii. Hypertension
iv. Seizures
v. Reduced dialysis clearance
vi. Hyperkalaemia
vii. Clotting of vascular access

Overall, erythropoietin (EPO) is still BENEFICIAL !

7. Social & Psychological Support

8. Renal replacement therapy

- Is initiated when GFR falls < 10ml/min/1.73m²
- Other indications are enumerated under Acute renal Failure e.g poor
growth, unresponsiveness to conservative therapy.

Types
i. Peritoneal dialysis
a. Continous Ambulatory Peritoneal Dialysis
- is favoured in children

b. Contionus Cycling Peritoneal Dialysis

 - is user’s friendly
- involves the use of personal machine
- it is usually done at night

i. Haemodialysis - Is faster, last for 3 – 4hours. It is done 3 times

a week

ii. Renal transplantation – Age is not a limiting factor

Prognosis
o The prognosis has improved in the last 25 years in the developed countries.
But in Nigeria, ESRF is still a death sentence.

N.B
Compare Peritoneal dialysis & Haemodialysis – for frequency/ prevalence of
anaemia.

Comparison of ARF & CRF

Acute Renal Failure Chronic Renal Failure
1. Previously healthy child - Poor growth
- Chronically ill appearance
- Family history of renal disease
2. Gross haematuria Previous abnormal urine or hypertension
3. Oedema Oedema (if terminal)
4. Large kidneys Small kidneys
5. Anaemia – Usually improves as Anaemia
thepatient gets better.
6. Hypertensive nephropathy
7. Bone disease
8. Broad waxy casts – due to tubular damage.
 RENAL BIOPSY

Introduction
Percutaneous renal biopsy is an invasive procedure. In doing it, the benefits should
outweigh the risks.

Uses
i. To make diagnosis
ii. To plan treatment
iii. In prognostication

Indications

1. Nephrotic syndrome
- All nephrotic patients in our environment who have no contra-indication
should have renal biopsy because most of them are steroid-resistant.

- In Caucassian children, it is done for

a. Those whose age < 1year or > 10 years. This is because those
between 1 and 5 years have >90% chance of having Minimal
change disease which is steroid –sensitive, therefore they are
just begun on steroid.
b. Those with low serum complement
c. Failing of corticosteroid therapy

Persistent low C3 after 8 weeks in MPGN, Lupus Nephritis.

2. Acute Nephritic syndrome

i. Atypical presentation e.g anuria
ii. Nephrotic syndrome
iii. Delayed resolution of persistent hypertension, azotaemia, gross
haematuria after 3 weeks.
iv. Low C3 after 8 weeks : in MPGN, Lupus Nephritis
v. Persistent proteinuria for > 6 months
vi. Microscopic haematuria > 1 year

3. Acute renal failure

i. When associated with no obvious cause
ii. Associated severe oliduria lasting > 2 – 3weeks
iii. Associated manifestations of more diffuse disease
 4. Chronic renal insufficiency
i. To determine the existing severity of morphologic lesions
ii. To determine the risk of recurrence in eventual renal treatment
iii. To know suitability of cadaveric and living related donor treatment
iv. For follow-up of disease – With improved therapeutic modalities now
available for intervention in chronic renal progressive renal disease,
sequential or follow-up biopsy will become increasingly necessary to
evaluate therapeutic efficacy.
v. For renal treatment biopsy
vi. To assess episodes of clinically suspected rejection
vii. To investigate the cause reduced renal function or reduced urine
output
viii. To detect de novo recurrent disease

Contra-indications to Percutaneous Renal Biopsy

Absolute
i. Solitary kidney
ii. Ectopic kidney
iii. Horse-shoe kidney
iv. Bleeding diathesis
v. Abnormal renal vascular support

Relative
i. Obesity – can be done under fluoroscopy or ultrasound-guided
ii. Unco-operative patient
iii. Hydonephrosis
iv. Ascites
v. Small shrunken kidneys
vi. Tumours
vii. Large cyst
viii. Abscess
ix. Pyelonephritis

Pre-biopsy Requirements
1. Full blood count
2. Absolute platelet count
3. Electrolytes, urea & creatinine
4. Clotting profile
i. Prothrombin time
ii. Plasma thrmboplastin time with kaolin
iii. Bleeding time
5. Group and crossmatch blood
6. Urine M/C/S
 7. Ultrasound – to exclude solitary or ectopic kidney
To determine the size & symmetry of the kidneys
8. Informed consent
9. Fasting for about 4 – 6 hours before biopsy
10. IV line
11. Premedication (light sedation that enable patient to cooperate during biopsy
o Promethazine – 0.5mg/kg
o Chlorpromazine
o Pentazocine – 1mg/kg
o Diazepam

Biopsy Suite
- Should air-conditioned
- Has ultrasoumd machine
- Has Microscope, Slide & Fixatives

Types of Needles Available

1. Vim Silverman
2. Trucut
3. Quick core biopsy needle
4. Biopsy needle/gun

Attendants
i. Renal physician
ii. Radiologist/Sonologist
iii. Pathologist - to determine the adequacy of tissue
iv. Technician

Complications
1. Haemorrhage
2. Perforation of adrenal glands
3. Infection
4. Arterio-venous fistula – may close spontaneously
5. Pneumothorax
 Chapter 19
HYPERTENSION IN CHILDHOOD

Definition

o Average systolic & diastolic pressure for age, gender, & height

Percentile
Normal < 90th
Borderline (High normal) 90 – 95th
Hypertension > 95th

In the borderline, search for the risk factors.

Epidemiology
Incidence: 1 – 2%

Below the age of 10years, secondary hypertension is more common than primary.
Out of which, 80% is due to renal disease.

Precautions to be Taken When Blood Pressure in Children.

1. The largest cuff which is comfortable for should be used and its inflatable part
(the Bladder) should at least 2/3rd of the circumference of the upper arm.

Standard
o Adult cuff bladder’ width is 12 – 14cm
o Children’s cuff = 8cm
o Infant cuff = 5 -6cm

2. The lower edge of the cuff should be 2.5cm above the cubital fossa.
3. The Child should be seated, relaxed and comfortable.
4. Arm should be positioned at the heart level to remove the effect of gravity.
5. Cuff should not be deflated rapidly, deflate at 2 -3mm/s.

Note, Doppler ultrasound or oscillametry devices are reliable in neonates.

Diagnosis of hypertension
Is made by Blood pressure > 95th percentile and confirmed by 2 further
examination.
Aetiology

A. Renal Casues (responsible for 80% cases)

i. Post-infectious glomerulonephritis
ii. Haemolytic uraemic syndrome
iii. Nephrotic syndrome
iv. Reflux Nephropathy
v. Renovascular hypertension
o Renal artery stenosis
o Renal artery thrombosis
o Polyarteritis nodosa
o Arterio-venous fistula
vi. Multicystic dysplastic kidney
vii. Renal tumours
viii. Acute renal failure
ix. Chronic renal failure
x. Post-renal transplant

B. Non-renal causes
i. Coarctaion of aorta
ii. Arteio-venous fistula
iii. Polycythaemia

C. Encdocrine
i. Steroid therapy
ii. Corticosteroid excess
o Congenital Adrenal Hyperplasia
o Conn’s syndrome
o Cushing’ syndrome
iii. Mineralocorticoid excess
iv. Hypothyroidism
v. Hyperthyroidism

D. Tumour
i. Wilm’s tumour (Nephroblastoma)
ii. Neuroblastoma
iii. Phaechromocytoma
 E. Central Nervous System
i. Seuzure
ii. Gullain Barre syndrome
iii. Poliomyelitis
iv. Increased intracranial pressure

F. Drugs
i. Erythropoietin
ii. Oral contraceptive pills
iii. Cocaine - in adolescent
iv. Sympathomimetics

G. Syndromes
i. Liddle’s syndrome – is Pseudohyperaldosteronism. It is characterized by
o Low rennin
o Low aldosterone
It is due to mutation of gene encoding renal epithelium sodium
channel.
ii. Gordon syndrome – is characterized by
o Hyperkalaemia
o Hyperchloraemia
o Metabolic acidosis
o Normal GFR
The defect is due to inability of the kidney to excrete sodium.

Causes of Transient Hypertension

1. Renal diseases – predominate
2. Acute glomerulonephritis
3. Haemolytic uraemic syndrome
4. Acute renal failure
5. Henoch-Schonlein purpura
6. Nephrotic syndrome secondary to Intravascular volume depletion
7. Following intravascular surgery.
8. Patient with Suprumbilical catheterization
9. Renal Transplantation
10. Increased Intra-cranial pressure
11. Steroid therapy

Essential Hypertension ( Read more in the textbook)

- There is reset of the set point of blood pressure.
- Inability of the kidney to maintain balance between blood pressure and total body
sodium. The effect of salt intake is more marked in blacks i.e the hypertension is
volume-dependent)
 - There is renal leak of calcium
- Impaired regulation of insulin
- Primary defect is in Renin-Angiotensin system.

Clinical Presentation

i. Is usually asymptomatic, it is detected during routine examination.

ii. There might be features of the underlying disease in secondary hypertension.
iii. With substantial elevation, patient may present with
ƒ Headache
ƒ Dizziness
ƒ Visual changes
ƒ Seizure

In infancy – the features of the are present

i. Congestive cardiac failure
ii. Respiratory distress
iii. Failure to thrive
iv. Vomiting
v. Irritability

In Older children
i. Headache vii. Polydipsia
ii. Nausea viii. Polyuria
iii. Vomiting ix. Visual symptoms
iv. Tiredness x Irritability
v. Heart failure xi. Facial weakness
vi. Epistaxis xii. Growth retardation

Other Features
i. Palpaitation
ii. Sweating
iii. Pallor – in catecholamine excess state
iv. Cardiomegaly
v. Hypertensive retinopathy
vi. Lower motor neurone facial palsy – due to damage to facial nerve vasa
vasorum in long standing hypertension.
vii. Permananet visual loss – Can be due of the following
o Retinal damage
o Disc oedema
o Cortical pathway interruption
o Vitreous haemorrhage
o Hih Ischaemic anterior optic neuropathy
Points to Note When Evaluating A Child With Hypertension

o Is the hypertension sustained?

o Will he benefit from treatment?
o Is there any target organ damage?
o Is any co-existing risk factor?
o Is there any definable cause?

Investigation

Aim : To identify secondary causes & assess the effect of the hypertension on target organs.

Scheme for investigation of hypertension

Step 1
1. Full blood count
2. Urea & Electrolytes
3. Serum creatinine
4. Urinalysis
5. Urine M/C/S
6. Serum protein & cholesterol
7. Blood sugar Chest X-ray
8. ECG
9. ECHO

Step 2
10. 24 hour urinay protein & creatinine clearance
11. Intravenous urogram
12. Renal scan or ultrasound
13. Renal biopsy

Step 3
14. Aortogram & Renal arterigram
15. Homovanillic acid (VMA) estimation - for neuroblastoma
16. Hydroxy-mechoxy-mendelic acid (MMA) – for phaechromocytoma or
neuroblastoma
17. Adrenal & Pituitary function
18. Peripheral plasma rennin level – to rule out hyper-reninnaemia.
Treatment
(- Note, drugs are used ia all cases)
A. Non-pharmacologic – involves lifestyle modification
i. Salt restriction
ii. Weight loss
iii. Exercise
iv. Mofication of risk factors e.g
o Obesity
o Hypertension
o Hyperlipidaemia
o Control of diabetes mellitus
o Control of oral contraceptive use
o Cigarrette smoking

v. Mineral supplements: e.g Magnesium

vi. Increased dietary potassium
vii. Reduced caffeine intake

B. Pharmacologic
o (Note – there is lack of age specific pharmacokinetics and efficacy data in
children is often an extrapolation from adult data.)
o Drugs are used if there is family history

o Factors Influencing the Use of Drugs

i. Family history of any complication of hypertension e.g renal failure
ii. Target organ involvement e.g retinopathy
iii. Presence of other risk factors e,g coronary heart diseas

Classes of Antihypertensives
1. Angiotensin-Converting Enzyme Inhibitors (ACEI)
- It blocks conversion of Angiotensin I to Angiotensin II
- It is renoprotective

Side effects
i. Reversible renal involvement especially in children with pre-existing renal
insufficiency and bilateral artery stenosis. This is because angiotensin
mediates efferent arteriole vasoconstriction which is necessary for
maintenance of GFR, this is reversed by ACE inhibitor.
ii. Hyperkalaemia
iii. Neutropaenia
iv. Anaemia
 Algorithm

First line CCB ACEI BB

Add Diuretics Add Diuretics Add Diuretics

No effect

Add BB or ACEI Add BB or CCB Add ACEI or CCB

No effect

Add Minoxidil Add Minoxidil Add Minoxidil

Abbreviations
CCB - Calcium channel blocker
ACEI – Angiotensin Concerting Enzyme Inhibitor
BB- B-adrenaergic blocker
 Drugs Commonly Used in Ibadan

Drug Dose in Neonate Dose in Child Route/Frequency

Diuretics

1. Hydrochlorothiazide 2 – 3mg/kg/day 0.5 -2 mg/kg/dose Oral, bd-qd

2. Frusemide 1 -4 mg/kg/dose 0.5 – 2 mg/kg/dose Oral, bd-qd
3. Spironolactone 1 - 3 mg/kg/dose 1 – 3 mg/kg/dose Oral, bd-qd
Calcium Channel Blockers

1. Nifedipine 0.25 - 2mg/kg/dose 0.25mg/kg/dose Oral, tid-qd

2. Amlodipine 0.1 – 0.2 mg/kg/day Oral daily
Angiotensin Converting Enzyme Inhibitors

1. Captopril 0.5 – 2mg/kg/day Oral/tid

2. Enalapril 0.05 -0.5mg/kg/day o.1 – 0.5mg/kg/day Oral /daily-bd
3. Lisinopril
B-adrenergic Blockers

Atenolol 1 – 2mg/kg/day Oral/daily

Others

Hydrallazine 0.25 – Oral bi-tid

0.5mg/kg/dose

Side effects

Diuretics B-blockers Vasodilators

1. Hypokalaemia 1. Bradycardia 1. Headache

2. Volume depletion 2. Syncope 2. Palpitations
3. Hypotension 3. Sleepiness 3. Tachycardia
4. Hypomagnesaemia 4. Visual disturbance 4. Flushing
5. Hypercalcaemia 5. Vivid dreams 5. Fluid & Sodium retention
6. Gulcose intolerance 6. Weakness
7. Hyperlipidaemia 7. Fatigue
8. Hyperuricaemia 8. Depression
9. Gastric irritation 9. Bronchospasm
10. Impotence 10. Impotence
Sympatholytics ACE Inhibitors Calcium channel
blockers
1. Sedation 1. Renal Insufficiency 1. Peripheral oedema
2. Dry mouth 2. Hyperkalaemia 2. Dizziness
3. Depression 3. Neutropenia 3. Light-headedness
4. Vivid dreams 4. Hypotension 4. Headache
5. Nightmares 5. Orthostatic hypotension 5. Flushing
6. Hallucination 6. Rash 6. Weakness
7. Rebound hypertension 7. Dry cough 7. Transient hypotension
8. Impotence 8. Bronchospasm 8. Constipation

Note
o Do NOT use ACE Inhibitors in bilateral renal artery stenosis

o In patients with chronic renal failure, the aim is to reduce volume expansion &
sodium retention, start with thiazide diuretics if the GFR > 50 and Loop diuretic if
GFR is < 50.

o Drug resistance hypertension in patient with end-stage renal failure - ?

Nephrectomy.

SEVERE HYPERTENSION

a. Hypertensive Emergency : It is such a high blood pressure that requires

urgent treatment to reduce the blood pressure within minutes to hours to avoid
life-threatening complications.

b. Hypertension Urgency : When blood pressure is markedly raised, but can be

gradually brought down within a few days to avoid sequelae

There is significant overlap between the two types, hence the term Severe hypertension
with end-organ involvement is preferred for Hypertensive Emergency and without organ
involvement for Hypertensive Urgency.

Complications of Severe hypertension in Children

a. Central Nervous System – constitutes 60%

i. Encephalopathy
ii. Convulsion
iii. Facial nerve palsy
iv. Visual impairment
 v. Hemiplegia
b. Retinopathy – constitutes 27%
c. Left ventricular hypertrophy – for 13%
d. Renal failure

Management of Severe Hypertension

The ideal agent should be in parenteral form and should also have short half-life.

Aim of treatment
- Achieve 1/3 reduction to target blood pressure in first 6 hours
- 1/3 over next 24 – 36hours
- Proposed level in 48 – 72 hours

Common Agents For Severe Hypertension

Drug Dose Route
1. Nifepidine 0.25 – 0.5mg/kg SL/Chew/Swallow

2. Labetalo 0.25 mg/kg/hour Continous IV

3. Sodium Nitorprusside 0.3 - 8µg/kg/minute Continous IV

4. Nicardipine 1 -10µg/kg/min Continous IV

5. Diazoxide 1 - 3 mg/kg IV can be repated if there is

no response
6. Hydrallazine 0.3 – 0.5 mg/kg/dose IV /tid-qd

7. Enalaprilat 5 – 10 µg/kg/dose IV / tid

 Chapter 20
URINARY TRACT INFECTIONS
IN CHILDHOOD

Introduction
Urinary tract infection is of special significance in children because

i. Recurrent symptoms are troublesome and may persist to adult (female cystits)
ii. May indicate structural defects
iii. Urinary tract infection and Reflux nephropathy may lead to chronic atrophic
pyelonephritis (Reflux nephropathy) which is a cause of hypertension and
end-satge rednal disease.

Definitions

Urinary tract infections

Are conditions in which there is growth of baterial within the urinary tract.

Based on Sites
Cystitis – bladder involvement

Pyelitis – Upper tract involvement without parenchymal involvement.

Pyelonephritis – with renal parenchymal involvement

Bacteriuria – Presence of bacteria in bladder urine

Significant bacteriuria : growth of 100,000 colony-forming units (CFU)/ml of freshly

voided urine or any growth from suprapubic specimen except 2,000 – 3,000 CFU/ml of
coagulase –ve staphylococci.

Bacteriuria may be
a. symptomatic
b. asymptomatic (covert)

o Covert Bacteriuria : is presence of bacteria in repeated samples from a child who

does NOT report any symptom. It is usually seen during health investigation or
routine check-up.
o Complicated Urinary tract infection – when associated with reduced renal
function
- Obstruction
 - Dilatation
- Reflux
- Neurogenic dysfunction of bladder
- Indwelling urinary catheter
- Foreign body in the urinary tract
- Diabetes mellitus

o Uncomplicated UTI – is the type in the absence of the above factors.

o Recurrent Infection – can either be

a. Relapse
b. Reinfection
- it occurs in 60% of girls and 20% of boys

o Relapse : is infection with identical organism within 6weeks of treatment,

but it is usually within 1week.
Causes :
i. Upper UTI treated with too short course of antibiotics
ii. Stones
iii. Abscess
iv. Gross urological abnormalities

o Re-infection : is infection by a different organism. It constitutes most

recurrent infections.

Epidemiology
- Most UTIs are asymptomatic

- Asymptomatic UTI is commoner preterm than term infants

- It develops most often in the first year of life.

- Sex & Age : In the first 6 months of life, UTI is commoner in males, bur after 6
months, it is commoner in females.
- Infancy, congenital structural anomalies of the urinary tract probably account
foe higher incidence in boys.

- Prevalence of UTI in children is 4.1 – 7.5% (Pittsburg)

- The incidence of Symptomatic UTI is 4.1/1,000 newborns.

- UTI is more in uncircumcised male infants.

- Sicklers are at higher risk of UTI.

Aetiopathogenesis
There are 2 factors
i. Virulence fators in the organisms
ii. Host factors

Virulence Factors
a. Fimbriae
– This is best studied in E. coli.
– They are pilli on organism which are used to attach to uroepithelium, thus
assisting in invasion of urinary tract
– They also assist in the delivery of exotoxins in the causation of disease.
– Thus, organisms with fimbriae are ,ore likely to cause pyelonephiritis.

b. Antigens : E.coli has O, K, H antigens

H – flagella antigen
K - Capsule antigen, it is very important. It helps the organism to
evade phagocytosis.

Host Factors
a. Urine as a culture medium
i. The temperature 37ºC is ideal for incubation
ii. Urinary glucose is bacterial nutrient

b. Physiological & Functional factors

i. Incomplete bladder emptying
ii. Dysfunctional voiding – is common in children < 5 years who are just
being trained on toileting.
iii. Neurogenic bladder e.g spina bifida
iv. Sexual intercourse

c. Anatomical Factors
i. Dilatation of the Urinary tract
ii. Obstruction
iii. Vesico-ureteric reflux
iv. Bladder diverticulum
v. Calculi
vi. Short urethra in females

d. Immunological & Cellular factors

i. Immature immune system in infants
ii. B blood group
iii. P1 blood group

e. Iatrogenic
 i. Catheterisation
ii. Surgery & Instrumentation of urinary tract
iii. Accidental trauma – Penetrating injuries

Agents
i. Escherichia coli – is the commonest causative organism worldwide
ii. Klebsiella – is more prominent in Ibadan
iii. Others : Proteus, Staphylococcus.

Note, Among neonates in Nigeria, Klebsiella is the commonest cause followed by E. coli,
others follow worldwide distribution.

Route of Infection
a. Haematogenou – is common in the first month of life
b. Ascending infection – after one month, organism are from gut and urethra.

Clinical Presenation
- Is variable
- High index of suspicion is required

a. Neonate (What the mother complains about)

i. Non-specific
ii. Poor feeding
iii. Diarrhoea
iv. Vomiting
v. Jaundice
vi. Fever
vii. Cyanosis
viii. Circulatory collapase

b. Older infants
i. Fever (after malaria, think of UTI next)
ii. Pyrexia of unknown origin
iii. Weight loss
iv. Failure to thrive

c. Older children
i. Urinary frequency
ii. Abdominal pians
iii. Dysuria – child cries while passing urine
iv. Haematuria
v. Secondary onset enuresis
vi. Renal angle tenderness
Laboratory Diagnosis

I. Urine Microscopic /Culture / Sensitivity

1. Mid-stream Urine (MSU) - 10⁵ CFU of a single urinary pathogen (KASS CRITERIA)

2. Catheter Specimen Urine (CSU) - ≥ 50 X 10³ CFU/ml

3. Suprapubic aspirate (SUA) – growth of urinary pathogen in any number except

coagulase staphylococcus as mentioned above)

4. In newborn, a positive bag specimen should always br confirmed by CSU ou SUA. It

is useful only when the result is negative. When positive, you will need to eliminate
contamination.

Pyuria
o Is when there are 10 pus cell/mm³of unspun urine – is more reliable
Or
o 5 pus cells under high power film of centrifuge urine
o Pyuria is usually in UTI, but it is not diagnostic

Sterile Pyuria
o Is when there are significant pus cell (WBC) under micrscope, but the culture
is negative.
o Causes :
i. Renal tuberculosis
ii. Nephrotic syndrome
iii. Contamination from vagina
iv. Viral infection

Note
The urine is transported in bottle containing Boric crystals which peforms
the following functions :
o Prevent the organism from multiplying
o Preserves cellular elements- wbc, pus cells
The alternatives are
i. keeping the specimen in refridgerator at 4ºC
ii. Examine it immediately
 Other Investigations
Every child should with a proven UTI should be adequately scrutinize to know
a. the cause
b. If there is associated structural abnormalities reuiring surgery
c. If there is renal scarring or risk of developing it.

1. Electrolytes & Urea & Creatinine – done if the child is sick to check for
complication
2. Abdominal Ultrasound – to detect structural abnormalities and scarring
3. Micturating Cystourethrogram (MCUG) – to detect reflux.
4. DMSA scan using Technitium Tc 99 (is non-invasive) – to detect reflux or scars
5. Intravenous Urogram

Note,
o Abdominal ultrasound & MCUG are for all cases.
o Multi Dipstick -
- Leucocytes (esterase)
- Nitrites : the normal nitrates in urine are converted to nitrite by
bacteria, thus when positive, take sample for culture & begin
treatment.

Treatment

a. Asymptomatic & no associated anomalies – don’t treat. It poses no threat.

b. Symptomatic UTI
- The choice of antibiotic depends on the sensitivity pattern of the local
environment.
- The common drugs are
i. Nitrofurantoin
ii. Nalidixic acid
iii. Amoxycillin
iv. Amoxicillin-clavulanate
v. Gentamicin
vi. Cotrimoxazole
vii. Ampicillin

Note, the last 2 drugs should be considered last as the resistance to

them is close to 100%
 - Duration : Is usually 5 -& 7 days. If the child is very sick (e.g with
pyelonephritis which cause fever, chills & rigors), extend it to 10 – 14 days.
Also use parenteral antibiotics e.g
o IV Ceftriaxone
o IM Gentamicin
- Do repeat culture 1 week after, if is negative, reduce the dose of antibiotic to
keep the urine sterile and
- Monitor urine for the next 1 - 2 years because of the risk of recurrence.

Prevention
1. Regular bowel training
2. Regular bowel voiding/Double micturition
3. Low dose prophylaxis – for patient with
i. Vesico-ureteric reflux
ii. Recuurent UTI
iii. Neurogenic bladder
The drug used is Nitrofurantoin, its dose is 1/3rd of the usual therapeutic dose.

Complications
1. Renal scarring – resulting into
o Hypertension
o Chronic renal failure

Risk Factors For Development of Pyelonephritic Renal Scarring

1. Obstruction (as in obstructive uropathy)
2. Reflux with dilatation
3. Age : < 3 – 4 years
4. Delay in treatment
5. Number of pyelonephritic attacks
VESICO - URETERIC REFLUX

Introduction
o It is backflow of urine from bladder to ureter through an incompetent ureteric
valve.
o It is found in 30 -40% of children with Urinary tract infection

Mechanisms
Normally, the entering of ureter into is slanting as shown below. Different
abnormalities are demonstrated.

Normal Vesico-ureteric Junction – Here, the increasing intravesical pressure shuts the
intramural ureter thus preventing back flow.

1. Transverse junction 2. Rigid ureter 3. Develomental

shortened
- is congenital - is acquired, due to UTI

In the abnormal junctions, the organisms are allowed to be transmitted from bladder to
renal pelvis and on return to the bladder, the reflux urine increases the residual volume
encouraging re-infection and risk of developing renal scars.

Grading of VUR

I II III IV V
 Grade Description

I Refflux into a non-dilated distal ureter

II Reflux into Upper collecting system without dilatation
III. Reflux into dilated ureter ± blunting of calyceal fornices
IV Reflux into a grossly dilated ureter
V Massive reflux with ureteral dilatation and tortousity &
enlargement of the calyceal details

Treatment
Involves rapid treatment and prevention of recurrent UTI by
o Low dose prophylaxis
o Bladder & bowel training
- Voiding 2 or 3 hourly with double micturition at bed time
- Anticipate infection
o Investigate infants with urinary tract dilatation, it can be detected
antenatally. Also screen siblings of index patient with VUR.
o Surgery – for grades IV & V
To reduce bladder pressure as in
i. Posterior urethral valve
ii. Stones
iii. Ureterocoele
o Do urine culture every 3 months and whenever the child is not well.

Complication
Renal scarring.
 Chapter 21
FLUIDS & ELECTROLYTES

Introduction

- These include
• Water
• Electrolytes – Na+, K+, Cl- , HCO3-
• Acid-base balance

A.
WATER/FLUID

Introduction
- Fluid & Electrolyte disturbance in children is commonly secondary to
gastrointestinal illness which causes dehydration e.g diarrhea, vomiting,
insensible water loss.
- Children are at higher risk of developing dehydration because they have
i. higher body surface area/body weight ratio
ii. higher body water content/ body weight
iii. higher basal metabolic rate

Physiology of Water

- Water is the largest single compartment of the body.

