Clinical, laboratory, imaging features and prognosis in pediatric patients with Congenital anomalies of the kidney and the urinary tract (CAKUT): a referral center experience

Abstract Background This study aims to evaluate the demographic, clinical, laboratory, and imaging findings in Iranian children diagnosed with congenital abnormalities of the kidney and urinary tract (CAKUT). Methods All patients with a diagnosis of CAKUT referred to the Nephrology clinics of Mofid Children’s Hospital between 2018 and 2023 were included, and their medical records were retrospectively investigated and analyzed. Results A total of 390 CAKUT patients [131 (33.6%) male and 259 (66.4%) female], at a median (IQR) age of 65.5 (44.8–115.3) months, were identified. The most frequent clinical manifestation was hydronephrosis (291, 74.6%), among whom 99 (25.4%) cases of vesicoureteral reflux (VUR), 94 (24.1%) cases of isolated hydronephrosis, 93 (23.8%) cases of posterior urethral valves (PUV), 67 (17.2%) cases of ureteropelvic junction obstruction (UPJO), 50 (12.8%) cases of neurogenic bladder, and 22 (5.6%) cases of ureterovesical junction obstruction (UVJO) were identified. 278 (71.3%) patients underwent surgery at a median (IQR) age of 24 (3–68.5) months. Patients were followed up for a median (IQR) duration of 42 (14.8–94) months, and 35 (9%) patients succumbed to their disease at a median (IQR) age of 34 (3.5–94.5) months. The most frequent causes of death were sepsis and infections (48% of all deaths) and ESRD (22% of all deaths). Conclusions These findings suggest a delay in the diagnosis and management of CAKUT cases in Iranian patients. Early diagnosis of CAKUT in pediatric patients and parental/public education reduces long-term renal sequela and mortality rate.