现把酸性麦芽糖酶缺陷分成婴儿、儿童和成年三型。以前,把婴儿型酸性麦芽糖酶缺陷看成是致命的。儿童和成年型酸性麦芽糖酶缺陷引起症状出现晚。本文作者报告三例儿童型酸性麦芽糖酶缺陷。病例Ⅰ:患者女性,15岁。因进行性肌无力,死于呼吸衰竭。病例Ⅱ:患者男性,4岁。因肌肉发育缓慢,于3岁时去就诊。患者足月顺产,出生时体重2.45公斤,4个月时,头抬不起来,7个月时能坐,到4岁时,患者尚不能站立和行走。患者的父母是堂 Acid maltase deficiency is now divided into three types of infants, children and adulthood. In the past, infant type maltase deficiency was considered fatal. Symptoms appear late in children and adults with maltase deficiency. The authors report three cases of childhood acid maltase deficiency. Case I: Female patient, 15 years old. Due to progressive muscle weakness, died of respiratory failure. Case II: Male patient, 4 years old. Due to the slow muscle development, at the age of 3 to see a doctor. The patient was delivered at full-term by birth and weighed 2.45 kg at birth. At 4 months, he could not lift his head and sat at 7 months. By the age of 4, the patient was still unable to stand and walk. Patient’s parents are hall