- It varies with age as shown below

Variations with Age

Age Total Body (%) ECF (%) ICF (%)
Term baby 75 35 - 44 33
4 – 6months 60 23 37
1 year 60 26 - 30 37
Puberty 60 20 40
Adult 50 – 60 20 40

Note
- Total body water decreases with age
- ECF decreases with age
- ICF increases with age
- In dehydration, children tend to loose more water from ECF.
Osmolality
- Is the toncity of effective concentration of plasma.
- That of ECF depends on Na+, Cl, HCO3, glucose, urea while ICF depends
on potassium.
- At steady state, the osmolality of ICF, ECF & Plasma are equal and the
value is
280 – 295mmol/kg

Maintenance of fluid Requirement

o Fluid requirement is the amount of fluid needed to maintain homeostasis is a

person in a resting basal state. or
o Amount of fluid and electrolytes need to achieve zero water and electrolyte
balance.
o Maintenance water = Natural loses

Types of Fluid Loses

i. Insensible (Evaporation) – the total loss is 30ml/kg day
a. Skin – accounts for 2/3rd
b. Lungs – accounts for 1/3rd

This is very important especially in changes in environmental

temperature e.g a child with pyrexia, a child on ventilator or
phototherapy. There is 12% loss (of 30%) of every degree rise in
temperature above 38C. This is very important in neonatology.

ii. Urinary Loss - 60ml/kg/day

- Is the amount of water in urine neede to excrete basal renal
solute load with normal specific gravity of 1.010

iii. Faecal loss : 5 – 10ml/kg/day

- It is negligible, but remember in the management of
dehydration i.e adding 5 – 10ml/kg body weight of ORS
for every faecal loss)
Calculation of Maintenance fluid Requirement

a. Is for children above 1.5kg. It is done by calculating the weight & height.

1,500 ml/m²/day

b. Based on weight

Weight (kg) Fluid /day

≤ 10 100ml/kg
11 -20 Add 50ml for every
kg above 10kg
> 20 Add 20ml for every
kg above 20kg

B.
ELECTROLYTES

Electrolytes Normal Maintenance Value

(mEq ormmol/kg/day)
Soidum 2 -3
Potassium 2 -3
Chloride 2-3

FLUID & ELECTROLYTES DISORDERS IN CHILDREN

Types of Dehydration
Is based on serum sodium level

Types Sodium Level

1. Isonatraemic (Isotonic) 130 – 150
2. Hyponatraemic (hypotonic) < 130
3. Hypernatraemic (Hypertonic) > 150

1. Isotonic dehdration
- is the commonest
- The net sodium and water losses are proportionate
2. Hypotonic dehydration
- Is not common
- Is caused by renal or extra-renal losses of electrolyte-rich fluid which are
replaced by plain water.
- Thirst is NOT marked
- Losses are mainly from the extracellular fluid.
- Signs of dehydration occurs early

- Rehydration method is the same with that of isotonic rehydration.

-
0.6 X Weight X Deficit
Deficit = Desired level (usually a low level) – Observed Na level

3. Hypertonic dehydration
- There is water in excess of sodium
Cause
i. Iatrogenic e.g using < 1 litre of fluid for ORS
ii. Artificial feeds
- Thirst is marked
- Water loss is principally from intracellular fluid, as extracellulae fluid is
relatively preserved.
- Classic signs of dehydration are frequently absent
- Neurological signs e.g irritability, jitteriness, convulsions are prominent
and are due to rupture of cerebral vessels.
- There is doughy abdomen
- Associated conditions are
i. Hyperglycaemia
ii. Metabolic acidosis
iii. Hypocalcaemia
- Rate of rehydration should be slower

Note, both severe hyponatraemia & hypernatraemia cause convulsion.

Correction of Dehydration

Hypotonic dehydration
- Don’t correct the total deficit
- Correct to low serum sodium level – 125mmol/l
- Start with normal saline and change to half saline.
- For severe hyponatraemia, correction should be made with 3% saline (<
120mmol)
- 1 ml = 0.5mmol of sodium
 - Rate of correction = 1 – 2mmol/L/hour (i.e rate of increasing the sodium
level)
- Further correction is proceed as with isotonic dehydration.

C.
ACID-BASE DISTURBANCES

Introduction
Acid-base balance is maintained by
o Lung excretion of CO2
o Renal excretion of excess H+
o Body buffer systems – which prevents acute changes. Out of the
buffers, bicarbonate is the most important.
CO2 + H2O H2CO3 H+ + HCO3-

Assessing Acid-Base Status

1. Serum electrolyte
o Serum bicarbonate (total PCO2)
o Anion gap = serum (Na+ + K+) – serum (HCO3 + Cl-)
o The normal value is 10 – 14mmol/L, it should be
< 12 in infant
< 17 in older child

2. Blood pH - The normal pH is 7.35 -7.45

3. Arterial blood gases

- PCO2 – to determine pulmonary ventilation, buffer base excess of
deficit.
Acid-Base Disturbances

1. Acidosis
- There is deficit in base or gain in buffer acid

Types
a. Respiratory acidosis

Causes (of pulmonary hypoventilation)

i. Pulmonary disease
ii. Neuromuscular disease
iii. CNS depression
 Picture
o Reduced pH
o Increased PCO2

Compensation
o Conservation of HCO3 by kidneys
o Increased excretion of H+ by kidneys

Treatment
Don’t give anything, just restore adequate ventilation.

b. Metabolic acidosis

Causes
a. Accumulation of net acid (H+)
i. Ingestion of acid e.g salicylate (Note, in salicylate
poisoning, there is both acidosis & alkalosis)
ii. Excess production of acid e.g Diabetic ketoacidosis
Lactic acidosis

b. Decreased excretion of hydrogen ion

i. Renal failure

c. Excess loss of HCO3

i. Diarrhoea
ii. Renal disease

Anion Gap in Metabolic acidosis

- It helps to clssfiy metabolic acidosis.

- It represents protein which are not usually measured e.g phosphates,
sulphates, creatinine.

Diabetic Ketoacidosis & Diarrhoea

Lactic Acidosis
- The anion gap is wide - It is normal because the bicarbonate loss
- There is normochloraemia is compensated for by chloride hence
resulting into hyperchloraemic acidosis.
 Picture of Arterial blood gases
o Reduced pH
o Reduced bicarbonate
o PCO2 may be normal or low when there is compensation by the
respiratory system.

Treatment
a. If it is mild or moderate with respiratory compensation and the
renal function is normal, just treat the underlying cause.

b. If Severe i.e pH < 7.2, give alkali (1ml =1mmol). The dose is

1 – 2mmol/kg of 8.4% NaHCO3, dilute to twice its volume

Give slowly.

c. Bicarbonate deficit
- Do half correction i.e using 0.3 and not 0.6 so as to
over-correction to alkalosis.

0.3 X Weight X Base deficit

d. Trihydoxymethyl amino-methan (THAM)

- in metabolic acidosis + hypernatraemia
or Respiratory acidosis

2. Alkalosis

a. Respiratory alkalosis

Causes (is due hyperventilation which causes loss of CO2)

i. Salicylate – this is centrally-nediated
ii. Hysteria
iii. Head injury

Picture
o Increased pH
o Reduced PCO2

Compensation – by kidney
o Increased bicarbonate excretion

Treatment
- Treat the cause of hyperventilation.
b. Metabolic alkalosis
Causes – loss of H+ or increase in base
i. Diuretic therapy – is one common cause in Paediatrics
ii. Recurrent vomiting e.g pyloric stenosis
iii. Excessive alkali administration – Iatrogenic
iv. Familial chloride loosing diarrhea

Picture
o Increased pH
o Increased bicarbonate

Compensation - is by Respiratory system

o It is small and unpredictable
o There is decrease in ventilation, but this only causes slight increase
in PCO2.

Treatment
i. Rehydrate – to restore the intravascular volume
ii. Replace Potassium & Chloride deficits. Note alkalosis is usually
associated with hypokalaemia.
iii. Treat underlying cause.
 Chapter 22
MENINGITIS

Definition
- Is inflammation of the meninges

Aetiology
a. Viruses – also called Aseptic
b. Bacterial
i. Pyogenic
ii. Tuberculosis
c. Fungi – is very rare
d. Protozoa
i. Malaria
ii. Toxoplasmosis

PYOGENIC MENINGITIS

Aetiology
- Almost any organism can cause meningitis, but it depends specifically on the age
and environment.

Age Agents
Neonate or < 2months Generally, organisms causing neonatal
sepsis which are
• Klebsiella sp
• Staphylococcus aureus
• Escherichia coli
2 months – 1 year • Haemophilus influenza (usually upto
5years). The invasive one is type B
(Hib)
• Streptococcus pneumoniae
(Pneumococcus)
• Neisseria meningitides
(meningococus)
> 12 years (Uncommon) • Streptococcus pneumoniae
• Neisseria meningitidis
Note
• Streptococcus pneumoniae is particularly common in the following groups of
people:
i. Haemoglobin SS
ii. Patients who have had Splenectomy
iii. Immunocompromised patient
iv. Those on steroids

• Neisseria meningitides is responsible for epidemic pyogenic meningitis in

Northern part of Nigeria.

Epidemiology
It is commoner in male.

Pathology
- The inflammation of the meninges causes exudate formation around the brain,
which may lead to cerebral oedema.
- There is also arteritis which cause reduced blood supply and consequently
infarction. This brain damage results in various neurologic deficits e.g cranial
nerve palsy, seizures.

Pathogenesis

Routes of infection are

i. Haematogenous – most common. It is usually secondary to chest or
lung infections
ii. Contiguous sites e.g
o Otitis media
o Sinusitis
o Mastoiditis
o Cerebral abscess

iii. Trauma e.g compound skull fracture which causes direct implantation
of bacteria (or any organism around the site of injury)
iv. Direct invasion e.g dermoid sinus, meningomyelocoele. Usually here,
any organism can be seen.

TUBERCULOUS MENINGITIS
Disseminated Tuberculosis
Primary focus

Haematogenous route
 Forms tuberculoma - which can be one huge mass or several tiny masses which
ruptures and results in meningitis.

Clinical Presntation of Pyogenic Meningitis

i. Signs & symptoms of preceding upper respiratory tract (URTI) or

gastrointestinal infections e.g
URTI : Fever, cough, respiratory distress
GIT : Vomiting
ii. Sypmtoms & Signs of meningeal irritation – these are minimal in infants
o Anorexia, Nausea & Vomiting (ANV)
o Headache
o Neck stiffness
o Seizures
o Coma
In infants, fever & irritability may be only signs

On Physical examination
- The patient is ill and febrile
- In infants, anterior fontanelle is bulging and tense
- Neck stiffness
- Positive Kernig’s & Brudzinki’s signs – these may be absent in infants
- ± Focal neurologic signs :
o Cranial nerve palsies
o Focal seizures
o Blindness
o Deafness

- Signs & symptoms of meningococcaemia : Waterhouse- Friedrichsen syndrome –

it is characterised by
o haemorrhage into the adrenal gland
o bleeding into skin (purpura, ecchymosis)
(It occurs in children < 10 years)

Differential Diagnosis

1. Other types of meningitis – CSF is used to differentiate these

2. Brain abscess – differentiated by
o Presence of abscess at other sites of focus of spread from other
parts of the body e.g osteomyelitis, congenital heart disease.
o Presence of localizing signs
o Presence of increased intracranial pressure
 3. Viral encephalitis – There is fever, seizure & coma. It is differentiated on CSF
analysis.
4. Brain tumour
o Tends to have subacute or chronic history.
o Increased intracranial pressure is very prominent
o There are focal neurological signs
o May be no fever
5. Intracranial haemorrhage
o History of sudden headache
o CSF would be bloody
6. Meningismus /Meningism – this is meningeal irritation without inflammation.
It is found in young children.

Investigation

a. Lumbar puncture for Cerebrospinal fluid Analysis

Parameters Normal Pyogenic Tuberculosis Viral Partially-
treated
Pyogenic
meningitis
1. Colour Clear or Cloudy/turbid/ Clear/slightly Clear Variable
colourless Purulent/hazy turbid/xantho-
chromic
2. Pressure < 180 Increased Increased Increased Variable
(mm of H20)
3. < 5, ↑↑↑, e.g 1,000s ↑↑ e.g 100s ↑ mainly Variable/↑mixed
Microscopy predominantly mainly Mainly lymphocytes /Lymphoctes
(no. of wbc) lymphocytes polymorphs or lymphocytes
pleocytosis
4. Gram stain No organism Positive (N.O.S) (N.O.S) (N.O.S)
seen (N.O.S)
5. Culture Negative Positive Negative, but Negative Negative
+ve in LJ
medium
6. Protein 10 – 40 ↑ ↑↑ ↑ ↑
(mg/dl)
7. Sugar ½ to 3/2rd ↓ i.e < 1/2 ↓ Normal Variable
of blood sugar

Note,
o In partially-treated meningitis, the patient has used one form of antibiotic
before presenting, thus the CSF finding is vaiable.
 o The CSF pressure is measured with an instrument attached to the needle.
With normal pressure, the CSF runs in fast drops. If it is increased, it
rushes out .
o Tuberculosis is positive in Lowenstein-Jensen medium
o Protein is increased in all, but higher in tuberculosis where it can form clot
or spider web.
o Sugar level is normal in viral meningitis
o On microscopy, the shapes are shown below

Organism Shape
Streptococcus pneumoniae Gram +Ve Diplococcus

Neisseria meningitides Gram –Ve Diplococcus, it is kidney or bean-

shaped
Haemophilu influenza Coccobacillus

o Neisseria meningitides
- are usually intracellular

b. Adjunct Investigations
i. Full blood count – shows leucocytosis if bacterila
ii. Electrolytes & urea – because of the risk of Syndrome of
Inappropiate ADH Secretion.
iii. Blood culture – 80 – 90% is usually positive.

Management
1. Admit the patient
2. Give antibiotics
- there is no room for oral or intramuscular drugs
- The drugs should be able to cross the blood brain barrier
- While waiting for the CSF result, start to cover for the above 3 main organism.

a. First choice
Crystalline penicillin + Chloramphenicol
Dose: 300,000 iu/kg/day 100 mg/kg/day
Organism: Streptococcus pneumoniae Haemophilus influenzae, type B
Neisseria meninditidis

b. Second choice : 3rd generation Cephalosporin e.g

o Ceftriaxone
o Cefotaxime
o Ceftaxidime
 - They are very expensive, therefore, are not usually used in
UCH.
- Don’t use Rocephin
3. After seeing laboratory result, change to recommended drug based on sensitivity
unless if the patient is already responding to the initial drugs.

4. Duration of antibiotic administration

- Depends on response of the patient, but give at least 10 – 14 days or
continue for 5days after fever has disappeared.

5. Studies have shown that IV dexamethasone steroid can reduce the inflammatory
response to the organism which has actually been a major cause of complications
e.g deafness. It should be given to patients 20 – 30minutes before the first dose of
antibiotics, then continue for 4 days after the stoppage of antibiotics. (Note, you
must be sure that you are using correct antibiotics because the steroid lowers the
body immune resistance.

6. Administration of intravenous fluids – give 2/3rd of maintenance fluid because

of the risk of SIADH.
7. Nasogastric feeding – if the patient cannot feed orally
8. Oral feeding
9. Anticonvulsants – for seizures
10. Monitor head circumference – to look for hydrocephalus

Complications
1. Subdural effusion
– Is very common and most common with H. influenza
– Is mostly frontal or parietal
– Is now regarded to be part of the disease
2. Cranial nerve palsy
3. Deafness – is the most common complication. It is associated with Mumps
meningitis
4. Blindness
5. Hemiparesis or Quadriparesis
6. Hypertonia
7. Ataxia
8. Seizure disorder
9. Hydrocephalus
10. Mental retardation
11. Cerebral palsy
12. Acute adrenal failure – meningococcal diseas
Prognosis – depends
i. Age of the patient – the younger the child, the worse the prognosis
ii. Duration of onset before appropriate treatment
iii. Type of organism involoved – Out of the 3 major organisms, it is worse with
streptococcus pneumoniae
iv. Number of organism

Prevention
1. Vaccination
a. Hib vaccine has been introduced as part of routine immunization in many
parts of developed countries. It can be combined DPT.

b. Pneumococcal vaccine
- Is available but not given routinely
- It is given to target populations:
ƒ Sickle cell disease
ƒ Immunocompromised patients
ƒ Splenectomy
ƒ Nephrotic syndrome
ƒ Acquired Immunne deficiency states

c. Meningococcal vaccine
- Is not part of routine vaccines
- Is used where the epidemic is common i.e Northern part of Nigeria.
- It occurs during dry season.
- It is given every 3 years at the

2. Chemopophylaxis
- Is given to the close-contacts because H. influenza & N. menigitidis are
transferred through the nose.
- The drugs used is Rifampicin with the dose of
 Chapter 23
SEIZURE DISORDERS

Definition
- Is sudden excessive hyper-synchronous discharge of neurons in part of the brain.

- It is manifested as
o Involuntary motor
Tonic – sustained contraction
Clonic – broken or jerky
Tonic -clonic
o Sensory – abnormal sensations
o Autonomic – salivation, palpitatios
o Psychic phenomenon – feeling of abnormal fear
whether alone or in combination.
- It is often accompanied by alteration of loss of consciousness
- Other associated terms
• Convulsion – is motor manifestation of a seizure

• Epilepsy – is tendency to have spontaneous recurrent seizure.

(Defined as chronic recurrent paroxysmal changes in neurological function due to
abnormalities in electrical activity)

Incidence & Prevalence

o Age : It varies, highest in neonates of about 5 – 6%. It decreases by 1% by
age 1 year.
o It is 1% of population
o 2.5% of population have had 1 seizure attack.
o In UCH, it constitutes 23% of neurological consult in Paediatrics. It is the
2nd commonest in the Paediatric Neurologic clinic after Cerebral palsy.
o It is commoner in male
o It is slightly commoner in lower socioeconomic class.

Classification
Can be based on the following
a. Aetiological – this helps in treatment
b. Seizure types – helps in treatment since the various types respond to
different drugs
c. Syndromic – for treatment and prognosis
I. Aetiological Classification

a. Congenital
i. CNS Malformation
• Neuromuscular syndromes
o Neufibromatoses
o Tuberous sclerosis
o Sturge Weber syndrome
• Cerebral dysgenesis
• Proencephaly

ii. Genetic syndromes

iii. Intrauterine infections

b. Acquired
i. Trauma
• Birth trauma – causes both seizure &
cerebral pasly
• Asphyxia

ii. Infections
• Meningitis
• Encephalitis
• Cerebral malaria

iii. Febrile sezure

iv. Metabolic
• Hypoglycaemia
• Hyponatraemia
• Hypernatraemia
• Hypocalcaemia
• Hypomagnaesamia

v. Toxic
• Lead poisoning
• Drugs (intoxication, withdrawal od drug
addiction)

vi. CNS Neoplasm

vii. Vascular
• Arteriovenous malformation
• Cardiovascular accident
 FEBRILE SEIZURES

Definition
• Is seizure occurring in young children usually from 3 months (used to be 6
months) to 5 years in which there is no evidence of intracranial infection or
any other cause.

Epidemiology
o The incidence is 2 – 5% among the young children.
o It is commoner in male

Aetiology
Hereditary – tends to occur in families, ? autosomal dominant.

Common causes of Fever

i. Malaria
ii. Upper respiratory tract infections
- Otitis media
- Sore throat/pharyngitis

Classification
Simple Febrile seizure Complex
1. It is relatively brief, lasting < 15minutes 1. Lasts > 15 minutes
2. Is usually single episode 2. Is usually multiple in 24 hours
3. It is generalised It is focal

Diagnosis
Before diagnosis of febrile seizure is made, rule out intracranial infections.

Management of Febrile Seizure

1. Stop the seizure
o Intravenous (parenteral) diazepam, given it slowly to avoid respiratory
arrest.
Dose: 0.1 -0.3kg
o Intravenous paraldehyde – Although is good enough as it causes necrosis,
but it is often used here.

Dose : 0.1ml/kg
 2. Control the temperature
o Tepid sponging
o Antipyretics

3. Investigate for the cause

o Full blood count
o Blood film for malarial parasite
o Lumbar puncture for CSF analysis – to rule out meningitis especially in
children < 18 months who don’t show the usual signs.

4. Reassure the parents

- That the disease in not fatal or that the child will become epileptic or has
brain damage.

5. Treat the underlying cause

Recurrence
o Occurs in 33% of patients. 75% of which occurs within the 12 months of the
first attack.
o The risk factors for it are:
i. Young age (< 18months) of the first attack
ii. Family history
iii. Short duration of fever
iv. Seizure at a relatively lower fever.

Risk for Development of Epilepsy

Note, this risk is small. Also, recurrence does not increase the risk of development of
epeilepsy.
i. Abnormal development before the first sseizure
ii. Family history of afebrile seizure
iii. Complex febrile seizure

Long-term Management
1. Parental counseling
- That the disease is a benign condition
- About recurrent management
- Likelyhood of developing epilepsy
- Note, drug is not the first line of treatment. There is no more need of
phenobarbitone for prophylaxis.

2. Diazepam - For
- Prevention or treatment of future episodes
- Young age
- Multiple attacks in the past
- Parental anxiety
 Oral : - given from onset of fever to 24 hours after.
Dose : 1mg/kg/day in 3
divided doses

Parenteral : Given till temperature is < 37.5C. It can be used to stop ongoing
seizure.
Dose: < 3 years – 5mg
> 3 years – 10mg

II. Classification by Seizure types

1. Partial seizure (Focal )

a. Simple
- shown by motor, somatosensory, autonomic or psychic
- there is no loss of consciousness

b. Complex
- there is some (not total) loss of consciousness
- There are subtypes
i. Simple partial - there is impairment of consciouness from onset.
There is automatism (Psychomotor epilepsy)

c. Partail seizure with secondary generalization – it is usually the pattern

in most cases of generalized.

2. Generalized Seizures
They are subtypes
a. Absence seizure (Petit mal)
- there are brief lapses in conciouness
- Usually lasts for a few seconds
- Has no aura
- Has no post-ictal symptoms
- Ocassionally, presents with alittle movement of eyelids,
though there is really no motor manifestation.
- Occurs many times a day, therefore it is very irritable.
- Can be divided into two
i. Typical – last for < 12 seconds
ii. Atypical – has motor seizure

b. Myoclonic - there is brief contraction of muscles. It is seen in several

syndromes
 c. Clonic – there is rhythmic contraction of limbs

d. Tonic – there is sustained contraction

e. Tonic-clonic (Grand mal)

f. Atonic – The patient loses muscle and collapses

3. Unclassified
4. Status Epilepticus

Grand Mal Epilepsy

- Is commonly seen
- Has aural (pre-attack sensation) which is (are) actually evidence of
partial seizure.
- Patient may utter a cry and fall, have tonic (sustained) seizure for few
minutes, and then clonic contraction.
- During the attack, there may be salivation or urination.
- In the post-ictal state, the patient might have
o Drowsiness
o Headache
o Severe weakness of the parts of the involved in the
contraction which does not last for > 24 hours, this is
called Todd’s Paralysis.

III. Classification based on syndromes

This mode of classification is based on
o Age of onset
o Aetiology (if known)
o Seizure type
o Genetics
o Natural history – that is response to drugs and remission.
o EEG pattern

The common examples are

i. Benign Childhood Epilepsy with Centrotemporal Spikes
(Rolandic)
- Usually occurs in primary school children
- Remit in adolescence
- Responds to drugs
- Almost always occurs in the night (from partial to
generalized seizure.
 ii. Childhood Absence Epilepsy (Petit mal)
- Usually starts within 3 – 8years
- Continues in the adolescence
- The attack is usually precipitated by hyperventilation
(you can do this in the hospital to check)
- There is characteristic EEG pattern of 3 cycles/second
spike wave pattern

iii. West Syndrome / Infantile spasm

- Occurs from the age > 3 months
- It is associated with some forms of brain damage or
abnormalities.
- Has poor prognosis i.e patient may die or develop mental
retardation.

Infantile Spasm
o There os brief spasm, starts with abduction, then
adduction.
o There is sudden flexion of the head – Sallam type
o It occurs in volleys with each attack very brief.
o Is common when the child is or
waking up from sleep.
o Has characteristic EEG pattern, called
Hypsarrhthmia, described as disorganized EEG
pattern of high voltage.
o Prognosis is poor because it is usually associated
with brain damage.

iv. Lennox-Gestaut
- Occurs in older age
- May be myoclonic, absence or tonic-clonic
- Is usually associated with brain damage.
- Has poor prognosis.
Diagnosis
Is clinical particularly from the history of good eye witness account.
Investigation

1. Electroencephalogram
- Is important as noted above
- Check for spikes
- Helps to point to a type of epilepsy

2. Computer Tomography scan/MRI

- Done in cases of abnormal history & examination or there is difficulty in
control the patient.

Note : Skull x-ray is of use.

3. Biochemical parameters
a. Glucose
b. Calcium
c. Phosphate
d. Magnesium
e. Electrolytes & Urea

Differential Diagnosis

1. Breath holding spells

2. Gastro-oesophageal reflex – in small infants
3. Benign Paroxysmal vertigo
4. Syncope – resembles atonic type
5. Night terrors
6. Tics
7. Pseudoseizures – false seizures in patient who have had it before.

Principles of Management of Epilepsy

1. Counselling – done at the first visit. About the long term treatment
2. Drug treatment
- Usually started after ≥ 2 episodes
- You start with small dose and increase gradually until the seizure is
controlled.
- Use single drug if possible as it control the seizure in about 70%
- Continue the drug for 2 – 4 years after the last seizure. (i.e 2 - 4
seizure-free years)
- On stoppage, you tail off gradually over 3 – 6months.
- Classes of drugs used are listed below
3. Ketogenic diet
4. Surgery
Drug Treatment of Seizure types

Simple Partial Complex Generalised Absence Myoclonic Infantile

Sz. (PPC) Partial Sz ( PPC. GNL) (EN) ( Ncl. N) (ACo V)
( CNG. LV)
Phenobarbitone Phenobarbitone
Phenytoin Phenytoin
Carbamazepine Carbamazepine Carbamaz-
pine
Na Valproate Na Na
Valproate Valproate
Gabapentin Gabapentin
Lamotrigine Lamotrigine
Vigabratin
Vigabratin
Ethosuxi-
mide
Clonazepam
Nitrazepam
Corticoste-
roid(Pred-
solone)
ACTH

Note
o In Simple partial seizure, phenobarbitone remains the first choice even with all
the disadvantages.
o In infantile spasm, ACTH (Corticotrophin) is the first choice

Management of Acute Seizure Attack

1. ABC of acute care
2. Prevent injury
3. Oxygen by mask or cannula
4. Antiepiletic drugs (AED) to stop the seizure.

STATUS EPILEPTICUS

Definition
- Is a life-threatening seizure that is prolonged for > 30 minutes with no
recovery in between the attacks.
- Generalized tonic-clonic is most alarming because of the risk of hypoxia.
Aetiology
a. Febrile seizure
b. Non-compliance with drugs
c. Stress including intercurrent infections
d. Brain pathology e.g tumour, trauma, infection, infarction, drugs etc.

