囊性纤维化

Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation consensus panel. J Pediatr. 1998;132:589-595.

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Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187:680-689.

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Flume PA, Robinson KA, O'Sullivan BP, et al. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009;54:522-537.

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Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176:957-969.

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Saiman L, Anstead M, Mayer-Hamblett N, et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2010;303:1707-1715.

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1.  Cystic fibrosis mutation database. April 2011. http://www.genet.sickkids.on.ca/ (last accessed 31 August 2016).

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2.  Kerem E. Atypical CF and CF related diseases. Paediatr Respir Rev. 2006;7(suppl 1):S144-S146.

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3.  Borowitz D, Robinson KA, Rosenfeld M, et al; Cystic Fibrosis Foundation. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155(suppl 6):S73-S93.

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4.  Orenstein DM, Rosenstein BJ, Stern RC. Cystic fibrosis: medical care. Philadelphia, PA: Lippincott Williams & Wilkins; 2000:365.

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6.  The Cystic Fibrosis Foundation. http://www.cff.org/ (last accessed 31 August 2016).

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7.  Eigen H, Rosenstein BJ, FitzSimmons S, et al. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr. 1995;126:515-523.

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8.  Hale JE, Parad RB, Comeau AM. Newborn screening showing decreasing incidence of cystic fibrosis. N Engl J Med. 2008;358:973-974.

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9.  Tarran R, Button B, Picher M, et al. Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections. J Biol Chem. 2005;280:35751-35759.

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10.  Moskwa P, Lorentzen D, Excoffon KJ, et al. A novel host defense system of airways is defective in cystic fibrosis. Am J Respir Crit Care Med. 2007;175:174-183.

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11.  Matsui H, Grubb BR, Tarran R, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell. 1998;95:1005-1015.

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12.  Knowles MR, Stutts MJ, Spock A, et al. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science. 1983;221:1067-1070.

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13.  Knowles M, Gatzy J, Boucher R. Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med. 1981;305:1489-1495.

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14.  Joo NS, Irokawa T, Robbins RC, et al. Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands. J Biol Chem. 2006;281:7392-7398.

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15.  Conner GE, Wijkstrom-Frei C, Randell SH, et al. The lactoperoxidase system links anion transport to host defense in cystic fibrosis. FEBS Lett. 2007;581:271-278.

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16.  Boucher RC. New concepts of the pathogenesis of cystic fibrosis lung disease. Eur Respir J. 2004;23:146-158.

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17.  Bals R, Weiner DJ, Meegalla RL, et al. Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid. Am J Respir Cell Mol Biol. 2001;25:21-25.

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18.  Ballard ST, Trout L, Mehta A, et al. Liquid secretion inhibitors reduce mucociliary transport in glandular airways. Am J Physiol Lung Cell Mol Physiol. 2002;283:L329-L335.

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19.  Rosenstein BJ, Cutting GR. The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation consensus panel. J Pediatr. 1998;132:589-595.

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20.  Comeau AM, Accurso FJ, White TB, et al. Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics. 2007;119:e495-e518.

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21.  Castellani C, Southern KW, Brownlee K, et al. European best practice guidelines for cystic fibrosis neonatal screening. J Cyst Fibros. 2009;8:153-173.

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22.  Sermet-Gaudelus I, Munck A, Rota M, et al. French guidelines for sweat test practice and interpretation for cystic fibrosis neonatal screening [in French]. Arch Pediatr. 2010;17:1349-1358.

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23.  Sermet-Gaudelus I, Mayell SJ, Southern KW, et al; European Cystic Fibrosis Society, Neonatal Screening Working Group. Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening. J Cyst Fibros. 2010;9:323-329.

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24.  Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al; Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187:680-689.

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25.  Mckoy NA, Wilson LM, Saldanha IJ, et al. Active cycle of breathing technique for cystic fibrosis. Cochrane Database Syst Rev. 2016;(7):CD007862.

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26.  Flume PA, Robinson KA, O'Sullivan BP, et al. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009;54:522-537.

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27.  Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176:957-969.

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28.  Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. 2006;354:229-240.

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29.  Wark P, McDonald VM. Nebulised hypertonic saline for cystic fibrosis. Cochrane Database Syst Rev. 2009;(2):CD001506.

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30.  Aitken ML, Bellon G, De Boeck K, et al; CF302 Investigators. Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study. Am J Respir Crit Care Med. 2012;185:645-652.

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31.  Balfour-Lynn IM, Welch K. Inhaled corticosteroids for cystic fibrosis. Cochrane Database Syst Rev. 2014;(10):CD001915.

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32.  Dovey M, Aitken ML, Emerson J, et al. Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study. Chest. 2007;132:1212-1218.

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33.  Auerbach HS, Williams M, Kirkpatrick JA, et al. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet. 1985;2:686-688.

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34.  Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2003;290:1749-1756.

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35.  Fennell PB, Quante J, Wilson K, et al. Use of high-dose ibuprofen in a pediatric cystic fibrosis center. J Cyst Fibros. 2007;6:153-158.

