Pathophysiology and treatment of sickle-cell disease;Pathofysiologie en behandeling van sikkelcelziekte

Nederlands Tijdschrift voor Geneeskunde

Volumn 149, Issue 21, 2005, Pages 1144-1149

  Van Beers, E J ^a   Peters, M ^a   Biemond, B J ^a  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

ARGININE; BETA GLOBIN; HEMOGLOBIN F; HEMOGLOBIN S; HYDROXYUREA; METHYLPREDNISOLONE; POLYMER; SALAZOSULFAPYRIDINE;

ALLOGENEIC STEM CELL TRANSPLANTATION; BLOOD CLOTTING; BLOOD TRANSFUSION; BLOOD VESSEL OCCLUSION; CELL ACTIVATION; DISEASE SEVERITY; ENDOTHELIUM CELL; ERYTHROCYTE ADHESIVENESS; ERYTHROCYTE SHAPE; GENE MUTATION; GLOBIN GENE; GRAFT VERSUS HOST REACTION; HEMOGLOBINOPATHY; HLA MATCHING; HUMAN; HYDRATION; HYPOXIA; LEUKOCYTE ADHERENCE; LIFE EXPECTANCY; MORTALITY; ORGAN INJURY; PAIN; PATHOPHYSIOLOGY; PHENOTYPE; RECURRENT DISEASE; REVIEW; SICKLE CELL ANEMIA; VASOCONSTRICTION;

ANEMIA, SICKLE CELL; ANTISICKLING AGENTS; CELL ADHESION; COMBINED MODALITY THERAPY; ERYTHROCYTES; HUMANS; HYDROXYUREA; STEM CELL TRANSPLANTATION;

EID: 19944372071     PISSN: 00282162     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Review

References (31)

1. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994;330:1639-44.
2. Sears DA. Sickle cell trait. In: Embury SH, Hebbel RP, Mohandas N, Steinberg MH, editors. Sickle cell disease: basic principles and clinical practice. New York: Raven Press; 1994. p. 381-94.
3. Heijboer H, Tweel XW van den, Peters M, Knuist M, Prins J, Heymans HSA. Een jaar neonatale screening op sikkelcelziekte in het Emma Kinderziekenhuis/ Academisch Medisch Centrum te Amsterdam. Ned Tijdschr Geneeskd 2001;145:1795-9.
4. Giordano PC, Bouva MJ, Harteveld CL. A confidential inquiry estimating the number of patients affected with sickle cell disease and thalassemia major confirms the need for a prevention strategy in the Netherlands. Hemoglobin 2004;28:287-96.
5. Stuart MJ, Nagel RL. Sickle-cell disease. Lancet 2004;364:1343-60.
6. Driscoll MC, Hurlet A, Styles L, McKie V, Files B, Olivieri N, et al. Stroke risk in siblings with sickle cell anemia. Blood 2003;101:2401-4.
7. Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000;342:1855-65.
8. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95.
9. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med 1997;337:762-9.
10. Kaul DK, Fabry ME, Costantini F, Rubin EM, Nagel RL. In vivo demonstration of red cell-endothelial interaction, sickling and altered microvascular response to oxygen in the sickle transgenic mouse. J Clin Invest 1995;96:2845-53.
11. Turhan A, Weiss LA, Mohandas N, Coller BS, Frenette PS. Primary role for adherent leukocytes in sickle cell vascular occlusion: a new paradigm. Proc Natl Acad Sci USA 2002;99:3047-51.
12. Solovey A, Lin Y, Browne P, Choong S, Wayner E, Hebbel RP. Circulating activated endothelial cells in sickle cell anemia. N Engl J Med 1997;337:1584-90.
13. Embury SH, Matsui NM, Ramanujam S, Mayadas TN, Noguchi CT, Diwan BA, et al. The contribution of endothelial cell P-selectin to the microvascular flow of mouse sickle erythrocytes in vivo. Blood 2004;104:3378-85.
14. Reiter CD, Wang X, Tanus-Santos JE, Hogg N, Cannon 3rd RO, Schechter AN, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med 2002;8:1383-9.
15. Xia Y, Dawson VL, Dawson TM, Snyder SH, Zweier JL. Nitric oxide synthase generates superoxide and nitric oxide in arginine-depleted cells leading to peroxynitrite-mediated cellular injury. Proc Natl Acad Sci USA 1996;93:6770-4.
16. Ergul S, Brunson CY, Hutchinson J, Tawfik A, Kutlar A, Webb RC, et al. Vasoactive factors in sickle cell disease: in vitro evidence for endothelin-1-mediated vasoconstriction. Am J Hematol 2004;76:245-51.
17. Ataga KI, Orringer EP. Hypercoagulability in sickle cell disease: a curious paradox. Am J Med 2003;115:721-8.
18. Setty BNY, Rao AK, Stuart MJ. Thrombophilia in sickle cell disease: the red cell connection. Blood 2001;98:3228-33.
19. Sugama Y, Tiruppathi C, Offakidevi K, Andersen TT, Fenton 2nd JW, Malik AB. Thrombin-induced expression of endothelial P-selectin and intercellular adhesion molecule-1: a mechanism for stabilizing neutrophil adhesion. J Cell Biol 1992;119:935-44.
20. Koshy M, Burd L, Wallace D, Moawad A, Baron J. Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study. N Engl J Med 1988;319:1447-52.
21. Wierenga KJJ. Neonatale screening op sikkelcelziekte. Ned Tijdschr Geneeskd 1997;141:184-7.
22. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. 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 1995;332:1317-22.
23. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA 2003;289:1645-51.
24. Kaul DK, Liu XD, Choong S, Belcher JD, Vercellotti GM, Hebbel RP. Anti-inflammatory therapy ameliorates leukocyte adhesion and microvascular flow abnormalities in transgenic sickle mice. Am J Physiol Heart Circ Physiol 2004;287:H293-301.
25. Solovey AA, Solovey AN, Harkness J, Hebbel RP. Modulation of endothelial cell activation in sickle cell disease: a pilot study. Blood 2001;97:1937-41.
26. Griffin TC, McIntire D, Buchanan GR. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. N Engl J Med 1994;330:733-7.
27. Belcher JD, Mahaseth H, Welch TE, Vilback AE, Sonbol KM, Kalambur VS, et al. Critical role of endothelial cell activation in hypoxia-induced vaso-occlusion in transgenic sickle mice. Am J Physiol Heart Circ Physiol [ter perse].
28. Pace BS, Shartava A, Pack-Mabien A, Mulekar M, Ardia A, Goodman SR. Effects of N-acetylcysteine on dense cell formation in sickle cell disease. Am J Hematol 2003;73:26-32.
29. Morris CR, Kuypers FA, Larkin S, Sweeters N, Simon J, Vichinsky EP, et al. Arginine therapy: a novel strategy to induce nitric oxide production in sickle cell disease. Br J Haematol 2000;111:498-500.
30. Weiner DL, Hibberd PL, Betit P, Cooper AB, Botelho CA, Brugnara C. Preliminary assessment of inhaled nitric oxide for acute vaso-occlusive crisis in pediatric patients with sickle cell disease. JAMA 2003;289:1136-42.
31. Vermylen C. Hematopoietic stem cell transplantation in sickle cell disease. Blood Rev 2003;17:163-6.