Predicting outcomes and improving care in children with congenital kidney anomalies
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EDITORIAL COMMENTARY
Predicting outcomes and improving care in children with congenital kidney anomalies Douglas G. Matsell 1
&
Marisa Catapang 1
Received: 14 June 2020 / Accepted: 16 June 2020 # IPNA 2020
Keywords Children . CAKUT . Congenital urinary tract obstruction . Obstructive nephropathy . Chronic kidney disease . Biomarkers . Outcome
Introduction Children born with congenital anomalies of the kidney and urinary tract (CAKUT) are at risk of progressive chronic kidney disease (CKD) and long-term kidney injury [1, 2]. These observations are supported by long-term observational data; however, reports are limited and some are hampered by their retrospective nature, small cohort sizes, short follow ups, and heterogeneous patient populations [3]. Although a variety of CAKUT conditions are thought to have universally good long-term outcomes, such as children born with a solitary functioning kidney due to contralateral renal agenesis or a multicystic dysplastic kidney, a progressive decline in kidney function over time has been reported [4].
Congenital urinary tract obstruction and obstructive nephropathy Congenital urinary tract obstruction (CUTO) resulting in obstructive nephropathy, the focus of the recent publication by McLeod et al. in this edition of Pediatric Nephrology [5], is singularly the most important and most common congenital kidney anomaly resulting in progressive CKD and kidney failure in young boys. Experimental models developed over the past 3 decades have defined the pathogenesis of the progressive and chronic kidney changes that occur, including the development of kidney dysplasia, medullary hypoplasia, * Douglas G. Matsell [email protected] 1
Division of Nephrology, British Columbia Children’s Hospital, University of British Columbia, 4480 Oak Street, Room K4-150, Vancouver, British Columbia V6H 2V2, Canada
interruption in nephron induction with a decrease in kidney size and nephron number, tubulointerstitial changes, and remodeling of the collecting duct epithelium [6]. The onset of obstructive kidney injury starts during fetal life, with the extent and scope of the pathological changes determined by the severity and duration of the obstruction. The causes are most commonly due to bladder outlet obstruction and posterior urethral valves (PUV) but include unilateral and bilateral functional and anatomical blockage of the ureters. Consequently, not all children are equally affected by the obstruction, some have associated anomalies or genetic conditions affecting multiple organ systems, and others have complicated antenatal and/or postnatal courses which negatively impact their longterm outcome [7]. Because of the variability of fetal kidney injury, there is a corresponding variability in the long-term postnatal clinical outcomes, including the development of hypertension and proteinuria, the extent of decline in kidney function, and the rate of progression to kidney failure. Previous investigations have focused on correlating the histopathological and functional changes seen in early
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