Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus

Abstract

Glycogenic hepatopathy (GH) is an uncommon cause of serum transaminase elevation in type I diabetes mellitus (DM). The clinical signs and symptoms of GH are nonspecific, and include abdominal discomfort, mild hepatomegaly, and transaminase elevation. In this report we describe three cases of patients presenting serum transaminase elevation and hepatomegaly with a history of poorly controlled type I DM. All of the cases showed sudden elevation of transaminase to more than 30 times the upper normal range (like in acute hepatitis) followed by sustained fluctuation (like in relapsing hepatitis). However, the patients did not show any symptom or sign of acute hepatitis. We therefore performed a liver biopsy to confirm the cause of liver enzyme elevation, which revealed GH. Clinicians should be aware of GH so as to prevent diagnostic delay and misdiagnosis, and have sufficient insight into GH; this will be aided by the present report of three cases along with a literature review.

Keywords: Glycogen hepatopathy; Transaminase; Type I diabetes mellitus.

Figure 1

Image findings. Abdominal ultrasonography showed mildly increased hepatic parenchymal echogenicity and mild hepatomegaly in case 1, which led to an initial suspicion of nonalcoholic fatty liver disease (A, B). The findings for cases 2 and 3 were similar.

Figure 2

Histological findings for case 1. Hematoxylin and eosin staining showed a preserved portal and hepatic lobular architecture without significant inflammation, fibrosis, or cholestasis. The hepatocytes were diffusely swollen with a pale cytoplasm (arrowhead). Numerous hepatocytes exhibited glycogenated nuclei (×200) (A). Strong positivity for periodic acid-Schiff (PAS) stain (arrow) suggested cytoplasmic glycogen deposits (×200) (B). The findings for cases 2 and 3 were similar.

Figure 3

Changes in serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), and HbA1c in cases 1 (A), 2 (B), and 3 (C). In all cases the status of glycemic control did not show a definitive correlation with the changes in liver enzyme levels; the latter showed various fluctuations in each case.