Murtaza L N, Stroud C E, Davis L R, Cooper D J
Br Med J (Clin Res Ed). 1981 Mar 28;282(6269):1048-51. doi: 10.1136/bmj.282.6269.1048.
Admissions to hospital of 171 children with sickle-cell anaemia, genotype Hb SS, were reviewed over a 20-year period. Altogether 887 admissions occurred in 797 patient-years. The commonest cause of admission was painful vaso-occlusive crisis. Appreciable morbidity also resulted from pulmonary disease, infection, and anaemic episodes. The complications resulting in the most severe illness were acute splenic sequestration, pneumococcal meningitis, and some episodes of erythroid hypoplasia resulting in very low haemoglobin concentrations. Most deaths occurred in children aged under 5. Mortality and morbidity could be reduced by measures including prophylaxis of pneumococcal infections and more active treatment of seemingly minor illness in children with sickle-cell anaemia.
对171例基因型为Hb SS的镰状细胞贫血患儿在20年期间的住院情况进行了回顾。在797个患者年中共发生了887次住院。最常见的住院原因是疼痛性血管闭塞危象。肺部疾病、感染和贫血发作也导致了明显的发病率。导致最严重疾病的并发症是急性脾滞留、肺炎球菌脑膜炎以及一些导致血红蛋白浓度极低的红细胞生成低下发作。大多数死亡发生在5岁以下的儿童中。通过包括预防肺炎球菌感染以及对镰状细胞贫血患儿看似轻微的疾病进行更积极治疗等措施,可以降低死亡率和发病率。