Treatment of sickling disorders

Current Opinion in Hematology

Volumn 3, Issue 2, 1996, Pages 139-144

  Charache, Samuel ^a  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BETA GLOBIN; BUTYRIC ACID; BUTYRIC ACID DERIVATIVE; CLOTRIMAZOLE; GAMMA GLOBIN; HEMOGLOBIN F; HEMOGLOBIN S; HYDROXYUREA; METHYLPREDNISOLONE; MICONAZOLE;

APHERESIS; BLOOD TRANSFUSION; CLINICAL TRIAL; DRUG MECHANISM; GLOBIN GENE; HEMOGLOBIN SYNTHESIS; HUMAN; HYPERTHERMIA; INTRAVENOUS DRUG ADMINISTRATION; IRON DEFICIENCY ANEMIA; MEAN CORPUSCULAR HEMOGLOBIN; NONHUMAN; PATIENT COUNSELING; PRIORITY JOURNAL; PSYCHOTHERAPY; REVIEW; RISK BENEFIT ANALYSIS; SICKLE CELL ANEMIA; SICKLE CELL TRAIT; SUDDEN DEATH;

EID: 0029892515     PISSN: 10656251     EISSN: None     Source Type: Journal    
DOI: 10.1097/00062752-199603020-00006     Document Type: Review

References (57)

