Evaluation of children with multicystic dysplastic kidney: The role of recurrent urinary tract infections

Öz

Purpose: Multicystic dysplastic kidney (MCDK) is the most common cystic renal disease in children and is often detected prenatally. This study evaluated the clinical features, associated anomalies, and renal outcomes of children with unilateral MCDK, focusing on the impact of recurrent urinary tract infections (UTIs) on renal function. Methods: A retrospective review of 77 patients (aged 1–18 years) diagnosed with unilateral MCDK by ultrasonography between 2019 and 2024 was conducted. Demographic, clinical, and laboratory data were analyzed, including estimated glomerular filtration rate (eGFR), UTIs, hypertension, and contralateral compensatory hypertrophy. Results: Of 77 patients, 64.9% were male and 55.8% had left-sided disease. Antenatal diagnosis was made in 77.9%, and 85.7% were asymptomatic. Urinary tract and extrarenal anomalies occurred in 27.3% and 40.3%, respectively. UTIs were observed in 31.2% and recurrent UTIs in 11.7%. Vesicoureteral reflux (VUR) was detected in 9.1% overall and in 33.3% of patients with recurrent UTIs (p=0.031). Mean follow-up was 7.2±4.4 years with a mean eGFR of 99.9±17.8 mL/min/1.73 m². Recurrent UTIs were more frequent in patients with eGFR <90 (p=0.018) and were associated with lower eGFR (p=0.021). Contralateral compensatory hypertrophy was present in 62.3% and correlated with older age and longer follow-up, but not with renal function. Conclusions: Unilateral MCDK usually has a favorable prognosis. However, the high rate of associated anomalies and the link between recurrent UTIs and reduced eGFR underscore the need for long-term follow-up, early detection of VUR, and prompt UTI management to preserve renal function.

Anahtar Kelimeler

• multicystic dysplastic kidney
• urinary tract infection
• vesicoureteral reflux
• compensatory hypertrophy
• renal function
• children

Multikistik displastik böbreği olan çocukların değerlendirilmesi: Tekrarlayan idrar yolu enfeksiyonlarının rolü

Öz

Amaç: Multikistik displastik böbrek (MKDB), çocuklarda en sık kistik böbrek hastalığıdır ve sıklıkla prenatal dönemde saptanır. Bu çalışmada, unilateral MKDB’li çocukların klinik özellikleri, eşlik eden anomalileri ve renal sonuçları değerlendirilmiş; tekrarlayan idrar yolu enfeksiyonlarının (İYE) böbrek fonksiyonu üzerindeki etkisine odaklanılmıştır. Yöntemler: 2019–2024 yılları arasında ultrasonografi ile unilateral MKDB tanısı alan 77 hastanın (1–18 yaş) retrospektif incelemesi yapıldı. Tahmini glomerüler filtrasyon hızı (eGFR), geçirilen İYE’ler, hipertansiyon ve karşı taraf böbrekte kompansatuvar hipertrofi varlığı dahil olmak üzere demografik, klinik ve laboratuvar verileri analiz edildi. Bulgular: Yetmiş yedi hastanın %64,9’u erkekti ve %55,8’inde hastalık sol taraftaydı. Antenatal tanı %77,9 oranında konuldu ve hastaların %85,7’si asemptomatikti. Üriner sistem ve ekstrarenal anomaliler sırasıyla %27,3 ve %40,3 oranında görüldü. İYE %31,2, tekrarlayan İYE ise %11,7 oranında saptandı. Vezikoüreteral reflü (VUR) genel olarak %9,1 oranında ve tekrarlayan İYE’si olan hastaların %33,3’ünde tespit edildi (p=0,031). Ortalama izlem süresi 7,2±4,4 yıl olup, ortalama eGFR 99,9±17,8 mL/dk/1,73 m² idi. eGFR <90 olan hastalarda tekrarlayan İYE daha sık görüldü (p=0,018) ve tekrarlayan İYE daha düşük eGFR ile ilişkiliydi (p=0,021). Karşı taraf kompansatuvar hipertrofisi %62,3 oranında mevcuttu ve daha ileri yaş ile daha uzun izlem süresiyle ilişkiliydi; ancak böbrek fonksiyonu ile ilişkili değildi. Sonuçlar: Unilateral MKDB genellikle iyi prognoza sahiptir. Ancak, eşlik eden anomalilerin yüksek oranı ve tekrarlayan İYE ile azalmış eGFR arasındaki anlamlı ilişki, böbrek fonksiyonunun korunması için uzun dönem izlemin gerekliliğini, VUR’un erken saptanması ve İYE’nin zamanında ve etkin bir şekilde tedavi edilmesi gerekliliğini ortaya koymaktadır.

