Sickle cell anemia

References

Key articles

National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report, 2014. Sep 2014 [internet publication].Full text

Wang WC, Ware RE, Miller ST, et al; BABY HUG investigators. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011 May 14;377(9778):1663-72.Full text  Abstract

Steinberg MH, McCarthy WF, Castro O, et al. The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: a 17.5 year follow-up. Am J Hematol. 2010 Jun;85(6):403-8.Full text  Abstract

Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017 Feb 2;376(5):429-39.Full text  Abstract

Vichinsky E, Hoppe CC, Ataga KI, et al. A phase 3 randomized trial of voxelotor in sickle cell disease. N Engl J Med. 2019 Aug 8;381(6):509-19.Full text  Abstract

Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet. 2013 Mar 16;381(9870):930-8. Abstract

Mantadakis E, Cavender JD, Rogers ZR, et al. Prevalence of priapism in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 1999 Nov-Dec;21(6):518-22. Abstract

Reference articles

1. Pass KA, Lane PA, Fernhoff PM, et al. US newborn screening system guidelines II: follow-up of children, diagnosis, management, and evaluation. Statement of the Council of Regional Networks for Genetic Services (CORN). J Pediatr. 2000 Oct;137(suppl 4):S1-46. Abstract

2. Dunston T, Rowland R, Huntsman RG, et al. Sickle-cell haemoglobin C disease and sickle-cell beta thalassaemia in white South Africans. S Afr Med J. 1972 Sep 30;46(39):1423-6. Abstract

3. Centers for Disease Control and Prevention (CDC). Data and statistics on sickle cell disease. Dec 2020 [internet publication].Full text

4. Lorey FW, Arnopp J, Cunningham GC. Distribution of hemoglobinopathy variants by ethnicity in a multiethnic state. Genet Epidemiol. 1996;13(5):501-12. Abstract

5. Ohene-Frempong K, Nkrumah FK. Sickle cell disease in Africa. In: Embry SH, Hebbel RP, Mohandas N, et al, eds. Sickle cell disease: basic principles and clinical practice. New York, NY: Raven Press Ltd; 1994.

6. Allison A. Abnormal haemoglobin and erythrocyte enzyme-deficiency traits. In: Harrison G, ed. Genetic variations in human populations. New York, NY; 1961.

7. Wethers DL. Sickle cell disease in childhood: part I. Laboratory diagnosis, pathophysiology and health maintenance. Am Fam Physician. 2000 Sep 1;62(5):1013-20, 1027-8.Full text  Abstract

8. Sundd P, Gladwin MT, Novelli EM. Pathophysiology of sickle cell disease. Annu Rev Pathol. 2019 Jan 24;14:263-92.Full text  Abstract

9. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017 Apr 20;376(16):1561-73.Full text  Abstract

10. Hebbel RP, Yamada O, Moldow CF, et al. Abnormal adherence of sickle erythrocytes to cultured vascular endothelium: possible mechanism for microvascular occlusion in sickle cell disease. J Clin Invest. 1980 Jan;65(1):154-60.Full text  Abstract

11. Hebbel RP, Osarogiagbon R, Kaul D. The endothelial biology of sickle cell disease: inflammation and a chronic vasculopathy. Microcirculation. 2004 Mar;11(2):129-51. Abstract

12. Yale SH, Nagib N, Guthrie T. Approach to the vaso-occlusive crisis in adults with sickle cell disease. Am Fam Physician. 2000 Mar 1;61(5):1349-56, 1363-4. Abstract

13. Kato GJ, Onyekwere OC, Gladwin MT. Pulmonary hypertension in sickle cell disease: relevance to children. Pediatr Hematol Oncol. 2007 Apr-May;24(3):159-70.Full text  Abstract

14. Naik RP, Smith-Whitley K, Hassell KL, et al. Clinical outcomes associated with sickle cell trait: a systematic review. Ann Intern Med. 2018 Nov 6;169(9):619-27.Full text  Abstract

15. Dormandy E, Gulliford M, Bryan S, et al. Effectiveness of earlier antenatal screening for sickle cell disease and thalassaemia in primary care: cluster randomised trial. 2010 Oct 5;341:c5132.Full text  Abstract

16. US Preventive Services Task Force. Sickle cell disease (hemoglobinopathies) in newborns: screening - referred topic. Sep 2007 [internet publication].Full text

