Hepatocellular adenoma in type Ia glycogen storage disease
โ Scribed by Masatoshi Kudo
- Publisher
- Springer Japan
- Year
- 2001
- Tongue
- English
- Weight
- 37 KB
- Volume
- 36
- Category
- Article
- ISSN
- 0944-1174
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๐ SIMILAR VOLUMES
Patients suffering from Type I glycogen storage disease frequently develop hepatic tumors. Some of these were classified as carcinoma, with the majority of tumors representing benign adenomata. However, no evidence exists of malignant transformation of adenomata in these patients. Here, we describe
Glycogen storage disease type Ia (GSD la, von Gierke disease) is an autosomal recessive inborn error of metabolism caused by the deficiency of ~-glucose-6-phosphatase (G6Pase). Since this enzyme is expressed primarily in hepatocytes, couples at risk for GSD type Ia relied on fetal liver biopsy for p