Interesting Case of the Month - (IGHP, ICOM) - April
april
Clinical History-
8-year-old child with asymptomatic hepatomegaly. No prior history of jaundice or sibling death. Full term normal vaginally delivered.
Examination: liver 5 cm palpable BCM, firm, smooth surface. No splenomegaly. No ascites.
LFT: total bill 0.8 / 0.4 mg/ dl SGOT 243 IU/ l SGPT 154 IU/ L SAP: 348 IU/ L
Eyes: Cherry red spot. Liver biopsy was performed for evaluation.
From the images below identify the likely disease?
Microscopic Images-
Histology showing foamy vacuolated cells within the sinusoids
Special Stains-
MT stain showed presence of thin pericellular fibrosis. The vacuolated cells are PAS negative.
Glycogen Storage Disease
Tyrosinemia
PFIC Type 2
Niemann Pick Disease
