PRACTICE TEACHING

ON
CHILDHOOD LIVER
CIRRHOSIS

SUBJECT:
NURSING
EDUCATION

SUBMITTED TO SUBMITTED BY
MADAM POULOMI CHOWDHURY SANGITA DEB
PROFESSOR ROLL NO – 03
CHARNOCK COLLEGE OF NURSING MSc NURSING FIRST YEAR
NEWTOWN, KOLKATA CHARNOCK COLLEGE OF NURSING
Introduction:
Cirrhosis occurs at the end stage of many chronic liver diseases, including biliary atresia (BA) and
chronic hepatitis. Cirrhosis can also result from infectious, autoimmune or toxic factors and from
chronic diseases such as haemophilia and cystic fibrosis. A cirrhotic liver is irreversibly damaged.
Childhood liver cirrhosis or Indian childhood cirrhosis is a chronic liver disease of infants and young
children usually 6 months to 4 years of age unique to the Indian subcontinent. Affected children
present with jaundice, pruritis, lethargy and hepatosplenomegaly with rapid progress of cirrhosis.
Cirrhosis occurs at the end stage of many chronic liver diseases, including biliary atresia and chronic
hepatitis.

Definition:
The WHO has defined cirrhosis as a diffuse liver process characterised by fibrosis and conversion of
normal liver architecture into abnormal nodules, consequent to diffuse hepatocyte injury.
Indian childhood cirrhosis is an autoimmune disorder characterized by fever, abdominal distention
and hepatosplenomegaly and most often seen in Indian children between the age of 6 months to 4
years.

Anatomy and physiology of liver:

Liver is the largest gland in the body. It is reddish
brown in colour, highly vascular and weighs
about 1.5 kg in adults. It occupies the right
hypochondrium and extends into the epigastrium
and left hypochondrium. It is wedge shaped. It
has an apex, base or right lateral surface and
anterior, superior and inferior surfaces.
Anatomically, liver has two lobes, a large right
lobe and a smaller left lobe. They are separated from each other by the line of attachment of the
falciform ligament, the fissure for ligamentum venosum and by the groove for ligamentum teres.
Caudate lobe is chiefly situated on the posterior surface of the right lobe.

Organs associated with liver:

- Superiorly and anteriorly: diaphragm and anterior abdominal wall.
- Inferiorly: stomach, bile ducts, duodenum, hepatic flexure of the colon, right kidney and adrenal
gland.
- Posteriorly: Oesophagus, inferior vena cava, aorta, gall bladder, vertebral column and diaphragm.
- Laterally: lower ribs and diaphragm.
Blood supply:
Liver gets blood from two sources:
- Hepatic artery- a branch of celiac trunk. It
carries oxygenated blood. It divides into right
and left branches.
- Portal vein- carries products of digestion from
the alimentary canal. It is formed by the union
of superior mesenteric vein and splenic vein.

Functions of Liver:
The liver performs numerous functions. Some of these are as follows:
 The liver as an exocrine gland for the secretion of bile.
 The liver plays a prominent role in metabolism of carbohydrates, proteins and fats. Metabolic
functions include synthesis of plasma proteins- fibrinogen and prothrombin and the regulation of
blood glucose and lipids.
 The liver acts as a storehouse for various substances including glucose, lipids, vitamins and iron.
When necessary, the liver can convert lipids and amino acids into glucose.
 The liver plays a protective role by detoxifying substances. Removal of bile pigments from blood
is part of this process.
 During fetal life, the liver is a center for hemopoiesis

metabolism of
proteins, fats and storage of glycogen,
carbohydrates aminoacids and
destruction of old vitamin B12 , A and D
RBCs

Synthesis of
glucose by
gluconeogenesis
hemopoeisis in fetus Functions
of liver

defense of body by secretion of bile

kupffer cells
excretion of
cholesterol, and bile
pigments
 Fig: Functions of liver

Incidence:
The disease is prevalent all over India in some particular communities. Male children are 4 times
more sufferer than female. The first-born child is at greater risk. Large number of cases belongs to
the lower middle-class family of rural areas with vegetarian food habits. Childhood liver cirrhosis or
Indian childhood cirrhosis first described by Sen in 1887 from Calcutta. The remarkable decline in
ICC incidence appears to be related to declining practice of employing copper/brass utensils for
boiling milk.

