All searches were executed on April 5, 2018, except for Cochrane Central which was run on May 1, 2018. Our primary outcome was silent stroke, secondary outcomes included stroke and adverse events attributed to hydroxyurea.Įlectronic searches were developed and performed by medical librarians (KS, RP) in PubMed, which includes Medline, EMBASE, Web of Science Core Collection, and Cochrane Central Register of Controlled Trials. Observational studies evaluating the role of hydroxyurea to prevent stroke and silent stroke in patients with sickle cell anemia or sickle cell β thalassemia were eligible for inclusion. Ethical approval by an institutional review board was waived as this was a systematic review. Randomized controlled trials (RCTs) comparing the use of hydroxyurea vs blood transfusions or observation alone to prevent stroke and silent stroke in patients with sickle cell anemia or sickle cell β thalassemia were eligible for inclusion. This systematic review was conducted according to the Cochrane guidelines. The goal of this systematic review is to summarize the available data on the use of hydroxyurea for the prevention of SCI in patients with sickle cell disease.Ģ.1. It is used as therapy for the prevention of complications of SCD, including pain and decreased organ function and it may be indicated to prevent stroke in patients with sickle cell disease. Hydroxyurea, first tested in sickle cell anemia in 1984, has emerged as a vital therapeutic option. Although SCIs may not have obvious clinical symptoms, there are clearly neurocognitive consequences.Ĭhronic blood transfusions are the standard treatment for stroke prevention, but this method is not cost effective and not without risks, including iron overload and alloimmunization. Children with SCD and SCI have higher global intellectual functioning scores than children who have had overt strokes however, their scores are significantly lower than those of children without the disease. This often results in lifelong neuropsychological effects, including lower intelligence quotients and poorer processing speeds compared to children without SCD and its complications. Children who have SCIs are more likely to experience further neurological events, including stroke, than children without evidence of SCIs. The prevalence of SCI in adults is not well studied. In children with sickle cell disease (SCD), silent cerebral infarct (SCI) occurs in 27% before the age of six years and 37% up to the age of 14 years, and approximately 11% of children with SCD will experience an overt stroke with the highest prevalence before the age of 6 years. Silent strokes, defined as ischemic changes in brain tissue visible on imaging without overt physical signs or symptoms of a stroke, are more common than overt strokes and are more likely to occur with increasing age.
Neurological complications are a major cause of morbidity and mortality in sickle cell disease.
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