Hepatic adenomata in type Ia glycogen storage disease

Liver adenomata are common in young adults and adults with type Ia glycogen storage disease. Complications that may arise in these patients include acute hemorrhage and malignant transformation. With appropriate dietary therapy, they may partially or completely regress. We describe a young woman with type Ia glycogenosis who developed liver adenomata. Because of its persistence and the significant potential of malignant transformation, liver transplantation was performed. This case is discussed in light of a review of the literature on the subject.