尼日尔儿童镰状细胞病380例临床研究

The clinical study of 380 children with sickle cell disease in Niger

目的探讨非洲儿童镰状细胞病的临床诊断和治疗。方法收集2000年11月至2002年11月在尼日尔马腊迪省住院中心住院,经血液学确诊的380例镰状细胞病进行临床分析。结果贫血和疼痛危象为最常见的临床表现,骨关节疼痛占50%为首位,其次为胸腹疼痛占40%;并发急性胸部综合征(ACS)占70%,是病情恶化的证据和各年龄段死亡的主要原因。有关节血肿者采用外科放血术可降低和预防骨痛危象的发生。结论随着国际交往增多和跨国婚配的增加,提高临床医生对本病的认识具有现实意义。

Objective To study the clinical diagnosis and treatment of drepanocytosis in African children. Methods To clinically diagnose the 380 cases who were identified as drepancocytosis by blood test （differentiation） and thus accepted clinical treatment in the Inpatient Center of Maradi of Niger. Results Anemia and painful crisis are the most common clinical manifestations;the cases appearing pains in joints of bones count for 50%, and the cases appearing pains in berry and chest count for 40%. Besides, the cases of ACS take up 70%, which is a proof of becoming worse and the main reason for the death of various ages. Bloodletting by surgery can decrease and prevent the cases of painful crisis for those who appear haematoma in the joints of bones. Conclusion With the increase in international communication and marriage, it is of significance to arouse the awareness of this disease in doctors.