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Renal Manifestations of Sickle Cell Disease

Renal Manifestations of Sickle Cell Disease Abstract Cortical morphologic changes in kidneys of patients with sickle cell anemia include vascular dilatation and engorgement of vessels with red blood cells, glomerular enlargement, hypertrophy and hypercellularity of glomerular mesangial cells, and iron deposits in various glomerular and tubular structures. Occasionally, glomerular sclerosis develops, with resultant end-stage renal failure. In the renal medulla, vascular congestion, edema, focal scarring, and occasional papillary necrosis are seen. Vascular dilatation and engorgement also occur in renal pelvis submucosa, where vessel rupture may cause hematuria. Young patients with sickle cell anemia have increased total renal blood flow while medullary blood flow is probably decreased. Glomerular filtration rate is also increased in young patients. A renal concentrating defect is reversible in younger age groups, becoming irreversible in older patients. References 1. Welt LG, Lyle CB Jr: The kidney in sickle cell anemia , in Strauss MB, Welt LG (eds): Diseases of the Kidney , ed 2. Boston, Little Brown & Co, 1971, pp 1207-1213. 2. Margolies MP: Sickle cell anemia . Medicine 30:357-443, 1951.Crossref 3. Schlitt LE, Keitel HG: Renal manifestations of sickle cell disease: A review . Am J Med Sci 239:773-778, 1960.Crossref 4. Pitcock JA, et al: Early renal changes in sickle cell anemia . Arch Pathol 90:403-410, 1970. 5. Bernstein J, Whitten CF: A histologic appraisal of the kidney in sickle cell anemia . Arch Pathol 70:407-418, 1960. 6. Etteldorf JN, et al: Renal hemodynamic studies in adults with sickle cell anemia . Am J Med 18:243-248, 1955.Crossref 7. Mostofi KF, Vorder Bruegge CF, Diggs LW: Lesions in kidneys removed for unilateral hematuria in sickle cell disease . Arch Pathol 63:336-351, 1957. 8. Statius Van Eps LW, et al: Nature of concentrating defect in sickle cell nephropathy . Lancet 1:450-452, 1970.Crossref 9. Hatch FE, et al: Renal circulatory studies in young adults with sickle cell anemia . J Lab Clin Med 76:632-640, 1970. 10. McCoy RC: Ultrastructural alterations in the kidney of patients with sickle cell disease and the nephrotic syndrome . Lab Invest 21:85-94, 1969. 11. Ellis JT: Glomerular lesions and the nephrotic syndrome in rabbits given saccharated iron oxide intravenously . J Exp Med 103:127-143, 1956.Crossref 12. Antonovych TT: Ultrastructural changes in glomeruli of patients with sickle cell disease and nephrotic syndrome , in Abstracts, Fifth Annual Meeting . Washington, DC, The American Society of Nephrology, 1971, p 3. 13. Walker BR, et al: Glomerular lesions in sickle cell nephropathy . JAMA 215:437-440, 1971.Crossref 14. Tellem M, Rubenstone AI, Frumin AM: Renal failure and other unusual manifestations in sickle cell trait . Arch Pathol 63:508-512, 1957. 15. Miller RE, et al: Sickle cell nephropathy . Ala J Med Sci 1:233-238, 1964. 16. Schreiner GE: The nephrotic syndrome , in Strauss MB, Welt LG (eds): Diseases of the Kidney , ed 2. Boston, Little Brown & Co, 1971, pp 503-636. 17. Etteldorf JN, et al: Renal hemodynamic studies in adults with sickle cell anemia . Am J Med 18:243-248, 1955.Crossref 18. Keitel HG, Thompson D, Itano HA: Hyposthenuria in sickle cell anemia: A reversible renal defect . J Clin Invest 35:998-1007, 1956.Crossref 19. Miller WA, Peck D, Lowman RM: Perirenal hematoma in association with renal infarction in sickle cell trait . Radiology 92:351-352, 1969.Crossref 20. Aach RH, Kissane J: A patient with sickle cell anemia surviving 48 years . Am J Med 48:226-234, 1970.Crossref 21. Evans PV, Symmes AT: Bone marrow infarction with fat embolism and nephrosis in sickle cell disease . J Indiana State Med Assoc 50:1101-1105, 1957. 