Management
o ABC of resuscitation
o Blood sugar estimation
o Start IV line
o Drugs
i. Diazepam
ii. Intravenous phenytoin, 20 mg/kg as slow infusion.
iii. Intravenous Phenobarnitone, 15 -20mg/kg, give slowly
iv. Intramuscular Paraldehyde, 0.1ml/kg
o Intubate after general anaesthesia for 2 hours.
 Chapter 24
CEREBRAL PALSY

Definition
o Is defined as a disorder of movement & posture resulting from non-
progressive permanent damage of developing brain.
o The disorder leaves a lasting effect.
o Brain development still occurs upto 18 years, however, the brain damage
occurs very early in life.
o The name was coined by Williams Oslez

Epidemiology

o The incidence is 2-2.5/1000 in developed countries

o It is the commonest neurological disease seen in Paediatric Neurologic Clinic,
the rate is about 27.7%

Aetiology
Is undertemined in a large number of cases.

a. Prenatal
i. Radiation.
ii. Anoxia
iii. Intrauterine infections e.g TORCHES manifested as skin rash & fever
during pregnancy.
iv. Developmental anomaly
v. Drugs.
vi. Metabolic
vii. Toxins
viii. Genetic – some syndromes are associated with brain dysgenesis
ix. Vascular accident resulting into infarction

b. Perinatal
i. Birth weight (low)
ii. Anoxia – resulting from diffilculty in breathing e.g
o Birth asphyxia → Hypoxic encephalopathy → Cerebral palsy.

c. Postnatal
i. Trauma e.g Falls, Child abuse, RTA
ii. Infections – meningitis, encephalitis, cerebral malaria
iii. Toxins e.g bilirubin (hyperbilirubinaemia resulting into kernicterus)
iv. Drugs
v. Metabolic – hypoglycaemia
Classification of Cerebral Palsy

a. Physiologic – this is with respect to muscle tone or based on movement)

i. Spastic – occurs in most cases
- there is muscle tone
- the intellect is spared

ii. Athetoid
- is slow writhing movement of limbs & trunk resulting
from damage to basal ganglia.
- Is common in kernicterus (bilirubin toxicity, Bb level >
20mg%)

iii. Rigid
- Is as a result of damage to basal ganglia. There are two types:
a. Lead pipe – is more common
b. Cog-wheel

iv. Ataxic
- Suggests involvement of cerebellum. It is associated with
nystagmus.

v. Tremor
vi. Atonic
vii. Mixed Unclassified

b. Topagraphic - depending on the parts of the body affected particularly the

limbs

i. Monoplegia – one limb, it is very uncommon, watch the other limbs

very well
ii. Hemiplegia – one side of the body is involved, there is associated
seizures. It is usually spastic
iii. Triplegia
iv. Quadri- / Tetraplegia
– there is poor mentation, cognitive dysfunction & mental
retardation occurs
- There are subtypes
 a. Diplegia – Lower limbs are more affected than the Upper
limbs. It is especially common in preterm infants with
anoxic brain.

b. Double hemiplegia – Upper limbs are more affected

(spastic) than lower limbs

v. Paraplegia – involvement of lower limbs

c. Aetiologic – as above
d. Functional – this is useful social rehabilitation
- There are 4 classes

Classes Description
I No Limitation of activity
II Slight to moderate limitation
III Moderate to great limitation
IV No useful physical activity

Clinical Presentation

i. Delayed developmental milestones (especially motor) – is the commonest. (The

classical features of cerebral palsy are maily neuromotor symptoms, those these
may not typical in early infancy.

ii. Associated abnormalities of

o Speech
o Vision
o Intellect
o Behaviour

Note, all these are not part of definition of cerebral palsy, though are often
associated.

iii. Pregnancy, Labour & Delivery

o Fever
o Antepartum haemorrhage
o Proloned labour
o Asphyxia or trauma during labour
o Neonatal jaundice, sepsis, or seizure

Physical Examination
- Disorders of movement
o Hemiplegic gait – Flexion of elbow & wrist +
 - Extension of lower limb +
- Circumduction movement

o Scissoring of lower limb – most noticeable in diplegia

o Spasticity
o Prolonged fisting
o Tongue thrusting – is common in athetoid type
o Microcephaly (Always remember to check the head circumference)
o Abnormal hand preference – Hand preference normally develops by
3 years.

Diagnosis
- Is clinical
- It may be difficult in very young children
- Sometimes, you look for the presence or absence of primitive reflexes e.g
o Persistent Moro’s reflex beyond 4 months
o Asymmetric Tonic Neck Reflex (ATNR) – Is present in about 2
months of life. To demonstrate it, you turn the head to one side,
the limb on the same side extends while there is flexion of the
other limb. Even, the child at normal age doesn’t always do it.
o Obligate ATNR is very abnormal, even during the neonatal
period.

Investigation

o The diagnosis is clinical, no need of chemical test.

o But exclude progressive disorders e.g
i. Degenerative CNS disorders
ii. Metabolic disorders
o Positive family history rules out cerebral palsy except in rare cases if the mother
is G6PD deficient.
o G6PD screening – for the above reason
o Blood group
o EEG

Differential Diagnosis
1. Muscular dystrophy
2. Spinal cord tumour
3. Werdnig Hoffman disease

CT scan – shows atrophy of the brain

- Cystic lesion

Management
 • It is multidisciplinary.
• Physiotherapy - is the main modality of treatment
• Counselling – for the parents
• Specialists involved are
i. Paediatric Neurologist
ii. Physiotherapist
iii. Ophthalmologist
iv. ENT Surgeons
v. Plastic & Orthopaedic surgeons – to release contracture, to correct hip
dislocation, for rhizotomy
vi. Psychologist
vii. Occupational therapist
viii. Social workers
• The earlier the rehabilitation, the better for the child.
• Drug treatment
- is limited.
- It is to reduce spasticity when it is very high.
- The drugs used are Muscle relaxants, but has a disadvantage of being
sedative.
- Examples : Nitrazepam
Dantrolene
Botulinum toxin – used in the recent past. It is injected
directly into the spastic mucles. Its effect is short-acting.
• Rehabilitation

Prevention

Primary
i. Proper supervision of pregnancy & delivery to prevent severe birth asphyxia
- Recognition of high risk pregnancy
- Referral

ii. Improvement in obstetric care

iii. Improvement in care of the newborn
iv. Proper management of neonatal jaundice

Secondary
Management – as above

Tertiary
Rehabilitation
 Chapter 25
NEUROMUSCULAR DISORDERS

a. Anterior Horn Cell Disorders

b. Neuropathies
c. Neuromuscular junction Diseases
d. Myopathies

Anatomy & Overview of Neuromuscular Disorders

1 - Anterior horn cell

2 - Motor Nerve Fibre
3 - Motor end plate
4 - Muscle
5 - Sensory receptor in muscle
6 - Sensory nerve fibre

Generally, disorders of Nervous system can be classified into

a. Diseases of the Brain
b. Diseases of the Spinal cord
c. Diseases of thr Nerves
d. Diseases of the Neuromuscular junction
e. Diseases of the Muscle

Neuromuscular disorders start from the anterior horn cell through the nerves and
neuromuscular junction and to the muscles. i.e the whole circuit.
Classification

a. Anterior horn cell Disorders

i. Poliomyelitis
ii. Werdnig Hoffman Disease

b. Nerve Fibres Disorders (Motor & Sensory) – Neuropathies

• Polyneuropathies
i. Gullain Barre Syndrome
ii. Diphthretic Polyneuropathies
iii. Tropical Ataxic Neuropathy - Read up

• Mononeuropathies
i. Erb’s Pasly
ii. Bell’s Palsy
iii. Klumpke’s Paralysis

c. Disorders of Motor-end plate

• Myasthenia gravis

d. Diseases of Muscles (Myopathies)

• Duchenne Muscular Dystrophy
• Fascioscapulohumeral Dystrophy
• Limb Girdle Muscular Dystrophy
• Becker Pseudohypertrophy

A.

ANTERIOR HORN CELL DISORDERS

POLIOMYELITIS

Introduction
Poliovirus is an RNA enterovirus with 3 serotyps 1, 2, 3

Pathogenesis
o The transmission is mainly by faeco-oral route. In addition it can be oro-oral
route i.e from saliva.
 o The virus enters the lymph node in the gastrointestinal system and multiplies
in the reticuloendothelial system.
o From there, it passes through the blood (stage called Viraemia) to migrate to
the nervous system. It has predilection for Nervous system especially
• Anterior horn cells in the Spinal cord where it causes cervical
and lumbar expansion and consequently paralysis of the limbs.
• Cranial nerve nuclei in the medulla
• Other sections e.g
i. Cerebral cortex
ii. Cerebellum
iii. Thalamus
iv. Hypothalamus
o The end result is flaccid paralysis

Epidemiology
- It is found worldwide, but has been eradicated in most parts of the world.
- There has been more than 80% decrease in the prevalence rate since 1988 through
the use Oral Polio Vaccine (OPV).
- It is still endemic in Nigeria.

Clinical Presentation
There are 4 modes

1. Asymptomatic infection
– occurs in 90 – 95% of people infected.
– It is detected by serology which shows high titre antibody.

2. Abortive Polio
– occurs in 4 – 8% of patients
– It is called Minor illness.
– It is more serious
– Diagnosis is made during epidemics

Clinical presentation
o The child is ill and feverish
o The following may or may not be present
Malaise Headache
Anorexia Sorethroat
Nausea Constipation
Vomiting Abdominal pain

– It lasts for about 1 – 3 days

 Treatment
o Rest for 1 week following the deferverscence (i.e temperature
falling back to normal). The reason is because muscle stress or
strain precipitate paralysis.

3. Non-paralytic Polio
- Is a major illness
- Affects 1- 5% of infected population

Clinical Presntation
o As in the minor illness, but they are more intense.
o ± Stiffness & thickness of muscles - at the back of neck, back
muscles & calf muscle. They are also painful and in spasm.
o There is increase or decrease in superficial or deep reflex
o No SENSORY deficit ( as it does not affect the posterior column
tract)

4. Paralytic Polio
o Affects < 1% of infected polio

Clinical Presntation
- As above
- Weakness of > 1 muscle groups
• Skeletal muscle – Spinal polio
• Cranial muscle – Bulbar polio
• Combination – Bulbospinal polio

- The paralysis in polio is characteristically spotty and in

hapharzard manner i.e asymmetrical.

Diagnosis
Is clinical – made from history and examination. But high index
of suspicion is required in the endemic areas.

Investigation
i. Lumabr puncture – Polio is differential diagnosis of
meningitis. Even though there is meningitis in
poliomyelitis, only that it is viral or aseptic.

ii. CSF examination

Protein – Increased
Sugar - Normal
Gram stain - Negative
Serology - High antibody titre
 iii. Viral culture – using blood and stool

Differential Diagnosis of Poiomyelitis

1. Other viral infections – can also result into paralysis

o Echo virus
o Coxsackie virus
o Mumps virus
2. Meningitis – Differentiated on CSF examination
3. Gullain Barre syndrome
4. Peripheral neuritis e.g Post-infection Sciatic nerve Toxic
5. Spinal cord neoplasm
6. Myasthenia gravis

Prognosis
- Worsens with
o Age
o Stress and fatigue

Treatment
a. In Acute phase
o Bed rest – is most important
o Analgesics
o Appropiate posture – to minimize skeletal deformities

- Recovery of paralysis starts soon after the infection and continue for 6 months,
then to 2 yaers, after this, the deficit is permanent.
- Weakness often resolves completely.
- Atrophy is apparent within 4 – 8weeks.

• In supine position, knee slightly flexed, ankle perpendicular,

trunk straight with sand bags.

o Apply warm packs to the muscle for relaxation

o Physiotherapy – Start when symptoms disappear. It begins with
passive movement, then to active.
o Intravenous fluid – via nasogastric tube
o Mechanical ventilation – if there is any evidence of respiratory
embrassment which can be form bulbar or bulbospinal.
o **** DON’T GIVE INTRAMUSCULAR INJECTIONS to any patient
with polio
 b. Long-term treatment
1. Physiotherapy – to strengthened the muscles which are not paralysed.
2. Surgery – to correct contracture
3. Social rehabilitation

Prevention
o No drug is used.

o Is mainly by vaccination. There are 2 types of vaccines.

a. Sabin (Oral polio vaccine)

o is live attenuated virus

b. Salk (Inactivated/killed virus)

- given by subcutaneous injection.

o It is part of routine immunization

OPV0 At birth
OPV1 6 weeks
OPV2 10 weeks
OPV3 14 weeks
OPV1, 2 & 3 are given with DPT.

WHO’s Eradication Programme

o Make use of OPV
o The former was 2,000 years, but now 2003.
o Is given to all children < 5years. (Most children above this age would
have had Y –virus.
o National Immunization Day makes use of age as a guide.

Advanteage of OPV
i. It is cheap
ii. It is easily admsitered
iii. It is effective
iv. It increase the herd immunity – especially in area with low hygienic
environment as in Nigeria.

Disadvantage of OPV
i. When given immunosuppresed individuals, it causes Vaccine-
Associated Polio Paralysis. This is very rare.

Advantage of IPV
 i. Is very effective
ii. It does not cause paralysis, therefore used for cases with
immunosuppression.
iii. It is used for household family.

Disadvantage
i. It is more expensive
ii. Requires more gadgets to administer e.g needles etc.
iii. Requires high technical skills.

WERDNIG HOFFMAN DISEASE

(Infantile Muscular Dystophy/Atrophy)

Introduction
Is an autosomal recessive disorder (note, it is not infection).

Pathology
o There is atrophy of the anterior horn cella (AHC) due to disturbance of
controlled cell growth & development and cell death.
o Gives

Clinica Presentation
o Features of typical lower motor neuron lesion
i. Weakness – It causes floppy infant.
ii. Hypotonia
iii. Hyporeflexia
iv. Fibrillation/Fasciculation

o Mental function is normal i.e they are intelligent

o May manifest in-utero as reduction in fetal movment(kicking)
o Death usually usually occurs in 2nd year of life

Investigation
1. Electromyography (EMG) – shows fibrillation
2. Muscle biopsy – shows degeneration of muscles
3. Biopsy of anterior horn cells (on Post-mortem) – shows atrophy of anterior horn
cell and cranial motor nuclei.

Cause of death
- In children is respiratory failure as muscles become paralysed.

B.
 NEUROPATHIES
(Motor & Sensory)

Aetiology
1. Post-infectious
2. Toxic
i. Drugs : Isoniazid
Vincristine
ii. Metabolic : Diabetes mellitus
Uraemia

Classification
a. Polyneuropathies
i. Gullain Barre Syndrome
ii. Diphthretic Polyneuropathies
iii. Tropical Ataxic Neuropathy - Read up

b. Mononeuropathies
i. Erb’s Pasly
ii. Bell’s Palsy
iii. Klumke’s Paralysis

GULLAIN BARRE SYNDROME

(Acute or Idiopathic Polyneuritis)

Introduction
- Is a typical post-infectious polyneuropathy
- It affects any age

Aetiology
i. Mumps
ii. Infectious mononucleosis
iii. Influenza
iv. Vaccination - Rabies, Influenza
Clinical Presentation
o Features suggestive of viral infection
- Mild fever
- Diarrhoea
- Vomiting
- Rhinorrhoea
o Neuromuscular development disorder – develop within 2 weeks
o Paraesthesia - Pins and needles or numbness
o Weakness – usually starts in the lower limbs, migrate upto upper lower
limb. It usually starts from peripheral parts i.e fingers & toes, it is
called Glove-and-Stocking description.

Physical examination
i. Hypotonia
ii. Absent deep tendon reflexes
iii. Paraltsis is usually symmetrical (different fron polio which is spotty
and hapharzard.
iv. Cranial nerve involvement
v. Sensory loss is rare (unlike in polio), but do have sensory impairment.

Diagnosis
i. CSF examination is typical
Protein - Increased
Cells - Few or absent

There is cytoalbiminologic dissociation - i.e pleocytosis may

occur which later drops and protein level becomes high.

Differential Diagnosis

1. Poliomyelitis
2. Transverse myelitis

Complications
Paralysis of intercostals muscle (just like in Poliomelitis) can lead to death.

Prognosis
 - Is not bad.
- Recovery is complete in few weeks in > 90% cases. A few die from
respiratory embarassment

Treatment
i. Bed rest
ii. Mechanical ventilation – if there is respiratory compromise.
iii. Steroids – may or may not be used
iv. Plasmapheresis – Filtering the patient’s blood, removing the red cells &
reconstitute the cells with donor plasma and re-infuse. Discard the patient’s
plasma. It is life-saving.

DIPHTHERITIC POLNEURITIS
o It is not very common
o It follows pharyngeal or laryngeal nerve infection with diphtheria.
o The latent period is 4 – 8weeks
o There is bilateral acute weakness of all 4 limbs
o Paralysis of palate, ocular and phrenic nerve may also occur.

MONONEUROPATHIES

BELLS’ PALSY
- Is a disorder of cranial nerve VII
- It is associated with herpes zoster or otitis media infection

Clinical Presentation
- It tends to be sudden in onset
- Involves weakness of one side of the face.
- The angle of the mouth is deviated to normal side –Bell’s sign or
phenomenon.
- Also, on the weak side, the eye cannot close, when the patient attempts, the
eyeball rolls up.
- Hyper-acusis : becauses the removal of dampening effect of the stapedius
due its damage.
 - Riniging in the ear.

Treatment
o There is no definite treatment
o It disappears within few weeks
o Protect the eye during the illness – most important step
- Instil artificial tears or normal saline
- Use plaster to cover at night

ERB’S PALSY
- Is due to damage to C5 & C6 nerves
- It usually occurs during the birth of a baby (especially big babies), due to
pulling of the head at the second stage of labour.
- It also occurs in breech-delivery i.e bottom first

Clinical Presentation
- Weakness of muscles supplied by these nerves, resulting into
o Loss of abduction
o Inability to external rotation
o Inability to supination of forearm
- Thus, the arm is held adducted, internally rotated and pronated.
- There is also loss of biceps reflex (C5,6)

Treatment
- Wearing of long-sleeve shirt for the baby and pin it down to pillow.
- Early physiotherapy
- Gentle massage and other physical exercise.
- If required, surgery should be done before 4years.

Prognosis
- Most recover fully, but a few do not.

KLUMPKE’S PARALYSIS
- Is due to damage to lower roots of brachial plexus (C7,8, T1)
- It gives rise to Waiter’s tip hand.

C.
 NEUROMUSCULAR JUNCTION

MYASTHENIA GRAVIS

Introduction
- It is an autoimmune disorder characterized by weakness or fatiguability of
muscles after exercise.
- Antibodies are produced against acetylcholine recptor in the muscles.
- Though, it is mainly an autoimmune disorder, a rare family of MG is
autosomal recessive.
- There are 3 types
i. Juvenile

Clinical Presentation
i. It usually occurs > 10 years in 75% cases
ii. It affects more girls
iii. It commonly affects the following cranial nerves
III, VII, IX, X, XI
3 7 9 10 11
iv. Respiratory muscle may also be affected.
v. Clinical signs & symptoms are
o Ptosis – Is usually bilateral (though may also be unilateral)
o Diplopia – due to weakness of ≥ 1 extra-ocular muscle
o Poor chewing and inability to close mouth properly – if masseter
is affected.
o Typically, the symptoms with rest.

Investigation
1. Clinically, tell the patient to do repititive movement e.g looking up and
down or sustaining an upward gaze, while doing, the eyelids drop.
2. Anti-acteylcholine receptor antibodies – if the facility is available
3. Edrophonium (Tensilon) Test
- Tensilon is a strong short-acting anticholinesterase that increases
the concentration of acetylcholine at neuromuscular junction.
- It is not used for treatment, but only given to make diagnosis.
- If given IV (Dose : 0.2mg/kg), within few minutes, the situation
improves.

4. Neostigmine (0.04mg/kg) – is long-acting, therefore it is not likely to be

used for diagnosis.
 5. Chest x-ray – to rule out thymic hyperplasia or Thymomas which is seen as
thymic shadow on x-ray.

Differential Diagnosis

1. Hyperthyroid myopathy
2. Hypothyroid myopathy
3. Aminoglycosides myopathy e.g gentamicin
4. Botulism

Treatment

1. Using anticholinesterases e.g Neostigmine

Pyridostigmine
Ambemonium
2. In non-responsive cases, give steroids.
3. Surgery – To remove the thymomas (Thymomectomy)

Prognosis
- With optimal treatment, most patient can live normal live.
- Remission occurs in about 25% after about 2 tears of treatment.
- In some, the weakness occurs and die of respiratory embarrassment

PRIMARY DISORDERS OF MUSCLES

(MYOPATHIES)

MUSCULAR DYSTROPHIES
There are different types, but the most common is Pseudohypertrophic (Duchenne).

1.
PSEUDOHYPERTROPHIC (DUCHENNE) MUSCULAR DYSTROPHY

Introduction
o It is an x-linked inherited disease with spontaneous mutation.
o It is characterized by degeneration of muscle fibres.

Pathogenesis
 It is due to deletion of short arm of x-chromosome at the point where it carries the
dystrophin gene. Dystrophin is needed for muscular contraction.

Clinical Presentation
o Age : Diagnosis is usually made after 3 years
o Delayed developmental milestones
o Walking/Standing on toes
o Characteristic waddling gait (like a duck) – This is NOT pathognomonic, it is
just an evidence of weakness of pelvic girdle muscles
o Difficulty in climbing staircase

Physical Examiantion
o Hypertrophy of calf muscles – this is typical. It is due to pseudohypertrophy
resulting from fatty infiltration. Ocassionally, there is pseudohypertrophy of
brachioradialis and other muscle.
o Gower’s sign – it is typical of MG, it is due to weakness of pelvic girdle
muscles. It is also NOT pathognomonic.
o Intelligence is below normal in 30% of cases.

Investigation
1. Serum creatinine kinase (CK) – this is the muscle enzyme released after muscle
death. It is > 10 times of upper limit of normal (160iu/L)
2. Muscle biopsy – shows degeneration of muscle fibres
3. Electromyography (EMG) – Shows myopathic changes which are different from
neuropathic changes.

Treatment
1. Steroids(prednisolone) – in low dose. It increase the period of ambulation for
about 2 yaers
2. Physiotherapy
3. Respiratory physiotherapy – to remove secretions
4. Orthopaedic
5. Genetic counselling

Prognosis
o By 12 years of age, ambulation becomes impossible.
o Death occurs before 20 years, resulting from cardiomyopathy.
2.
FASCIOSCAPULOHUMERAL DYSTROPHY

Introduction
o Is less common
o Is milder
o Has autosomal dominance inheritance
o Patient usually present in the first decade of life

Clinical Features
i. Weakness of facial muscle
ii. Expressionless face
iii. Orbicularis oculi or oris involvement

Investigation
i. Creatinine kinase – is normal or slightly increased

Prognosis
- Is compatible with life

3.
LIMB GRIDLE MUSCULAR DYSTROPHY

o Is an autosomal recessive disorder

o The onset is late in childhood
o It is slowly progressive.
o It affects pelvic girdle mainly, though may affect shoulder girdle as well.
o Is compatible with life.

4.

BECKER PSEUDOHYPERTROPHY

o Is a mild form of Duchenne Muscular Dystrophy

o It is x-linked
o It presents later than Duchenne Muscular Dystrophy.
o It progresses slowly.
o There is increased creatinine kinase
o Prgnosis is better.
Dandy-Walker Syndrome

Despite being described nearly a century ago, the exact definition of the Dandy-Walker syndrome is
still debated. Classically, it is characterized by aplasia of the vermis, cystic enlargement of the fourth
ventricle, rostral displacement of the tentorium, and absence or atresia of the foramina of Magendie
and Luschka. Although hydrocephalus is usually not present congenitally, it develops within the first
few months of life. Ninety percent of patients who develop hydrocephalus do so by age 1 year.
Variants have cerebellar hypoplasia without dilation of the fourth ventricle and hydrocephalus and
may suggest other subtle cortical abnormalities not classically present and could be confused with
other disorders such as Joubert syndrome and its variants. On physical examination, a rounded
protuberance or exaggeration of the cranial occiput often exists. In the absence of hydrocephalus and
increased intracranial pressure, few physical findings may be present to suggest neurologic
dysfunction. An ataxic syndrome occurs in fewer than 20% of patients and is usually late in
appearing. Many long-term neurologic deficits result directly from hydrocephalus. Diagnosis of
Dandy-Walker syndrome is confirmed by CT or MRI scanning of the head. Treatment is directed at
the management of hydrocephalus.

Craniosynostosis

Craniosynostosis, or premature closure of cranial sutures, is usually sporadic and idiopathic.

However, some patients have hereditary disorders, such as Apert syndrome and Crouzon disease, that
are associated with abnormalities of the digits, extremities, and heart. Occasionally craniosynostosis
may be associated with an underlying metabolic disturbance such as hyperthyroidism and
hypophosphatasia. The most common form of craniosynostosis involves the sagittal suture and results
in scaphocephaly, an elongation of the head in the anterior to posterior direction. Premature closure
of the coronal sutures causes brachycephaly, an increase in cranial growth from left to right. Unless
many or all cranial sutures close prematurely, intracranial volume will not be compromised, and the
brain's growth will not be impaired. Closure of only one or a few sutures will not cause impaired
brain growth or neurologic dysfunction. Management of craniosynostosis is directed at preserving
normal skull shape and consists of excising the fused suture and applying material to the edge of the
craniectomy to prevent reossification of the bone edges. The best cosmetic effect on the skull is
achieved when surgery is done during the first 6 months of life.

Abnormalities of Neural Tube Closure

Defects of neural tube closure constitute some of the most common congenital malformations
affecting the nervous system. Spina bifida with associated meningomyelocele or meningocele is
commonly found in the lumbar region. Depending on the extent and severity of the involvement of
the spinal cord and peripheral nerves, lower extremity weakness, bowel and bladder dysfunction, and
hip dislocation may be present. Delivery via cesarean section followed by early surgical closure of
meningoceles and meningomyeloceles is usually indicated. Additional treatment is necessary to
manage chronic abnormalities of the urinary tract, orthopedic abnormalities such as kyphosis and
scoliosis, and paresis of the lower extremities. Hydrocephalus associated with meningomyelocele
usually requires ventriculoperitoneal shunting.

Arnold-Chiari Malformations
Arnold-Chiari malformation type I consists of elongation and displacement of the caudal end of the
brainstem into the spinal canal with protrusion of the cerebellar tonsils through the foramen magnum.
In association with this hindbrain malformation, minor to moderate abnormalities of the base of the
skull often occur, including basilar impression (platybasia) and small foramen magnum. Arnold-
Chiari malformation type I may remain asymptomatic for years, but in older children and young
adults it may cause progressive ataxia, paresis of the lower cranial nerves, and progressive vertigo;
rarely it may present with apnea or disordered breathing. Posterior cervical laminectomy may be
necessary to provide relief from cervical cord compression. Ventriculoperitoneal shunting is required
for hydrocephalus.

Arnold-Chiari malformation type II consists of the malformations found in Arnold-Chiari type I plus
an associated lumbar meningomyelocele. Hydrocephalus develops in approximately 90% of children
with Arnold-Chiari malformation type II. These patients may also have aqueductal stenosis,
hydromyelia or syringomyelia, and cortical dysplasias. The clinical manifestations of Arnold-Chiari
malformation type II are most commonly caused by the associated hydrocephalus and
meningomyelocele. In addition, dysfunction of the lower cranial nerves may be present. Up to 25%
may have epilepsy, likely secondary to the cortical dysplasias. With the advent of "aggressive-
selective" therapy, mortality is 14%; of survivors 74% are ambulatory and 73% have a normal IQ.

Arnold-Chiari malformation type III is characterized by occipital encephalocele, a closure defect of

the rostral end of the neural tube. Hydrocephalus is extremely common with this malformation.

