Glycogenic hepatopathy: A narrative review

Abstract

Glycogenic hepatopathy (GH) is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction with elevated liver enzymes and associated hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes. It is predominantly seen in patients with longstanding type 1 diabetes mellitus and rarely reported in association with type 2 diabetes mellitus. Although it was first observed in the pediatric population, since then, it has been reported in adolescents and adults with or without ketoacidosis. The association of GH with hyperglycemia in diabetes has not been well established. One of the essential elements in the pathophysiology of development of GH is the wide fluctuation in both glucose and insulin levels. GH and non-alcoholic fatty liver disease (NAFLD) are clinically indistinguishable, and latter is more prevalent in diabetic patients and can progress to advanced liver disease and cirrhosis. Gradient dual-echo MRI can distinguish GH from NAFLD; however, GH can reliably be diagnosed only by liver biopsy. Adequate glycemic control can result in complete remission of clinical, laboratory and histological abnormalities. There has been a recent report of varying degree of liver fibrosis identified in patients with GH. Future studies are required to understand the biochemical defects underlying GH, noninvasive, rapid diagnostic tests for GH, and to assess the consequence of the fibrosis identified as severe fibrosis may progress to cirrhosis. Awareness of this entity in the medical community including specialists is low. Here we briefly reviewed the English literature on pathogenesis involved, recent progress in the evaluation, differential diagnosis, and management.

Keywords: Glycogenic hepatopathy; Diabetes mellitus; Hepatomegaly; Mauriac syndrome; Elevated liver enzymes; Liver biopsy; Gradient dual-echo MRI

Core tip: Glycogenic hepatopathy (GH) is considered as a benign reversible condition. Elevation in transaminases is a common finding in patients with diabetes mellitus, and non-alcoholic fatty liver disease (NAFLD) and GH are the two primary underlying pathologies in most cases. It is essential to distinguish NAFLD from GH as this can progress to advanced liver disease. However, recent studies have identified the varying degree of fibrosis in glycogen hepatopathy as well, further emphasizing the need for future studies. We briefly reviewed the literature on mechanisms involved in the development of GH, evaluation of these patients, recent progress made on diagnostic tests and management.