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.

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“…Glycogenic hepatopathy is diagnosed by the presence of hepatomegaly and reversible glycogen accumulation in hepatocytes. Although their pathophysiology has not been fully explored, it is believed that adequate glycemic control could alleviate the observed histological abnormalities [ 31 ]. Our data confirmed the relationship between the EL-mediated glycemic control and the reduction in glycogen accumulation in the liver.…”

Hepatocellular Glycogen Accumulation in the Setting of Poorly Controlled Type 1 Diabetes Mellitus: Case Report and Review of the Literature

“…Follow-up repeat CT of the abdomen did not show a reduction in liver size which could be explained by the suboptimal glucose control, noncompliance with insulin regimen evident by HbA1c of 12.6%, and temporary loss to follow-up. e patient remains at risk for relapse due to inadequate glycemic control [9,37].…”

Glycogenic hepatopathy as a cause of severe deranged liver enzymes in a young patient with type 1 diabetes mellitus

“…However, the clinical or radiological discrimination between GH and NAFLD is not always easy, although recently imaging studies—particularly, the gradient dual-echo magnetic resonance imaging—have been proposed as effective tools for the distinction of glycogen deposition from the deposition of fat in the liver 37–40. Apart from diabetes mellitus, many other disorders have been infrequently associated with GH such as dumping syndrome after gastrectomy, anorexia nervosa, administration of high-dose corticosteroids, azathioprine and insulin overdose 41–43. However, these conditions were excluded easily according to the history of our patient.…”

“…Indeed, in patients with diabetes mellitus, hepatomegaly and increased levels of aminotransferase, the differential diagnosis is wide. Apart from GH, the differential diagnosis in young patients with T1DM should include celiac disease and autoimmune hepatitis while investigation for serological markers of viral hepatitis A, B, C and E seems also mandatory 15 41. Finally, haemochromatosis and Wilson’s disease should also be considered depending on the extent and pattern of liver dysfunction at presentation.…”