In general, the diagnosis of neural tube defects is obvious at the time of birth. The diagnosis may be
strongly suspected prenatally on the basis of ultrasonographic findings and the presence of elevated

-fetoprotein in the amniotic fluid. All women of childbearing age should take prophylactic
folate, which can prevent these defects and decrease the risk of recurrence by 70%.
 Chapter 26
INTRODUCTION TO NEONATAL MEDICINE

• Adaptation to Extrauterine life

• High risk Pregnancies
• Examination of the Newborn
• Temperature Control
• Nutrition
• Fetal Distress

Objectives of the Lecture

i. Ability to appreciate neonate as an individual
ii. Ability to recognize and manage common neonatal problems

Definition of terms

o Terms : ≥ 37 – 42 weeks of gestation

o Preterm : < 37 weeks

o Post term : > 42 weeks

o Perinatal period - is from 28 weeks of gestation to 1st week of life

o Perinatal Period - is the first 28 days or 4 weeks of extrauterine life

o Low Birth weight - < 2,500 at birth irrespective of gestation

Goal of Perinatal Care

Is to present to each mother a healthy infant who has the maximal potential for
physical growth and neurological development.i.e social smile, neck control etc.

Objectives
1. Early and prompt identification of high risk pregnancies
2. Skilled perinatal care
 HIGH RISK PREGNANCY

The Patient

i. Teenage (< 16 at conception) – because of complications of cephalopelvic

disproportion e.g birth asphyxia, VVF
ii. Elderly mother, > 40 at conception – due to common illnesses associated with
increasing age e.g hypertension, DM
iii. Underweight – those who are 2SD from mean when compered to standard chart
appropriate for race
iv. Overweight – there would be problems to palpate
v. Low socio-economic status e.g nutrition

The Medical History

i. Hypertension – there is reduced surface area of vessels due to atherosclerosis
which causes reduction in nutrient intake thus resulting into LBW
ii. Renal disease
iii. Diabetes mellitus – they are prone to infections
iv. Cancer
v. Thyroid disease
vi. Cardiovascular disease
vii. Rhesus sensitization – not a problem here
viii. Tuberculosis
ix. Lupus erythematosus
x. Mental retardation –
xi. Major psychosis
xii. Neurological disease
xiii. Sickle cell disease – problems associated with severe anaemia
xiv. Thalassemias

Previous Pregnancy History

i. Grand multiparity
ii. Previous surgical delivery
iii. Previous prolonged labour (> 24 hours)
iv. Previous fetal loss
v. Previous live premature infant or SGA infant
vi. Previous infant death in first week of life
vii. Previous damaged infant e.g birth trauma, cerebral palsy, mental retardation
viii. Previous infant with respiratory distress syndrome/ Hyaline membrane disease
Pregnancy-Related Medical Condition (Past or present)
i. Toxaemia
ii. Bleeding after 12 weeks of gestation : Placenta praevia, abruption placenta
iii. Multiple pregnancy
iv. Abnormal presentation or position of the foetus
v. Hydramnious – Oligo & Poly
vi. General anaesthesia during pregnancy
vii. Adminstration of certain drugs to mother e.g sulphonamide (causing jaundice),
diazepam, proylthiouracil
viii. Anaemia
ix. Indifference to health needs

High risk babies

i. LBW
ii. Birth Asphyxia
iii. Neonatal jaundice
iv. Infant of diabetic mother
v. Seizures
vi. Metabolic disorder e.g hypo- and hypercalcaemia

FETAL DISTRESS

Definition
• Is fetal heart rate of < 100 (bradycardia) or > 160b/min. (The normal HR is 100
– 160b/min).
• Acidosis, pH < 7.20 ( ≤ 7.15 is ominous)
• Meconium staining of liquor. Normally, no fetus should pass meconium in-
utero. It is seen when there is asphyxia.

Fresh meconium – is a sign of acute asphyxia, It is very thick and the

colour is bottle-green. It blocks the airway causing air-trapping which
may result into pneumomediastinum or pneumothroax.

Stale mecoinum – is a sign of chronic hypoxia. It is lemon-green and it

is seen in patient with pneumonia.
Clinical Presentation
• Cyanosis
• ± Grunting
• ± Retraction
• ± Tachypnoea - > 60/min.
• ± Apnoea
Shock

Management
1. Reposition of the mother – to remove the compressive effect grand uterus on the
inferior vena cava, and consequently increase cardiac output.
2. Correction of maternal hypotension with IV fluids or blood
3. Reduce uterine contraction i.e stop oxytocin
4. Give oxygen administration
5. Assisted /Operative delivery

Differental Diagnosis of Respiratory Distress in the Newborn

Period

a. Respiratory

Common Less common Rare

Respiratory Distress Pneumonia Upper airway
Syndrome /HDS * obstruction e.g choanal
atresia
Transient tachypnoea* Pulmonary haemorrhage Space occupying lesion
e.g diaphragmatic hernia,
lung cysts etc *
Meconium aspiration Pneumothorax *
Primary pulmonary Pulmonary dysmaturity
hypertension (Persistent
fetal circulation)
 b. Extra-Pulmonary
Heart Metabolic * Brain Blood *
Congenital heart Metabolic acidosis Haemorrhage Acute blood loss
disease
Hypoglycaemia Edema Hypovolaemia
Hypothermia Drugs Twin-twin
transfusion
Hyperviscosity

* - Common important ones

APPROACH TO NEWBORN PROBLEMS

History
The purpose is to identify high risk pregnancies and babies.

• Prenatal /Perinatal history

• Preganancy history
• Apgar score
• Membrane rupture etc
• Neonatal

Examination
a. At birth – Is very brief. The aim is to check for life-threatening conditions

b. Within 24 hours – after the baby has settled down form labour distress
c. Subsequentl Examination - depends

Investigation
The minimum requirements are
1. Haematocrit
2. Dextrostix
3. Blood sugar (Glucometer)

Therapy
General
Specific

Prevention
 PHYSIOLODICAL ADJUSTMENTS

a. Respiratory Adaptation
- Stimuli for onset of respiration are obscure, but the following are known
i. Cold, light, noise & pain
ii. Respiratory acidosis – in form of hypoxia
- The first breath is responsible for
i. Conversion of fetal to adult circulation
ii. Emptying of lung field. Fetal lungs contain 40 – 80ml of fluid at
term.Thoracic squeeze during vaginal delivery helps to remove
part of this.
Note, suctioning if too much, it may stimulate vagus nerve in
pharyngeal wall, thus it should be discouraged.
iii. Establish of neonatal lung volume & pulmonary function.
Respiratory rate – approx 40/min, note > 60/min is abnormal.

b. Circulatory Adjustments

Fetal Circulation
• Consists of parallel circuits i.e both circuits are have the same
resistance
• Comparable levels of pressure in the ventricles because both
ventricles pump against systemic resistance.
• High pulmonary vascular resistance
• Persistence of PDA and foramen ovale

Adjustment at Birth
1. Removal of placenta from circulation causes increase in systemic
resistance and there is transfer of gas exchange to lungs.
2. The first breath brings about decrease in pulmonary vascular
resistance and consequently blood flow to the lung.
3. Closure of PDA & Foramen ovale leading to “4”
4. Serial pumping i.e Body → Right ventricle → Lungs → Left
ventricle → Aorta
Ductus Arteriosus
Closure “OFIB” Re-opening
1. Oxygen 1. Hypoxia from birth
2. Fluid restriction 2. Increased fluid intake
3. Indomethacin (within 2 3. Prostaglandin E2
weeks)
4. Blood 4. Anaemia
c. Renal Functions
• Output : 2 -3ml
• Specific gravity : 1.006 – 1.012
• There is increasing GFR with gestation age, thus antibiotics are given 12 –
hourly e.g penicillin instead of 6 hours. But after 1 week, it is changed to 6
hours.

d. Gastrointestinal

Meconium
• Majority of the babies pass meconium within 12 hours, 95% pass in the first
24 hours.
• It is passed for 3 -4 dyas, then
• Subsequently, the character of the stool depends on type of food.
Breastfeed – Soft yellow
Artificial - Pale firm or dry, making the child to be prone
to constipation.
Greenisk stool – is indicative of starvation
• Meconium consists of
i. swallowd amniotic fluid
ii. Mucous
iii. Intestinal secretion
iv. Desquamated cells

Feeding

a. Routes
a. Orogastric
i. Cup & Spoon
ii. Breastfeeding
iii. Nasogastric tube feeding
Note, In passing NG tube via mouth, don’t grease. Also to
ensure the tube is in the stomach, pass in air and listen over the
stomach.

b. Parenteral : Note, the maximal glucose that can be given via

peripheral vein is 10 – 12.5%. If larger is to be given, a central vein
cannulation is needed.

b. Frequency of feeding : 2- 3 hourly

 c. Amount
Term Preterm
Amount (ml/kg/day) 60 - 70 80 – 90

- This is increased by 10 – 20ml/day

Calorie Intake : 100 – 120 cal/kg/day

Weight gain : 20 – 30g/day

Weight change in Early life of Neonates

Age in days

Appropiate for Gestational Age (AGA) Babies

- Loses about 5-10% of birth weight within 3 – 4 days
- Regains birth weight over 7 – 10 days
- Then start to increase on birth weigth by 20 – 30g/day

Small for Gestational Age Babies

- Loses 0.3% within 3- 4 days, then start to gain rapidly.

e. Haematological

Normal Values in Neonate

a. PCV (Venous)
1st week : 45 – 65
Remaining 3 weeks : ≥ 35%
 b. WBC : 5 – 20,000 cells/mm³
Days
1 -3 Neutrophils > Lymphocyte
3-5 Equal
5-8 Lymphocyte > Neutrophil (Infantile
Picture)
Neonatal Neutrophils > Lymphocyte
Period

c. Platelet : ≥ 100, 000

d. Blood Volume : 80 – 90 ml/kg

f. Biochemical Parameters
Parameters
Glucose ≥ 40mg/100ml (Irrespective of
age)
Sodium 130 – 145 mEq/L
Potassium 3.5 – 4.5 mEq/L
Bicarbonate 18 – 25 mEq/L

g. Growth Parameters
Age Weight (kg) OFC (cm) CHL (cm)
Birth 3 35 50
6 months 6 44
1 year 9 47 75cm

Increase
Weight gain : 20 -30 g/day
Occipito-frontal CIrcumference
Term baby : 0.5 cm/week
Pre-term baby : 0.8 cm/week

Phone Number of OFC : 432111

- Head grows faster in the first 6 months i.e by 9cm and slowly in the last 6
months, by 3cm.
 h. Primitve Reflexes

Reflexes Onset (weeks of Well-established Disappearance

gestation)
1. Moro 28 -32 37 4 months
2. Palmar 28 32 4 months
3. Tonic neck 33 1 month of life 7 months
4. Pincer grasp --- 9 months of life For life
5. Parachute --- 9 months of life For life
6. --- 32 -34 weeks By 6 -9months, it
Sucking/Swallowing becomes
voluntary
7. Rooting 29 --- 9months
 Chapter 27
NEONATAL HAEMATOLOGY

HAEMATOLOGIC DISORDERS IN THE NEWBORN PERIOD

Anatomy /Physiology

Fetal circulation
Ductus arteriosus → Right atrium → Foramen ovale→ Left atrium → Left
ventricle → Aorta

• Aortic oxygen saturation in foetus is 45%, thus requiring compensatory menhanisms

: Polycythemia (due to increased erythropoietin production) & HbF.
• Retic count is 3 -7%
• Fetal haemoglobin increases wth gestational age, at about 28 weeks, fetal Hb is about
45%. From here, it progressively increases. Below 28 weeks, the PCV is low.
• Fetal blood volume
In-utero : 115ml/kg ( 75ml/kg in foetus & 40ml/kg in placenta)
- the volume (rbc & plasma) increases with weight

At birth : 80 – 90ml/kg. This is because some blood in placenta goes to the

baby.
• After birth, oxygen saturation increases to 95%, thus there is no need for
polycythemia and the erythropoietin production falls. The rbc die after exhausting its
life span.
Newborn - 90 days
Preterm - 75 days
Adult - 120 days

• The haemoglobin level falls. It decreases to a minimum by 8 – 12 weeks of life after

which erythropoiesis resumes again.
• HbA /HbF starts to increase
• 2,3 –DPG increases
• Oxygen delivery to tissue increases
HAEMATOLOGICAL INDICES

1. Haemoglobin (g/dl)
Term 14 – 20
Preterm 14 at 28 weeks of gestation. It is 1 -
2g/dl less.

2. Haematocrit 45 – 65%, it is lower in preterm

3. White blood cells (Varies

with post-natal age)
Day 1 6,000 – 35,000/µL (very wide)
Week 1 8,000 – 16,000/µL (On average, there is
fall
First month 6,000 – 14,000 (On average, there is fall)
- The values are a little lower in preterm.
- Differentials also vary with age.

Differentials

a. Neutrophils
%
Day 1 50 – 80 (Neutrophil predominance)
Day 4 35 – 60 (Is falling)
Day 7 35 – 45 (Is falling) (Approx % of lymphocytes)
3 months – 7 years 25 – 45 ( typical childhood lymphoctes
predominance
- At about 7th day of life, neutorphils and lymphocytes are about equal.

b. Lymphocte
%
Day 1 31
Day 7 41
Day 14 48

4. Platelets : 150,000 – 400,000/µL (May be up to 600,000/µL by 2 – 4months )

 5. Retic count
%
Day 1 2–8
Day 7 0.5
1 month 0 – 0.5

- In haemolytic disease and congenital heart disease, it is higher.

N.B - Don’t just memorize all the parameters, understand the trend.

ANAEMIA OF PREMATURITY

Introduction
- Is an exaggeration of physiological anaemia
- Is common in preterm because of lower rbc life span (75days) and higher
tendency to be sick.

Reasons For Higher Incidence Rate in Preterm

i. Minimum value of PVC is reached earlier in preterm than in term babies which is
about 6 weeks (1- 3 months)
ii. Their rbcs have reduced life span
iii. Iatrogenic – samples for investigation.
iv. Because of relative more rapid somatic growth rate. There is fluid redistribution.
v. Due to Vitamin E deficiency – from relative fat absorption

Preterm infants start to produce erythropoietin again when Hb falls to 7 – 9g/dl in

contrast to 10 – 11g/dl in term infants because their tissues require lower oxygen
requirement.

Clinical Presentation
i. May be asymptomatic
ii. Pallor
iii. Apnoea
iv. Poor weight gain
v. Tachypnoea
vi. Tachycardia
vii. Re-opening of Patent ductus arteriousus
N.B – The difference between physiological and anatomical closure of PDA.

Management

i. If symptomatic – give blood transfusion slowly with 10ml/kg of packed cells

over 1 – 3 hours. Note, haematinics are not used because it takes longer time to
act.)

Prevention

i. Adminstration of folic acid 5mg weekly to babies < 2kg from 2 weeks of age.
ii. Adminstration of 10mg α – tocopherol acetate (Vit E) daily to babies < 1.5kg from
2 weeks of age
iii. Administration of Ferous sulphate, 50 mg/day or elemental iron 6mg/kg/day
from 2kg or 10 – 14 weeks of age. This should not be given early to avoid iron
excess. (unlike Folic acid & Vitamin B12 which do not result into excessive state)
iv. Use of recombinant human erythropoietin, 100 -200iu/kg, given 5d /week,
400u/kg/d, 3d/week + iron + vit. E. This is forcing baby to start erythropoieis,
after which iron can be given in this case.

ANAEMIA

Introduction
- Is the most common haematological disoder
- The definition is not categorical
- Is reduced haemoglobin below normal for gestational and post-natal age.

Classification
a. Early-onset Anaemia
b. Late-onset Anaemia
 EARLY-ONSET ANAEMIA
- Occurs in first few days after birth
- Is most frequently due to haemolytic disease or haemorrhage

Causes
i. Rhesus haemolytic disease
ii. ABO haemolytic disease
iii. G6PD deficiency – is common here, 20 – 25% of male population
iv. Infections – causes suppression of bone marrow and excessive
haemolysis
v. Neonatal haemorrhage –
a. Birth trauma - May result into subperiosteal haemorrhage
(because of its loose areolar tissue)
b. Cephal haematoma
c. Subgaleal haematoma
d. Ruptured Liver or spleen etc
e. Intra-abdominal haemorrhage
f. Fracture of femur
vi. Maternal infevtions e.g parvo-virus
vii. Others
a. Spherocytosis
b. Α – thalassemias
c. Fetal haemorrhage
- Abruptio placentae
- Vasa praevia
d. Fetomaternal transfusion
e. Twin-twin transfusion
f. Congenital hypoplastic anaemia (Diamond–Blackfan Anemia)

Clinical Presentation

History of
• antepartum haemorrhage (APH)
• multiple delivery
• instrumental delivery
• poor feeding
• breathlessness
 Physical examination
• Colour
• Bruises – scalp, shoulder
• Cephal haematoma – is limited at suture line
• Rigid abdomen
• Small pulse volume
• Tachycardia
• Hypotension
• Tachypnoea
• Fever /Hypothermia – suggesting infections

Investigation

i. PCV
ii. Hb estimation
iii. WBC - pancytopaenia is a feature of aplastic anaemia
iv. Blood film – may show spherocytosis

Treatment
Depends on severity

a. Asymptomatic – leave the child

b. If in shock (and the child is term) i.e BP < 25mmHg, PCV < 30%, pH < 7.1
- Immediate transfusion with 15 – 20ml/kg of whole blood over 5 –
10minutes ( if it is due to haemorrhage)

c. If not in shock, but anemia is severe

- Transfuse 20ml/kg over 2 – 3hours with IV frusemide, 2mg/kg
or
- Packed cell 10 – 15ml/kg (2 -3 mls/kg/hour)
Note : 2ml/kg of packed cells raises haemoglobin by 0.5 – 1g/dl or PCV of 10%.

d. Very severe anaemia Hb < 8g/dl or the child is in congestive

cardiac failure
- Single volume EBT with packed cells
- The procedure involves
o calculating the total volume
o Taking of blood in aliquots (10mls for each round)

- At the end, the PCV will have risen by 65%

LATE ONSET ANAEMIA
- This appears later in the neonatal period

Causes
1. Mild haemorrhagic disease of the newborn
2. Haemolytic disease of the newborn – ABO incompatibility (Note Rhesus
isommunization often presents as early-onset anaemia
3. Chronic blood loss e.g GI bleeding (Merkel’s diverticulum)
4. Infection – causing DIC by the toxins which damage to the endothelium,
stimulating consumption coagulopathy. It also depresses bone marrow.
5. Chronic infactions e.g rubella, parvovirus
6. G6PD deficiency
7. Spherocytosis
8. α – thalassemia
9. Congenital hypolplastic aplasia
10. Repeated venpuncture

Management
If it is severe, do exchange blood transfusion. But often, it is not because the bleeding
is slow giving time for compensation, thus you just need to transfuse,
 HYPERVSCOSITY STATES

Introduction
- It is important that plasma osmolality is maintained.

POLYCYTHAEMIA

Definiton
- Is venous PCV > 65% ( the more the PCV above 65%, the low the oxygen carrying
capacity due to increase in viscosity.

Aetiology
1. Placenta insufficiency – due to placental infarcts e.g in diabetes mellitus
2. Maternal –fetal transfusion – due to delayed cord clamping
3. Twin-twin transfusion e.g monozygotic twins
4. Infants of diabetic mothers (IDM) - due to hypoxia caused by vascular problems
5. Dehydration
6. Others
a. Congenital adrenal hyperplasia
b. Trisomy 13, 18, 21
c. Neontal thyrotoxicosis
d. Becwith-Weidemann syndrome
e. Maternal drugs e.g propanolol

Clinical Presentation

May be asymptomatic

General
o Plethora
o Jaundice
o Cyanosis (≥ 5g/dl of deoxygenated blood) - may be a cause or effect.
o Prolonged capillary refill

Respiration
o Tachypnoea
o Dyspnoea
 GIT
o Feeding problem
o Necrotizing enterocolitis

CNS – Mostly undesirable

o Irritability
o Jitteriness
o Lethargy
o Seizures

Investigation

1. Bilirubin - hyperbilirubinaemia
2. Blood sugar – hypoglycaemia
3. Serum calcium – hypocalcaemia
4. Chest X-ray – shows prominent pulmonary vascular markings

Complications

1. Thromboembolism
2. Pulmonary haemorrhage
3. Congestive cardiac failure
4. Brain damage

Management

a. If PC < 70% and the child is asymptomatic

- Admit and observe
- Ensure no dehydration

b. If < 70% and symptomatic

- Do erythropoiesis ( note, not plasmapharesis)

c. If > 70% and asymptomatic

- Treat

Calculation

Volume (ml) = Blood volume X ( Observed – Desired PCV )

To be removed Actual PCV

Blood Volume = 80 – 90ml X Weight

 The following can be used
i. plasma
ii. Fresh frozen plasma
iii. Normal saline
iv. Haemacel

Precaution
i. At any time, don’t take > 25% of blood volume.
e.g Calculate the volume of blood to be removed from a child
that is 4kg. The answer is 94mls. This is mors than 25% of the patient’s
volume, thus you take 80mls.

BLEEDING DISORDERS

Introduction

Clotting cascade

Intrinsic Extrinsic

Common Pathway

Thrombin

( Fibriongen → Fibrin monomer → Fibrin polymer → Stable fibrin + platelet plug

Plasmin

Fibrin degradation products (FDP)

– in excess, it causes haemorrhage
Tests of Coagulation

1. Prothrombin time
- Is for extrinsic system
- It measures the activity of factors 2,7,9,10. These are Vitamin K-
dependent factors.
- Normal range is 13 – 20s

2. Plasma thromboplastin time with kaolin

- Is for intrinsic pathway
- It measures 12, 11, 9, 8 and factors in common pathway.
- It also measures some vitamin K-dependent factors, 2 & 10
- It is used to monitor heparin therapy
- Normal range is 30 – 45s

Note, In vitamin Vitamin k deficiency, both PT & PTTK are done.

3. PT ratio ( Test/Control )
Normal range = 0.9 – 1.2. If ≥ 1.3, you intervene even if the patient is
not bleeding.

4. Fibrinogen – Normal range is 150 – 300mg/dl

Disease Clinica Platele PT PTTK Other tests ( to
l state t confirm)
1. Haemorrhagic Well Normal ↑ ↑ Fibrinogen/FDP –
disease of the Normal, because there
newborn is no excessive
breakdown of clots
2. Idiopathic Well ↓ Normal Normal Maternal platelet
thrombocytopaenic count – decrease
purpura (ITP confirms it
3. Large Haemangioma Well ↓ Normal Normal Maternal platelet
count – normal
4. Bone Marrow Well ↓ Normal Normal Peripheral blood film –
Hypoplasia shows pancytopaenia
5. Haemophilia Well Normal Normal ↑ Factors 8 & 9 Assay
6. Liver disease Sick Normal ↑ ↑ Albumin, Fibrinogen &
LFTs are deranged
7. Intrauterine TORCH Sick ↓ (may N or ↑ N or ↑ Fibrinogen, Albumin
Infection be)
8. Infections Without Sick ↓ Normal Normal FBC, Blood culture
DIC e.g Septicaemia
9. Infections + DIC (or Sick ↓ ↑ ↑ Fibrinogen - ↓
in any cause of DIC ) FDP - ↑
PBF – fragmented rbc due
to trapping

o ITP – there is autoimmune antibody against platelets

o In Large haemangioma, platelets are trapped at the bleeding site – Kasabach

Meritt syndrome

o In Intrauterine TORCH infection, the bone marrow may be suppressed. PT &

PTTK may (not) be deranged depending whether liver is affected or not.
HAEMORRHAGIC DISEASE OF THE NEWBORN

Introduction

o It is due to deficiency of vitamin K in newborn.

o Normally, the gut flora of baby is sterile and breast milk is not rich in vitamin K.
o It takes about a week for the baby to acquire gut flora.
o Vitamin K received from the mother disappears after few days, 2 – 7days, leading
to the inactivation of vitamin K dependent factors, thus prone to HDN.
o It may result into provoked or unprovoked bleeding. The common sites of
haemorrhage are :
i. GIT
ii. Nose
iii. Cord
iv. Intracranial
v. Circumcision
vi. Trauma

Types

a. Early HDN
- Occurs < 24 hours of age. Here, you should search for other possible causes

b. Late HDN
- Occurs > 1 week

Predispocing Factors to Early or Late HDN

1. Birth Asphyxia
2. Maternal drug ingestion e.g phenobarbitone, aspirin, anticoagulant (coumarin) –
leading to vitamin K deficiency in the mother
3. Exclusive breasfeeding – since breast milk is not rich in vitamin K
4. Broad spectrum antibiotics – reduce the normal flora in the gut
5. Total parenteral nutrition – delays the aquire of normal gut flora through oral
feeding.

Clinical Presentation

• Bleeding – provoked or unprovoked

• On examination, patient may look well in the absence of other conditions
Investigation - as shown in the table above

1. Packed cell volume

2. FBC - normal
3. Platelet count – normal
4. Prothrombin time - prolonged
5. PTTK – prolonged
6. Fibrinogen – normal
7. FDP – normal

Treatment

a. Non-severe
• Give Intravenous Vitamin K1, 1 – 5mg stat. ( K2 & K3 are not used if
patient is G6PD deficient because they cause haemolysis.

b. Severe & Life non-threatening

• Intravenous Vitamin K stat
• Double volume EBT with fresh whole blood - to remove inactivated
clotting factors, called Protein-induced Vitamin K factors with vitamin
K replenishment.

Note
Single volume EBT – 65%
Double volume EBT – 83 -85%. It is used often used as the maximal dose.

Prevention

1. Rotine intramuscular vitamin K1, 1 mg stat, to every newborn baby. For preterm,
the dose is 0.5mg stat.
2. For special babies (with any of the conditions listed above e.g total parenteral
nutrition), you continue to give 0.5mg / week until the baby starts feeding.
DISSEMINATED INTRAVASCULAR COAGULOPATHY

Predisposing Factors
- are causes of genralised vascular damage

i. Severe birth asphyxia

ii. Septicaemia
iii. Hypothermia
iv. Hypotension
v. Acidosis
vi. Hypoxia

Pathogenesis
Is due to consumption of clotting factors

Clinical Presentation
i. Provoked bleeding - from puncture sites
ii. Unprovoked bleeding e.g petechiae, spontaneous massive bleeding form
orifices.

