title: 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.
authors.affiliation: Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA.
authors.forename: Simone
authors.initials: S
authors.lastname: Sanna-Cherchi
authors.forename: Pietro
authors.initials: P
authors.lastname: Ravani
authors.forename: Valentina
authors.initials: V
authors.lastname: Corbani
authors.forename: Stefano
authors.initials: S
authors.lastname: Parodi
authors.forename: Riccardo
authors.initials: R
authors.lastname: Haupt
authors.forename: Giorgio
authors.initials: G
authors.lastname: Piaggio
authors.forename: Maria L Degli
authors.initials: ML
authors.lastname: Innocenti
authors.forename: Danio
authors.initials: D
authors.lastname: Somenzi
authors.forename: Antonella
authors.initials: A
authors.lastname: Trivelli
authors.forename: Gianluca
authors.initials: G
authors.lastname: Caridi
authors.forename: Claudia
authors.initials: C
authors.lastname: Izzi
authors.forename: Francesco
authors.initials: F
authors.lastname: Scolari
authors.forename: Girolamo
authors.initials: G
authors.lastname: Mattioli
authors.forename: Landino
authors.initials: L
authors.lastname: Allegri
authors.forename: Gian Marco
authors.initials: GM
authors.lastname: Ghiggeri
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007668:Kidney / Q000002:abnormalities* / Q000473:pathology; D007674:Kidney Diseases / Q000401:mortality; D008297:Male; D011379:Prognosis; D006435:Renal Dialysis; D014551:Urinary Tract / Q000002:abnormalities*