Amaç: Ektopik böbrek (EB), konjenital böbrek ve üriner sistem anomalileri [congenital anomalies of kidney and urinary tract (CAKUT)]nin nedenlerinden biridir. Bu çalışmada, EB nedeniyle izlediğimiz hastaların demografik özelliklerinin ve izlem sonuçlarının değerlendirilmesi, eşlik eden üriner ve diğer sistem anomalilerinin sıklığının belirlenmesi amaçlandı. Gereç ve Yöntemler: Çocuk nefroloji polikliniğimizde 2000 ve 2019 yılları arasında EB tanısı ile takip edilen toplam 67 hasta (24 kız, 43 erkek) geriye dönük olarak değerlendirildi. Bulgular: On altı (%23,9) hasta, antenatal tanı almış olup, diğer 51 hastanın ortanca tanı yaşı 3 [çeyrekler arası aralık (ÇAA): 5,4] yıl, tüm hastaların ortanca takip süresi 3,8 (ÇAA: 5,6) yıl idi. On (%14,9) hastada akraba evliliği, 10 (%14,9) hastada ailede CAKUT öyküsü mevcuttu. Elli altı (%83,6) hastada basit pelvik ektopi, 11 (%16,4) hastada çapraz ektopi mevcuttu. Elli sekiz hastaya, işeme sistoüretrografisi [voiding cystourethrography (VCUG)] uygulandı. On bir (%19) hastada 14 böbrek ünitesine vezikoüreteral reflü [vesicoureteral reflux (VUR)] saptandı. Diğer üriner sistem anomalileri 7 (%10,5) hastada saptandı. On altı (%23,9) hastada idrar yolu enfeksiyonu (İYE) öyküsü olup, bu hastaların 10 (%62,5)'unda üriner sistem anomalisi mevcuttu. Çapraz ve basit pelvik EB arasında İYE ve VUR açısından fark saptanmadı (p=0,29 ve p=0,16), ancak çapraz EB'li hastalarda teknesyum99m-dimerkaptosüksinik asit (99mTc-DMSA)teki ortalama fonksiyonel kortikal kitleye katkı anlamlı olarak düşük bulundu (p=0,015). Ek üriner sistem anomalisi olan hastalarda İYE sıklığı anlamlı olarak yüksekken (p=0,009); 99mTc-DMSA'da skar gelişimi açısından fark saptanmadı (p=0,26). Toplam 4 (%6) hastada hipertansiyon saptandı. Sonuç: EB tanısı, sadece böbreğin farklı anatomik yerleşim gösterdiği masum bir durum değildir. EB tanılı hastalar, doğumdan itibaren eşlik edebilecek ek üriner anomaliler, diğer sistemlerin doğumsal anomalileri, İYE, hipertansiyon gibi komplikasyonlar açısından düzenli izlenmelidir.
Anahtar Kelimeler: CAKUT; ektopik böbrek; idrar yolu enfeksiyonu; vezikoüreteral reflü
Objective: Ectopic kidney (EK) is a congenital anomalies of kidney and urinary tract (CAKUT). In this study, we aimed to evaluate the demographic characteristics, follow-up results of the patients with EK and to determine the frequency of urinary and other system anomalies. Material and Methods: A total of 67 patients (24 girls, 43 boys) were evaluated retrospectively in our pediatric nephrology outpatient clinic between 2000-2019. Results: The median age at diagnosis of was 3 [interquartile range (IQR): 5.4] years and the median follow-up time was 3.8 (IQR: 5.6) years. Sixteen (23.9%) patients were diagnosed antenatally, ten (%14.9) patients had parental consanguinity and ten (%14.9) had a family history of CAKUT. Simple pelvic ectopia was present in 56 (83.6%) patients, and cross ectopia in 11 (%16.4) patients. Voiding cystourethrography (VCUG) was performed for 58 patients and vesicoureteral reflux (VUR) was detected in 14 kidney units in 11 (%19) patients. Other urinary system abnormalities were detected in seven (%10.5) patients. Sixteen (%23.9) patients had urinary tract infection (UTI), ten (%62.5) of whom had urinary system abnormalities. There was no difference between cross and simple EK in terms of UTI and VUR (p=0.29 and p=0.16), but the relative renal function in technetium-99m-dimercaptosuccinic acid (99mTc-DMSA) was significantly lower in cross EK (p=0.015). The frequency of UTI was significantly higher in patients with urinary system abnormalities (p=0.009), but there was no difference in terms of scarring in 99mTc DMSA (p=0.26). Four patients (%6) had hypertension. Conclusion:Ectopic kidney is not an innocent diagnosis defined as abnormal anatomical location. Patients with EK should be followed-up regularly for urinary and other system abnormalities, UTI and hypertension.
