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Hepatic adenomata in type Ia glycogen storage disease and liver transplantation: report of a case

✍ Scribed by C. Fontolliet; J.M. Matthieu; M. Gillet


Book ID
118568582
Publisher
Elsevier Science
Year
1998
Tongue
English
Weight
146 KB
Volume
28
Category
Article
ISSN
0168-8278

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Glycogen storage disease type Ia (GSD Ia) is a rare metabolic disorder due to hepatic glucose-6-phosphatase deficiency. Although great progress has been made in managing affected patients, severe hypoglycemia, lactic acidosis, hyperlipidemia, hepatic cytolysis, and impaired kidney function are frequ