Pediatric glycogen storage disease type IV

Glycogen storage disease (GSD) is a class of disorders of glycogen metabolism caused by congenital enzyme defects. Glycogen storage disease type Ⅳ (glycogen storage disease type Ⅳ), also known as amylopectinosis (Amylopectinosis), Andersen disease (Andersens disease), is caused by the deficiency of starch 1,4-1,6 transglucosidase. Patients with abnormal glycogen storage in the liver, kidney, spleen, muscles and nervous system, especially in liver cells, the most accumulation, the most serious damage to the liver. Examination of the branching enzymes of the liver, leukocytes and fibroblasts can confirm the diagnosis. There is no special treatment for this disease, only symptomatic treatment. Clinical manifestations are normal.