Therapy of the Renal Complications of Sickle Cell Hemoglobinopathy

  • Carlos A. Vaamonde
  • James R. Oster


The possibility of offering renal replacement therapy to patients with sickle cell hemoglobinopathy has greatly increased the interest of nephrologists in the treatment of this disease. Sickle cell hemoglobinopathy has many potential renal-fluid–electrolyte complications (1). For many of these conditions, no particular therapy is indicated; for some, prevention is the most important aspect of management; and unfortunately, for several, therapeutic maneuvers are of relatively little benefit (Table 1). Not surprisingly, chronic dialysis and renal transplantation are the mainstays of therapy for the most serious renal abnormality—end-stage renal disease (ESRD).


Nephrotic Syndrome Sickle Cell Disease Sickle Cell Renal Tubular Acidosis Renal Plasma Flow 
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  1. 1.
    Vaamonde CA, Oster JR: Kidney in sickle cell hemoglobinopathies. In: SG Massry, RJ Glassock, eds, Textbook of Nephrology, 3rd ed. Williams and Wilkins, Baltimore, pp 751–756, 1989.Google Scholar
  2. 2.
    Powars DR, Elliott-Mills DD, Chan L, Niland J, Hiti AL, Opas LM, Johnson C: Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality. Ann Intern Med 115: 614–620, 1991.PubMedCrossRefGoogle Scholar
  3. 3.
    Kontesses P, Mayopoulou-Symvoulidis D, Symvoulidis A, Kontopoulou-Griva I: Renal involvement in sickle cell beta thalassemia: Nephron 61: 10–15, 1992.CrossRefGoogle Scholar
  4. 4.
    Falk RJ, Scheinman J, Phillips G, Orringer E, Johnson A, Jennette JC: Prevalence and pathologic features of sickle cell nephropathy and response to inhibition of angiotensinconverting enzyme. N Engl J Med 326: 910–915, 1992.PubMedCrossRefGoogle Scholar
  5. 5.
    Vaamonde CA, Oster JR, Strauss J: The kidney in sickle cell disease. In: WN Suki, G Eknoyan, eds, The Kidney in Systemic Disorders, 2nd ed. John Wiley & Sons, New York, pp 159–195, 1981.Google Scholar
  6. 6.
    Antonarakis SE, Boehm CD, Serjeant GR, Theisen CE, Dover GJ, Kazazian HH Jr: Origin of ßs globin gene in blacks: the contribution of recurrent mutation or gene conversion or both. Proc Natl Acad Sci USA 81: 853–856, 1984.PubMedCrossRefGoogle Scholar
  7. 7.
    Powars DR: Sickle cell anemia: beta-S-gene-cluster haplotypes as prognostic indicators of vital organ failure. Semin Hematol 28: 202–208, 1991.PubMedGoogle Scholar
  8. 8.
    Rodgers GP: Recent approaches to the treatment of sickle cell anemia. JAMA 265: 2097–2101, 1991.PubMedCrossRefGoogle Scholar
  9. 9.
    De Jong PE, Statius Van Eps LW: Sickle cell nephropathy: new insights into its pathophysiology. Kidney Int 27: 711–717, 1985.PubMedCrossRefGoogle Scholar
  10. 10.
    Vaamonde CA, Oster JR: Urinary potassium excretion in sickle-cell disorders. Miner Electrolyte Metab 2: 142–149, 1979.Google Scholar
  11. 11.
    Sklar AH, Campbell H, Caruana RI, Lightfoot BO, Gaier JG, Milner P: A population study of renal function in sickle cell anemia. Int J Artif Organs 13: 231–236, 1990.PubMedGoogle Scholar
  12. 12.
