肝糖元累积病为一种糖元代谢紊乱性疾病,又称Von Gierke氏病.本病多见于婴幼儿,以肝、肾肿大,生长发育不良,严重低血糖倾向,血浆中乳酸增多,酸中毒,血浆中磷脂增高,酮酸中毒为其特点.国内本病于1939年始有报告,两例发病与同一家族中也 Liver glycogen accumulation disease is a disorder of glycogen metabolism disorder, also known as Von Gierke’s disease. The disease more common in infants and young children with liver and kidney enlargement, poor growth and development, severe hypoglycemia, increased plasma lactic acid, Acidosis, increased plasma phospholipids, ketoacidosis is characterized by the domestic report of the disease began in 1939, the incidence of two cases with the same family also