A Primary Prevention

B Secondary Prevention
C Tertiary Prevention
D Health Promotion
E Health Education
F Child Health Surveillance
G Public health

The definition below are best describe by which of the options above. Each option may be
used once, more than once, or not at all.
It is important to consider the child in society and the importance of looking at child health
in the community as a whole. Secondary prevention is reduction of prevalence of disease
by shortening duration or diminishing the impact through early detection and prompt
intervention (eg screening). Health promotion is any measure that improves health or
prevents disease. Public health is a science preventing disease, prolonging life and
promoting health through the organised efforts of society.

Scenario 1

Correct
Reduction in number of new cases of a disease, disorder or condition (accidents)

A Correct answer

Primary Prevention

Scenario 2

Correct
Any activity which promotes health through learning

E Correct answer

Health Education

Scenario 3

Incorrect
Reduction of impairment and disability, and minimizing suffering (eg multidisciplinary
approach of a child with Downs syndrome)

D Your answer

C Correct answer
Tertiary Prevention

Which of the following are associated with Bulimia?

Correct
Cardiac arrhythmias True False

Correct
Enhanced self image True False

Incorrect
Good response to anti-depressant medication True False

Incorrect
Previous history of anorexia nervosa True False

Incorrect
Feelings of guilt True False

Bulimia nervosa comprises: a preoccupation with food associated with episodes of gross
overeating or 'bingeing'; a fear of fatness; and the development of techniques to
counteract the fattening effect of binges, (self-induced vomiting, use of laxatives).

Bulimia is far more common in girls. In some cases of bulimia there is evidence of a
previous episode of anorexia but most often bulimia develops de novo as a syndrome in its
own right. The disorder tends to persist. It is often kept secret from others and the
repetitive eating binges can interfere with social relationships, the patient becoming guilty
and depressed. The repeated vomiting and/or purging may lead to a variety of physical
side-effects ranging from eroded dental enamel to hypokalemia and impaired renal
function. Cardiac arrhythmias may be a consequence of electrolyte abnormalities.

The current treatment of choice is cognitive-behavioural therapy.

The following are true regarding gastro-oesophageal reflux?

Correct
It can be treated successfully non pharmacologically True False

Correct
Is usually associated with blood loss True False

Correct
Most children fail to thrive True False

Correct
It is usually self limiting True False

Correct
It is commoner in cerebral palsy True False

Gastro-oesophageal reflux is the passive regurgitation of gastric contents into the

oesophagus. Gastro-oesophageal reflux will result if there is incompetence of the
sphincteric mechanisms at the gastroesophageal junction or if raised intragastric or intra-
abdominal pressures are able to overcome this mechanism. In conditions where gastric
emptying is delayed or in chronic respiratory diseases, such as cystic fibrosis, where
coughing increases intra-abdominal pressure, Gastro-oesophageal reflux is exacerbated.

Gastro-oesophageal reflux is a physiological phenomenon which occurs in all individuals for

between 1 and 5% of any 24-h period. Whether Gastro-oesophageal reflux is deemed to be
trivial or pathological is dependent on many factors including the presence of complicating
sequelae (eg aspiration, symptoms of heartburn or vomiting, behavioral sequelae (e.g
refusal to eat), FTT).

Simple measures such as positioning are often sufficient to reduce Gastro-oesophageal

reflux. Where these simple measures fail to reduce the Gastro-oesophageal reflux the use
of prokinetic agents should be considered. In patients with oesophagitis, H2 blocking or
proton pump inhibitors seem to help in the healing of oesophagitis.

5-year-old child presented with oedema of the lips after ingesting a peanut. Which of the
following are true regarding management of this patient?

Incorrect
Hyposensitisation therapy should be performed True False

Incorrect
Peanut allergy can be reliably diagnosed by skin testing True False

Incorrect
Peanut allergy can be reliably diagnosed by serum RAST True False

Correct
If peanut allergy is confirmed, allergy to other nuts should be considered True False

Incorrect
A history of apparent allergic reaction to nuts is usually inadequate for a True False
diagnosis

Hyposensitisation therapy can be dangerous and is not routinely recommended. IgE titres
and skin tests are limited by false positive and false negative results. The definitive
diagnostic test for nut allergy in the hospital setting is direct oral challenge.

