Texas Children's Center for Global Health, Houston, Texas 77030, USA.
Cold Spring Harb Perspect Med. 2013 Aug 1;3(8):a011817. doi: 10.1101/cshperspect.a011817.
Proper management of sickle cell anemia (SCA) begins with establishing the correct diagnosis early in life, ideally during the newborn period. The identification of affected infants by neonatal screening programs allows early initiation of prophylactic penicillin and pneumococcal immunizations, which help prevent overwhelming sepsis. Ongoing education of families promotes the early recognition of disease-released complications, which allows prompt and appropriate medical evaluation and therapeutic intervention. Periodic evaluation by trained specialists helps provide comprehensive care, including transcranial Doppler examinations to identify children at risk for primary stroke, plus assessments for other parenchymal organ damage as patients become teens and adults. Treatment approaches that previously highlighted acute vaso-occlusive events are now evolving to the concept of preventive therapy. Liberalized use of blood transfusions and early consideration of hydroxyurea treatment represent a new treatment paradigm for SCA management.
镰状细胞贫血（SCA）的恰当管理始于在生命早期、理想情况下在新生儿期尽早确立正确的诊断。通过新生儿筛查计划确定受影响的婴儿，可以及早开始预防性青霉素和肺炎球菌免疫接种，这有助于预防严重败血症。持续向家庭提供教育，促进早期识别疾病相关并发症，从而可以及时进行适当的医学评估和治疗干预。由训练有素的专家进行定期评估有助于提供全面的护理，包括经颅多普勒检查以识别有发生原发性中风风险的儿童，以及随着患者成长为青少年和成年人评估其他实质器官损伤。以前强调急性血管阻塞性事件的治疗方法现在正在演变为预防性治疗的概念。放宽输血的使用和早期考虑羟基脲治疗代表了 SCA 管理的新治疗范例。
