Beckwith J B
Department of Pathology and Human Anatomy, Loma Linda University, California 92350, USA.
Am J Med Genet. 1998 Oct 2;79(4):268-73. doi: 10.1002/(sici)1096-8628(19981002)79:4<268::aid-ajmg7>3.0.co;2-i.
Nephrogenic rests (NR) are abnormally persistent clusters of embryonal cells, representing microscopic malformations (dysplasias) of the developing kidney. Though NR are best known as precursors of Wilms tumor (WT), many alternative fates are observed, and most rests are destined for eventual atresia. Biological and clinical distinctions between the two major NR categories, perilobar and intralobar rests (PLNR and ILNR) are emphasized. PLNR occur in fetal overgrowth and with certain overgrowth syndromes. ILNR are frequently associated with deletions or mutations of WT1. Data are presented concerning the prevalence of NR in general pediatric autopsy populations, and in selected syndromes. The age at diagnosis of WT was determined for the largest series of patients with WT-associated syndromes reported to date. These data provide a basis for determining how long patients with these conditions are at risk for WT development.
肾源性残留（NR）是胚胎细胞异常持续存在的团块，代表发育中的肾脏的微观畸形（发育异常）。尽管NR最为人所知的是肾母细胞瘤（WT）的前体，但也观察到许多其他转归，并且大多数残留最终会闭锁。文中强调了两种主要的NR类型，即叶周型和叶内型残留（PLNR和ILNR）之间的生物学和临床差异。PLNR发生于胎儿过度生长以及某些过度生长综合征中。ILNR常与WT1的缺失或突变相关。文中给出了关于NR在一般儿科尸检人群以及特定综合征中的患病率的数据。确定了迄今为止报道的最大系列WT相关综合征患者的WT诊断年龄。这些数据为确定患有这些疾病的患者发生WT的风险期提供了依据。