1. Kark JA, Ward FT: Exercise and hemoglobin S. Semin Hematol 1994, 31:181-225. Sudden death in military recruits with sickle cell trait is usually due to the consequences of heat disease.
2. Gardner JW, Kark JA: Fatal rhabdomyolysis presenting as mild heat illness in military training. Mil Med 1994, 159:160-163.
3. Le Gallais D, Prefaut C, Mercier J, Bile A, Bogui P, Lonsdorfer J: Sickle cell trait as a limiting factor for high-level performance in a semimarathon. Int J Sports Med 1994, 15:399-402.
4. Thiriet P, Le Hasran JY, Wouassi D, Bitanga E, Gozal D, Louis FJ: Sickle cell trait performance in a prolonged race at high altitude. Med Sci Sports Exerc 1994, 26:914-918.
5. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug P: Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med 1994, 330:1639. Median ages at death were 42 and 48 years for men and women with sickle cell anemia. Risk factors for early death include acute chest syndrome, renal failure, seizures, a high leukocyte count, and a low fetal hemoglobin concentration.
6. James TN, Riddick L, Massing GK: Sickle cells and sudden death: morphologic abnormalities of the cardiac conduction system. J Lab Clin Med 1994, 124:507-520.
7. Charache S: Sickle cells and sudden death (editorial). J Lab Clin Med 1994, 124:473-474.
8. Barbarin OA, Whitten CF, Bonds SM: Estimating rates of psychosocial problems in urban and poor children with sickle cell anemia. Health Soc Work 1994, 19:112-119.
9. Lemanek KL, Horwitz W, Ohene-Frempong K: A multiperspective investigation of social competence in children with sickle cell disease. J Pediatr Psychol 1994, 19:443-456.
10. Cohen MJ, Branch WB, McVie VC, Adams RJ: Neuropsychological impairment in children with sickle cell anemia and cerebrovascular accidents. Clin Pediatr 1994, 33:517-524.
11. Thompson RJ Jr, Gil KM, Gustafson KE, George LK, Keith BR, Spock A, Kinney TR: Stability and change in the psychological adjustment of mothers of children and adolescents with cystic fibrosis and sickle cell disease. J Pediatr Psychol 1994, 19:171-188.
12. Bennett DS: Depression among children with chronic medical problems: a meta-analysis. J Pediatr Psychol 1994, 19:149-169.
13. Sharpe JN, Brown RT, Thompson NJ, Eckman J: Predictors of coping with pain in mothers and their children with sickle cell syndrome. J Am Acad Child Adolesc Psychiatry 1994, 33:1246-1255.
14. Thompson RJ Jr, Gil KM, Keith BR, Gustafson KE, George LK, Kinney TR: Psychological adjustment of children with sickle cell disease: stability and change over a 10-month period. J Consult Clin Psychol 1994, 62:856.
15. Kunkel N, Rackoff WR, Katolik L, Ohene-Frempong K: Utilization of a pediatric emergency department by patients with sickle cell disease. Pediatr Emerg Care 1994, 10:79-82.
16. Hand R, Koshy M, Dorn L, Patel M: Health insurance status and the use of emergency and other outpatient services by adults with sickle cell disease. Ann Emerg Med 1995, 25:224-229.
17. Yang Y-M, Shah AK, Watson M, Mankad VN: Comparison of costs to the health sector of comprehensive and episodic health care for sickle cell disease patients. Public Health Rep 1995, 110:80-86.
18. Griffin TC, McIntire D, Buchanan GR: High-dose intravenous methyl-prednisolone therapy for pain in children and adolescents with sickle cell disease. N Engl J Med 1994, 330:733-737. Methylprednisolone therapy shortened painful sickle cell crises, but they tended to recur and it was not clear if pain was only covered up.
19. West TB, West DW, Ohene-Frempong K: The presentation, frequency, and outcome of bacteremia among children with sickle cell disease and fever. Pediatr Emerg Care 1994, 10:141-143.
20. Hassell KL, Eckman JR, Lane PA: Acute multiorgan failure syndrome: a potentially catastrophic complication of severe sickle cell pain episodes. Am J Med 1994, 96:155-162.
21. Anderson R, Cassell M, Mullinax G, Chaplin H: Effect of normal cells on viscosity of sickle cell blood. Arch Intern Med 1963, 111:286-294.
22. Zinkham WH, Seidler AJ, Kickler TS: Variable degrees of suppression of hemoglobin S synthesis in subjects with hemoglobin SS disease on a long-term transfusion regimen. J Pediatr 1994, 124:215-219.
23. Styles LA, Vichinsky E: Effects of a long-term transfusion regimen on sickle cell-related illnesses. J Pediatr 1994, 125:909-911.
24. Wong W-Y, Powars DR, Operskalski EA, Hassett J, Parker JW, Sarnaik S, Pegelow CH, Hilgartner MW, Johnson CS, Zhou Y, Ku P-Y, Mosley JW, The Transfusion Safety Study Group: Blood transfusions and immunophenotypic alterations of lymphocyte subsets in sickle cell anemia. Blood 1995, 85:2091-2097.
25. Hurlet-Jensen AM, Prohovnik I, Pavlakis SG, Piomelli S: Effects of total hemoglobin and hemoglobin S concentration on cerebral blood flow during transfusion therapy to prevent stroke in sickle cell disease. Stroke 1994, 25:1688-1692.
26. Venketasubramanian N, Prohovnik I, Hurlet A, Mohr JP, Piomelli S: Middle cerebral artery velocity changes during transfusion in sickle cell anemia. Stroke 1994, 25:2153-2158.
27. Howard RJ, Tuck SM, Pearson TC: Blood transfusion in pregnancies complicated by maternal sickle cell disease: effects on blood rheology and transplacental velocimetry. Clin Lab Haematol 1994, 16:253-259.
28. Vichinsky EP, Haberkern CM, Neumayr L, Earles AN, Black D, Koshy M1 Pegelow C, Abboud M, Ohene-Frempong K, Iyer RV, Preoperative Transfusion in Sickle Cell Disease Study Group: A comparison of conservative and aggressive transfusion regimens in the perloperative management of sickle cell disease. N Engl J Med 1995, 333:206-213. An aggressive transfusion regimen was no more effective than a conservative one in preventing perioperative complications of sickle cell disease but was associated with an increased risk of transfusion-related complications.
29. Pearlman ES, Ballas SK: When to transfuse blood in sickle cell disease? Lessons from Jehovah's Witnesses. Ann Clin Lab Sci 1994, 24:396.
