Glycogenic hepatopathy in a Korean girl with poorly controlled type 1 diabetes mellitus

Abstract

Glycogenic hepatopathy (GH) is a rare complication of type 1 diabetes mellitus. We report the case of a 13-year-old diabetic female with poorly controlled blood sugar levels who presented with abdominal pain and distention 1 month in duration. She exhibited tender hepatomegaly, an elevated lipid profile, and elevated serum transaminase levels. Her liver histology was consistent with GH. The pathophysiology and/or underlying genetic background of GH remains unclear. The optimum treatment for GH is optimal glycemic control, and the prognosis is favorable. Clinicians should be aware of the possibility of GH and observe the clinical response to optimal glycemic control prior to invasive investigation.

Keywords: Glycogenic hepaptopathy; Hepatomegaly; Type 1 diabetes mellitus.

Fig. 1

Abdominal computed tomography revealed hepatomegaly and marked liver attenuation.

Fig. 2

(A) Intact lobular architecture; swollen hepatocytes exhibited pyknotic nuclei and intranuclear glycogen granules (X200). (B) Periodic acid-Schiff staining revealed abundant accumulation of cytoplasmic glycogen (×400). (C) Diastase removed all glycogen (×400).