2. I.C.C
• Is a disease
Found in Indian Subcontinent & West Indies.
Peculiar to Indian Infants & Children.
• ICC is a progressive disease with
abdominal distension,
marked irritability,
unexplained irregular low grade fever
Hepatospleenomegaly with hepatic failure, ascites &
jaundice.
• Usually occurs between 6 months to 4 years of age.
3. Incidence
• Male: female::4 : 1
• First born at greater risk.
• Low- middle class of rural areas with veg food
habits.
• Declined incidence r/t declining practice of
use of copper/brass utensils for boiling milk.
• There is familial predisposition of the disease.
4. ETIOLOGY
• MULTIFACTORIAL/NO SINGLE FACTOR
• Hepatotoxic agents
• Guttis/aspergillus flavus that grow on
groundnuts,maize & rice; cause-effect-
unknown.
• Copper vessels use- boiling milk, cooking food.
• Early weaning with milk supplement before
age of 3 months
6. PATHOLOGY
Due to disease process
• Variation in size of the liver
• Colour ranges from gray to frank green
• Diffuse liver damage by degeneration
• Necrosis & replacement fibrosis
• Capsule shows patchy thickening & surface is
finely nodular.
7. MICROSCOPIC STUDY
• Marked hepatocyte damage as degenerative
changes of cytoplasm.
• Kuppfers cell- show mild degree of
proliferation.
• Hepatic lobules- gross pericellular fibrosis.
• Intracellular hyaline called mallory’s hyaline
seen.
• There is gross excess of copper & copper
associated protein - ORCEIN
8. CLINICAL MANIFESTATIONS
• ONSET: insidious/acute
• INSIDIOUS ONSET-3 arbitrary stages- which
tend to merge each other
Early stage
Intermediate stage
Terminal stage
10. INTERMEDIATE STAGE
• Progressive growth failure inspite of adequate diet;
Looks very ill.
Within few months to years:
Hepatomegaly- firm with sharp leafy well defined
marginal edges.
Jaundice
Prominent abdomen; features of portal HTN incl
spleenomegaly, ascites, hematemesis, anemia
Prominent superficial abdominal veins
Thrombocytopenia, intravascular hemolysis
Edema of ankles, puffiness of face ( S. Albumin)
12. Acute onset
• Sudden appearance of jaundice, fever, clay
coloured stools & hepatomegaly.
• Rapid development of hepatic coma- fatal
outcome
13. diagnosis
• Liver biopsy (C.I- prolonged PTT)
• CUPRIURESIS test- performed by oral
administration of D- penicillamine: using
urinary copper/creatinine ratio as the index
parameter.
14. management
• Few case improve spontaneously & survive
without specific treatment.
• D- PENICILLAMINE THERAPY- used as copper
chelating agent from liver- improve survival.
• Immunomodulating agent:
levamisole/cortocosteroids/gamma globulins.
• Symptomatic treatment: for infection; vitamin
& mineral deficiency
15. • Anemia: haematincs
• Bleeding : Vit K
• DIURETICS
Edema: spironolactone
Ascites: frusemide ( with oral potassium
supplement)
16. Supportive care
• Rest
• Balanced Diet with good quality proteins
• I.V glucose drip
• O2 therapy
• Antibiotics (neomycin)
• In compensated/intermediate stage- edema, ascites:
salt restriction, protein supplements with additional
glucose.
Detect early: hepatic/pre coma- for prompt management.
17. • Exchane transfusion: to remove circulating
toxins.
• SENGSTAKEN TUBE- to control esophageal
bleeding-Portal HTN causing hematemesis.
• PORTOCAVAL ANASTOMOSIS- to relieve portal
HTN & control of hypersplenism.
18. Prevention
• sibling screening
• EBF esp known history.
• Avoid boiling milk in copper/copper alloy pots.
• Avoid boiling/storing infant feeds in
copper/copper alloy pots.
• Increase public awareness about preventive
measures: lowering of copper intake- copper
rich food, water & utensils