22. Barnett HL, Bernstein J: Clinical Pathologic Conference . J Pediatr 73:936-941, 1968.Crossref 23. Susmano S, Lewy JE: Sickle cell disease with acute glomerulonephritis . Am J Dis Child 118:615-618, 1969. 24. Strom T, Muehrcke RC, Smith RD: Sickle cell anemia with the nephrotic syndrome and renal vein obstruction . Arch Intern Med 129:104-108, 1972.Crossref 25. Schrier RW, Earley LE: Effects of hematocrit on renal hemodynamics and sodium excretion in hydropenic and volume expanded dogs . J Clin Invest 49:1656-1667, 1970.Crossref 26. Thompson DD, et al: Evaluation of the cell separation hypothesis of autoregulation of renal blood flow and filtration rate: Blood flow, filtration rate and PAH extraction as functions of arterial pressure in normal and anemic dogs . Am J Physiol 191:493-500, 1957. 27. Bradley SE, Bradley GP: Renal function during chronic anemia in man . Blood 2:192-202, 1947. 28. Bruch E: Renal function in anemia . Am J Dis Child 86:511-512, 1953. 29. Statius Van Eps LW, et al: The influence of red blood cell transfusions on the hyposthenuria and renal hemodynamics of sickle cell anemia . Clin Chim Acta 17:449-461, 1967.Crossref 30. Bahlman J, et al: The effect on urinary sodium excretion of blood volume expansion without changing the composition of blood in the dog . Clin Sci 32:403-413, 1967. 31. Barreras L, Diggs LW, Lipscomb A: Plasma volume in sickle cell disease . South Med J 59:456-458, 1966.Crossref 32. Deen WM, Robertson CR, Brenner BM: A model of glomerular ultrafiltration in the rat . Am J Physiol 223:1178-1183, 1972. 33. Brenner BM, et al: Dynamics of glomerular ultrafiltration in the rat: II. Plasma-flow dependence of GFR . Am J Physiol 223:1184-1190, 1972. 34. Pitts RF: Renal circulation , in Physiology of the Kidney and Body Fluids , ed 2. Chicago, Yearbook Medical Publishers, 1968, pp 144-161. 35. Earley LE, Friedler RM: Changes in renal blood flow and possibly the intrarenal distribution of blood during the natriuresis accompanying saline loading in the dog . J Clin Invest 44:929-941, 1965.Crossref 36. Pilkington LA, et al: Intrarenal distribution of blood flow . Am J Physiol 208:1107-1113, 1965. 37. Johnston HH, Herzog JP, Lauler DP: Effect of prostaglandin E, on renal hemodynamics, sodium and water excretion . Am J Physiol 213:939-946,1967. 38. Kountz SL, et al: Serial hemodynamics after renal allotransplantation in man . Circulation 41:217-224, 1970.Crossref 39. Velasquez MT, Notargiacomo AV, Cohn JN: Influence of cortical plasma transit-time on p-aminohippurate extraction during induced renal vasodilatation in anesthetized dogs . Clin Sci 43:401-411, 1972. 40. Perillie PE, Epstein FH: Sickling phenomenon produced by hhyposthenuriautions: A possible explanation for the hyposthenuria of sicklemia . J Clin Invest 42:570-580, 1963.Crossref 41. Moffat DB, Fourman J: The vascular pattern of the rat kidney . J Anat 97:543-553, 1963. 