3028 Glycogenic Hepatopathy Masquerading as Acute Relapsing Hepatitis: A Diagnostic Pitfall

Abstract

INTRODUCTION: Glycogenic hepatopathy (GH) is a rare conditioned characterized by elevated liver enzymes, hepatomegaly with or without right upper quadrant pain, typically seen in poorly controlled diabetes type 1, rarely in type II. It is related to glycogen accumulation during periods of high blood glucose, and tends to resolve with improvement of glycemic control. The course of liver enzyme abnormalities is transient. We present a case of glycogenic hepatopathy masquerading as biliary stones. CASE DESCRIPTION/METHODS: A 54 year old female with no known liver, history of HTN, CVA and poorly controlled type 2 diabetes mellitus (T2DM), complicated by multiple episodes of DKA and hyperosmolar hyperglycemic state, presented to the hospital with abdominal pain, nausea, constipation and high liver enzyme abnormalities. This was preceded by an admission 2 months prior with hyperglycemia above 600, ketones, and similar liver enzyme abnormalities. Work up was initiated including liver US, CT scan, MRCP, viral and autoimmune serologies which were normal or negative (Graph 1). Liver biopsy in progress. DISCUSSION: The patient in this case report has risk factors for metabolic syndrome with HTN, BMI of 26, T2DM with poor control, with episodes of hyperglycemia, associated with peaks of liver enzymes that mimic hepatocellular or mixed injury, which resolved slowly with control of hyperglycemia, being completely normal between spikes. This pattern of presentation is typical for GH, a metabolic entity that was linked to poorly controlled diabetes, and is caused by transient accumulation of glycogen within hepatocytes that follows passive influx of glucose into hepatocytes utilizing hepatic glucose transporter-2. Long-term prognosis is unclear and may in recurrent cases progress to fibrosis and cirrhosis, similar to NAFLD. Clinical suspicion for GH is based on correlation between peaks of blood sugar and acute rise in LFTs as well as periods of normalization of LFTs corresponding to resolution of acute hyperglycemia. Recognition of this entity is highly significant, as not only direct the effort to correct the underlying metabolic abnormalities, it also avoids unnecessary and costly work up knowing its transient and self-limited course. Liver biopsy if confirmatory. Glycogenic hepatopathy is an underdiagnosed metabolic hepatopathy in the setting poorly controlled diabetes, completely reversible, and should be considered in patients with T1 or T2DM with risk factors for NAFLD who present with acute and relapsing hepatitis.