30. Viele MK, Weiskopf RB: What can we learn about the need for transfusion from patients who refuse blood? The experience with Jehovah's Witnesses. Transfusion 1994, 34:396-401.
31. Tahhan HR, Holbrook CT, Braddy LR, Brewer LD, Christie JD: Antigen-matched donor blood in the transfusion management of patients with sickle cell disease. Transfusion 1994, 34:558-560.
32. Issitt PD: Race-related red cell alloantibody problems. Br J Biomed Sci 1994, 51:158-167.
33. Norol F, Nadjahi J, Bachir D, Desaint C, Guillou-Bataille M, Beaujean F, Bierling P, Bonin P, Galacteros F, Duedari N: Transfusion and alloimmunization in sickle cell anemia patients. Transfus Clin Biol 1994, 1:27-34.
34. Eaton WA, Hofrichter J: The biophysics of sickle cell hydroxyurea therapy. Science 1995, 268:1142-1143.
35. Brown AK, Sleeper LA, Pegelow CH, Miller ST, Gill FM, Waclawiw MA: The influence of infant and maternal sickle cell disease on birth outcome and neonatal course. Arch Pediatr Adolesc Med 1994, 148:1156-1162.
36. de Simone J, Heller P, Hall L, Zwiers D: 5-Azacytidine stimulates fetal hemoglobin synthesis in anemic baboons. Proc Natl Acad Sci U S A 1982, 79:4428-4431.
37. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR, and the investigators of the Multicenter Study of Hydroxyurea: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 1995, 332:1317-1322. Treatment with hydroxyurea decreases the frequency of sickle cell crises, hospitalizations, attacks of acute chest syndrome, and use of blood transfusion. No adverse effects were encountered, but long-term safety is uncertain.
38. Lu Z-H, Steinberg MH, Multicenter Study of Hydroxyurea: Fetal hemoglobin concentration in sickle cell anemia (HbSS): relation to potential regulatory regions cis to the beta-globin gene [abstract]. Blood 1994, 84:404a.
39. Vichinsky EP, Lubin BH: A cautionary note regarding hydroxyurea in sickle cell disease. Blood 1994, 83:1124-1128.
40. Claster S, Vichinsky E: Splenic regeneration in two sickle cell patients treated with hydroxyurea [abstract]. Blood 1994, 84:219a.
41. Voskaridou E, Kalotychou V, Loukopoulos D: Clinical and laboratory effects of long-term administration of hydroxyurea to patients with sickle-cell/beta thalassaemia. Br J Haematol 1995, 89:479-484.
42. Wiger R, Hongslo JK, Evenson DP, De Angelis P, Schwarze PE, Holme JA: Effects of acetaminophen and hydroxyurea on spermatogenesis and sperm chromatin structure in laboratory mice. Reprod Toxicol 1995, 9:21-23.
43. Weinfeld A, Swolin B, Westin J: Acute leukaemia after hydroxyurea therapy in polycythaemia vera and allied disorders: prospective study of efficacy and leukaemogenicity with therapeutic implications. Eur J Haematol 1994, 52:134-139. Patients treated with hydroxyurea for myeloproliferative disorders had a significantly increased frequency of acute leukemia compared with patients treated only by phlebotomy.
44. Fruchtman SM, Kaplan ME, Peterson P, Mack K, Berk PD, Wasserman LR for the Polycythemia Vera Study Group: Acute leukemia, hydroxyurea and polycythemia vera: an analysis of risk from the polycythemia vera study group [abstract]. Blood 1994, 84:518a. Patients treated with hydroxyurea for polycythemia vera had an increased frequency of acute leukemia when compared with phlebotomized patients, but the difference between the two groups was not statistically significant.
45. Triadou P, Maier-Redelsperger M, Krishnamoorty R, Deschamps A, Casadevall A, Dunda O, Ducrocq R, Elion J, Girot R, Labie D, Dover G, Cornu P: Fetal haemoglobin variations following hydroxyurea treatment in patients with cyanotic congenital heart disease. Nouv Rev Fr Hematol 1994, 36:367-372. Patients treated with hydroxyurea for secondary polycythemia did not develop acute leukemia after follow-up of 2 to 15 years.
46. Dover GJ, Brusilow S, Charache S: Induction of fetal hemoglobin production in subjects with sickle cell anemia by oral sodium phenylbutyrate. Blood 1994, 84:339-343.
47. Collins AF, Pearson HA, Giardina P, McDonagh KT, Brusilow SW, Dover GJ: Oral sodium phenylbutyrate therapy in homozygous beta thalassemia: a clinical trial. Blood 1995, 85:43-49.
48. Perrine SP, Dover GJ, Daftari P, Walsh CT, Jin Y-X, Mays A, Faller DV: Isobutyramide, an orally available butyrate analogue, stimulates fetal globin gene expression in vitro and in vivo. Br J Haematol 1994, 88:555-561.
49. Stamatoyannopoulos G, Blau CA, Nakamoto B, Josephson B, Li Q, Liakopoulou E, Pace B, Papayannopoulou T, Brusilow SW, Dover GJ: Fetal hemoglobin induction by acetate, a product of butyrate catabolism. Blood 1994, 84:3198-3204.
50. Collins AF, Dover GJ, Luban NLC: Increased fetal hemoglobin production in patients receiving valproic acid for epilepsy. Blood 1994, 84:1690-1691.
51. Newmark HL, Lupton JR, Young CW: Butyrate as a differentiating agent: pharmacokinetics, analogues and current status. Cancer Lett 1994, 78:1-5.
52. Safaya S, Ibrahim A, Rieder RF: Augmentation of gamma-globin gene promoter activity by carboxylic acids and components of the human beta-globin locus control region. Blood 1994, 84:3929-3935.
53. Blau CA, Constantoulakis P, Shaw CM, Stamatoyannopoulos G: Fetal hemoglobin induction with butyric acid: efficacy and toxicity. Blood 1993, 81:529-537.
54. De Franceschi L, Saadane N, Trudel M, Alper SL, Brugnara C, Beuzard Y: Treatment with oral clotrimazole blocks Ca(2+)-activated K+ transport and reverses erythrocyte dehydration in transgenic SAD mice. J Clin Invest 1994, 93:1670-1676. Inhibition of the Gardos channel of ion transport increases erythrocyte hydration and may provide a new approach to prevention of sickling in patients.
55. Poillon WN, Kim BC, Labotka RJ, Hicks CU, Kark JA: Antisickling effects of 2,3-diphosphoglycerate depletion. Blood 1995, 85:3289-3296.
56. Stuart J, Mojiminiyi FB, Stone PC, Culliford SJ, Ellory JC: Additive in vitro effects of anti-sickling drugs. Br J Haematol 1994, 86:820-823.
57. Castro O, Poillon WN, Finke H, Massac E: Improvement of sickle cell anemia by iron-limited erythropoiesis. Am J Hematol 1994, 47:74-81.