12-year-old girl was diagnosed with meningococcal meningitis. She was treated successfully
and made a full recovery. Appropriate further action would include which of the following?

Correct
Treatment of immediate family with rifampicin True False

Incorrect
Treatment of the entire class with rifampicin True False

Correct
Immediate notification of the local Public Health Laboratory Service True False

Incorrect
Nasal swabbing of the childs entire class to detect carriers True False

Correct
Provision of information leaflets to the childs school True False

People who have come into close contact with meningococcal meningitis and/or
meningococcal septicaemia require antibiotics. Close contacts are people who are living or
sleeping in the same household or who have intimately kissed the patient. School friends
and colleagues of the patient with meningococcal disease are rarely at higher risk. The
patient will have come into contact with other people during the incubation period. These
people are deemed as casual contacts and they are not at any increased risk.

Long-term benefits of breast feeding include:

Incorrect
Prevention of gastrointestinal infections True False

Correct
Decreased incidence of LDL:HDL cholesterol ratio True False

Correct
Protection against the development of inflammatory bowel disease True False

Correct
Decreased incidence of atopic disease True False

Correct
Reduced risk of developing neoplastic disease True False

Short-term benefits of breastfeeding include decreased incidence of:

GIT infection
Atopic disease
Ear infections
NEC in preterms and low birth weight babies
Systemic infections in preterms

Studies carried out have provided evidence indicating that breastfeeding decreases risk
factors for the development of cardiovascular disease at an older age by decreasing:
1. Blood pressure: blood pressure in adults studied was lower in individuals who were
breast fed when compared to those who were formula fed. Prevalence of
hypertension was decreased by 17%, ischaemic heart disease by 16% and stroke /
TIAs by 15 %
2. Decreased LDL:HDL cholesterol ratio : breast feeding is associated with an
increased in total and LDL cholesterol concentrations in infancy, but decreased
values in adult life.
3. Decreased incidence of obesity
Breast feeding was not found to be protective against the development of inflammatory
bowel disease, whilst evidence to support association between breastfeeding and the
development of neoplastic disease is inconclusive.

Correct
The secretion of growth hormone is increased by?

Hyperglycaemia

Exercise
Your answer

Somatostatin

Growth hormone

Free fatty acids

Growth hormone (GH) is synthesised, stored, and secreted by the endocrine cells of the
anterior pituitary. Its release is stimulated by growth hormone-releasing hormone and
inhibited by somatostatin. Numerous factors serve as a stimulus for GH release, including
hypoglycaemia (e.g. insulin administration), moderate to severe exercise, stress due to
emotional disturbances, illness, and fever, and dopamine agonists such as bromocriptine.
Which are true regarding the routine neonatal examination?

Incorrect
It must be carried out by a doctor True False

Correct
It should include full abduction of hips to exclude dislocation True False

Correct
It may miss serious congenital cardiac defects True False

Incorrect
Ideally the examination should take place with a parent present True False

Correct
Measurement of head circumference is not required as intrauterine
moulding will affect measurement True False

A midwife trained in neonatal examination could do the initial assessment. Even the most
expert examiner will miss many cases of congenital heart disease if duct dependent since
they may not be detectable in the early days of life, before the ductus closes.

Recognised causes of methaemaglobinaemia include:

Incorrect
Dapsone True False

Incorrect
Isoniazid True False

Correct
Prilocaine True False

Incorrect
Nitric oxide True False

Incorrect
Nitrates True False

Methaemoglobinaemia is caused by uncoupling of oxidative phosphorylation and any of the

nitrates, local anaesthetics or dapsone may be responsible. Treatment is with methylene
blue.

Which of the following are common features of Juvenile chronic polyarthritis (Still's
disease)?

