【摘 要】
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Glycogen storage disease type I (GSD type I), also known as von Gierke disease, is an autosomal recessive disorder of the glycogen metabolism pathway, caused by a deficiency of glucose- 6-phosphatase (GSD type Ia) or glucose-6-phosphate translocase (GSD t
【机 构】
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Department of Radiology,Centro Hospitalar Universitário do Porto EPE,Porto 4099-001,Portugal;Departm
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Glycogen storage disease type I (GSD type I), also known as von Gierke disease, is an autosomal recessive disorder of the glycogen metabolism pathway, caused by a deficiency of glucose- 6-phosphatase (GSD type Ia) or glucose-6-phosphate translocase (GSD type Ib) [1] . These enzymes are crucial in the last step of both glycogenolysis and gluconeogenesis, and their deficiency re- sults in excessive glycogen and fat accumulation in the liver, kid- ney and intestinal mucosa [1] .
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