Ann Pediatr Endocrinol Metab.  2014 Mar;19(1):49-52. 10.6065/apem.2014.19.1.49.

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

• Jeong HR
• Shim YS
• Kim YB
• Lee HS
• Hwang JS

• KMID: 1702665
• DOI: http://doi.org/10.6065/apem.2014.19.1.49

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.

Keyword

Glycogenic hepaptopathy; Type 1 diabetes mellitus; Hepatomegaly

MeSH Terms

Abdominal Pain
Adolescent
Blood Glucose
Diabetes Mellitus, Type 1*
Female
Glycogen*
Hepatomegaly
Humans
Liver
Prognosis
Blood Glucose
Glycogen

Figure

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  Fig. 1 Abdominal computed tomography revealed hepatomegaly and marked liver attenuation.

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  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).

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