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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
Abstract:
Reported are the clinical and pathologic features of glycogenic hepatopathy, a pathologic overloading of hepatocytes with glycogen that is associated with poorly controlled diabetes mellitus. Fourteen cases were studied by stains, including hematoxylin and eosin, trichrome, periodic acid-Schiff, and periodic acid-Schiff with diastase. Ultrastructural analysis was performed in 2 cases. Medical records were reviewed for clinical presentations, laboratory findings, and clinical outcomes. The individuals ranged from 8 to 25 years of age. All had type I diabetes mellitus with poor glycemic control. The clinical presentations included hepatomegaly, abdominal pain, and elevated transaminases (range, 50-1600 IU/L). The transaminases were dramatically elevated in 3 cases to greater than 10 times the upper limit of normal. All biopsies showed diffusely pale staining hepatocytes on hematoxylin and eosin stains, with excessive glycogen accumulation demonstrated by periodic acid-Schiff stains. Ultrastructural examination revealed marked glycogen accumulation in the cytoplasm and nuclei. Most cases showed no evidence for fatty liver disease: steatosis was absent in 12 of 14 cases, simple steatosis was seen in 1 of 14 cases, and mild steatohepatitis was present in 1 of 14 cases. Mallory hyaline was absent in all cases, acidophil bodies were only rarely seen, and inflammation was absent or minimally present. Fibrosis was typically absent, with only 2 cases demonstrating focal mild fibrosis. Three patients had adequate follow-up and demonstrated improvement of liver enzyme levels with control of blood glucose. We conclude that glycogenic hepatopathy can cause hepatomegaly and significant transaminase elevations in individuals with type I diabetes mellitus. The pathology is distinct from steatohepatitis.
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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