42. Thorburn GD, et al: Intrarenal distribution of nutrient blood flow determined with krypton85 in the unanesthetized dog . Circ Res 13:290-307, 1963.Crossref 43. Berliner RW, Bennett CM: Concentration of urine in the mammalian kidney . Am J Med 42:777-789, 1967.Crossref 44. Kokko JP, Rector FC Jr: Countercurrent multiplication system without active transport in inner medulla . Kidney Int 2:214-223, 1972.Crossref 45. Stephenson JL: Concentration of urine in a central core model of the renal counterflow system . Kidney Int 2:85-94, 1972.Crossref 46. Stewart J, Valtin H: Computer simulation of osmotic gradient without active transport in renal inner medulla . Kidney Int 2:264-270, 1972.Crossref 47. Schaefer JA, Adreoli TE: The effect of antidiuretic hormone on solute flows in mammalian collecting tubules . J Clin Invest 51:1279-1286, 1972.Crossref 48. Pitts RF: Volume and osmolar concentration of extracellular fluid , in Physiology of the Kidney and Body Fluids , ed 2. Chicago, Yearbook Medical Publishers, 1968, pp 213-227. 49. Goldberg M, McCurdy DK, Ramirez MA: Differences between saline and mannitol diuresis in hydropenic man . J Clin Invest 44:182-192, 1965.Crossref 50. Whitten CF, Younes AA: A comparative study of renal concentrating ability in children with sickle cell anemia and in normal children . J Lab Clin Med 55:400-415, 1960. 51. Levitt MF, et al: The renal concentrating defect in sickle cell disease . Am J Med 29:611-622, 1960.Crossref 52. Scaglione PR, Dell RB, Winters RW: Lactate concentration in the medulla of rat kidney . Am J Physiol 209:1193-1198, 1965. 53. Lief PD, Sullivan A, Goldberg M: Physiological contributions of thin and thick loops of Henle to the renal concentrating mechanism, abstracted . J Clin Invest 48:52a, 1969. 54. Jamison RL: Intrarenal heterogeneity . Am J Med 54:281-289, 1973.Crossref 55. Goossens JP, et al: Incomplete renal tubular acidosis in sickle cell disease . Clin Chim Acta 41:149-156, 1972.Crossref 56. Ho Ping Kong H, Alleyne GAO: Studies on acid excretion in adults with sickle-cell anemia . Clin Sci 41:505-518, 1971. 57. Harris JW, et al: Studies on the destruction of red blood cells: X. The biophysics and biology of sickle cell disease . Arch Intern Med 97:145-168,1956.Crossref 58. Oliver RE: Severe acidosis during a painful sickle cell crisis . Proc R Soc Med 62:1097, 1969. 59. Barreras L, Diggs LW: Sodium citrate orally for painful sickle cell crises . JAMA 215:762-768,1971.Crossref 60. Gennaris FJ, Goldstein MB, Schwartz WB: The nature of the renal adaptation to chronic hypocapnia . J Clin Invest 51:1722-1730, 1972.Crossref 61. Gold MS, et al: Sickle cell anemia and hyperuricemia . JAMA 206:1572-1573, 1968.Crossref 62. Walker BR, Alexander F: Uric acid excretion in sickle cell anemia . JAMA 215:255-258, 1971.Crossref 63. Immergut MA, Stevenson T: The use of epsilon amino caproic acid in the control of hematuria associated with hemoglobinopathies . J Urol 93:110-111, 1965. 64. Knochel JP: Hematuria in sickle trait: The effect of intravenous administration of distilled water, urinary alkalinization and diuresis . Arch Intern Med 123:160-165, 1969.Crossref 65. Goldberg M: Ethacrynic acid . Ann NY Acad Sci 139:443-452, 1966.Crossref 66. Sweeney MJ, Dobbins WT, Etteldorf JN: Renal disease with elements of the nephrotic syndrome associated with sickle cell anemia . J Pediatr 60:42-51, 1962.Crossref 67. Berman LB, Tublin I: The nephropathies of sickle cell disease . Arch Intern Med 103:602-606, 1959.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Renal Manifestations of Sickle Cell Disease

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American Medical Association
Copyright
Copyright © 1974 American Medical Association. All Rights Reserved.
