Diagnostic Dilemma - Pediatric Oncall
Is it Wilson’s disease or Indian Childhood Cirrhosis_?
Author: Pediatric Oncall
Question
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
ICC most likely.
4 years ago
B
BHARAT PARMAR
Indian Childhood Cirrhosis
4 years ago
P
Pravin G Patel
On liver biopsy Mallory’s hyaline ,a sine qua non of ICC is widely recognised .There is also micro-nodular cirrosis, and copper positive on liver biopsy ,, we do not know how earliar wilson disese occurs but at 1 y 8 nonths we expect it to be in In the earlier stages of the disease when biopsy typically shows steatosis {deposition of fatty material}, increased glycogen in the nucleus ,Levels of ceruloplasmin are abnormally low in wilsons,
4 years ago
K
kusum kalla
indian chidhood cirrhosis
4 years ago
D
Dr.Ravi Bhatia
since liver biopsy was done what was the copper content in the liver tissue obtained for biopsy!

what was the slit lamp finding!

ICC is very rare

4 years ago
12
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