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Cystic Fibrosis – An Introduction

Cystic Fibrosis - An Introduction


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‘Cystic Fibrosis Handbook’, 6th Edition, produced by Cystic Fibrosis Association of Victoria, 1998

LESSON 2: Genetics and Incidence

Bell etal, Cystic fibrosis in Australia, 2009: results from a data registry. Med J Aust 2011; 195 (7): 396-400.

The Clinical and Functional Translation of CFTR (CFTR2). Available at 18 October 2018)

LESSON 3: Prognosis

Elborn, S. Cystic Fibrosis. Lancet; 388(10059): 2519-2531


Morrell, M et al. Lung Transplantation for Cystic Fibrosis. Clinical Chest Medicine. Mar 2016; 37:127-138

LESSON 4: Multidisciplinary care

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Dominique Hubert · Nicholas Simmonds (editors) Living longer with Cystic Fibrosis (2015) E-book

Standards for the Clinical Care of Children and Adults with cystic fibrosis in the UK Second edition. December 2011. UK CF Trust website:

Physiotherapy for people with Cystic Fibrosis: from infant to adult(“Blue booklet”) 2009

Button BM etal,Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline. Respirology. 2016 May; 21(4): 656–667.

Quittner AL etal, 8 the International Committee on Mental Health 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.

Quittner, Alexandra L.; Saez-Flores, Estefany; Barton, John D. The psychological burden of cystic fibrosis . Current Opinion in Pulmonary Medicine: March 2016 – Volume 22 – Issue 2 – p 187–191


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Bryant, J. M etal. Population-level genomics identifies the emergence and global spread of a human transmissible multidrug-resistant nontuberculous mycobacterium. Science 2016 (New York, N.Y.), 354(6313), 751–757.

Cystic Fibrosis Australia Infection control guidelines 2012

Garber E, Desai M, Zhou J, Alba L, Angst D, Cabana M, et al. Barriers to adherence to cystic fibrosis infection control guidelines. Pediatr Pulmonol. 2008;43(9):900–907.

Gibson RL, Burns JL, Ramsey BW. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med. 2003;168(8):918–951.

Hutchinson GR, Parker S, Pryor JA, Duncan-Skingle F, Hoffman PN, Hodson ME, et al. Home-use nebulizers: a potential primary source of Burkholderia cepacia and other colistin-resistant, gram-negative bacteria in patients with cystic fibrosis. J Clin Microbiol. 1996;34(3):584–587.

Knibbs LDetal. Viability of Pseudomonas aeruginosa in cough aerosols generated by persons with cystic fibrosis. Thorax. 2014 Aug;69(8):740-5.

Lester MK, Flume PA, Gray SL, Anderson D, Bowman CM. Nebulizer use and maintenance by cystic fibrosis patients: a survey study. Respir Care. 2004;49(12):1504–1508.

Qvist T etal. Comparing the harmful effects of nontuberculous mycobacteria and Gram negative bacteria on lung function in patients with cystic fibrosisJ Cyst Fibrosis. 2016 May; 15(3): 380–385.

Reychler G, Aarab K, Van Ossel C, Gigi J, Simon A, Leal T, et al. In vitro evaluation of efficacy of 5 methods of disinfection on mouthpieces and facemasks contaminated by strains of cystic fibrosis patients. J Cyst Fibros. 2005;4(3):183–187.

Rosenfeld M, Emerson J, Astley S, Joy P, Williams-Warren J, Standaert TA, et al. Home nebulizer use among patients with cystic fibrosis. J Pediatr. 1998;132(1):125–131.

Saiman L, Siegel J, Cystic Fibrosis Foundation Infection control recommendations for patients with cystic fibrosis: microbiology, important pathogens, and infection control practices to prevent patient-to-patient transmission. Infect Control Hosp Epidemiol. 2003;24(5 ) Suppl:S6–52.

Somayaji Retal. Infection control knowledge, beliefs and behaviours amongst cystic fibrosis patients with epidemic Pseudomonas aeruginosa. BMC Pulm Med. 2015 Nov 5;15:138.

Zuana AD etal. Effect that an educational program for cystic fibrosis patients and caregivers has on the contamination of home nebulizers. J Bras Pneumol. 2014 Mar-Apr; 40(2): 119–127.

Zuckerman JB, Clock SA, Prato BS, McDevitt JJ, Zhou JJ, Leclair LW, Lucas FL, Saiman L.Air contamination with bacteria in cystic fibrosis clinics: implications for prevention strategies. Am J Respir Crit Care Med. 2015 Mar 1;191(5):598-601.


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