American Journal of Kidney Diseases
Volume 54, Issue 3 , Pages 450-458 , September 2009

Kidney Function and Volume Progression in Unilateral Autosomal Dominant Polycystic Kidney Disease With Contralateral Renal Agenesis or Hypoplasia: A Case Series

  • Diane Poster, MD

      Affiliations

    • Division of Nephrology, University Hospital, Zürich, Switzerland
    • ,
  • Andreas D. Kistler, MD

      Affiliations

    • Division of Nephrology, University Hospital, Zürich, Switzerland
    • ,
  • Fabienne Krauer

      Affiliations

    • Division of Nephrology, University Hospital, Zürich, Switzerland
    • ,
  • Jon D. Blumenfeld, MD

      Affiliations

    • The Rogosin Institute and Department of Medicine, Weill Cornell Medical College, New York, NY
    • ,
  • Hanna Rennert, PhD

      Affiliations

    • Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY
    • ,
  • Dominik Weishaupt, MD

      Affiliations

    • Division of Diagnostic Radiology, University Hospital, Zürich, Switzerland
    • ,
  • Rudolf P. Wüthrich, MD

      Affiliations

    • Division of Nephrology, University Hospital, Zürich, Switzerland
    • ,
  • Andreas L. Serra, MD

      Affiliations

    • Division of Nephrology, University Hospital, Zürich, Switzerland
    • Corresponding Author InformationAddress correspondence to Andreas L. Serra, MD, Division of Nephrology, University Hospital, Rämistrasse 100, 8091 Zürich, Switzerland

Received 12 November 2008 ,Accepted 11 March 2009.

