Renal outcome in patients with congenital anomalies of the kidney and urinary tract

Abstract

Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) are a major cause of morbidity in children. We measured the risk of progression to end-stage renal disease in 312 patients with CAKUT preselected for the presence of anomalies in kidney number or size. A model of dialysis-free survival from birth was established as a function of the renal CAKUT categories of solitary kidney; unilateral and bilateral hypodysplasia; renal hypodysplasia associated with posterior urethral valves; and multicystic and horseshoe kidney. Cox regression analysis took into account the concomitant presence of vesicoureteral reflux, year of diagnosis, and time-varying values of serum creatinine, proteinuria, and hypertension. By 30 years of age, 58 patients had started dialysis, giving a yearly incidence of 0.023 over a combined 2474 patient risk years. The risk for dialysis was significantly higher for patients with a solitary kidney or with renal hypodysplasia associated with posterior urethral valves (hazard ratios of 2.43 and 5.1, respectively) compared to patients with unilateral or bilateral renal hypodysplasia, or multicystic or horseshoe kidney, and was independent of other prognostic factors. Our study shows that sub-clinical defects of the solitary kidney may be responsible for a poorer prognosis compared to more benign forms of CAKUT. Prospective studies are needed to validate these results.

Timing and Outcome of Renal Replacement Therapy in Patients with Congenital Malformations of the Kidney and Urinary Tract

“…It is possible that a major fraction of male patients in these categories had posterior urethral valves, a male-limited condition often associated with particularly severe renal dysplasia. In keeping with a major effect of sex-specific underlying kidney diseases on renal survival, a recent single-center CAKUT study from Italy showed that male sex predicted a shorter renal survival by univariate analysis but not after adjustment for diagnosis categories (11).…”

“…During adolescence, accelerated progression of CKD to ESRD is frequently observed (10). Altogether, approximately 25% of children born with bilateral CAKUT and kidney dysfunction require RRT during the first two decades of life (2,10,11). However, little information is available about the long-term prognosis of patients with CAKUT as they advance into adult life.…”

“…The natural course of CAKUT is very heterogeneous (10,11). Although the most severely affected newborns progress to ESRD within the first few months of life, kidney function improves in most children born with CAKUT, typically reaching a peak around age 3-4 years.…”

Chronic Kidney Disease: A Life Course Health Development Perspective

“…Hypoplastic or dysplastic kidneys, obstructive uropathy, and vesicoureteral reflux (VUR) account for 30% of children on dialysis in the USA (NAPRTCS 2011;Chen et al 2012;Bagby 2015;Puddu et al 2009;Chiang et al 2010;Hsu et al 2014;Greenbaum et al 2011;Horbar et al 2012;Hack et al 2002;Stelloh et al 2012;Gilbert et al 1990;LelievrePegorier et al 1998;Sutherland et al 2012a;Ortiz et al 2001;Brenner and Anderson 1987;Reidy and Kaskel 2007;Fogo2007;Lopez-Hernandez and Lopez-Novoa 2012;Gubhaju et al 2009;Sutherland et al 2012b;White et al 2009;Bacchetta et al 2009;Keijzer-Veen et al 2010;Rademacher and Sinaiko 2009;Salgado et al 2009; Centers for Disease Control and Prevention (CDC) 2007; Hodgin et al 2009a;Harambat et al 2012;Warady and Chadha 2007;Tabel et al 2010;Sanna-Cherchi et al 2009). The development of end-stage renal disease in infancy/childhood results in poor growth and altered health development.…”

The Life Cycle of the Kidney

“…4 Although the majority of renal failure in adults results from diabetes and hypertension, recent reports demonstrate that renal failure as a result of congenital renal disorders is more likely to develop in adulthood than childhood. 5,6 In this issue of JASN, investigators from Rotterdam, The Netherlands, report two studies based on the Generation R Study, a population-based prospective cohort of healthy participants followed from fetal life through 5-8 years of age. In one study, fetal growth was measured by ultrasonography in the second and third trimesters, during which nephron number increases 50-fold.…”