A. Midhat ELMACI, FATİH AKIN

Konjenital böbrek ve üriner kanal anomalisi bulunan çocukların klinik ve demografik özellikleri

Amaç: Konjenital böbrek ve üriner kanal anomalisi (CAKUT), çocuklarda son dönem böbrek hastalığına neden olabilen, yapısal ve fonksiyonel malformasyonları içerir. Bu çalışmada CAKUT tanısı ile takip edilen vakaların klinik ve demografik özellikleri değerlendirildi. Yöntemler: Diyarbakır Çocuk Hastalıkları Hastanesinde Ekim 2008 ile Haziran 2011 tarihleri arasında CAKUT tanı- sı alan vakaların dosyaları geriye dönük olarak incelendi. Dosya bilgilerinden yaş, cinsiyet, aile öyküsünde CAKUT, akraba evliliği ve görüntüleme çalışmaları kaydedildi. Bulgular: Çalışmaya 232 hasta alındı, 129u erkek (%55,6), 103ü kızdı (%44,4), ortalama yaş 4.4 yıl (6 gün- 17 yıl). Ailede CAKUT öyküsü vakaların %15,2sinde ve akraba evliliği %50,9unda tespit edildi. Çalışmamızda vakaların 66sında (%28,5) üretero-pelvik darlık (UPD), 49unda (%21,1) renal agenezi, 25inde (%10,8) ektopik böbrek, 25inde (%10.8) at nalı böbrek, 20sinde (%8,6) multikistik displastik böbrek (MKDB), 20sinde (%8,6) primer veziko-üreteral reflü (VUR), 12sinde (%5,2) polikistik böbrek (PKB), 7sinde (%3) posterior üretral valv (PUV), 4ünde (%1,7) üretero-vezikal darlık (UVD), 2sinde (%0,9) renal hipoplazi ve 2sinde (%0,9) füzyone böbrek tespit edildi. Vakaların 32sinde (%13,8) böbrekte skar ve 7sinde (%3) kronik böbrek hastalığı (KBH) mevcuttu. Sonuçlar: Çocukluk çağında CAKUT ciddi morbiditeye neden olabilir. Antenatal dönemden itibaren vakaların dikkatli ve düzenli takibi önemlidir. Tedavi gereken vakalarda erken dönemde teşhis, kalıcı renal hasar riskini azaltır.

Clinical and demographic characteristics of children with congenital anomaly of kidney and urinary tract

Objective: Congenital anomaly of kidney and urinary tract (CAKUT) includes functional and structural anomalies that can cause end-stage renal disease in children. Clinical and demographic characteristics of patients with CAKUT are evaluated in this study. Methods: The files of patients who were followed up with the diagnosis of CAKUT between November 2008 and June 2011 in Diyarbakır Childrens Hospital were reviewed retrospectively. Patient characteristics including age, gender, family history of CAKUT, consangineous marriage, and radiological imaging results were recorded. Results: The study was consisted of 232 patients 129 (55.6%) boys, and 103 (44.4%) girls. The mean age was 4.4 years. A family history of CAKUT was found in 15.2% of patients and 50.9% of parents had consanguineous marriage. Anomalies that were observed in the study included, ureteropelvic junction obstruction in 66 (28.5%), renal agenesis in 49 (21.1%), ectopic kidney in 25 (10.8%), horseshoe kidney in 25 (10.8%), multicystic dysplastic kidney in 20 (8.6%), vesicoureteral reflux in 20 (8.6%), polycystic kidney in 12 (5.2%), posterior urethral valve in 7 (3%), ureterovesical obstruction in 4 (1.7%), renal hypoplasia in 2 (0.9%), and fusion of kidneys in 2 (%0.9) patients. Renal scarring and chronic kidney disease was present in 32 (13.8%) and 7 (3%) patients, respectively. Conclusion: Childhood CAKUT can cause serious morbidities. Cases should be followed up carefully even from the antenatal period. Early intervention of cases requiring treatment will minimize the risk of permanent renal damage.

PDF

___

1. Yosypiv IV Congenital anomalies of the kidney and urinary tract: a genetic disorder? Int J Nephrol 2012; 2012:909083 doi: 10.1155/2012/909083.
2. Rosenblum ND, Salomon R. Disorders of kidney formation. In: Geary DF, Schaefer F (eds).Comprehensive Pediatric Nephrology. Mosby-Elsevier, Philadelphia, 2008; 132-141.
3. Bulum B, Ozçakar ZB, Ustüner E, et al. High frequency of kidney and urinary tract anomalies in asymptomatic firstdegree relatives of patients with CAKUT. Pediatr Nephrol 2013;28:2143-2147.
4. Bek K, Akman S, Bilge I, et al. Chronic kidney disease in children in Turkey. Pediatr Nephrol 2009;24:797-806.
5. Şimşek F, Tinay İ. Çocuklarda üreteropelvik bileşke obstrüksiyonları. Klinik Gelişim 2008;21:24-27.
6. Ulman İ, Divarcı E. Çocuklarda obstrüktif üropatiler. Türk Ped Arş 2010; 45:85-89.
7. Quirino IG, Diniz JS, Bouzada MC, et al. Clinical course of 822 children with prenatally detected nephrouropathies. Clin J Am Soc Nephrol 2012;7:444-451.
8. Becker AM. Postnatal evaluation of infants with an abnormal antenatal renal sonogram. Curr Opin Pediatr 2009;21:207- 213.
9. Westland R, Schreuder MF, Bökenkamp A, et al. Renal injury in children with a solitary functioning kidney--the KIMONO study. Nephrol Dial Transplant 2011;26:1533-1541.
10. Schreuder MF, Westland R, van Wijk JA. Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney. Nephrol Dial Transplant 2009;24:1810-1818.
11. Tiryaki S, Alkac AY, Serdaroglu E, et al. Involution of multicystic dysplastic kidney: is it predictable? J Pediatr Urol 2013;9:344-347.
12. Kaneko K, Suzuki Y, Fukuda Y, et al. Abnormal contralateral kidney in unilateral multicystic dysplastic kidney disease. Pediatr Radiol 1995;25:275-277.
13. Kibar Y, Ashley RA, Roth CC, et al. Timing of posterior urethral valve diagnosis and its impact on clinical outcome. J Pediatr Urol 2011;7:538-542.