Section of Pediatric Nephrology, Department of Pediatrics, Hypertension and Renal Center of Excellence, Tulane University Health Sciences Center, 1430 Tulane Avenue, New Orleans, LA, 70112, USA.
Pediatr Nephrol. 2021 Mar;36(3):479-489. doi: 10.1007/s00467-020-04496-5. Epub 2020 Feb 18.
Mutations in the genes of the renin-angiotensin system result in congenital anomalies of the kidney and urinary tract (CAKUT), the main cause of end-stage renal disease in children. The molecular mechanisms that cause CAKUT are unclear in most cases. To improve the care of children with CAKUT, it is critical to determine the underlying mechanisms of CAKUT. In this review, we discuss recent advances that have helped to better understand how disruption of the renin-angiotensin system during kidney development contributes to CAKUT.
肾素-血管紧张素系统基因的突变导致先天性肾和尿路畸形(CAKUT),这是儿童终末期肾病的主要原因。在大多数情况下,导致 CAKUT 的分子机制尚不清楚。为了改善 CAKUT 患儿的护理,确定 CAKUT 的潜在机制至关重要。在这篇综述中,我们讨论了最近的进展,这些进展有助于更好地了解肾发育过程中肾素-血管紧张素系统的破坏如何导致 CAKUT。