1. Weatherall DF, Cleg SB, Higs DR, et al. The hemoglobinopathies. In: Scriver CR, Beaudet AL, Sly WS, et al., eds. The metabolic basis of inherited disease, 6th ed. New York: McGraw-Hill, 1992:2293–2335.
2. Oksenhendler E, Bourbigot B, Desbazeille F, et al. Recurrent hematuria in four white patients with sickle cell trait. J Urol 1984;132:1201–1203.
3. Osegbe DN. Haematuria and sickle cell disease. A report of 12 cases and review of the literature. Trop Geograph Med 1990;42:22–27.
4. Statius van Eps LAW, de Jong PE. Sickle cell disease. In: Scherer WR, Gottschalk KW, eds. Diseases of the kidney, 4th ed. Boston: Little, Brown, 1988:2561–2681.
5. Davies SC, Dewitt PE. Sickle cell disease. Br J Hosp Med 1984;31:440–444.
6. Audit JC, Ugbodaga CI, Okafor LA, et al. Urographic changes in homozygous sickle cell disease. Diagn Imag 1983;52:259–263.
7. Pandya KK, Koshy M, Brown N, et al. Renal papillary necrosis in sickle cell hemoglobinopathies. J Urol 1976;115: 497–501.
8. Chauhan PM, Kondlapoodi P, Natta CL. Pathology of sickle cell disorders. Pathol Ann 1983;18:253–276.
9. Bunn HF. Mechanisms of disease—pathogenesis and treatment of sickle cell disease. N Engl J Med 1997;337:762–769.
10. de Jong PE, Statius van Eps LW. Sickle cell nephropathy: new insights into its pathophysiology. Kidney Int 1985;27: 711–717.
11. Burry A, Cross R, Axelsen R. Analgesic nephropathy and the renal concentrating mechanism. Pathol Ann 1977;12:1–31.
12. Avery RA, Harris JE, Davis CJ, et al. Renal medullary carcinoma: clinical and therapeutic aspects of a newly described tumor. Cancer 1996;78:128–132.
13. Schultz PK, Strife JL, Strife CF, et al. Hyperechoic renal medullary pyramids in infants and children. Radiology 1991;181: 163–167.
14. Walker TM, Serjeant GR. Increased renal reflectivity in sickle cell disease: prevalence and characteristics. Clin Radiol 1995;50:566–569.
15. McCall IW, Moule N, Desai P, et al. Urographic findings in homozygous sickle cell disease. Radiology 1978;126:99–104.
16. Mapp E, Karasick S, Pollack H, et al. Uroradiological manifestations of S-hemoglobinopathy. Semin Roentgenol 1987; 22:186–194.
17. Braden GL, Kozinn DR, Hampf FE, et al. Ultrasound diagnosis of early renal papillary necrosis. J Ultrasound Med 1991;10:401–403.
18. Eknoyan G, Qunibi WY, Grissom RT, et al. Renal papillary necrosis: an update. Medicine 1982;61:55–73.
19. Sabatini S. Pathophysiologic mechanisms of abnormal collecting duct function. Semin Nephrol 1989;9:179–202.
20. Keeler R, Wilson N. Natriuretic response to hypervolemia is absent in rats with papillary necrosis. Am J Physiol 1989;257: R422–R426.
21. Allon M, Lawson L, Eckman JR, et al. Effects of nonsteroidal antiinflammatory drugs on renal function in sickle cell anemia. Kidney Int 1988;34:500–506.
22. Beutler E. Erythrocyte disorders: anemias related to abnormal globulin. In: Williams WJ, Beutler E, Erslev AJ, et al., eds. Hematology, 4th ed. New York: McGraw-Hill, 1992:613–626.
23. Allon M. Renal abnormalities in sickle cell disease. Arch Intern Med 1990;150:501–504.
24. Buckalew VM, Someren A. Renal manifestations of sickle cell disease. Arch Intern Med 1974;133:660–669.
25. Devereux S, Knowles SM. Rhabdomyolysis and acute renal failure in sickle cell anaemia. BMJ 1985;290:1707.
26. de Jong PE, de Jong-van den Berg LT, Schouten H, et al. The influence of indomethacin on renal acidification in normal subjects and in patients with sickle cell anemia. Clin Nephrol 1983;19:259–264.