Etiopathogenesis:
It is an autoimmune disorder. Indian childhood cirrhosis continues to be a disease of obscure
etiology. The following factors may predispose to the illness:
1. Toxic (copper intoxication):
- Studies reported that there is evidence of excess of copper binding proteins in the liver of
patient with ICC.
- If the baby is weaned earlier and if the milk supplements were added to the breast milk often
before 3 months of age.
- Use of copper or copper alloy pots for boiling milk and cooking food. During boiling milk,
copper is released from the copper pots and this is probably absorbed in excess from the gut
by infant.

2. Viral infection of the liver:

- It is a consequence of neonatal or infective hepatitis.

3. Immunological factors:
 - A variety of immunological disturbances were reported in patients with Indian childhood
cirrhosis. High levels of circulating immune complexes may indicate that an environmental
insult might alter the hepatocyte or tissue proteins and initiate an immune mediated injury to
the liver.

4. Nutritional factors:
- It is believed that malnutrition is an important cause of cirrhosis.

5. Hepatotoxic agents: some hepatotoxic agents like aflatoxin, produced by Aspergillus flavus that
grows on ground nuts, maize and rice can predispose to this disease.

6. Familial history of the disease : a definite family predisposition is the hallmark of ICC. An
increased prevalence of peptic ulcer, asthma, diabetes and migraine in the pedigrees affected by
ICC has been observed.

7. Metabolic factors: a child with inborn error of tryptophan metabolism, aminoaciduria

aminoacidemia, disturbed lactose, zinc, copper and magnesium metabolism can predispose to the
disease. So, a child at risk should be put on low tryptophan diet.

Pathophysiology:
Etiologic factors

Marked damage of the hepatocytes

Complete disorganization of liver architecture, that is size of liver varies, and its colour ranges from
grey tan to frank green

Formation of micronodules in the surface of the liver. But the portal vein and the biliary passages of
the patient, the lymphatic appear normal

Regeneration nodules in the liver are encircled by the banks of fibrous tissue

Absence of regenerative activity and manifesting degenerative changes in the liver

Necrosis and fibrosis of the hepatic lobules

Due to etiological factors like copper intoxication, viral infection of the liver, immunological factors,
malnutrition and family history of the disease marked damage of the hepatocytes occur. The liver
architecture completely disorganize, size of the liver varies and its colour ranges from grey tan to
frank green. Micronodules will form in the surface of the liver. But the portal vein and the biliary
passages of the patient with the lymphatic appear normal. Regeneration of nodules in the liver are
encircled by the banks of fibrous tissue. Absence of regenerative activity occur and manifestation of
degenerative changes in the liver started. Necrosis and fibrosis of the hepatic lobules starts.

Clinical Features:
1. Insidious onset: In this, the disease will last for 6 months to 3 years. Symptoms are grouped under
two headings- pre cirrhotic symptoms and cirrhotic symptoms.
Pre cirrhotic symptoms:
- Irritability
- Disturbed appetite
- Disturbed appetite
- Chalky, pasty stools and distension of abdomen
- Often slight irregular fever