Investigation
_ As in the table above

Treatment

i. Treat the underlying cause

ii. Specific treatment
• Give Platelet concentrate /Platelet riach plasma + transfusion with
Fresh frozen plasma using 10 – 15ml/kg
• Give Vitamin K1 intravenously

If Severe
Do EBT with hreash whole blood – to remove the toxins & FDP which causes
haemorrhage
 Chapter 29
RESPIRATORY PROBLEMS IN THE NEWBORN

• Outline of Causes of Respiratory problems in the Newborn

• Discussed Below
1. Transient Tachypnoea of the Newborn
2. Respiratory Distress Syndrome /HDM
3. Meconium Aspiration Syndrome
4. Congenital pneumonia
5. Neonatal pneumonia

CAUSES

a. Respiratory System
6. Aspiration syndromes – Amniotic squames, Meconium, Milk, Blood
7. Pneumonia
8. Transient tachypnoeaof the Newborn
9. Redspiratory distress syndrome
10. Pneumonthorax
11. Diaphragmatic hernia
12. Other malformations – Renal atresia, Lobar emphysema

b. Vascular
8. Persistent fetal circulation
9. Heart failure
10. Congenital malformation
11. Hypovolaemia
12. Anaemia
13. Polycythemia

c. Metabolic
14. Hypoglycaemia
15. Acidosis
16. Hypothermia
d. Neuromuscular
17. Cerebral oedema
18. Cerebral haemorrhage
19. Drugs
20. Mucsular disorder
21. Phrenic nerve damage
 I

TRANSIENT TACHYPNOEA OF THE NEWBORN

Introduction
o It is also called Wet lung disease or Delayed Clearance of lung water.
o In-utero, the respiratory rate is 30 – 70, but this is not effective

Pathogenesis
o Is due to presence of fluid in the lungs in the amount more than what should
be there at birth.
o Towards birth, the rate of secretion of fluid is reduced and reasorption is
increasing. Also almost 75% is lost during labour, ans some as well during the
passage of baby through the birth canal.
o The effect is transient

Risk Factors
o Maternal diabetes mellitus – it interferes with fluid clearance at term and
during labour
o Maternal sedation – also reduces birth canal effect
o Caesarian delivery – no birth canal effect

Clinical Presentation

i. Tachypnoea ( Respiratory rate : ≥ 60/min)

ii. Grunting
iii. Chest wall indrawing
iv. Nasal flaring
v. Hyperinflation
vi. ± mild depression at birth

Diagnosis
Is made by ecluding other conditions
Investigation

1. Chest x-ray : shows sunburst appearance ( due to engorgement of lymphatic

vessels) which clears in 24 hours .

Treatment
i. It is benign and self-limiting. The child recovers in 24 – 72 hours by which the
lymphatics would h ave ultimately reabsorbed the fluid.

II
RESPIRATORY DISTRESS SYNDROME
(Hyaline Membrane Disease)

Introduction
o Is progressive atelectasis of prematuirty

Epidemiology
o Occurs worldwide, but prevalence is less in this environment
o Male > Female

Predisposing Factors
1. Prematurity
2. Elective Caesarian section
3. Infant of diabetic mother – glucose suppresses with surfactant maturation, even at
full term.
4. Second twin
5. Perinatal asphyxia
6. Antepartum haemorrhage
7. Shock

The risk is reduced in

1. Premature rupture of membrane (PROM)
 2. IUGR
i. Placental insufficiency
ii. Anaemia
iii. Maternal stress – becauses stress induces steroid ( surfactant) formation
3. Maternal stress e.g pre-eclampsia
4. Exogenous steroid

Pathophysiology
o Surfactant, a phospholipid which reduces surface tension especially expiration.
o Components of surfactant are Lecithin & Sphingomyelin. With age, lecithin
increases more than sphingomyelin. Mature surfactant lecithin/sphingomyelin ratio
is 2: 1
o It is due to inadequate surfactant synthesis which results in high alveolar surface
tension and reduced lung compliance. The alveoli close during expiration and do
not open up during inspiration.
o This leads to progressive expiration atelectasis which leads to hypoxia. There is
capillary permeability resulting into exudation of fluids into already compromised
lung with surfactant deficiency.

Clinical Presentation

i. Onset – near time of birth

ii. Tachypnoea
iii. Chest wall indrawing
iv. Expiration grunt
v. Cyanosis
vi. Fine inspiratory crepitattions
vii. Hypothermia ( from tachypnoea)
viii. There is progressive deterioration (unlike in Transient Tachypnoea of
Newborn ) in 3 -5 days. If the child does not die, it begins to recover
becauses the production of mature surfactant will start by this time.

Investigation

1. Chest x-ray
- The finding is characteristic, but not particular to RDS.
- There is fine reticulogranular appearance (ground glass ) with air
bronchogram sign – air is still seen in bronchial tree and signs seen in
bronchopneumonia which develops during the first 6 hours of life.
Treatment

- It is self-limitng, as the endogenous production begins, it relieves, thus the

treatment is supportive.

i. Oxygen therapy
ii. Broad spectrum antibiotics (prophylactic)
iii. Adequate fluid balance – the fluid accumulation may result into infection
iv. Maintenance of body temperature
v. Ventilatory support

Indications for Mechanical Ventilation

i. Hypoxia – that is resistant to other measure
ii. Hypercapnia - PCO2 > 60mmhg, pH < 7.25
iii. Apnoea

Prognosis
The infants rarely die

Prevention
1. Prenatal glucocorticoid e.g dexamethasone for > 24 - 48hours prior to delivery.
2. Synthetic surfactant replacement within 1 – 2 hours of life via endotracheal tube.
 III

MECONIUM ASPIRATION SYNDROME

Introduction
- Is often encountered here
- Is often a problem of term infants

Aetiopathogenesis

o During intrauterine life, anal sphincter is closed, therefore baby does not pass
meconium until near of at term.
o Passage is caused by hypoxia or under stressful conditions which causes
opening of the sphincter. (i.e risk factors are hypoxia & IUGR)
o The same hypoxia makes the baby gasp in-utero, taking the meconium-stained
liquor into airways.
o Fresh meconium (colour is bottle green) more often causes airway obstruction
& air trapping due to the particulate in the meconium while stale meconium
(colour is brownish green) often causes chemical pneumonitis.
o The obstruction results into hyperinflation due to the ball-valve effect of the
partial obstruction – Fresh Meconium Aspiration Syndrome)
o Complications
i. Pneumothorax
ii. ± Pneumomediastinum
iii. Chemical pneumonitis
iv. Bacterial infection
v. Persistent of hyperinflation for some years

Clinical Signs
- Is a syndrome constituted by
1. Respiratory distress – that cannot be explained by any other condition in a
baby that has passed meconium-stained liquor.
2. Meconium staining of the skin (appears yellowish), nails, umbilical cord
3. Respiratory distress soon after birth

Crepitations or rhonchi (air trapping) in lung fields

Increased anteroposterior diameter of the chest
Investigation

Chest X-ray
- Shows overinflation, streaking atelectasis or diffuse opacification.

Note, all these features are not pathognomonic.

Course
Recovers in 7 – 10 days if the baby does not die

Management
o Anticipation – most important
o Paediatrcian must present at delivery
o Visualization and direct suctioning of trachea immediately after birth.
Endotracheal tube is used to suck below the vocal cords.
o Blood gas monitoring
o Oxygen therapy
o Antibiotics
o Assisted ventilation

CONGENITAL PNEUMONIA

Defintion
Is pneumonia acquired from the mother prior to delivery.

Routes of Infection
i. Ascending infection – from the genital tract
ii. Blood borne – via the pacenta

Onset
Is usually within first 3 days of life (if > 3days, it is acquired pneumonia)

Predisposing factors
1. Premature labour
2. Premature rupture of memebrane
3. Prolonged rupture of membrane ( > 24 hours before delivery )
4. Prolonged labour
5. Frequent digital examination
Infective Agents
i. Group B streptococcus
ii. E. coli
iii. Klebsiella species
iv. H. influenza
v. Listeria monocytogenes – causes greenish-yellowish liquor in preterm
vi. Anaerobes

Note : ii & iii are common in Ibadan

Clinical Presentation
i. May have lower Apgar scote
ii. Tachypnoea
iii. Grunting
iv. Chest wall indrawings
v. Apnoea
vi. Shock

Investigation
- For any infection in babies, do full sepsis screening

i. Chest x-ray
ii. Blood culture
iii. FBC
iv. Gastric and tracheal aspirate m/c/s

Treatment
1. Oxygen therapy
2. Keeping the baby warm
3. Broad spectrum antibiotics – Cefuroxime + Gentamicin to cover for Gram +ve & -
ve, but does not affect Listeria monocytogenes, though it is not common here.
 NEONATAL PNEUMONIA

Introduction
- Occurs after the first 3 dyas of life.
- NO evidence of preceding amnionitis or maternal infection.

Agents
i. Gram –ve : E. coli, Klebsiella
ii. Gram +ve : Staphylococcus

Investigation & Management

- As above.
 Chapter 29
BIRTH ASPHYXIA/TRAUMA

Definition
o Is the condition in the newborn where there is reduced oxygen saturation
(hypoxaemia) and increased acid in blood ( acidaemia) from CO2 retention
and lactic acid accumulation.

Concept of Primary & Secondary Apnoea

1. Primary apnoea – Asphyxia Livida

2. Secondary apnoea – Asphyxia Pallida

Course
Hypoxia Gasping Hypoxia Cyanosis A. Livida

With subsequent gasping and intervention is not offered, it results into Asphyxia
Pallida – there is circulatory collapse and the patient is pale.

Resuscutation has a good prognosis in primary apnoea i.e 1st gasp.

Note, human beings can only sustain hypoxia for about 5 minutes, after then , it
affects the brain.

Aetiology / Predisposing Factors

1. Maternal (Prenatal) Factors

i. Infection – chorioamnionitis

ii. Lungs – Pneumonia, Asthmatic attack etc

iii. Cardiac – Arrythmias, hear failure etc

iv. Vascular –
a. Anaemia due to haemoglobinopathy, poor nutirional and
leukaemia
b. Diabetes mellitus – due to arterioslecrosis
c. Hypertension - due to arterioslecrosis
d. Hypoperfusion

v. Uterus
 a. Uterine hypertonia – titanic contractions reduces blood flow to the
baby.
b. Malformations
c. Uterine rupture

vi. Others
a. Narcotics – via respiratory depression in mother of direct effect on
the baby
b. Anaesthetic agent
c. Alcohol

2. Prolonged obstructed labour

3. Placental problems
a. Abruptio placenta
b. Placenta praevia
c. Placental insufficiency

4. Foetal factors
a. Premaurity – affect respiratory centre

b. Postmaturity – due to placental insufficiency

c. Developmental anomalies
i. Diaphragmatic hernia
ii. Hypolplasia
iii. Choanal atresia

d. Cord problems : Knotting, Compression or Proplapse

e. Infection : Congenital pneumonia

Assessment

o Anticipation is the KEY to good care

o Maternal & Fetal Monitoring before & during labour is essential
o Fetal heart rate
o Scalp blood pH
o Cardiotocography – to monitor variability in FHR.

o Apgar Score
Is assessed at 1 & 5 minuites, though it may be extended to assess the
extent of resuscitation required.
 a. 1 minute : to determine the amount of resuscitation

b. 5 minutes : To prognosticate ( especially on brain)

If it is low after 5 minutes, the child is likely to have long term sequelae.

Apgar Score
Score 0 1 2
Appearance /Colour Pale or central Peripheral cyanosis Completely pink
cyanosis
Pulse rare /Heart Absent < 100 b/min > 100b/min
rate
Grimace/Irritability None Grimace Cry /sneezing

Activity/Muscle tone Flaccid Some flexion Well flexed

Respiration Absent Weak irregular Regular

Maximal score = 10

Grading
Score Grade Management
≥7 Resguires no active resuscitation
6 Mild birth asphyxia Suctioning

4–5 Moderate birth asphyxia • More suctioning, but avoid vigorous suctioning to
avoid vagal reflex
• Oxygen by face mask, occasionally bag & mask
1–3 Severe Birth Asphyxia • Clearing of airways
• Endotracheal intubation & ventilation by bag and
mask

Complications of Severe Birth Asphyxia

1. Central Nervous system

i. Syndrome of Inappropiate ADH secretion
- Inadequate urine output
- Hyponatraemia
- Oedema
 - Weight gain
ii. Intracranial haemorrhage
iii. Hypoxic Ischaemic Encephalopathy
• Seizures
• Hypotonia
• Mental retardation
• Learning disability
• Speech disorders
• Cerebral palsy

2. Respiratory System
i. Respiratory distress
ii. Respiratory distress syndrome, tyoe II
iii. Persistent pulmonary hypertension

3. Cardiovascular System
i. Left ventricular dysfunction - due to myocardial ischaemia
ii. Tricuspid incompetence
iii. Patent ductus arteriosus
iv. Patent fetal circulation
v. Cardiogenic shock

4. Renal
i. Bladder atony – different kidney failure which requires fluid challenge
ii. Acute tubular necrosis – causing haematuria
iii. Acute cortical necrosis
iv. Haematuria

5. Gastrointestinal
i. Necrotising enterocolitis – is due ischaemia which causes bacterial
proliferation
ii. Liver dysfunction

6. Haemopoeitic
i. Marrow suprresion
ii. DIC – due to generalized hypxia
iii. Increased risk of infection

7. Metabolic
i. Hypo or hyperglycaemia
ii. Hyponatraemia – from renal failure
iii. Hypocalcaemia
iv. Acidosis
Management

Resuscitation in delivery room – most important

A – Clear airway
B- Ensure ventilation, 100% oxygen
C- Ensure circulation by gicing volume expander if in shock
D. Drugs e.g
i. Naloxone, 0.1mg/kg to reverse effects of narcotics
ii. Bicarbonate, 1 – 2mEq/L, diluted & only give when the baby has
started breathing
iii. 50% dextrose, 0.5 -1mg/kg diluted – to treat hypoglycaemia.
 Chapter 30
LOW BIRTH WEIGHT INFANTS

• Gestational Age Assessment

• Low birth weight Infant
- Definition
- Incidence
- Aetiology
- Clinical Features
- Comparison of SGA & Immature infants
- Management
- Prevention

GESTATIONAL AGE ASSESSMENT

Aim
i. Identification of high risk infants
ii. Interpretation of neurological & behavioural evaluation of
developmental progress

Criteria
i. Physical signs e.g Farr scoring system – consists of skin, lanugo hair,
immature external genitalia, palmar & planter crease
ii. Neurological signs e.g Amiel-Tison
iii. Combined physical & Neurological Signs e.g Dubowitz & Ballard
Scoring System.

Checking Appropiateness of Weight for Gestataion – Using Olowe’s or Freeman’s

chart

Weeks of gestation

¾ 90% centile – Large for gestational age

¾ < 10% centile – Low birth weight (SGA)
¾ 10 – 90% centile – Appropiate for gestational age (AGA)
THE LOW BIRTH WEIGHT

Definiton
- Is an infant weighing < 2.5kg at birth irrespective of gestational age.

Incidence : 7 – 8% in Ibadan. It is 21% in India

England ( %) Nigeria ( %) India ( %)

Low Birth Weight
AGA 60 30 20
SGA 30 60 80

Aetiology

1. Placental insufficiency
i. Toxaemia (Pre-eclampsia & Eclampsia)
ii. Hypertension – causes placental infarction
iii. Malaria

2. Chronic intrauterine infection

i. Cytomegalovirus } - both cause SGA
ii. Rubella }

iii. Toxoplasmosis } - both cause preterm birth (AGA)

iv. Syphilis }

Note – Bacterial infections are not associated with growth retardation

3. Inherited factors - Familial

i. Achondroplasia
ii. Trisomies 13, 18 & 21 ( E P D )
iii. Cri-du-chat
iv. Turner’s syndrome

4. Others
i. Multiple pregnancy – contributes to about 25% of LBW
ii. Antepartum haemorrhage
iii. Smoking – Asphyxia causes reduced oxygen supply to the fetus.
iv. Alcohol consumption – causing Fetal Alcoholo Syndrome which
consists of the following :
 • Small for gestational age
• Microcephaly
• Mental retardation
• Limb defects
• Cleft lip & palate
• Cardiovascular anomalies

v. Noxious agent
• Radiation
• Phenytoin
• Antimetabolites
vi. High altitude – has low oxygen causing SGA & polycythemia
vii. First borns – are usually smaller than other siblings
viii. Anaemias
ix. Sickle cell disease

5. Unknown – seen in 2/3rd of the cases.

Important Causes of LBW in Nigerira

1. Multiple pregnancy
2. Toxaemia of pregnancy
3. Sickle cell anaemia

Weight change in Early life of Neonates

Age in days

Appropiate for Gestational Age (AGA) Babies

- Loses about 5-10% of birth weight within 3 – 4 days
- Regains birth weight over 7 – 10 days
- Then start to increase on birth weigth by 20 – 30g/day
 Small for Gestational Age Babies
- Loses 0.3% within 3- 4 days, then start to gain rapidly.
Age in days

Clinical Features
- To assess the age of gestation

Size
This is very important as there are 2 groups of SGA, and it depends on
the stage of gestation at which the growth is retarded.

a. Uniformly retarded /Symmetrical

- Occurs earlier in pregnancy e.g congenital
rubella syndrome

b. Disproportionate retardation
- Occurs late in pregnancy
- The head size is normal ( as brain growth is least
affected by IUGR resulting into Head-body
disproportion that may so marked that you suspect
hydrocephalus.
- The length of the child is normal
- The weight would be small.
- Has Old man’s appearance (Wizened)
- There is loss of buccal fat
- Has hungry looking
Has scaphoid abdomen because of shrunken liver
 Comparison of Problems of SGA & Immature Infants

Factors Small For Gestational Immature/Appropiate GA

Age
1. Birth weight Low Normal
2. Early Weight 0 - 3% los, then gain rapidly 5 – 10% loss, then slowly gain
change
3. Pulmonary problems o Aspiration syndrome o Hyaline membrane disease
o Pneumomediastinum (Lecithin/Sphigomyelin
o Pneumothorax ratio < 2: 1)
4. Apnoea Spells ± +++

5. Infection +++ ±
(Congenital)
6. Hyperbilirubinaemia + ++++
7. Hypoglcaemia +++ +
8. Haematocrit Normal or Polycythemia Normla or low
9. Congenital +++ ±
malformation
10. Intracranial + +++
haemorrhage
11. Persistent Fetal ++ +
Circulation
12. Growth Catch-up by 6 months or Normal
remain small

o Hypoglycaemia in SGA is due to

i. reduced hepatic glycogen stores
ii. reduced gluconeogenesis
iii. Increased basal metabolic rate
o The intracranial haemorrhage in Immature infants is due to immaturity of germinal
matrix underlying the ventricle.
o Problems of SGA infants ot very short gestation become less distinguishable from
those of immature ones.
Management of LBW

1. Temperature control
- Improves survival by 3-fold increase
- The child may be nursed in incubator until the child is about 1.5kg,
corresponding to 32 weeks of gestation/

2. Feeding

Weight Frequency
< 1.5kg 2 – hourly
> 1.5kg 3 - hourly

Using orogastric tube feeding. After passing orogastric tube, to confirm

if it is stomach or trachea, do these tests
i. Test aspirate with litmus paper (acidic)
ii. Put the open of the tube in water, if it is in trachea, it bubbles.

3. Control of infection

4. Treatment of complications
i. Patent ductus arteriosus
ii. Necrotising enterocolitis
iii. Respiratory problems e.g RDS, Apnoea

5. Prevention of deficiencies of iron & folic acid. This is started at

postnatal age of 4 -6 weeks because that is when anaemia of prematurity occurs.

Prevention of LBW

1. Improved maternal nutrition

2. Careful monitoring of high risk pregnancy such as multiple pregnancy,
antepartum haemorrhage, pregnant women with sickle cell disease.
 Chapter 31
NEONATAL INFECTIONS

• Bacterial Infections in the Neonate

• Neonatal Immunology
• Neonatal Septicaemia
• Cutaneous Infections
• Conjunctivitis

BACTERIAL INFECTIONS IN THE NEONATE

Introduction
Neonates are prone to infections due to
i. deficiency in his immunological make up
ii. the prevalence of predisposing factors
iii. the quality of obstetrical/neonatal care.

All these contribut to an increase in Neonatal Morbidity and Mortality.

Classification of Bacterial Infection in the Neonates

1. Based on Time of onset

a. Early onset : < 48 hours of age. The child must have got the infections
from the mother or at birth.
b. Late onset

2. Based on Characteristics of Infecting agents

a. Gram –ve
b. Gram +ve
c. Anaerobes

3. Based on Organ/System involved

a. CNS - Meningitis
b. Skeletal – Osteomyelitis/arthritis
c. Urinary – Urinary tract infections
d. Blood – Neonatal Septicaemia
 4. Superficial /Deep
a. Superficial – e.g Cutaneous infections, Conjunctivitis
b. Deep - Septicaemia, meningitis, UTI, osteomyelitis

5. Primary or Secondary
a. Primary – occur de-novo without any prior infection
b. Secondary

NEONATAL IMMUNOLOGY
The high vulnerability of neonates to infections is due to multiple defects in the
immune mechanism and these include

a. Non-Specific Methods

i. Decreased Complement
- Complements do not cross the placenta and therefore not
transferred to the baby.
- Fetal production of complements starts from the 10 weeks of
gestational age and increases to about 50 – 75% of maternal level at
birth, thus the complement in preterm & low birth weight is lower
than in full term babies.

ii. Neutrophil Dysfunction : Due to

- Reduced chemotactic & phagocytic responses
- Impairment of bacterial activity
- Reduced opsonic activity

iii. Low Level of Fibronectin

- Fibronectin is a high molecular weight glycoprotein which
participates in the immunologival clearance of complement of
antibodies coated materials e.g bacteria
- Newborns have low plasma concentration
- There is a direct correlation with the gestational age.
 b. Specific Immune Mechanism
o IgG crosses the placenta with the gestational age
o IgM does not cross the placenta. If IgM is seen in a child, it means the
child has been challenged.
o IgM contains the specific bactericidal activity against Gram –ve
organism

NEONATAL SEPTICAEMIA

Definition
o Is bacterial infection in infants aged < 4 weeks of life, who are clinically sick
and have a positive blood culture.
o It increases the morbidity & mortality
o It is a critical determinant of the outcome in VLBW.

Definitions of Terms

a. Primary & Secondary Septicaemia – as above

b. Presumed Septicaemia : when there are +ve predisposing factors

c. Probable Septicaemia : when there are clinical features & abnormal white
blood cells

d. Definitive Septicaemia – as above

Aetiology

Tropics England
Gram +ve : S. aureus Group B-haemolytic strept.
Gram –ve : Klebsiella
Anaerobes - not very common
Incidence
- It varies depending on
a. the population of infants studied – is commoner in preterm
b. the prevalence of predisposing factors
c. the level of obstetric care
- Is about 5 – 17.9/1,000 live births in the tropics.
- In Europe & America, it is 1/1,000 in full term & 4/1,000 preterm

Predisposing factors

1. Maternal Factors
i. Socio-economic status – there is reduced bacterial activity of the
amniotic fluid
ii. Illness – Septicaemia, UTI, Intrapartum pyrexia (is associated with
prolonged rupture of membrane – Amniotic Fluid Infections Syndrome
iii. Maternal Diabetes mellitus
iv. Obstetrical problems –
- SROM
- PROM (proloned)
- Prolonged Obstructed labour
- Antepartum haemorrhage – blood clot culture organism
suggesting of Amniotic Fluid Infection Syndrome which includes
discoloured foul semlling amniotic fluid
- Chorioamnionitis

2. Host Factors
i. Prematurity
ii. Impaired immunologic defenc mechanism
iii. Congenital anomalies e.g spina bifida, ectopia vesica etc.
iv. Birth Asphyxia requiring active resuscitation (Umbilical vessel
catheterization (UVC) Endotracheal Titubation (ETT) )
v. Sex : There is increased incidence in males because the gene for the
production of immnuoglobulins is on X-chromosomes

3. Environemtnal Factors
i. Resuscitative procedures - indwelling catheters
ii. Monitoring devices & ventilatory support

Pathogenesis

a. In-utero (Transplacental)
b. Intra-partum (Ascending)
c. Postpartum ( Nosocomial)
 Routes of Antenatal Infection
i. Descending infection
ii. Ascending
iii. Haematogenous

Routes of Postnatal Infection

i. Hands of attendants
ii. From other babies
iii. Equipments
iv. Feeding

Ascending Infection

Amniotic Fluid Infection

Aspiration Ingestion

Pneumonia GIT Colonisation

± Invasion of capillary wall

Bacteraemia

Aspiration Ingestion
Bacteraemia

Vaginal Secretions
Clincal Features of Neonatal Septicaemia
- Are non-specific and can mimic anyd disease, therefore a high index of
suspicion is needed for early diagnosis.
- The features include

a. General
o Not doing well
o Poor temperature control e.g fever, hypothermia

b. CNS
o Lethargy/irritability
o Jitteriness
o Hyporeflexia
o Tremors
o Seizures
o Coma
o Full fontanelle ( Normal is flat)
o Abnorml eye movements
o Hypotonia or hypertonia

c. Skin
o Rashes
o Erythema
o Pustlus
o Serelema – wooden, firm & hard skin, subcutaneous tissue due
to poor perfusion. It occurs in overwhelming
septicemia and marked hypothermia
- Occurs on cheeks, thighs or may be generalised
- Is a bad sign of infection

o Paronychia
o Omphalitis

d. Respiratory System
o Cyanosis
o Grunting
o Apnoea
o Irregular respiration
o Tachypnoea

e. Haematopoetic
o Jaudice – esecially conjugated
o Bleeding
o Purpura
 o Ecchymosis
o Splenomegaly

f. Gastrointestinal
o Poor feeding
o Vomiting
o Abdominal distension
o Erthema of anterior abdominal wall
o Oedema
o Hepatosplenomegaly
o Diarrhoea

g. Cardiovascular
o Pallor
o Cyanosis/mottling
o Hypotension
o Heart failure

Laboratory Diagnosis

a. Direct methods
i. Blood culture
ii. CSF culture
iii. Urine culture
iv. Additional culture – nasopharynx, ear, skin & gastric aspirate)
v. Chest x-ray
vi. Counter-Immunoelectrophoresis (CIE) – to detect bacterial antigen in
body fluis – is not done here.

b. Indirect methods
i. Full blood count
ii. Erythrocyte sedimentation rate
iii. C-reactive protein/Nitrobule-Tetrazoline test

Infection/Sepsis Screen -

1. F – Ful blood count

2. B – Blood culture
3. C – Chest x-ray
4. U – Urine culture
5. L – Lumbar puncture
Associated findings

1. Meningitis
2. Osteomyelitis
3. Arthritis
4. DIC
5. Urinary tract infection
6. Cardiac failure

Haematological Indices in Neonatal Septicaemia

1. Neutropenia
o First few days : < 5,000/mm³
o End of first week : < 1,000/mm³

2. Immature/Total Neutrophil ratio (I/T)

o > 16%
o or there is a shift to the left

3. Toxic granulations – t the neutrophils

4. Thrombocytopaenia : < 100,000/mm³

Note
Age Picture
First 4 days N>L
5th day N=L
7 - 9 days L > N (Infantile picture)
Therapy

1. General
i. Maintenance of thermoneutral environment
ii. Nutrition – Total partenteral nutrition
iii. Oxygen/Nursing etc

2. Specific
i. Antibiotics

Combination
a. In Cefuroxime 100mg/kg daily + Gentamicin

b. IV Ceftazidime/Cefotaxime + Gentamicin

c. IV Quinolones e.g Ciprotab

Duration : is determined by
i. Portal of entry of organism or systems involved
ii. Response to treatment
iii. Clinical status of the infant

a. Neonatal Septicaemia : 7 – 10 days, but you should

continue if the child is not better.

b. Meningitis : at least 21 days

c. Osteomyelitis : 6 weeks

ii. Immunotherapy e.g

o Exchange blood transfusion
- done for overwhelming septicaemia to remove
bacterial endotoxins & improve peripheral
perfusion.

o Granulocte transfusion
o Immunoglobulin
o Fresh frozen plasma

Mortality
Is about 18 – 25%
Prevention

1. Provision & utilization of antenatal care facilities

2. Delivery must be conducted under sterile conditions
3. Prevention of overcrowding in nursery
4. Handwashing technique
5. Isolation of outborn or infeted inborn
6. Individual toiletries
7. Gowning
8. Care of equipment
- Sterilese before use
- Wash incubators/cots weeklt and after occupancy

9. Cord care
10. Autoclave milk – not done here
11. Exclude personnel/parents with pyrexia of unknown origin or communicable
disease.

