Diagnosis and management of sickle cell disorders

Methods in Molecular Biology

Volumn 663, Issue , 2010, Pages 291-307

  Mousa, Shaker A ^a   Qari, Mohamad H ^b  

^a ALBANY COLLEGE OF PHARMACY AND HEALTH SCIENCES   (United States)

^b KING ABDULAZIZ UNIVERSITY   (Saudi Arabia)

Author keywords

diagnosis; guidelines; low molecular weight heparin; management; Painful crisis; prophylaxis; sickle cell; vaso occlusive crisis

Indexed keywords

ANIMAL; FEMALE; HUMAN; PAIN; PATHOLOGY; PREGNANCY; PREGNANCY COMPLICATION; REVIEW; SICKLE CELL ANEMIA;

ANEMIA, SICKLE CELL; ANIMALS; FEMALE; HUMANS; PAIN; PREGNANCY; PREGNANCY COMPLICATIONS;

EID: 77957662581     PISSN: 10643745     EISSN: None     Source Type: Book Series    
DOI: 10.1007/978-1-60761-803-4_13     Document Type: Article

References (81)

1. Jeremiah, Z.A. (2006) Abnormal haemoglobin variants, ABO and Rh blood groups among student of African descent in Port Harcourt, Nigeria Afr Health Sci 6, 177-81.
2. Hiran, S. (2005) Multiorgan dysfunction syndrome in sickle cell disease J Assoc Physic India 53, 19-22.
3. Alhamdan, N.A., Almazrou, Y.Y., Alswaidi, F.M., and Choudhry, A.J. (2007) Premarital screening for thalassemia and sickle cell disease in Saudi Arabia Genet Med 9, 372-7.
4. Al-Riyami, A., and Ebrahim, G.J. (2003) Genetic blood disorders survey in the Sultanate of Oman J Trop Pediatr 49(Suppl 1), i1-20.
5. Mohammed, A.M., Al-Hilli, F., Nadkarni, K.V., Bhagwat, G.P., and Bapat, J.P. (1992) Hemoglobinopathies and glucose-6- phosphate dehydrogenase deficiency in hospital births in Bahrain Ann Saudi Med 12, 536-9.
6. el-Hazmi, M.A., and Warsy, A.S. (1999) Appraisal of sickle-cell and thalassaemia genes in Saudi Arabia East Mediterr Health J 5, 1147-53.
7. El-Hazmi, M.A., and Warsy, A.S. (1994) The frequency of glucose-6-phosphate dehydrogenase phenotypes and sickle cell genes in Al-Qatif oasis Ann Saudi Med 14, 491-4.
8. Qari, M.H., Aljaouni, S.K., Alardawi, M.S., Fatani, H., Alsayes, F.M., Zografos, P., Alsaigh, M., Alalfi, A., Alamin, M., Gadi, A., andMousa, S.A. (2007) Reduction of painful vaso-occlusive crisis of sickle cell anaemia by tinzaparin in a double-blind randomized trial Thromb Haemost 98, 392-6.
9. Aslan, M., and Freeman, B.A. (2007) Redoxdependent impairment of vascular function in sickle cell disease Free Radic Biol Med 43, 1469-83.
10. Hebbel, R.P., Osarogiagbon, R., and Kaul, D. (2004) The endothelial biology of sickle cell disease: Inflammation and a chronic vasculopathy Microcirculation 11, 129-51.
11. Mehta, S.R., Afenyi-Annan, A., Byrns, P.J., and Lottenberg, R. (2006) Opportunities to improve outcomes in sickle cell disease Am Fam Physician 74, 303-10.
12. Hebbel, R.P., Boogaerts, M.A., Eaton, J.W., and Steinberg, M.H. (1980) Erythrocyte adherence to endothelium in sickle-cell anemia. A possible determinant of disease severity N Engl J Med 302, 992-5.
13. Grigg, A.P. (2001) Granulocyte colonystimulating factor-induced sickle cell crisis and multiorgan dysfunction in a patient with compound heterozygous sickle cell/beta+thalassemia Blood 97, 3998-9.
14. Ataga, K.I., and Key, N.S. (2007) Hypercoagulability in sickle cell disease: New approaches to an old problem Hematol Am Soc Hematol Educ Program 2007, 91-6.
