Bodaghi E, Honarmand M T, Ahmadi M
Children's Hospital Medical Center (Ahari Hospital), Teheran University, Iran.
Int J Pediatr Nephrol. 1987 Oct-Dec;8(4):207-10.
Juvenile nephronophthisis is a common cause of end stage renal failure in children and adolescents. The early occurrence with rapid progression to advanced renal failure has not previously been described. In this report we present three babies in whom failure to thrive, dehydration and a renal concentrating defect presented soon after birth; they were subsequently investigated for renal failure during the first year of life. In two cases the association with congenital hepatic fibrosis was noted. A family history of nephronophthisis was not present but the parents were consanguineous and one sibling had died in infancy.
青少年肾单位肾痨是儿童和青少年终末期肾衰竭的常见病因。早期发病并迅速进展至晚期肾衰竭的情况此前未见报道。在本报告中，我们介绍了三名婴儿，他们出生后不久即出现生长发育迟缓、脱水和肾浓缩功能缺陷；随后在出生后第一年内对其进行了肾衰竭检查。在两例中发现了与先天性肝纤维化的关联。虽然没有肾单位肾痨的家族史，但父母为近亲结婚，且有一个兄弟姐妹在婴儿期死亡。