CUTANEOUS INFECTIONS

Types
1. Pustules
2. Expanded Scalded Skin Syndrome (ESSS) – it include
i. Ritter’s disease
ii. Bullous impetigo
iii. Toxic Epidermal Necrolysis
iv. Scalded Skin Syndrome

- Is a serious generalized exfoliative staphylococcal skin infection

- The Skin is moist and red
- It is due to release of exotoxin which causes tenderness & oedema
- There is constitutional upset.

3. Umbilical Sepsis - True Omphalitis

4. Necrotising Fascitis

Management
i. Sample for m/c/s
ii. Antibiotics : Cloxacillin or methicillin
iii. Intravenous fluid
iv. Local treatment – G.V paint aqeous
Prevention
Srupulous handwashing technique

CONJUNCTIVITIS

Aetiology
i. Neisseria gonorrhoea
ii. Chlamydia trachomatis
iii. Staphylococcus aureus
iv. Secondary to Silver nitrate

Incidence – is increased when there is PROM or Prematurity

Features
i. Silver nitrate
o starts within 6 -12 hours of birth. It clears within 24 hours

ii. N. gonorrhoea
o Starts about 5 -7 days after birth
o Initially there is watery serosanguinous discharge
o Then becomes purulent with conjunctival hyperaemia
o Complications are
i. Corneal perforation
ii. Panophthalmitis

iii. Chlamydial
o Occurs in 2 – 3 weeks
o There is pseudomemebranous formation of tasal conjuntiva

iv. Viral
o Associated with upper respiratory tract infection
o The discharge is watery
o Occurs in 2 -3 weeks

Investigation
i. Eye swab
Treatment

o Gonococcal - Penicillin (IV or eye drops)

o Staphylococcal – Cloxacillin or methicillin
o Chlamydial – Oral erythromycin + Topical
 Chapter 32
NEONATAL JAUNDICE

Introduction

o Jaundice is yellowish discolouration of the skin and mucous membrane in the

newborn under 28 days.
o Is a manifestation of hyperbilirubinaemia
o Physical evidence of jaundice is observable at the level of 5mg/dl
(17umol/L) or in range of 4 – 7mg/dl
o It usually starts from the face (sclera) and progresses to the feet.
- Sclera : 5mg/dl
- Abdomen : 15mg/dl
- Sole : 20mg/dl
o It can be seem by blanching the skin in the forehead or nose.
o Normal conjugated bilirubin is 0.1 – 0.5mg/dl. It is seen normally in urine and
high in obstructive jaundice.

Incidence
o 25 – 30% of newborn babies develop it
o In preterm, it may be as high as 80%
o It is the commonest clinical sign seen in neonates

Pathogenesis

Red blood cells

Haemoglobin

Biliverdin

Bilirubin

UDP-Glucuronyl transferase

Conjugated bilirubin in Liver

 Excreted in urine

o Water soluble bilirubin does not cross the blood brain barrier
o It is excreted into bile, then into duodenum where it changes stercobilin which is
yellowish brown. This gives the stool the typical normal colour of stool. A pale
stool does not stercobilin
o In newborn where the gut is relatively sterile, the bilirubin is hydrolysed back to
unconjugated and via the enterohepatic circulation, the deconjugated bilirubin is
reabsorbed back leading to unconjugated hyperbilirubinaemia.

o Lipid soluble or Uuconjugated indirect (with van der bergh test) bilirubin is
transported by albumin, thus hypoalbuminaemia can also cause jaundice.

o In Liver, ligandins are carrier protein required to transport bilirubin into the hepatic
cells.

o Babies with enhanced enterohepatic circulation are those

i. on exclusive breasfeeding
ii. on broad spectrum antibiotics
iii. not feeding well

o Also, note that newborns have s 2 – 3 fold greater rate of bilirubin production
because of the following reasons
i. High red cell mass – 1g of haemoglobin in a child yiel more bilirubin
than in afults.
ii. Shortened red cell life span (70 – 90 days)
iii. The concentration of ligandin & glucuronyl trasnferase are lower i.e
immature in newborn infant especially in preterm.
iv. Low gut flora – this enhances enterohepatic circulation
v. Delayed passage of meconium which also contains meconium.
o All these are referred to as Physiologic Jaundice, but one of them can be reduced
to become pathological.

Clinical Patterns of Physiologic Jaundice

1. When the peak level of indirect or unconjugated bilirubin is not > 12mg/dl
rd
on 3 day of life in term and 15mg/dl in preterm.
2. It is higher in exclusive breastfeeding infants - due to
- decreased fluid intake
- absent flor
- breast milk contains glucuronidase
 days
- In physiologic jaundice, the bilirubin rises to peak at about
5th day and begins to drop from 7th day. At 14th day it is at
the minimum.
- Whereas in Breast milk jaundice, it takes a longer time to
come down.

Characteristics of Physiologic Jaundice

i. Appears after the first 48 hours of life
ii. Peaked by 4th day
iii. Disappears by 7 – 10 days
iv. Peak level is < 170umol/L

Characteristics of Non-physiologic Jaundice

- Definition of Significant jaundice : > 12.5mg/dl

1. Jaundice manifesting within 24 hours of age. Normally, it takes about 2 days for
bilirubun to reach 5mg/dl except if there is something else causing it.

2. If peak serum bilirubin level is > 12mg/dl of unconjugated in term babies and >
15mg/dl of direct bilirubin (conjugated) or in an eclusively breastfed babies.

3. If the rate of rise is ≥ 5mg/dl/day or 0.5mg/dl/hour

th
4. If conjugated bilirubin concentration is > 2mg/dl or 1/5 of total bilirubin.

5. If associated with hepatomegaly or anaemia

6. If the jaundice persists for > 2 weeks in terms and 3 weeks in preterm. It
suggest conjugated hyperbilirubinaemia.

7. Jaundice in a sick neonate e.g septicaemia, Necrotising enterocolitis.

Causes of Non-physiologic Jaundice

1. Excessive breakdown of red cell (Haemolysis)

i. G6PD deficiency – commonest cause in this environment
ii. Polycythemia
iii. Hereditary spherocytosis
iv. ABO incompatibility – worse in group B individual. An example is if
the mother is O and the child is A or B.
v. Rhesus incompatibility
vi. Septicaemia
vii. α – thalassemia
viii. Polycythaemia
ix. Extravasated blood : Cephalhaematoma, Fracture of thigh

2. Impairment of conjugation
i. Prematurity – Note that preterm conjugating ability is even more
limited.

3. Enhanced Enterohepatic circulation

i. Broad-spectrum antibiotic therapy
ii. Total parenteral nutrition (Hyperalimentation)
iii. GI obstruction – Hirschprung disease, Upper GI obstruction
iv. Undernutririon – due to reduced intake

Note : “i” and “ii” are likely to be seen in preterm due to their susceptibility to
infections.

4. Obstruction to flow/Conjugated Hyperbilirubinaemia

a. Extrahepatic biliary obstruction
i. Biliary atresia
b. Intrahepatic biliary obstruction
ii. Septicaemia – swelling of hepatocytes which compress the bile
ductules. Really, it gives a mixed pattern.
iii. Neonatal hepatitis syndrome
- Giant cell
- TORCH

5. Mixed
iv. Inssipated bowel syndrome – It is due to viscosity of bile (stasis).
It is also seen in sickle cell anaemia. Initially, it is unconjugated,
but later changes to conjugated hyperbilirubinaemia.
 Differential Diagnosis of Unconjugated hyperbilirubinaemia in 1st week
and then Changes to Conjugated hyperbilirubinaemia

1. Inssipated bowel syndrome

2. Biliary atresia
3. Septicaemia
4. Annular pancreas – examine for masses in the abdomen
5. Galactosemia
6. Dubin-Johnson syndrome – is disorder of transport
7. Rotor syndrome – is disorder of transport
8. Familial unconjugated – Criggler Najjar syndrome. It is not common
here.
9. Rhesus isoimmunisation.

N.B
Sickle cell anaemia does not manifest in newborn. It takes about 6 months .

History
i. Maternal drugs e.g
o Camphor
o Sulphonamides
o Septrin

Management

Investigation
1. Serum bilirubin – to exclude carotinaemia
2. Full blood count – for anaemia, infection
3. G6PD status
4. Blood film : High retic count suggests haemolysis
5. Blood group of mother & child
6. Coombs test
7. Liver function test
8. Peroxidase haemolysis test –

Treatment

For Unconjugated hyperbilirubinaemia

- be aggressive. Note that preterm develop kernicterus earlier because of loer
serum protein level and immature blood brain barrier.

1. Phototherapy : used when it is > 12.5mg/dl

2. Exchange blood transfusion : when approaching > 20mg/dl
3. Fluid intake
Complications of Phototherapy
1. Increase in insensible water loss and dehydration
2. Diarrhoea
3. maculopapaular rash
4. Lethargy
5. Masking of cyanosis
6. Nasal obstruction by eye pads
7. Retinal damage
8. Bronze baby syndrome – due to effect of phototherapy on
direct/conjugated bilirubin
9. Hypocalcaemia

Care during Phototherapy

1. Give extra fluid allowance (20 – 30ml/kg/day) for insensitive water loss i
2. Protect the eye with googles
3. The wavelength of the ultraviolet light is 420nm while distance is 45cm, if further,
it is less effective.

Exchange Blood Transfusion

- Fresh whole blood that is compatible with both mother & child is used
Conjugated hyperbilirubinaemia
1. Treat the cause

Phenobarbitone

Disadvantage
- Manifestation after about 3 days
- Less effective than phtotherapy in low serum bilirubin concentration
- Has sedative effect
 Chapter 33
GROWTH & DEVELOPMENT
• Growth and Development
• Measurements of Growth
- Reference standards
- Percentile & Standard Deviation
• Growth Curves
• Neural growth
• Control of Growth
• Assessment of Maturity
• Abnormal Growth
• Development

Introduction

o Growth is a complex process which distinguishes a child from an adult. It is

defined as an increase in size and number of cells of the tissue of size of an
organ.
o Development refers to changes in function of an organ on an increase in
complexity due to maturation of the nervous system.

- Growth and development are interrelated.

- Physical growth and development thus encompasses changes in the size and
function of organisms. These changes range from those at the molecular level
in foetal life to the complex changes associated with puberty and adolescence.
- Growth and development of an individual begin at conception and continue
through tout life, their rate however change.

Measurement of Growth

- Growth is measured in dimension such as height, weight, volume and tissue

thickness and known reference standard is required for comparison.

Reference Standards
- Are obtained by taking measurements of an adequate cross-sectional sample
of healthy children of all social classes in a country/community. The means ±
2S.D of the measurement wil give the rabge of normal of the various
parameters for the particular country /community.
- A commonly used growth standard is based on percentiles. In the percentile
growth standard all the measurements of a large sample of children are
ranked in size from the smallest to the largest and are assigned percentiles that
correspond to their positions in the rank oreder. The middle measurement or
median is called the 50th percentile. Half the children in a normal distribution
can be expected to fall between the 25th and 75th percentiles which are
 equidistant from the median. The 3rd and 97th percentiles are used as limits or
normal range in clinical work corresponding to ± 2 S.D or precisely 1.9.
- Development cannot be measured, but must be assessed by other procedure
e.g description of sequence of changes.

Growth Curves
- The two types of growth curves are used in describing patterns of growth,
these are
a. Cummulative or Distance curve
b. Incremental or Velocity curve

Cummulative or Distance Curve

- Is curve obtained by plotting a series of consecutive measurements against age
(e.g heights, weights, against age)
- Again it shows the total amount of growth that occurred over time.

Incremental or Velocity Curve

- Is obtained by plotting consecutive growth increments or changes over a
series of time intervals. It shows the rate of growth or the amount of growth
per unit time.
- Growth velocity = Increase in height over time
Interval expressed as a decimal of the year (3/12 = 0.25 of
a year)

o During growing years, there are periods of rapid growth and periods of
slow growth. Increase in rate of growth is referred to as accelerating and
decrease as decelerating.
o Growth may be accelerating or decelerating irrespective of the growth rate
e.g during the first year of life, the growth rate is very rapid, but growth is
decelerating, during 13th year, the growth rate is again rapid and growth is
accelerating e.g a child returns to birth weight by 10 days and doubles
birth weight by 5 months and triples by 1 year and quadriples by 2.5 years.
o During the first year of life, the average increase in head circumference is
also about 4 inches.
o Measurements regularly used are
i. Height (length)
ii. Weight
iii. Head circumference
iv. Mid upper arm circumference
v. Chest circumference
vi. Skin fold thickness – is mainly for research

o Length is difficult to measure accurately in a baby.

o Important ratio
i. Upper body /Lower body
 ii. Sitting height /Standing height
iii. Total height / Arm length

o A child shape changes as he gets older. There is a relatively preponderance

of rowth a the cephalic part of the body from foetal life to infancy and
early childhood. This is followed by growth of the trunk and extremities.
It is referred to as Cephalocaudal progression. It corresponds to the
elaboration of function necessary at that stage i.e the development of the
cerebral cortex. Functions of musculature controlled movements of upper
limbs become possible while movements of lower are voluntary. Thus at
conception age of 0.42 i.e about 5th months of pregnancy, the head is
about a quarter of the total length of the child. At birth 0.75 – 9/12 the
head is about 1/5th of the total length.
o The main components of the body do not grow at the same rate. The brain
and head approach the their adult size very early inlife and by the age of
5 or 6 years would have reached about 90% of their unltimate size, while
the body as a whole has reached only 50%. The reproductive system
shows little growth until puberty. The lymphoid tissue on the other hand
shows remarkable pre-pubertal growth reaching nearly twice their
ultimate size about the time the adolescent growth spurt begins. Body fat
increases rapidly during the 1st year, this is followed by a slow steady loss
until the age of 7 years. ( Note sugar babies – high calories , low protein).
There is then another slow increase till puberty when fat then continues
on the trunk in both sexes and also on the limbs in girls.
o Neural growth
Neural growth (representing particularly) brain growth spurt begins in
mid-pregnancy and is largely complete by the 3rd year. There are actually 2
growth spurts : the first is a neuronal cell multiplication which occur between 15
– 20 weeks gestation. The 2nd is predominantly a glial cell multiplication starting
at 25 weeks and extending to the 2 nd year with myelination continuing into 10th
and 12th year.

Control of growth
Growth is controlled by integrated action of genes, endocrine and
metabolism of the body and is greatly influenced by nutritional and
environmental factors. Food enzymes, hormones e.g insulin, glucagons, lipase
trypsin etc. However genetic factors have overriding influence.

1. Growth hormone (Pituitary) – stimulates chondrogenesis in the presence of

somatomedins.
2. Thyroid hormone – skeletal maturation (affects osteogensis)
3. Parathyroid hormones – Calcitonin is essential for bone growth which leads
to pubertal growth in males
4. Adrenal & Testicular hormones – in males, ovarian oestrogen in females
Adrenal androgens – gonadal oestrogens and growth hormone may lead to
pubertal growth in females.
Note
Linear growth occurs primarily as a result of chondrogensis or the formation
of new cells and collagen in the growth cartilage of long bones.
 Chapter 35
GENERAL PRINCIPLE OF ONCOLOGY

• Introduction
• Aetiology : Genetic & Environmental
• Investigation
• Staging
• Treatment
• Tumour Lysis Syndrome

Introduction
Oncology is study of new growth.

Aetiology
Most cases are unknown. The factors associated are grouped into genetic and
environmental

Genetic
i. Mutations in Tumour suppressor gene (anti‐onocogens) e.g in retinoblastoma
ii. Activation of proto‐oncogens e.g by chromosomal tanslocation thereby
deregulating the gene expression
iii. Cancer predisposition syndromes e.g Xeroderma pigmentosum – there is
defect in repair of DNA caused by ultraviolet light, thus leading to cancer.

Environmental
i. Ionizing radiation (atomic bomb) e.g Leukaemia
ii. Ultraviolet radiation e.g Xeroderma pigmentosum
iii. Drugs e.g
• Intrauetrine diethylstilbesterol causes adenocarcinoma
• Treatment of aplastic anaemia with anabolic steroids predisposes to
hepatocellular carcinoma
• Immunosuppresive agents : Non‐Hodgkin’s Lymphoma

iv. Viruses e.g Ebstein Barr virus predisposes to

• Burkitt’s lymphoma
• Hodgkin’s disease
• Nasopharyngeal carcinoma

Investigations
 1. Full blood count – is mandatory
- It helps in diagnosis
- To determine the minimum counts before chemotherapy

Minimum Counts For Treatment

PCV 30%, the lowest min. is

25%
WBC (Absolute 1,000mm³
neutrophil count)
Platelet count 100,000mm³
e.g of how to calculate absolute neutrophil count is below
if total is 4,000, and using 50%, the absolute count is 2,000.

2. Electrolyte & Urea

3. Creatinine
4. Uric acid
5. Calcium & phosphate

N.B 2 – 6 are done to

• know the baseline for treatment
• to monitor treatment from metabolic complications

6. CSF cytology
7. X‐rays
8. Bone marrow aspiration
9. Liver function tests
10. Abdominal ultrasound – for abdominal tumour e.g Burkitt’s Lymphoma
11. Intravenous urogram
12. CT scan
13. MRI

For Confirmation of Diagnosis

1. FNAB for Cytology or FNAC
2. Incisional biopsy
3. Excisional biopsy

‐ The type of biopsy used depend son the site of tumour. FNAC is used for superficial
tumour.

Requirements For Biopsy

i. Full blood count
ii. Platelet count
 iii. PT/PTTK

Staging : Is used
a. For prgognosis
b. As guide to treatment ‐ chemotherapy or surgery

Treatment
‐ Is multidisciplinary
‐ Is divided into Specific & Supportive

a. SPECIFIC TREATMENT

‐ Is multimidal
‐ Determinats of modalities are

i. Histology – as certain tumours respond to chemotherapy e.g anaplastic

ii. Size / Stage
- Large size ‐ Surgery
- Early stage – Surgery
- Metastasis of multiple tumour – Systemic chemotherapy to mop up
- Advanced stage – Radiotherapy, Chemotherapy

iii. Experience
- e.g Burkitt’s Lymphoma in ovary respnds well to chemotherapy and so
there is no need for surgery.

iv. Location of the tumour e.g Radiotherapy rather than surgery would be used
for Brainstem glioma as it is very close to the vital centres (Respiratory &
RAS)

Treatment Options

1. Surgery
i. Complete excision e.g in early nphroblastoma
ii. Debulking surgery (i.e subtotal excision) e.g in rhabdomyosarcoma
iii. May follow neo‐adjuvant chemotherapy i.e after cytotoxics have given to reduce the
bulk.
( Adjuvant chemotherapy – is after surgery)

2. Radiotherapy
- May be the main modality e.g in brainstem glioma
- May precede or follow surgery
 Side effecs
Early
i. Alopecia
ii. Skin erythema & desquamation
iii. Vomiting
iv. Diarrhoea
v. Bone marrow suppression

Late
i. Impaired growth & dvelopmen of epiphyseal growth centres
ii. Secondary tumour e.g radiation of retinoblastoma causing
secondary tumour of bone

3. Chemotherapy
Principles
- May be given as adjuvant chemotherapy
- Are also given as combination chemotherapy – to reduce the likelihood
tumour resistance. The drugs act on different stagesof life cell cycle,
thus giving additional effects.

M – Mitosis
G1 – Presynthetic phase
G2 – Post –synthetic / Premitotic
S – Synthetic phase

- Cytotoxic drugs are grouped into

a. Phase‐specfic
b. Phase non‐specific

- A drug may push the tumour cells into another state in which another
drug can act on it.
- The aim of chemotherapy is to destroy cancer cells without endangering
the host.
- Most drugs attack DNA and rapidly dividing tissues. Rapidly dividing
tumours like ALL respond to agents that affect DNA synthesis e.g
methotrexate, cytarabine. Wheras slowly proliferating tumours like
 myeloma respond to alkylating agents that damage the helical structure
of DNA irrespective of the phase of the cycle.

Groups of Cancer Chemotherapeutic Agents

1. Alkylating agents
- Are cell cyle non‐specific e.g cyclophosphamide

Mechanism of Action : Inhibits DNA replication, RNA transcription and

causes chromosomal breakages.

Side effects
i. Haemorrhagic cystitis & fibrosis – prevented by giving excess fluid
intake and frequent voiding.
ii. Myelosuppresion
iii. Pulmonary fibrosis
iv. Infertility
v. Darkening/grayish formation of the nail bed.

2. Antibiotics
- e.g Dactinomycin, Daunorubicin, Doxorubicin
- Are cell cycle non‐specific

Mechanism of Action : Intercalate DNA helix and prevents DNA replication

and RNA transcription.

Side effects
i. Local vesicant – ensure IV line is n vein
ii. Cardiomyopathies : Daunorubicin & Doxorubicin are cardiotoxic
iii. Arrhythmias
ECG should be done before giving these drugs.

3. Antimetabolites
e.g Methotrexate, 5‐Fluouracil, Cytosine, Arabinoside
‐ Are specific for S‐phase

Mechanism of Action : Inhibit enymatic steps for synthesis of nucleic acids.

Methotrexate inhibits dihydrofolate reductase.

Side effects
i. myelosuppresion
ii. Stomatitis – causing diarrhea
iii. Mucositis
- Generally, it affects all fast growing cells in the body.
4. Vinca alkaloids e.g Vincristine, viblastine
- Are specific at M‐phase

Mechanism of Action
- Inhibitis microtubule formation, causing mitotic arrest at metaphase.

Side effects
i. Local vesicant
ii. Neuropathies e.g foot drop

5. Hormones e.g prednisolone

- Casues ? lymphocyte modification

6. Enzymes e.g L‐asparaginase

- Are specific for G1 phase

Mechanism of Action
- Converts asparagine to aspartate & ammonia. This leads to inhibition of
protein synthesis.

Side effects
i. Hypersensitivity : urticaria, anaphylaxis.

7. Miscellaneous : Nitrosourea, Cisplatin

4. Immunotherapy
- Involves boosting immune system to fight tumours
- It can be active or passive

a. Active
‐ Antigen is given to stimulate immune system e.g
• BCG ‐‐ Burkitt’s Lymphoma, the response is very poor, so it has been
dropped
• BCG ‐ Acute Leukaemia, the response is still better.
• Interferons and Interleukin‐2 ‐ Burkitt’s Lymphoma
• Hepatitis B vaccine – to prevent the occurrence of primary liver cell
carcinoma.

b. Passive
• Is divided into
i. Cell‐mediated type (active) – using culture lymphocytes e.g
Lymphocytes Activated Killer cells
ii. Humoral ( Passive)
 ‐ Using monoclonal antibodies.

TUMOUR LYSIS SYNDROME

Definition
• A syndrome of metabolic abnormality in patient with tumours that results from
breakdown of tumour cells.
• It may occur spontaneously or due to treatment‐related tumour necrosis.
• It usually occurs in large burden tumour like Leukaemias, Burkitt’s Lymphoma.

Components

1. Hyperkalaemia – from necrotic cells. It can causes arrhythmias and eventually cardiac
arrest.

2. Hyperphosphataemia : occurs because lymphoblast are very rich in phosphate

3. Hypocalcaemia : results from the hyperphosphataemia as calcium and phosphate are

must in equilibrium.

4. Hyperuricaemia : may lead to Uric acid nephropathy and eventually renal failure.

5. Metabolic acidosis (↓ HCO3)

Treatment
Is prevention which is done 24 ‐48 hours before commencement of treatment.

1. Hydration : Give plenty of fluids ( 1½ of maintenance volume) before

chemotherapy. This is to promote excretion of uric acid and prevent uric acid
nephropathy.
2. Alkaline diuresis : Adding Sodium bicarbonate to IV fluid being given. It is given
for those who already have hyperuricaemia as it facilitates the ecxcretion of uric
acid in kidney.
3. Maintain urine specific gravity at < 1.010
4. Giving allopurinol – a xanthine oxidase inhibitor.
 5. Diuretics.

1 and 4 should not be forgotten at all.

b. SUPPORTIVE TREATMENT

1. Blood products
i. Packed cells – for anaemia
ii. Granulocytes concentrate – for leucopaenia
iii. Platelet concentrate – for thrombocytopaenia
iv. Colony stimulating factor e.g G‐CSF

2. Treatment of infections
- Note, because of immune suppression, they may show full signs of
infection.
- It is usually caused by staphylococcus aureus, Gram –ve organism. Take
culture and place on antibiotics

3. Nutrition
• Oral
• Nasogastric tube
• Parenteral feeds
• Hyperalimentation

4. Antiemetic e.g Phenothiazines , Chlorpomazine

- Nausea & vomiting are usually not severe in paediatric age group

5. Analgesic – the type used depends on the severity of pain

i. Mild – Paracetamol
ii. Moderate ‐ DF 118 (Dihydrocodeine)
iii. Severe – Morphine

6. Psychological support
 Chapter 36
SPECIFIC TUMOURS IN CHILDHOOD

Specific Tumours
i. Lymphomas : BL., HL, NHL
ii. Wilm’s tumour
iii. Neuroblastoma
iv. Acute Leukaemias
v. CNS Malignancies
vi. Retinoblastoma
vii. Rhabdomyosarcoma

Incidence in Ibadan (1990)

Specific Tumours %
1. Lymphomas 45 - (37.1% Burkitt’s
Lymphoma)
2. Retinoblastoma 10
3. Nephroblastoma 8.5
4. Intracranial tumours 7.5
5. Sarcomas 7.5
6. Leukaemias 7.4
7. Carcinomas 3.1
8. Neuroblastoma 2.9
9. Germ cell Gonadal Neoplasia 1.4
10. Bone noeplasia 1.3
11. Hepatic neoplasia 1.2

Current Review
Burkitt’s lymphoma is the commonest childhood tumour in Tropical Africa, in
Europe, it is Leukaemia.
1.

LYMPHOMAS

- Are proliferative malignant disorders of lymhoctes and/or macrophage system.

- They are heterogenous.
- They include
i. Burkitt’s Lymphoma
ii. Non-hodgkin Lymphoma
iii. Hodgkin Lymphoma

BURKITT ‘S LYMPHOMA

Definition
- Is a malignant lymphoma of B-lymphocytes origin.
- Is the fastest growing tumour in man with a 24-hour doubling time, hence the
interval of onset of symptoms and presentation is usually short.

Types
a. Endemic – in Tropical Africa
b. Sporadic – seen in Far East, Central & South America & Europe.

Incidence - Endemic BL
- Is the commonest childhood malignant tumor in Ibadan. It accounts for 37.1% of
malignant tumour in Ibadan in children.

Aetiology – not known, but there are 3 aetiological factors

1. Ebstein Barr virus (EBV)

2. Malaria infection (chronic)
3. Chromosomal translocation
a. 8 : 14 - constitute about 80%
b. 8 : 22 - 15%
c. 2 : 8
Note, chromosome 8 is common to all and it possesses the c-myc gene.
It is critical in the regulation of cell proliferation.
Pathogenesis

a. Mechanism of EBV : Its viral genome is found in 95% of cases in the endemic area.
Chronic EBV infection is thought to cause proliferation of lymphocytes.
b. Chronic malarial infection – impairs immune surveillance ( P. falcparum)
c. Sporadic cases : Here, EBV occurs in very smaller proportion.

Overall, the full reason is not known.

