Risk factors and prediction of outcomes in children and adolescents who have sickle cell anemia

Hematology/Oncology Clinics of North America

Volumn 18, Issue 6 SPEC.ISS., 2004, Pages 1339-1354

  Quinn, Charles T ^a,b,d   Miller, Scott T ^c  

^a UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

^b 5323 Harry Hines Boulevard   (United States)

^c KINGS COUNTY HOSPITAL CENTER   (United States)

^d UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BETA GLOBIN; HEMOGLOBIN;

ACUTE CHEST SYNDROME; ALPHA THALASSEMIA; DISEASE SEVERITY; FOLLOW UP; GENE MUTATION; GENETIC POLYMORPHISM; GENOTYPE; HAPLOTYPE; HEMATOCRIT; HEMOGLOBIN SC DISEASE; HLA SYSTEM; HUMAN; LABORATORY TEST; LEUKOCYTE COUNT; MORBIDITY; MORTALITY; PATHOGENESIS; PREDICTION; PRIORITY JOURNAL; PROGNOSIS; REVIEW; RISK ASSESSMENT; RISK FACTOR; SICKLE CELL ANEMIA; STEADY STATE; STROKE; THALASSEMIA;

EID: 7044235524     PISSN: 08898588     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.hoc.2004.07.004     Document Type: Review

References (93)

