Practice Patterns of Stroke Screening and Hydroxyurea Use in Children With Sickle Cell Disease: A Survey of Health Care Providers

Practice Patterns of Stroke Screening and Hydroxyurea Use in Children With Sickle Cell Disease: A Survey of Health Care Providers
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DOI:
10.1097/mph.0000000000000160
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发表时间:
2014-08-01
影响因子:
1.2
通讯作者:
Woods, Gerald
Woods, Gerald
中科院分区:
医学4区
文献类型:
--
作者:
Madden, Nicholas A.;Jones, Gary L.;Woods, Gerald

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随着经颅多普勒超声和长期输血治疗的使用,镰状细胞病(SCD)的卒中发生率有所下降。关于它们在HbSS和HbS β(0)以外的基因型中使用的信息很少。通过磁共振成像(MRI)在SCD患者中发现了无症状性脑梗死(SCI),并且认为这些可能增加明显卒中的风险。关于SCI的MRI筛查,目前尚无循证指南。羟基脲是有急性胸部综合征和重度、复发性SCD相关疼痛发作病史的患者的标准治疗,但尚未确定用于其他镰刀相关疾病。共有102家机构接受了一项调查(62份回复),以评估经颅多普勒超声用于卒中筛查、MRI用于SCI筛查以及处方羟基脲的机构模式。19%的机构筛查HbSS和HbSb0以外的基因型,24%的机构使用MRI筛查SCI。26%的机构在发现有SCI的患者中处方了羟基脲。结果表明,中风筛查和羟基脲使用的显着变化往往与诊所规模,医生提供者的数量和地理位置相关。目前还没有循证指南来支持其中许多做法。
Incidence of stroke in sickle cell disease (SCD) has declined with the use of transcranial Doppler ultrasound and chronic transfusion therapy. There is little information regarding their use in genotypes other than HbSS and HbS beta(0). Silent cerebral infarcts (SCIs) have been identified by magnetic resonance imaging (MRI) in SCD patients and it is believed that these may increase the risk of overt stroke. No evidence-based guidelines exist regarding MRI screening for SCIs. Hydroxyurea is a standard therapy in patients with history of acute chest syndrome and severe, recurrent, SCD-associated pain episodes, but has not been established for use with other sickle-associated morbidities. A total of 102 institutions received a survey (with 62 responses) to assess the use of transcranial Doppler ultrasound for stroke screening, use of screening MRI for SCIs, and institutional patterns for prescribing hydroxyurea. Nineteen percent of institutions screen genotypes other than HbSS and HbSb0, and 24% use MRI to screen for SCIs. Twenty-six percent of institutions prescribed hydroxyurea in patient found to have SCIs. Results indicate significant variation in stroke screening and hydroxyurea use often correlating with clinic size, number of physician providers, and geographic location. There are currently no evidence-based guidelines to support many of these practices.