    Aparicio SAJR, Mojiminiyi S, Kay JDS, Shepstone BJ, de Ceulaer K, Serjeant GR: Measurement of glomerular filtration rate in homozygous sickle cell disease: a comparison of 51Cr-EDTA clearance, creatinine clearance, serum creatinine, and ßZ microglobulin. J Clin Pathol 43: 370–372, 1990.PubMedCrossRefGoogle Scholar
  13. 12a.
    Bank N, Aynedjian NS, Qiu J-N, Osei SY, Ahima RS, Fabry ME, Nagel RL: Renal nitric oxide synthases transgenic sickle cell mice. Kidney Int 50: 184–189, 1996.PubMedCrossRefGoogle Scholar
  14. 13.
    Moncada S, Higgs A: The L-arginine-nitric oxide pathway. N Engl J Med 329: 2002–2012, 1993.PubMedCrossRefGoogle Scholar
  15. 14.
    Allon M, Lawson L, Eckman JR, Delaney V, Bourke E: Effects of nonsteroidal anti-inflammatory drugs on renal function in sickle cell anemia. Kidney Int 34: 500–506, 1988.PubMedCrossRefGoogle Scholar
  16. 15.
    Oster JR, Lespier LE, Lee SM, Pellegrini EL, Vaamonde CA: Renal acidification in sickle cell disease. J Lab Clin Med 88: 389–401, 1976.PubMedGoogle Scholar
  17. 16.
    De Jong PE, De Jong-van den Berg LTW, Schouten H, Donker AJM, Statius van Eps LW: The influence of indomethacin on renal acidification in normal subjects and in patients with sickle cell anemia. Clin Nephrol 19: 259–264, 1983.PubMedGoogle Scholar
  18. 17.
    Oster JR, Lee SM, Lespier LE, Pellegrini EL, Vaamonde CA: Renal acidification in sickle cell trait. Arch Intern Med 136: 3035, 1976.Google Scholar
  19. 18.
    Baffle D, Itsarayoungyuen K, Arruda JAL, Kurtzman NA: Hyperkalemic hyperchloremic metabolic acidosis in sickle cell hemoglobinopathies. Am J Med 71: 188–192, 1982.Google Scholar
  20. 19.
    De Fronzo RA: Hyperkalemia and hyporeninemic hypoaldosteronism. Kidney Int 17: 118–134, 1980.CrossRefGoogle Scholar
  21. 20.
    De Fronzo RA, Taufield PA, Black H, McPhedran P, Cooke CR: Impaired renal tubxular potassium secretion in sickle cell disease. Ann Intern Med 90: 310–316, 1979.CrossRefGoogle Scholar
  22. 21.
    Aluoch JR: Renal and electrolyte profile in steady state sickle cell disease: observations in patients with sickle cell disease in the Netherlands. Trop Geograph Med 41: 128–132, 1989.Google Scholar
  23. 22.
    Oster JR, Lanier DC Jr, Vaamonde CA: Renal response to potassium loading in sickle cell trait. Arch Intern Med 140: 534536, 1980.Google Scholar
  24. 23.
    Yurbasiyan-Gurkan VA, Brewer GJ, Vander AJ, Guenther MJ, Prasad AS: Net renal tubular reabsorption of zinc in healthy man and impaired handling in sickle cell anemia. Am J Hematol 31: 87–90, 1989.CrossRefGoogle Scholar
  25. 24.
    De Jong PE, de Jong-van der Berg LTW, Sewrajsingh GS, Schouten H, Donker AJM, Statius van Eps LW: Beta-2microglobulin in sickle cell anemia—evidence of increased tubular reabsorption. Nephron 29: 138–141, 1981.PubMedCrossRefGoogle Scholar
  26. 25.
    Johnson CS, Giorgio AJ: Arterial blood pressure in adults with sickle cell disease. Arch Intern Med 144: 891–893, 1981.CrossRefGoogle Scholar
  27. 26.
    Grell GAC, Alleyne GAO, Serjeant GR: Blood pressure in adults with homozygous sickle cell disease. Lancet 2: 1166, 1981.PubMedCrossRefGoogle Scholar
  28. 27.
