Davis L R, Huehns E R, White J M
Br Med J (Clin Res Ed). 1981 Dec 5;283(6305):1519-21. doi: 10.1136/bmj.283.6305.1519.
The incidence and the clinical course of patients suffering from sickle-cell syndrome (Hb SS; Hb SC; Hb S thal) in England and Wales are not known. In 1979 an ad hoc committee was formed to investigate these problems. Initially, a questionnaire was sent to 227 haematologists in England and Wales to determine the number of cases in these countries. The replies have indicated that 1367 cases were seen in 1978 and 1979. Probably this may represent only half the total number of cases. From this survey it has been possible to draw up a composite map showing the location of patients, which has provided a basis to determine the clinical course of the disease, and for further studies into the complications and management of sickle-cell disease in England and Wales. From a second questionnaire preliminary data about the general management and mortality in England and Wales have been recorded.
镰状细胞综合征(血红蛋白SS型;血红蛋白SC型;血红蛋白S与地中海贫血型)在英格兰和威尔士的发病率及临床病程尚不清楚。1979年成立了一个特别委员会来调查这些问题。最初,向英格兰和威尔士的227位血液学家发送了一份调查问卷,以确定这些国家的病例数量。答复表明,1978年和1979年共发现1367例病例。这可能仅占病例总数的一半。通过这项调查,已绘制出一张综合地图,显示了患者的分布位置,这为确定该病的临床病程以及进一步研究英格兰和威尔士镰状细胞病的并发症及治疗方法提供了依据。通过第二份调查问卷,记录了英格兰和威尔士有关总体治疗及死亡率的初步数据。