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Home > West Medicine > Pediatrics > Pediatric Internal Med

Classroom classification

百科

Glycogen storage disease type I in children

儿科综合

Common symptoms

酮症酸中毒、凝血功能障碍、对称性肌无力、生长缓慢、血脂异常、肝肿大、乳酸蓄积过多

The pathology of disease

Glycogen storage disease type I in children (von Gierke) is glucose-6-phosphatase deficiency; severe hypoglycemia; lactic acidosis; hyperlipidemia; children; cornstarch; pediatric metabolic/genetics.

Symptoms

Severe hypoglycemia; lactic acidosis; hyperlipidemia; child; hepatomegaly; cornstarch

Causes

G6PC mutation; AR; von Gierke; GSD I; child.

Treatment

Cornstarch; frequent feeds; avoid fasting; allopurinol; pediatric metabolic/genetics; liver transplant selected

Dept

Pediatrics / metabolic / medical genetics / pediatric gastroenterology.

Warm reminder: Health education reference only; seek care at regular hospital.

Family room

儿科综合Overview of the Western Medical Room