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Journal of Applied Genetics

, Volume 47, Issue 2, pp 171–176 | Cite as

Urological anomalies in children with renal agenesis or multicystic dysplastic kidney

  • Grażyna Krzemień
  • Maria Roszkowska-Blaim
  • Izabella Kostro
  • Julita Wojnar
  • Monika Karpińska
  • Renata Sękowska
Article

Abstract

This study aimed to determine the frequency of associated urological abnormalities in children with unilateral renal agenesis (RA) or multicystic dysplastic kidney (MCDK). In total, 38 children (10 girls, 28 boys) were studied: 21 with RA and 17 with MCDK. In 14 children (37%) anomalies of the urinary tract were suspected prenatally in ultrasound studies. In the remaining 24 children the diagnosis of RA/MCDK was made postnatally: in 13 (34%) in the first 7 days of life, in 11 (29%) at the age of 8 days to 34 months, mean 10.6 ± 8.05 months. Voiding cystourethrography was done in 36 (95%) children, the isotopic99mTc-EC/DMSA scan of the kidney in 29 (67%), and urography in 8. Urological anomalies were present in 11 (29%) children: in 7 (33%) with RA and in 4 (24%) with MCDK. Vesicoureteral reflux was diagnosed in 8 children: grade II in 4, III in 3, and IV in 1 (in 1 child to duplicated, in 1 to ectopic kidney); ureterovesical junction obstruction in 2 (9.5%); and ureteropelvic junction obstruction in 1 (4.8%). Among them, 2 children demanded surgery on the contralateral urinary tract: pyeloplasty in 1, antireflux procedure in 1; while 9 children were treated conservatively. Compensatory hypertrophy of the contralateral kidney was found in 90% of children. Thus due to an increased risk of pathological changes in the single functioning kidney, lifelong nephrological care is recommended in patients with unilateral RA/MCDK.