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0003-9926
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1538-3679
DOI
10.1001/archinte.1974.00320160154014
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Abstract

Abstract Cortical morphologic changes in kidneys of patients with sickle cell anemia include vascular dilatation and engorgement of vessels with red blood cells, glomerular enlargement, hypertrophy and hypercellularity of glomerular mesangial cells, and iron deposits in various glomerular and tubular structures. Occasionally, glomerular sclerosis develops, with resultant end-stage renal failure. In the renal medulla, vascular congestion, edema, focal scarring, and occasional papillary necrosis are seen. Vascular dilatation and engorgement also occur in renal pelvis submucosa, where vessel rupture may cause hematuria. Young patients with sickle cell anemia have increased total renal blood flow while medullary blood flow is probably decreased. Glomerular filtration rate is also increased in young patients. A renal concentrating defect is reversible in younger age groups, becoming irreversible in older patients. References 1. Welt LG, Lyle CB Jr: The kidney in sickle cell anemia , in Strauss MB, Welt LG (eds): Diseases of the Kidney , ed 2. Boston, Little Brown & Co, 1971, pp 1207-1213. 2. Margolies MP: Sickle cell anemia . Medicine 30:357-443, 1951.Crossref 3. Schlitt LE, Keitel HG: Renal manifestations of sickle cell disease: A review . Am J Med Sci 239:773-778, 1960.Crossref 4. Pitcock JA, et al: Early renal changes in sickle cell anemia . Arch Pathol 90:403-410, 1970. 5. Bernstein J, Whitten CF: A histologic appraisal of the kidney in sickle cell anemia . Arch Pathol 70:407-418, 1960. 6. Etteldorf JN, et al: Renal hemodynamic studies in adults with sickle cell anemia . Am J Med 18:243-248, 1955.Crossref 7. Mostofi KF, Vorder Bruegge CF, Diggs LW: Lesions in kidneys removed for unilateral hematuria in sickle cell disease . Arch Pathol 63:336-351, 1957. 8. Statius Van Eps LW, et al: Nature of concentrating defect in sickle cell nephropathy . Lancet 1:450-452, 1970.Crossref 9. Hatch FE, et al: Renal circulatory studies in young adults with sickle cell anemia . J Lab Clin Med 76:632-640, 1970. 10. McCoy RC: Ultrastructural alterations in the kidney of patients with sickle cell disease and the nephrotic syndrome . Lab Invest 21:85-94, 1969. 11. Ellis JT: Glomerular lesions and the nephrotic syndrome in rabbits given saccharated iron oxide intravenously . J Exp Med 103:127-143, 1956.Crossref 12. Antonovych TT: Ultrastructural changes in glomeruli of patients with sickle cell disease and nephrotic syndrome , in Abstracts, Fifth Annual Meeting . Washington, DC, The American Society of Nephrology, 1971, p 3. 13. Walker BR, et al: Glomerular lesions in sickle cell nephropathy . JAMA 215:437-440, 1971.Crossref 14. Tellem M, Rubenstone AI, Frumin AM: Renal failure and other unusual manifestations in sickle cell trait . Arch Pathol 63:508-512, 1957. 15. Miller RE, et al: Sickle cell nephropathy . Ala J Med Sci 1:233-238, 1964. 16. Schreiner GE: The nephrotic syndrome , in Strauss MB, Welt LG (eds): Diseases of the Kidney , ed 2. Boston, Little Brown & Co, 1971, pp 503-636. 