Avanoglu Ali, Tiryaki Sibel
Division of Pediatric Urology, Department of Pediatric Surgery, Ege University, Izmir, Turkey.
Gaziantep Maternity and Children's Hospital, Pediatric Urology, Gaziantep, Turkey.
Front Pediatr. 2020 Apr 7;8:137. doi: 10.3389/fped.2020.00137. eCollection 2020.
Kidney parenchyma and collecting system arise from two different embryologic units as a result of a close interaction between them. Therefore, their congenital abnormalities are classified together under the same heading named CAKUT (congenital abnormalities of the kidney and urinary tract). The pathogenesis of CAKUT is thought to be multifactorial. Ureteropelvic junction obstruction (UPJO) is the most common and most investigated form of CAKUT. Despite years of experimental and clinical research, and the information gained on the embryogenesis of the kidney; its etiopathogenesis is still unclear. It involves both genetic and environmental factors. Failure in development of the renal pelvis, failure in the recanalization of ureteropelvic junction, abnormal pyeloureteral innervation, and impaired smooth muscle differentiation are the main proposed mechanisms for the occurrence of UPJO. There are also single gene mutations like AGTR2, BMP4, Id2 proposed in the etiopathogenesis of UPJO.
肾实质和集合系统源于两个不同的胚胎学单元，这是它们之间密切相互作用的结果。因此，它们的先天性异常被归为同一类，称为CAKUT（先天性肾脏和尿路异常）。CAKUT的发病机制被认为是多因素的。肾盂输尿管连接部梗阻（UPJO）是CAKUT最常见且研究最多的形式。尽管经过多年的实验和临床研究，以及在肾脏胚胎发生方面获得的信息，但UPJO的病因发病机制仍不清楚。它涉及遗传和环境因素。肾盂发育失败、肾盂输尿管连接部再通失败、肾盂输尿管神经支配异常以及平滑肌分化受损是UPJO发生的主要提出机制。在UPJO的病因发病机制中也提出了如AGTR2、BMP4、Id2等单基因突变。
