A man with type III glycogenosis associated with cirrhosis and portal hypertension.
case_report · Level V
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- Record sourced from PubMed, PMID 8253364.
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Abstract
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.
Medical subject headings
- Glycogen Storage Disease Type III
- Hypertension, Portal
- Liver Cirrhosis