Data Resource Profile: The UK Cystic Fibrosis Registry.
Data Resource Profile: The UK Cystic Fibrosis Registry.
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DOI：
10.1093/ije/dyx196
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发表时间：
2018-02-01
影响因子：
7.7
通讯作者：
CF-EpinNet collaboration
中科院分区：
文献类型：
--
作者：
Taylor-Robinson D;Archangelidi O;Carr SB;Cosgriff R;Gunn E;Keogh RH;MacDougall A;Newsome S;Schlüter DK;Stanojevic S;Bilton D;CF-EpinNet collaboration
英国囊性纤维化登记处是一个全国性的、安全的、集中的数据库，由囊性纤维化信托基金赞助和管理，并获得英国国家卫生服务（NHS）研究伦理批准和每个收集数据的人的同意。它于1995年首次建立，记录了英格兰、威尔士、苏格兰和北方爱尔兰所有囊性纤维化（CF）患者的纵向健康数据，迄今已收集了12000多人的数据。囊性纤维化是一种遗传性、慢性、进行性疾病，在英国每2500名活产婴儿中就有1例发生，每年约有200-300例新诊断。儿童通常在出生后的头几个月被诊断出来，2007年在英国实施了普遍的新生儿筛查，尽管有些人被诊断为成年人。例如，2015年在英国有29名16岁以上的人被诊断出患有CF。1确诊患有CF的患者随后需要家庭和医疗保健服务的强化支持。大多数患者因呼吸衰竭而过早死亡，在20世纪30年代和40年代，儿童期后存活的情况很少。[2]在随后的几十年里，生存率有了令人印象深刻的改善;例如，1990年出生的囊性纤维化儿童的平均预期寿命估计为40岁，是20年前估计的两倍。3在英国，患有CF的儿童在33个专科中心之一接受治疗（与100多个较小的网络诊所有关）。16至18岁的儿童转到27个成人专科中心之一。所有中心和网络诊所都以标准化方式定期收集数据。当CF患者在英国的一个新的CF中心就诊时，他们或他们的父母同意他们的健康和治疗信息被收集并存储在CF登记处。患者信息和同意书还涵盖了将登记数据与英国国家统计局联系起来的问题。当过渡到成人服务时，年轻人有机会确认或撤回同意。患有CF的人如果改变他们的初级护理中心也会重新同意。注册表记录有关健康和VC作者2017的信息。由牛津大学出版社代表国际流行病学协会出版9
The UK Cystic Fibrosis Registry is a national, secure, centralized database sponsored and managed by the Cystic Fibrosis Trust, with UK National Health Service (NHS) research ethics approval and consent from each person for whom data are collected. First established in 1995, it records longitudinal health data on all people with cystic fibrosis (CF) in England, Wales, Scotland and Northern Ireland, and to date has captured data on over 12 000 individuals. Cystic fibrosis is an inherited, chronic, progressive condition occurring in around 1 in 2500 live births in the UK, with around 200–300 new diagnoses annually. Children are generally diagnosed in the first few months of life with universal newborn screening being implemented in 2007 in the UK, though some people are diagnosed into adulthood. For instance, 29 people aged over 16 years were diagnosed with CF in the UK in 2015. 1 Patients diagnosed with CF subsequently require intensive support from family and health care services. Most patients die prematurely from their disease through respiratory failure, and in the 1930 s and 40 s survival beyond childhood was rare. 2 There have been impressive improvements in survival over subsequent decades; for instance, the median life expectancy of children with cystic fibrosis born in 1990 was estimated to be 40 years, double that of estimates 20 years earlier. 3 In the UK, children with CF are treated in one of 33 specialist centres (associated with over 100 smaller network clinics). At between 16 years and 18 years of age, children transfer to one of 27 adult specialist centres. All centres and network clinics routinely collect data in a standardized fashion. When patients with CF attend a new CF centre in the UK, they or their parents consent to information on their health and treatment being collected and stored in the CF Registry. The patient information and consent form also covers the issue of linking registry data to the UK Office for National Statistics. When transitioning to adult services, the young adult is given the opportunity to confirm or withdraw consent. People with CF will also reconsent if they change their primary centre of care. The Registry records information about the health andVC The Author 2017. Published by Oxford University Press on behalf of the International Epidemiological Association 9
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影响因子：
5.2
作者：
Stanojevic, Sanja;Stocks, Janet;Bilton, Diana
通讯作者：
Bilton, Diana
影响因子：
5.1
作者：
Sims, EJ;McCormick, J;Mehta, A
通讯作者：
Mehta, A
影响因子：
16.2
作者：
Adler AI;Shine BS;Chamnan P;Haworth CS;Bilton D
通讯作者：
Bilton D
影响因子：
24.3
作者：
Dodge, J. A.;Lewis, P. A.;Wilsher, J.
通讯作者：
Wilsher, J.
DOI：
10.1016/j.jcf.2005.01.001
发表时间：
2005-05-01
期刊：
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
影响因子：
--
作者：
McCormick, Jonathan;Sims, Erika J;Mehta, Anil
通讯作者：
Mehta, Anil