Fatal liver cirrhosis and esophageal variceal hemorrhage in a patient with type IIIa glycogen storage disease

Intern Med. 1998 Dec;37(12):1055-7. doi: 10.2169/internalmedicine.37.1055.

Abstract

A 45-year-old woman with type IIIa glycogen storage disease (GSD IIIa) died of variceal hemorrhage secondary to liver cirrhosis. The postmortem examination disclosed increased intracellular glycogen in the liver as well as in the heart and skeletal muscle. Although most liver injuries in GSD IIIa have been considered to be non-progressive in adulthood, liver cirrhosis can be a cause of death in some patients.

Publication types

  • Case Reports

MeSH terms

  • Diagnosis, Differential
  • Endoscopy, Digestive System
  • Esophageal and Gastric Varices / etiology*
  • Esophageal and Gastric Varices / metabolism
  • Esophageal and Gastric Varices / pathology
  • Fatal Outcome
  • Female
  • Follow-Up Studies
  • Gastrointestinal Hemorrhage / etiology*
  • Gastrointestinal Hemorrhage / metabolism
  • Gastrointestinal Hemorrhage / pathology
  • Glycogen Storage Disease Type III / complications*
  • Glycogen Storage Disease Type III / metabolism
  • Glycogen Storage Disease Type III / pathology
  • Humans
  • Liver Cirrhosis / complications*
  • Liver Cirrhosis / metabolism
  • Liver Cirrhosis / pathology
  • Liver Glycogen / metabolism*
  • Middle Aged
  • Recurrence

Substances

  • Liver Glycogen