15. Inwald, D.P., Kirkham, F.J., Peters, M.J., Lane, R., Wade, A., Evans, J.P., and Klein, N.J. (2000) Platelet and leucocyte activation in childhood sickle cell disease: Association with nocturnal hypoxaemia Br J Haematol 111, 474-81.
16. Tomer, A., Harker, L.A., Kasey, S., and Eckman, J.R. (2001) Thrombogenesis in sickle cell disease J Lab Clin Med 137, 398-407.
17. Key, N.S., Slungaard, A., Dandelet, L., Nelson, S.C., Moertel, C., Styles, L.A., Kuypers, F.A., and Bach, R.R. (1998) Whole blood tissue factor procoagulant activity is elevated in patients with sickle cell disease Blood 91, 4216-23.
18. Ataga, K.I., Moore, C.G., Hillery, C.A., Jones, S., Whinna, H.C., Strayhorn, D., Sohier, C., Hinderliter, A., Parise, L.V., and Orringer, E.P. (2008) Coagulation activation and inflammation in sickle cell disease-associated pulmonary hypertension Haematologica 93, 20-6.
19. Etienne-Julan, M., Belloy, M.S., Decastel, M., Dougaparsad, S., Ravion, S., and Hardy- Dessources, M.D. (2004) Childhood sickle cell crises: Clinical severity, inflammatory markers and the role of interleukin-8 Haematologica 89, 863-4.
20. Ballas, S.K. (2007) Current issues in sickle cell pain and its management Hematol Am Soc Hematol Educ Prog 2007, 97-105.
21. Frenette, P.S., and Atweh, G.F. (2007) Sickle cell disease: Old discoveries, new concepts, and future promise J Clin Invest 117, 850-8.
22. Al-Absi, I.K., Al-Subaie, A.M., Ameen, G., Mahdi, N., Mohammad, A.M., Fawaz, N.A., and Almawi, W.Y. (2006) Association of the methylenetetrahydrofolate reductase A1298C but not the C677T single nucleotide polymorphism with sickle cell disease in Bahrain Hemoglobin 30, 449-53.
23. Mid-Atlantic Regional Human Genetics Network (MARHGN). Sickle cell disease in children and adolescents: Diagnosis, guidelines for comperhensive care, and protocols for management of acute and chronic complications. In: Mid-Atlantic Sickle Cell Disease Consortium (MASCC) Practice Guidelines Workgroup;2002.
24. Shapiro, B.S. (1989) The management of pain in sickle cell disease Pediatr Clin North Am 36, 1029-45.
25. Platt, O.S., Thorington, B.D., Brambilla, D.J., Milner, P.F., Rosse, W.F., Vichinsky, E., and Kinney, T.R. (1991) Pain in sickle cell disease. Rates and risk factors N Engl J Med 325, 11-6.
26. Platt, O.S., Brambilla, D.J., Rosse, W.F., Milner, P.F., Castro, O., Steinberg, M.H., and Klug, P.P. (1994) Mortality in sickle cell disease. Life expectancy and risk factors for early death N Engl J Med 330, 1639-44.
27. Barakat, L.P., Patterson, C.A., Daniel, L.C., and Dampier, C. (2008) Quality of life among adolescents with sickle cell disease: Mediation of pain by internalizing symptoms and parenting stress Health Qual Life Outcomes 6, 60.
28. Hassell, K.L., Eckman, J.R., and Lane, P.A. (1994) Acute multiorgan failure syndrome: A potentially catastrophic complication of severe sickle cell pain episodes Am J Med 96, 155-62.