Anahtar Kelimeler

• Multikistik displastik böbrek
• üriner sistem enfeksiyonu
• vezikoüreteral reflü
• kompansatuvar hipertrofi
• böbrek fonksiyonu
• çocuk

Kaynakça

1. Cardona-Grau D, Kogan BA. Update on Multicystic Dysplastic Kidney. Curr Urol Rep. 2015;16(10):67. Crossref
2. Baker HM, Jnah AJ. Supporting Infants with Multicystic Dysplastic Kidney Disease: A Comprehensive Approach. Neonatal Netw. 2024;43(5):286-294. Crossref
3. 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(6):1810-1818. Crossref
4. Alamir A, Al Rasheed SA, Al Qahtani AT, Almosa MS, Aljehani ND, Alanazi ED, et al. The Outcome of Multicystic Dysplastic Kidney Disease Patients at King Abdulaziz Medical City in Riyadh. Cureus. 2023;15(4):e37994. Crossref
5. Mashat SD, El-Desoky SM, Abdulaziz Kari J. Outcome of Multi-Cystic Dysplastic Kidneys in Children. Iran J Pediatr. 2015;25(5):e2991. Crossref
6. Rosenbaum DM, Korngold E, Teele RL. Sonographic assessment of renal length in normal children. AJR Am J Roentgenol. 1984;142(3):467-469. Crossref
7. Kim SY, Jang MS, Kim J. Impact of Third-Generation Cephalosporin Resistance on Recurrence in Children with Febrile Urinary Tract Infections. J Pers Med. 2022;12(5):773. Crossref
8. Schwartz GJ, Munoz 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):629-637. Crossref

9. Kuwertz-Broeking E, Brinkmann OA, Von Lengerke HJ, Sciuk J, Fruend S, Bulla M, et al. Unilateral multicystic dysplastic kidney: experience in children. BJU Int. 2004;93(3):388-392. Crossref
10. Sharada S, Vijayakumar M, Nageswaran P, Ekambaram S, Udani A. Multicystic dysplastic kidney: a retrospective study. Indian Pediatr. 2014;51(8):641-643. Crossref
11. Blachman-Braun R, Camp MM, Becerra MF, Guevara CG, Velasquez MC, Moscardi PRM, et al. Voiding Cystourethrogram in Children With Unilateral Multicystic Dysplastic Kidney: Is It Still necessary? Urology. 2020;139:156-160. Crossref
12. Meyers ML, Treece AL, Brown BP, Vemulakonda VM. Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 2020;50(13):1921-1933. Crossref
13. Akbalik Kara M, Taktak A, Alparslan C. Retrospective evaluation of the pediatric multicystic dysplastic kidney patients: experience of two centers from southeastern Turkey. Turk J Med Sci. 2021;51(3):1331-1337. Crossref
14. Mansoor O, Chandar J, Rodriguez MM, Abitbol CL, Seeherunvong W, Freundlich M, et al. Long-term risk of chronic kidney disease in unilateral multicystic dysplastic kidney. Pediatr Nephrol. 2011;26(4):597-603. Crossref
15. Hains DS, Bates CM, Ingraham S, Schwaderer AL. Management and etiology of the unilateral multicystic dysplastic kidney: a review. Pediatr Nephrol. 2009;24(2):233-241. Crossref
16. Shin YS, Kim SW, Heo JE, Lee MJ, Park J, Han SW, et al. Abnormal parenchymal features in the contralateral kidney of patients with multicystic dysplastic kidney. Pediatr Nephrol. 2025. Crossref
17. Zambaiti E, Sergio M, Baldanza F, Corrado C, Di Pace MR, Cimador M. Correlation between hypertrophy and risk of hypertension in congenital solitary functioning kidney. Pediatr Surg Int. 2019;35(1):167-174. Crossref
18. Sugaya K, Ogawa Y, Hatano T, Koyama Y, Miyazato T, Naito A, et al. Compensatory renal hypertrophy and changes of renal function following nephrectomy. Hinyokika Kiyo. 2000;46(4):235-240.
19. Gaither TW, Patel A, Patel C, Chuang KW, Cohen RA, Baskin LS. Natural History of Contralateral Hypertrophy in Patients with Multicystic Dysplastic Kidneys. J Urol. 2018;199(1):280-286. Crossref
20. Huettinger M, Bogner G, Fischer T, Tovilo K, Fazelnia C. Clinical outcome of children with prenatally diagnosed isolated unilateral multicystic dysplastic kidney. Eur J Obstet Gynecol Reprod Biol. 2025;312:114112. Crossref