Page 716 - Hematology_ Basic Principles and Practice ( PDFDrive )
P. 716
Chapter 42 Sickle Cell Disease 607.e1
REFERENCES 27. Smith JA, Espeland M, Bellevue R, et al: Pregnancy in sickle cell
disease: experience of the Cooperative Study of Sickle Cell Disease.
1. Embury SH: The clinical pathophysiology of sickle cell disease. Annu Obstet Gynecol 87:199, 1996.
Rev Med 37:361, 1986. 28. Platt OS, Thorington BD, Brambilla DJ, et al: Pain in sickle cell
2. Pagnier J, Mears JG, Dunda-Belkhodja O, et al: Evidence for the disease. Rates and risk factors. N Engl J Med 325:11, 1991.
multicentric origin of the sickle cell hemoglobin gene in Africa. Proc 29. Embury SH, Dozy AM, Miller J, et al: Concurrent sickle-cell anemia
Natl Acad Sci USA 81:1771, 1984. and α-thalassemia: effect on severity of anemia. N Engl J Med 306:270,
3. Nagel RL, Fleming AF: Genetic epidemiology of the beta s gene. 1982.
Baillieres Clin Haematol 5:331, 1992. 30. Higgs DR, Aldridge BE, Lamb J, et al: The interaction of α-thalassemia
4. Motulsky AG: Frequency of sickling disorders in U.S. blacks. N Engl J and homozygous sickle-cell disease. N Engl J Med 306:1441, 1982.
Med 288:31, 1973. 31. Steinberg MH, Rosenstock W, Coleman MB, et al: The Cooperative
5. Fleming AF: The presentation, management and prevention of crisis in Study of Sickle Cell Disease: effects of thalassemia and microcytosis on
sickle cell disease in Africa. Blood Rev 3:18, 1989. the hematologic and vasoocclusive severity of sickle cell anemia. Blood
6. Lorey F, Cunningham G, Shafer F, et al: Universal screening for 63:1353, 1984.
hemoglobinopathies using high-performance liquid chromatogra- 32. Felice AE, McKie KM, Cleek MP, et al: Effects of α-thalassemia-2 on
phy: clinical results of 2.2 million screens. Eur J Hum Genet 2:262, the developmental changes of hematological values in children with
1994. sickle cell disease from Georgia. Am J Hematol 25:389, 1987.
7. Bunn HF: Subunit assembly of hemoglobin: an important determinant 33. Kwiatkowski JL, Granger S, Brambilla DJ, et al: Elevated blood flow
of hematologic phenotype. Blood 69:1, 1987. velocity in the anterior cerebral artery and stroke risk in sickle cell disease:
8. Dover GJ, Boyer SH, Charache S, et al: Individual variation in the extended analysis from the STOP trial. Br J Haematol 134:333, 2006.
production and survival of F cells in sickle-cell disease. N Engl J Med 34. Valadi N, Silva GS, Bowman LS, et al: Transcranial Doppler ultraso-
299:1428, 1978. nography in adults with sickle cell disease. Neurology 67:572, 2006.
9. Nagel RL, Ranney HM: Genetic epidemiology of structural mutations 35. Wong WY, Overturf GD, Powars DR: Infection caused by Strepto-
of the beta-globin gene. Semin Hematol 27:342, 1990. coccus pneumoniae in children with sickle cell disease: epidemiology,
10. Gaston MH, Verter JI, Woods G, et al: Prophylaxis with oral penicillin immunologic mechanisms, prophylaxis, and vaccination. Clin Infect
in children with sickle cell anemia. A randomized trial. N Engl J Med Dis 14:1124, 1992.
314:1593, 1986. 36. John AB, Ramlal A, Jackson H, et al: Prevention of pneumococcal
11. Consensus Conference: Newborn screening for sickle cell disease and infection in children with homozygous sickle cell disease. Br Med J
other hemoglobinopathies. JAMA 258:1205, 1987. (Clin Res Ed) 288:1567, 1984.
12. Tsevat J, Wong JB, Pauker SG, et al: Neonatal screening for sickle cell 37. Falletta JM, Woods GM, Verter JI, et al: Discontinuing penicillin
disease: a cost-effectiveness analysis. J Pediatr 118:546, 1991. prophylaxis in children with sickle cell anemia. Prophylactic Penicillin
13. O’Brien RT, McIntosh S, Aspnes GT, et al: Prospective study of sickle Study II. J Pediatr 127:685, 1995.
cell anemia in infancy. J Pediatr 89:205, 1976. 38. Hord J, Byrd R, Stowe L, et al: Streptococcus pneumoniae sepsis and
14. Jones S, Shickle DA, Goldstein AR, et al: Acceptability of antenatal meningitis during the penicillin prophylaxis era in children with sickle
diagnosis for sickle-cell disease among Jamaican mothers and female cell disease. J Pediatr Hematol Oncol 24:470, 2002.
patients. West Indian Med J 37:12, 1988. 39. Hongeng S, Wilimas JA, Harris S, et al: Recurrent Streptococcus
15. Diggs LW: Bone and joint lesions in sickle-cell disease. Clin Orthop pneumoniae sepsis in children with sickle cell disease. J Pediatr 130:814,
Relat Res 52:119, 1967. 1997.