Correct
Maculopapular rash True False

Correct
Endocarditis True False

Correct
Lymphadenopathy True False

Correct
Hypotension True False

Correct
Hypothermia True False
The most common age of onset of the disease is under 5 years, but it can occur throughout
childhood. Boys are affected as frequently as girls.

Children with JCA usually present apparently toxic and irritable usually with swinging
fever. Splenomegaly and general lymphadenopathy are almost invariably present but
arthritis may often be delayed for weeks or even months. A large number of children have
a typical maculopapular rash which is usually nonpruritic . Pericarditis may occur but is
usually benign. Rarely, myocarditis can occur and may lead to congestive cardiac failure
Endocarditis is not a feature of JCA. Hepatitis is an uncommon manifestation during the
systemic phase

Incorrect
Consider the options presented below. What screening does not occur in the United
Kingdom.

Heel Prick Test to screen for congenital hypothyroidism

Red Reflex to screen for congenital cataract

Echocardiogram in children at 6 weeks to screen for / diagnose cardiac

structural disease Correct
answer

Otoacoustic emission to screen for hearing impairment

Your answer

Heel prick test to screen for phenylketonuria

Auscultation is used to pick up murmurs in children, not echocardiograms.

Following are associated with trinucleotide repeats:

Incorrect
Spinocerebellar atrophy 7 True False

Correct
Fragile X syndrome True False
 Correct
Myotonic dystrophy True False

Incorrect
Huntingtons disease True False

Correct
Friedriechs ataxia True False

Myotonic dystrophy is inherited in an autosomal dominant manner. It arises

due to the presence of an unstable tri-nucleotide repeat expansion, and the
larger the expansion, the earlier the age of onset of symptoms. The
expansion tends to increase in size in successive generations, leading to an
increased severity of the condition. This is known as anticipation.
Huntingtons disease, SCA 7 and Fragile X syndrome also display this
phenomenon. Cystic fibrosis (autosomal recessive) and Marfans syndrome
(autosomal dominant) do not have unstable tri-nucleotide repeat
expansions. Friedreich's ataxia (autosomal recessive) is due to tri-
nucleotide repeat expansions in the mitochondrial protein frataxin.

Which of the following necessitate a prompt mental health assessment?

Incorrect
A 13 year old who has been absent from school for 3 months
due to abdominal pain which has been investigated and no True False
cause has been identified

Correct
A 7 year old who tells his parents he is afraid of the dark True False
 Correct
A 12 year old boy who, having been previously sociable, has True False
become withdrawn at school

Correct
A 15 year old who has taken a deliberate overdose of 10mg True False
diazepam

Correct
A 6 year old boy who tells his teacher he has been miserable True False
for a few days

A referral for a specialist mental health assessment is mandatory when

there are signs suggestive of a major psychotic illness, major emotional
illness, (e.g. obsessional-compulsive disorder or school refusal) or eating
disorders if symptoms have persisted for more than 3 months. Referral is
also strongly recommended when children present with a suicide attempt or
there is disclosure of sexual abuse or for perpetrators of sexually abusive
activity. It is also probably recommended for conduct disorders, poor family
function significantly impairing maturation and development, alcohol,
solvents and opiates dependency and poor coping with the psychological
effects of chronic physical illness or handicap.

Regarding Handwashing

Correct
Ordinary soap and water is not effective against bacteria True False

Correct
Staff should wash hands after every patient contact True False

Correct
Staff should wash hands before examining every patient True False

Correct
Hand transmission is a major source of serious infection True False

Correct
Pseudomonas aeruginosa cannot be removed with the use of alcohol rubs True False

Formal handwashing with soap and water is required when there is soiling. When there is
none the hand hygiene liaison group now advocates that staff should use an alcohol-glycerol
hand rub between patients.

Which of the following biochemical parameters are consistent with a diagnosis of pyloric
stenosis?

