Diagnostic Dilemma - Pediatric Oncall
Is it Wilson’s disease or Indian Childhood Cirrhosis_?
Author: Pediatric Oncall
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.

Answers of this discussion
Author :- Pravin G Patel on 25 August 2012.
Answer :- It is more likely ICC Age is less than 5 Years.wilson above 5 years eye is normalin ICC, In wilson cataract or kf rings seen S. serulospasmin is N OR H IN icc, iT IS lOW IN WILSON L.biopsy is already suggestive of ICC

Author :- Dr.Ravi Bhatia on 26 August 2012.
Answer :- 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

Author :- kusum kalla on 26 August 2012.
Answer :- indian chidhood cirrhosis

Author :- Pediatric Oncall on 30 August 2012.
Answer :- ICC is now obselete in India. Is is still possible that this is ICC_? How young an age can wilsons disease present_?

Author :- Pravin G Patel on 30 August 2012.
Answer :- 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,

Author :- BHARAT PARMAR on 31 August 2012.
Answer :- Indian Childhood Cirrhosis

Author :- Pediatric Oncall on 03 September 2012.
Answer :- The earliest Wilsons` {communicated orally to us} is about 2 years of age. Thus, it would be really unusual to be Wilsons disease. If not wilsons is this ICC_? Would electron microscopy help in this child_?

Author :- mehernosh asli on 04 September 2012.
Answer :- ICC most likely.

Author :- Pediatric Oncall on 04 September 2012.
Answer :- 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.

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