糖原累积病是先天性糖原代谢所需酶的缺乏而造成的糖原代谢紊乱。根据其主要受累器官及其临床特点分为:肝、心、肌肉3大类型,其中以心型最为罕见。现将笔者遇见1例报告如下。患儿,女,5个月,因咳嗽、气喘、唇周发绀4天,发热2天,于1984年3月12日入院。入院前,曾在本厂卫生所用青霉素、止咳糖浆等治疗无效,且咳喘加剧伴发热。患儿出生时无青紫现象,生后混合喂养,生长良好。其姐生后36天,因气促、咳喘在上海某医院诊断为“先心”、“肺炎”,治疗无效死亡。体检:神清,精神差,呼吸急促,鼻搧,面色苍白,唇周发绀。心前区未见明显隆起,心浊音界明显向两侧扩大,以左侧为主,心音低纯,心率(?)16次,律齐,未闻及杂音。两肺均可闻及哮鸣音及湿性啰音。腹平软,肝在肋缘下可触及1cm,质软。辅助检查:Hb11g,WBC 6,500,中性46%,淋巴54%。心电图:T 波在Ⅰ、Ⅱ、Ⅲ、aVF、V_3、V_5低平、双向、倒置。ST 段在Ⅱ、Ⅲ、aVF、 Glycogen storage disease is congenital glycogen metabolism required for the lack of enzymes caused by glycogen metabolism disorders. According to its main affected organs and their clinical characteristics are divided into: liver, heart and muscle 3 major types, of which the most heart-shaped. Now I met one case reported as follows. Children, women, 5 months, due to cough, asthma, cyanosis lips 4 days, fever 2 days, on March 12, 1984 admission. Before admission, once in our factory use penicillin, cough syrup and other treatment is invalid, and cough and asthma exacerbated with fever. Children born without cyanosis, mixed feeding after birth, good growth. His sister 36 days after birth, due to shortness of breath, cough and asthma in Shanghai, a hospital diagnosed as “heart”, “pneumonia”, the treatment died. Physical examination: God clear, poor spirit, shortness of breath, nose fan, pale, cyanosis lip weeks. No obvious uplift precordial area, heart voiced sound bound to both sides of the expansion to the left, heart sound low purity, heart rate (?) 16 times, law Qi, did not smell and noise. Both lungs can smell wheezing and wet rales. Abdomen soft, liver palpable under the ribs 1cm, soft. Assisted examination: Hb11g, WBC 6,500, 46% neutral, lymph 54%. ECG: T wave in Ⅰ, Ⅱ, Ⅲ, aVF, V_3, V_5 low flat, bidirectional, inverted. ST segment in Ⅱ, Ⅲ, aVF,