17. Etteldorf JN, et al: Renal hemodynamic studies in adults with sickle cell anemia . Am J Med 18:243-248, 1955.Crossref 18. Keitel HG, Thompson D, Itano HA: Hyposthenuria in sickle cell anemia: A reversible renal defect . J Clin Invest 35:998-1007, 1956.Crossref 19. Miller WA, Peck D, Lowman RM: Perirenal hematoma in association with renal infarction in sickle cell trait . Radiology 92:351-352, 1969.Crossref 20. Aach RH, Kissane J: A patient with sickle cell anemia surviving 48 years . Am J Med 48:226-234, 1970.Crossref 21. Evans PV, Symmes AT: Bone marrow infarction with fat embolism and nephrosis in sickle cell disease . J Indiana State Med Assoc 50:1101-1105, 1957. 22. Barnett HL, Bernstein J: Clinical Pathologic Conference . J Pediatr 73:936-941, 1968.Crossref 23. Susmano S, Lewy JE: Sickle cell disease with acute glomerulonephritis . Am J Dis Child 118:615-618, 1969. 24. Strom T, Muehrcke RC, Smith RD: Sickle cell anemia with the nephrotic syndrome and renal vein obstruction . Arch Intern Med 129:104-108, 1972.Crossref 25. Schrier RW, Earley LE: Effects of hematocrit on renal hemodynamics and sodium excretion in hydropenic and volume expanded dogs . J Clin Invest 49:1656-1667, 1970.Crossref 26. Thompson DD, et al: Evaluation of the cell separation hypothesis of autoregulation of renal blood flow and filtration rate: Blood flow, filtration rate and PAH extraction as functions of arterial pressure in normal and anemic dogs . Am J Physiol 191:493-500, 1957. 27. Bradley SE, Bradley GP: Renal function during chronic anemia in man . Blood 2:192-202, 1947. 28. Bruch E: Renal function in anemia . Am J Dis Child 86:511-512, 1953. 29. Statius Van Eps LW, et al: The influence of red blood cell transfusions on the hyposthenuria and renal hemodynamics of sickle cell anemia . Clin Chim Acta 17:449-461, 1967.Crossref 30. Bahlman J, et al: The effect on urinary sodium excretion of blood volume expansion without changing the composition of blood in the dog . Clin Sci 32:403-413, 1967. 31. Barreras L, Diggs LW, Lipscomb A: Plasma volume in sickle cell disease . South Med J 59:456-458, 1966.Crossref 32. Deen WM, Robertson CR, Brenner BM: A model of glomerular ultrafiltration in the rat . Am J Physiol 223:1178-1183, 1972. 33. Brenner BM, et al: Dynamics of glomerular ultrafiltration in the rat: II. Plasma-flow dependence of GFR . Am J Physiol 223:1184-1190, 1972. 34. Pitts RF: Renal circulation , in Physiology of the Kidney and Body Fluids , ed 2. Chicago, Yearbook Medical Publishers, 1968, pp 144-161. 35. Earley LE, Friedler RM: Changes in renal blood flow and possibly the intrarenal distribution of blood during the natriuresis accompanying saline loading in the dog . J Clin Invest 44:929-941, 1965.Crossref 36. Pilkington LA, et al: Intrarenal distribution of blood flow . Am J Physiol 208:1107-1113, 1965. 37. Johnston HH, Herzog JP, Lauler DP: Effect of prostaglandin E, on renal hemodynamics, sodium and water excretion . Am J Physiol 213:939-946,1967. 38. Kountz SL, et al: Serial hemodynamics after renal allotransplantation in man . Circulation 41:217-224, 1970.Crossref 39. Velasquez MT, Notargiacomo AV, Cohn JN: Influence of cortical plasma transit-time on p-aminohippurate extraction during induced renal vasodilatation in anesthetized dogs . Clin Sci 43:401-411, 1972. 40. Perillie PE, Epstein FH: Sickling phenomenon produced by hhyposthenuriautions: A possible explanation for the hyposthenuria of sicklemia . J Clin Invest 42:570-580, 1963.Crossref 41. Moffat DB, Fourman J: The vascular pattern of the rat kidney . J Anat 97:543-553, 1963. 