1. G.R. Serjeant, and B.E. Serjeant Sickle cell disease 3rd edition 2001 Oxford University Press New York
2. O.S. Platt, D.J. Brambilla, W.F. Rosse, P.F. Milner, O. Castro, and M.H. Steinberg Mortality in sickle cell disease: life expectancy and risk factors for early death N Engl J Med 330 23 1994 1639 1644
3. S.L. Leikin, D. Gallagher, T.R. Kinney, D. Sloane, P. Klug, and W. Rida Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease Pediatrics 84 3 1989 500 508
4. C.T. Quinn, Z.R. Rogers, and G.R. Buchanan Survival of children with sickle cell disease Blood 103 11 2004 4023 4027
5. D.J. Weatherall Phenotype-genotype relationships in monogenic disease: lessons from the thalassaemias Nat Rev Genet 2 4 2001 245 255
6. G.R. Serjeant, R. Richards, P.R. Barbor, and P.F. Milner Relatively benign sickle-cell anaemia in 60 patients aged over 30 in the West Indies BMJ 3 610 1968 86 91
7. M.H. Steinberg, B.J. Dreiling, F.S. Morrison, and T.F. Necheles Mild sickle cell disease. Clinical and laboratory studies JAMA 224 3 1973 317 321
8. O.S. Platt, B.D. Thorington, D.J. Brambilla, P.F. Milner, W.F. Rosse, and E. Vichinsky Pain in sickle cell disease. Rates and risk factors N Engl J Med 325 1 1991 11 16
9. K. Ohene-Frempong, S.J. Weiner, L.A. Sleeper, S.T. Miller, S. Embury, and J.W. Moohr Cerebrovascular accidents in sickle cell disease: rates and risk factors Blood 91 1 1998 288 294
10. American Academy of Pediatrics, Section on Hematology/Oncology, Committee on Genetics Health supervision for children with sickle cell disease Pediatrics 109 3 2002 526 535
11. S. Charache, M.L. Terrin, R.D. Moore, G.J. Dover, F.B. Barton, and S.V. Eckert Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia N Engl J Med 332 20 1995 1317 1322
12. M.H. Steinberg, F. Barton, O. Castro, C.H. Pegelow, S.K. Ballas, and A. Kutlar Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment JAMA 289 13 2003 1645 1651
13. M. de Montalembert, and S.C. Davies Is hydroxyurea leukemogenic in children with sickle cell disease? Blood 98 9 2001 2878 2879
14. W.C. Wang, R.W. Helms, H.S. Lynn, R. Redding-Lallinger, B.E. Gee, and K. Ohene-Frempong Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS Study J Pediatr 140 2 2002 225 229
15. S.T. Miller, E. Wright, M. Abboud, B. Berman, B. Files, and C.D. Scher Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia J Pediatr 139 6 2001 785 789
16. W. Reed, and E.P. Vichinsky Transfusion therapy: a coming-of-age treatment for patients with sickle cell disease J Pediatr Hematol Oncol 23 4 2001 197 202
17. M.O. Russell, H.I. Goldberg, L. Reis, S. Friedman, R. Slater, and M. Reivich Transfusion therapy for cerebrovascular abnormalities in sickle cell disease J Pediatr 88 3 1976 382 387
18. C.H. Pegelow, R.J. Adams, V. McKie, M. Abboud, B. Berman, and S.T. Miller Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions J Pediatr 126 6 1995 896 899
19. D.J. Scothorn, C. Price, D. Schwartz, C. Terrill, G.R. Buchanan, and W. Shurney Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke J Pediatr 140 3 2002 348 354
20. A.S. Wayne, S.V. Kevy, and D.G. Nathan Transfusion management of sickle cell disease Blood 81 5 1993 1109 1123
21. B. Files, D. Brambilla, A. Kutlar, S. Miller, E. Vichinsky, and W. Wang Longitudinal changes in ferritin during chronic transfusion: a report from the Stroke Prevention Trial in Sickle Cell Anemia (STOP) J Pediatr Hematol Oncol 24 4 2002 284 290
22. M.C. Walters, M. Patience, W. Leisenring, J.R. Eckman, J.P. Scott, and W.C. Mentzer Bone marrow transplantation for sickle cell disease N Engl J Med 335 6 1996 369 376
23. A. Ashley-Koch, Q. Yang, and R.S. Olney Sickle hemoglobin (HbS) allele and sickle cell disease: a HuGE review Am J Epidemiol 151 9 2000 839 845
24. O. Castro, D.J. Brambilla, B. Thorington, C.A. Reindorf, R.B. Scott, and P. Gillette The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease Blood 84 2 1994 643 649
25. J. Watson, A.W. Stahman, and F.P. Biello The significance of the paucity of sickle cells in newborn Negro infants Am J Med Sci 215 1948 419 423