29. Robieux, I.C., Kellner, J.D., Coppes, M.J., Shaw, D., Brown, E., Good, C., O'Brodovich, H., Manson, D., Olivieri, N.F., Zipursky, A., et al. (1992) Analgesia in children with sickle cell crisis: Comparison of intermittent opioids vs. continuous intravenous infusion of morphine and placebo-controlled study of oxygen inhalation Pediatr Hematol Oncol 9, 317-26.
30. Yale, S.H., Nagib, N., and Guthrie, T. (2000) Approach to the vaso-occlusive crisis in adults with sickle cell disease Am Fam Physic 61, 1349-56, 63-4.
31. Benjamin, L., Dampier, C., Jacox, A., Odesina, V., Pheonix, D., Shapiro, B., Strafford, M., and Treadwell, M. Guideline for the Management of Acute and Chronic Pain in Sickle-Cell Disease. American Pain Society;1999.
32. American Academy of Pediatrics, S.o.H.O. C.o.G.A.A.o. (2002) Health supervision for children with sickle cell disease Pediatrics 109, 526-35.
33. Preboth, M. (2000) Management of pain in sickle cell disease Am Fam Physician 61, 1544, 9-50.
34. Reid, C., Charache, S., and Lubin, B.e. Management of Therapy of Sickle Cell Disease, 3rd edition. Bethesda, MD: National Institutes of Health;1995.
35. Jacobson, S.J., Kopecky, E.A., Joshi, P., and Babul, N. (1997) Randomised trial of oral morphine for painful episodes of sickle-cell disease in children Lancet 350, 1358-61.
36. Zempsky, W.T., Loiselle, K.A., McKay, K., Blake, G.L., Hagstrom, J.N., Schechter, N.L., and Kain, Z.N. (2008) Retrospective evaluation of pain assessment and treatment for acute vasoocclusive episodes in children with sickle cell disease Pediatr Blood Cancer 51, 265-8.
37. Panel, S.C.D.G. Sickle Cell Disease: Screening, Diagnosis, Management and Counseling in Newborns and Infants. Rockville, MD: AHCPR;1993.
38. Field, J.J., Knight-Perry, J.E., and Debaun, M.R. (2009) Acute pain in children and adults with sickle cell disease: Management in the absence of evidence-based guidelines Current Opin Hematol 16, 173-8.
39. Morrissey, L.K., Shea, J.O., Kalish, L.A., Weiner, D.L., Branowicki, P., and Heeney, M.M. (2009) Clinical practice guideline improves the treatment of sickle cell disease vasoocclusive pain Pediatr Blood Cancer 52, 369-72.
40. Niscola, P., Sorrentino, F., Scaramucci, L., de Fabritiis, P., and Cianciulli, P. (2009) Pain syndromes in sickle cell disease: An update Pain Med(Malden, Mass 10), 470-80.
41. Pack-Mabien, A., and Haynes, J., Jr. (2009) A primary care provider's guide to preventive and acute care management of adults and children with sickle cell disease J Am Acad Nurse Practition 21, 250-7.
42. Richard, R.E. (2009) The management of sickle cell pain Curr Pain Headache Rep 13, 295-7.
43. Meremikwu, M.M. (2009) Sickle cell disease Clin Evid (Online) 2009, 2402.
44. Chaplin, H., Jr., Monroe, M.C., Malecek, A.C., Morgan, L.K., Michael, J., and Murphy, W.A. (1989) Preliminary trial of minidose heparin prophylaxis for painful sickle cell crises East Afr Med J 66, 574-84.
45. Embury, S.H., Matsui, N.M., Ramanujam, S., Mayadas, T.N., Noguchi, C.T., Diwan, B.A., Mohandas, N., and Cheung, A.T. (2004) The contribution of endothelial cell P-selectin to the microvascular flow of mouse sickle erythrocytes in vivo Blood 104, 3378-85.
46. Kirkham, F.J., Lerner, N.B., Noetzel, M., DeBaun, M.R., Datta, A.K., Rees, D.C., and Adams, R.J. (2006) Trials in sickle cell disease Pediatr Neurol 34, 450-8.
