Prognostic Insights into Congenital Solitary Functioning Kidneys among the Turkish Pediatric Population: A Comparative Study of Renal Agenesis and Multicystic Dysplastic Kidney Disease

Öz

Objective: Our study aims to assess the outcomes of solitary functioning kidneys (SFKs) resulting from unilateral renal agenesis (URA) and multicystic dysplastic kidney (MCDK) in order to identify the factors influencing kidney damage in these patients and to elucidate the potential contrasts between these two conditions. Methods: The study retrospectively analyzes 154 pediatric patients (ages 0-18) with SFK treated at a tertiary center in Türkiye. Results: Among the 154 SFK patients, 91 are male, 74 were diagnosed with MCDK, and 80 with URA. The median age is one month at diagnosis and 69.5 months at the last follow-up. Most MCDK cases were identified antenatally, while URA was most commonly detected incidentally. Congenital anomalies of the kidney and urinary tract (CAKUT) in the functioning kidney were identified in 21.6% of MCDK patients and 7.5% of URA patients, with a significantly higher occurrence in MCDK patients (p = 0.012). Vesicoureteral Reflux (VUR) was the most prevalent CAKUT, occurring in 45% of patients. A low glomerular filtration rate (GFR) was observed in 12.4% of patients, and 15% exhibited signs of renal damage. No significant disparity was found in low GFR or kidney damage between MCDK and URA patients. Those with a low GFR showed increased rates of hydronephrosis, CAKUT in the functioning kidney, recurrent urinary tract infections (UTIs), renal scarring, hypoplastic kidneys, proteinuria, and hypertension (HT). Conclusions: The findings underline that CAKUT in the functioning kidney, recurrent UTIs, and renal scarring significantly influence GFR and kidney injury. VCUG is useful in select cases for identifying CAKUT, especially VUR. No significant distinction was observed between MCDK and URA concerning the eventual renal injury.

Anahtar Kelimeler

• Solitary functioning kidney
• unilateral renal agenesis
• multicystic dysplastic kidney disease
• chronic kidney disease

Kaynakça

1. Harambat J, van Stralen KJ, Kim JJ, Tizard EJ. Epidemiology of chronic kidney disease in children. Pediatr Nephrol. 2012;27(3):363-73. google scholar
2. Brenner BM, Garcia DL, Anderson S. Glomeruli and blood pressure. Less of one, more the other? Am J Hypertens. 1988;1(4 Pt 1):335-47. google scholar
3. Hostetter TH, Olson JL, Rennke HG, Venkatachalam MA, Brenner BM. Hyperfiltration in remnant nephrons: a potentially adverse response to renal ablation. Am J Physiol. 1981;241(1):F85-93. google scholar
4. Schreuder MF, Nyengaard JR, Fodor M, van Wijk JA, Delemarre-van de Waal HA. Glomerular number and function are influenced by spontaneous and induced low birth weight in rats. J Am Soc Nephrol. 2005;16(10):2913-9. google scholar
5. Schreuder MF, van Wijk JA, Delemarre-van de Waal HA. Intrauterine growth restriction increases blood pressure and central pulse pressure measured with telemetry in aging rats. J Hypertens. 2006;24(7):1337-43. google scholar
6. Schreuder MF, Langemeijer ME, Bokenkamp A, Delemarre-Van de Waal HA, Van Wijk JA. Hypertension and microalbuminuria in children with congenital solitary kidneys. J Paediatr Child Health. 2008;44(6):363-8. google scholar
7. Shirzai A, Yildiz N, Biyikli N, Ustunsoy S, Benzer M, Alpay H. Is microalbuminuria a risk factor for hypertension in children with solitary kidney? Pediatr Nephrol. 2014;29(2):283-8. google scholar
8. Urisarri A, Gil M, Mandia N, Aldamiz-Echevarria L, Iria R, Gonzalez-Lamuno D, et al. Retrospective study to identify risk factors for chronic kidney disease in children with congenital solitary functioning kidney detected by neonatal renal ultrasound screening. Medicine (Baltimore). 2018;97(32):e11819. google scholar