27. Kurtzman NA. Acquired distal renal tubular acidosis. Kidney Int 1983;24:807–819.
28. Bakir AA, Hathiwala SC, Ainis H, et al. Prognosis of the nephrotic syndrome in sickle glomerulopathy. A retrospective study. Am J Nephrol 1987;7:110–115.
29. DeFronzo RA, Taufield PA, Black H, et al. Impaired renal tubular potassium secretion in sickle cell disease. Ann Intern Med 1979;90:310–316.
30. Morgan AG, de Ceulaer K, Serjeant GR. Glomerular function and hyperuricemia in sickle cell disease. J Clin Pathol 1984;37:1046–1049.
31. de Jong PE, de Jong-van den Berg LT, Donker AJ, et al. The role of prostaglandins and renin in sickle-cell nephropathy. A hypothesis. Neth J Med 1978;21:67–72.
32. Berman LB, Tublin I. The nephropathies of sickle-cell disease. Arch Intern Med 1959;103:602–606.
33. Sklar AH, Campbell H, Caruana RJ, et al. A population study of renal function in sickle cell anemia. Int J Artif Organs 1990;13:231–236.
34. Falk RJ, Scheinman JI, Phillips G, et al. Prevalence and pathologic features of sickle cell nephropathy and response to inhibition of angiotensin-converting enzyme. N Engl J Med 1992;326:910–915.
35. Guasch A, Cua M, Mitch WE. Early detection and the course of glomerular injury in patients with sickle cell anemia. Kidney Int 1996;49:786–791.
36. Guasch A, Cua M, You W, et al. Sickle cell anemia causes a distinct pattern of glomerular dysfunction. Kidney Int 1997;51: 826–833.
37. Lonsdorfer A, Comoe L, Yapo AE, et al. Proteinuria in sickle cell trait and disease: an electrophoretic. Clin Chim Acta 1989;181:239–248.
38. Powars DR, Elliott-Mills DD, Chan L, et al. Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality. Ann Intern Med 1991;115:614–620.
39. Zamurovic D, Churg J. IDiopathic and secondary mesangiocapillary glomerulonephritis. Nephron 1984;38:145–153.
40. Morgan AG, Shah DJ, Williams W. Renal pathology in adults over 40 with sickle-cell disease. West Indian Med J 1987;36:241–250.
41. Bhathena DB, Sondheimer JH. The glomerulopathy of homozygous sickle hemoglobin (SS) disease: morphology and pathogenesis. J Am Soc Nephrol 1991;1:1241–1252.
42. de Jong PE, Saleh AW, de Zeeuw D, et al. Urinary prostaglandins in sickle cell nephropathy: a defect in 9-ketoreductase activity? Clin Nephrol 1984;22:212–213.
43. Strauss J, Pardo V, Koss MN, et al. Nephropathy associated with sickle cell anemia: an autologous immune complex nephritis. I. Studies on nature of glomerular-bound antibody and antigen identification in a patient with sickle cell disease and immune deposit glomerulonephritis. Am J Med 1975;58:382–387.
44. Jennette JC, Charles L, Grubb W. Glomerulomegaly and focal segmental glomerulosclerosis associated with obesity and sleep-apnea syndrome. Am J Kidney Dis 1987;10:470–472.
45. Olson JL, Hostetter TH, Rennke HG, et al. Altered glomerular permselectivity and progressive sclerosis following extreme ablation of renal mass. Kidney Int 1982;22:112–126.
46. Chen YT, Coleman RA, Scheinman JI, et al. Renal disease in type I glycogen storage disease. N Engl J Med 1988;318:7–11.
47. Spear G. Glomerular alterations in cyanotic congenital heart disease. Bull Johns Hopkins Hosp 1960;106:347–367.
48. Lande IM, Glazer GM, Sarnaik S, et al. Sickle-cell nephropathy: MR imaging. Radiology 1986;158:379–383.
49. Yoshida Y, Fogo A, Ichikawa I. Glomerular hemodynamic changes vs. hypertrophy in experimental glomerular sclerosis. Kidney Int 1989;35:654–660.
50. Hostetter TH, Olson JL, Rennke HG, et al. Hyperfiltration in remnant nephrons: a potentially adverse response to renal ablation. Nephron 1981;241:F85-F93.
