S2596 Glycogenic Hepatopathy: A Rare Cause of Reversible Severe Hepatitis

Abstract

Introduction: Glycogenic hepatopathy (GH) is a rare reversible complication of poorly controlled diabetes mellitus. Its presentation can range from mild to severe hepatitis and diagnosis can be challenging. Here we present a case of GH presenting as severe hepatitis. Case Description/Methods: A 51-year-old male with a history of hypertension and poorly uncontrolled T1DM presented with two days of intractable nausea and vomiting. Physical examination revealed cachectic male only oriented to name. Abdominal exam was normal without tenderness to palpation or organomegaly. Laboratory results were significant for bicarbonate < 5.0 mmol/L, glucose 1169 mg/dL, total bilirubin (T bili.) 0.4 mg/dL, alkaline phosphatase (ALKP) 141 U/L, alanine aminotransferase (ALT) 161 U/L, aspartate aminotransferase (AST) 107 U/L, albumin 3.6 g/dL, INR 1.0, hemoglobin A1c 13.7%. Head computed tomography showed no acute intracranial pathology. He was admitted to the intensive care unit, diagnosed with DKA and treated with insulin infusion therapy. On day 4, laboratory results showed T bili. 0.4 mg/dL, ALKP 295 U/L, ALT 1570 U/L, AST 5250 U/L, INR 1.0. An ultrasound showed mild hepatomegaly 14cm, hyperechoic liver parenchyma, no intra or extra hepatic biliary dilation, and patent hepatic and portal veins. Workup of viral hepatitis A, B and C, autoimmune hepatitis, Wilson’s disease, alpha 1 antitrypsin deficiency, and hemochromatosis was negative. An ultrasound guided liver biopsy showed diffuse swollen hepatocytes, minimal focal fibrosis and mild inflammation composed of scattered lymphocytes, plasma cells and few polymorphonuclear leukocytes. Periodic acid-Schiff (PAS) stain with and without diastase highlighted abundant cytoplasmic glycogen. Patient was diagnosed with GH and a rapid downtrend in liver enzymes was noted with DKA treatment. Discussion: GH was first described by Peter Mauriac in 1930 in children with uncontrolled T1DM. Its incidence is unknown as there have been less than 100 cases reported so far in English literature. Pathophysiology of GH remains unclear but rapid fluctuations in glucose and insulin leading to excessive glycogen deposition in hepatocytes has been suggested. Distinguishing GH from NASH, DILI and other chronic liver disease is challenging without a liver biopsy. With proper glycemic control, GH is quickly reversible and has a good prognosis. No long term hepatic complications are known. GH remains underdiagnosed due to a combination of diagnostic challenges and lack of awareness amongst specialists.Figure 1.: Histopathological section of the liver showing: A) Hepatocytes with clear and fine granular cytoplasm under hematoxylin and eosin stain, B) PAS stain showing large amount of dark red-colored glycogen in hepatocytes, C) Complete digestion of glycogen after adding diastase stain and reappearance of clear hepatocytes.