Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study

被引：16

作者：

Kouis, Panayiotis ^{[1
]}

Goutaki, Myrofora ^{[2
]}

Halbeisen, Florian S. ^{[2
]}

Gioti, Ifigeneia ^{[1
]}

Middleton, Nicos ^{[3
]}

Amirav, Israel ^{[4
,26
]}

Barbato, Angelo ^{[5
]}

Behan, Laura ^{[6
,7
]}

Boon, Mieke ^{[8
]}

Emiralioglu, Nagehan ^{[9
]}

Haarman, Eric G. ^{[10
]}

Karadag, Bulent ^{[11
]}

Koerner-Rettberg, Cordula ^{[12
]}

Lazor, Romain ^{[13
,14
]}

Loebinger, Michael R. ^{[15
]}

Maitre, Bernard ^{[16
]}

Mazurek, Henryk ^{[17
]}

Morgan, Lucy ^{[18
]}

Nielsen, Kim Gjerum ^{[19
]}

Omran, Heymut ^{[20
]}

Ozcelik, Ugur ^{[9
]}

Price, Mareike ^{[21
]}

Pogorzelski, Andrzej ^{[17
]}

Snijders, Deborah ^{[5
]}

Thouvenin, Guillaume ^{[22
]}

Werner, Claudius ^{[20
,27
]}

Zivkovic, Zorica ^{[23
,24
]}

Kuehni, Claudia E. ^{[2
]}

Yiallouros, Panayiotis K. ^{[1
,25
]}

机构：

[1] Univ Cyprus, Med Sch, Resp Physiol Lab, Nicosia, Cyprus

[2] Univ Bern, Inst Social & Prevent Med, Bern, Switzerland

[3] Cyprus Univ Technol, Sch Hlth Sci, Dept Nursing, Limassol, Cyprus

[4] Univ Alberta Edmonton, Dept Pediat, Edmonton, AB, Canada

[5] Univ Padua, Dept Womens & Childrens Hlth SDB, Primary Ciliary Dyskinesia Ctr, Padua, Italy

[6] NHS Fdn Trust, Univ Hosp Southampton, Primary Ciliary Dyskinesia Ctr, Southampton, Hants, England

[7] Univ Southampton, Southampton, Hants, England

[8] Univ Hosp Gasthuisberg Leuven, Dept Paediat & Paediat Pulmonol, Leuven, Belgium

[9] Hacettepe Univ, Pediat Pulmonol, Ankara, Turkey

[10] Vrije Univ, Emma Childrens Hosp, Dept Pediat Pulmonol, Amsterdam UMC, Amsterdam, Netherlands

[11] Marmara Univ, Sch Med, Dept Pediat Pulmonol, Istanbul, Turkey

[12] Ruhr Univ Bochum, Univ Childrens Hosp, Dept Paediat Pulmonol, Bochum, Germany

[13] Lausanne Univ Hosp, Dept Resp Med, Lausanne, Switzerland

[14] Natl Reference Ctr Rare Pulm Dis, Dept Resp Med, Lyon, France

[15] Royal Brompton & Harefield NHS Fdn Trust, Host Def Unit, London, England

[16] Univ Paris Est Crete, DHU ATVB, Serv Pneumol, Hop Intercommunal Crete, Paris, France

[17] Instytut Gruilicy & ChorobPluc, Klin Pneumonol & Mukowiscydozy, Rabka, Poland

[18] Univ Sydney, Concord Hosp Clin Sch, Dept Resp Med, Sydney, NSW, Australia

[19] Copenhagen Univ Hosp, Danish PCD Ctr Copenhagen, Paediat Pulm Serv, Copenhagen, Denmark

[20] Univ Hosp Muenster, Dept Gen Paediat & Adolescent Med, Munster, Germany

[21] Hannover Med Sch, Clin Paediat Pulmonol Allergiol & Neonatol, Hannover, Germany

[22] Sorbonne Univ, Hop Trousseau, AP HP, Serv Pneumol Pediat,INSERM,CRSA, Paris, France

[23] Med Ctr Dr Dragisa Misovic, Childrens Hosp Lung Dis & TB, Belgrade, Serbia

[24] Business Acad Novi Sad, Fac Pharm Novi Sad, Novi Sad, Serbia

[25] Shakolas Educ Ctr Clin Med, Palaios Dromos Lefkosias Lemesou 215-6, CY-2029 Nicosia, Cyprus

[26] Dana Dwek Childrens Hosp, Tel Aviv Med Ctr, Tel Aviv, Israel

[27] Helios Hosp Schwerin, Dept Pediat, Schwerin, Germany

来源：

RESPIRATORY RESEARCH | 2019年 / 20卷 / 01期

基金：

瑞士国家科学基金会;

关键词：

Kartagener syndrome (MeSH); Ciliary motility disorders (MeSH); Lobectomy; CYSTIC-FIBROSIS PATIENTS; MIDDLE LOBE SYNDROME; CHILDREN; MANAGEMENT; DIAGNOSIS; FEATURES;

D O I：

10.1186/s12931-019-1183-y

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

Background Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (- 2.41 vs - 1.35, p = 0.0001) and FEV1 z-scores (- 2.79 vs - 1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope: - 0.037/year Vs - 0.009/year, p = 0.047 and FEV1 z-score slope: - 0.052/year Vs - 0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.

引用

页数：12

共 33 条

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