Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography

Robert J. Adams, Virgil C. Mckie, Lewis Hsu, Files Beatrice Files, Elliott Vichinsky, Charles Pegelow, Miguel Abboud, Dianne Gallagher, Abdullah Kutlar, Fenwick T Nichols, Duane R. Bonds, Donald Brambilla, Gerald Woods, Nancy Olivieri, Catherine Driscoll, Scott Miller, Winfred Wang, Anne Hurlett, Charles Scher, Brian Berman & 6 others Elizabeth Carl, Anne M. Jones, E. Steve Roach, Elizabeth Wright, Robert A. Zimmerman, Myron Waclawiw

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Abstract

Background Blood transfusions prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in preventing a first stroke is unknown. We used transcranial Doppler ultrasonography to identify children with sickle cell anemia who were at high risk for stroke and then randomly assigned them to receive standard care or transfusions to prevent a first stroke. Methods To enter the study, children with sickle cell anemia and no history of stroke had to have undergone two transcranial Doppler studies that showed that the time-averaged mean blood-flow velocity in the internal carotid or middle cerebral artery was 200 cm per second or higher. The patients were randomly assigned to receive standard care or transfusions to reduce the hemoglobin S concentration to less than 30 percent of the total hemoglobin concentration. The incidence of stroke (cerebral infarction or intracranial hemorrhage) was compared between the two groups. Results A total of 130 children (mean [±SD] age, 8.3±3.3 years) were enrolled; 63 were randomly assigned to receive transfusions, and 67 to receive standard care. At base line, the transfusion group had a slightly lower mean hemoglobin concentration (7.2 vs. 7.6 g per deciliter, P=0.001) and hematocrit (20.4 vs. 21.7 percent, P=0.002). Ten patients dropped out of the transfusion group, and two patients crossed over from the standard-care group to the transfusion group. There were 10 cerebral infarctions and 1 intracerebral hematoma in the standard-care group, as compared with 1 infarction in the transfusion group - a 92 percent difference in the risk of stroke (P<0.001). This result led to the early termination of the trial. Conclusions Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.

Original languageEnglish (US)
Pages (from-to)5-11
Number of pages7
JournalNew England Journal of Medicine
Volume339
Issue number1
DOIs
StatePublished - Jul 2 1998

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Doppler Transcranial Ultrasonography
Sickle Cell Anemia
Stroke
Cerebral Infarction
Hemoglobins
Sickle Hemoglobin
Blood Flow Velocity
Intracranial Hemorrhages
Cerebral Hemorrhage
Middle Cerebral Artery
Hematocrit
Blood Transfusion
Hematoma
Infarction
Incidence

ASJC Scopus subject areas

  • Medicine(all)

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Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. / Adams, Robert J.; Mckie, Virgil C.; Hsu, Lewis; Beatrice Files, Files; Vichinsky, Elliott; Pegelow, Charles; Abboud, Miguel; Gallagher, Dianne; Kutlar, Abdullah; Nichols, Fenwick T; Bonds, Duane R.; Brambilla, Donald; Woods, Gerald; Olivieri, Nancy; Driscoll, Catherine; Miller, Scott; Wang, Winfred; Hurlett, Anne; Scher, Charles; Berman, Brian; Carl, Elizabeth; Jones, Anne M.; Roach, E. Steve; Wright, Elizabeth; Zimmerman, Robert A.; Waclawiw, Myron.

In: New England Journal of Medicine, Vol. 339, No. 1, 02.07.1998, p. 5-11.

Research output: Contribution to journalArticle

Adams, RJ, Mckie, VC, Hsu, L, Beatrice Files, F, Vichinsky, E, Pegelow, C, Abboud, M, Gallagher, D, Kutlar, A, Nichols, FT, Bonds, DR, Brambilla, D, Woods, G, Olivieri, N, Driscoll, C, Miller, S, Wang, W, Hurlett, A, Scher, C, Berman, B, Carl, E, Jones, AM, Roach, ES, Wright, E, Zimmerman, RA & Waclawiw, M 1998, '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, vol. 339, no. 1, pp. 5-11. https://doi.org/10.1056/NEJM199807023390102
Adams, Robert J. ; Mckie, Virgil C. ; Hsu, Lewis ; Beatrice Files, Files ; Vichinsky, Elliott ; Pegelow, Charles ; Abboud, Miguel ; Gallagher, Dianne ; Kutlar, Abdullah ; Nichols, Fenwick T ; Bonds, Duane R. ; Brambilla, Donald ; Woods, Gerald ; Olivieri, Nancy ; Driscoll, Catherine ; Miller, Scott ; Wang, Winfred ; Hurlett, Anne ; Scher, Charles ; Berman, Brian ; Carl, Elizabeth ; Jones, Anne M. ; Roach, E. Steve ; Wright, Elizabeth ; Zimmerman, Robert A. ; Waclawiw, Myron. / Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. In: New England Journal of Medicine. 1998 ; Vol. 339, No. 1. pp. 5-11.
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abstract = "Background Blood transfusions prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in preventing a first stroke is unknown. We used transcranial Doppler ultrasonography to identify children with sickle cell anemia who were at high risk for stroke and then randomly assigned them to receive standard care or transfusions to prevent a first stroke. Methods To enter the study, children with sickle cell anemia and no history of stroke had to have undergone two transcranial Doppler studies that showed that the time-averaged mean blood-flow velocity in the internal carotid or middle cerebral artery was 200 cm per second or higher. The patients were randomly assigned to receive standard care or transfusions to reduce the hemoglobin S concentration to less than 30 percent of the total hemoglobin concentration. The incidence of stroke (cerebral infarction or intracranial hemorrhage) was compared between the two groups. Results A total of 130 children (mean [±SD] age, 8.3±3.3 years) were enrolled; 63 were randomly assigned to receive transfusions, and 67 to receive standard care. At base line, the transfusion group had a slightly lower mean hemoglobin concentration (7.2 vs. 7.6 g per deciliter, P=0.001) and hematocrit (20.4 vs. 21.7 percent, P=0.002). Ten patients dropped out of the transfusion group, and two patients crossed over from the standard-care group to the transfusion group. There were 10 cerebral infarctions and 1 intracerebral hematoma in the standard-care group, as compared with 1 infarction in the transfusion group - a 92 percent difference in the risk of stroke (P<0.001). This result led to the early termination of the trial. Conclusions Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.",
author = "Adams, {Robert J.} and Mckie, {Virgil C.} and Lewis Hsu and {Beatrice Files}, Files and Elliott Vichinsky and Charles Pegelow and Miguel Abboud and Dianne Gallagher and Abdullah Kutlar and Nichols, {Fenwick T} and Bonds, {Duane R.} and Donald Brambilla and Gerald Woods and Nancy Olivieri and Catherine Driscoll and Scott Miller and Winfred Wang and Anne Hurlett and Charles Scher and Brian Berman and Elizabeth Carl and Jones, {Anne M.} and Roach, {E. Steve} and Elizabeth Wright and Zimmerman, {Robert A.} and Myron Waclawiw",
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T1 - Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography

AU - Adams, Robert J.

AU - Mckie, Virgil C.

AU - Hsu, Lewis

AU - Beatrice Files, Files

AU - Vichinsky, Elliott

AU - Pegelow, Charles

AU - Abboud, Miguel

AU - Gallagher, Dianne

AU - Kutlar, Abdullah

AU - Nichols, Fenwick T

AU - Bonds, Duane R.

AU - Brambilla, Donald

AU - Woods, Gerald

AU - Olivieri, Nancy

AU - Driscoll, Catherine

AU - Miller, Scott

AU - Wang, Winfred

AU - Hurlett, Anne

AU - Scher, Charles

AU - Berman, Brian

AU - Carl, Elizabeth

AU - Jones, Anne M.

AU - Roach, E. Steve

AU - Wright, Elizabeth

AU - Zimmerman, Robert A.

AU - Waclawiw, Myron

PY - 1998/7/2

Y1 - 1998/7/2

N2 - Background Blood transfusions prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in preventing a first stroke is unknown. We used transcranial Doppler ultrasonography to identify children with sickle cell anemia who were at high risk for stroke and then randomly assigned them to receive standard care or transfusions to prevent a first stroke. Methods To enter the study, children with sickle cell anemia and no history of stroke had to have undergone two transcranial Doppler studies that showed that the time-averaged mean blood-flow velocity in the internal carotid or middle cerebral artery was 200 cm per second or higher. The patients were randomly assigned to receive standard care or transfusions to reduce the hemoglobin S concentration to less than 30 percent of the total hemoglobin concentration. The incidence of stroke (cerebral infarction or intracranial hemorrhage) was compared between the two groups. Results A total of 130 children (mean [±SD] age, 8.3±3.3 years) were enrolled; 63 were randomly assigned to receive transfusions, and 67 to receive standard care. At base line, the transfusion group had a slightly lower mean hemoglobin concentration (7.2 vs. 7.6 g per deciliter, P=0.001) and hematocrit (20.4 vs. 21.7 percent, P=0.002). Ten patients dropped out of the transfusion group, and two patients crossed over from the standard-care group to the transfusion group. There were 10 cerebral infarctions and 1 intracerebral hematoma in the standard-care group, as compared with 1 infarction in the transfusion group - a 92 percent difference in the risk of stroke (P<0.001). This result led to the early termination of the trial. Conclusions Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.

AB - Background Blood transfusions prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in preventing a first stroke is unknown. We used transcranial Doppler ultrasonography to identify children with sickle cell anemia who were at high risk for stroke and then randomly assigned them to receive standard care or transfusions to prevent a first stroke. Methods To enter the study, children with sickle cell anemia and no history of stroke had to have undergone two transcranial Doppler studies that showed that the time-averaged mean blood-flow velocity in the internal carotid or middle cerebral artery was 200 cm per second or higher. The patients were randomly assigned to receive standard care or transfusions to reduce the hemoglobin S concentration to less than 30 percent of the total hemoglobin concentration. The incidence of stroke (cerebral infarction or intracranial hemorrhage) was compared between the two groups. Results A total of 130 children (mean [±SD] age, 8.3±3.3 years) were enrolled; 63 were randomly assigned to receive transfusions, and 67 to receive standard care. At base line, the transfusion group had a slightly lower mean hemoglobin concentration (7.2 vs. 7.6 g per deciliter, P=0.001) and hematocrit (20.4 vs. 21.7 percent, P=0.002). Ten patients dropped out of the transfusion group, and two patients crossed over from the standard-care group to the transfusion group. There were 10 cerebral infarctions and 1 intracerebral hematoma in the standard-care group, as compared with 1 infarction in the transfusion group - a 92 percent difference in the risk of stroke (P<0.001). This result led to the early termination of the trial. Conclusions Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.

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