- incidence decreasing in India ,Sri lanka
- affects 1-3 yrs age group
- >1 children in family affected
- growth retardation
- ascites
- liver-- enlarged, firm, sharp edges, fine granular surface, grainy leathery appearance, mallory hyaline
- cells are strongly+ive for Copper on orcein stain
- excess copper intake from utensils responsible
- may be ass. with genetic defect also
- fine creeping fibrosis---- pericellular fibrosis
- no nodule formation
- scanty inflammatory infilterate
- Cu chiefly concentrated in lysosomes as CuSO4 aggregates
- also scattered Cu in cytoplasm
- orcein/rhodamine staining +++ ive
- ceruloplasmin NOT decreased
- serum Cu, urinary Cu,hair Cu ------ increased
what are sources of Cu in ICC?
- lack of maternal Breast feeding
- top feeds--animal milk
- brass / Cu utensils
- genetic----- often ass. with viral hepatitis
Treatment
- D-penicillamine
- Prednisolone
- i.m. Gamma globulin
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