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Liver transplantation in patients with type IIIa glycogen storage disease, cirrhosis and hepatocellular carcinoma.
Revista Española de Enfermedades Digestivas 2018 October 16
Type III glycogen storage disease (GSD-III) is an autosomal recessive disorder due to the deficiency of the glycogen debrancher enzyme. 80% of the patients have hepatic and muscular involvement (IIIa), compared to 15% with only liver involvement (IIIb). As the life expectancy improves in these patients, the possible liver complications are better understood.
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