26. N. Murray, B.E. Serjeant, and G.R. Serjeant Sickle cell-hereditary persistence of fetal haemoglobin and its differentiation from other sickle cell syndromes Br J Haematol 69 1 1988 89 92
27. Y.P. Chang, M. Maier-Redelsperger, K.D. Smith, L. Contu, R. Ducroco, and M. de Montalembert The relative importance of the X-linked FCP locus and beta-globin haplotypes in determining haemoglobin F levels: a study of SS patients homozygous for beta S haplotypes Br J Haematol 96 4 1997 806 814
28. G.R. Serjeant Fetal haemoglobin in homozygous sickle cell disease Clin Haematol 4 1 1975 109 122
29. G.J. Dover, K.D. Smith, Y.C. Chang, S. Purvis, A. Mays, and D.A. Meyers Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2 Blood 80 3 1992 816 824
30. R.L. Nagel, and H.M. Ranney Genetic epidemiology of structural mutations of the beta-globin gene Semin Hematol 27 4 1990 342 359
31. J.E. Craig, J. Rochette, C.A. Fisher, D.J. Weatherall, S. Marc, and G.M. Lathrop Dissecting the loci controlling fetal haemoglobin production on chromosomes 11p and 6q by the regressive approach Nat Genet 12 1 1996 58 64
32. Y.C. Chang, K.D. Smith, R.D. Moore, G.R. Serjeant, and G.J. Dover An analysis of fetal hemoglobin variation in sickle cell disease: the relative contributions of the X-linked factor, beta-globin haplotypes, alpha-globin gene number, gender, and age Blood 85 4 1995 1111 1117
33. P.W. Thomas, D.R. Higgs, and G.R. Serjeant Benign clinical course in homozygous sickle cell disease: a search for predictors J Clin Epidemiol 50 2 1997 121 126
34. M. Koshy, R. Entsuah, A. Koranda, A.P. Kraus, R. Johnson, and R. Bellvue Leg ulcers in patients with sickle cell disease Blood 74 4 1989 1403 1408
35. D.R. Powars Sickle cell anemia: beta s-gene-cluster haplotypes as prognostic indicators of vital organ failure Semin Hematol 28 3 1991 202 208
36. D.R. Powars Beta s-gene-cluster haplotypes in sickle cell anemia. Clinical and hematologic features Hematol Oncol Clin North Am 5 3 1991 475 493
37. D. Labie, R. Srinivas, O. Dunda, C. Dode, C. Lapoumeroulie, and V. Devi Haplotypes in tribal Indians bearing the sickle gene: evidence for the unicentric origin of the beta S mutation and the unicentric origin of the tribal populations of India Hum Biol 61 4 1989 479 491
38. S. el-Kalla, and E. Baysal Genotype-phenotype correlation of sickle cell disease in the United Arab Emirates Pediatr Hematol Oncol 15 3 1998 237 242
39. P.F. Milner, A.P. Kraus, J.I. Sebes, L.A. Sleeper, K.A. Dukes, and S.H. Embury Sickle cell disease as a cause of osteonecrosis of the femoral head N Engl J Med 325 21 1991 1476 1481
40. M.H. Steinberg, W. Rosenstock, M.B. Coleman, J.G. Adams, O. Platica, and M. Cedeno Effects of thalassemia and microcytosis on the hematologic and vasoocclusive severity of sickle cell anemia Blood 63 6 1984 1353 1360
41. H.S. Zarkowsky, D. Gallagher, F.M. Gill, W.C. Wang, J.M. Falletta, and W.M. Lande Bacteremia in sickle hemoglobinopathies J Pediatr 109 4 1986 579 585
42. M.T. Gladwin, V. Sachdev, M.L. Jison, Y. Shizukuda, J.F. Plehn, and K. Minter Pulmonary hypertension as a risk factor for death in patients with sickle cell disease N Engl J Med 350 9 2004 886 895
43. R.P. Hebbel, O. Yamada, C.F. Moldow, H.S. Jacob, J.G. White, and J.W. Eaton Abnormal adherence of sickle erythrocytes to cultured vascular endothelium: possible mechanism for microvascular occlusion in sickle cell disease J Clin Invest 65 1 1980 154 160
44. R.P. Hebbel, M.A. Boogaerts, J.W. Eaton, and M.H. Steinberg Erythrocyte adherence to endothelium in sickle-cell anemia. A possible determinant of disease severity N Engl J Med 302 18 1980 992 995
45. E. Fadlon, S. Vordermeier, T.C. Pearson, A.R. Mire-Sluis, D.C. Dumonde, and J. Phillips Blood polymorphonuclear leukocytes from the majority of sickle cell patients in the crisis phase of the disease show enhanced adhesion to vascular endothelium and increased expression of CD64 Blood 91 1 1998 266 274
46. I. Okpala, Y. Daniel, R. Haynes, D. Odoemene, and J. Goldman Relationship between the clinical manifestations of sickle cell disease and the expression of adhesion molecules on white blood cells Eur J Haematol 69 3 2002 135 144
