The Clinical Features of Bronchiectasis Associated with Alpha-1 Antitrypsin Deficiency, Common Variable Immunodeficiency and Primary Ciliary Dyskinesia--Results from the U.S. Bronchiectasis Research Registry.
| Autor: | Eden E; Icahn School of Medicine Mt Sinai, New York, New York., Choate R; University of Kentucky College of Public Health, Lexington., Barker A; Oregon Health Sciences University Hospital, Portland., Addrizzo-Harris D; New York University School of Medicine, New York., Aksamit TR; Pulmonary Disease and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota., Daley CL; Division of Mycobacterial and Respiratory Infections, National Jewish Health, Denver, Colorado., Daniels MLA; University of North Carolina at Chapel Hill., DiMango A; Columbia College of Physicians and Surgeons, Center for Chest Disease, New York, New York., Fennelly K; National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland., Griffith DE; University of Texas at Tyler., Johnson MM; Mayo Clinic Florida, Pulmonary and Critical Care, Jacksonville., Knowles MR; University of North Carolina at Chapel Hill., Metersky ML; Division of Pulmonary and Critical Care Medicine, University of Connecticut School of Medicine, Farmington., Noone PG; University of North Carolina at Chapel Hill., O'Donnell AE; Georgetown University Medical Center, Washington, DC., Olivier KN; National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland., Salathe MA; University of Kansas Medical School, Kansas City., Schmid A; University of Miami Miller School of Medicine, Miami, Florida., Thomashow B; Columbia College of Physicians and Surgeons, Center for Chest Disease, New York, New York., Tino G; University of Pennsylvania Perelman School of Medicine, Philadelphia., Turino GM; Icahn School of Medicine Mt Sinai, New York, New York., Winthrop KL; Oregon Health Sciences University Hospital, Portland.; Department of Infectious Disease, Oregon Health and Science University School of Medicine, Portland. |
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| Jazyk: | angličtina |
| Zdroj: | Chronic obstructive pulmonary diseases (Miami, Fla.) [Chronic Obstr Pulm Dis] 2019 Apr 09; Vol. 6 (2), pp. 145-153. Date of Electronic Publication: 2019 Apr 09. |
| DOI: | 10.15326/jcopdf.6.2.2018.0156 |
| Abstrakt: | Objective: This study compares and contrasts the clinical features of non-cystic fibrosis bronchiectasis with 3 uncommon disorders known to be associated with bronchiectasis but with distinctly different underlying defined pathophysiologic derangements, namely severe alpha-1 antitrypsin deficiency (AATD), common variable immunodeficiency (CVI) and primary ciliary dyskinesia (PCD). Methods: The Bronchiectasis Research Registry provides a central database for studying patients with non-cystic fibrosis bronchiectasis. This report consists of information from 13 U.S. sites pertaining to the 3 study diagnoses. Patients with AATD (SZ and ZZ phenotypes only), CVI (patients with IgG≤500), PCD (history of physician diagnosed Kartagener's syndrome or PCD), and patients with confirmed absence of the above 3 diagnoses (idiopathic control group) were included in the study. Descriptive statistics were computed for the main demographic and clinical characteristics of the sample stratified by group. Values between the groups were compared using Kruskal-Wallis test, and Chi-squared/ Fisher's exact tests respectively. The significance level was set at 0.05. Software SAS 9.4 was used to perform the statistical analyses. Results: Of the 2170 participants in the database enrolled as of January 2017, 615 respondents had sufficient data and were included in the analyses. Patients with PCD (n=79, mean age 41.9 years [standard deviation (SD)=14.5]) were significantly younger than patients with AATD (n=58, mean age 66.9 [SD=10.7]), CVI (n=18, mean age 66.7 years [SD=10.5]) or the idiopathic group (n=460, mean age 64.2 [SD=15.9]), p <.0001. Compared to other groups, those with PCD had lower pulmonary function (forced expiratory volume in 1 second [FEV Conclusion: This report from the U.S. Bronchiectasis Research Registry compares and contrasts differences in the clinical features of patients suffering from 3 rare conditions, with different underlying causes, to those without. The group with PCD had more symptoms, greater morbidity, lower lung function and more commonly were infected by Pseudomonas aeruginosa . A greater percentage of those with AATD reported mycobacterial lung involvement. Competing Interests: Kenneth Olivier is supported in part by the Division of Intramural Research, National Heart, Lung and Blood Institute, National Institutes of Health. Doreen Addrizzo-Harris reports personal fees from AIT Therapeutics and Insmed. Alan Barker is supported in part by a grant provided by the COPD Foundation. Charles Daley is supported in part by a grant provided by the COPD Foundation. Leigh Anne Daniels reports personal fees from Insmed and Spark Partners as well as grants from Zambon and Parion/Vertex. Mark Metersky is supported in part by a grant provided by the COPD Foundation. Anne O’Donnell is supported in part by a grant provided by the COPD Foundation. Matthias Salathe is supported in part by a grant provided by the COPD Foundation. Byron Thomashow reports personal fees from Boehringer Ingelheim, GlaxoSmithKline, and AstraZeneca. Additionally, Byron Thomashow helped co-found the COPD Foundation and served as Board Chairman for the COPD Foundation for several years. Gregory Tino reports personal fees from Bayer, Grifols, Aradigm, and Cipla. Additionally, Gregory Tino is supported in part by a grant provided by the COPD Foundation. Kevin Winthrop reports grants and personal fees from Insmed and Bayer. All other authors have nothing to declare. (JCOPDF © 2019.) |
| Databáze: | MEDLINE |
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