    Rodgers GP, Walker EC, Podgor MJ: Is “relative” hypertension a risk factor for vaso-occlussive complications in sickle cell disease? Am J Med Sci 305: 150–156, 1993.PubMedCrossRefGoogle Scholar
  29. 28.
    Lande IM, Glazer GM, Sarnaik S, Aisen A, Rucknagel D, Martel W: Sickle-cell nephropathy: MR imaging. Radiology 158: 379–383, 1986.PubMedGoogle Scholar
  30. 29.
    Osegbe DN: Haematuria and sickle cell disease: a report of 12 cases and review of the literature. Trop Geograph Med 42: 2227, 1991.Google Scholar
  31. 30.
    Baldree LA, Ault BH, Chesney CM, Stapleton FB: Intravenous desmopressin acetate in children with sickle cell trait and persistent macroscopic hematuria. Pediatrics 86: 238–243, 1990.PubMedGoogle Scholar
  32. 31.
    Qunibi WY: Renal autotransplantation for severe sickle-cell hematuria. Lancet 1: 236–237, 1988.PubMedCrossRefGoogle Scholar
  33. 32.
    Vaamonde CA: Renal papillary necrosis in sickle cell hemoglobinopathies. Semin Nephrol 4: 48–64, 1984.Google Scholar
  34. 32a.
    Davis CJ Jr, Mostofi FK, Sesterhenn IA: Renal medullary carcinoma. The seventh sickle cell nephropathy. Am J Surg Pathol 19: 1–11, 1995.PubMedCrossRefGoogle Scholar
  35. 32b.
    Davidson AJ, Choyke PL, Hartman DS, Davis CJ Jr.: Renal medullary carcinoma associated with sickle cell trait: Radio-logic findings. Radiology 195: 83–85, 1995.PubMedGoogle Scholar
  36. 33.
    Whalley PJ, Martin FG, Pritchard JA: Sickle cell trait and urinary tract infection during pregnancy. JAMA 189: 903–906, 1964.PubMedCrossRefGoogle Scholar
  37. 34.
    Amin UF, Ragbeer MMS: The prevalence of pyelonephritis among sicklers and non-sicklers in an autopsy population. West Indian Med J 21: 166, 1984.Google Scholar
  38. 35.
    Sklar AH, Perez JC, Harp RJ, Caruana RJ: Acute renal failure in sickle cell anemia. Int J Artif Organs 13: 347–351, 1990.PubMedGoogle Scholar
  39. 36.
    Diggs LW: The sickle cell trait in relation to the training and assignment of duties in the Armed Forces: III. Hypostheruria, hematuria, sudden death, rhabdomyolysis and acute tubular necrosis. Aviat Space Environ Med 55: 358–364, 1984.PubMedGoogle Scholar
  40. 37.
    Helzlsouer KJ, Hayden FG, Rogol AD: Severe metabolic complications in a cross-country runner with sickle cell trait. JAMA 249: 777–779, 1983.PubMedCrossRefGoogle Scholar
  41. 38.
    Kelly CJ, Singer I: Acute renal failure in sickle-cell disease. Am J Kidney Dis 8: 146–150, 1986.PubMedGoogle Scholar
  42. 38a.
    Walker TM, Beardsall K, Thomas PW, Serjeant GR: Renal length in sickle cell disease: Observations from a cohort study. Clin Nephrol 46: 384–388, 1996.PubMedGoogle Scholar
  43. 39.
    Halankar A, Frumkin E, Dunn I, Seligman BS, Dosik H: Renal findings in sickle cell anemia. In: JI Hercules, AN Schechter, WA Eaton, RE Jackson, eds, Proceedings First National Symposium on Sickle cell Disease, Washington, DC, June 1974, DHEW. Publication No. 75–723, pp 311–312, 1974.Google Scholar
  44. 40.
    Tejani A, Phadke K, Adamson O, Nicastri A, Chen CK, Sen D: Renal lesions in sickle cell nephropathy in children. Nephron 39: 352–355, 1985.PubMedCrossRefGoogle Scholar
  45. 41.
