BMJ. 1988 Dec 17;297(6663):1599-602. doi: 10.1136/bmj.297.6663.1599.
A national survey was conducted of patients with cystic fibrosis who were known to paediatricians, chest physicians, and others or whose deaths were reported through the death certification authorities in the United Kingdom during 1977 to 1985. From this population based study a revised incidence figure of one affected baby in 2500 live births was produced. Mortality was very high in the first year of life (7.6%) and was substantially greater for females than for males under age 20 years. A temporal improvement in mortality was found during the period under study, with about 100 more births than deaths occurring each year. This improvement was notable in the first five years of life. Meconium ileus, which used to be a primary cause of early mortality, is becoming increasingly rare as a cause of death. The total prevalence of cystic fibrosis in the UK in mid-1985 was estimated to be about 5000.
对1977年至1985年间英国儿科医生、胸科医生及其他人员所知晓的囊性纤维化患者，或通过死亡证明机构报告其死亡情况的患者进行了一项全国性调查。基于这项人群研究，得出了一个修订后的发病率数据，即每2500例活产中有1例患病婴儿。一岁以内的死亡率非常高（7.6%），20岁以下女性的死亡率明显高于男性。在研究期间发现死亡率有时间上的改善，每年出生人数比死亡人数多约100人。这种改善在生命的头五年尤为显著。胎粪性肠梗阻过去是早期死亡的主要原因，现在作为死亡原因正变得越来越罕见。据估计，1985年年中英国囊性纤维化的总患病率约为5000人。