47. Matsui, N.M., Varki, A., and Embury, S.H. (2002) Heparin inhibits the flow adhesion of sickle red blood cells to P-selectin Blood 100, 3790-6.
48. Mousa, S.A. (2005) Elevation of plasma von Willebrand factor and tumor necrosis factor-a in obese subjects and their reduction by the low molecular weight heparin tinzaparin Int Angiol 24, 278-81.
49. Mousa, S.A. (2005) Effect of low molecular weight heparin and different heparin molecular weight fractions on the activity of the matrix-degrading enzyme aggrecanase: Structure-function relationship J Cell Biochem 95, 95-8.
50. Mousa, S.A. (2006) Inhibitory effect of C-reactive protein on the release of tissue factor pathway inhibitor from human endothelial cells: Reversal by low molecular weight heparin Int Angiol 25, 10-3.
51. Mousa, S.A., Bozarth, J., and Barrett, J.S. (2003) Pharmacodynamic properties of the low molecular weight heparin, tinzaparin: Effect of molecular weight distribution on plasma tissue factor pathway inhibitor in healthy human subjects J Clin Pharmacol 43, 727-34.
52. Mousa, S.A., and Johansen, K. (2005) Pharmacodynamic effects of low molecular weight heparin in obese subjects following subcutaneous administration of 75 IU/kg on plasma tissue factor pathway inhibitor and nitric oxide Int Angiol 24, 40-2.
53. Stevenson, J.L., Choi, S.H., and Varki, A. (2005) Differential metastasis inhibition by clinically relevant levels of heparins- correlation with selectin inhibition, not antithrombotic activity Clin Cancer Res 11, 7003-11.
54. Mousa, S.A. (2002) The low molecular weight heparin, tinzaparin, in thrombosis and beyond Cardiovasc Drug Rev 20, 199-216.
55. Puskas, A., Balogh, Z., Hadadi, L., Imre, M., Orban, E., Kosa, K., Brassai, Z., and Mousa, S.A. (2007) Spontaneous recanalization in deep venous thrombosis: A prospective duplex ultrasound study Int Angiol 26, 53-63.
56. Bellet, P.S., Kalinyak, K.A., Shukla, R., Gelfand, M.J., and Rucknagel, D.L. (1995) Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases N Engl J Med 333, 699-703.
57. Vichinsky, E.P., Neumayr, L.D., Earles, A.N., Williams, R., Lennette, E.T., Dean, D., Nickerson, B., Orringer, E., McKie, V., Bellevue, R., Daeschner, C., and Manci, E.A. (2000) Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group N Engl J Med 342, 1855-65.
58. Greenberg, J., Ohene-Frempong, K., Halus, J., Way, C., and Schwartz, E. (1983) Trial of low doses of aspirin as prophylaxis in sickle cell disease J Pediatr 102, 781-4.
59. Osamo, N.O., Photiades, D.P., and Famodu, A.A. (1981) Therapeutic effect of aspirin in sickle cell anaemia Acta Haematol 66, 102-7.
60. Quinn, C.T., and Buchanan, G.R. (1999) The acute chest syndrome of sickle cell disease J Pediatr 135, 416-22.
61. Salvaggio, J.E., Arnold, C.A., and Banov, C.H. (1963) Long-term anti-coagulation in sickle-cell disease. A clinical study N Engl J Med 269, 182-6.
62. Wolters, H.J., ten Cate, H., Thomas, L.L., Brandjes, D.P., van der Ende, A., van der Heiden, Y., and Statius van Eps, L.W. (1995) Low-intensity oral anticoagulation in sicklecell disease reverses the prethrombotic state: Promises for treatment? Br J Haematol 90, 715-7.