    Pardo V, Strauss J, Kramer H, Czawa T, McIntosh RM: Nephropathy associated with sickle cell anemia: an autologous immune complex nephritis II. Clinicopathologic study of seven patients. Am J Med 59: 560–569, 1975.CrossRefGoogle Scholar
  46. 42.
    Bathena DB, Sondheimer JH: The glomerulopathy of homozygous sickle hemoglobin (SS) disease: morphology and pathogenesis. J Am Soc Nephrol 1: 1241–1252, 1991.Google Scholar
  47. 42a.
    Guasch A, Cua M, Mitch WE: Early detection and the course of glomerular injury in patients with sickle cell anemia. Kidney lnt 49: 786–791, 1996.CrossRefGoogle Scholar
  48. 43.
    Bakir AA, Hathiwala SC, Ainis H, Hryhorczuk DO, Rhee HL, Levy PS, Dunea G: Prognosis of the nephrotic syndrome in sickle glomerulopathy. A retrospective study. Am J Nephrol 7: 110–115, 1987.PubMedCrossRefGoogle Scholar
  49. 44.
    Morgan AG, Sergeant GR: Renal function in patients over 40 with homozygous sickle cell disease. Br Med J 282: 181–183, 1981.Google Scholar
  50. 45.
    U.S. Renal Data System: URSDS 1996 Annual Data Report. NIH, NIDDKD, Bethesda, MD, pp 1–164, April 1996.Google Scholar
  51. 46.
    U.S Renal Data System: URSDS 1993 Annual Data Report. NIH, NIDDKD, Bethesda, MD, pp 1–130, March 1993.Google Scholar
  52. 47.
    Nissenson AR, Por FK: Outcome of end-stage renal disease in patients with rare causes of renal failure. I. Inherited and metabolic disorders. Q J Med 73: 1055–1062, 1989.PubMedGoogle Scholar
  53. 48.
    Tomson CR: End-stage renal disease in sickle cell disease: future directions. Postgrad Med J 68: 775–778, 1992.PubMedCrossRefGoogle Scholar
  54. 49.
    Vichinsky EP, Earles A, Johnson RA, Hoag S, Williams A, Lubion B: Alloinmmunization in sickle cell anemia and transfusion of racially unmatched blood. N Engl J Med 322: 1617 1621, 1990.Google Scholar
  55. 50.
    Roger SD, MacDougall IC, Thuraisingham RC, Raine AEG: Erythropoietin in anemia of renal failure in sickle cell disease. N Engl J Med 325: 1175–1176, 1991.PubMedCrossRefGoogle Scholar
  56. 51.
    Tomson CR, Edmunds ME, Chambers K, Brucknell S, Freehally J, Walls J: Effect of recombinant human erythropoietin on erythropoiesis in homozygous sickle cell anaemia and renal failure. Nephrol Dial Transplant 7: 817–821, 1992.PubMedGoogle Scholar
  57. 52.
    Chatterjee SN: National study in natural history of renal allografts in sickle-cell disease or trait: A second report. Transplant Proc 19: 33–35, 1987.PubMedGoogle Scholar
  58. 52a..
    Montgomery R, Zibari G, Hill GS, Ratner LE: Renal transplantation in patients with sickle cell nephropaty. Transplantation 58: 618–620, 1994.PubMedCrossRefGoogle Scholar
  59. 53.
    Barber WH, Deierhoi MH, Julian BA, Curtis JJ, Luke RG, Alexander RW, Diethelm AG: Renal transplantation in sickle cell anemia and sickle disease. Clin Transplant 1: 169–175, 1987.Google Scholar

Copyright information

© Springer Science+Business Media New York 1998

Authors and Affiliations

  • Carlos A. Vaamonde
    • 1
  • James R. Oster
    • 1
  1. 1.Nephrology Section and Research Services, Veterans Affairs Medical Center, Department of MedicineUniversity of Miami School of MedicineMiamiUSA

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