Yawn Barbara P, John-Sowah Joylene
Olmsted Medical Center, Rochester, MN, USA.
National Heart, Lung, and Blood Institute, Bethesda, MD, USA.
Am Fam Physician. 2015 Dec 15;92(12):1069-76.
Family physicians are the primary and sometimes only health care resource for families affected by sickle cell disease. Recently published guidelines provide important recommendations for health maintenance, acute care, and monitoring of disease-modifying therapy in persons with this condition. This overview highlights some of the most important clinical activities that can and should be carried out in the community care setting. Children with sickle cell anemia should receive prophylactic penicillin from birth through at least five years of age, and all persons with sickle cell disease require vaccination to prevent invasive pneumococcal disease. Annual screening with transcranial Doppler ultrasonography is recommended for all children with sickle cell disease beginning at two years of age and continuing through adolescence to evaluate the risk of stroke and to initiate transfusion therapy in those at high risk. Vasoocclusive crises require immediate and adequate analgesia appropriate to the level of patient-reported pain. Antibiotics, hospitalization, and incentive spirometry are indicated for those with acute chest syndrome. There is strong evidence to support the promotion and use of hydroxyurea therapy in patients nine months and older who have sickle cell anemia because its use can decrease the frequency of vasoocclusive crises and acute chest syndrome with limited adverse effects.
家庭医生是受镰状细胞病影响家庭的主要甚至有时是唯一的医疗保健资源。最近发布的指南为患有这种疾病的人的健康维护、急性护理和疾病修饰疗法监测提供了重要建议。本概述重点介绍了在社区护理环境中能够且应该开展的一些最重要的临床活动。镰状细胞贫血患儿应从出生起至至少五岁接受预防性青霉素治疗，所有镰状细胞病患者都需要接种疫苗以预防侵袭性肺炎球菌疾病。建议对所有两岁及以上且持续至青春期的镰状细胞病患儿每年进行经颅多普勒超声检查，以评估中风风险，并对高危患儿启动输血治疗。血管闭塞性危机需要根据患者报告的疼痛程度立即给予充分的镇痛治疗。对于患有急性胸综合征的患者，需要使用抗生素、住院治疗并进行激励性肺量测定。有强有力的证据支持在九个月及以上的镰状细胞贫血患者中推广和使用羟基脲疗法，因为其使用可减少血管闭塞性危机和急性胸综合征的发生频率，且不良反应有限。
