Overcoming challenges in the management of primary ciliary dyskinesia: The UK model

被引：23

作者：

Lucas, Jane S. ^{[1
,2
]}

Chetcuti, Philip ^{[3
]}

Copeland, Fiona

Hogg, Claire ^{[4
]}

Kenny, Tom ^{[5
]}

Moya, Eduardo ^{[6
]}

O'Callaghan, Christopher ^{[7
,8
]}

Walker, Woolf T. ^{[1
,2
]}

机构：

[1] Univ Hosp Southampton NHS Fdn Trust, Primary Ciliary Dyskinesia Ctr, Southampton NIHR Resp Biomed Res Unit, Southampton, Hants, England

[2] Univ Southampton, Fac Med, Clin & Expt Sci Acad Unit, Southampton, Hants, England

[3] Leeds Childrens Hosp, Leeds, W Yorkshire, England

[4] Royal Brompton & Harefield Fdn Trust, Primary Ciliary Dyskinesia Ctr, Dept Paediat, London, England

[5] Natl Specialised Commissioning Team, London, England

[6] Bradford Royal Infirm, Div Serv Women & Children, Womens & Newborn Unit, Bradford BD9 6RJ, W Yorkshire, England

[7] UCL, Inst Child Hlth, Dept Resp Med, Portex Unit, London, England

[8] Leicester Royal Infirm, Primary Ciliary Dyskinesia Ctr, Leicester, Leics, England

来源：

PAEDIATRIC RESPIRATORY REVIEWS | 2014年 / 15卷 / 02期

关键词：

Primary ciliary dyskinesia; Co-ordinated care; Multi-disciplinary team; Equity of access; LUNG-FUNCTION; DIAGNOSIS;

D O I：

10.1016/j.prrv.2013.04.007

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Primary ciliary dyskinesia (PCD) is an autosomal recessive disease associated with bronchiectasis, chronic rhinosinusitis, infertility and situs inversus. Estimates of prevalence vary widely, but is probably between 1:10,000- 1:40,000 in most populations. A number of observational studies indicate that access to services to diagnose and manage patients with PCD vary both between and within countries. Diagnosis is often delayed and frequently missed completely. The prognosis of patients with PCD is variable, but evidence suggests that it is improved by early diagnosis and specialist care. This article briefly reviews the literature concerning PCD and the evidence that specialist care will improve healthcare outcomes. The article specifically refers to a new national service in the UK. (C) 2013 Elsevier Ltd. All rights reserved.

引用

页码：142 / 145

页数：4

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