17. Gill FM, Sleeper LA, Weiner SJ, et al; Cooperative Study of Sickle Cell Disease. Clinical events in the first decade in a cohort of infants with sickle cell disease. Blood. 1995 Jul 15;86(2):776-83.Full text  Abstract

18. Miller ST, Sleeper LA, Pegelow CH, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. 2000 Jan 13;342(2):83-9.Full text  Abstract

19. Gualandro SF, Fonseca GH, Yokomizo IK, et al. Cohort study of adult patients with haemoglobin SC disease: clinical characteristics and predictors of mortality. Br J Haematol. 2015 Nov;171(4):631-7.Full text  Abstract

20. Mukisi-Mukaza M, Elbaz A, Samuel-Leborgne Y, et al. Prevalence, clinical features, and risk factors of osteonecrosis of the femoral head among adults with sickle cell disease. Orthopedics. 2000 Apr;23(4):357-63. Abstract

21. Centers for disease control and prevention (CDC). Hemoglobinopathies: current practices for screening, confirmation and follow-up. Dec 2015 [internet publication].Full text

22. Vosmaer A, Pereira RR, Koenderman JS, et al. Coagulation abnormalities in Legg-Calvé-Perthes disease. J Bone Joint Surg Am. 2010 Jan;92(1):121-8. Abstract

23. Eckman J. Neonatal screening. In: Embry SH, Hebbel RP, Mohandas N, et al, eds. Sickle cell disease: basic principles and clinical practice. New York, NY: Raven Press Ltd.; 1994.

24. DeBaun MR, Jordan LC, King AA, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020 Apr 28;4(8):1554-88.Full text  Abstract

25. Cherry MG, Greenhalgh J, Osipenko L, et al. The clinical effectiveness and cost-effectiveness of primary stroke prevention in children with sickle cell disease: a systematic review and economic evaluation. Health Technol Assess. 2012;16(43):1-129.Full text  Abstract

26. Alexandrov AV, Sloan MA, Tegeler CH, et al; American Society of Neuroimaging Practice Guidelines Committee. Practice standards for transcranial Doppler (TCD) ultrasound. Part II. Clinical indications and expected outcomes. J Neuroimaging. 2012 Jul;22(3):215-24. Abstract

27. Stephens AD, Angastiniotis M, Baysal E, et al; International Council for the Standardisation of Haematology (ICSH). ICSH recommendations for the measurement of haemoglobin F. Int J Lab Hematol. 2012 Feb;34(1):14-20. Abstract

28. Ryan K, Bain BJ, Worthington D, et al. Significant haemoglobinopathies: guidelines for screening and diagnosis. Br J Haematol. 2010 Apr;149(1):35-49.Full text  Abstract

29. Lobitz S, Telfer P, Cela E, et al. Newborn screening for sickle cell disease in Europe: recommendations from a Pan-European Consensus Conference. Br J Haematol. 2018 Nov;183(4):648-60.Full text  Abstract

30. Asnani MR, Quimby KR, Bennett NR, et al. Interventions for patients and caregivers to improve knowledge of sickle cell disease and recognition of its related complications. Cochrane Database Syst Rev. 2016 Oct 6;(10):CD011175.Full text  Abstract

31. National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease: expert panel report, 2014. Sep 2014 [internet publication].Full text

32. Rankine-Mullings AE, Owusu-Ofori S. Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev. 2021 Mar 8;3:CD003427.Full text  Abstract

33. Dekker LH, Fijnvandraat K, Brabin BJ, et al. Micronutrients and sickle cell disease, effects on growth, infection and vaso-occlusive crisis: a systematic review. Pediatr Blood Cancer. 2012 Aug;59(2):211-5. Abstract

34. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease - life expectancy and risk factors for early death. N Engl J Med. 1994 Jun 9;330(23):1639-44.Full text  Abstract

35. Redwood AM, Williams EM, Desai P, et al. Climate and painful crisis of sickle-cell disease in Jamaica. BMJ. 1976 Jan 10;1(6001):66-8.Full text  Abstract

36. Mohan J, Marshall JM, Reid HL, et al. Peripheral vascular response to mild indirect cooling in patients with homozygous sickle cell (SS) disease and the frequency of painful crisis. Clin Sci (Lond). 1998 Feb;94(2):111-20. Abstract

37. Beutler E. Disorders of hemoglobin structure: sickle cell anemia and related abnormalities. In: Lichtman MA, Beutler E, Kaushansky K, et al, eds. Williams hematology. 7th ed. New York, NY: McGraw-Hill; 2006:667-700.

38. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995 May 18;332(20):1317-22.Full text  Abstract

39. Lanzkron S, Strouse JJ, Wilson R, et al. Systematic review: hydroxyurea for the treatment of adults with sickle cell disease. Ann Intern Med. 2008 Jun 17;148(12):939-55.Full text  Abstract

40. Segal JB, Strouse JJ, Beach MC, et al. Hydroxyurea for the treatment of sickle cell disease. Evid Rep Technol Assess (Full Rep). 2008 Mar;(165):1-95. Abstract

41. Hankins JS, Ware RE, Rogers ZR, et al. Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood. 2005 Oct 1;106(7):2269-75.Full text  Abstract

42. Scott JP, Hillery CA, Brown ER, et al. Hydroxyurea therapy in children severely affected with sickle cell disease. J Pediatr. 1996 Jun;128(6):820-8. Abstract

43. Wang WC, Helms RW, Lynn HS, et al. Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS Study. J Pediatr. 2002 Feb;140(2):225-9. Abstract

44. Wang WC, Ware RE, Miller ST, et al; BABY HUG investigators. Hydroxycarbamide in very young children with sickle-cell anaemia: a multicentre, randomised, controlled trial (BABY HUG). Lancet. 2011 May 14;377(9778):1663-72.Full text  Abstract

45. Zimmerman SA, Schultz WH, Davis JS, et al. Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood. 2004 Mar 15;103(6):2039-45.Full text  Abstract

46. Nevitt SJ, Jones AP, Howard J. Hydroxyurea (hydroxycarbamide) for sickle cell disease. Cochrane Database Syst Rev. 2017 Apr 20;(4):CD002202.Full text  Abstract

47. Ware RE, Helms RW; SWiTCH Investigators. Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood. 2012 Apr 26;119(17):3925-32.Full text  Abstract

48. Kinney TR, Helms RW, O'Branski EE, et al. Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group. Blood. 1999 Sep 1;94(5):1550-4.Full text  Abstract

49. Steinberg MH, Barton F, Castro O, 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 Apr 2;289(13):1645-51.Full text  Abstract

50. Voskaridou E, Christoulas D, Bilalis A, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2010 Mar 25;115(12):2354-63.Full text  Abstract

51. Steinberg MH, McCarthy WF, Castro O, et al. The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: a 17.5 year follow-up. Am J Hematol. 2010 Jun;85(6):403-8.Full text  Abstract

52. Cieri-Hutcherson NE, Hutcherson TC, Conway-Habes EE, et al. Systematic review of l-glutamine for Prevention of vaso-occlusive pain crisis in patients with sickle cell disease. Pharmacotherapy. 2019 Nov;39(11):1095-104.Full text  Abstract

53. Niihara Y, Miller ST, Kanter J, et al. A phase 3 trial of L-glutamine in sickle cell disease. N Engl J Med. 2018 Jul 19;379(3):226-35. Abstract

54. Quinn CT. L-glutamine for sickle cell anemia: more questions than answers. Blood. 2018 Aug 16;132(7):689-93. Abstract

55. Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017 Feb 2;376(5):429-39.Full text  Abstract

56. Kutlar A, Kanter J, Liles DK, et al. Effect of crizanlizumab on pain crises in subgroups of patients with sickle cell disease: a SUSTAIN study analysis. Am J Hematol. 2019 Jan;94(1):55-61.Full text  Abstract

57. Han J, Saraf SL, Gordeuk VR. Systematic review of crizanlizumab: a new parenteral option to reduce vaso-occlusive pain crises in patients with sickle cell disease. Pharmacotherapy. 2020 Jun;40(6):535-43.Full text  Abstract

58. Çanak B, Eşkazan AE. Voxelotor: a new kid on the block in the treatment of sickle cell disease. Br J Clin Pharmacol. 14 Feb 2022 [internet publication].Full text  Abstract

59. Vichinsky E, Hoppe CC, Ataga KI, et al. A phase 3 randomized trial of voxelotor in sickle cell disease. N Engl J Med. 2019 Aug 8;381(6):509-19.Full text  Abstract

60. Han J, Saraf SL, Gordeuk VR. Systematic review of voxelotor: a first-in-class sickle hemoglobin polymerization inhibitor for management of sickle cell disease. Pharmacotherapy. 2020 Jun;40(6):525-34.Full text  Abstract

