A man with type III glycogenosis associated with cirrhosis and portal hypertension.

Journal Article (Journal Article)

Type III glycogenosis, an inherited disorder of glycogen metabolism that results from reduced or absent activity of the enzyme amylo-1,6-glycosidase (debranching enzyme), has not been frequently associated with cirrhosis and portal hypertension in adults. An adult Caucasian man with well-document type IIIa glycogenosis, who presented with a variceal hemorrhage secondary to hepatic cirrhosis, is described here. No other cause of cirrhosis was found.

Full Text

Duke Authors

Cited Authors

  • Markowitz, AJ; Chen, YT; Muenzer, J; Delbuono, EA; Lucey, MR

Published Date

  • December 1993

Published In

Volume / Issue

  • 105 / 6

Start / End Page

  • 1882 - 1885

PubMed ID

  • 8253364

International Standard Serial Number (ISSN)

  • 0016-5085

Digital Object Identifier (DOI)

  • 10.1016/0016-5085(93)91088-y


  • eng

Conference Location

  • United States