16. Platt OS, Brambilla DJ, Rosse WF, et al: Mortality in sickle cell disease. 40. Perrine RP, Pembrey ME, John P, et al: Natural history of sickle cell
Life expectancy and risk factors for early death. N Engl J Med 330:1639, anemia in Saudi Arabs. A study of 270 subjects. Ann Intern Med 88:1,
1994. 1978.
17. Powars DR, Chan LS, Hiti A, et al: Outcome of sickle cell anemia: a 41. Goldberg MA, Husson MA, Bunn HF: Participation of hemoglobins A
4-decade observational study of 1056 patients. Medicine (Baltimore) and F in polymerization of sickle hemoglobin. J Biol Chem 252:3414,
84:363, 2005. 1977.
18. Steinberg MH, Barton F, Castro O, et al: Effect of hydroxyurea on 42. Nagel RL, Bookchin RM, Johnson J, et al: Structural bases of the
mortality and morbidity in adult sickle cell anemia: risks and benefits inhibitory effects of hemoglobin F and hemoglobin A2 on the polym-
up to 9 years of treatment. JAMA 289:1645, 2003. erization of hemoglobin S. Proc Natl Acad Sci USA 76:670, 1979.
19. Saraf S, Farooqui M, Infusino G, et al: Standard clinical practice 43. Charache S, Barton FB, Moore RD, et al: Hydroxyurea and sickle cell
underestimates the role and significance of erythropoietin deficiency in anemia. Clinical utility of a myelosuppressive “switching” agent. The
sickle cell disease. Br J Haematol 153:386, 2011. Multicenter Study of Hydroxyurea in Sickle Cell Anemia. Medicine
20. Thomas PW, Higgs DR, Serjeant GR: Benign clinical course in homo- (Baltimore) 75:300, 1996.
zygous sickle cell disease: a search for predictors. J Clin Epidemiol 44. Charache S, Terrin ML, Moore RD, et al: Effect of hydroxyurea on
50:121, 1997. the frequency of painful crises in sickle cell anemia. Investigators of
21. Steinberg MH: Predicting clinical severity in sickle cell anaemia. Br J the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl
Haematol 129:465, 2005. J Med 332:1317, 1995.
22. Platt OS, Brambilla DJ, Rosse WF, et al: Mortality in sickle cell disease. 45. Orringer EP, Blythe DS, Johnson AE, et al: Effects of hydroxyurea on
Life expectancy and risk factors for early death. N Engl J Med 330:1639, hemoglobin F and water content in the red blood cells of dogs and of
1994. patients with sickle cell anemia. Blood 78:212, 1991.
23. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al: Cerebrovascular 46. Kinney TR, Helms RW, O’branski EE, et al: Safety of hydroxyurea in
accidents in sickle cell disease: rates and risk factors. Blood 91:288, children with sickle cell anemia: results of the HUG-KIDS study, a
1998. phase I/II trial. Pediatric Hydroxyurea Group. Blood 94:1550, 1999.
24. Guasch A, Cua M, Mitch WE: Early detection and the course of 47. Ware RE, Eggleston B, Redding-Lallinger R, et al: Predictors of fetal
glomerular injury in patients with sickle cell anemia. Kidney Int 49:786, hemoglobin response in children with sickle cell anemia receiving
1996. hydroxyurea therapy. Blood 99:10, 2002.
25. Faulkner M, Turner EA, Deus J, et al: Severe anemia: a risk factor 48. Ferster A, Tahriri P, Vermylen C, et al: Five years of experience with
for glomerular injury in sickle cell disease. J Natl Med Assoc 87:209, hydroxyurea in children and young adults with sickle cell disease. Blood
1995. 97:3628, 2001.
26. Morris JS, Dunn DT, Poddar D, et al: Haematological risk factors for 49. Ware RE, Zimmerman SA, Schultz WH: Hydroxyurea as an alternative
pregnancy outcome in Jamaican women with homozygous sickle cell to blood transfusions for the prevention of recurrent stroke in children
disease. Br J Obstet Gynaecol 101:770, 1994. with sickle cell disease. Blood 94:3022, 1999.

