Sevgi YAVUZ, Aysel KIYAK, Ferhat DEMİR, Ayşe Nur AKINEL

Renal ektopili çocukların ürolojik ve nefrolojik analizi

Amaç: Renal ektopi (RE)nin morfolojik ve klinik özelliklerini, ektopi lateralizasyonunun prognozu etkileyip etkilemediğini değerlendirmek. Yöntemler: Merkezimizde 2004-2014 yılları arasında RE tespit edilen 61 pediatrik hastanın klinik, laboratuvar ve görüntüleme sonuçları retrospektif olarak gözden geçirildi. Bulgular: Hastaların 32si kız (%52,5), 29u erkek (%47,5), yaş ortalaması 48 (2-204) ay, takip süresi 60 (2- 120) aydı. RE 33 hastada (%54) sağ, 26 hastada (%46) sol yerleşimliydi. 52 hastada (%85,2) basit RE, 9 hastada (%14,8) çapraz RE tespit edildi. 27 hastada (%44.3) ek üriner anomali, 15inde (%24.6) ek hastalık mevcuttu. En sık görülen üriner sistem bozukluğu vezikoüreteral reflü (VUR) (%21) idi. DMSA sintigrafisinde 17 hastada (%27,9) renal skar (RS) tespit edildi. Takiplerde 32 hastada (%52.5) idrar yolu enfeksiyonu (İYE), 5inde (%8,2) hipertansiyon, 11inde (%11) proteinüri ve 5inde (%8) kronik böbrek hastalığı (KBH) görüldü. Hastalar ektopi lokalizasyonuna göre karşılaştırıldığında ek üriner ve sistemik anomali, İYE, RS, hipertansiyon, proteinüri ve KBH gelişim riski açısından sağ ve sol taraflar arasında istatiksel açıdan anlamlı fark saptanmadı (p> 0,05). Sonuç: REli çocuklar ek üriner ve sistemik hastalık açı- sından dikkatle incelenmelidir. Artmış VUR riski nedeniyle bu hastalara voiding sistoüretrografi çekilmesi önerilebilir. REde İYE ve RSa yatkınlık yüksek olup proteinüri, hipertansiyon ve KBH gibi uzun dönem komplikasyonların ortaya çıkışını önlemek için hastalar yakından takip edilmelidir. Ektopi lateralizasyonu ek hastalık insidansını ve geç komplikasyonları etkilemeyebilir

The urological and nephrological analysis of children with renal ectopia

Objective: To assess the morphological and clinical features of children with renal ectopia (RE) and assess whether the lateralization of ectopia determines the prognosis. Methods: The clinical, laboratory and radiological data of 61 patients diagnosed as RE in our center between the years of 2004 and 2014 were retrospectively reviewed. Results: There were 32 girls (52.5%) and 48 boys (47.5%). The median age was 48 (2-204) months and follow-up time was 60 (2-120) months. RE was right-sided in 33 (54%) and left-sided in 26 (46%) patients. 52 patients (85.2%) had simple RE whereas 9 (14.8%) had crossed RE. 27 children (44.3%) had additional urinary abnormality, 15 (24.6%) had extra-renal disease. The most common urinary tract malformation was vesicoureteral reflux (VUR) (21%). Renal scarring (RS) was detected in 17 patients (27.9%) on DMSA scan. 32 patients (52.5%) experienced urinary tract infection (UTI), 5 (8.2%) had hypertension, 11 (11%) had proteinuria, 5 (8%) had chronic renal disease (CKD). No significant difference was found between ectopia sides for development of additional urinary or systemic disease, UTI, proteinuria, hypertension and CKD (p> 0.05). Conclusion: The children with RE should be carefully examined for additional urinary or systemic abnormalities. Voiding cystourethrogram might be recommended in those patients for the increased risk of VUR. The patients with RE should be closely followed-up for avoiding long term complications including proteinuria, hypertension and CKD because of susceptibility to UTI and RS. The lateralization of ectopia might not affect the incidence of additional abnormality or late complications.

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1. Woolf AS. A molecular and genetic view of human renal and urinary tract malformations. Kidney Int 2000;58:500-512.
2. Bauer SB. Abnormalities of the upper urinary tract. In: Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA, eds. Campbell Walsch Urology, 9th ed. Philedelphia: Saunders Elsevier; 2007:3278-3281.
3. Rushton HG. The evaluation of acute pyelonephritis and renal scarring with technetium 99m-dimercaptosuccinic acid renal scintigraphy: evolving concepts and future directions. Pediatr Nephrol 1997;11:108-120.
4. Lebowitz RL, Olbing H, Parkkulainen KV, et al. International system of radiographic grading of vesicoureteral reflux. International Reflux Study in Children. Pediatr Radiol 1985;15:105-109.
5. Schwartz GJ, Munoz A, Schneider MF, et al. New equations to estimate GFR in children with CKD. J Am Soc Nephrol 2009;20:629-637.
6. National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents. The fourth report on the diagnosis, evaluation and treatment of high blood pressure in children and adolescents. Pediatrics 2004;114:555-576.
7. Van den Bosch CM, van Wijk JA, Beckers GM, et al. Urological and nephrological findings of renal ectopia. J Urol 2010;183:1574-1578.
8. Guarino N, Tadini B, Camardi P, et al. The incidence of associated urological abnormalities in children with renal ectopia. J Urol 2004;172:1757-1759.
9. Arena F, Arena S, Paolata A, et al. Is a compete urological evaluation necessary in all newborns with asymptomatic renal ectopia? Int Urol Nephrol 2007;14:491-495.
10. Dursun H, Bayazıt AK, Büyükçelik M, et al. Associated anomalies in children with congenital solitary functioning kidney. Pediatr Surg Int 2005;21:456-459.
11. Pagon RA, Graham JM Jr, Zonona J, Yong SL. Coloboma, congenital heart disease and choanal atresia with multiple anomalies: CHARGE association. J Pediatr 1981;99:223- 227.
12. Evans J, Stranc LC, Kaplan P, Hunter AG. VACTERL with hydrocephalus: further delineation of the syndrome(s). Am J Med Genet 1989;34:177-182.
13. Gleason PE, Kelalis PP, Husmann DA, Kramer SA. Hydronephrosis in renal ectopia: incidence, etioogy and significance. J Urol 1994;151:1660-1661.
14. Ubetagoyena Arrieta M, Areses Trapote R, Arruebarrena Lizarraga D. Renal position and fusion abnormalities. An Pediatr Barc 2011;75:329-333.
15. Schreuder MF. Unilateral abnormalities of kidney development: why is left not right? Kidney Int 2011;80:740-745.