61. U.S. National Library of medicine. Clinical trials. Study to evaluate the effect of GBT440 on TCD in pediatrics with sickle cell disease (HOPE Kids 2). Dec 2020 [internet publication].Full text

62. Vichinsky EP, Haberkern CM, Neumayr L, et al; The Preoperative Transfusion in Sickle Cell Disease Study Group. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. N Engl J Med. 1995 Jul 27;333(4):206-13.Full text  Abstract

63. Estcourt LJ, Kimber C, Trivella M, et al. Preoperative blood transfusions for sickle cell disease. Cochrane Database Syst Rev. 2020 Jul 2;7:CD003149.Full text  Abstract

64. Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet. 2013 Mar 16;381(9870):930-8. Abstract

65. Lee MT, Piomelli S, Granger S, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. 2006 Aug 1;108(3):847-52.Full text  Abstract

66. Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. 2005 Dec 29;353(26):2769-78.Full text  Abstract

67. Compernolle V, Chou ST, Tanael S, et al. Red blood cell specifications for patients with hemoglobinopathies: a systematic review and guideline. Transfusion. 2018 Jun;58(6):1555-66. Abstract

68. Chou ST, Alsawas M, Fasano RM, et al. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020 Jan 28;4(2):327-55.Full text  Abstract

69. Trompeter S, Massey E, Robinson S, et al. Position paper on International Collaboration for Transfusion Medicine (ICTM) Guideline 'Red blood cell specifications for patients with hemoglobinopathies: a systematic review and guideline'. Br J Haematol. 2020 May;189(3):424-27.Full text  Abstract

70. Fasano RM, Meyer EK, Branscomb J, et al. Impact of Red Blood Cell Antigen Matching on Alloimmunization and Transfusion Complications in Patients with Sickle Cell Disease: A Systematic Review. Transfus Med Rev. 2019 Jan;33(1):12-23.Full text  Abstract

71. Oteng-Ntim E, Pavord S, Howard R, et al. Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline. Br J Haematol. 2021 Sep;194(6):980-95.Full text  Abstract

72. Boafor TK, Olayemi E, Galadanci N, et al. Pregnancy outcomes in women with sickle-cell disease in low and high income countries: a systematic review and meta-analysis. BJOG. 2016 Apr;123(5):691-8.Full text  Abstract

73. Malinowski AK, Shehata N, D'Souza R, et al. Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis. Blood. 2015 Nov 19;126(21):2424-35.Full text  Abstract

74. Brandow AM, Carroll CP, Creary S, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv. 2020 Jun 23;4(12):2656-701.Full text  Abstract

75. Jacobson SJ, Kopecky EA, Joshi P, et al. Randomised trial of oral morphine for painful episodes of sickle-cell disease in children. Lancet. 1997 Nov 8;350(9088):1358-61. Abstract

76. Cooper TE, Hambleton IR, Ballas SK, et al. Pharmacological interventions for painful sickle cell vaso-occlusive crises in adults. Cochrane Database Syst Rev. 2019 Nov 14;2019(11):CD012187.Full text  Abstract

77. Aboursheid T, Albaroudi O, Alahdab F. Inhaled nitric oxide for treating pain crises in people with sickle cell disease. Cochrane Database Syst Rev. 2019 Oct 11;10:CD011808.Full text  Abstract

78. Dampier CD, Wager CG, Harrison R, et al; Investigators of the Sickle Cell Disease Clinical Research Network (SCDCRN). Impact of PCA strategies on pain intensity and functional assessment measures in adults with sickle cell disease during hospitalized vaso-occlusive episodes. Am J Hematol. 2012 Oct;87(10):E71-4.Full text  Abstract

79. Vichinsky EP, Neumayr LD, Earles AN, 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 Jun 22;342(25):1855-65.Full text  Abstract

80. Dolatkhah R, Dastgiri S. Blood transfusions for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2020 Jan 16;1:CD007843.Full text  Abstract

81. Bundy DG, Richardson TE, Hall M, et al. Association of guideline-adherent antibiotic treatment with readmission of children with sickle cell disease hospitalized with acute chest syndrome. JAMA Pediatr. 2017 Nov 1;171(11):1090-9.Full text  Abstract

82. Kanter J, Liem RI, Bernaudin F, et al. American Society of Hematology 2021 guidelines for sickle cell disease: stem cell transplantation. Blood Adv. 2021 Sep 28;5(18):3668-89.Full text  Abstract

