Williams-Campbell 2-3 Marfan's syndrome / % ~ 48% common variable immunodeficiency CVID X- X-linked agammaglobulinemia XLA lga - IgA Ig

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1 COPD [1-2] 2010 [3-4] [5] 3.7 / 10 [6] 52 / 10 [5] 100 / ~ / / 10 [7] CT 50 CT - COPD 15% ~ 30% COPD 50% [11] DOI: / cma. j. issn

2 Williams-Campbell 2-3 Marfan's syndrome / % ~ 48% common variable immunodeficiency CVID X- X-linked agammaglobulinemia XLA lga - IgA IgG acquired immune deficiency syndrome AIDS 40% primary ciliary dyskinesia PCD Kartagener Young's syndrome 2 60% ~ 80% CD4 +

3 317 IL-16 IL-8 IL-10 -α tumor necrosis factor-α TNF-α allergic hronchopulmonary aspergillosis ABPA

4 %~ 69% [A] 6% ~ 48% CVID XLA IgA ABPA [12] % ~ 5.2% CT

5 319 59% α1- α1-1. > 90% 75% ~ 100% 72% ~ 83% FEV 1 CT X IgG IgA IgM CT CT IgE 1. 1 X X X

6 320 X X X 2 CT CT 0.62 ± 0.13 < 80% CT CT 4 CT CT CT ABPA CT CT CT 3 CT 2. 1 ESR C 2 IgG IgA IgM [A] 3 IgE ABPA 4 5 ABPA l h 3 6 anti-neutrophil

7 321 cytoplasmic antibody ANCA IgE IgG B CT 2 FEV 1 FVC 1 4 > 80% 33% ~ 76% FEV 1 40% FEV 1 FVC 24 h

8 CT CT COPD COPD 2 COPD COPD 2 X CT FEV 1 / FVC < 70%) FEV 1

9 323 CT 1 [B] CT 4 1 ~ 2 h 2 3 [A] 3 [13] 4 β2- [B] 5 min FEV 1 5 [A] ~ 2 20 ~ 30 min [B]

10 324 60% ~ 80% [14-15] [15] [ FEV < 30% 4 2 > ] [2] 5 5 / / + β- / / 14 d ml 24 h 500 ml

11 ~ 5 min 20 ~ 30 min 5 ~ 10 U 5% 20 ~ 40 ml 15 min 10 ~ 20 U 5% 500 ml 0.1 U kg -1 h -1 2 ~ 3 d 2 4 ~ 6 g ml 15 ~ 30 min 1 g / h 100 ~ 200 mg 5% 40 ml 2 / d 250 ~ 500 mg 2 ~ 3 / d l ~ 2 ku 5 ~ 10 min 24 h mg 30 ml l ~ 2 / d 0.25% 0.1 ml 5 ~ 10 mg 20 ~ 40 ml 10 ~ 20 mg 500 ml % 1 70% [16] 2 93% 1 51% 2 39% [17] 34.5% DNA [A] 2. β2-3.

12 326 [B] % ~ 19% < 5% RCT 1+ RCT 1- RCT A B C 1++ RCT D A B 500 mg / kg [18]

13 Woodhead M, Blasi F, Ewig S, et al. European Respiratory Society; European Society of Clinical Microbiology and Infectious Diseases. Guidelines for the management of adult lower respiratory tract infections. Eur Respir J. 2005, 26 (6): Woodhead M, Blasi F, Ewig S, et al. Guidelines for the management of adult lower respiratory tract infections-full version. Clin Microbiol Infect. 2011, 17 Suppl 6: E1-E59. 3 Pasteur MC, Bilton D, Hill AT, et al. British Thoracic Society guideline for non-cf bronchiectasis. Thorax. 2010, 65 Suppl 1: i1-i Weycker D, Edelsberg J, Oster G, et al. Prevalence and economic burden of bronchiectasis. Am J Respir Crit Care Med, 2004, 169: A Twiss J, Metcalfe R, Edwards E, et al. New Zealand national incidence of bronchiectasis "too high" for a developed country. Arch Dis Child, 2005, 90 (7): Weycker D, Edelsberg J, Oster G, et al. Prevalence and economic burden of bronchiectasis. Clin

14 328 Pulmon Med, 2005, 12: Crofton J. Bronchiectasis. In: Crofton J, Douglas A, eds. Respiratory diseases. 3 rd eds. Oxford: Blackwell Scientific, 1981: Patel IS, Vlahos I, Wilkinson TM, et al.. Bronchiectasis, exacerbation indices, and inflammation in chronic obstructive pulmonary disease. Am J Respir Crit Care Med, 2004, 70 (4): O'Brien C, Guest PJ, Hill SL, et al. Physiological and radiological characterisation of patients diagnosed with chronic obstructive pulmonary disease in primary care. Thorax, 2000, 55 (8): King PT. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis, 2009, 4: Pang JA, Cheng A, Chan HS, et al. The bacteriology of bronchiectasis in Hong Kong investigated by protected catheter brush and bronchoalveolar lavage. Am Rev Respir Dis, 1989, 139 (1): Angrill J, Agustí C, de Celis R, et al. Bacterial colonisation in patients with bronchiectasis: microbiological pattern and risk factors. Thorax, 2002, 57: Anuradha C, Shyamkumar NK, Vinu M, et al. Outcomes of bronchial artery embolization for lifethreatening hemoptysis due to tuberculosis and post-tuberculosis sequelae. Diagn Interv Radiol, 2012, 18(1): Samara KD, Tsetis D, Antoniou KM, et al. Bronchial artery embolization for management of massive cryptogenic hemoptysis: a case series. J Med Case Rep, 2011, 5: Chang CC, Singleton RJ, Morris PS, et al. Pneumococcal vaccines for children and adults with bronchiectasis. Cochrane Database Syst Rev. 2009, 2: CD [ J/ CD] ( 5)

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untitled Miles Weinberger, MD and Mutasim Abu-Hasan Pediatric Allergy and Pulmonary Division, Department of Pediatrics, University of Iowa, Iowa City, Iowa (asthma); (cough); (wheezing); (dyspnea) Address correspondence