Sickle Cell Disease
Sickle Cell Disease
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DOI：
10.1542/pir.33-5-195
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发表时间：
2012-05-01
影响因子：
1.3
通讯作者：
McCavit, Timothy L.
中科院分区：
文献类型：
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作者：
McCavit, Timothy L.
镰状细胞病（SCD）是一组普遍存在的、可能危及生命的慢性血红蛋白（Hgb）疾病。Hgb聚合是SCD病理生理学的基础。患有SCD的儿童受益于儿科血液科医生和初级保健儿科医生的定期健康维护就诊。SCD中侵袭性肺炎球菌疾病（IPD）的高发病率证明新生儿筛查、每日预防性青霉素、血管闭塞性疼痛危象是SCD的临床标志，在儿童期发生的频率越来越高。这些发作需要积极的止痛剂和水化治疗，并可以预防与hydroxyurea（HU）治疗。每年经颅多普勒（TCD）筛查的患者年龄2至16岁确定那些在急性中风的高风险，定期输血可以大大降低这种风险。启动HU治疗的常见适应症是严重或频繁的血管闭塞性危象或急性胸部综合征，但这种疗法可以考虑在年轻和症状较轻的患者中使用。2 SCD儿童的预后有所改善，绝大多数儿童可以存活到成年，这促使人们关注改善向成人护理过渡的过程。
Sickle cell disease (SCD) is a heterogeneous group of prevalent, potentially life-threatening, chronic disorders of hemoglobin (Hgb).Hgb polymerization underlies the pathophysiology of SCD.Children who have SCD benefit from regular health maintenance visits with a pediatric hematologist and a primary care pediatrician.The high incidence of invasive pneumococcal disease (IPD) in SCD justifies newborn screening, daily prophylactic penicillin, and immunization with the pneumococcal conjugate and polysaccharide vaccines.Vaso-occlusive pain crises are the clinical hallmark of SCD and occur with increasing frequency through childhood. These episodes warrant aggressive treatment with analgesics and hydration and may be prevented with hydroxyurea (HU) therapy.Annual transcranial Doppler (TCD) screening for patients ages 2 to 16 years identifies those at high risk for acute stroke, and regular blood transfusions can reduce this risk greatly.Common indications for initiating HU therapy have been severe or frequent vaso-occlusive crises or acute chest syndrome, but this therapy may be considered in younger and less symptomatic patients.The prognosis for children with SCD has improved, with the vast majority surviving into adulthood, prompting a focus on improving the process of transition to adult care.