51. Haycock G. Creatinine, body size and renal function. Pediatr Nephrol 1989;3:22–24.
52. Schwartz GJ, Haycock GB, Edelmann CM, et al. A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 1976;58:259–263.
53. Mulhern JG, Perrone RD. Accurate measurement of glomerular filtration rate. Int Yearb Nephrol 1990;277–291.
54. Excerpts from the United States Renal Data System 1996 Annual Data Report. Am J Kidney Dis 1996;28:S1–165.
55. Walser M. Progression of chronic renal failure in man. Kidney Int 1990;37:1195–1210.
56. Coakley DF, Roland CL, Falk RJ, et al. Renal function assessment with HPLC analysis of iothalamate (IOTH) and para-aminohippurate (PAH) compared to inulin, PAH, and creatinine as measured by standard analytical methods. Pharmacotherapy 1991;11:265.
57. Woolf AS, Fine LG. Do glomerular hemodynamic adaptations influence the progression of human renal disease? Pediatr Nephrol 1991;5:88–93.
58. Morozumi K, Thiel G, Gudat F, et al. Studies on morphological outcome of cyclosporine-associated arteriolopathy after discontinuation of cyclosporine in patients with renal allografts. Transplant Proc 1993;25:537–539.
59. Raymond NG, Dwyer JT, Nevins P, et al. An approach to protein restriction in children with renal insufficiency. Pediatr Nephrol 1990;4:145–151.
60. Foucan L, Bourhis V, Bangou J, et al. A randomized trial of captopril for microalbuminuria in normotensive adults with sickle cell anemia. Am J Med 1998;104:339–342.
61. Heeg JE, de Jong PE, van der Hem GK, et al. Reduction of proteinuria by angiotensin converting enzyme inhibition. Kidney Int 1987;32:78–83.
62. Murthy VS, Haywood J. Survival analysis by sex, age, group and hemotype in sickle cell disease. J Chronic Dis 1981;34:3 13–319.
63. Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle-cell disease in Jamaica. BMJ 1982;285:633–635.
64. Powars DR, Meiselman HJ, Fisher TC, et al. Beta-S gene cluster haplotypes modulate hematologic and hemorheologic expression in sickle cell anemia. Use in predicting clinical severity. Am J Pediatr Hematol Oncol 1994;16:55–61.
65. Guasch A, Zayas CF, Eckman JR, et al. Evidence that microdeletions in the alpha globin gene protect against the development of sickle cell glomerulopathy in humans. J Am Soc Nephrol 1999;10:1014–1019.
66. Tomson CR, Edmunds ME, Chambers K, et al. Effect of recombinant human erythropoietin on erythropoiesis in homozygous sickle-cell anaemia and renal failure. Nephrol Dial Transplant 1992;7:817–821.
67. Rodgers GP, Dover GJ, Uyesaka N, et al. Augmentation by erythropoietin of the fetal-hemoglobin response to hydroxyurea in sickle cell disease [see comments]. N Engl J Med 1993;328:73–80.
68. Ojo AO, Govaerts TC, Schmouder RL, et al. Renal transplantation in end-stage sickle cell nephropathy. Transplantation 1999;67:291–295.
69. Bleyer AJ, Donaldson LA, McIntosh M, et al. Relationship between underlying renal disease and renal transplantation outcome. Am J Kidney Dis 2001;37:1152–1161.
70. Spector D, Zachary JB, Sterioff S, et al. Painful crises following renal transplantation in sickle cell anemia. Am J Med 1978;64:835–839.
71. Chatterjee SN, Lundberg GD, Berne TV. Sickle cell trait: possible contributory cause of renal allograft failure. Urology 1978;11:266–268.
72. Donnelly PK, Edmunds ME, O’Reilly K. Renal transplantation in sickle cell disease [Letter]. Lancet 1988;2:229.
73. Miner DJ, Jorkasky DK, Perloff LJ, et al. Recurrent sickle cell nephropathy in a transplanted kidney. Am J Kidney Dis 1987;10:306–313.
74. Mauer SM, Steffes MW, Connett J, et al. The development of lesions in the glomerular basement membrane and mesangium after transplantation of normal kidneys to diabetic patients. Diabetes 1983;32:948–952.
75. Khamashta MA, Williams FM, Hunt BJ. Anticoagulation for venous thromboembolism [Letter]. N Engl J Med 1999; 341:539–540.
