Doğumsal böbrek ve üriner anomalili çocukların klinik ve demografik özelliklerinin retrospektif değerlendirilmesi

Abstract

Bu çalışmaya göre merkezimize başvuran hastalarda DBÜSA sıklığı %3,9 bulundu. Olguların erkek/kız oranı %48,8(225)/ %51,2(236) bulundu. Çalışmaya dahil edilen olguların %31,5'inde (145) VUR saptandı. Bu hastaların %71,7'si (104) kız, %28,3'si (41) erkekti.Çalışmaya alınan olguların 75'inde (%16,2) hidronefroz vardı. Bu hastaların antenatal tanı oranı %30,7 (23) idi. Hidronefrozun eşlik ettiği doğumsal böbrek ve üriner sistem anomalileri içinde en sık UPD 26 (%70,2), ikinci sıklıkta ise VUR 21 (%14,4) tespit edildi. Olguların %50,5'inde (233) İYE geçirme öyküsü vardı . Bu sıklık VUR tanılı hastalarda %90,3 oranında belirgin yüksekti. Çalışma grubundaki 8 hastada KBH tanı sırasında mevcuttu. Bu hastalardan 4'ünde (%50) altta yatan neden VUR'du. Çalışmadaki olguların %7,5'inde (35) aile öyküsü pozitifti. Anne-baba akrabalığı hastaların %5,2'sinde mevcuttu. SonuçSon yıllarda antenatal ultrasonografinin de yaygınlaşması nedeniyle özellikle geçici hidronefrozlar nefroloji polikliniklerine yoğun olarak başvurmaktadır. Çalışmamızda geçici olmayan ve uzun dönemde İYE ya da KBH progresyonu nedeniyle morbiditeye yol açabilecek DBÜSA tanılı hastalar % 3,9 sıklığında bulunmuştur. Bu hastaların izleminin çocuk nefrolojisi ve ürolojisinin bulunduğu 3. basamak merkezlerde yapılması böbrek işlevlerinin korunması açısından önemlidir.Anahtar sözcükler: Doğumsal böbrek ve üriner sistem anomalisi, VUR, idrar yolu enfeksiyonu

Purpose In this study, the clinical and demographic characteristics of children with congenital abnormalities of kidney and urinary tract (CAKUT) were retrospectively investigated. We aimed to determine the long term epidemiologic, demographic and clinical features of the cases with CAKUT in our tertiary care reference center. Patients and method A total of 461 CAKUT patients, of 11725 patients aged 0-18 years, who were followed up by Kocaeli University School of Medicine Pediatric Nephrology Department between April 2012 and January 2016 were included in the study. The records of the patients studied were retrospectively evaluated and analyzed by SPSS 20.0 program. ResultsAccording to this study, CAKUT frequency was 3.9% in patients who applied to our center. The male / female ratio of the cases was 48.8% (225) / 51.2% (236). VUR was detected in 31.5% (145) of the cases. Of these patients, 71.7% (104) were female and 28.3% (41) were male.Hydronephrosis was present in 75 (16.2%) of the cases studied. The antenatal diagnosis rate of these patients was 30.7% (23). UPJ obstruction (26-70.2%) and VUR (21-14.4%) were the most common congenital renal and urinary system anomalies associated with hydronephrosis. 50.5% (233) of the cases had a history of urinary tract infection. This frequency was significantly higher in patients with VUR ( 90.3%). Chronic kidney disease was present at the time of diagnosis in 8 patients in the study group. The underlying cause in 4 (50%) of these patients was VUR. In 7.5% (35) of the cases in the study family history for CAKUT was positive. Parental consanguinity was present in 5.2% of patients.ConclusionBecause of the widespread use of antenatal ultrasonography in recent years, transient hydronephrosis has been diagnosed and followed extensively in pediatric nephrology clinics. Patients with CAKUT in our study were found to have a frequency of 3.9% with considerable morbidity and long term risk of progression to chronic kidney disase secondary to recurrent urinary tract infections. Therfore close follow-up of these patients in tertiary care centers with a multidisciplinary approach by pediatric nephrology, urology and other relevant clinics is important for the protection of kidney function.