Congenital Anomalies of the Kidney and Urinary Tract: A Clinical Review.
Congenital Anomalies of the Kidney and Urinary Tract: A Clinical Review.
复制标题
DOI：
10.1007/s40746-019-00166-3
复制
发表时间：
2019-01-01
影响因子：
--
通讯作者：
Spencer, John David
中科院分区：
文献类型：
--
作者：
Stonebrook, Emily;Hoff, Monica;Spencer, John David
审查目的：这篇综述强调了在儿科实践中遇到的最常见的先天性肾脏和泌尿道异常（CAKUT）。CAKUT是产前诊断发育畸形的最常见原因，包括一系列影响下尿路发育以及肾脏发育和功能的疾病。在儿童和青少年人群中，发育异常是终末期肾病的主要原因。本次审查的目标是提供儿科供应商的框架，适当的临床管理，以及强调转诊时subspecialty care是need.Recent发现：虽然确切的病因CAKUT没有完全定义，新的证据表明，遗传和分子的变化影响胚胎肾脏和尿路发育。因此，表型和临床结局可能会受到影响。摘要：由于儿科提供者提供一线护理的儿童和青少年发育性肾脏和尿路异常，CAKUT发病机制，胚胎学，临床管理和患者的结局的更新知识是必要的。这篇文章回顾了CAKUT的病因学和基本的诊断、预后和管理策略。
PURPOSE OF REVIEW: This review highlights the most common congenital anomalies of the kidney and urinary tract (CAKUT) that are encountered in pediatric practices. CAKUT are the most common cause of prenatally diagnosed developmental malformations and encompass a spectrum of disorders impacting lower urinary tract development as well as kidney development and function. In pediatric and adolescent populations, developmental abnormalities are the leading cause of end-stage kidney disease. The goal of this review is to provide pediatric providers a framework for appropriate clinical management as well as highlight when referral to subspecialty care is needed.RECENT FINDINGS: While the exact etiologies of CAKUT are not completely defined, new evidence demonstrates that genetic and molecular changes impact embryonic kidney and urinary tract development. As a result, phenotypes and clinical outcomes may be affected.SUMMARY: Because pediatric providers provide front-line care to children and adolescents with developmental kidney and urinary tract anomalies, updated knowledge of CAKUT pathogenesis, embryology, clinical management, and patient outcomes is needed. This manuscript reviews CAKUT etiologies and essential diagnostic, prognostic, and management strategies.