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

Abstract

SummaryBackground and objectives Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of ESRD in children, but the proportion of patients with individual CAKUT entities progressing to ESRD during adulthood and their long-term clinical outcomes are unknown. This study assessed the age at onset of renal replacement therapy (RRT) and patient and renal graft survival in patients with CAKUT across the entire age range.Design, setting, participants, & measurements Patients with CAKUT were compared with age-matched patients who were undergoing RRT for other renal disorders on the basis of data from the European Renal AssociationEuropean Dialysis and Transplant Association Registry. Competing risk and Cox regression analyses were conducted.Results Of 212,930 patients commencing RRT from 1990 to 2009, 4765 (2.2%) had renal diagnoses consistent with CAKUT. The proportion of incident RRT patients with CAKUT decreased from infancy to childhood and then increased until age 15-19 years, followed by a gradual decline throughout adulthood. Median age at RRT start was 31 years in the CAKUT cohort and 61 years in the non-CAKUT cohort (P,0.001). RRT was started earlier (median, 16 years) in patients with isolated renal dysplasia than in those with renal hypoplasia and associated urinary tract disorders (median, 29.5-39.5 years). Patients with CAKUT survived longer than age-and sex-matched non-CAKUT controls because of lower cardiovascular mortality (10-year survival rate, 76.4% versus 70.7%; P,0.001).Conclusions CAKUT leads to ESRD more often at adult than pediatric age. Treatment outcomes differ from those of acquired kidney diseases and vary within CAKUT subcategories.

Renal transplantation in infants

“…It is important to note that acute kidney injury in the neonatal period, particularly in premature infants, often recovers and rarely leads to ESRD and RTx in infancy. Similarly, most infants with CAKUT show slow progression and the patients manage into adolescence or adulthood without RRT [7]. The progression of renal failure in inherited CNS may also be slow and RTx can be performed at the age of 2-5 years.…”

Poststreptococcal glomerulonephritis in children with congenital anomalies of the kidney and urinary tract

“…[1][2][3][4] The prevalence of CAKUT varies between 3 and 6 per 1000 births. The prognosis is dependent on the severity of malformations (unilateral, bilateral), presence of hypoplasia, dysplasia, and associated urinary tract infections.…”

Día Mundial del Riñón 2016. Prevención del legado de la nefropatía, con enfoque en la niñez

“…Sin embargo, según la evidencia reciente, muchos pacientes con tipos más leves de ACRVU podrían avanzar a IRT durante la adultez, alcanzando su pico máximo durante la cuarta década de vida. 15 Existen diferencias nacionales y regionales en los tipos y la evolución de la lesión renal aguda y la IRC durante la niñez y más allá de este período. La mortalidad causada por nefropatías es mayor en los países en vías de desarrollo, y es necesario abordar las disparidades nacionales y regionales en el acceso a la atención y los resultados.…”