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

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-documented...

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Veröffentlicht in:	Gastroenterology (New York, N.Y. 1943) N.Y. 1943), 1993-12, Vol.105 (6), p.1882-1885
Hauptverfasser:	Markowitz, Arnold J., Chen, Yuan-Tsong, Muenzer, Joseph, Delbuono, Elizabeth A., Lucey, Michael R.
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Biological and medical sciences Carbohydrates (enzymatic deficiencies). Glycogenosis Errors of metabolism Glycogen Storage Disease Type III - complications Glycogen Storage Disease Type III - metabolism Glycogen Storage Disease Type III - pathology Humans Hypertension, Portal - etiology Liver - metabolism Liver - pathology Liver Cirrhosis - etiology Male Medical sciences Metabolic diseases Muscles - metabolism
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Zusammenfassung:	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-documented type IIIa glycogenosis, who presented with a variceal hemorrhage secondary to hepatic cirrhosis, is described here. No other cause of cirrhosis was found.
ISSN:	0016-5085 1528-0012
DOI:	10.1016/0016-5085(93)91088-Y