Üriner Sistemin Konjenital Anomalisi (USKA) Olan Çocukların Klinik Seyirleri ve Bu Hastalarda Son Durum Hakkında Fikir Verebilecek Ilk Değişkenlerin Saptanması

Abstract

Congenital anomalies of kidney and urinary tract (CAKUT) is one of the most common causes of chronic renal failure (CRF) in childhood. CAKUT is a spectrum of renal, ureteral, bladder and urethral anomalies and results from genetic abnormalities . There are several studies related to pathogenesis, progression and outcome of CAKUT but there is no consensus on them . In this retrospective study, we aimed to characterize subgroups of CAKUT and their particular course. Futhermore we also aimed to determine, if any, initial factors that would be related to CRF. Three hundred patients (203 male, 97 female) were included in the study. They were divided into 16 subgroups according to their diagnosis. Antenatal hydronephrosis and multicystic dysplastic kidney diseases were found as the most common subgroup of CAKUT. Males were dominant in all sub-groups. Patients with posterior urethral valve (PUV) had the worst prognosis in CAKUT and these patients progressed to CRF earlier than the other subgroups. PUV was the leading cause that needs surgery in early in life.. Proteinuria and vesicourethral reflux (VUR) were found to be more prevalent in those patients who progressed to CRF. Olygohydramniosis was found to be a significant predictor for CRF and these patients reached end stage renal failure earlier when compared to other subgroups. There was no difference in glomerular filtration rate between patients with obstruction of ureteropelvic junction (UPJ) who underwent surgery and who did not. We detected increased risk of hypertension in patients with multicystic dysplastic kidney and renal agenesis , and increased risk of VUR in patients with horse shoe and ectopic kidney . VUR was prevalent in males than in females with increased risk of risk of urinary tract infection. Factors related to worse prognosis were PUV, male gender, proteinuria, presence of VUR, urinary tract infection and olygohydramniosis. This study provides important findings in terms of disease course and outcome that would be a guide in routine practice.