Question of the Week

Question :
Posted On : 21 Sep 2008
An 8 year old male child, resident of Aligarh presented to us with recurrent fever for the past 3 years,3 episodes of jaundice in the last 3 years each lasting for 15 days and swelling in abdomen for past 3 days.No history of bleeding from any site, blood transfusion, urinary or bowel, neurological complaint. On examination he had severe pallor, icterus with no signs of liver failure other than ascites. Liver span was 8 cm, spleen was 9 cm palpable and ascitis present. Hemogram revealed pancytopenia,LFT and PT, PTTK raised, S.Albumin decreased.Patient was given blood transfusion and FFP. LFT and Coagulaion profile became normal.VIRAL Hepatitis serology was negative. USG abdomen revealed normal liver, splenic collaterals at hilumand ascitis. Upper GI Endoscopy was normal and KF Ring negative. WHAT IS THE LIKELY DIAGNOSIS?
4
Expert Answer :
No expert answer available.
Answer Discussion :
M
mohd elsayed mohd mohd
Profile
ifamilial meditrinian fever
16 years ago
M
mohd elsayed mohd mohd
Profile
buddchiari
16 years ago
P
pediatriconcall
Profile
Rule out autoimmune liver disease. Also which viral markers have been done? Have you ruled out EBV and CMV? What is the pancytopenia due to ? Is it due to hypersplenism or bone amrrow suppression? If due to bone marrow suppression, a bone marrow exam will also be useful. Once the acute liver cell failure resolves, a liver biopsy will be useful.
16 years ago
E
eugene ngono
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HEREDITARY SPHEROCYTOSIS
16 years ago




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