对1例具典型家族遗传史的葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症报告并家系分析如下。1病历摘要先证者Ⅳ1(图1),男,32个月,发病前进食加有豆瓣酱的菜肴,潜伏期8 h,1 d内入院。临床表现为尿呈浓茶色,明显血红蛋白尿,发热,呕吐,哭闹不安,抽搐,贫血貌,黄疸,肝肿大。实验室检查红细胞渗 A case report of glucose-6-phosphate dehydrogenase (G6PD) deficiency with a typical family history and pedigree analysis is as follows. 1 medical records probands IV1 (Figure 1), male, 32 months before eating onset with bean paste dishes, the incubation period of 8 h, 1 d admission. Clinical manifestations of urine was dark brown, significant hemoglobinuria, fever, vomiting, crying uneasiness, convulsions, anemia appearance, jaundice, hepatomegaly. Laboratory examination of erythrocyte infiltration