Pathology
- is a multifocal tumour e.g arising form jaw, abdomen etc, hence no metastasis.

a. On Fine Needle Aspiration Cytology

- The cells have basophilic & moderate cytoplasm containing fat vacuoles., the
nucleus has about 2 – 5 nucleoli. These are called Burkitt’s cells

b. Excisional b iopsy
- Done if FNAC fails
- It shows typical starry-sky appearance
• Stars – histiocytes which are interspersed among the lymphoid cells
• Blue sky – is basophilic lymphoid cells

Clnical Presentation
- Age incidence : 2 – 16 years of age with peak age of 7 years. Note, it very rare <
2years and > 20 years.
- Male/Female ratio – 1.7 : 1
- Incidence is 1 in 10,000

Sites

1. Jaw - is commoner in the maxilla (than mandible) or orbit resulting into

proptosis. Here, it causes l
• Loosening of teeth
• Dental anarchy or malalighment
• Jaw swelling

2. Abdomen - Is the commonest site in Ibadan. It occurs in Bowels, Kidneys,

Mesenteries & Ovaries.

3. Central Nervous System : It causes

• Cranial nerve palsies
• Paraplegia – usually flaccid
 • CSF Pleocytosis ( of Burkitt’s cells) – therefore, always do CSF tap in
these patients

4. Others
i. Thyroid iv. Skin
ii. Breast v. Parotid
iii. Testes

5. It is very rare in bone marrow, lymph node, lung, spleen and mediastinum.

Staging
Stage
A Solitary extra-abdominal site like jaw
B Multiple extra-abdominal site
C Intra-abdominal tumour ± Facial tumour

AR Abdominal tumour which has been completely (90%)

resected

Investigation

1. Tumour aspirate for cytology. Percutaneous aspiration under ultrasound

guidance.
2. X-ray of jaw – It shows
• loss of dental lamina
• dural thin rim of cortical bone outlining teeth
• Osteolytic lesion
• Dental anarchy
These features are seen even in the absence of obvious jaw mass.
3. Full blood count – for all cases and baseline for chemotherapy.
4. Electrolyte, Urea & Creatinine – to monitor metabolic complications e.g
Tumour Lysis Syndrome.
5. Calcium & Phosphorus
6. Abdominal ultrasound
7. IVU – for kidney masses
8. Chest x-ray
9. Spinal myelogram – for those with lumbar myelogram
10. Spinal CSF for cytology – is mandatory for all cases.
11. Serum LDH – is a useful marker for tumour burden. It is used to monitor
treatment : tumour regression & relapse
Treatment

1. Chemotherapy
- Is the mainstay of treatment

Drugs : “COMP or COAP”

• Oral Prednisolone
• IV Cyclophosphamide
• IV Vincristine
• IV Cytosine arabinoside or methotrexate
• Intrathecal cytosine arabinoside or methotrexate

Course
• 6 cycles are given and each cycle of treatment is 2 weeks. It is given for
the first 5 days for each cycle except if there is bone marrow
complication.

2. Radiotherapy - Is not very successful

3. Surgery – Not essential because of chemotherapy
4. Use of Intravenous BCG as immunomodulator. It is not successful. ( But it is now
used for renal cell carcinoma & melanoma)

Prognosis
Depends on the stage

Better Worse
1. A, AR & B 1. C, D
2. CNS Involvement
3. Bone marrow involvement
4. Peripheral blood manifestation
5. Male gender
Age 6. Age > 13 years

• It is worse in male because testes are sanctuary sites.

• Most patient regress with chemotherapy, but relapse is common.
• Some reports show 40 – 80% survival rate but not in Ibadan.
Extra information

Differential Diagnosis
a. Jaw tumour
1. Adamantinoma
2. Dentigenous cysts
3. Dental abscess
4. Embronal rhabdomyosarcoma
5. Ossifying fibroma

b. Orbital involvement
6. Retinoblastoma
7. AML with Chloroma
8. ALL

c. Abdominal swelling
9. Nephroblastoma
10. Neuroblastoma
11. Abdominal tuberculosis

Medical treatment
i. Blood complications
ii. Management of metabolic disturbance
iii. 10% die due to Tumour Lysis Syndrome
iv. Antiemetic
v. Nutritional support
vi. Psychological support

Pre-chemotherapy Management
Allopurinol, 100mg/m²
 HODGKIN’S DISEASE

Introduction
Presents with progressive painless enlargement of lymph nodes

Types
1. Childhood form : < 15 years
2. Youg adult form : 15 – 34 years
3. Older adult form : 55 – 74years

Incidence
- There are 2 peak ages : 15 – 35years and > 50 years of age.
- It is unusual < 5 years

Pathology
- Is associated with Ebstein barr virus (EBV)
- The characteristic cell is Reed-Sternberg cells which has Owl-eye appearance.
- Other cells are
o Lymphocytes
o Histiocytes
o Plasma cells

- There are 4histologic types

i. Lymphocte-predominant : has the best prognosis
ii. Nodular sclerosis
iii. Mixed celullarity
iv. Lymphocte-depleted – has the worst prognosis

Clinical Presentation

i. It may be nodal or extranodal

ii. Lymphadenopathy – most common site is cervical manifestinag as airway
obstruction
iii. May also show hepatic symptoms
iv. Constitutional/Systemic symptoms are
o Fever
o Weight loss
o Drenching night sweats
Thus Tuberculosis is a strong differential diagnosis of it.
Diagnosis

1. Lympho node biopsy

2. Others – to assist in staging & treatment
i. Full blood count
ii. Liver function test
iii. Chest x-ray
iv. Abdominal CT
v. CT chest
vi. Bone marrow aspiration

Note, if you see lymph node enlargement, examine other parts of the reticuloendothelial
system i.e Axilla, Liver & Spleen

Treatment

a. Early Stages : Radiotherapy and/or Chemotherapy

Chemotherapy Regimen : “MOPP”

i. Nitrogen mustard
ii. Oncovin
iii. Procarbazine
iv. Prednisolone

Note, though Hodgkins lmpymphoma responds well to radiotherapy, it is avoided

for children becauses of affectation of growth plates.
 NON - HODGKIN LYMPHOMA

Incidence
Age : 5 – 15 years
Sex : Male > Female

Clinical Features

i. Abdomen : enlargement of para-aortic lymph node or extranodal e.g

manisfestinf as intussuception in children > 6 years.
ii. Mediastinal involvement
iii. Head & Neck glands
iv. Peripheral nodes
v. Others : Bones, Breast, Orbits, Gonads, Skin

Treatment

1. Chemotherapy – is the mainstay

Regimen : CHOP
i. Cyclophosphamide
ii. Adriamycin/Doxorubicin
iii. Oncovin
iv. Prednisone

2. Radiotherapy
3. Surgery –depending on the site
 NEPHROBLASTOMA
(Wilm’s Tumour)

Introduction
Is malignant tumour of kidney. It involves the embyonal cell in kidney.

Incidence
Age : 1 – 5years, with peak age 3 - 4 years

Sex : Equal

Aetiology
It may be associated with
1. Congenital abnormality of genitourinary tract like
o Hypospadias
o Cryptoorchidism
o Other genitourinary abnormalities e.g pelvic kidneys, horse-shoe
kidney

2 Aniridia – absence of iris

3. Beckwith-Wiedmann syndrome – It consists of

o Hemihyperrophy
o Macroglossia
o Visceromegaly
o Many embryonal tumours

- It is associated with lesion of chromosome 11.

4. WAGR syndrome – which consists of

o W – Wilms tumour
o A – Aniridia
o G – Genitourinary abnormality
o R – Mental retardation

It is sometimes inherited as Autosomal dominant.

Pathology

- On gross appearance, it is lobulated, gray to pink tumour that is initially

encapsulated, but later due to contiguous spread loses the capsule.
- It metastasis to Lymph node, Lungs, Liver, Bone, Brain.

Clinical Features

i. Abdominal (flank) mass – that usually does NOT cross the midline.
ii. It is painless, becomes painful if there is haemorrhage into tumour.
iii. Haematuria
iv. Hypertension – resulting from vascular obstruction
v. Polycythemia – due to production of erythropoietin as paraneoplastic syndrome
vi. Constitutional symptoms
o Fever
o Anorexia
o Vomiting

Investigation

1. Urinalysis
2. Intravenous urogram – shows distorted calyces or failure to excrete the dye (non-
functional kidney)
3. Abdominal ultrasound

Differential Diagnosis

1. Neuroblastoma
2. Hepatoblastoma
3. Rhabdomyosarcoma
4. Benign Cystic tumour of kidney

Staging

Stage
I Limited to one kidney
II Extend beyond the capsule, but can be completely
resected
III Residual (after resection), no hematogenous
extension
IV Hematogenous metastasis
V Bilateral tumour
Treatment

Early Stages : Nephrectomy ± Radiotherapy

Advanced cases : Chemotherapy – VAC

a. V - Vincristine
b. A – Actionmycin D /Adriamycin
c. C - Cyclophosphamide

Prognosis
- Depends on age, histology and stage
a. Age : it is better in < 2 years

b. Histology
i. Favourable histologic type
ii. Unfavourable - has analasia. It has poor prognosis.

c. Stage : Prognosis worsens with advancing stage.

5 –year Survival Rate

Stage 5 –year Survival Rate
I 97%
II 94%
 NEUROBLASTOMA

Introduction
o Is tumour originating from neural crest cells i.e either from adrenal medulla or
sympathetic ganglia.

Incidence
o Age : the children are usually younger than Wilm’s tumour, it is less in <
4yaesr of age
o Sex : Male > Female

Pathology
- Small round cells forming clusters with surrounding fibrillar material
- Is an invasive tumour
- It metastasizes to bone, liver, bone marrow, subcutaneous tissue.
- Most cases ( 60%) arises from abdomen as firm to hard mass which is painless,
irregular, craggy surface.
- It usually extends beyond midline.
- It may also present as a mass along sympathetic chain on medi

Clinical Presentation
i. Patients are usually more ill
ii. Paresis or paralysis of limbs
iii. Bone or joint pains
iv. Orbital features
o Ecchymosis
o Proptosis
o Horner’s syndrome
v. Constitutional symptoms
vi. Hypertension – from secretion of catecholamines from adrenal medulla
vii. Diarrhoea – often intractable. It is paraneoplastic. It is due to release of
vasoactive intestinal polypepetide.

Investigation

1. Biopsy – to confirm the diagnosis

2. Full blood count
3. Bone marrow aspiration – to stage
4. Urine sample : to check for Vanil mandelic acid (VMA) & Homovanillic acid (HVA).
It is increased in > 90% of patients.
5. Serum CEA
 6. Abdominal X-ray
7. Intravenous urogram – shows displaced kidney downwards and laterally, but
calyces are normal.

Treatment

1. Chemotherapy : VCAC
i. Vincristine
ii. Cyclophosphamide
iii. Adriamycin
iv. CispLatin/Cisplatinum

2. Surgery

Prognosis

1. Age : it is better in < 1 year

2. Site : Worse in abdominal tumour
3. Stage : Worsens with advancing stage
4. Pathology : the less the differentiation, the worse the prognosis

- There may be spontaneous regression of differentiation into benign ganglioneuroma.

 ACUTE LEUKAEMIAS

Introduction
- Is primary disorder of bone marrow
- It is rapid uncontrolled proliferation of lymphoctes.

Incidence
- It is the commonest childhood malignancy in Caucassians.
- It accounts for 7.1% in Ibadan.
- Age : any age, but peak is 2 – 5 years.
- It occurs more often in Down’s syndrome

Classification
a. ALL – commoner in children
b. AML

Clinical Features

General
i. Fever
ii. Lassitude
iii. Pallor

Bone Marrow
i. Anaemia
ii. Infections
iii. Bleeding – especially skin & buccal mucosa

Extrameduallry invasion
i. Lymphadenopathy
ii. Hepatosplenomegaly

Others
i. CNS
ii. Testes
iii. Bones & Jonits
iv. Skin
Investigation

1. Full blood count – shows anaemia

2. WBC – It is usually high, but may be low or normal.

3. Platelet count – is usually low due to associated DIC

4. Perpheral blood film – blast cells are seen

5. Bone marrow aspiration – shows leukaemia cells

Diagnosis
- With bone marrow aspiration
- Presence of blasts in peripheral film is NOT diagnostic.

Differential diagnosis

1. Haematological disease
i. Infectious leukaemoid reaction
ii. Idiopathic thrombocytopaenic purpura
iii. Aplastic anaemia

2. Non-heamatogenous – also presenting with bone pains

i. Rheumatic fever
ii. Rheumatoid arthritis

Treatment
1. Chemotherapy : COAP

i. Cyclophosphamide
ii. Oncovin
iii. Adriamycin
iv. Prednisolone
- Note, there are other regimen

2. Radiotherapy – can also be used

3. Bone marrow transplantation
Relapse
Is relatively quite common
Bone marrow is commonest site
CNS
Testes

Prognosis

1. Age : Is better for those between 2 – 10 years, i.e worse for those < 2years and > 10
years

2. Race : It is worse in blacks

3. Sex : Worse in male

4. WBC : Worse if the initial value is > 50,000/mm³

5. Types : Prognosis is worse with AML. In good centres, 80% cure can be achieved in
ALL. ( It is better in Non-B, non-T cell and worse with B cell, T cell & Pre-B)
 CNS MALIGNANCIES

Types
a. Primary - are commoner
b. Secondary – are rare

Pathology
- Majority arise from glial cells e.g Astrocytoma, Ependymomas

Inicidence
i. Generally or worldwide, > 50% arise in posterior fossa o.e infratentorial. In
Ibadan, most of the CNS tumours are hemispheric i.e supratentorial (Akang,
Nigeria).
ii. Peak age : is common in second half of first decade

Clinical Features
Depends on histological characteristic & rate of growth of tumour.

i. Focal neurological defcitis

ii. Features of raised intracranial pressure
• Early morning headache, relived by standing
iii. Rapid head enlargement
iv. 6th cranial nerve palsy – note, 6th cranial nerve doe not have any localosong
value.
v. Papilloedema
vi. Vomiting – usually projectile & on waking from sleep i.e early morning.
vii. Diplopia
viii. Head tiliting – to one side.

Other signs depnd on the side the brain affected.

Investigation

1. Skull X-ray – showing cupper beating appearance or silver beating

2. Plain radiography – raised ICP
3. Angiography
4. Myelography
5. Cranial CT
6. MRI
7. Nuclear MRI
8. CSF Cytology
 9. EEG – for localization of tumour
10. Endocrine test – depending on tumour
• ACTH, TSH, GH, LH & FSH

Treatment

- Depends on localization

Modalities are
i. Surgery – for craniopharyngioma
ii. Radiotherapy – Brainstem glioma
iii. Combination chemotharpy
 RETINOBLASTOMA

Introduction
- Is a malignant tumour arising from retina.
- 2/3rd of cases are unilateral, 10 – 20% of these unilateral inherited in autosomal
dominant fashion.
- 1/3rd of cases are bilateral and almost all are inherited in autosomal in autosomal
fashion.
- It arises from mutation or deletion of chromosome 13

Incidence : 1 – 3 years

Clinical Features

i. Cat-eye reflex or leucocoria – is an early feature

ii. Mass protruding the eye
iii. Strabismus
iv. Proptosis – in late cases

Pathology
It metastasizes to
Brain Face
Orbits Lung
Liver Lymph nodes
Bones Bone marrow

Treatment
Depends on the stage

1. Earlier stage
i. Laser therapy – photocoagulation
ii. Cryotherapy – the use of cold to freeze

2. Advanced stage
i. Chemotherapy
ii. Radiotherapy – external beam is done here. It causes secondary orbit
tumour.
 Note :
In Ibadan, surgery (Enucleation) is done for most cases ( with
radiotherapy).

RHABDOMYOSARCOMA
Introduction
- Arises from the same embryonic mesenchyme as striated muscle
- Is the commonest soft tissue sarcomatous

Incidence
Age : common in all ages, but has 3 peak ages
a. 2 – 6 years
b. 15 – 19 years

Sex : Male > Female

Clinical Presentation
The sites are
• Head & neck region - 40% ( commonest site). It mat present as jaw tumour,
therefore it is a strong differential diagnosis of Burkitt’s Lymphoma.
• Extremities – 20%
• Urinary tract – 20%
• Others ; Trunk, Retroperitoneal, GIT

SARCOMA BOTYROIDES
- Is a form of rhabdomyosarcoma
- It occurs in girls.
- It presents as a grape-like tumour projecting into body cavity e.g vagina, uterus,
bladder, nasopharynx.

Treatment of Rhabdomysarcoma
- Depends on site and extent
Modalities are
i. Surgery – Subtotal excision or debulking surgery.
ii. Radiotherapy
iii. Chemotherapy
Vincristine
Adriamycin
Cyclophosphamide.
 Chapter 44
MALARIA

Introduction
- Malaria derived its name from “bad air”
- It is a thoroughly pervasive disease i.e many conditions mimic it.

Clinical Definition of Malaria

o Is fever without any localizing/lateralizing sign e.g ear, chest, diarrhea,
viral infections, bacteraemia, protozoal & neoplastic disease.
o It affects all systems
o It is the commonest cause of convulsion (Febril convulsion)

Aetiology
It is caused by 4 main parasites
a. Plasmodium falciparum
– is the commonest worldwide. Responsible for 95% of malaria.
– It causes malignant tertian malaria. It is called “malignant” because
it often kills and “tertian” because its half life is 48 hours.

b. P. vivax
– is rare in Western regions because of Duffy blood antigen as most
people (about 75%) are Duffy –ve.
– It causes benign tertian. It is called “benign” because it does not kill.

c. P. malariae
– Responsible for 4%
– Has half life of 72 hours, hence called Quartan malaria.

d. P. ovale
- accounts for 1%
- It is also called tertian
Life Cycle of Malaria
- It was put together in 1896 by Ronold who worked on Chicken parasites.

Mosquito saliva

Sporozoites

Liver

Merozoites - some re-infect live

Red blood cells

Schizonts

Gametocytes – move from 37 C to ambient temperature

Mosquito

Male & Female gametes

o After mosquito bite a man, it injects the sporozoites into the blood stream
o Within 30 minutes, the sporozoites enter into the liver
o And causes hepatocytes rupture to release the merozoites. This is pre-
erythrocytic stage.
o Ex-flagellation : grows from single nucleus to 8-gamete within 20 minutes.
This is caused by the drop in temperature
o Malarial parasites are haploid and is only diploid after fertilization.
o Fever is due to erythrocytic stage as the liver stage is asymptomatic.
o In falciparum malaria, some merozoites enter liver to become hypnozoites
which can reactivate.
o Note, P. vivax has extrerythrocytic stage.

Varying Degree of Susceptibility in Pouplations

1. With Age
Birth – 6 months
o Congenital malaria is relatively uncommon here because
i. Maternal antibody protection on the child
ii. Cellular mechanism – Fetal haemoglobin (HbF) does not support
the growth of parasites because of its high oxygen affinity,
remembering “ the switch” from embryonal to Fetal Hb, then to
Adult haemoglobin.
iii. Entomological reason i.e mosquitoes are more likelt to bite larger
creatures i.e the mother than the child. The is because of the higher
chemoreceptors & thermorecpetors as mosqiotoes fly along CO2
gradient.

Note , the first 2 reasons are more convincing.

At 6 months – 2 years
o By the end of 6 months, the child becomes maximally susceptible to
malaria, and this continue to 2 years till the child begins to produce its
own cellular and humoral mechanisms.

Above 5 years
o The chills is relatively immune. This immunity wanes again in
pregnancy, especially in primigravida.

2. AS haemoglobin ( Sickle cell trait)

- Note, it is not protective against acute uncomplicated malaria, but the AS
patients don’t die from it.

- The reason is because parasitized AS red cells sickle faster than non-
parasitised red cells, thereby get easily removed by reticuloendothelial
system and consequently resulting into decrease in the level of
parasitaemia. The sickling is irreversible.

- Note in SS patient, it is better, however both the parasitized and non-

parasitised red cells sickle massively. This is due to the lactic acidosis
caused by the malaria parasites. Note, high acidosis is a feature of severe
malaria. These patient become very ill, though not from malaria, but from
sickling, thus prophylactic anti-malaria are used by these group of
patients.

3. G6PD deficiency
- In the past, only the heterozygous was protected against life severe
threatening malaria, but recently, it’s been said that both homozygous
female & hemizygous male are protected.
 Mechanism
- The malarial parasites cause increase in oxidant damage in the g6PD
deficiency red cells which are thus removed by the reticuloendothelial
cells.
- Summary

Old Recent
1. Heterozygous 1. Heterozygous -
2. Hemizygous male
3. Homozygous female

4. Elliptocytosis – is also protective against falciparum malaria.

5. HbC – some evidence

ACUTE UNCOMPLICATED MALARIA

Introduction
Is the commonest form

Clinical Presentation

1. Fever – commonest, initiallt it may be without localizing signs e.g

splenomegaly.

2. Febrile convulsions – seen in 6 months – 5 years. Malaria is responsible for

about 50%

3. Combined Fever + Anaemia + Metabolic acidosis

Note :
o Metabolic acidosis manifests as deep rapid breathing which doesn’t have
Kussmaul breathing that is described as “air hunger” seen commonly in
Emergency unit. It is different from rapid & shallow breathing which is due
to reduced lung compliance in pulmonary oedema & pneumonia.

Laboratory Diagnosis

1. Microscopy – is the gold standard

a. Thick film – to count malarial parasitwa
b. Thin film – is for species identification.
 Stain
i. Giemsa stain is for both thick and thin films
ii. Leishman is for thin film.
It has limit of detection at 1/100,000 i.e one parasite seen in 100,000 red blood cells.

Thin film
i. Central disc pallor
ii. Bacterial infection : wbc > rbc ( Normal is 1 – 2wbc/ rbc using X40,
X 1,000 is used to see parasites
iii. Sickle cell
iv. Immature red cells – are nucleated
v. Rouleaux formation – due to increased globulin & acute haemolysis
vi. Moncytes-containing pigment – is seen at times.
vii. In P . falciparum, ring forms are seen in red blood cells in the
superficial vessels. It also has multiple infected red cells.
viii. Characteristically-shaped gametocytes (falciform /banana-shaped)
ix. Toxic neutrophil in karyorrhexis

Note – Normal sizes of White blood cells

a. Monocytes : 3 X rbc
b. Lymphocytes : almost equal
c. Polymorphs : 2X rbc

2. Polymerase chain reaction (PCR) – Is used for batches of specimen.

3. Quantitative Buffy Coat – the parasite is stained with acridine orange and
examine under ultraviolet light. It is faster than microscopy.

Disadvantge
i. It cannot count – and this is needed for monitoring the drug
treatment. 50% drops after 24 hours and to 0% after 48 hours, but in
drug-resistant parasites, the pattern does not follow this.

4. Deep stick devices – is based on specific protein produced by parasites. One of

them is histidine-ricj protein. This procedure is very expensive.

Treatment of Acute Uncomplicated Malaria

a. Specific – anti-malarial drugs. It is most important.

1. Chloroquine

Dose
 25mg/kg (base) for 3 days

Oral
0 day - 10mg/kg (stat)
Ist day - 10mg/kg
2nd day – 5kg/kg

Subcutaneous – used if the child is vomiting. Dilute at 2

different sites.
0 day – 5mg/kg
1st day – 10mg/kg
2nd day – 10mg/kg

Monitoring
i. Fever resolves by 72 hours.
ii. Parasitaemia should start disappearing, if parasites are
stil seen on day 4, they are resistant to the drug.

Side effects of Chloroguine

i. Chloroquine associated pruritus
- is common here
- It is not life-threatening, but may be intense
- It starts 24-48 hours, with maximal intensity over
48 – 72 hours and wanes over the 2 days
- It is associated with no rash
- It is almost exclusively a disease of Negroids
- It is self-limiting
- It is not mediated by histamine, thus anti-
histamines given 30 min to 1 hour before giving the
CQ does really improve the condition.
- The affected people are slow-metabolisers of CQ.
- AS individuals do not itch as much as AA.
- It is absent in Caucassians who often use
chloroquine for rheumatoid disease. It is also
useful for amoebic liver abscess.

ii. Choroidretinitis
- the half life of CQ is very especially choroids.
- Choroidretinitis is irreversible.
Second Line Drug
i. Sulphadoxine (500mg)-pyrimethamine (25mg) (Fansidar)
 b. Supportive care
i. Tepid sponging or paracetamol
ii. Oral antiemetics e.g phenegal, 0.5 – 1.0mg/kg body weight per oral or
intramuscular (lateral thigh) for child vomiting

Complications

1. Jaundice - is rare
2. Haemoglobinuria – is due to breakdown of red cells in kidney and not to
haemoglobinaemia. It causes acute renal failure. It is called Black water fever.
3. Renal dysfunction (ARF) –is common and is usually pre-renal
4. Hypoglycaemia
5. Hyponatraemia
6. Anaemia ( hct < 30%)
- is a very common cause of death
- Patients affected are younger ( 1-2 years)
- Thereis reticulocytopaenia

Prevention
i. Safety netting
ii. Malaria chemoprophylais – The use is controversial. Generally, it is not
practised in children as early diagnosis and treatment is preferred.
iii. Use of pathogen e.g bacillus thuringiensis to kill mosquitoes
iv. Vaccination
i. Mass vaccination – as part of EPI in endemic areas
ii. Targeted vaccination e.g for immigrants, temporary visitors,
pregnanct women and sickle cell patient.

Note
Severe anaemia : is haematocrit < 15%

CEREBRAL MALARIA
Introduction
It is the most severe form of malaria. It is life-threatening

Definition : It consists of
a. Asexual parasitaemia of P. falciparum
b. Coma : lasting > 30 minutes. There is either no response of non-purposeful
response to painful stimuli.
c. Normal CSF – protein, sugar, microscopy & culture
d. Absence of other causes of coma – most are viral, also measles

Research Definition
Incidence
o Is 1 – 2%
o Is commoner in male
o It occurs in < 5years and even up to 13 years
o

Pathology
o Slaty-gray discolouration of the brain
o The brain is congested, showing shallow, flattened gyri with narrowed sulci.
o Also shows punctuate haemorrhage in the white matter.
o On histology, macrophages-containing granules which form from digestion of
parasitized red cells

Pathophysiology of Coma
1. Sludging hypothesis – from parasitized red blood cells form a sludge which
blocks brain vessels, thus in resulting into coma

2. Permeability hypothesis –

3. Mechanical hypothesis – due to reduced deformability of parasitized red cells and

cytoadherence of the parasites which is histidine rich. The adhesion molecule is
CD48.

4. Immunological hypothesis – This is important in Quartan Malarial Nephropathy

and Tropical splenomegaly syndrome (now called Hypertrophic Malarial
Syndrome).
- Cytokines
a. Pro-inflammatory : TNF, IL-6
b. Anti-inflammatory : IL-10 which counteracts TNF.

Clinical Feature

Symptoms
i. Fever
ii. Convulsion
iii. Vomiting
iv. Restlessness
v. Abdominal pain
vi. Headache
vii. Reduced urine output

Signs
i. Pyrexia – seen in 100%
ii. Pallor - in 2/3rd of cases
 iii. Hepatomegaly
iv. Acidotic breathing (rapid & deep) – seen in 18 -20%
v. Abnormal posture
Decorticate – is more common
Decerebrate
vi. Neck stiffness
vii. Sqattting
viii. Spasticity
ix. Retinal haemorrhage
x. Monoparesis

Investigation

1. Full blood count

2. Bilirubin – Jaundice is uncommon
3. Biochemical parameters – are generally normal

Treatment
1. Antimalaria
i. Quinine – giving as IV drip

Dose
10mg/kg

Vehicle
- 5% dextrose
- Run for 4 hours, repeat the dose after 8 hours.
– 5 or 10% dextrose is used as many of these patients are
hypoglycaemic which result from poor feeding and also the
quinine being given is associated with hyperinsulinaemia. (
Normal saline is not used )
– Change to oral and continue for 7 days, but if parasitaemia has
cleared and there is some evidence of renal impairment, reduce the
dose of quinine.

