Raafat F, Morita M, Lau M, Taylor C M, White R H
Department of Histopathology, Children's Hospital, Birmingham, England.
Arch Pathol Lab Med. 1988 Jun;112(6):630-3.
Juvenile nephronophthisis is a major cause of progressive renal failure in children. Its manifestations are varied and protean. It is a multisystem syndrome, encompassing and overlapping related disorders. We treated a 4-year-old girl who presented with various manifestations of progressive renal failure characteristic of juvenile nephronophthisis, in addition to calcification of the basal ganglia and pancreatic lipomatosis, two associated conditions hitherto undescribed. It is hoped that further reports of other associated conditions may help to clarify further the nature of this complex syndrome.
青少年肾单位肾痨是儿童进行性肾衰竭的主要原因。其表现多样且变化多端。它是一种多系统综合征，涵盖并重叠相关疾病。我们治疗了一名4岁女孩，她除了基底节钙化和胰腺脂肪瘤病（这两种相关病症迄今尚未被描述）外，还出现了青少年肾单位肾痨特征性的进行性肾衰竭的各种表现。希望关于其他相关病症的进一步报告可能有助于进一步阐明这种复杂综合征的本质。