Correct
Hyperchloraemia True False

Correct
Alkalosis True False

Correct
Low PaCO2 True False

Correct
Raised haematocrit True False

Correct
Low total body volume True False
In pyloric stenosis, volume depletion and H+/Cl- loss occur.

Loss of H+ stimulates more carbonic anhydrase activity to attempt to replace the lost H +
which results in more HCO3- as well. If dudodenal secretion and renal excretion of
bicarbonate are not able to correct this rise in plasma HCO3- then hypochloraemic alkalosis
develops.

Well established causes of neonatal hypotonia include which of the following?

Correct
Maternal myasthenia gravis True False

Correct
Werdnig Hoffman disease True False

Correct
Neonatal infection True False

Correct
Hypothyroidism True False

Incorrect
Maternal treatment with diazepam True False

Incorrect
A newborn baby boy has facial deformities and a small-misproportioned head. He is irritable,
hypotonic and has severe tremors.

What diagnosis, related to antenatal care, would best account for these symptoms?
 Fetal alcohol syndrome
Correct answer

AIDS

Downs syndrome
Your answer

Microcephalus

Hydrocephalus

FAS was first reported as a syndrome in 1973 and is now thought to be one of the major
causes of mental retardation, having an incidence of 0.23 per 1000 live births. It has been
estimated that between 10 and 20% of mild mental retardation cases are caused by
maternal alcohol use. Severity and timing of alcohol consumption, bingeing, polydrug use
(including smoking) during pregnancy, genetic variation and low socioeconomic status are
all aetiological factors. Alcohol inhibits N-methyl-d-aspartate (NMDA) receptors, which
mediate the postsynaptic excitatory effects of glutamate, and this is thought to have an
effect on cell proliferation.

Affected newborns are often irritable, hypotonic, experience severe tremors and show
other signs of alcohol withdrawal. The cardinal signs are facial features, growth deficit and
central nervous system impairment. Facial features include epicanthic folds, microcephaly,
short palpebral fissure, underdeveloped philtrum and a thin upper lip. There are often
associated behavioural difficulties including hyperactivity and sleep disturbance. Optic
nerve hypoplasia with poor visual acuity, hearing loss and receptive and expressive
language deficits can also be seen. Cardiac and renal abnormalities include atrial and
ventricular septal defects, renal hypoplasia and bladder diverticula
A 9-year-old boy was diagnosed with Diabetes mellitus. Which of the following are true
regarding his management?

Correct
Proper control should involve no glycosuria True False

Incorrect
Ketones in early morning urine specimens are normal True False

Correct
A young child can be easily controlled on once daily regimens True False
 Incorrect
Children should not inject insulin into the anterior abdominal wall True False

Incorrect
Control should a im for a HbA1C of <7.5 True False

Previous question Next question

Correct
Which of the following statements regarding Status Epilepticus is true:

Defined as convulsion lasting for more than 1 hour

Always seen in a known epileptic

The drugs can be administered intramuscularly if IV access is difficult

The mode of treatment: ABC management Maintain blood glucose- Up to 2

doses of IV lorazapam/ Diazapam Rectal Paraldehyde- IV Phenytoin-Consider
General Anaesthesia if still continues to fit. Your
answer

Pre-hospital management includes rectal diazepam and buccal midazolam

given both together.

Notes: Defined as continuous convulsion lasting longer than 30 minutes or the occurrence
of serial convulsions between which there is no return of consciousness.
These are common in poorly controlled epileptics but also seen in febrile seizures,
electrolyte abnormalities, encephalitis, inborn error of metabolism & HIE in newborns,
tumours and Reye syndrome.
Pre-hospital treatment include either buccal midazolam or per-rectal diazepam.
In the hospital use always intravenous route. Intramuscular route not preferred as drugs
are sequestered in the muscle. If IV access not available, try intra-osseous route.
Not more than 2 doses of benzodiazapines recommended. Either per-rectal diazepam or
buccal midazolam used. Overdose of benzodiazapines cause respiratory depression.

