Diagnostic Dilemma

Acute liver injury with cardiac involvement


Author:
Question
A 5 years old boy presented with generalized edema, 2 episodes of vomiting and respiratory distress. He required ventilatory care and ionotropes in view of ejection fraction of 57 percent. He was detected to have elevated liver enzymes {SGOT = 6210 IU, L, SGPT of 5480 IU, L} with normal bilirubin and ammonia of 186 mg, dl. Initially PT, INR were deranged which gradually normalised. He had history of jaundice a year ago. He also had acidosis and low serum ceruloplasmin. His dengue, leptospira IgM were negative, HIV, HBsAg, Anti Hepatitis C were negative. Urine aminoacidogram, 24 hrs urine copper and ophthalmological examination were normal and USG abdomen showed coarse echotexture of liver.

What is the likely cause of his hepatitis_?
Expert Opinion :
This child has acidosis, hepatitis, Cardiac dysfunction, increased ammonia and previous jaundice. Suspicion of metabolic liver disease. Urine aminoacidogram normal. Serum Carnitine was found to be low. He is suspected to have fatty acid oxidation defect
Answer Discussion :
T
Tahir Ahmad
bubble
Urea cycle defect
8 years ago
R
rahiul Islam
bubble
drugs induced
8 years ago

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