Effect of a comprehensive clinical care program on disease course in severely in children with sickle cell anemia in a sub-Saharan African setting
Effect of a comprehensive clinical care program on disease course in severely in children with sickle cell anemia in a sub-Saharan African setting
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DOI：
10.1182/blood-2002-05-1453
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发表时间：
2003-08-01
期刊：
影响因子：
20.3
通讯作者：
Alihonou, E
中科院分区：
文献类型：
--
作者：
Rahimy, MC;Gangbo, A;Alihonou, E
镰状细胞性贫血（SS）在非洲的临床严重程度可能不仅仅是由遗传因素决定的。本研究评估了贝宁儿童SS过程中强化父母教育和充分的临床护理的效果。研究纳入了转诊至科托努国家教学医院的党卫军儿童。关于SS的教学经常重复，强调保持门诊预约的重要性，改善受影响儿童的营养，并制定抗肺炎球菌和抗疟疾预防措施。SS相关事件的频率和严重程度、身体生长的变化、疟疾发作的频率、输血原因和死亡原因是评估的主要变量。从1993年7月1日到1999年12月31日（983患者年），对236名患有反复SS相关急性并发症的幼儿进行了研究。观察到SS相关急性事件的频率和严重程度显著降低。在184例患者（78%）中观察到一般状况和身体发育改善;此外，其余52例患者中的22例在重新激励父母依从性后显示出类似的改善。有10例死亡，主要发生在该队列的52例患者中。在撒哈拉以南非洲，密集的社会医学干预可以使许多严重患病的党卫军儿童的临床状况得到持续改善。
Clinical severity of sickle cell anemia (SS) in Africa may not be solely determined by genetic factors. This study evaluated the effects of intensive parental education and adequate clinical care on the course of SS in children in Benin. SS children referred to the National Teaching Hospital in Cotonou were included in the study. Teaching about SS was repeated frequently, emphasizing the importance of keeping clinic appointments, improving the nutrition of the affected children, and instituting antipneumococcal and antimalarial prophylaxis. Frequency and severity of SS-related events, changes in physical growth, frequency of malarial attacks, causes of transfusion, and causes of death were the principal variables assessed. 236 young children with repeated SS-related acute complications were studied from July 1, 1993, to December 31, 1999 (983 patient-years). A marked reduction in the frequency and severity of SS-related acute events was observed. Improvement in general status and physical growth was noted in 184 patients (78%); in addition, 22 of the remaining 52 patients showed Similar improvement after remotivating the parents for compliance. There were 10 deaths, primarily in this cohort of 52 patients. Intensive sociomedical intervention can produce sustained clinical improvement in many severely ill SS children in sub-Saharan Africa.