Diagnosis of asthma is likely in a 4-year-old child if:

Correct
Peak expiratory flow rate (PEFR) is below 200 True False

Incorrect
FEV1 increases by 10% after bronchodilators True False

Correct
Chest X-ray (CXR) shows focal bronchial wall thickening True False

Correct
Symptoms tend to worsen overnight True False

Incorrect
Wheeze is present True False

PEFR is a very good guide for diagnosis of asthma, but is not a reliable tool in children
younger than 5 years of age. An increase of 20% in FEV1, as a response to an adequate dose
of nebulised bronchodilators, is diagnostic of asthma but its absence does not rule it out.
CXR is not very helpful in diagnosis of asthma but if unifocal changes are noticed, diagnosis
should be questioned and foreign body inhalation or infection should be considered. A
symptom record diary should be kept and points towards diagnosis of asthma if symptoms
tend to get worse at night. Wheeze on its own is not diagnostic of asthma as it can be
caused by various different causes.

Previous question Next question

 Correct
You review a 17-year-old who has suffered from Still's disease since childhood.
Which of the following are common features of Still's disease?
Negative rheumatoid factor
Your answer

Normal erythrocyte sedimentation rate (ESR)

Normal C-reactive prote in (CRP)

Thrombocytopenia

Splenic atrophy

Stills disease (which accounts for 10% of cases of juvenile idiopathic arthritis (JIA) affects
boys and girls equally up to the age of five years of age, after this point, girls are more
commonly affected. Adult-onset Stills is rare. Clinical features include fever,
maculopapular rash, myalgia and generalised lymphadenopathy. Hepatosplenomegaly,
pericarditis and pleurisy also occur. Laboratory tests reveal raised ESR and CRP,
neutrophilia and thrombocytosis, though autoantibodies are negative.

Incorrect

A 10-year-old Egyptian boy who has recently immigrated to the UK is found to have hepatitis C
infection. He is unsure as to how he could have acquired this disease. His mother died of
jaundice 10 years ago. He was treated in Egypt 3 years ago for a bladder infection and the
passage of blood in his urine.

What is the most likely method of transmission in this case?

Contact with the local population

Vertical transmission
Your answer

Sexual transmission

Contaminated drinking water

 Intramuscular injections
Correct answer

In this case, the most probable cause is parenteral antimony treatment for schistosomiasis
and improper sterilisation of the equipment used to administer this. The rate of hepatitis C
infection in Egypt is estimated to be as high as 20% due to this means of communication of
the virus. Vertical transmission can occur but is very rare.

Which of the following may be physiological?

Correct
Breast development in a 3-day-old boy True False

Correct
Breast development in an 8-year-old girl True False

Correct
Breast development in a 14-year-old boy True False

Correct
Pubic hair in a 2-year-old girl True False

Correct
Pubic hair and breast development in a 5-year-old girl True False

Breast development is relatively common at certain times in childhood and can be

considered a normal variant. Neonatal gynaecomastia is probably due to maternal hormone
effects. An eight-year-old girl might be in the early stages of puberty especially if >50
percentile for weight. Teenage boys frequently develop gynaecomastia which is usually self
resolving though can be embarassing and may require cosmetic surgery.

In a younger girl (2-3 years old) the cause might be idiopathic premature thelarche which is
again usually self resolving. Other sexual characteristics (pubic hair, penile changes etc.)
ocurring in young children are signs of precocious puberty and should be investigated and
treated accordingly.
a child has abdominal pain and blood in the stools, the following diagnoses should be
considered?