Keywords: CAKUT; ectopic kidney; urinary tract infection; vesicoureteral reflux
- Queisser-Luft A, Stolz G, Wiesel A, Schlaefer K, Spranger J. Malformations in newborn: results based on 30,940 infants and fetuses from the Mainz congenital birth defect monitoring system (1990-1998). Arch Gynecol Obstet. 2002;266(3):163-7.[Crossref] [PubMed]
- Dogan CS, Dorterler ME, Aybar MD, Ciftci H, Gulum M, Akin Y, et al. Associated anomalies and clinical outcome in children with ectopic kidney. Saudi J Kidney Dis Transpl. 2017;28(2):330-5.[Crossref] [PubMed]
- Rosenblum ND, Gupta IR. Disorders of kidney formation. In: Geary DF, Schaefer F, eds. Pediatric Kidney Disease. 2nd ed. Berlin, Germany: Springer; 2016. p.277-302.[Crossref]
- Jain S, Chen F. Developmental pathology of congenital kidney and urinary tract anomalies. Clin Kidney J. 2018;12(3):382-99.[Crossref] [PubMed] [PMC]
- Yavuz S, Kıyak A, Demir F, Akınel AN. [The urological and nephrological analysis of children with renal ectopia]. Dicle Tıp Dergisi. 2014;41(3):529-33.[Crossref]
- Lebowitz RL, Olbing H, Parkkulainen KV, Smellie JM, Tamminen-Möbius TE. International system of radiographic grading of vesicoureteric reflux. International Reflux Study in Children. Pediatr Radiol. 1985;15(2):105-9.[Crossref] [PubMed]
- Nguyen HT, Herndon CD, Cooper C, Gatti J, Kirsch A, Kokorowski P, et al. The Society for Fetal Urology consensus statement on the evaluation and management of antenatal hydronephrosis. J Pediatr Urol. 2010;6(3):212-31.[Crossref] [PubMed]
- Schwartz GJ, Brion LP, Spitzer A. The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents. Pediatr Clin North Am. 1987;34(3):571-90.[Crossref] [PubMed]
- Schwartz GJ, Mu-oz A, Schneider MF, Mak RH, Kaskel F, Warady BA, et al. New equations to estimate GFR in children with CKD. J Am Soc Nephrol. 2009;20(3):629-37.[Crossref] [PubMed] [PMC]
- Elmacı AM, Dönmez Mİ, Soran M. Is voiding cystourethrography necessary for evaluating unilateral ectopic pelvic kidney? Turk J Urol. 2019;45(Supp. 1):S108-S110.[Crossref] [PubMed] [PMC]
- Arena F, Arena S, Paolata A, Campenni A, Zuccarello B, Romeo G. Is a complete urological evaluation necessary in all newborns with asymptomatic renal ectopia? Int J Urol. 2007;14(6):491-5.[Crossref] [PubMed]
- Guarino N, Tadini B, Camardi P, Silvestro L, Lace R, Bianchi M. The incidence of associated urological abnormalities in children with renal ectopia. J Urol. 2004;172(4 Pt 2):1757-9.[Crossref] [PubMed]
- Hiraoka M, Hori C, Tsukahara H, Kasuga K, Kotsuji F, Mayumi M. Voiding function study with ultrasound in male and female neonates. Kidney Int. 1999;55(5):1920-6.[Crossref] [PubMed]
- Smellie JM, Normand IC, Katz G. Children with urinary infection: a comparison of those with and those without vesicoureteric reflux. Kidney Int. 1981;20(6):717-22.[Crossref] [PubMed]
- Orellana P, Baquedano P, Rangarajan V, Zhao JH, Eng ND, Fettich J, et al. Relationship between acute pyelonephritis, renal scarring, and vesicoureteral reflux. Results of a coordinated research project. Pediatr Nephrol. 2004;19(10):1122-6.[Crossref] [PubMed]
- van den Bosch CM, van Wijk JA, Beckers GM, van der Horst HJ, Schreuder MF, Bökenkamp A. Urological and nephrological findings of renal ectopia. J Urol. 2010;183(4):1574-8.[Crossref] [PubMed]
- Gleason PE, Kelalis PP, Husmann DA, Kramer SA. Hydronephrosis in renal ectopia: incidence, etiology and significance. J Urol. 1994;151(6):1660-1.[Crossref] [PubMed]
- Polak-Jonkisz D, Fornalczyk K, Musiał K, Zaleska-Dorobisz U, Apoznański W, Zwolińska D. Crossed renal ectopia: can it be a diagnostic problem? Postepy Hig Med Dosw (Online). 2012;66:210-4.[Crossref] [PubMed]
.: Process List