63. Zago, M.A., Costa, F.F., Ismael, S.J., Tone, L.G., and Bottura, C. (1984) Treatment of sickle cell diseases with aspirin Acta Haematol 72, 61-4.
64. Ballas, S.K. (1990) Treatment of pain in adults with sickle cell disease Am J Hematol 34, 49-54.
65. Anie, K.A., and Green, J. (2002) Psychological therapies for sickle cell disease and pain Cochrane Database Syst Rev CD001916.
66. Bodhise, P.B., Dejoie, M., Brandon, Z., Simpkins, S., and Ballas, S.K. (2004) Non-pharmacologic management of sickle cell pain Hematology 9, 235-7.
67. Thomas, V.J., Gruen, R., and Shu, S. (2001) Cognitive-behavioural therapy for the management of sickle cell disease pain: Identification and assessment of costs Ethn Health 6, 59-67.
68. Serjeant, G.R. (1997) Sickle-cell disease Lancet 350, 725-30.
69. Sun, P.M., Wilburn, W., Raynor, B.D., and Jamieson, D. (2001) Sickle cell disease in pregnancy: Twenty years of experience at Grady Memorial Hospital, Atlanta, Georgia Am J Obstet Gynecol 184, 1127-30.
70. Villers, M.S., Jamison, M.G., De Castro, L.M., and James, A.H. (2008) Morbidity associated with sickle cell disease in pregnancy Am J Obstet Gynecol 199, 125 e1-5.
71. Smith, J.A., Espeland, M., Bellevue, R., Bonds, D., Brown, A.K., and Koshy, M. (1996) Pregnancy in sickle cell disease: Experience of the cooperative study of sickle cell disease Obstet Gynecol 87, 199-204.
72. Rees, D.C., Olujohungbe, A.D., Parker, N.E., Stephens, A.D., Telfer, P., and Wright, J. (2003) Guidelines for the management of the acute painful crisis in sickle cell disease Br J Haematol 120, 744-52.
73. Koshy, M., Burd, L., Wallace, D., Moawad, A., and Baron, J. (1988) Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study N Engl J Med 319, 1447-52.
74. Faron, G., Corbisier, C., Tecco, L., and Vokaer, A. (2001) First sickle cell crisis triggered by induction of labor in a primigravida Eur J Obstet Gynecol Reprod Biol 94, 304-6.
75. Sanson, B.J., Lensing, A.W., Prins, M.H., Ginsberg, J.S., Barkagan, Z.S., Lavenne- Pardonge, E., Brenner, B., Dulitzky, M., Nielsen, J.D., Boda, Z., Turi, S., Mac Gillavry, M.R., Hamulyak, K., Theunissen, I.M., Hunt, B.J., and Buller, H.R. (1999) Safety of low-molecular-weight heparin in pregnancy: A systematic review Thromb Haemost 81, 668-72.
76. Bates, S.M., Greer, I.A., Hirsh, J., and Ginsberg, J.S. (2004) Use of antithrombotic agents during pregnancy: The seventh ACCP conference on antithrombotic and thrombolytic therapy Chest 126, 627-44S.
77. Ginsberg, J.S., Greer, I., and Hirsh, J. (2001) Use of antithrombotic agents during pregnancy Chest 119, 122-31S.
78. Greer, I.A. (2005) Venous thromboembolism and anticoagulant therapy in pregnancy Gend Med 2(Suppl A), S10-7.
79. Villa-Forte Gomes, M.P. (2009) Venous thromboembolism in pregnancy Curr Treatment Opt Cardiovasc Med 11, 104-13.
80. Smith, M.P., Norris, L.A., Steer, P.J., Savidge, G.F., and Bonnar, J. (2004) Tinzaparin sodium for thrombosis treatment and prevention during pregnancy Am J Obstet Gynecol 190, 495-501.
81. Gynaecologists, R.C.o.O.a. Thromboembolic Disease in Pregnancy and the Puerperium: Acute Management. Guideline No. 28 Update. London: RCOG Press; 2007.