Cirrhotic symptoms:
(stage I)
- Slight fever
- Liver is enlarged to 3-5 cm, edges become sharp and giving an appearance of leafy border.
- Children exhibits jaundice.
- Poor growth
- Anorexia
- Constipation or diarrhoea
- Clay coloured stools
- Growth failure
(stage II)
- Diffuse hepatomegaly
- Splenomegaly
- Ascites
- Esophageal varices
- Hematemesis
- Anemia
- Muscle weakness
- Lethargy
- GI bleeding
(stage III)
- Terminal stage of illness
- Restlessness
- Confusion
- Dyspnoea and cyanosis on exertion
- Evidences of hepatocellular failure in the form of Palmer erythema and spider nevi
appearance on the upper torso.
- A peculiar garlic odour is present in patients with impending liver cell failure.
- Enlarged and hard spleen.
- Terminally, there is jaundice and hepatic coma and is often associated with GI bleeding.
- Child may die at this stage either from hepatic failure or inter current infections.
2. Acute onset:
Sudden onset of disease and sometimes child becomes symptomatic for a variable period and then
shows the manifestations of insidious onset.
- Sudden onset of fever
- Jaundice
- Clay-coloured stools
- Hepatomegaly
- Child may die with hepatic coma

Diagnostic Evaluation:
 History collection and complete physical examination.
 Liver can be palpable, very firm in consistency and its borders will be sharp. On auscultation
hepatic bruit in severe cases.
 In case of ascites, fluid thrill test can be done.
 Liver function test shows elevated ALT, GGT.
 Prothrombin time, clotting time and bleeding time should be assessed.
 Prolonged prothrombin time.
 Liver biopsy- can help to find out the sclerosis of liver.
 Cup uresis- testing the presence of copper in urine after administration of d-penicillamine.

Management:
If the disease condition can diagnose in early stage, it can be treated.
Initial stage:
Diet: Adequate diet with enough of good quality proteins, vitamins and minerals is desirable.
Antibiotics for infection or infestations: D-penicillamine 20-40 mg/kg per day for 12-18 months.
Symptomatic treatment will be done.
Immune modulators such as levamisole can be used. Corticosteroids and gamma globulins are also
helpful.
IV fluids can be administered in case of dehydration.
Strict aseptic technique need to be maintained for prevention of infection.
Terminal stage:
If the patient has entered the precoma or coma stage protein intake should be reduced, administration
of neomycin by gavage and 20% IV glucose drip are helpful. Exchange transfusion will be helpful to
remove the circulating toxins with that oxygen administration should be done when necessary.
Surgical management:
1. Liver transplantation in End stage liver disease.
Liver transplantation: Liver transplantation is a surgical procedure used to replace a diseased or failing
liver with a healthy one from a donor. It’s often considered for conditions that have caused severe liver
damage and when other treatments haven’t been effective.

Donor types:
 Deceased Donor: The liver comes from someone who has died. The process involves matching the
donor’s liver with the recipient based on factors like blood type and size.

 Living Donor: A portion of the liver is taken from a living person, usually a relative or close
friend, and transplanted into the recipient. The liver has the ability to regenerate, so both the donor's
and recipient's livers can grow back to near normal size.
Procedure: The surgery is performed under general anaesthesia and usually lasts several hours. The
diseased liver is removed, and the donor liver is then implanted. The procedure involves connecting
blood vessels and bile ducts.
Recovery: Post-surgery recovery typically involves a stay in the hospital, followed by a period of
rehabilitation. Patients need to take immunosuppressive medications to prevent rejection of the new
liver and may require regular follow-up appointments to monitor liver function and overall health.
Risks and Complications: As with any major surgery, there are risks involved, including infection,
bleeding, and organ rejection. Long-term risks include complications from the immunosuppressive
drugs, such as increased susceptibility to infections and potential damage to other organs.
2. Sengstaken- Blakemore tube may help to control the esophageal bleeding in case of portal
hypertension and hematemesis.
Sengstaken-Blakemore tube: The Sengstaken-Blakemore tube is a medical device used primarily to
manage and treat upper gastrointestinal bleeding, particularly from esophageal variceal haemorrhage,
which is a serious complication of liver cirrhosis and portal hypertension.
Components and Function
Tube Structure: The Sengstaken-Blakemore tube is a long, flexible tube with multiple lumens. It
typically has:

o A balloon for esophageal compression: This balloon is inflated to apply pressure to the
oesophagus and control bleeding from esophageal varices.
o A balloon for gastric compression: This balloon is inflated to apply pressure to the stomach,
helping to manage bleeding from the gastric varices or to stabilize the tube.
o An aspiration lumen: This allows for the removal of gastric contents, which can help reduce the
risk of aspiration and facilitate a clearer view of the oesophagus and stomach.