42. Thorburn GD, et al: Intrarenal distribution of nutrient blood flow determined with krypton85 in the unanesthetized dog . Circ Res 13:290-307, 1963.Crossref 43. Berliner RW, Bennett CM: Concentration of urine in the mammalian kidney . Am J Med 42:777-789, 1967.Crossref 44. Kokko JP, Rector FC Jr: Countercurrent multiplication system without active transport in inner medulla . Kidney Int 2:214-223, 1972.Crossref 45. Stephenson JL: Concentration of urine in a central core model of the renal counterflow system . Kidney Int 2:85-94, 1972.Crossref 46. Stewart J, Valtin H: Computer simulation of osmotic gradient without active transport in renal inner medulla . Kidney Int 2:264-270, 1972.Crossref 47. Schaefer JA, Adreoli TE: The effect of antidiuretic hormone on solute flows in mammalian collecting tubules . J Clin Invest 51:1279-1286, 1972.Crossref 48. Pitts RF: Volume and osmolar concentration of extracellular fluid , in Physiology of the Kidney and Body Fluids , ed 2. Chicago, Yearbook Medical Publishers, 1968, pp 213-227. 49. Goldberg M, McCurdy DK, Ramirez MA: Differences between saline and mannitol diuresis in hydropenic man . J Clin Invest 44:182-192, 1965.Crossref 50. Whitten CF, Younes AA: A comparative study of renal concentrating ability in children with sickle cell anemia and in normal children . J Lab Clin Med 55:400-415, 1960. 51. Levitt MF, et al: The renal concentrating defect in sickle cell disease . Am J Med 29:611-622, 1960.Crossref 52. Scaglione PR, Dell RB, Winters RW: Lactate concentration in the medulla of rat kidney . Am J Physiol 209:1193-1198, 1965. 53. Lief PD, Sullivan A, Goldberg M: Physiological contributions of thin and thick loops of Henle to the renal concentrating mechanism, abstracted . J Clin Invest 48:52a, 1969. 54. Jamison RL: Intrarenal heterogeneity . Am J Med 54:281-289, 1973.Crossref 55. Goossens JP, et al: Incomplete renal tubular acidosis in sickle cell disease . Clin Chim Acta 41:149-156, 1972.Crossref 56. Ho Ping Kong H, Alleyne GAO: Studies on acid excretion in adults with sickle-cell anemia . Clin Sci 41:505-518, 1971. 57. Harris JW, et al: Studies on the destruction of red blood cells: X. The biophysics and biology of sickle cell disease . Arch Intern Med 97:145-168,1956.Crossref 58. Oliver RE: Severe acidosis during a painful sickle cell crisis . Proc R Soc Med 62:1097, 1969. 59. Barreras L, Diggs LW: Sodium citrate orally for painful sickle cell crises . JAMA 215:762-768,1971.Crossref 60. Gennaris FJ, Goldstein MB, Schwartz WB: The nature of the renal adaptation to chronic hypocapnia . J Clin Invest 51:1722-1730, 1972.Crossref 61. Gold MS, et al: Sickle cell anemia and hyperuricemia . JAMA 206:1572-1573, 1968.Crossref 62. Walker BR, Alexander F: Uric acid excretion in sickle cell anemia . JAMA 215:255-258, 1971.Crossref 63. Immergut MA, Stevenson T: The use of epsilon amino caproic acid in the control of hematuria associated with hemoglobinopathies . J Urol 93:110-111, 1965. 64. Knochel JP: Hematuria in sickle trait: The effect of intravenous administration of distilled water, urinary alkalinization and diuresis . Arch Intern Med 123:160-165, 1969.Crossref 65. Goldberg M: Ethacrynic acid . Ann NY Acad Sci 139:443-452, 1966.Crossref 66. Sweeney MJ, Dobbins WT, Etteldorf JN: Renal disease with elements of the nephrotic syndrome associated with sickle cell anemia . J Pediatr 60:42-51, 1962.Crossref 67. Berman LB, Tublin I: The nephropathies of sickle cell disease . Arch Intern Med 103:602-606, 1959.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Apr 1, 1974

References