Hepatic Sarcodosis presenting as Portal hypertension in a young boy
Gastroenterology and Hepatology from Bed to Bench,
A 13-year-old boy, known case renal stone disease came with the complaints of abdominal pain along with low grade fever. On examination, hepatosplenomegaly was noted while his lab reports showed a low hemoglobulin with a raised ESR. His blood and urine cultures showed no growth. Viral markers, autoimmune profile, C and p ANCA were all negative apart from a raised serum IgG level. Ultrasound abdomen showed a hyperechoic liver with an enlarged spleen along with splenic varices and minimum ascites. Ultrasound hepatic doppler was normal. Serum AFP levels were normal while workup for Wilson’s disease was negative. Fibroscan showed F4 fibosis. CT scan abdomen showed an enlarged left lobe of the liver along with an enlarged spleen. His EGD revealed varices. So liver biopsy was done that was suggestive of chronic granulomatous disease with ZN stain testing negative for TB.PPD, urine for AFB were both negative. Serum ACE levels were raised. He started ATT therapy but his condition did not improve. So, on the suspicion of hepatic sarcoidosis, he started on steroids and had a drastic improvement in his condition.
Keywords: Hepatic sarcoidosis; chronic granulomatous disease; steroids; Serum ACE levels.
(Please cite as: Achakzai IK, Majid Z, Khalid MA, Khan SA, Laeeq SM, Luck NH. Hepatic Sarcodosis presenting as portal hypertension in a young boy. Gastroenterol Hepatol Bed Bench 2018;11(1):220-228).
- Hepatic sarcoidosis
- chronic granulomatous disease
- Serum ACE levels
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