Indian childhood cirrhosis is an autoimmune disorder peculiar to Indian children most often seen between ages 6 months to 4 years. It is a chronic degenerative state of the liver leading to fibrosis and nodular regeneration. Symptoms vary from mild fever and enlarged liver to jaundice, ascites, and hepatic failure. Treatment focuses on preventing infection, managing symptoms, and in late stages liver transplantation may be required.

2. KING GEORGE’S MEDICAL UNIVERSITY,
LUCKNOW
K.G.M.U. COLLEGE OF NURSING
TOPIC-INDIAN CHILDHOOD CIRRHOSIS

3. CONTENTS
 Introduction
 Definition
 Causes
 Pathophysiology
 Clinical Features
 Diagnostic Evaluation
 Medical & Surgical Management
 Nursing Management

5. Term cirrhosis of liver introduced by Laennec in 1826 . Originated from the
Greek term "scirrhus" and is used to describe the orange or tawny surface.
When we talk about Indian Childhood Cirrhosis ,it is an auto immune
disorder peculiar to Indian children is most often seen in children between
the age of 6 months to 4 years.
INTRODUCTION

6. DEFINITION
Cirrhosis of liver is chronic degenerative state
of liver which leads to development of band of
fibrous tissue, firm nodules and scarring of
liver ,ultimately leading to irreversible cell
damage.

7.  Copper Intoxication
 Viral Infections
 Genetic
CAUSES

8. PATHOPHYSIOLOGY
Due to etiological factors
Marked damage of the hepatocytes
Complete disorganization of liver architecture, i.e., size of liver varies,
and its color change.
Formation of macronodules and micronodules in the surface of the liver, while the
portal vein and the biliary passages are patent, the lymphatics appear normal.

9. Absence of regenerative activity and manifesting
degenerative changes in the liver.
Necrosis and fibrosis of the hepatic lobules
Indian childhood cirrhosis
Regenerating nodules in the liver are encircled by the
bands of fibrous tissue

10. CLINICAL FEATURES
Insidious Onset s: The disease will
last for 6 months to 3 years.
Symptoms are grouped in 2 headings.
The pre - cirrhotic symptoms:
• Irritability and disturbed appetite
• Chalky, pasty stools
• Distension of abdomen

11. • Constipation
• Diarrhoea
• Often slight irregular fever

12. The cirrhotic symptoms: Cirrhotic symptoms
are grouped under 3 stages.
Stage I:
• Slight fever, enlarged liver to 3-5 cm with
sharp edges , poor growth , anorexia,
constipation/diarrhoea and jaundice, clay
colored stools, and growth failure.

13. Stage II:
• Diffuse hepatomegaly , splenomegaly ,
ascites , esophageal varices, hemoptysis ,
anemia , muscle weakness , lethargy and
GI bleeding.

14. Stage III:
 It is the terminal stage of the
disease featuring restlessness,
confusion, dyspnea and cyanosis
on exertion, evidences of
hepatocellular failure in the
form of palmar erythema

15.  Spider nevi appearance on the upper
torso a peculiar garlic odor in
patients with impending liver cell
failure, enlarged and hard spleen
and terminally, there is jaundice and
hepatic coma and is often associated
with gastrointestinal bleeding.

16. • Child may die at this stage either from hepatic failure or recurrent
infections.
Acute Onset
It has a sudden onset of disease and sometimes child becomes
symptomatic for a variable period and then shows the manifestations
of insidious onset. The symptoms include-

17. Sudden onset of fever
Jaundice with clay- colored stools.
Hepatomegaly
Death with hepatic coma

18. History collection
Physical examination : Liver can be
palpable, very firm in consistency and its
boarders will be sharp . On auscultation ,
hepatic bruit is heard in severe cases , if
there is ascites, fluid thrill test can be done.
DIAGNOSTIC EVALUATION

19. Liver function Test : Increased
Alanine Transaminase (ALT),
Increased Gama Glutaryl
Transpeptidase (GGT) Prothrombin
time , clotting time ,and bleeding time
will be prolonged.

