Madden Nicholas A, Jones Gary L, Kalpatthi Ram, Woods Gerald
Children's Mercy Hospitals and Clinics, Kansas City, MO.
J Pediatr Hematol Oncol. 2014 Aug;36(6):e382-6. doi: 10.1097/MPH.0000000000000160.
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 HbSβ. 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 HbSβ, 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.
镰状细胞病（SCD）患者中，随着经颅多普勒超声和慢性输血疗法的应用，中风发病率有所下降。关于这些疗法在除HbSS和HbSβ之外的其他基因型中的应用，相关信息较少。通过磁共振成像（MRI）已在SCD患者中发现无症状脑梗死（SCI），据信这些可能会增加明显中风的风险。目前尚无关于MRI筛查SCI的循证指南。羟基脲是有急性胸部综合征病史以及严重、复发性SCD相关疼痛发作患者的标准疗法，但尚未确定其在其他镰状细胞相关疾病中的应用。共有102家机构收到了一项调查（收到62份回复），以评估经颅多普勒超声用于中风筛查的情况、筛查MRI用于SCI的情况以及羟基脲的处方机构模式。19%的机构筛查除HbSS和HbSβ之外的其他基因型，24%的机构使用MRI筛查SCI。26%的机构对发现患有SCI的患者开具羟基脲。结果表明，中风筛查和羟基脲使用情况存在显著差异，这通常与诊所规模、医生数量以及地理位置相关。目前尚无循证指南支持其中许多做法。