Correct
Mesenteric adenitis True False

Correct
Meckels diverticulum True False

Correct
Campylobacter infection True False

Correct
Abdominal migraine True False

Correct
Ulcerative colitis True False

Common causes of abdominal pain with blood in the stools include constipation (anal
fissure) and infection with campylobacter or salmonella. Ulcerative colitis, Meckels
diverticulum, volvulus, HSP, peptic ulcer disease and gastritis are other causes. Mesenteric
adenitis and abdominal migraine can both present with abdominal pain, however blood in
the stools is not a recognised feature.
The criteria for registration on the Child Protection Register are:

Correct
Is a medical emergency True False

Correct
Sexual abuse True False
 Incorrect
Failure to thrive True False

Correct
General concern True False

Incorrect
Bullying True False

The criteria for registration on the child protection register are physical injury, sexual
abuse, failure to thrive, emotional abuse and physical neglect. There are cases were there
might be issues in more than one of these areas, but one has to be chosen as the primary
reason for registration and only this is recorded on the register.

Correct
What BMI is diagnostic of anorexia?

< 13.0

< 15.5

< 17.5
Your answer

< 19.5

< 22.0

This is one of the diagnostic criteria. According to ICD-10 all the following are required
for a definite diagnosis of anorexia nervosa:
Quetelets body mass index is 17.5 or less
The weight loss is self-induced
There is body-image distortion and a dread of fatness
There is disturbance of the hypothalamicpituitarygonadal axis (manifest in
women as amenorrhoea and in men as a loss of sexual interest and potency)
If the onset is prepubertal, the sequence of pubertal events is delayed or
even arrested.

The risk of septicaemia in a pyrexial child is increased by which of the following:

Correct
Generalised convulsions for 5 minutes True False

Correct
Sickle cell trait True False

Correct
Purpura True False

Incorrect
Floppiness True False

Correct
White cell count < 3x109/l True False

There are many ways children may respond in septicaemia. High or low fever, convulsions,
irritable or floppy, high or low white count, purpura (especially in meningococcal disease).
Sickle cell crisis presents with pain and sometimes fever and will require treatment with
pain relief, antibiotics and fluids.

Correct
A 2-year-old boy has vitamin D-resistant rickets. Investigations show: serum calcium 2.6
mmol/l, phosphate 0.5 mmol/l and alkaline phosphatase 1040 U/l. Parathyroid hormone and
bicarbonate levels are normal.

What is the most probable diagnosis?

 Distal renal tubular acidosis

Hypophosphataemic rickets
Your answer

Vitamin D-dependent rickets

Proximal renal tubular acidosis

Hyperparathyroidism

Disorder Serum Serum Alkaline Parathyroid

calcium phosphate phosphatase hormone
Hypophosphataemic rickets Normal or Decreased Increased Normal
decreased
Vitamin D-dependent Decreased Normal or Increased Increased
rickets decreased
Hyperparathyroidism Increased Decreased Increased Increased
Nutritional rickets Decreased Decreased Increased Increased

Proximal tubular acidosis also causes hypophosphataemic rickets but the bicarbonate level
will be reduced. Distal tubular acidosis causes vitamin D-dependent rickets.

A 12 months
B 15 months
C 18 months
D 2 years
E 2 years
F 3 years
G 4 years
H 5 years
I 6 years

For each of the following cases select the most likely developmental age from the list
above:

Scenario 1

Correct
Hops on one foot, throws ball over arm, goes to toilet alone, draws square from copy.

G Correct answer

G - 4 years

Other milestones of a 4-year-old include: standing on one foot well, building steps
with cubes, drawing a man with two to four parts, counting to ten, able to undress,
sharing toys and beginning of social interaction. A useful mnemonic for
remembering the order of copying shapes is CCST: Circle (3 years), Cross (3
years), Square (4 years) and Triangle (5 years)

Scenario 2

Incorrect
Rides tricycle, washes hands, knows age and sex, imitates bridge of three cubes.

H Your answer

F Correct answer

F - 3 years

Other milestones of a 3-year-old include: briefly balancing on one foot, walking on

tiptoes, building tower of nine cubes, copying circle, knows name, speaking three to
four word sentences, helping with dressing, eating with knife and fork. It is easy to
remember some of the three-year-old milestones; such as riding a tricycle and
imitating a bridge with 3 cubes.

Scenario 3

Incorrect
Walks up and down stairs one step at a time, jumps with both feet, scribbles
spontaneously, can build tower of more than 4 cubes.