47. S.T. Miller, L.A. Sleeper, C.H. Pegelow, L.E. Enos, W.C. Wang, and S.J. Weiner Prediction of adverse outcomes in children with sickle cell disease N Engl J Med 342 2 2000 83 89
48. S. Charache Mechanism of action of hydroxyurea in the management of sickle cell anemia in adults Semin Hematol 34 3 Suppl 3 1997 15 21
49. F.G. Moser, S.T. Miller, J.A. Bello, C.H. Pegelow, R.A. Zimmerman, and W.C. Wang The spectrum of brain MR abnormalities in sickle-cell disease: a report from the Cooperative Study of Sickle Cell Disease AJNR Am J Neuroradiol 17 5 1996 965 972
50. S.G. Pavlakis, I. Prohovnik, S. Piomelli, and D.C. DeVivo Neurologic complications of sickle cell disease Adv Pediatr 36 1989 247 276
51. M. Koshy, C. Thomas, and J. Goodwin Vascular lesions in the central nervous system in sickle cell disease (neuropathology) J Assoc Acad Minor Phys 1 3 1990 71 78
52. F.D. Armstrong, R.J. Thompson Jr, W. Wang, R. Zimmerman, C.H. Pegelow, and S. Miller Cognitive functioning and brain magnetic resonance imaging in children with sickle Cell disease. Neuropsychology Committee of the Cooperative Study of Sickle Cell Disease Pediatrics 97 6 Pt 1 1996 864 870
53. D. Powars, B. Wilson, C. Imbus, C. Pegelow, and J. Allen The natural history of stroke in sickle cell disease Am J Med 65 3 1978 461 471
54. D.H. Wood Cerebrovascular complications of sickle cell anemia Stroke 9 1 1978 73 75
55. D.R. Powars, W.A. Schroeder, J.N. Weiss, L.S. Chan, and S.P. Azen Lack of influence of fetal hemoglobin levels or erythrocyte indices on the severity of sickle cell anemia J Clin Invest 65 3 1980 732 740
56. R.J. Adams, A. Kutlar, V. McKie, E. Carl, F.T. Nichols, and J.C. Liu Alpha thalassemia and stroke risk in sickle cell anemia Am J Hematol 45 4 1994 279 282
57. B. Balkaran, G. Char, J.S. Morris, P.W. Thomas, B.E. Serjeant, and G.R. Serjeant Stroke in a cohort of patients with homozygous sickle cell disease J Pediatr 120 3 1992 360 366
58. K.J. Wierenga, B.E. Serjeant, and G.R. Serjeant Cerebrovascular complications and parvovirus infection in homozygous sickle cell disease J Pediatr 139 3 2001 438 442
59. R.A. Seeler, and J.E. Royal Commentary: sickle cell anemia, stroke, and transfusion J Pediatr 96 2 1980 243 244
60. S.A. Sarnaik, and J.M. Lusher Neurological complications of sickle cell anemia Am J Pediatr Hematol Oncol 4 4 1982 386 394
61. R. Adams, V. McKie, F. Nichols, E. Carl, D.L. Zhang, and K. McKie The use of transcranial ultrasonography to predict stroke in sickle cell disease N Engl J Med 326 9 1992 605 610
62. S. Kugler, B. Anderson, D. Cross, Z. Sharif, M. Sano, and R. Haggerty Abnormal cranial magnetic resonance imaging scans in sickle-cell disease. Neurological correlates and clinical implications Arch Neurol 50 6 1993 629 635
63. P.E. Houston, S. Rana, S. Sekhsaria, E. Perlin, K.S. Kim, and O.L. Castro Homocysteine in sickle cell disease: relationship to stroke Am J Med 103 3 1997 192 196
64. L.A. Styles, C. Hoppe, W. Klitz, E. Vichinsky, B. Lubin, and E. Trachtenberg Evidence for HLA-related susceptibility for stroke in children with sickle cell disease Blood 95 11 2000 3562 3567
65. F.J. Kirkham, D.K. Hewes, M. Prengler, A. Wade, R. Lane, and J.P. Evans Nocturnal hypoxaemia and central-nervous-system events in sickle-cell disease Lancet 357 9269 2001 1656 1659
66. S.T. Miller, E.A. Macklin, C.H. Pegelow, T.R. Kinney, L.A. Sleeper, and J.A. Bello Silent infarction as a risk factor for overt stroke in children with sickle cell anemia: a report from the Cooperative Study of Sickle Cell Disease J Pediatr 139 3 2001 385 390
67. J.G.T. Taylor, D.C. Tang, S.A. Savage, S.F. Leitman, S.I. Heller, and G.R. Serjeant Variants in the VCAM1 gene and risk for symptomatic stroke in sickle cell disease Blood 100 13 2002 4303 4309
68. C.H. Pegelow, L. Colangelo, M. Steinberg, E.C. Wright, J. Smith, and G. Phillips 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 102 2 1997 171 177
