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Hepatocyte transplantation as a treatment for glycogen storage disease type 1a

โœ Scribed by Maurizio Muraca; Giorgio Gerunda; Daniele Neri; Maria-Teresa Vilei; Anna Granato; Paolo Feltracco; Muzio Meroni; Gianpiero Giron; Alberto B Burlina


Book ID
117279717
Publisher
The Lancet
Year
2002
Tongue
English
Weight
58 KB
Volume
359
Category
Article
ISSN
0140-6736

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Glycogen storage disease type 1b (GSD-1b) is due to an autosomal recessive inborn error of carbohydrate metabolism caused by defects in glucose-6-phosphatase translocase. Patients with GSD-1b have severe hypoglycemia with several clinical manifestations of hepatomegaly, obesity, a doll-like face, an