Feasibility and efficacy of chronic transfusion for stroke prevention in children with sickle cell disease

@article{Mirre2010FeasibilityAE,
  title={Feasibility and efficacy of chronic transfusion for stroke prevention in children with sickle cell disease},
  author={Elsa Mirre and Valentine Brousse and Laureline Berteloot and Karen Lambot-Juhan and Suzanne Verlhac and Claire Boulat and Marie Dumont and G{\'e}rard Lenoir and Mariane de Montalembert},
  journal={European Journal of Haematology},
  year={2010},
  volume={84}
}
Objectives:  In children with sickle cell disease (SCD), chronic transfusion to maintain haemoglobin S (HbS) below 30% markedly decreases both the risk of a first stroke when transcranial Doppler (TCD) ultrasonography shows abnormal cerebral blood flow velocities and the risk of recurrent stroke. Maintaining HbS below 30% may be difficult, especially in countries where blood donors and recipients belong to different ethnic groups and where the availability of closely matched blood products is… Expand
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[Transfusion in children with sickle cell disease].
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  • 2012
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TLDR
Receiving scheduled transfusions on time was the most predictive variable for maintaining HbS at the ≤30% goal. Expand
Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography.
TLDR
Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography. Expand
Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease.
TLDR
Discontinuation of transfusion for the prevention of stroke in children with sickle cell disease results in a high rate of reversion to abnormal blood-flow velocities on Doppler studies and stroke. Expand
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TLDR
Evidence is offered that a target pretransfusion HbS level of 50% affords a continuing high rate of protection against recurrent cerebral infarction in sickle cell disease after 4 years of a conventional transfusion program. Expand
The use of transcranial ultrasonography to predict stroke in sickle cell disease.
TLDR
Transcranial ultrasonography can identify the children with sickle cell disease who are at highest risk for cerebral infarction and periodic ultrasound examinations and the selective use of transfusion therapy could make the primary prevention of stroke an achievable goal. Expand
Does regular blood transfusion prevent progression of cerebrovascular lesions in children with sickle cell disease?
TLDR
Optimal transfusion therapy does not prevent progression of cerebral vasculopathy in SCD children with a history of stroke and within-patient score changes evaluated using Wilcoxon’s paired rank test indicated lesion progression in the secondary-prevention group. Expand
Long-term follow-up of pediatric sickle cell disease patients with abnormal high velocities on transcranial Doppler
TLDR
It is postulated that patients with normal MRA findings and abnormal TCD velocities that normalized on a transfusion program could be safely treated with hydroxyurea (HU), and six other transplanted patients remain transfusion-free. Expand
Blood transfusion for preventing stroke in people with sickle cell disease.
TLDR
A trial compared a chronic transfusion regime to maintain sickle haemoglobin at less than 30% with standard care in 130 children with sickle cell disease who were judged to be at high risk of a first stroke through transcranial doppler ultrasonography, demonstrating a significantly reduced risk of stroke. Expand
Prevention of secondary stroke and resolution of transfusional iron overload in children with sickle cell anemia using hydroxyurea and phlebotomy.
TLDR
For children with SCA and stroke, hydroxyurea effectively prevents secondary stroke and serial phlebotomy leads to complete resolution of transfusional iron overload. Expand
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TLDR
The epidemiology, clinical spectrum, and pathophysiology of stroke in sickle-cell disease is reviewed to identify potential therapeutic targets and interventions directed towards the potential mechanisms that promote vasculopathy and occlusion in sickre-cell anaemia should be investigated. Expand
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