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Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus

Michael Torbenson1, Yunn-Yi Chen, Elizabeth Brunt

  • 1Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA. mtorben@jhmi.edu

Insights

Glycogenic hepatopathy, a liver glycogen overload, affects young patients with poorly controlled type I diabetes mellitus, causing enlarged livers and elevated liver enzymes. Improved glycemic control led to better liver enzyme levels in observed cases.

Area of Science:

  • Hepatology
  • Endocrinology
  • Pathology

Background:

  • Glycogenic hepatopathy is characterized by excessive glycogen accumulation in hepatocytes.
  • It is frequently associated with poorly controlled diabetes mellitus, particularly type I diabetes.

Purpose of the Study:

  • To report the clinical and pathologic features of glycogenic hepatopathy.
  • To differentiate glycogenic hepatopathy from other liver conditions like steatohepatitis.

Main Methods:

  • Analysis of 14 cases using various stains (H&E, trichrome, PAS, PAS-D).
  • Ultrastructural analysis in 2 cases.
  • Review of clinical records, laboratory findings, and outcomes.

Main Results:

  • Patients (8-25 years) had type I diabetes with poor glycemic control.
  • Clinical signs included hepatomegaly, abdominal pain, and elevated transaminases (up to 1600 IU/L).
  • Histology showed diffuse pale hepatocytes with excessive glycogen; steatosis and inflammation were minimal or absent.

Conclusions:

  • Glycogenic hepatopathy can cause significant hepatomegaly and transaminase elevations in type I diabetes.
  • The condition is histologically distinct from steatohepatitis.
  • Improved glycemic control can lead to normalization of liver enzyme levels.

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