患者男,2岁。病史祖母代述:患儿生后所用尿布。衣物尿湿后,逐渐变为黑色,似白布被火烤焦样,洗之不退色。尿盆中的尿久放也变为黑色。这种现象逐渐明显,不伴其它不适。于1975年来门诊就诊疑为黑酸尿症予以门诊检查及随访至今。患儿为头胎,足月顺产,产程顺利,弟妹父 Male patient, 2 years old. History of the grandmother on behalf of: diapers used after birth. After the clothing is wetted, it gradually turns black, like a white cloth is baked with fire like, wash it without fading. Urine urine in the long put also become black. This phenomenon is obvious, not with other discomfort. Outpatient clinics in 1975 suspected black aciduria to be out-patient examination and follow-up so far. Children with first-born, term full-term, labor smoothly, brother and sister father