76. Cazzola M, Pootrakul P, Huebers HA, et al. Erythroid marrow function in anemic patients. Blood 1987;69:296–301.
77. Goldberg JS, Dunning SP, Bunn HF. Regulation of the erythropoietin gene: evidence that the oxygen sensor is a heme protein. Science 1988;242:1412–1415.
78. Sztajzel J, Ruedin P, Stoermann C, et al. Effects of dialysate composition during hemodialysis on left ventricular function. Kidney Int 1993;41:S60–S66.
79. Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease. Rates and risk factors. N Engl J Med 1991;325: 11–16.
80. Ponez M, Kane E, Gill FM. Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J Pediatr 1985; 107:861–866.
81. Powars DR. Sickle cell anemia and major organ failure. Hemoglobin 1990;14:573–598.
82. Aslan M, Thornley-Brown D, Freeman BA. Reactive species in sickle cell disease. Ann N Y Acad Sci 2000;899:375–391.
83. Nath KA, Grande JP, Haggard JJ, et al. Oxidative stress and induction of heme oxygenase-1 in the kidney in sickle cell disease. Am J Pathol 2001;158:893–903.
84. Nath KA, Vercellotti GM, Grande JP, et al. Heme protein-induced chronic renal inflammation: suppressive effect of induced heme oxygenase-1. Kidney Int 2001;59:106–117.
85. Sklar AH, Perez JC, Harp RJ, et al. Acute renal failure in sickle cell anemia. Int J Artif Organs 1990;13:347–351.
86. Simckes AM, Chen SS, Osorio AV, et al. Ketorolac-induced irreversible renal failure in sickle cell disease: a case report. Pediatr Nephrol 1999;13:63–67.
87. Koppes GM, Daly JJ, Coltman CA, et al. Exertion-induced rhabdomyolysis with acute renal failure and disseminated intravascular coagulation in sickle cell trait. Am J Med 1977;63:313–317.
88. Kark JA, Posey DM, Schumacher HR, et al. Sickle-cell trait as a risk factor for sudden death in physical training. N Engl J Med 1987;317:781–787.
89. Johnson CS, Giorgio AJ. Arterial blood pressure in adults with sickle cell disease. Arch Intern Med 1981;141:891–893.
90. Pegelow CH, Colangelo L, Steinberg M, et al. Natural history of blood pressure in sickle cell disease: risks for stroke and death associated with relative hypertension in sickle cell anemia. Am J Med 1997;102:171–177.
91. Sty JR, Babbitt DP, Sheth K. Abnormal Tc-99m-methylene diphosphonate accumulation in the kidneys of children with sickle cell disease. Clin Nucl Med 1980;5:445–447.
92. Adams RA, McKie V, Nichols F, et al. The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 1992;326:605–610.
93. Shannon KM. Recombinant erythropoietin in pediatrics: a clinical perspective. Pediatr Ann 1990;19:197–206.
94. Baliga BS, Pace BS, Chen HH, et al. Mechanism for fetal hemoglobin induction by hydroxyurea in sickle cell erythroid progenitors. Am J Hematol 2000;65:227–233.
95. Charache S, Dover GJ, Moyer MA, et al. Hydroxyurea-induced augmentation of fetal hemoglobin production in patients with sickle cell anemia. Blood 1987;69:109–116.
96. Noguchi CT, Rodgers GP, Serjeant G, et al. Current concepts: levels of fetal hemoglobin necessary for treatment of sickle cell disease. N Engl J Med 1988;318:96–99.
97. Charache S, Dover GJ, Moore RD, et al. Hydroxyurea: effects on hemoglobin F production in patients with sickle cell anemia. Blood 1992;79:2555–2565.
98. Ferster A, Tahriri P, Vermylen C, et al. Five years of experience with hydroxyurea in children and young adults with sickle cell disease. Blood 2001;97:3628–3632.
99. Wang WC, Helms RW, Lynn HS, et al. Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS Study. J Pediatr 2002;140:225–229.
100. Iyamu EW, Fasold H, Roa D, et al. Hydroxyurea-induced oxidative damage of normal and sickle cell hemoglobins in vitro: amelioration by radical scavengers. J Clin Lab Anal 2001;15:1–7.
101. Steinberg MH. Management of sickle cell disease. N Engl J Med 1999;340:1021–1030.
102. Schleuning M, Stoetzer O, Waterhouse C, et al. Hematopoietic stem cell transplantation after reduced-intensity conditioning as treatment of sickle cell disease. Exp Hematol 2002; 30:7–10.