I Your answer

D Correct answer

D - 2 years

Other milestones of a 2-year-old include: running well, kicking ball, climbing on

furniture, building tower of six cubes, copying vertical line, using plurals, two to
three word sentences, feeding with fork and spoon. Children initially learn to climb
stairs one step at a time around 22 years. By around 3 they can walk upstairs one
foot per step, but come down with two. Between 45 they learn to descend with one
foot per step.

It is important to remember that whilst the age at which milestones are attained
differs between children, the order of skill development does not. Children with
severe developmental delay acquire milestones in exactly the same order as
everybody else, albeit at a different rate and with a different end-point.

Tetracycline
B Isoniazid
C Warfarin
D Sodium valproate
E Carbimazole
F Phenytoin
G Amiodarone
H Salbutamol via inhaler
I Haloperidol
J Chloramphenicol

Which of the above medications if ingested via breast milk will produce the
following adverse effects?

Tetracycline causes yellowish discoloration of the teeth. Isoniazid, an

antituberculous drug, is associated with an increased risk of convulsions and
neuropathies in the newborn. Chloramphenicol may cause bone marrow
toxicity in an infant. Grey baby syndrome, i.e. abdominal distension,
cyanosis and circulatory collapse is rare but can occur. Carbimazole appears
in breast milk, but breastfeeding is not contraindicated as long as low doses
are used and neonatal development is monitored. Warfarin, Sodium
valproate, Salbutamol, and Haloperidol can be used safely by breastfeeding
mothers.

Scenario 1

Correct
A 1-year-old baby boy with discoloration of the teeth

A Correct answer

Tetracycline
Scenario 2

Incorrect
A 4-day-old girl admitted for investigation following a convulsion

I Your answer

B Correct answer

Isoniazid

Scenario 3

Incorrect
A 6-week-old baby boy is brought to the emergency. He is hypotensive, tachypnoeic and
cyanosed. His abdomen is distended.

D Your answer

J Correct answer

Chloramphenicol

Theme: Treatment options

A Whey hydrolysate milk

B Sucrose solution
C Soya based infant feeds
D Herbal tea
E Intranasal Desmopressin
F Carbamazepine
G Lamotrigine
H Paracetamol
I Ibuprofen
J Clear fluids

For each of the following scenarios select the most likely beneficial
treatment option from the list above. Each option may be used once, more
than once, or not at all.
Scenario 1

Incorrect
A 9-year-old boy develops sudden episodes, only lasting a few seconds, of
unconsciousness. The EEG shows regular symmetrical three cycles a second generalised
spike and wave complexes.

F Your answer

G Correct answer

Lamotrigine

This child has typical absence seizures and valproate, ethosuximide and lamotrigine
are used to treat this.

Scenario 2

Correct
An 8-year-old boy is still wetting the bed at night and wants to go to a sleep over party.

E Correct answer

Intranasal Desmopressin

This boy is having nocturnal enuresis and as a temporary measure desmopressin

has been shown to be of benefit.

Scenario 3

Incorrect
A 3-month-old is crying at the same time as pulling up his legs for more than 3 hours.
This occurs for more than 3 days a week. All investigations have been normal.

D Your answer

A Correct answer

Whey hydrolysate milk

This is infantile-colic, a very common problem and Whey hydrolysate milk is likely
to be beneficial in such cases (clinical evidence, BMJ).

Neurocutaneous Syndromes

A Struge Weber Syndrome

B Neurofibromatosis 2
C Neurofibromatosis 1
D Tuberous Sclerosis
E Von Hippel Lindau
F Hypomelanosis of Ito

Scenario 1

Incorrect
A Child has facial naevus at birth on R side involving upper face and eyelid. At 8 months
she develops L sided focal tonic clonic convulsions, resistant to anticonvulsants.
Following this she is found to develop L sided hemiparesis and developmental regression
in 2nd year of life. CT Scan head was done and there was evidence of intracranial
calcification showing railtrack appearance.