83. Oringanje C, Nemecek E, Oniyangi O. Hematopoietic stem cell transplantation for people with sickle cell disease. Cochrane Database Syst Rev. 2020 Jul 3;7:CD007001.Full text  Abstract

84. Shenoy S, Angelucci E, Arnold SD, et al. Current results and future research priorities in late effects after hematopoietic stem cell transplantation for children with sickle cell disease and thalassemia: a consensus statement from the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects after Pediatric Hematopoietic Stem Cell Transplantation. Biol Blood Marrow Transplant. 2017 Apr;23(4):552-61.Full text  Abstract

85. Smith WR, Penberthy LT, Bovbjerg VE, et al. Daily assessment of pain in adults with sickle cell disease. Ann Intern Med. 2008 Jan 15;148(2):94-101. Abstract

86. Anie KA, Green J. Psychological therapies for sickle cell disease and pain. Cochrane Database Syst Rev. 2015 May 8;(5):CD001916.Full text  Abstract

87. Webster J, Osborne S, Rickard CM, et al. Clinically-indicated replacement versus routine replacement of peripheral venous catheters. Cochrane Database Syst Rev. 2019 Jan 23;(1):CD007798.Full text  Abstract

88. Martí-Carvajal AJ, Solà I, Agreda-Pérez LH. Treatment for avascular necrosis of bone in people with sickle cell disease. Cochrane Database Syst Rev. 2019 Dec 5;12:CD004344.Full text  Abstract

89. US Food and Drug Administration. FDA drug safety communication: FDA restricts use of prescription codeine pain and cough medicines and tramadol pain medicines in children; recommends against use in breastfeeding women. Apr 2017 [internet publication].Full text

90. Medicines and Healthcare Products Regulatory Agency. Codeine: restricted use as analgesic in children and adolescents after European safety review. Jun 2013 [internet publication].Full text

91. European Medicines Agency. Restrictions on use of codeine for pain relief in children - CMDh endorses PRAC recommendation. Jun 2013 [internet publication].Full text

92. Misra H, Lickliter J, Kazo F, et al. PEGylated carboxyhemoglobin bovine (SANGUINATE): results of a phase I clinical trial. Artif Organs. 2014 Aug;38(8):702-7. Abstract

93. Misra H, Bainbridge J, Berryman J, et al. A Phase Ib open label, randomized, safety study of SANGUINATE™ in patients with sickle cell anemia. Rev Bras Hematol Hemoter. 2017 Jan-Mar;39(1):20-7.Full text  Abstract

94. Valentino J, Misra H, Lopez L, Paulino GM. Use of pegylated-carboxyhemoglobin bovine for the treatment of sickle cell disease associated leg ulcers: results from a phase 2 safety study. Presented at: Haematologica. 22th congress of the european hematology association. Spain, 2017.Full text

95. McArthur JG, Svenstrup N, Chen C, et al. A novel, highly potent and selective phosphodiesterase-9 inhibitor for the treatment of sickle cell disease. Haematologica. 2019 May 30 [Epub ahead of print].Full text  Abstract

96. U.S. National library of medicine. Clinical trials. A study of IMR-687 in subjects with sickle cell disease. Jul 2020 [internet publication].Full text

97. ClinicalTrials.gov. A study evaluating the efficacy and safety of Mitapivat (AG-348) in participants with sickle cell disease. ClinicalTrials.gov Identifier: NCT05031780. Mar 2022 [internet publication].Full text

98. Rab MAE, Van Oirschot BA, Kosinski PA, et al. AG-348 (Mitapivat), an allosteric activator of red blood cell pyruvate kinase, increases enzymatic activity, protein stability, and ATP levels over a broad range of PKLR genotypes. Haematologica. 2021 Jan 1;106(1):238-49.Full text  Abstract

99. Shah FT, Porter JB, Sadasivam N, et al. Guidelines for the monitoring and management of iron overload in patients with haemoglobinopathies and rare anaemias. Br J Haematol. 2022 Jan;196(2):336-50.Full text  Abstract

100. Qureshi A, Kaya B, Pancham S, et al. Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease: a British Society for Haematology Guideline. Br J Haematol. 2018 May;181(4):460-75.Full text  Abstract

101. National Institute for Health and Care Excellence. Sickle cell disease: managing acute painful episodes in hospital. Jun 2012 [internet publication].Full text

102. Liem RI, Lanzkron S, D Coates T, et al. American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease. Blood Adv. 2019 Dec 10;3(23):3867-97.Full text  Abstract

103. Meschia JF, Bushnell C, Boden-Albala B, et al. Guidelines for the primary prevention of stroke: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2014 Dec;45(12):3754-832.Full text  Abstract

104. Elmariah H, Garrett ME, De Castro LM, et al. Factors associated with survival in a contemporary adult sickle cell disease cohort. Am J Hematol. 2014 May;89(5):530-5.Full text  Abstract

105. Grosse SD, Odame I, Atrash HK, et al. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med. 2011 Dec;41(6 suppl 4):S398-405.Full text  Abstract

106. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998 Jan 1;91(1):288-94.Full text  Abstract

107. Stockman JA, Nigro MA, Mishkin MM, et al. Occlusion of large cerebral vessels in sickle-cell anemia. N Engl J Med. 1972 Oct 26;287(17):846-9. Abstract

108. Ware RE, Davis BR, Schultz WH, et al. Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial. Lancet. 2016 Feb 13;387(10019):661-70.Full text  Abstract

109. Miller ST, Jensen D, Rao SP. Less intensive long-term transfusion therapy for sickle cell anemia and cerebrovascular accident. J Pediatr. 1992 Jan;120(1):54-7. Abstract

110. Estcourt LJ, Kohli R, Hopewell S, et al. Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease. Cochrane Database Syst Rev. 2020 Jul 27;7:CD003146.Full text  Abstract

111. Hulbert ML, McKinstry RC, Lacey JL, et al. Silent cerebral infarcts occur despite regular blood transfusion therapy after first strokes in children with sickle cell disease. Blood. 2011 Jan 20;117(3):772-9.Full text  Abstract

112. Estcourt LJ, Kimber C, Hopewell S, et al. Interventions for preventing silent cerebral infarcts in people with sickle cell disease. Cochrane Database Syst Rev. 2020 Apr 6;4:CD012389.Full text  Abstract

113. Fortin PM, Hopewell S, Estcourt LJ. Red blood cell transfusion to treat or prevent complications in sickle cell disease: an overview of Cochrane reviews. Cochrane Database Syst Rev. 2018 Aug 1;(8):CD012082.Full text  Abstract

114. Vichinsky EP, Neumayr LD, Gold JI, et al; Neuropsychological Dysfunction and Neuroimaging Adult Sickle Cell Anemia Study Group. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010 May 12;303(18):1823-31. Abstract

115. Krauss JS, Freant LJ, Lee JR. Gastrointestinal pathology in sickle cell disease. Ann Clin Lab Sci. 1998 Jan-Feb;28(1):19-23. Abstract

116. Omata M, Johnson CS, Tong M, et al. Pathological spectrum of liver diseases in sickle cell disease. Dig Dis Sci. 1986 Mar;31(3):247-56. Abstract

117. Schubert TT. Hepatobiliary system in sickle cell disease. Gastroenterology. 1986 Jun;90(6):2013-21. Abstract

118. Seguier-Lipszyc E, de Lagausie P, Benkerrou M, et al. Elective laparoscopic cholecystectomy. Surg Endosc. 2001 Mar;15(3):301-4. Abstract

119. Bonatsos G, Birbas K, Toutouzas K, et al. Laparoscopic cholecystectomy in adults with sickle cell disease. Surg Endosc. 2001 Aug;15(8):816-9. Abstract

120. Owusu-Ofori S, Remmington T. Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database Syst Rev. 2017 Nov 7;(11):CD003425.Full text  Abstract

121. Oligbu G, Fallaha M, Pay L, et al. Risk of invasive pneumococcal disease in children with sickle cell disease in the era of conjugate vaccines: a systematic review of the literature. Br J Haematol. 2019 May;185(4):743-51.Full text  Abstract

122. Claster S, Vichinsky EP. Managing sickle cell disease. BMJ. 2003 Nov 15;327(7424):1151-5.Full text  Abstract

123. Leitão Guerra RL, Leitão Guerra CL, Bastos MG, et al. Sickle cell retinopathy: What we now understand using optical coherence tomography angiography. A systematic review. Blood Rev. 2019 May;35:32-42. Abstract