20. Liver biopsy: To find out the
sclerosis of liver. It is a reliable
method of arriving at a fool proof
of diagnosis.

21.  Cupriuresis: Testing the presence of copper in
urine after administration of d-penicillamine .

22. TREATMENT
Medical Management
ICC was dubbed as a "frustrating
situation" as there was no specific
treatment available. If the diagnosis is
made on an early stage (before the
development of jaundice and ascites), ICC
is potentially treated.

23. The drug of choice is d-pencillamine
(which chelate copper) in a dose of 20-40
mg/kg/day for 12-18 months, leads to
marked improvement and even total
reversal in the histopathologic picture.

24. Initial Stage
Adequate diet with enough of good
quality proteins, vitamins and minerals is
desirable.
Antibiotics should be given to treat the
intercurrent infections/infestations.

25. Symptomatic treatment should be given.
Immunomodulators such as levamisole can
be used.
Corticosteroids and gammaglobulins are
also helpful.
Administer IV fluids if there is dehydration.
Prevention of infection: Follow aseptic
techniques and prophylactic antibiotics .

26. Terminal Stage
If the patient has entered precoma or coma, the
protein intake should be reduced.
Administration of neomycin by gavage and 20%
IV glucose drip are helpful .
 Oxygen can be administered, if necessary.
 Exchange transfusion to remove the circulating
toxins.

27. Surgical Management
 No special surgical correction The only
successful treatment for end stage liver
disease is liver tansplantation . If there is
portal hypertension with hematemesis ,
sengestaken tube to control esophageal
bleeding .

28.  A portocaval anastamosis may be done to relieve the portal
hypertension and complications of hypersplenism .

29. Nursing Management
Nursing Diagnosis
Hyperthermia related to inflammatory process in the liver.
Impaired breathing pattern related to pressure on diaphragm
secondary to ascites.
Impaired liver function related to damage of hepatic cells

30. Diarrhoea or constipation related to acute abdominal condition .
Delayed growth and development related to chronic illness .
Parental anxiety related to management of the disease.
Nursing Interventions
Follow aseptic techniques to prevent infection.
Intake and output chart should be maintained.

31. Health Education
Explain the cause, symptoms, and management of the disease.
Avoid food rich in copper like dry nuts, chocolates
liver, etc.
Provide small and frequent diet to the child.
Advise the mother to breast feed their baby for longer
period and not to introduce food supplements beyond
the age of 6 months.

32. Milk used for infant should not be boiled and stored in copper and copper
alloy pots.
Reduce the use of brass and copper vessels.
Use aluminium and steel utensils.
Foods rich in tryptophan (milk, eggs, meat, nuts, beans, fish, and cheese)
should be reduced.
Provide more vitamin B, rich foods like potato, banana, spinach, soya bean,
fruits and vegetables (vitamin B help to convert tryptophan to niacin).

33. Cirrhosis of liver is a chronic, degenerative state
of liver, which leads to development of bands of
fibrous tissue, firm nodules and connections
between the central and portal areas of liver.
Cirrhosis is peculiar to Indian children. Indian
childhood cirrhosis is an auto immune disorder
is most often seen in Indian children between the
age of 6 months to 4 years.
SUMMARY

34. ASSIGNMENT
 Prepare an assignment on Liver transplantation .
It is manifested by slight fever, enlarged liver to 3-5 cm,
with sharp edges and giving an appearance of leafy
boarder, jaundice etc. Antibiotics , good quality protein
, minerals ,vitamins etc are very much helpful in
providing relief in case of liver cirrhosis .In case of 3rd
stage liver cirrhosis liver transplantation is suggested.

35. RECAPITULATION
 What is Indian Childhood cirrhosis ?
 What are the factors that cause liver cirrhosis ?
 Enlist the symptoms of stage – I ?
 Listdown the clinical features of stage –II .
 What treatment modalities can be used for the
patient with liver cirrhosis ?

36. REFERENCES
• Pal Panchali. Textbook of pediatric nursing. Edition 1.
Paras Medical Publishers, 2016 p.313-315.
• Sharma Rimple. Essentials of pediatric nursing. Edition 2.
Paras Medical Publishers, Delhi: Jaypee Publication, 2017
p. 329-331
• Datta Parul. Pediatric Nursing . Edition 4. Jaypee
Publication.p. 315-317