肝糖元累积病又称Von Gierke氏病,为一种糖元代谢紊乱性疾病。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,血浆中磷酯增高,酮酸中毒为其特点。国内1939年始有报告。曾有报告两例发生于同一家族中。我科1979年6月遇见一家兄、弟、妹3例,报告如下: Liver glycogen accumulation disease, also known as Von Gierke’s disease, is a disorder of glycogen metabolism. 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 phosphorus esters, ketoacidosis is characterized. Domestic report began in 1939. Two cases have been reported in the same family. Our department met a brother, brother and sister in June 1979 and reported the following three cases: