Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease

Cochrane Database of Systematic Reviews

Volumn 2017, Issue 1, 2017, Pages

  Estcourt, Lise J ^a   Fortin, Patricia M ^b   Hopewell, Sally ^c   Trivella, Marialena ^c   Wang, Winfred C ^d  

^a NHS BLOOD AND TRANSPLANT   (United Kingdom)

^b JOHN RADCLIFFE HOSPITAL   (United Kingdom)

^c UNIVERSITY OF OXFORD   (United Kingdom)

^d ST JUDE CHILDREN S RESEARCH HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

HEMOGLOBIN S; HYDROXYUREA; ANTISICKLING AGENT; IRON CHELATING AGENT;

ACUTE CHEST SYNDROME; ALLOIMMUNIZATION; BIBLIOGRAPHIC DATABASE; BLOOD DONOR; BLOOD TRANSFUSION REACTION; CEREBROVASCULAR ACCIDENT; COMPARATIVE STUDY; COMPUTER ASSISTED TOMOGRAPHY; DISEASE REGISTRY; ERYTHROCYTE TRANSFUSION; HEMOGLOBIN BLOOD LEVEL; HUMAN; IMMUNOCOMPETENCE; INCIDENCE; IRON OVERLOAD; LONG TERM CARE; NATIONAL HEALTH ORGANIZATION; NUCLEAR MAGNETIC RESONANCE IMAGING; OUTCOME ASSESSMENT; PHLEBOTOMY; PRIMARY PREVENTION; PRIMARY STROKE; PRIORITY JOURNAL; QUALITY OF LIFE; REVIEW; RISK FACTOR; RISK REDUCTION; SECONDARY PREVENTION; SECONDARY STROKE; SICKLE CELL ANEMIA; SYSTEMATIC REVIEW; TRANSIENT ISCHEMIC ATTACK; TREATMENT DURATION; ADOLESCENT; ADVERSE EFFECTS; BLOOD; BLOOD TRANSFUSION; CHILD; COMPLICATION; EARLY TERMINATION OF CLINICAL TRIAL; META ANALYSIS; PRESCHOOL CHILD; STROKE; YOUNG ADULT;

ADOLESCENT; ANEMIA, SICKLE CELL; ANTISICKLING AGENTS; BLOOD TRANSFUSION; CHILD; CHILD, PRESCHOOL; EARLY TERMINATION OF CLINICAL TRIALS; ERYTHROCYTE TRANSFUSION; HEMOGLOBIN, SICKLE; HUMANS; HYDROXYUREA; IRON CHELATING AGENTS; PHLEBOTOMY; PRIMARY PREVENTION; SECONDARY PREVENTION; STROKE; YOUNG ADULT;

EID: 85009813220     PISSN: 14651858     EISSN: None     Source Type: Journal    
DOI: 10.1002/14651858.CD003146.pub3     Document Type: Review

References (162)

1. Bean CJ, Boulet SL, Ellingsen D, Pyle ME, Barron-Casella EA, Casella JF, et al. Heme oxygenase-1 gene promoter polymorphism is associated with reduced incidence of acute chest syndrome among children with sickle cell disease. Blood 2012;120(18):3822-8. [CENTRAL: 899251; CRS: 5500050000000004; 2987038; EMBASE: 2012654658]
2. Beverung LM, Strouse JJ, Hulbert ML, Neville K, Liem RI, Inusa B, et al. Health-related quality of life in children with sickle cellanemia: Impact of blood transfusion therapy. American Journal of Hematology 2015;90:139-43. [2987039]
3. Bhatnagar P, Purvis S, Barron-Casella E, DeBaun MR, Casella JF, Arking DE, et al. Genome-wide association study identifies genetic variants influencing F-cell levels in sickle cell patients. Journal of Human Genetics 2011;56(4):316-23. [2987040]
4. Bhatnagar P, Purvis S, Barron-Casella E, DeBaun MR, Casella JF, Arking DE, et al. Genome-wide association study identifies genetic variants influencing F-cell levels in sickle-cell patients. Journal of Human Genetics 2011;56(4):316-23. Supplementary information. www.nature.com/jhg/journal/v56/n4/suppinfo/jhg201112s1.html?url=/jhg/journal/v56/n4/full/jhg201112a.html. [2987041]
5. Casella JF, King AA, Barton B, White DA, Noetzel MJ, Ichord RN, et al. Design of the silent cerebral infarct transfusion (SIT) trial. Pediatric Hematology and Oncology 2010;27(2):69-89. [2987042]
6. DeBaun M. Epidemiology and treatment of silent strokes in sickle cell anemia. 28th Annual Meeting of the National Sickle Cell Disease Program; 2005 Apr 9-13; Cincinnati, Ohio. 2005:Plenary. [2987043]
7. DeBaun MR, Gordon M, McKinstry RC, Noetzel MJ, White DA, Sarnaik SA, et al. Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. New England Journal of Medicine 2014;371:399-10. [2987044; NCT00072761: Silent Cerebral Infarct Transfusion Multi-Center Clinical Trial (SIT)]
8. DeBaun MR, Sarnaik SA, Rodeghier MJ, Minniti CP, Howard TH, Iyer RV, et al. Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure. Blood 2012;119(16):3684-90. [4680998]
9. ISRCTN52713285 Silent cerebral infarct multi-center clinical trial. www.isrctn.com/ISRCTN52713285 (accessed 1 April 2016). [4680999]
10. Jordan LC, McKinstry RC 3rd, Kraut MA, Ball WS, Vendt BA, Casella JF, et al. Incidental findings on brain magnetic resonance imaging of children with sickle cell disease. Pediatrics 2010;126(1):53-61. [2987045]
11. NCT00072761 Silent cerebral infarct transfusion multi-center clinical trial (SIT). clinicaltrials.gov/ct2/show/NCT00072761 (accessed 1 April 2016). [4681000]
12. Quinn CT, McKinstry RC, Dowling MM, Ball WS, Kraut MA, Casella JF, et al. Acute silent cerebral ischemia occurs more frequently than silent cerebral infarction in children with sickle cell anemia. Blood 2010;116(21):268. [4681001]
13. Vendt BA, McKinstry RC, Ball WS, Kraut MA, Prior FW, Barton B, et al. Silent Cerebral Infarct Transfusion (SIT) trial imaging core: application of novel imaging information technology for rapid and central review of MRI of the brain. Journal of Digital Imaging 2009;22(3):326-43. [2987046]
14. Abboud M, Cure J, Gallagher D, Berman B, Hsu L, Wang W, et al. Magnetic resonance angiography (MRA) in children with abnormal transcranial doppler (TCD) velocities in the STOP study. Blood 1999;94(10 Suppl 1):645a. [2987048]
15. Abboud MR, Cure J, Gallagher D, Berman B, Hsu L, Wang W, et al. Magnetic resonance angiography in children with abnormal TCD in the STOP study. 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:49. [2987049]
16. Abboud MR, Cure J, Granger S, Gallagher D, Hsu L, Wang W, et al. Magnetic resonance angiography in children with sickle cell disease and abnormal transcranial Doppler ultrasonography findings enrolled in the STOP study. Blood 2004;103(7):2822-6. [2987050]
17. Adamkiewicz T, Abboud M, Barredo J, Cavalier ME, Peterson J, Rackoff B, et al. Serum Ferritin in children with SCD on chronic transfusion: correlation with serum alanine aminotransferase and liver iron (STOP/STOP II liver iron ancillary study). 35th Annivesary Convention of the National Sickle Celll Disease Program and the Sickle Cell Disease Association of America; 2007 Sep 17-22; Washington DC, USA. 2007:316. [2987052]
18. Adamkiewicz T, Abboud MR, Barredo JC, Kirby-Allen M, Alvarez OA, Casella JF, et al. Serum ferritin in children with sickle cell disease on chronic transfusion: measure of iron overload or end organ injury? STOP/ STOP II liver iron ancillary study. Blood. 2006; Vol. 108, issue 11. [Abstract no: 791; 2987053]
19. Adamkiewicz TV, Abboud MR, Paley C, Olivieri N, Kirby-Allen M, Vichinsky E, et al. Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood 2009;114(21):4632-8. [2987054]
20. Adams RJ. Lessons from the stroke prevention trial in sickle cell anemia (STOP) study. Journal of Child Neurology 2000;15(5):344-9. [2987069; NCT00000592: Stroke Prevention in Sickle Cell Anemia (STOP 1)]
21. Adams RJ, Brambilla DJ. Stroke prevention trial in sickle cell anemia: STOP. 21st Annual Meeting of the National Sickle Cell Disease Program. 1996:19. [2987064]
22. Adams RJ, Brambilla DJ, Granger S, Gallagher D, Vichinsky E, Abboud MR, et al. Stroke and conversion to high risk in children screened with transcranial Doppler ultrasound during the STOP study. Blood 2004;103(10):3689-94. [2987055]
23. Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Risk of stroke in children with sickle cell disease and abnormal transcranial doppler ultrasound (TCD). Blood 1999;94(10 Suppl 1):419a. [2987056]
24. Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): Baseline characteristics of trial patients. 22nd Annual Meeting of the National Sickle Cell Disease Program. 1997:41. [2987057]
25. Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): final results. 25th Annual Meeting of the National Sickle Cell Disease Program. 2001. [Abstract no: #8; 2987058]
26. Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): final results. Blood 2000;96(11 Pt 1):10a. [2987059]
27. Adams RJ, Brambilla DJ, McKie V, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): follow-up after closure of the trial. Blood 1998;92(10 Suppl 1):527a. [2987060]
28. Adams RJ, Brambilla DJ, McKie VC, Files B, Vichinsky E, Abboud M, et al. Stroke prevention in sickle cell disease (STOP): baseline characteristics of trial patients. Blood 1997;90(10 Suppl 1):123a. [2987061]
29. Adams RJ, Brambilla DJ, Vichinsky E, Abboud M, Pegelow C, Carl EM, et al. Risk of stroke and conversion to abnormal TCD in children screened with transcranial doppler (TCD) during the STOP study. National Sickle Cell Disease Program 30th Annual Meeting; 2002 Sept 17-21. 2002:3. [2987062]
30. Adams RJ, Brambilla DJ, Vichinsky E, Abboud M, Pegelow C, Carl EM, et al. Stroke prevention trial in sickle cell anemia (STOP study): Risk of stroke in 1933 children screened with transcranial doppler (TCD). 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:54. [2987063]
31. Adams RJ, Carl EM, McKie VC, Odo NA, Kutlar A, Phillips M, et al. A pilot trial of hydroxyurea to prevent strokes in children with sickle cell anemia. 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:53. [2987065]
32. Adams RJ, McKie V, Files B, Vichinsky E, Abboud M, Hsu L, et al. Stroke prevention in sickle cell disease (STOP): Results of transcranial doppler ultrasound screening stroke risk. Stroke 1998;29(1):308. [2987066]
33. Adams RJ, McKie VC, Brambilla DJ, Carl E, Gallagher D, Nichols FT, et al. Stroke prevention trial in sickle cell anemia. Controlled Clinical Trials 1998;19(1):110-29. [2987067]
34. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography. New England Journal of Medicine 1998;339(1):5-11. [2987068; NCT00000592: Stroke Prevention in Sickle Cell Anemia (STOP 1)]
35. Anonymous Transfusion to prevent first stroke in children with sickle cell anemia. Journal of the American Ostoepathic Association 1998;98(7):363-4. [2987070]
36. Bulas DI, Jones A, Seibert JJ, Driscoll C, O'Donnell R, Adams RJ. Transcranial Doppler (TCD) screening for stroke prevention in sickle cell anemia: pitfalls in technique variation. Pediatric Radiology 2000;30(11):733-8. [2987071]
37. Cohen AR. Sickle cell disease - new treatments, new questions. New England Journal of Medicine 1998;339(1):42-4. [2987072]
38. Duncan GW. Ischemic stroke in sickle cell disease: a review. Tennessee Medicine 1997;90(12):498-9. [2987073]
39. Files B, Brambilla D, Kutlar A, Miller S, Pegelow C, Vichinsky E, et al. A randomized trial of chronic transfusion to prevent stroke in sickle cell anemia: changes in ferritin with chronic transfusion. Blood 1998;92(10 Suppl 1):528a. [2987074]
40. Files B, Brambilla D, Kutlar A, Miller S, Vichinsky E, Wang W, et al. Longitudinal changes in ferritin during chronic transfusion: a report from the Stroke Prevention Trial in Sickle Cell Anemia (STOP). Journal of Pediatric Hematology/Oncology 2002;24(4):284-90. [2987075]
41. Gates A, Rogers MA, Puczynski M. Stroke prevention trial in sickle cell anemia: comments on effects of chronic transfusion on pain. Journal of Pediatrics 2002;141(5):742-3. [2987076]
42. Hsu LL, Miller ST, Wright E, Kutlar A, McKie V, Wang W, et al. Alpha thalassemia is associated with decreased risk of abnormal transcranial doppler ultrasonography in children with sickle cell anemia. Journal of Pediatric Hematology/Oncology 2003;25(8):622-8. [2987077]
43. Hyacinth HI, Gee BE, Voeks JH, Adams RJ, Hibbert J. High frequency of RBC transfusions in the STOP study was associated with reduction in serum biomarkers of neurodegeneration, vascular remodeling and inflammation. Blood. 2012; Vol. 120, issue 21. [Abstract no: 244; CENTRAL: 999896; CRS: 5500127000000021; 2987078]
44. Kutlar A, Brambilla D, Clair B, Haghighat A, Bakanay S, Adams G, et al. Candidate gene polymorphisms and their association with TCD velocities in children with sickle cell disease. Blood. 2007; Vol. 110, issue 11. [Abstract no: 429; 2987079]
45. Kutlar A, Harbin J, Jackson B, Holley L, Gallagher D, Clair B, et al. Laboratory parameters in patients randomized in the STOP study and their modification by transfusion. Blood 2000;96(11 Pt 2):18b-9b. [2987080]
46. Kutlar A, Harbin J, Jackson BB, Holley LGD, Clair B, Brambilla D, et al. Baseline laboratory parameters in patients randomized to the STOP study and their modification by transfusion. 24th Annual Meeting of the National Sickle Cell Disease Program. 2000:82a. [2987081]
47. Kwiatkowski JL, Brambilla DJ, Granger S, Adams RJ. Elevated blood flow velocity in the anterior cerebral artery and stroke risk in sickle cell disease. Blood. 2003; Vol. 102, issue 11. [Abstract no: 408; 2987082]
48. Kwiatkowski JL, Granger S, Brambilla DJ, Brown RC, Miller ST, Adams RJ. Elevated blood flow velocity in the anterior cerebral artery and stroke risk in sickle cell disease: extended analysis from the STOP trial. British Journal of Haematology 2006;134(3):333-9. [2987083]
49. Kwiatkowski JL, Morales K, Brambilla DJ, Files B, Adamkiewicz T, Adams RJ. Long-term follow-up of transcranial doppler ultrasonography in children with sickle cell disease: Results of the STOP and STOP II patient cohorts. Blood 2002;100(11 Pt 1):663a. [2987084]
50. Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood 2006;108(3):847-52. [2987085]
51. Lee SB, Ramsingh D, Kutlar A, Holley L, McVie VC, Adams RJ. C-reactive protein in children with sickle cell disease at risk for stroke in the STOP study. Stroke 2002;33(1):373. [2987086]
52. Lezcano NE, Odo N, Kutlar A, Brambilla D, Adams RJ. Regular transfusion lowers plasma free hemoglobin in children with sickle-cell disease at risk for stroke. Stroke 2006;37(6):1424-6. [2987087]
53. Miller ST, Wright E, Abboud M, Berman B, Files B, Scher C, et al. Impact of chronic transfusion on non-neurological events during the Stroke Prevention Trial in sickle cell anemia (STOP). Pediatric Research 2000;47(4 Pt 2):251A. [2987088]
54. Miller ST, Wright E, Abboud M, Berman B, Files B, Scher C, et al. Impact of chronic transfusion on non-neurological events during the stroke prevention trial in sickle cell anemia (TOP). 24th Annual Meeting of the National Sickle Cell Disease Program. 2000:61a. [2987089]
55. Miller ST, Wright E, Abboud M, Berman B, Files B, Scher CD, et al. Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. Journal of Pediatrics 2001;139(6):785-9. [2987090]
56. Nichols FT, Jones AM, Adams RJ. Stroke prevention in sickle cell disease (STOP) study guidelines for transcranial Doppler testing. Journal of Neuroimaging 2001;11(4):354-62. [2987091]
57. Olivieri N, Brambilla D, McKie V, Piomelli S, Kutlar A, Files B, et al. Changes in cerebral blood flow velocities during chronic transfusion therapy to prevent stroke in sickle cell disease. Blood 2000;96(11 Pt 1):486a. [2987092]
58. Pegelow CH, Adams R, Hsu L, McKie V, Wang W, Zimmerman R, et al. Children with silent infarct and elevated transcranial doppler ultrasonography velocity are at increased risk of subsequent infarctive events. 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:137. [2987093]
59. Pegelow CH, Wang W, Granger S, Hsu LL, Vichinsky E, Moser FG, et al. Silent infarcts in children with sickle cell anemia and abnormal cerebral artery velocity. Archives of Neurology 2001;58(12):2017-21. [2987094]
60. Sayer G, Bowman L, Clair B, Cail A, Blanchard B, Natrajan K, et al. Long term outcome of patients enrolled into STOP and STOP II trials: a single center experience. Blood. 2012; Vol. 120, issue 21. [Abstract no: 3219; CENTRAL: 999895; CRS: 5500127000000020; 2987095]
61. Styles L, De Jong K, Vichinsky E, Lubin B, Adams R, Kuypers F. Increased RBC phosphatidylserine exposure in sickle cell disease patients at risk for stroke by transcranial Doppler screening. Blood 1997;90(10 Suppl 1):604a-5a. [2987096]
62. Vichinsky E, Luban E, Wright E, Olivieri N, Driscoll C, Pegelow C, et al. Prospective red cell phenotype matching in STOP - a multi-centre transfusion trial. 23rd Annual Meeting of the National Sickle Cell Disease Program. 1999:163. [2987097]
63. Vichinsky E, Luban N, Wright E, Olivieri N, Driscoll C, Pegelow C, et al. Prospective red cell phenotype matching in STOP - a multi-center transfusion trial. Blood 1998;92(10 Suppl 1):528a. [2987098]
64. Vichinsky EP, Luban NL, Wright E, Olivieri N, Driscoll C, Pegelow CH, et al. Prospective RBC phenotype matching in a stroke-prevention trial in sickle cell anemia: a multicenter transfusion trial. Transfusion 2001;41(9):1086-92. [2987099]
65. Wang W, Morales K, Olivieri N, Styles L, Scher C, Adams R, et al. Effect of chronic transfusion on growth in children with sickle cell anemia: results of the STOP trial. National Sickle Cell Disease Program 30th Annual Meeting Conference; 2002 Sept 17-21. 2002:100. [2987100]
66. Wang WC, Morales KH, Scher CD, Styles L, Olivieri N, Adams R, et al. Effect of long-term transfusion on growth in children with sickle cell anemia: results of the STOP trial. Journal of Pediatrics 2005;147(2):244-7. [2987101]
67. ASH News Daily 2004. NHLBI stops the STOP II trial early. www.hematology.org/publications/newsdaily/2004/issue_3/NHLBI.cfm (accessed 22 November 2005). [2987103]
68. Abboud MR, Yim E, Adams RJ. The progression and development of silent infarcts in children with sickle cell anemia is prevented by chronic transfusions and is unrelated to level of hemolysis. Blood. 2008; Vol. 12. [Abstract no: 712; 2987104]
69. Abboud MR, Yim E, Musallam KM, Adams, RJ Discontinuing prophylactic transfusions increases the risk of silent brain infarction in children with sickle cell disease: data from STOP II. Blood 2011;118(4):894-98. [2987105]
70. Adamkiewicz T. Transcranial doppler measures in patients with sickle cell disease at high risk for stroke and receiving hydroxyurea: the HyRetro ancillary study. 52nd ASH Annual Meeting and Exposition; 2010 Dec 4-7; Orlando, Florida. 2010. [Abstract no: 1620; 2987108]
71. Adamkiewicz T, Abboud M, Barredo J, Cavalier ME, Peterson J, Rackoff B, et al. Serum Ferritin in children with SCD on chronic transfusion: correlation with serum alanine aminotransferase and liver iron (STOP/STOP II liver iron ancillary study). 35th Anniversary Convention of the National Sickle Celll Disesae Program and the Sickle Cell Disease Association of America; 2007 Sep 17-22; Washington DC, USA. 2007:316. [2987106]
72. Adamkiewicz T, Abboud MR, Barredo JC, Kirby-Allen M, Alvarez OA, Casella JF, et al. Serum ferritin in children with sickle cell disease on chronic transfusion: measure of iron overload or end organ injury? STOP/ STOP II liver iron ancillary study. Blood. 2006; Vol. 108, issue 11. [Abstract no: 791; 2987107]
73. Adamkiewicz TV, Abboud MR, Paley C, Olivieri N, Kirby-Allen M, Vichinsky E, et al. Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood 2009;114(21):4632-8. [2987109]
74. Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. New England Journal of Medicine 2005;353(26):2769-78. [2987111; NCT00006182: Stroke Prevention in Sickle Cell Anemia (STOP 2)]
75. Adams RJ, Brambilla D, Miller ST. Optimizing primary stroke prevention in children with sickle cell. 28th Annual Meeting of the National Sickle Cell Disease Program; 2005 April 9-13; Cincinnati, Ohio. 2005:3. [2987110]
76. Adams RJ, Brambilla DJ, STOP II investigators. The study design of optimizing primary stroke prevention in children with sickle cell anemia. 27th Annual Meeting of the National Sickle Cell Disease Program; 2004 April 18-21; Los Angeles, California. 2004:174. [2987112]
77. Alvarez O, Miller S, Berman B, Brown C, Casella J, Coates T, et al. Evaluation of chronic transfusion practices in children with sickle cell disease: a survey of STOP II investigators. Blood 2004;104(11):Abstract no: 3732. [Abstract no: 3732; 2987113]
78. Brambilla DJ, Adams RJ. Stroke prevention in sickle cell anemia (STOP 2). National Institutes of Health, Clinical Trials Database Started in 06/2000. [2987114]
79. Brown C, Miller S, Kwiatkowski J, Brambilla D, Adams R. Optimizing primary stroke prevention in sickle cell anemia (STOP 2): an argument for prolonged transfusion?. 29th Annual Meeting of the National Sickle Cell Disease Program; 2006 April 8-12; Memphis, USA. 2006:78. [2987115]
80. Kutlar A, Brambilla D, Clair B, Haghighat A, Bakanay S, Adams G, et al. Candidate gene polymorphisms and their association with TCD velocities in children with sickle cell disease. Blood 2007;110(11):Abstract no: 429. [Abstract no: 429; 2987116]
81. Kwiatkowski JL, Morales K, Brambilla DJ, Files B, Adamkiewicz T, Adams RJ, et al. Long-term follow-up of transcranial doppler ultrasonography in children with sickle cell disease: results of the STOP and STOP II patient cohorts. Blood 2002;100(11 Pt 1):663a. [2987117]
82. Medical College of Georgia. STOP II Questions and Answers. www.mcg.edu/neurology/Research/sicklecell/STOPIIQandA.htm (accessed 22 November 2005). [2987118]
83. Medical News Today. NHLBI stops sickle cell anemia transfusion study. www.medicalnewstoday.com/printerfriendlynews.php?newsid=17363 (accessed 22 November 2005). [2987119]
84. Sayer G, Bowman L, Clair B, Cail A, Blanchard B, Natrajan K, et al. Long term outcome of patients enrolled into STOP and STOP II trials: a single center experience. Blood. 2012; Vol. 120, issue 21. [Abstract no: 3219; CENTRAL: 999895; CRS: 5500127000000020; 2987120]
85. Alvarez O, Yovetich NA, Scott JP, Owen W, Miller ST, Schultz W, et al. Pain and other non-neurological adverse events in children with sickle cell anemia and previous stroke who received hydroxyurea and phlebotomy or chronic transfusions and chelation: results from the SWiTCH clinical trial. American Journal of Hematology 2013;88(11):932-8. [CENTRAL: 963136; CRS: 5500125000000520; 2987122; PUBMED: 23861242]
86. Aygun B, Mortier NA, Kesler K, Schultz WH, Alvarez OA, Rogers ZR, et al. Therapeutice phlebotomy in children with sickle cell anemia, stroke, and iron overload: the SWiTCH experience. 53rd ASH Annual Meeting and Exposition; 2011 Dec 10-13; San Diego, California. 2011. [Abstract no: 1044; 2987123]
87. Helton KJ, Adam RJ, Kesler KL, Lockhart A, Aygun B, Driscoll C, et al. Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial. Blood 2014;124(6):891-898. [4681002]
88. Kwiatkowski JL, Cohen AR, Garro J, Alvarez O, Nagasubramanian R, Sarnaik S, et al. Transfusional iron overload in children with sickle cell anemia on chronic transfusion therapy for secondary stroke prevention. American Journal of Hematology 2012;87(2):221-3. [CENTRAL: 864015; CRS: 5500100000011226; 2987124; PUBMED: 22120913]
89. NCT00122980 Stroke with transfusions changing to hydroxyurea (SWiTCH). www.clinicaltrials.gov/show/NCT00122980 (accessed 16th August 2011). [2987125]
90. Sheehan VA, Howard TA, Sabo A, Nagasaswamy U, Crosby JR, Davis B, et al. Genetic predictors of hemoglobin F response to hydroxyurea in sickle cell anemia. Blood. 2012; Vol. 120, issue 21. [Abstract no: 241; CENTRAL: 977454; CRS: 5500125000000531; 2987126]
91. Ware RE, Helms RW. Stroke with transfusions changing to hydroxyurea (SWiTCH): a phase 3 randomised clinical trial for treatment of children with sickle cell anemia. 52nd ASH Meeting and Exposition; 2010 Dec 4-7; Orlando, Florida. 2010. [Abstract no: 844; 2987128]
92. Ware RE, Helms RW, SWiTCH Investigators. Stroke with transfusions changing to hydroxyurea (SWiTCH). Blood 2012;119(17):3925-32. [2987127]
93. Ware RE, McMurray MA, Schultz WH, Alvarez OA, Aygun B, Cavalier ME, et al. Academic community standards for chronic transfusion therapy in children with sickle cell anemia and stroke. Blood. 2006; Vol. 108, issue 11. [Abstract no: 1213; 2987129]
94. Ware RE, Schultz WH, Yovetich N, Mortier NA, Alvarez O, Hilliard L, et al. Stroke with transfusions changing to hydroxyurea (SWiTCH): A phase III randomized clinical trial for treatment of children with sickle cell anemia, stroke, and iron overload. Pediatric Blood & Cancer 2011;57(6):1011-7. [2987130]
95. Aygun B, Wruck LM, Schultz WH, Mueller BU, Brown C, Luchtman-Jones L, et al. Chronic transfusion practices for prevention of primary stroke in children with sickle cell anemia and abnormal TCD velocities. American Journal of Hematology 2012;87(4):428-30. [CENTRAL: 1139772; CRS: 5500135000001510; 4681003; PUBMED: 22231377]
96. Helton KJ, Roberts D, Schultz WH, Davis BR, Kalfa TA, Pressel SL, et al. Effects of chronic transfusion therapy on MRI and MRA in children with sickle cell anemia at risk for primary stroke: baseline imaging from the Twitch Trial. Blood 2014;124(21):4052. [4681004]
97. Imran H, Aygun B, Davis BR, Pressel SL, Schultz WH, Jackson S, et al. Effects of chronic transfusion therapy on transcranial doppler ultrasonography velocities in children with sickle cell anemia at risk for primary stroke: Baseline findings from the twitch trial. Blood 2014;124(21):87. [4681005]
98. NCT01425307 Transcranial doppler (TCD) with transfusions changing to hydroxyurea (TWiTCH). clinicaltrials.gov/ct2/show/NCT01425307 accessed 29 May 2016). [4681006]
99. Ware RE, Davis BR, Schultz WH, Brown C, Aygun B, Sarnaik SA, et al. TCD with transfusions changing to hydroxyurea (TWITCH): Hydroxyurea therapy as an alternative to transfusions for primary stroke prevention in children with sickle cell anemia. Blood 2015;126(23):3. [4681007]
100. Ware RE, Davis BR, Schultz WH, Brown RC, Aygun B, Sarnaik S, 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;387(10019):661-70. [CENTRAL: 1138971; CRS: 5500050000000362; 4681008; EMBASE: 20160126001; NCT01425307: TCD With Transfusions Changing to Hydroxyurea (TWiTCH): A Phase III Randomized Trial to Compare Standard Therapy (Erythrocyte Transfusions) With Alternative Therapy (Hydroxyurea) for the Maintenance of Lowered TCD Velocities in Pediatric Subjects With Sickle Cell Anemia and Abnormal Pre-treatment TCD Velocities]
101. Wood JC, Cohen A, Aygun B, Imran H, Luchtman-Jones L, Thompson AA, et al. Extrahepatic iron deposition in chronically transfused children with sickle cell anemia - Baseline findings from the twitch trial. Blood 2013;122(21):2238. [4681009]
102. Wood JC, Cohen AR, Pressel SL, Aygun B, Imran H, Luchtman-Jones L, et al. Organ iron accumulation in chronically transfused children with sickle cell anaemia: Baseline results from the TWiTCH trial. British Journal of Haematology 2016;172(1):122-30. [CENTRAL: 1133502; CRS: 5500050000000361; 4681010; EMBASE: 20151011308]
103. Wood JC, Pressel S, Rogers ZR, Odame I, Kwiatkowski JL, Lee MT, et al. Liver iron concentration measurements by MRI in chronically transfused children with sickle cell anemia: Baseline results from the TWiTCH trial. American Journal of Hematology 2015;90(9):806-10. [CENTRAL: 1090263; CRS: 5500050000000271; 4681011; EMBASE: 2015311270]
104. Bernaudin FV, Ducros-Miralles S, Ducros-Miralles E, Delatour RP, Dalle J-H, Petras E, et al. French national drepagreffe trial: Cognitive performances and neuroimaging at enrollment and after 12 months on transfusion program or transplantation (AP-HP: NCT 01340404). Blood 2015;126(23):544. [4681013]
105. Hankins JS, McCarville MB, Rankine-Mullings A, Reid ME, Lobo CL, Moura PG, et al. Prevention of conversion to abnormal transcranial Doppler with hydroxyurea in sickle cell anemia: A Phase III international randomized clinical trial. American Journal of Hematology 2015;90(12):1099-105. [2987134]
106. NCT01531387 Sparing conversion to abnormal TCD (transcranial doppler) elevation (SCATE). clinicaltrials.gov/show/NCT01531387 (accessed 06 November 2013). [2987135]
107. Adams RJ, Nichols FT, Figueroa R, McKie V, Lott T. Transcranial Doppler correlation with cerebral angiography in sickle cell disease. Stroke 1992;23(8):1073-7.
108. Adams RJ, McKie VC, Brambilla D, Carl E, Gallagher D, Nichols FT, et al. Stroke prevention trial in sickle cell anemia. Controlled Clinical Trials 1998;19(1):110-29.
109. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography. New England Journal of Medicine 1998;339(1):5-11.
110. Ansong D, Osei Akoto A, Ocloo D, Ohene-Frempong K. Sickle cell disease: management options and challenges in developing countries. Mediterranean Journal of Hematology and Infectious Diseases 2013;5(1):e2013062.
111. Aygun B, McMurray MA, Schultz WH, Kwiatkowski JL, Hilliard L, Alvarez O, et al. Chronic transfusion practice for children with sickle cell anaemia and stroke. British Journal of Haematology 2009;145(4):524-8.
112. Bernaudin F, Verlhac S, Arnaud C, Kamdem A, Chevret S, Hau I, et al. Impact of early transcranial Doppler screening and intensive therapy on cerebral vasculopathy outcome in a newborn sickle cell anemia cohort. Blood 2011;117(4):1130-40.
113. Chou ST, Jackson T, Vege S, Smith-Whitley K, Friedman DF, Westhoff CM. High prevalence of red blood cell alloimmunization in sickle cell disease despite transfusion from Rh-matched minority donors. Blood 2013;122:1062-1071.
114. Cohen AR, Martin MB, Silber JH, Kim HC, Ohene-Frempong K, Schwarz E. A modified program for prevention of stroke in sickle cell disease. Blood 1992;79(7):1657-61.
115. Cohen AR, Norris CF, Smith-Whitley K. Transfusion therapy for sickle cell disease. In: Capon SM, Chambers LA editor(s). New Directions in Pediatric Hematology. Bethesda MD: American Association of Blood Banks, 1996:39-85.
116. Veritas Health Innovation. Covidence systematic review software. Version accessed prior to 28 November 2016. Melbourne, Australia: Veritas Health Innovation, 2015.
117. DeBaun MR, Derdeyn CP, McKinstry RC. Etiology of strokes in children with sickle cell anemia. Mental Retardation and Developmental Disabilities Research Reviews 2006;12(3):192-9.
118. Deeks JJ, Higgins JPT, Altman DG. Chapter 9: Analysing data and undertaking meta-analyses. In: Higgins JP, Green S, editor(s). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Version 5.1.0 (updated March 2011). The Cochrane Collaboration.
119. Enninful-Eghan H, Moore RH, Ichord R, Smith-Whitley K, Kwiatkowski JL. Transcranial doppler ultrasonography and prophylactic transfusion program is effective in preventing overt stroke in children with sickle cell disease. Journal of Pediatrics 2010;157(3):479-84.
120. Estcourt LJ, Fortin PM, Hopewell S, Trivella M, Doree C, Abboud MR. Interventions for preventing silent cerebral infarcts in people with sickle cell disease. Cochrane Database of Systematic Reviews 2016, Issue 10. [DOI: 10.1002/14651858.CD012389]
121. Fullerton HJ, Adams RJ, Zhao S, Johnston SC. Declining stroke rates in Californian children with sickle cell disease. Blood 2004;104(2):336-9.
122. Gravitz L, Pincock S. Sickle-cell disease. Nature 2014;515:S1.
123. Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, PhD, et al. Sickle cell disease in Africa. A neglected cause of early childhood mortality. American Journal of Preventive Medicine 2011;41:S398-405.
124. Guilliams KP, Fields ME, Hulbert ML. Higher-than-expected prevalence of silent cerebral infarcts in children with hemoglobin SC disease. Blood 2015;125:416-417.
125. Higgins J P T, Deeks J J. Chapter 7: Selecting studies and collecting data. In: Higgins JP, Green S, editors(s).Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Version 5.1.0 (updated March 2011). The Cochrane Collaboration.
126. Higgins J P T, Altman D G, Sterne J A C. Chapter 8: Assessing risk of bias in included studies. In: Higgins JP, Green S, editor(s).Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Version 5.1.0 (updated March 2011). The Cochrane Collaboration.
127. Higgins J P T, Deeks J J, Altman D G. Chapter 16: Special topics in statistics. In: Higgins JP, Green S, editor(s). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Version 5.1.0 (updated March 2011). The Cochrane Collaboration.
128. Inati A, Khoriaty E, Musallam KM. Iron in sickle-cell disease: what have we learned over the years?. Pediatric Blood & Cancer 2011;56(2):182-90.
129. Kinney TR, Sleeper LA, Wang WC, Zimmerman RA, Pegelow CH, Ohene-Frempong K, et al. Silent cerebral infarcts in sickle cell anemia: a risk factor analysis. Pediatrics 1999;103(3):640-5.
130. Kwiatkowski JL, Zimmerman RA, Pollock AN, Seto W, Smith-Whitley K, Shults J, et al. Silent infarcts in young children with sickle cell disease. British Journal of Haemtology 2009;146(3):300-5.
131. Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood 2006;108(3):847-52.
132. McCarville MB, Goodin GS, Fortner G, Li CS, Smeltzer MP, Adams R, et al. Evaluation of a comprehensive transcranial doppler screening program for children with sickle cell anemia. Pediatric Blood & Cancer 2008;50(4):818-21.
133. Nagel RL, Fabry ME, Steinberg MH. The paradox of hemoglobin SC disease. Blood Reviews 2003;17(3):167-78.
134. (NICE) National Institute for Health and Care Excellence. Sickle cell disease 2010. cks.nice.org.uk/sickle-cell-disease#!backgroundsub:3 (accessed April 2016).
135. Ohene-Frempong K. Sickle cell disease in the United States of America and Africa. Hematology (American Society of Hematology Education Program). American Society of Hematology, 1999:64-72.
136. Pegelow CH, Adams RJ, McKie V, Abboud M, Berman B, Miller ST, et al. Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. Journal of Pediatrics 1995;126(6):896-9.
137. Piel F B, Patil A P, Howes R E, Nyangiri O A, Gething P W, Dewi M, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet 2012;381:142-51.
138. Pleasants S. Epidemiology: a moving target. Nature 2014;515:S2-3.
139. Powars DR. Management of cerebral vasculopathy in children with sickle cell anaemia. British Journal of Hematology 2000;108(4):666-78.
140. Rana S, Houston PE, Surana N, Shalaby-Rana EI, Castro OL. Discontinuation of long-term transfusion therapy in patients with sickle cell disease and stroke. Journal of Pediatrics 1997;131(5):757-60.
141. Rees D C, Williams T N, Gladwin M T. Sickle-cell disease. Lancet 2010;376:2018-31.
142. Nordice Cochrane Centre, The Cochrane Collaboration. Review Manager (RevMan). Version 5.3. Copenhagen: Nordice Cochrane Centre, The Cochrane Collaboration, 2014.
143. Rumaney MB, Ngo Bitoungui VJ, Vorster AA, Ramesar R, Kengne AP, Ngogang J, et al. The co-inheritance of alpha-thalassemia and sickle cell anemia Is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. PLoS ONE 2014;9:e100516.
144. Sarnaik S, Soorya D, Kim J, Ravindranath Y, Lusher J. Periodic transfusions for sickle cell anemia and CNS infarction. American Journal of Diseases of Children 1979;133(12):1254-7.
145. Schünemann HJ, Oxman AD, Higgins JPT, Vist GE, Glasziou P, Guyatt GH. Chapter 11: Presenting results and 'Summary of findings' tables. In: Higgins JPT, Green S, editor(s). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Version 5.1.0 (updated March 2011). The Cochrane Collaboration.
146. Schünemann HJ, Oxman AD, Vist GE, Higgins JPT, Deeks JJ, Glasziou P, et al. Chapter 12: Interpreting results and drawing conclusions. In: Higgins JP, Green S, editors(s). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Version 5.1.0 [updated March 2011]. The Cochrane Collaboration.
147. Scothorn DJ, Price C, Schwartz D, Terrill C, Buchanan GR, Shurney W, et al. Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke. Journal of Pediatrics 2002;140(3):48-54.
148. Serjeant GR. Surgery and anaesthesia. Sickle Cell Disease. 2nd Edition. Oxford: Oxford University Press, 1992:455-8.
149. Smith-Whitley K, Thompson AA. Indications and complications of transfusions in sickle cell disease. Pediatric Blood & Cancer 2012;59(2):358-64.
150. Steinberg MH. Management of sickle cell disease. New England Journal of Medicine 1999;340(13):1021-30.
151. Steinberg MH, Sebastiani P. Genetic modifiers of sickle cell disease. American Journal of Hematology 2012;87(8):795-803.
152. Sterne JAC, Egger M, Moher D. Chapter 10: Addressing reporting biases. In: Higgins JP, Green S, editor(s). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from handbook.cochrane.org. Version 5.1.0 (updated March 2011). The Cochrane Collaboration.
153. Styles LA, Vichinsky E. Effects of a long-term transfusion regimen on sickle cell-related illnesses. Journal of Pediatrics 1994;125(6 Pt 1):909-11.
154. Telfer P, Coen P, Chakravorty S, Wilkey O, Evans J, Newell H, et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica 2007;92(7):905-12.
155. Venketasubramanian N, Prohovnik I, Hurlet A, Mohr JP, Piomelli S. Middle cerebral artery velocity changes during transfusion in sickle cell anemia. Stroke 1994;25(11):2153-8.
156. Wang W, Kovnar EH, Tonkin IL, Mulhern RK, Langston JW, Day S, et al. High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease. Journal of Pediatrics 1991;118(3):377-82.
157. Wang WC, Morales KH, Scher CD, Styles L, Olivieri N, Adams R, et al. Effect of long-term transfusion on growth in children with sickle cell anemia: results of the STOP trial. Journal of Pediatrics 2005;147(2):244-7.
158. Ware RE, Zimmermann SA, Sylvestre PB, Mortier NA, Davis JS, Treem WR, et al. Prevention of secondary stroke and resolution of transfusional iron overload in children with sickle cell anemia using hydroxyurea and phlebotomy. Journal of Pediatrics 2004;145(3):346-52.
159. Ware RE. How I use hydroxyurea to treat young patients with sickle cell anemia. Blood 2010;115(26):5300-11.
160. Wilimas J, Goff JR, Anderson HR, Langston JW, Thomson E. Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents. Journal of Pediatrics 1980;96(2):205-8.
161. Hirst C, Wang WC. Blood transfusion for preventing stroke in people with sickle cell disease. Cochrane Database of Systematic Reviews 2002, Issue 1. [DOI: 10.1002/14651858.CD003146]
162. Wang WC, Dwan K. Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease. Cochrane Database of Systematic Reviews 2013, Issue 11. [DOI: 10.1002/14651858.CD003146.pub2]