Cystic fibrosis mortality and survival in the UK: 1947-2003
Cystic fibrosis mortality and survival in the UK: 1947-2003
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DOI：
10.1183/09031936.00099506
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发表时间：
2007-03-01
影响因子：
24.3
通讯作者：
Wilsher, J.
中科院分区：
文献类型：
--
作者：
Dodge, J. A.;Lewis, P. A.;Wilsher, J.
截至1995年，在1968-1992年期间出生在英国的囊性纤维化（CF）患者的3年生存率数据已经发表。目前的研究报告了截至2003年底的生存数据以及2003年的人口估计。所有在1968-1992年期间出生在英国的CF患者都是通过认可的CIF诊所和其他医院顾问积极询问确定的，直到1997年。补充了截至2003年底死亡证明当局提供的资料。无法与英国囊性纤维化调查记录相匹配的死亡证明进行了调查并核对了数据。1978年出生的CF患者到2003年的观察生存率为男性55%，女性49%。1988年和1992年的数据分别为91%和88%，97%和96%。估计2003年中CIF人口为8,284人。囊性纤维化患者在连续队列中生存的持续改善意味着，即使没有针对纠正基本囊性纤维化缺陷的有效治疗，先前对2000年出生的个体的中位生存期为50岁的预测仍然是现实的。
Data up to 1995 on the survival of 3-yr cohorts of patients with cystic fibrosis (CF) born in the UK in the period 1968-1992 have previously been published. The present study reports survival data up to the end of 2003 together with a 2003 population estimate.All subjects with CF born in the UK in the period 1968-1992 were identified up to 1997 by active enquiry through recognised CIF clinics and other hospital consultants. Information from the death certification authorities up to the end of 2003 was added. Death certificates that could not be matched with UK Cystic Fibrosis Survey records were investigated and the data reconciled.The observed survival up to 2003 of CF patients born in 1978 was 55% for males and 49% for females. For 1988 and 1992 the data were 91 and 88%, and 97 and 96%, respectively. The estimated 2003 mid-year CIF population was 8,284.The continuing improvement in survival of cystic fibrosis patients in successive cohorts means that the previous prediction of median survival of > 50 yrs of age for individuals born in 2000 continues to look realistic, even in the absence of proven effective therapy aimed at correcting the basic cystic fibrosis defect.