Living donor liver transplantation for glycogen storage disease type Ib
نویسندگان
چکیده
منابع مشابه
Outcome of living donor liver transplantation for glycogen storage disease.
GLYCOGEN storage diseases (GSD) are inherited disorders in which the amount and/or structure of glycogen in body tissues are abnormal. GSD I (von Gierke disease) is caused by a deficiency of glucose 6-phosphatase activity in the liver, kidney, and intestinal mucosa with glycogen overloading in these organs. The clinical manifestations are seizures, systemic acidosis, hyperlipidemia, hyperuricem...
متن کاملLiver transplantation for type I glycogen storage disease.
STUDIES of haptoglobin, 1-3 group-specific component,3,4 and numerous other products of hepatic synthesis59 have shown that liver homografts permanently retain their original metabolic specificity after transplantation. Consequently, liver transplantation has been regarded for some years4 as a potentially decisive way to treat those inborn errors of metabolism which result partly or completely ...
متن کاملLiver transplantation for glycogen storage disease type Ia.
BACKGROUND/AIMS Hepatocellular carcinoma (HCC) most often occurs within hepatocellular adenomas (HCAs) in glycogen storage disease Ia (GSD Ia) patients. The objective of this retrospective study is to assess outcomes after liver transplantation (LT) for GSD Ia where the principal indication for transplantation was prevention of HCC. METHODS Petitions to the United Network for Organ Sharing re...
متن کاملLiver transplantation for type IV glycogen storage disease.
T YPE IV glycogen storage disease is a rare autosomal recessive disorder (also called Andersen's diseasel or amylopectinosis) in which the activity of branching enzyme alpha-I, 4-g1ucan: alpha-I, 4-g1ucan 6-g1ucosyltransferase is deficient in the liver as well as in cultured skin fibroblasts and other tissues.2,3 This branching enzyme is responsible for creating branch points in the normal glyc...
متن کاملLiver transplantation in glycogen storage disease type I
Glycogen storage disease type I (GSDI), an inborn error of carbohydrate metabolism, is caused by defects in the glucose-6-transporter/glucose-6-phosphatase complex, which is essential in glucose homeostasis. Two types exist, GSDIa and GSDIb, each caused by different defects in the complex. GSDIa is characterized by fasting intolerance and subsequent metabolic derangements. In addition to these ...
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ژورنال
عنوان ژورنال: Liver Transplantation
سال: 2009
ISSN: 1527-6465
DOI: 10.1002/lt.21929