124. Rodgers GP, Roy MS, Noguchi CT, et al. Is there a role for selective vasodilation in the management of sickle cell disease? Blood. 1988 Mar;71(3):597-602.Full text  Abstract

125. Myint KT, Sahoo S, Thein AW, et al. Laser therapy for retinopathy in sickle cell disease. Cochrane Database Syst Rev. 2015 Oct 9;(10):CD010790.Full text  Abstract

126. Morgan AG. Sickle cell leg ulcers. Int J Dermatol. 1985 Dec;24(10):643-4. Abstract

127. Castro O, Gladwin MT. Pulmonary hypertension in sickle cell disease: mechanisms, diagnosis, and management. Hematol Oncol Clin North Am. 2005 Oct;19(5):881-96. Abstract

128. Klings ES, Machado RF, Barst RJ, et al.; American Thoracic Society Ad Hoc Committee on Pulmonary Hypertension of Sickle Cell Disease. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014 Mar 15;189(6):727-40. Abstract

129. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996 Feb 1;334(5):296-301.Full text  Abstract

130. McLaughlin VV, Genthner DE, Panella MM, et al. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med. 1999 May 4;130(9):740-3. Abstract

131. Machado RF, Martyr S, Kato GJ, et al. Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension. Br J Haematol. 2005 Aug;130(3):445-53.Full text  Abstract

132. Olaosebikan R, Ernest K, Bojang K, et al. A randomized trial to compare the safety, tolerability, and effectiveness of 3 antimalarial regimens for the prevention of malaria in Nigerian patients with sickle cell disease. J Infect Dis. 2015 Aug 15;212(4):617-25.Full text  Abstract

133. Mantadakis E, Cavender JD, Rogers ZR, et al. Prevalence of priapism in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol. 1999 Nov-Dec;21(6):518-22. Abstract

134. Arduini GAO, Trovó de Marqui AB. Prevalence and characteristics of priapism in sickle cell disease. Hemoglobin. 2018 Mar;42(2):73-7. Abstract

135. Chinegwundoh FI, Smith S, Anie KA. Treatments for priapism in boys and men with sickle cell disease. Cochrane Database Syst Rev. 2020 Apr 6;4:CD004198.Full text  Abstract

136. Phebus CK, Gloninger MF, Maciak BJ. Growth patterns by age and sex in children with sickle cell disease. J Pediatr. 1984 Jul;105(1):28-33. Abstract

137. Fung EB, Harmatz PR, Milet M, et al. Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions. Transfusion. 2008 Sep;48(9):1971-80. Abstract

138. Kwiatkowski JL, Hamdy M, El-Beshlawy A, et al. Deferiprone vs deferoxamine for transfusional iron overload in SCD and other anemias: a randomized, open-label noninferiority study. Blood Adv. 2022 Feb 22;6(4):1243-54.Full text  Abstract

139. Meerpohl JJ, Schell LK, Rücker G, et al. Deferasirox for managing transfusional iron overload in people with sickle cell disease. Cochrane Database Syst Rev. 2014 May 27;(5):CD007477.Full text  Abstract

140. Sridharan K, Sivaramakrishnan G. Efficacy and safety of iron chelators in thalassemia and sickle cell disease: a multiple treatment comparison network meta-analysis and trial sequential analysis. Expert Rev Clin Pharmacol. 2018 Jun;11(6):641-50. Abstract

141. Nelson DA, Deuster PA, Carter R 3rd, et al. Sickle Cell Trait, Rhabdomyolysis, and Mortality among U.S. Army Soldiers. N Engl J Med. 2016 Aug 4;375(5):435-42.Full text  Abstract

142. DeBaun MR, Jordan LC, King AA, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020 Apr 28;4(8):1554-1588.Full text  Abstract

143. World Health Organization. Medical eligibility for contraceptive use. 5th ed. Geneva: World Health Organization; 2015.Full text

144. Lottenberg R, Hassell KL. An evidence-based approach to the treatment of adults with sickle cell disease. Hematology Am Soc Hematol Educ Program. 2005:58-65.Full text  Abstract

145. Haddad LB, Curtis KM, Legardy-Williams JK, et al. Contraception for individuals with sickle cell disease: a systematic review of the literature. Contraception. 2012 Jun;85(6):527-37. Abstract

146. Rankine-Mullings AE, Owusu-Ofori S. Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev. 2021 Mar 8;3:CD003427.Full text  Abstract

Use of this content is subject to our disclaimer