本文报告的一例,具有病程长,充分发展的骨关节病变,伴发一侧双肾盂双输尿管畸形,中西结合治疗有一定收效等特点。复习文献,扼要地探讨了尿黑酸尿症—褐黄病的病因,病理,生化等方面的异常表现、临床表现、诊断及治疗。并简述人类对这一罕见疾病的认识史作一回顾。 This article reports an example of a long duration, full development of bone and joint disease, with bilateral ureteral double ureteral malformations, the combination of Chinese and Western treatment has some effect and so on. Review the literature, briefly discussed the urine black aciduria - brown yellow disease etiology, pathology, biochemistry and other aspects of the abnormal performance, clinical manifestations, diagnosis and treatment. And a brief review of human history of this rare disease to make a review.