69. J.W. Moohr, H. Wilson, and E.J.M. Pang Strokes and their management in sickle cell disease W. Fried Comparative clinical aspects of sickle cell disease 1982 Elsevier North Holland New York 101 111
70. D.R. Powars, W.Y. Wong, and L.A. Vachon Incomplete cerebral infarctions are not silent J Pediatr Hematol Oncol 23 2 2001 79 83
71. C.H. Pegelow, E.A. Macklin, F.G. Moser, W.C. Wang, J.A. Bello, and S.T. Miller Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease Blood 99 8 2002 3014 3018
72. M.C. Driscoll, A. Hurlet, L. Styles, V. McKie, B. Files, and N. Olivieri Stroke risk in siblings with sickle cell anemia Blood 101 6 2003 2401 2404
73. C. Hoppe, W. Klitz, J. Noble, L. Vigil, E. Vichinsky, and L. Styles Distinct HLA associations by stroke subtype in children with sickle cell anemia Blood 101 7 2003 2865 2869
74. C. Hoppe, W. Klitz, S. Cheng, R. Apple, L. Steiner, and L. Robles Gene interactions and stroke risk in children with sickle cell anemia Blood 103 6 2004 2391 2396
75. L.L. Hsu, S.T. Miller, E. Wright, A. Kutlar, V. McKie, and W. Wang Alpha thalassemia is associated with decreased risk of abnormal transcranial Doppler ultrasonography in children with sickle cell anemia J Pediatr Hematol Oncol 25 8 2003 622 628
76. R.J. Adams, V.C. McKie, L. Hsu, B. Files, E. Vichinsky, and C. Pegelow Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography N Engl J Med 339 1 1998 5 11
77. R.J. Adams, S. Pavlakis, and E.S. Roach Sickle cell disease and stroke: primary prevention and transcranial Doppler Ann Neurol 54 5 2003 559 563
78. S. Charache, J.C. Scott, and P. Charache Acute chest syndrome" in adults with sickle cell anemia. Microbiology, treatment, and prevention Arch Intern Med 139 1 1979 67 69
79. C.T. Quinn, and G.R. Buchanan The acute chest syndrome of sickle cell disease J Pediatr 135 4 1999 416 422
80. E.P. Vichinsky, L.D. Neumayr, A.N. Earles, R. Williams, E.T. Lennette, and D. Dean Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group N Engl J Med 342 25 2000 1855 1865
81. E.P. Vichinsky, L.A. Styles, L.H. Colangelo, E.C. Wright, O. Castro, and B. Nickerson Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease Blood 89 5 1997 1787 1792
82. E.P. Vichinsky Pulmonary hypertension in sickle cell disease N Engl J Med 350 9 2004 857 859
83. D. Powars, J.A. Weidman, T. Odom-Maryon, J.C. Niland, and C. Johnson Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure Medicine (Baltimore) 67 1 1988 66 76
84. L.A. Styles, A.J. Aarsman, E.P. Vichinsky, and F.A. Kuypers Secretory phospholipase A(2) predicts impending acute chest syndrome in sickle cell disease Blood 96 9 2000 3276 3278
85. L.A. Styles, C.G. Schalkwijk, A.J. Aarsman, E.P. Vichinsky, B.H. Lubin, and F.A. Kuypers Phospholipase A2 levels in acute chest syndrome of sickle cell disease Blood 87 6 1996 2573 2578
86. E. Vichinsky, R. Williams, M. Das, A.N. Earles, N. Lewis, and A. Adler Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia Blood 83 11 1994 3107 3112
87. D.R. Powars, A. Hiti, E. Ramicone, C. Johnson, and L. Chan Outcome in hemoglobin SC disease: a four-decade observational study of clinical, hematologic, and genetic factors Am J Hematol 70 3 2002 206 215
88. P.D. Fox, D.T. Dunn, J.S. Morris, and G.R. Serjeant Risk factors for proliferative sickle retinopathy Br J Ophthalmol 74 3 1990 172 176
89. R.J. Hayes, P.I. Condon, and G.R. Serjeant Haematological factors associated with proliferative retinopathy in homozygous sickle cell disease Br J Ophthalmol 65 1 1981 29 35
90. J. Brugarolas, B.F. Haynes, and J.R. Nevins Towards a genomic-based diagnosis Lancet 357 9252 2001 249 250
91. R.E. Banks, M.J. Dunn, D.F. Hochstrasser, J.C. Sanchez, W. Blackstock, and D.J. Pappin Proteomics: new perspectives, new biomedical opportunities Lancet 356 9243 2000 1749 1756
92. M.H. Steinberg, and G.P. Rodgers Pathophysiology of sickle cell disease: role of cellular and genetic modifiers Semin Hematol 38 4 2001 299 306
93. E. Kodish, J. Lantos, C. Stocking, P.A. Singer, M. Siegler, and F.L. Johnson Bone marrow transplantation for sickle cell disease. A study of parents' decisions N Engl J Med 325 19 1991 1349 1353