Insertion and Use

1. Insertion: The Sengstaken-Blakemore tube is inserted through the patient’s nose or mouth and
advanced into the oesophagus and stomach. Proper placement is confirmed with imaging or
endoscopy.
2. Balloon Inflation: Once in place, the balloons are inflated to exert pressure on the bleeding
vessels. This action can help control bleeding temporarily until more definitive treatments (like
endoscopic therapy or surgery) can be arranged.
3. Monitoring and Care: Continuous monitoring is essential. The tube must be carefully managed
to prevent complications such as esophageal or gastric necrosis due to prolonged pressure or
improper inflation.

Complications

 Esophageal Perforation: The pressure from the balloon can cause damage to the esophageal
wall.
 Aspiration: If the tube is not properly positioned, there is a risk of aspiration of stomach contents
into the lungs.
 Pressure Necrosis: Prolonged pressure can lead to necrosis of the esophageal or gastric tissue.
 Tube Displacement: The tube may need to be repositioned or replaced if it shifts or becomes
dislodged.

3. Portocaval anastomosis may be done to relieve the portal hypertension and complications of
hypersplenism.
Portocaval anastomosis: Portocaval anastomosis is a surgical procedure used to create a connection
between the portal vein and the inferior vena cava. This procedure is typically performed to manage
complications arising from portal hypertension, a condition often associated with liver cirrhosis.
Purpose

 Manage Portal Hypertension: Portal hypertension occurs when there is increased blood
pressure in the portal vein, which can lead to complications such as esophageal variceal bleeding,
ascites, and splenomegaly. Portocaval anastomosis helps to reduce this pressure by diverting
blood flow from the portal vein to the systemic circulation.
Procedure

1. Surgical Approach: The procedure is performed through an abdominal incision. The surgeon
accesses the portal vein and the inferior vena cava.
2. Creating the Anastomosis: The portal vein is connected to the inferior vena cava to create a new
pathway for blood flow. This reduces the pressure in the portal vein by allowing blood to bypass
the liver and flow directly into the systemic circulation.
3. Types of Portocaval Anastomosis:
o End-to-Side Anastomosis: The end of the portal vein is connected to the side of the inferior
vena cava.
o Side-to-Side Anastomosis: A section of the portal vein and the inferior vena cava are
connected side-to-side.

Benefits

 Reduction in Portal Pressure: By diverting blood flow from the portal vein, the pressure in the
portal system is reduced, which can alleviate symptoms associated with portal hypertension.
 Prevention of Complications: It helps prevent or manage complications such as variceal
bleeding and ascites.

Risks and Complications

 Postoperative Bleeding: As with any major surgery, there is a risk of bleeding.

 Infection: There is a risk of infection at the surgical site.
 Hepatic Encephalopathy: Reduced blood flow through the liver may lead to the accumulation
of toxins in the blood, potentially causing confusion or altered mental status.
 Portal Thrombosis: There is a risk of blood clot formation in the portal vein.

Postoperative Care

 Monitoring: Patients are closely monitored for signs of complications such as bleeding,
infection, or hepatic encephalopathy.
 Medications: They may be prescribed medications to manage symptoms and prevent
complications, including antibiotics and medications to reduce the risk of blood clots.
 Follow-up: Regular follow-up visits are necessary to assess liver function and ensure the
anastomosis is functioning properly.

