Diagnosis, Monitoring, and Treatment of Primary Ciliary Dyskinesia: PCD Foundation Consensus Recommendations Based on State of the Art Review

被引：287

作者：

Shapiro, Adam J. ^{[1
]}

Zariwala, Maimoona A. ^{[2
]}

Ferkol, Thomas ^{[3
]}

Davis, Stephanie D.

Sagel, Scott D. ^{[4
,5
,6
]}

Dell, Sharon D. ^{[7
,8
]}

Rosenfeld, Margaret ^{[9
,10
]}

Olivier, Kenneth N. ^{[11
]}

Milla, Carlos ^{[12
]}

Daniel, Sam J. ^{[13
]}

Kimple, Adam J. ^{[14
]}

Manion, Michele ^{[15
]}

Knowles, Michael R. ^{[16
]}

Leigh, Margaret W. ^{[17
]}

机构：

[1] McGill Univ, Montreal Childrens Hosp, Dept Pediat, 2300 Tupper,D-380, Montreal, PQ H3H 1P3, Canada

[2] Univ N Carolina, Sch Med, Marsico Lung Inst, Dept Pathol & Lab Med, Chapel Hill, NC USA

[3] Washington Univ, Sch Med, Dept Pediat, St Louis, MO 63110 USA

[4] Indiana Univ, James Whitcomb Riley Hosp Children, Dept Pediat, Indianapolis, IN 46204 USA

[5] Childrens Hosp Colorado, Dept Pediat, Aurora, CO USA

[6] Univ Colorado, Sch Med, Aurora, CO USA

[7] Hosp Sick Children, Dept Pediat, Toronto, ON M5G 1X8, Canada

[8] Univ Toronto, Toronto, ON, Canada

[9] Seattle Childrens Hosp, Dept Pediat, Seattle, WA USA

[10] Univ Washington, Seattle, WA 98195 USA

[11] NHLBI, Bldg 10, Bethesda, MD 20892 USA

[12] Stanford Univ, Dept Pediat, Palo Alto, CA 94304 USA

[13] McGill Univ, Montreal Childrens Hosp, Dept Otolaryngol, Montreal, PQ H3H 1P3, Canada

[14] Univ N Carolina, Sch Med, Dept Otolaryngol Head & Neck Surg, Chapel Hill, NC USA

[15] PCD Fdn, Minneapolis, MN USA

[16] Univ N Carolina, Dept Med, Marsico Lung Inst, Chapel Hill, NC USA

[17] Univ N Carolina, Dept Pediat, Marsico Lung Inst, Chapel Hill, NC USA

来源：

PEDIATRIC PULMONOLOGY | 2016年 / 51卷 / 02期

基金：

美国国家卫生研究院;

关键词：

primary ciliary dyskinesia; PCD; kartagener; consensus statement; PCD Foundation; NASAL NITRIC-OXIDE; CYSTIC-FIBROSIS BRONCHIECTASIS; MUCOCILIARY CLEARANCE DISORDER; SITUS-INVERSUS TOTALIS; LONG-TERM; LUNG-FUNCTION; IMMOTILE CILIA; OTITIS-MEDIA; PULMONARY EXACERBATIONS; INHALED ANTIBIOTICS;

D O I：

10.1002/ppul.23304

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, rare lung disease resulting in chronic oto-sino-pulmonary disease in both children and adults. Many physicians incorrectly diagnose PCD or eliminate PCD from their differential diagnosis due to inexperience with diagnostic testing methods. Thus far, all therapies used for PCD are unproven through large clinical trials. This review article outlines consensus recommendations from PCD physicians in North America who have been engaged in a PCD centered research consortium for the last 10 years. These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long-term therapeutics in PCD patients. (C) 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc.

引用

页码：115 / 132

页数：18

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