References 

  1. Dalgaard OZ. Bilateral polycystic disease of the kidneys; A follow-up of two hundred and eighty-four patients and their families. Acta Med Scand Suppl. 1957;328:S1–S255
  2. Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. 2007;369:1287–1301
  3. Zeier M, Geberth S, Gonzalo A, Chauveau D, Grunfeld JP, Ritz E. The effect of uninephrectomy on progression of renal failure in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1992;3:1119–1123
  4. Bear RA. Solitary kidney affected with polycystic disease: A report of 2 cases. J Urol. 1974;111:566–567
  5. Todorov VV. The diagnostic dilemma of the unilateral cystic kidney-ADPKD with aplasia of one kidney. Nephrol Dial Transplant. 1999;14:2775;(ltr)
  6. Jeong GH, Park BS, Jeong TK, et al. Unilateral autosomal dominant polycystic kidney disease with contralateral renal agenesis: A case report. J Korean Med Sci. 2003;18:284–286
  7. Sirvent AE, Enriquez R, Ardoy F, Amoros F, Gonzalez C, Reyes A. Autosomal dominant polycystic kidney disease with congenital absence of contralateral kidney. Int Urol Nephrol. 2006;38:773–774
  8. Hwang DY, Ahn C, Lee JG, et al. Unilateral renal cystic disease in adults. Nephrol Dial Transplant. 1999;14:1999–2003
  9. Ravine D, Gibson RN, Walker RG, Sheffield LJ, Kincaid-Smith P, Danks DM. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. Lancet. 1994;343:824–827
  10. Kistler A, Poster D, Krauer F, et al. Kidney volume progression in ADPKD is detectable within 6 months by magnetic resonance imaging without gadolinium. Kidney Int. 2009;75:235–241
  11. Cockcroft DW, Gault MH. Prediction of creatinine clearance from serum creatinine. Nephron. 1976;16:31–41
  12. Tan YC, Blumenfeld JD, Anghel R, et al. Novel method for genomic analysis of PKD1 and PKD2 mutations in autosomal dominant polycystic kidney disease. Hum Mutat. 2009;30:264–273
  13. Serra AL, Kistler AD, Poster D, et al. Clinical proof-of-concept trial to assess the therapeutic effect of sirolimus in patients with autosomal dominant polycystic kidney disease: SUISSE ADPKD study. BMC Nephrol. 2007;8:13
  14. Grantham JJ, Cook LT, Torres VE, et al. Determinants of renal volume in autosomal-dominant polycystic kidney disease. Kidney Int. 2008;73:108–116
  15. Campbell MF. Congenital absence of one kidney: Unilateral renal agenesis. Ann Surg. 1928;88:1039–1044
  16. Sakuma T, Ogawa O. [Ultrasonographic screening in healthy 3-month-old children for congenital malformations of the urinary tract]. Nippon Hinyokika Gakkai Zasshi. 1998;89:468–476
  17. Jojart G, Nagy G. [Ultrasonic screening of neonates]. Orv Hetil. 1996;137:623–627
  18. Kiprov DD, Colvin RB, McCluskey RT. Focal and segmental glomerulosclerosis and porteinuria associated with unilateral renal agenesis. Lab Invest. 1982;46:275–281
  19. Yuksel A, Batukan C. Sonographic findings of fetuses with an empty renal fossa and normal amniotic fluid volume. Fetal Diagn Ther. 2004;19:525–532
  20. Woolf AS, Hillman KA. Unilateral renal agenesis and the congenital solitary functioning kidney: Developmental, genetic and clinical perspectives. BJU Int. 2007;99:17–21
  21. MacDermot KD, Saggar-Malik AK, Economides DL, Jeffery S. Prenatal diagnosis of autosomal dominant polycystic kidney disease (PKD1) presenting in utero and prognosis for very early onset disease. J Med Genet. 1998;35:13–16
  22. Hill LM, Nowak A, Hartle R, Tush B. Fetal compensatory renal hypertrophy with a unilateral functioning kidney. Ultrasound Obstet Gynecol. 2000;15:191–193
  23. Grantham JJ, Torres VE, Chapman AB, et al. Volume progression in polycystic kidney disease. N Engl J Med. 2006;354:2122–2130
  24. Baudoin P, Provoost AP, Molenaar JC. Renal function up to 50 years after unilateral nephrectomy in childhood. Am J Kidney Dis. 1993;21:603–611
  25. Robitaille P, Mongeau JG, Lortie L, Sinnassamy P. Long-term follow-up of patients who underwent unilateral nephrectomy in childhood. Lancet. 1985;1:1297–1299
  26. Slack TK, Wilson DM. Normal renal function: CIN and CPAH in healthy donors before and after nephrectomy. Mayo Clin Proc. 1976;51:296–300
  27. Strandgaard S, Kamper A, Skaarup P, Holstein-Rathlou NH, Leyssac PP, Munck O. Changes in glomerular filtration rate, lithium clearance and plasma protein clearances in the early phase after unilateral nephrectomy in living healthy renal transplant donors. Clin Sci (Lond). 1988;75:655–659
  28. Shimamura T, Morrison AB. A progressive glomerulosclerosis occurring in partial five-sixths nephrectomized rats. Am J Pathol. 1975;79:95–106
  29. 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:F85–F93
  30. Sommerer C, Morath C, Andrassy J, Zeier M. The long-term consequences of living-related or unrelated kidney donation. Nephrol Dial Transplant. 2004;19(suppl 4):Siv45–Siv47
  31. Simon J, Zamora I, Mendizabal S, Castel V, Lurbe A. Glomerulotubular balance and functional compensation in nephrectomized children. Nephron. 1982;31:203–208
  32. Wikstad I, Pettersson BA, Elinder G, Sokucu S, Aperia A. A comparative study of size and function of the remnant kidney in patients nephrectomized in childhood for Wilms' tumor and hydronephrosis. Acta Paediatr Scand. 1986;75:408–414
  33. Argueso LR, Ritchey ML, Boyle ET, Milliner DS, Bergstralh EJ, Kramer SA. Prognosis of children with solitary kidney after unilateral nephrectomy. J Urol. 1992;148:747–751
  34. Gonzalez E, Gutierrez E, Morales E, et al. Factors influencing the progression of renal damage in patients with unilateral renal agenesis and remnant kidney. Kidney Int. 2005;68:263–270
  35. Nielsen FS, Gall MA, Parving HH. Acquired oligonephropathy and diabetic nephropathy. Am J Kidney Dis. 1995;26:898–903
  36. Gretz N. Accelerated renal death after unilateral nephrectomy in a rat strain suffering from autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1994;4:1925–1926
  37. Kang SH, Oyama TT, Kennefick TM, Thompson MM, Anderson S. Impaired adaptation to renal mass reduction in the polycystic rat. Am J Kidney Dis. 2000;35:923–929
  38. Zeier M, Wagner J, Ritz E. Effect of uninephrectomy in ADPKD. J Am Soc Nephrol. 1995;5:1535–1536
  39. Seeman T, Patzer L, John U, et al. Blood pressure, renal function, and proteinuria in children with unilateral renal agenesis. Kidney Blood Press Res. 2006;29:210–215
  40. Argueso LR, Ritchey ML, Boyle ET, Milliner DS, Bergstralh EJ, Kramer SA. Prognosis of patients with unilateral renal agenesis. Pediatr Nephrol. 1992;6:412–416
  41. Wikstad I, Celsi G, Larsson L, Herin P, Aperia A. Kidney function in adults born with unilateral renal agenesis or nephrectomized in childhood. Pediatr Nephrol. 1988;2:177–182

 Originally published online as doi: 10.1053/j.ajkd.2009.03.020 on June 11, 2009.

 D.P. and A.D.K. contributed equally to this article.

PII: S0272-6386(09)00650-7

doi: 10.1053/j.ajkd.2009.03.020

American Journal of Kidney Diseases
Volume 54, Issue 3 , Pages 450-458 , September 2009

Article Tools

* Image Usage & Resolution