尿黑酸尿症(Alkaptonuria)是一种较少见的先天性氨基酸代谢障碍疾病,作者发现1例,现报道如下.患儿女,18个月.于1990年2月25日来院就诊。患儿出生第2天起出现酱油色尿,诊断为“新生儿自然出血症”,入院治疗3天,治愈出院。出院后有间歇性黑尿.尿稍久置渐变红褐色,最后变成黑 Alkaptonuria is a rare congenital disease of amino acid metabolism disorders, the authors found in 1 case, are reported as follows. Children, 18 months, came to hospital on February 25, 1990. Soy sauce color urine appeared on the 2nd day after birth, diagnosed as “natural bleeding in newborn”, admitted to hospital for 3 days and was discharged. Discharged intermittent black urine after discharge. Urine slightly gradient pale red brown, eventually turning black