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36.  Lands LC, Stanojevic S. Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis. Cochrane Database Syst Rev. 2016;(4):CD001505.

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37.  Ramsey BW, Davies J, McElvaney NG, et al; VX08-770-102 Study Group. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365:1663-1672.

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38.  De Boeck K, Munck A, Walker S, et al. Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation. J Cyst Fibros. 2014;13:674-680.

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39.  Wainwright CE, Elborn JS, Ramsey BW, et al; TRAFFIC Study Group; TRANSPORT Study Group. Lumacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR. N Engl J Med. 2015;373:220-31.

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40.  Douglas TA, Brennan S, Gard S, et al. Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis. Eur Respir J. 2009;33:305-311.

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41.  Plummer A, Wildman M. Duration of intravenous antibiotic therapy in people with cystic fibrosis. Cochrane Database Syst Rev. 2013;(5):CD006682.

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42.  Smyth AR, Bhatt J. Once-daily versus multiple-daily dosing with intravenous aminoglycosides for cystic fibrosis. Cochrane Database Syst Rev. 2014;(2):CD002009.

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43.  Aaron SD, Vandemheen KL, Ferris W, et al. Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria: a randomised, double-blind, controlled clinical trial. Lancet. 2005;366:463-471.

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44.  Colombo C. Liver disease in cystic fibrosis. Curr Opin Pulm Med. 2007;13:529-536.

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45.  Shidrawi RG, Murugan N, Westaby D, et al. Emergency colonoscopy for distal intestinal obstruction syndrome in cystic fibrosis patients. Gut. 2002;51:285-286.

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46.  Holmes M, Murphy V, Taylor M, et al. Intussusception in cystic fibrosis. Arch Dis Child. 1991;66:726-727.

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47.  Saiman L, Anstead M, Mayer-Hamblett N, et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2010;303:1707-1715.

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48.  Southern KW, Barker PM, Solis-Moya A, et al. Macrolide antibiotics for cystic fibrosis. Cochrane Database Syst Rev. 2012;(11):CD002203.

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49.  Ratjen F, Saiman L, Mayer-Hamblett N, et al. Effect of azithromycin on systemic markers of inflammation in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa. Chest. 2012;142:1259-1266.

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50.  Lee TW, Southern KW. Topical cystic fibrosis transmembrane conductance regulator gene replacement for cystic fibrosis-related lung disease. Cochrane Database Syst Rev. 2016;(6):CD005599.

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51.  Stalvey MS, Anbar RD, Konstan MW, et al. A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatr Pulmonol. 2012;47:252-263.

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52.  Phung OJ, Coleman CI, Baker EL, et al. Recombinant human growth hormone in the treatment of patients with cystic fibrosis. Pediatrics. 2010;126:e1211-e1226.

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53.  Schuster A, Haliburn C, Döring G, et al; Freedom Study Group. Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in patients with cystic fibrosis: a randomised study. Thorax. 2013;68:344-350.

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54.  Geller DE, Flume PA, Staab D, et al; Mpex 204 Study Group. Levofloxacin inhalation solution (MP-376) in patients with cystic fibrosis with Pseudomonas aeruginosa. Am J Respir Crit Care Med. 2011;183:1510-1516.

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55.  Amin R, Noone PG, Ratjen F. Chemical pleurodesis versus surgical intervention for persistent and recurrent pneumothoraces in cystic fibrosis. Cochrane Database Syst Rev. 2012;(12):CD007481.

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56.  Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med. 2000;162:891-895.

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57.  Peterson ML, Jacobs DR Jr, Milla CE. Longitudinal changes in growth parameters are correlated with changes in pulmonary function in children with cystic fibrosis. Pediatrics. 2003;112:588-592.

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58.  Moran A, Pillay K, Becker DJ, et al; International Society for Pediatric and Adolescent Diabetes. ISPAD Clinical Practice Consensus Guidelines 2014. Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2014;15(suppl 20):65-76.

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59.  Beer H, Southern KW, Swift AC. Topical nasal steroids for treating nasal polyposis in people with cystic fibrosis. Cochrane Database Syst Rev. 2015;(6):CD008253.

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60.  Tangpricha V, Kelly A, Stephenson A, et al; Cystic Fibrosis Foundation Vitamin D Evidence-Based Review Committee. An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab. 2012;97:1082-1093.

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61.  Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2014;(3):CD002010.

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62.  Konstan MW, Butler SM, Wohl ME, et al. Investigators and coordinators of the epidemiologic study of cystic fibrosis: growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr. 2003;142:624-630.

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63.  Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med. 2010;182:298-306.

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64.  Quittner AL, Abbott J, Georgiopoulos AM, et al. International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax. 2016;71:26-34.

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65.  Nixon PA, Orenstein DM, Kelsey SF, et al. The prognostic value of exercise testing in patients with cystic fibrosis. N Engl J Med. 1992;327:1785-1788.

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66.  Orenstein DM, Hovell MF, Mulvihill M, et al. Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial. Chest. 2004;126:1204-1214.

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