Is it Wilson’s disease or Indian Childhood Cirrhosis_?

 
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A 1 year 8 months old boy born of non consanguineous marriage presented with abdominal distension for a month and jaundice for 15 days. On examination, he had hepatosplenomegaly with firm hepatomegaly and a leafy border. There were no other signs of liver cell failure. Investigations showed:
• Bilirubin = 0.6 mg, dl, SGOT = 1235 IU, L, SGPT = 235 IU, L, GGTP = 640 IU, L,
Albumin = 3 gm, dl, Alkaline phosphatase = 607 IU, L.
• HBsAg, HIV, Hepatitis C – Negative
• ANA, Anti LKM, Anti smooth muscle antibody = Normal
• Plasma aminoacidogram, urine aminoacidogram = Normal
• USG Abdomen = hepatosplenomegaly. No portal hypertension.
• Ophthalmological examination = Normal
• Liver biopsy = micronodular cirrhosis with mallory hyaline disease and copper stain positive suggestive of Indian childhood cirrhosis.
• Serum copper = 243 µg, dl {Normal = 80–190}
• S. ceruloplasmin = 378 mg, dl {Normal = 30-65}
• 24 hours urine copper = 269.82
• Bone marrow = No storage cells.

Expert Opinion :
Mallory bodies are seen in 73 percent Indian childhood cirrhosis and in 25 percent Wilson`s disease. Thus they are not specific for ICC. On electron microscopy, a variety of peculiar degenerative changes at the level of organelles,
in particular, the mitochondria have been observed in Wilson`s disease. In addition, the presence of lipofuscin granules have been described in the hepatocytes as a characteristic. However, they can also occur in ICC. Thus there is no specific histological finding that can differentiate between the two.

In this child, there was history of drinking milk heated in copper utensils. Thus with age of cirrhosis and this history, it was most likely to be ICC.
feature.
Answer Discussion :
M
mehernosh asli
bubble
ICC most likely.
5 years ago
B
BHARAT PARMAR
bubble
Indian Childhood Cirrhosis
5 years ago

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