Sunday, 22 December 2013

INDIAN CHILDHOOD CIRRHOSIS

  • 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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