Glycogenic Hepatopathy: A Reversible Condition

Abstract

Purpose: A 19 year old boy with insulin dependent diabetes (DM), presented with 2 weeks of nausea, vomiting and right sided abdominal (abd) pain. He denied any recreational drug or alcohol use. His AST was 1531 U/L, ALT 1006 U/L, AKP 154 U/L, normal bilirubin and INR. CT scan showed hepatomegaly with a liver span of 23 cm (1). Doppler showed good flow in the vessels. Acetaminophen, ceruloplasmin, viral and autoimmune work up was negative. HgbA1C was 13%. With hydration, liver enzymes improved significantly leading to an initial diagnosis of ischemic hepatitis. He presented a week later with worsening abd pain. Transaminases were >700 U/L again. Liver biopsy showed benign liver tissue with scattered PAS positive glycogen inclusions in hepatocyte nuclei (2). He was diagnosed with GH and insulin drip started. Within a few days there was significant improvement and follow up imaging 6 months later showed normalization of liver size. GH or DM associated glycogen storage is an under recognized condition occurring in poorly controlled insulin dependent diabetics. It is a variant of Mauriac's syndrome, first described in 1930. The usual age of presentation is 8 to 25 years of age with nausea, vomiting and abd pain. The average HbA1C is 10 to 13%. Transaminases can be markedly elevated, mimicking an ischemic or viral etiology. Liver biopsy shows abundant glycogen in the hepatocyte nuclei with minimal or no steatosis which is in contrast to the fat accumulation seen in non alcoholic fattly liver disease. Treatment with intensive insulin therapy for 4-8 weeks usually leads to improvement in liver enzymes.Figure: No Caption available.Figure: No Caption available.