0 Your answer

A Correct answer

Sturge Weber syndrome

Struge Weber: Charecterised by facial naevus(port wine stain),seizures, hemipareis,

Intracranial calcifications, developmental regression around 2nd year of life, mental
retardation and buphthalmos and glaucoma.

Not all children with facial naevus have Struge Weber Syndrome.

Scenario 2

Incorrect
4-year-old Harry has numerous caf-au-lait spots on his body. On slit lamp examination
of his eyes hamartomatous lesions consistent with Lisch nodules are found. His father
was diagnosed to have a similar condition.

0 Your answer
C Correct answer

Neurofibromatosis type 1

NF-1: Autosomal dominant.Called Von Recklinghausen Disease.

Any 2 of the following signs are present;

1. 6 or more caf-au-lait macules > 5mm in prepubertal and > 15 mm in

postpubertal children. These are the hallmark of NF1 and present in 100% of cases.
Present at birth, may increase in number and size

2. Axillary or inguinal freckling- Multiple hyperpigmented areas of 2-3mm diameter

3. 2 or more iris Lisch nodules- Hamartomas seen on slit lamp exam of eye. Its
prevelance increases with age. By 21 years up to 100% of them have them

4. A distinctive osseous lesion called sphenoid dysplasia, may cause pulsating

exophthalmos or cortical thinning of long bones

5. 2 or more neurofibroma or one plexiform neurofibroma

6. Optic Glioma

7. A 1st degree relative with NF 1 being diagnosed based on same criteria.

Risk of Developing hydrocephalus due to acqueductal stenosis

Developing malignant neoplasms like neurofibrosarcoma, Phaeochromocytoma,

Wilms and Optic glioma.

Scenario 3

Incorrect
14-year-old Jack has bilateral 8th nerve masses consistent with acoustic neuroma as seen
on MRI.

0 Your answer

B Correct answer

Neurofibromatosis type 2

NF-2:Bilateral acoustic neuromas- most distinctive feature of NF-2.

If a parent or sibling has NF2 and child has either unilateral acoustic neuroma or
Any 2 of the following- Neurofibroma, Meningioma, Glioma, Schwannoma or
Juvenile posterior subcapsular lenticular opacities diagnosis of NF2 can be made.

Scenario 4

Incorrect
8-month-old Charlotte has infantile spasms with hypsarrythmic EEG. On careful
examination she is found to have ash leaf like hypopigmented lesions on the skin. CT
Scan of the head shows tubers in the subependymal region.

0 Your answer

D Correct answer

Tuberous sclerosis

Tuberous Sclerosis: Autosomal dominant.

Clinical spectrum varies from severe mental retardation and seizures to normal
intelligence and no seizures. Characteristic brain lesions consists of tubers., they
may undergo calcification. They are present in subependymal region and project
into ventricular cavity causing hydrocephalus due to obstruction to CSF flow.

In infancy: Infantile spasms with Hypsarrythmic EEG, resistant to anticonvulsants.

May be responsive to Vigabatrin rather than ACTH.

Ash leaf macules may be seen on skin- they are hypopigmented lesions, enhanced by
Woods lamp examination.

Later in childhood: Child would be having generalised seizure disorder with

pathognomonic skin lesions Adenoma Sabaceum: Tiny red nodules over nose and
cheek resembling acne

Shagreen Patches: Roughened raised areas with an orange peel like consistency
present on back

Subungual or periungual fibromas develop in adolosence.

Retinal tumors cammon, brain tumors less common when compared to NF1.

Rhabdomyomas of heart are common

Von Hippel Lindau: Autosomal dominant. Involves multiple organs-
Hemangioblastoma of cerebellum, spinal cord, medulla, retina.

Cystic lesion of kidneys, pancreas, liver.

Phaeochromocytoma common.

Hypomelanosis of ito: Very rare, sporadic inheritance.

Hypopigmented areas on skin + CNS involvement in the form of seizures and

hemimegalancephaly.