Renal outcome in patients with congenital anomalies of the kidney and urinary tract.
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.
Sanna-Cherchi
Simone
S
Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA.
Ravani
Pietro
P
Corbani
Valentina
V
Parodi
Stefano
S
Haupt
Riccardo
R
Piaggio
Giorgio
G
Innocenti
Maria L Degli
ML
Somenzi
Danio
D
Trivelli
Antonella
A
Caridi
Gianluca
G
Izzi
Claudia
C
Scolari
Francesco
F
Mattioli
Girolamo
G
Allegri
Landino
L
Ghiggeri
Gian Marco
GM
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*