Controversy : Loading dose

o Data show no advantage of loading dose over infusion
o Dose – 20mg/kg over 4 hours
o Disadvanatages
i. Deafness
ii. Arrthythmia with prolonged QRS complex.

ii. IM Arthemeter – if quinine fails

 2. Anticonvulsants
3. Antipyretics or tepid sponging
4. Antibiotics
5. Others
i. Correction of dehydration & base deficit
ii. Blood transfusion – nearly 2/3rd require this

Prognosis
o Is better in > 5years and worse in < 3years
o Poor prognosis is associated with
- Acidotic brething
- Impaired renal function – increased serum creatinine

o Biochemical parameters signifying high severity are

iii. Hypoglycaemia
iv. Metabolic acidosis ( ↑ HCO3)
v. Azotaemia (↑ urea)
vi. Impaired renal function
o

Outcome %
1. Survived & Intact 67
2. Transient Neurologival Sequlaes 10
3. Persistent neurological Sequelae 10
4. Case fatality rate 10

Outcome is also grouped into

a. Favourable - consists of “1” and “ 2” i.e total of about 80%
b. Unfavorable – consists of “3” and “4” i.e total of 20%

Summary
Cerebral malaria is seen in >5 years (Toddlers)

o Outcome is worse in malnourished children.

 Chapter 45
MEASLES

Introduction
Is an acute communicable disease

Epidemiology
- Has worldwide distribution
- Is infectious disease.
- Usually because acquired immunity is seen till 4 – 6 months of age after birth,
immunization is given at 9 months. (Some advocate earlier administration)
- In Nigeria, the median age is
o By 1 year, 1/3rd of all children will have had it
o By 3 years, 3/4th will have it.
- Has seasonal variation. It is commoner in dry season ( February – March) because
of dispersal of germs in dust.
- 1,000 cases are seen per year, but this is now decreasing due to immunization.

Pathogenesis

1. Incubation period
- Lasts for 10 – 12 days
- Has very few or no symptoms at all

2. Prodromal stage/Catarrhal stage

- Is marked by en-anthem i.e rash within the body
- Koplit’s spot is the hallmark of diagnosis
- The features are
i. Low grade or moderate fever
ii. Conjunctivitis
iii. Coryza
iv. Increasing severity of cough
v. Catarrh

3. Final stage : there is

- ex-anthem i.e rash outside the body. This is typical maculopapular rash
- high grade fever

Pathology
- Lesion is found
i. Skin
ii. Nasopharynx
iii. Mucous membrane of bronchi, GIT, mouth (koplit’s spot) & conjunctiva
- Inflammation of buccal mucosa – reddened mouth, eye
 iv. Inflammation of trcheobronchial tree – Croup
v. May also cause interstitial pneumonia
vi. Encephalitis – due to viral invasion (this is acute) or immunologic
reaction called Subacute Sclerosing Panencephalitis (SSPE) which occur
after years.

Histology
- Lesions are made up of serous exudates and proliferation of mononuclear cells
with few polymorphs around capillaries.

Aetiology
o Measles virus is an RNA virus (like polio virus) which belongs to paramyxovirus.
o It has only ONE antigenic strain which can be present in nasopharynx, secretion,
urine & blood.
o Transmission is by droplet strains (polio is faeco-polio) i.e it is airborne or by
direct spread.
o Is higly infectious
o Period of infectivity – is period from time of incubation (9 -10 days) to 3 days after
disappearance or rash.

Clinical Presentation

a. Incubation Period
- has very few symptoms & signs
- there is slight temperature
- Coryza

b. Prodromal / Catarrhal stage

- Lasts 3 -5days
- Febrile convulsion
- Bacterial pneumonia – seen in some
- Fever & cough increase in severity up to the time the rash has covered
the body.
- Mouth examination reveals grayish-white dot on reddish alveolar i.e
salt on pepper appearance. The dots are occasionally haemorhagic.

c. Final stage : Ex-anthem

- Temperature rises abruptly as the rash appears
- In uncomplicated cases, the pyrexia should subside within 2 days. If it
persists, suspect secondary infection.
- The rash
o Is typical
o Starts as macules (discoloured spot) behind the ear & along the
hair line.
o It then covers the face, upper chest and arm
 o Within 24 hours, it becomes maculopapular
o During next 24 hours, it sreads to the back & thigh
o By 3rd day, it gets to the feet.
o When disappearing, it starts from the cranial as well
o In summary, the spread and disappearance are cephalocaudal in
direction.
o The severity is related to extent of spread and confluence of rash.
i.e
Mild Severe
1. Few lesions on legs 1. Palms are affected
2. Rash not confluent 2. Confluent & ecchymotic

- Black measles is a severe haemorrhagic firm measles. There is bleeding from

orifices e.g nose , bowel
- As the rash fades, there is brownish desquamation of the skin (peeling off) and
this should disapper within 7 – 10 days. It is associated with itching

Diagnosis

- Mainly clinical – Koplit’s spot

- But don’t make the diagnosis in absence of
• Fever
• Cough
• Conjunctivitis
• Running nose (coryza)

Investigatiom

1. Serology – compare the titre in acute phase and convalescent phase to see it is rising.
2. Full blood count – Leucopaenia ± Lypmhocytosis
3. CSF – to rule out encephalitis in which there is ↑ protein, ↑ lymphocytes and normal
glucose.
4. Viral culture – to isolate the virus.

Differential Diagnosis

1. Rubella (German measles ) – read more

- rashes are not stiking
- There is tender lymphadenopathy over the mastoid area

2. Drug reactions – Steven-Johnson Syndrome

3. Roseola infantum /Ex-anthem subitun/6th day Fever
- Its rash appears when fever disappears
 4. Others
i. Meningococcaemia
ii. Infectioun mononucleosis
iii. Toxoplasmosis

Complications

1. Chest
i. Laryngotracheobronchitis
ii. Bronchopneumonia – due to secondary bacterial infection (Viral
pneumonia is rare)
iii. Activation of old tuberculous lesion or worsening of active tuberculosis

2. Cardiovascular
iv. Myocarditis
v. Arrhythmias
vi. Heart failure

3. Gastrointestinal
vii. Diarrhoea
viii. Vomiting
ix. Cancrum oris
x. Marasmus
xi. Kwashiorkor
Note
- Hypernatraemic dehydration is common in well nourished while hyponatraemic
in malnourished.
- Fast rehydration of hypernatraemic dehydration leads to seizures.

4. Eye
xii. Vitamin A deficiency
xiii. Ulceration of cornea – leading to scarring & blindness
xiv. Purulent conjunctivitis

5. Neurological – are commoner in measles than in any other ex-anthems

xv. Febrile convulsions
xvi. Encephalitis – is seen in acute phase. It occurs in 1 – 2/1,000 of cases. It
occurs within 2 -5 days after appearance of the rash.
xvii. Subacute sclerosing panencephalitis – occurs years (2 -5years) later. It is
due immulonogic slow measles virus invasion. It causes degeneration of
white matter.
xviii. Others – rare
• Hemiplegia
• Gullain Barre syndrome
• Cerebral thrombophlebitis
 • Retrobulbar neuritis

Treatment
Is mainly supportive

1. Antipyretics
2. Eye drops/ointment – after cleaning. Drops is better during the day and ointment
for night.
3. Calamine lotion
4. Adequate fluid intake – because of increase in insensitive water loss
5. Manage complications
6. Magic bullet – vitamin A, 1,000iu for 0, 3 7 days.
7. During the period of photophobia, don’t expose to strong light

Prognosis
Morbidity & Mortality rates in developing are substantial because of underlying
malnutrition & secondary infections.

Prevention

1. Vaccination (active): Measles vaccine is sensitive to heat and light. It is stored at 4C

temp. It has long lasting protection.
Complications
i. Thrombocytopaenia purpura
ii. Febrile seizures
iii. Encephalitis – It is veryrare, it occurs in 1 in 1milion as opposed
to 1-2/1,000 cases in acute encephalitis), therefore, it should not
hinder immunization.

- Note, active immunization is not given to immunocompromised patient.

Immun serum globulin is rather used
2. Quarantine
- Is of little or no value because of the contagiousness during the prodromal
phase when mesles is not suspected.
 Chapter 46
DIPTHERIA, PERTUSIS & TETANUS
Outline
• Introduction
• Epidemiology
• Aetiology
• Incubation Period
• Pathogenesis
• Clinical Features
• Diagnosis
• Differential Diagnosis
• Treatment
• Prevention

Section I
DIPTHERIA

Introduction
- Is a vaccine preventable disease
- Is an acute toxicoinfection (not really an infection)

Epidemiology
- Has worldwide distribution
- Is rare in developed countries, but still found in developing countries.
- Is associated with poverty, poor hygiene and homelessness & overcrowding.
- Peak age : 2- 14 years, though all ages can be affected if not immunized.

Aetiology
- Corynebacterium diphtheriae – is a Gram +ve aerobic non-spore-froming bacillus
- The transmission is airborne, found in the respiratory droplets
- Is also contracted from skin infection – from exudates or due to direct contact.
- Incubation period is 2 -4 days, but may be as long as 17 days.

Pathogenesis
- The bacteria elaborate exotoxins (toxins produced by live bacteria).
- It causes tissue necrosis at sites where it is secreted. It also causes
leucocytosis, fibrin deposition, all these with red blood cells & the organism
 matted together to form grayish-brownish pseudomembrane which is firmly
adherent to the tissue where destruction occurs.
- Symptoms depend on the site of infection
- Occasionally, it is absorbed into blood causing
i. Renal – Necrosis of renal tubules
ii. Bone marrow – Thrombocyotpaenia
iii. Heart – Cardiomyopathy (myocarditis)
iv . Nerves – Peripheral neuropathy (demyelination)

- Another mechanism is via the formation of antigen-antibody complex

deposition in heart & nerves. This is not a direct manifestation, it takes about 2 -3
weeks for it to manifest.

Clinical Features
Depend on the site

a. Upper respiratory tract – is most common. Tonsil and pharynx are most common
and it is referred to as Classical Diphtheria.

b. Skin/Cutaneous – the extremities are more likely affected than trunk and head.
c. Ears – causing otitis media
d. Vulva – causing vulvovaginitis
e. Nose
f. Larynx
g. Eye – conjunctivitis

Symptoms
i. Constitutional – are not so much
o Malaise
o Low gradefever

ii. Tonsils – unilateral or bilateral

iii. Throat – sorethroat which manifests as

o dysphagia or
o horseness.

iv. Nose – most common site in infants

o Serosanguinous or sometime purulent discharge

v. Skin
o Shallow ulcers on nose or upper limb. Is covered by typical
membrane & exudates. The membrane has well defined
(circusmscribed) margin.
 o Is precede by painful sensation (hyperasethsia) , → tingling
sensation → redness & tendernedd → ulcers
vi. Larynx
o Hoarseness
o Stridor
o Has high risk of death

vii. Uvula
o Paralysis from direct affecetation of the toxins may be seen in the
acute phase or via neuritis of palatal or pharyngeal nerves

viii. Lymph node

o Cervical lymphadenitis – node is tender and there is oedema of
soft tissue of the neck, causing Bull’s neck. It may cause respiratory
obstructionwhich is a life threatening event.

Diagnosis
Is mostly clinical

Investigation

1. Culture of nose/throat/cutaneous lesions

2. Serum antitoxin (antibody) titre against the diphtheria toxin.

Differential Diagnosis

a. Nose
1. Foreign body in the nose – even at the purulent stage

b. Pharynx
2. Pharyngitis – throat is angry red, and has constitutional symptoms i.e
patient is more toxic. Organisms responsible are streptococci & EBV
3. Peritonsillar abscess
4. Pharyngeal candidiasis (oral thrush) – it has grayish-whitish patches
/plaques/membrane (and not a single membrane)
5. Vincent’s angina

c. Larynx (Croup - stridor)

6. Acute LTB – viral croup
7. Acute Epiglotittis – H . influenzae. It is life threatening.

d. Peripheral neuropathy
8. Poliomyelitis
9. Gullain Barre syndrome – Post-infectious polyneuropathies
Complications

1. Cardiomyopathy
- Is immune-mediated
- Takes 2- 10 weeks to set
- It is fatal
- Clinical feature is that of unexplained heart failure – cardiomegaly.

2. Neuropathy
- Is immune mediated
- May involve any nerves (Cranial & Peripheral).
- In peripheral polyneuropathy, it is symmetrical i.e level of involvement is the
same on the two sides
- It affects proximal muscle more distal
- i.e it is symmetric & progresses distally ( GBS is also symmetric but progresses
proximally)
- It does not leave permanent damage id patient survives it

3. Respiratory obstruction – is often the cause of death

4. Bronchopneumonia

Treatment

1. Give specific diphtheria anti-toxins

2. Antibiotics – to kill the bacteria therby stopping the production of more toxins.
i. IV crystalline penicillin, 100 – 140iu/kg for 2 weeks in divided doses or
ii. Erythromycin – 40 – 50mg/kg/day for 2 weeks

3. Good supportive care

- Clear up secretions
- Maintain good hygiene
- Maintain fluid

Prognosis
Is quite por because of cardiomyopathy or respiration obstruction.

Prevention
- Is most useful
- Notify appropriate authorities – the risk of infection after contact is very high
(2/100) with a carrier, it is 0.6% . Therefore, treat all the household or school
contact with erythromycin for 7 days.
- Large scale prevention – using Diphtheroid toxin. Its protection is not life-
long, but confers herd immunity.
-
Section II
PERTUSIS

Section
TETANUS
 ANAEMIA
Definition
Is reduced red cell mass.

Measurement
1. Haematocrit (PCV)
2. Haemoglobin concentration – is more accurate
Hb (1g/dl) = 37hct
Reference values for adult
Hb – 15g/dl
Hct – 45%

Diference between anaemia in Children & Adults

1. Children are growing faster than adults, therefore required more haematinics
2. Haemopoetic equilibrium is less well established in children
3. Infection is common in children, causing marrow depression.

Age-related Definitions
• Physiological anaemia
• Anaemia of prematurity

Neonate : < 45%

Infancy : < 30%,
Childhood : < 35%

• Haematocrit is higher in venous than arterial capillary.

• It is higher at birth, 55± 5% . Is SGA, it is higher
• Over the first 7 days of life, the fetal haemoglobin drops because fetal Hb higher oxygen
affinity than adult hemoglobin

Anaemia of Prematurity
Types of infants
a. Preterm infant
b. Term infant

Generally, haematocrit of preterm infant is lower than that of term infants. This is
because the preterm babies have low stores of haematinics (in 3rd trimester). The
treatment usually involves blood transfusion. Large doses of erythropoietin is also used .

Phases of Blood formation (Haemopoeisis)

1. Mesoblastic phase – yolk sac
2. Hepatic phase – liver
3. Myeloid phase phase – bone marrow
N.B Erythropoietin is produced by the juxtaglomerular cells.

Classification of Anaemia
This is based on the underlying mechanism

a. Increased destruction
b. Reducued production
c. Blood loss

I. Anaemia Due to Increased destruction of red blood cells

a. Intracorpuscular (Intrinsic)
1. Defect in Red cell Membrane
2. Defect in Haemoglobin structure
3. Defect in Enzymes

b. Extrinsic
1. Microangiopathic anaemia
2. Hyperslpenism
3. Burns
4. Paroxysmal Norcturnal Haemoglobin
5. Malaria

II. Anaemia Due to Reduced Production (Deficiency anaemias)

1. Iron-deficiency
2. Folate deficiency
3. Vitamin 12 Deficiency
4. Vitamin B6 Deficinecy
5. Anaemia of Chronic infection /diseases
6. Micronutrient and macronutrient deficiency (malnutrition)
7. Blackfan diamond anaemia (pure Red cell Apasia) :
- Its features include hypocellularity
- Idiosyncratic hyperplasia
- Rare and some respond to drugs

8. Infections
Bacteria : e.g Pneumonia, the bacterial toxins depress the bone marrow
Viral : e.g acute aplastic anaemia produced by HPV-B-19 (Parvo-virus) infection.
The retic count drops to zero. The virus switches off marrow for a week
or two.
9. Leukaemias
– there is replacement of red cell population by the malignant white cell. The
normal myeloid : Erythroid ratio is 4 : 1
 10. Renal failure
11. Heavy metals e.g lead, mercury

III. Anaemia Due to Blood Loss

- Can be divided into

a. Coagulation defect : vWD, Haemophilia

b. Platelet dysfunction : Idiopathic thrombocytopaenic purpura

c. Abnormalities of blood vessels

d. Autoimmune disease : Autoimmune haemolytic anaemia, Coomb’s test is

used to make the diagnosis.
e. Haemorrhage
In newborns, it can be
a. Subdural
b. Subgaleal
c. Subperiosteal – its swelling does not cross the suture line

f. Rupture of spleen – following fracture

g. Acute Sequestration syndrome in HbS

Common Causes of Anaemia

a. Infancy
i. Haemolytic disease of the newborn (Neonatal jaundice)
ii. Infections
iii. Iatrogenic : from blood samples being taken for investigations. It is
commoner in low birth weight babies. As the total blood volume of
newborn baby is 80mls/kg of body weight.

b. Children
i. Malaria
ii. Haemoglobinopathies
iii. Infections : Septicaemia, Emphysema, Pneumonia, Osteomyelitis

N.B
- Classical hemolytic anaemia : Increased bilirubin levels
Increased reticulocytes count

- Post-circumcision bleeding - has nothing to do with haemorrhagic disorders, so also

anaemia due to hookworm infection.
Investigations
1. Haematocrit/Packed cell volume – can also be used to calculate MCV & MCHC in
order to distinguish macrocytic from microcytic.
2. White blood cell count

3. Blood films
Thick films – for malarial parasites
Thin film – shows hypersegmented blood cells, elliptocytes, spherocytes

4. Stool microscopy – check for hookworm ova, schistosoma mansoni ova, Entamoeba
histolytical cyst etc.

5. Haemoglobin electrophoresis

6. Ferokinetic studies – Serum iron, Free protoporphyrin

7. Serum folate level
8. Clotting profile : Prothrombin time
9. Platelet count
10. Bone marrow aspiration/biopsy
11. Drug level concentrations
12. Heavy metal concentration level especially lead.

N.B the first 5 tests are very important.

Management
1. Antimalarials
2. Antibiotics
3. Antihelminthics
4. Replacement therapy – for iron, folate, vitamin E, B12, B6(rarely) and vitamin C
5. Blood transfusion – usedin life threatening anaemia. Before giving blood, you
must do first 5 tests with G6PD screening.

Rules About Transfusion

1. Don’t give a child > 20mls/kg body weight for whole blood & 10 -15mls/kg body
weight for packed cells.
2. After giving, you wait for 24 -48 hours before you give more
3. Use whole blood for acute haemorrhage
4. Use fraction of blood when indicated e.g platelet concentrate for DIC
5. Give frusemide at the beginning of transfusion to contract the blood volume because
of the cardiovascular compensation in anaemia.
Prevention

1. Malaria control
2. Proper weaning practices – encourage high protein diet and prevent iron deficiency in
the first 2 years of life
3. Immunisation
4. Control of infection
5. Good hygienic practices.
 Chapter 54
INTRODUCTION & CYTOGENETIC DISORDERS
(CLINICAL GENETICS I)

Introduction

Genes are defined as lengths of DNA (genetic material of all chromosomes in a cell) which
constitute a major component of chromosomes. There are about 50,000 – 100,000 human
structural genes encoded in the DNA.

Some DNAs are found outside the nucleus e.g in Mitochondria which has DNA that is quite
different from nuclear DNA.

Parts of genes
i. Exons – codes
ii. Introns – do not code
iii. Regulatory sequences

Basic Types of Cell Division

a. Mitosis – give rise to 2 daughter cells

b. Meiosis – Has 2 functions
i. For reduction division
ii. The chiasma formation and exchange of chromatin
material to shuffle and create variation, thus increasing
the chance of survival.

Fertilization
- Is fusion of haploid gametes leading to the formation zygote with diploid
chromososmes.
- All cytoplasm comes from the mother and the most important of its component is
mitochondria, thus mitochondrial disorders show maternal inheritance.
(Mitochondrial Eve – all mitochondria came from Eve, though there have some
mutations along the line.)

CLINICAL APPLICATION : THE SPECTRUM OF GENETIC DISORDERS

The spectrum of genetic disorders consists of 5 types

1. Single gene /Monogenic /Mendelian disorders

2. Chromosomal/Cytogenetic disorders
3. Multifactorial disorders /Polygenic
4. Somatic cell disorders
5. Mitochondrial disorders
6. Others

1. Single gene /Monogenic /Mendelian disorders

– Is common
– is due to single gene mutations and inherited as recessive (effect produced only
when lesion affects both alleles) or as dominant (effect produced when lesion
affects either of the alleles)

– Every autosome has 2 copies, but for sex chromosomes, only female has 2 copies
while male has one. But note, one of the female X-chromosomes is quiescent. The
process of inactivation of one of the X-chromosome is random and is called
Lyonization (Lyon, a female Scientisit). This means every female is a mosaic i.e
with 2 different cell lines.

– The consequence of this is that only a female can be homozygous or

heterozygous, male is referred to as hemizygous.

Types
a. Autosomal disorders –
b. Sex-linked disorders –

a. Autosomal Disorders

- Can be
a. Autosomal Recessive - arise from homozygous genes e.g Sickle cell
disease
b. Autosomal dominant - arise from heterozygous genes e.g
Achondroplasia

Sickle cell disease

AA – normal
AS – normal because the effect of the S gene is recessive to A gene, it is
autosomal reccessive
SS - affected

Achondroplasia
AÃ - Achondroplasia, the effect of abnormal gene is dominant
ÃÃ - Affected
AA – Normal

Note : Codominance e.g in ABO blood groups

 b. Sex chromosomes
- Most sex chromosome disorders are X-linked and most of these X-
linked are recessive
- There are 2 – 3 times more in males
- Only female can be heterozygous or homozygous, male is referred to as
hemizygous.
- Y –chromosome is one of the smallest chromosomes, it contains very
little genes (only TDF which forms male gonads). In contrast, X-
chromosome contains many genes such most fetuses hardly without X-
chromosome.

Female
XX – normal
XX – most times, the female is not affected at all since there is 50 : 50
chance of manifestation (Lyonization)
XX

Abnormal example – female haemophiliacs. It is seen in

i. Homozygous
ii. Turner’s syndrome (45X)
iii. Testicular feminizing syndrome
iv. Heterozygote with abnormal lionization i.e
not 50 : 50

Examples
Autosomal Recessive Autosomal dominant
1. Phenylketonuria 1. Marfan syndrome
2. Galactosaemia 2. Achondroplasia
3. Tyrosinaemia 3. Nail Patella syndrome
4. Homocystinuria 4. Alports syndrome
5. MPS 5. Noonan syndrome
I - Hurler
III – Sanfilipo
IV - Morquio
VI - Maroteau
6.

Sex chromosome disorders

X-linked Recessive X-linked Dominant
1. MPS 2 - Hunter’s Vitamin D-resistant Ricket
(Hypophosphataemia)
2. Fabry disease
3. Menke’s kinky hair
 4. Lesch-Nyan Syndrome

2. Chromosomal /Cytogenetic Disorders

- Is caused by abnormalities in number & structure of chromosomes.

- Are very common at ( 7.5%) conception but far less common among live birth
becauses of high rate of first trimester abortions.
a. 60% of early spontaneous abortions have cnromosomal abnormality
b. 5% of late spontaneous abortions
c. 4 – 5% of stillbirths have chromosomal aberrations

Classification

Chromosomal Aberrations

Numerical Structural
e.g
1. Translocation
2. Deletion
3. Duplication
Polyploidy Aneuploidy 4. Inversion
- exact multiple of - not exact multiple of multiple 5. Isochromosome
haploid number of haploid number 6. Ring fragments
- e.g 69XXY tryloidy
- Largely compatible
With life

Monosomy Trisomy
(Missing copy) ( Extra copy)
e.g 45X Turner’s syndrome e.g Trisimy 21 (Down’s)

Note
n - gametes
2n - somatic
3n - Megakaryoblast, Osteoblast

Principles of Cytogenetis

• Cytogenetics – is the study of chromosomes in cells

• The chromosomes are named from their ability to take up stains (Chromo means
colour, some means body
 • They are present in all nucleated cells and contain DNA with associated proteins
• Each species has a chromosome complement characteristics in number and form
– referred to as Karyotype
• The karyotype can also be referred to as the photographic representation of
stained chromosomes arranged in order of decreasing length.
i.e Chromosome 1 - largest
Chromosome 2 - smallest
XY is always put at the end
• The act oc producing it is called Karyotyping. ( The simplest method is using
lymphocyte culture and stimulate then with PHA. These arrest them at
metaphase.

Nomenclature
• Karyotypes are usually describe using a short hand system of symbols (Paris
nomenclature) which usually has the order
i. Total number of chromosomes
ii. Sex chromosome constitution
iii. Description of abnormality

e.g
46 XY means that the total number of chromosomes is 46, the sex chromosomes
complements is XY.
46 XX 5p- : Criduchat
46XY t(8 : 14) - Burkitt’s lymphoma

Types of chromosomes
1. Acrocentric
2. Submetacentric
3. Metacentric

Definition of terms

1. Hypertelorism - if the distance between medial and lateral canthi is less than distance
between medial canthi

2. Hypotelorism - if the distance between medial and lateral canthi is more than
distance between medial canthi

3. Upward Slanting palpebral fissure - Palpebral fissure slanting upwards from medial
canthi laterally.

4. Antimongoloid palpebral fissure – Palpebral fissure slanting downwards from medial

canthi laterally.

5. Brachycephaly – Reduced anteroposterior distance of head

6. Low set ears – Portion of pinna above imaginary line from lateral canthus to occiput is
less than a third

7. Widely spaced nipples – If inter nipple distance in > 25% of chest circumference at
nipple at level.

8. Ectrodactlyly - Absence of a digit, usually and mostly unilateral

9. Coxa vara – Reduction of angle between femoral neck and shaft (normal is 120 – 135)

10. Clinodactyly – Incurved digit, whether medially or laterally

11. Brachydactyly – Short digits

12. Syndactyly – Fused digits

13. Polydactyly - > 5 digits (usually bilateral)

 Chapter 55
CONGENITAL MALFORMATIONS
Introduction

• These are gross structural defects that present at birth. (Congentins, a Latin word
means born with)
• Congenital malformations = Congenital anomalies = Birth defects
• An understanding of congenital malformations is firmly rooted in a thorough
knowledge of embryology.

Other Definitions
a. Tetralogy : is the branch of science that studies the cause, mechanism and
patterns of abnormal developments

b. Dysmorphology : is an area of Clinical Genetics that is concerned with

diagnosis and interpretation of patterns of structural defects.

Types of Birth Defects

a. Major defects – are those with major medical, surgical and cosmetic significance.
If the defect is left uncorrected, it significantly impairs normal body function or
reduce life expectancy e.g Cleft lip, Spinal bifida

b. Minor defect – is an unusual morphologic feature that has no serious medical or

cosmetic consequence to the patient. E.g
• Preauricula pit
• Clinodactyly
• Polydactyly – commonest in blacks where frequency is about 1%

c. Normal Variant – Physical features that fell at the far end of the spectrum of
normal configuration e.g Low anterior hairline, bulbous nose.

Frequency of Occurence
- It occurs in 10 – 15% in early conceptions but most spontaneously abort during
the first 6 weeks.
- 2 – 3% of newborns have major congenital malformations while 14% has minor
CM.
- Additional malformations are detected after birth, thus the frequency of CM is 6%
in 2 years old and 8% in 5 years old. An additional 2% will be detected later in life
(during imaging, procudres, surgery and autopsy)
-