9. Westland R, Schreuder MF, Bökenkamp A, Spreeuwenberg MD, van Wijk JA. Renal injury in children with a solitary functioning kidney--the KIMONO study. Nephrol Dial Transplant. 2011;26(5):1533-41. google scholar Steiger J. Why did mother nature provide us with two kidneys? Nephrol Dial Transplant. 2011;26(7):2076-8. google scholar
10. Marzuillo P, Guarino S, Di Sessa A, Rambaldi PF, Reginelli A, Vacca G, et al. Congenital Solitary Kidney from Birth to Adulthood. J Urol. 2021;205(5):1466-75. google scholar
11. Sanna-Cherchi S, Ravani P, Corbani V, Parodi S, Haupt R, Piaggio G, et al. Renal outcome in patients with congenital anomalies of the kidney and urinary tract. Kidney International. 2009;76(5):528-33. google scholar
12. Xu Q, Wu H, Zhou L, Xie J, Zhang W, Yu H, et al. The clinical characteristics of Chinese patients with unilateral renal agenesis. Clin Exp Nephrol. 2019;23(6):792-8. google scholar
13. 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):1810-8. google scholar
14. Matsell DG, Bao C, Po White T, Chan E, Matsell E, Cojocaru D, et al. Outcomes of solitary functioning kidneys-renal agenesis is different than multicystic dysplastic kidney disease. Pediatr Nephrol. 2021;36(11):3673-80. google scholar
15. Flynn JT, Kaelber DC, Baker-Smith CM, Blowey D, Carroll AE, Daniels SR, et al. Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents. Pediatrics. 2017;140(3). google scholar
16. 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):629-37. google scholar
17. 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. google scholar Coulthard MG. Maturation of glomerular filtration in preterm and mature babies. Early Hum Dev. 1985;11(3-4):281-92. google scholar
18. Hogg RJ, Furth S, Lemley KV, Portman R, Schwartz GJ, Coresh J, et al. National Kidney Foundation’s Kidney Disease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics. 2003;111(6 Pt 1):1416-21. google scholar
19. Pottel H, Adebayo OC, Nkoy AB, Delanaye P. Glomerular hyperfiltration: part 1 - defining the threshold - is the sky the limit? Pediatr Nephrol. 2022. google scholar
20. Nguyen HT, Benson CB, Bromley B, Campbell JB, Chow J, Coleman B, et al. Multidisciplinary consensus on the classification of prenatal and postnatal urinary tract dilation (UTD classification system). J Pediatr Urol. 2014;10(6):982-98. google scholar
21. Obrycki L, Sarnecki J, Lichosik M, Sopinska M, Placzynska M, Stanczyk M, et al. Kidney length normative values - new percentiles by age and body surface area in Central European children and adolescents. Pediatr Nephrol. 2023;38(4):1187-93. google scholar
22. 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. google scholar
23. Ezel ÇElakil M, AktaŞ ÖZgÜR M, Bek K. Agenezi veya Multikistik Displazi: Doğuştan Tek Böbrekli Çocuklarda Hangisi Daha Kötü? Kocaeli Üniversitesi Sağlık Bilimleri Dergisi. 2020;6(2):113-7. google scholar
24. Robson WL, Leung AK, Thomason MA. Multicystic dysplasia of the kidney. Clin Pediatr (Phila). 1995;34(1):32-40. google scholar
25. Schreuder MF. Unilateral anomalies of kidney development: why is left not right? Kidney Int. 2011;80(7):740-5. google scholar
26. Groen In ‘t Woud S, Roeleveld N, Westland R, Renkema KY, Steffens MG, Gracchi V, et al. Uncovering risk factors for kidney injury in children with a solitary functioning kidney. Kidney Int. 2023;103(1):156-65. google scholar
27. Krzemien G, Roszkowska-Blaim M, Kostro I, Wojnar J, Karpinska M, Sekowska R. Urological anomalies in children with renal agenesis or multicystic dysplastic kidney. J Appl Genet. 2006;47(2):171-6. google scholar
28. Merrot T, Lumenta DB, Tercier S, Morisson-Lacombes G, Guys JM, Alessandrini P. Multicystic dysplastic kidney with ipsilateral abnormalities of genitourinary tract: experience in children. Urology. 2006;67(3):603-7. google scholar
29. Kara A, Gurgoze MK, Aydin M, Koc ZP. Clinical features of children with multicystic dysplastic kidney. Pediatr Int. 2018;60(8):750-4. google scholar
30. Baker R, Maxted W, Maylath J, Shuman I. Relation of age, sex, and infection to reflux: Data indicating high spontaneous cure rate in pediatric patients. J Urol. 1966;95(1):27-32. google scholar
31. Elder JS, Peters CA, Arant BS, Jr., Ewalt DH, Hawtrey CE, Hurwitz RS, et al. Pediatric Vesicoureteral Reflux Guidelines Panel summary report on the management of primary vesicoureteral reflux in children. J Urol. 1997;157(5):1846-51. google scholar
32. Kolvek G, Podracka L, Rosenberger J, Stewart RE, van Dijk JP, Reijneveld SA. Solitary functioning kidney in children--a follow-up study. Kidney Blood Press Res. 2014;39(4):272-8. google scholar
33. Schreuder MF. Life with one kidney. Pediatr Nephrol. 2018;33(4):595-604. google scholar
34. 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):280-6. google scholar
35. Sharma M, Sharma R, McCarthy ET, Savin VJ, Srivastava T. Hyperfiltration-associated biomechanical forces in glomerular injury and response: Potential role for eicosanoids. Prostaglandins Other Lipid Mediat. 2017;132:59-68. google scholar