Key words

multicystic dysplastic kidney renal agenesis urological anomalies 

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References

  1. Abidari JM, Park KH, Kennedy WA, Shortliffe LD, 2002. Serial follow-up of the contralateral renal size in children with multicystic dysplastic kidney. J Urol 168: 1821–1825.CrossRefPubMedGoogle Scholar
  2. Argueso LR, Ritchey ML, Boyle ET Jr, Milliner DS, Bergstralh EJ, Kramer SA, 1992. Prognosis of patients with unilateral agenesis. Pediatr Nephrol 6: 412–416.CrossRefPubMedGoogle Scholar
  3. Beckwith JB, 1992. Should asymptomatic unilateral multicystic dysplastic kidneys be removed because of future risk of neoplasia? Pediatr Nephrol 6: 511.CrossRefPubMedGoogle Scholar
  4. Beckwith JB: 1997. Editorial comment. J Urol 158: 2259.CrossRefGoogle Scholar
  5. Belk RA, Thomas DF, Mueller RF, Godbole P, Markham AF, Weston MJ, 2002. A family study and the natural history of prenatally detected unilateral multicystic dysplastic kidney J Urol 167: 666–669.CrossRefPubMedGoogle Scholar
  6. Cascio S, Paran S, Puri P, 1999. Associated urological anomalies in children with unilateral renal agenesis. J Urol 162: 1081–1083.CrossRefPubMedGoogle Scholar
  7. Dinkel E, Ertel M, Dittrich M, Peters H, Schulte-Wissermann H, 1985. Kidney size in childhood. Sonographical growth charts for kidney length and volume. Pediatr Radiol 15: 38–43.CrossRefPubMedGoogle Scholar
  8. Doray B, Gasser B, Reinartz I, Stoll C, 1999, Hereditary renal adysplasia in a three generation family. Genet Couns 10: 251–257.PubMedGoogle Scholar
  9. Farnham SB, Adams MC, Brock JW., Pope JC, 2005. Pediatric urological causes of hypertension. J Urol 173: 697–704.CrossRefPubMedGoogle Scholar
  10. Feldenberg LR, Siegiel NJ, 2000. Clinical course and outcome for children with multicystic dysplastic kidneys. Pediatr Nephrol 14: 1098–1101.CrossRefPubMedGoogle Scholar
  11. Goldraich NP, Goldraich IH, 1984. Reflux nephropathy. The clinical picture in south Brazilian children. Contr Nephrol 39: 52–67.Google Scholar
  12. John U, Rudnik-Schoneborn S, Zerres K, Misselwitz J, 1998. Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol 12: 567–571.CrossRefPubMedGoogle Scholar
  13. Kaneyama K, Yamataka A, Satake S, Yanai T, Lane GJ, Kaneko K, et al. 2004. Associated urologic anomalies in children with solitary kidney. J Pediatr Surg 39: 85–87.CrossRefPubMedGoogle Scholar
  14. Kuwertz-Broeking E, Brinkmann OA, Von Lengerke HJ, Sciuk J, Fruend S, Bulla M, et al. 2004. Unilateral multicystic dysplastic kidney: experience in children. BJU Int 93: 388–392.CrossRefPubMedGoogle Scholar
  15. Miller DC, Rumohr JA, Dunn RL, Bloom DA, Park JM, 2004. What is the fate of the refluxing contralateral kidney in children with multicystic dysplastic kidney? J Urol 172: 1630–1634.CrossRefPubMedGoogle Scholar
  16. Moerman P, Fryns JP, Sastrowijoto SH, Vandenberghe K, Lauweryns JM, 1994. Hereditary renal adysplasia: new observations and hypothesis. Pediatr Pathol 14: 405–410.CrossRefPubMedGoogle Scholar
  17. Morse RP, Rawnsley E, Crowe HC, Martin-Padilla M, Graham JM Jr, 1987. Bilateral renal agenesis in three consecutive siblings Prenat Diagn 7: 573–579.CrossRefPubMedGoogle Scholar
  18. McPherson R, Carey J, Kramer A, Hall JG, Pauli RM, Schimke RN, Tasin MH, 1987. Am J Med Genet 26: 863–872.CrossRefPubMedGoogle Scholar
  19. [OMIM™] Online Mendelian Inheritance in Man 2000. McKusick-Nathans Institute for Genetic Medicine, John Hopkins University (Baltimore, MD) and National Center for Biotechnology Information, National Library of Medicine. Bethesda, MD: www.ncbi.nlm.nih.gov/omim/.Google Scholar
  20. Parikh CR, McCall D, Engelman C, Schrier RW, 2002. Congenital renal agenesis: case-control analysis of birth characteristics. Am J Kidney Dis 39: 689–694.CrossRefPubMedGoogle Scholar
  21. Rabelo EA, Oliveira EA, Silva GS, Pezzuti IL, Tatsuo ES, 2005. Predictive factors of ultrasonographic involution of prenatally detected multicystic dysplastic kidney. BJU Int 95: 868–871.CrossRefPubMedGoogle Scholar
  22. Ranke A, Schmitt M, Didier F, Droulle P, 2001. Antenatal diagnosis of Multicystic Renal Dysplasia. Eur J Pediatr Surg 11: 246–254.CrossRefPubMedGoogle Scholar
  23. Selzman AA, Elder JS, 1995. Contralateral vesicoureteral reflux in children with a multicystic kidney. J Urol 153: 1252–1254.CrossRefPubMedGoogle Scholar
  24. Ylinen E, Ahonen S, Ala-Houhala M, Wikstrom S, 2004. Nephrectomy for multicystic dysplastic kidney: if and when? Urology 63: 768–771.CrossRefPubMedGoogle Scholar

Copyright information

© Institute of Plant Genetics, Polish Academy of Sciences, Poznan 2006

Authors and Affiliations

  • Grażyna Krzemień
    • 1
  • Maria Roszkowska-Blaim
    • 1
  • Izabella Kostro
    • 1
  • Julita Wojnar
    • 1
  • Monika Karpińska
    • 1
  • Renata Sękowska
    • 1
  1. 1.Department of Pediatrics and NephrologyMedical University of WarszawaWarszawaPoland

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