Nursing Management:
Nursing diagnosis:
- Hyperthermia related to the inflammatory process in the liver
- Ineffective breathing pattern related to pressure on diaphragm secondary to ascites
- Imbalanced nutrition less than body requirement related to anorexia
- Diarrhoea or constipation related to acute abdominal condition
- Parenteral anxiety related to management of the disease condition
Nursing Interventions:
- Provide symptomatic management
- Adequate rest
- Semi-fowlers position
- Daily abdominal girth measurement
- IV fluids as per order
- Small and frequent diet
- Protein rich foods and massive doses of vitamin B6
- Aseptic precautions
- Input and output charts
Parental education:
- Disease condition and management
- Avoid food rich in copper like drynuts, chocolates etc.
- Breastfeeding and weaning at 6 months
- Milk should not be boiled and stored in copper or copper alloy pots
- Reduce use of brass and copper vessels
- Use aluminium and steel utensil
- Food rich in tryptophan should be reduced. E.g.: milk, eggs, meat, nuts, beans, fish and
cheese.
- Provide more vitamin B6 foods such as potato, banana, spinach, soya bean, fruits and
vegetables.

Strategy of preventing ICC through lowering of the copper intake:

Source of high dietary copper Action plan to lower it

 Brass and copper vessels for transportation,  Use of aluminium and steel for tin-coating
storage and boiling of milk. on brass
 Copper and brass pots for storing drinking  Change over to earthenware or steel and
water. aluminium
 Food cooked and stored in brass and copper  Encourage use of aluminium and steel
utensils
 Introduction of animal milk before 2 to 3  Promotion of breast feeding
months of age
 Copper content of drinking water under 0.1  Demineralize water
mg/L.
 Foods rich in copper content (liver, nuts,  Avoid them or minimize their consumption
chocolates)
Related Research:

A retrospective study was done on Indian childhood cirrhosis by Surbhi Goyal, Akanksha Singh et.al.
on October, 2023.
The aim of this retrospective study was to emphasises the need of awareness for clinicopathological
attributes of Indian childhood cirrhosis in order to enable timely diagnosis and management. This
study was done on liver archival tissue from January 2016 to December 2022. Five children in
infancy to middle childhood presented with features of chronic liver disease in the form of jaundice
and abdominal distention, were included in the study. Characteristic firm hepatomegaly with sharp
margins and transaminitis was noted in all cases. Autoimmune, viral and metabolic workup were
negative in all these patients except one which showed positive autoimmunity and another whose
Coomb's test was positive. The histological features of marked ballooning degeneration with diffuse
Mallory Denk, pericellular fibrosis, absence of steatosis and pan lobular copper deposits clinched the
diagnosis of ICC. ICC once believed to be extinct has still not vanished and remains underdiagnosed
in routine practice. It is a rapidly fatal disease with a debatable pattern of inheritance and
controversial role of copper as etiological agent. The clinical presentation is often deceptive and lack
of awareness leads to misdiagnosis.

Conclusion:

Childhood liver cirrhosis is a complex and challenging condition. Timely identification of liver
cirrhosis through regular screening, particularly in at-risk populations, is crucial for improving
prognosis. Advances in diagnostic technologies and biomarkers play a critical role in early detection.
That requires a comprehensive and multidisciplinary approach for effective management and
prevention. A tailored treatment plan based on the underlying cause of cirrhosis whether it be viral
hepatitis, metabolic disorders, or autoimmune diseases is essential. This may include medical
therapies, nutritional support, and, in severe cases, liver transplantation. Prevention strategies, such
as vaccination programs (e.g., hepatitis B), public health education, and improved prenatal and
postnatal care, are fundamental in reducing the incidence of cirrhosis. Comprehensive care should
encompass not only medical treatment but also psychological and social support for affected children
and their families. Addressing the emotional and practical challenges associated with chronic illness
is vital. By focusing on these areas, healthcare systems can enhance the quality of life for children
with liver cirrhosis, reduce the disease burden, and improve overall outcomes for affected
individuals.
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