Cystic fibrosis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Cystic fibrosis (CF) is a genetic multisystem disease associated with abnormalities in salt and water transport across epithelial surfaces.
Carrier screening can help those at risk make informed decisions about pregnancy, while newborn screening can ensure prompt diagnosis and treatment. A sweat test is the most conclusive for diagnosis and is performed in addition to genetic testing.
Symptom onset is often in early infancy; however, in milder cases, symptoms may not develop until later childhood.
Treatment of respiratory disease includes mucus thinners, airway clearance, and antibiotics. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators can improve care and outcomes by partially restoring the function of mutated CFTR.
Gastrointestinal disease is treated with supplemental pancreatic enzymes, calorie support, and fat-soluble vitamins to support growth and nutrition.

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History & Exam
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Finger clubbing
CT--Coronal view of the paranasal sinuses showing the blockage of the osteomeatal complexes (Green Circle)
Pale-color soft tissue mass in the right nasal cavity

Citations

Key Articles

Farrell PM, White TB, Ren CL, et al. Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. J Pediatr. 2017 Feb;181S:S4-15.e1.[Abstract][Full Text]
Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013 Apr 1;187(7):680-9.[Abstract][Full Text]
Flume PA, Robinson KA, O'Sullivan BP, et al; Clinical Practice Guidelines for Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care. 2009 Apr;54(4):522-37.[Abstract][Full Text]
Ramos KJ, Smith PJ, McKone EF, et al. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J Cyst Fibros. 2019 May;18(3):321-33.[Abstract][Full Text]
Kapnadak SG, Dimango E, Hadjiliadis D, et al. Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease. J Cyst Fibros. 2020 May;19(3):344-54.[Abstract][Full Text]

Other Online Resources

Cystic Fibrosis Trust (UK): diet and nutrition leaflets
Cystic Fibrosis Foundation: drug development pipeline

Referenced Articles

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58. Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2017 Feb 20;(2):CD002769.[Abstract][Full Text]
59. Smith S, Rowbotham NJ, Edwards CT. Short-acting inhaled bronchodilators for cystic fibrosis. Cochrane Database Syst Rev. 2022 Jun 24;6(6):CD013666.[Abstract][Full Text]
60. Yang C, Montgomery M. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2021 Mar 18;(3):CD001127.[Abstract][Full Text]
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82. Rosenfeld M, Wainwright CE, Higgins M, et al; ARRIVAL study group. Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study. Lancet Respir Med. 2018 Jul;6(7):545-53.[Abstract][Full Text]
83. Davies J, Wang LT, Panorchan P, et al. WS06-4 ivacaftor (IVA) treatment in patients 6 to <12 months old with cystic fibrosis with a CFTR gating mutation: results of a 2-part, single-arm, phase 3 study. Paper presented at: 42nd European Cystic Fibrosis Conference. 5-8 Jun 2019. Liverpool, UK. J Cyst Fibros. 2019 Jun 1;18(suppl 1):S11.[Full Text]
84. Davies JC, Wainwright CE, Sawicki GS, et al. Ivacaftor in infants aged 4 to <12 months with cystic fibrosis and a gating mutation. Results of a two-part phase 3 clinical trial. Am J Respir Crit Care Med. 2021 Mar 1;203(5):585-93.[Abstract][Full Text]
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86. Mitchell RM, Jones AM, Stocking K, et al. Longitudinal effects of ivacaftor and medicine possession ratio in people with the Gly551Asp mutation: a 5-year study. Thorax. 2021 Sep;76(9):874-9.[Abstract]
87. Bailey J, Rozga M, McDonald CM, et al. Effect of CFTR modulators on anthropometric parameters in individuals with cystic fibrosis: an Evidence Analysis Center systematic review. J Acad Nutr Diet. 2021 Jul;121(7):1364-78.e2.[Abstract]
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94. Goralski JL, Hoppe JE, Mall MA, et al. Phase 3 open-label clinical trial of elexacaftor/tezacaftor/ivacaftor in children aged 2-5 years with cystic fibrosis and at least one F508del allele. Am J Respir Crit Care Med. 2023 Jul 1;208(1):59-67.[Abstract][Full Text]
95. Zemanick ET, Taylor-Cousar JL, Davies J, et al. A phase 3 open-label study of elexacaftor/tezacaftor/ivacaftor in children 6 through 11 years of age with cystic fibrosis and at least one F508del allele. Am J Respir Crit Care Med. 2021 Jun 15;203(12):1522-32.[Abstract][Full Text]
96. Heijerman HGM, McKone EF, Downey DG, et al. Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial. Lancet. 2019 Nov 23;394(10212):1940-8.[Abstract]
97. Barry PJ, Mall MA, Álvarez A, et al. Triple therapy for cystic fibrosis Phe508del-gating and -residual function genotypes. N Engl J Med. 2021 Aug 26;385(9):815-25.[Abstract][Full Text]
98. Sutharsan S, McKone EF, Downey DG, et al. Efficacy and safety of elexacaftor plus tezacaftor plus ivacaftor versus tezacaftor plus ivacaftor in people with cystic fibrosis homozygous for F508del-CFTR: a 24-week, multicentre, randomised, double-blind, active-controlled, phase 3b trial. Lancet Respir Med. 2022 Mar;10(3):267-77.[Abstract]
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100. Okroglic L, Sohier P, Martin C, et al. Acneiform eruption following elexacaftor-tezacaftor-ivacaftor treatment in patients with cystic fibrosis. JAMA Dermatol. 2023 Jan 1;159(1):68-72.[Abstract]
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106. Saadh MJ, Lohrasbi A, Ghasemian E, et al. The status of carbapenem resistance in cystic fibrosis: a systematic review and meta-analysis. Yale J Biol Med. 2022 Dec;95(4):495-506.[Abstract]
107. Bonyadi P, Saleh NT, Dehghani M, et al. Prevalence of antibiotic resistance of Pseudomonas aeruginosa in cystic fibrosis infection: A systematic review and meta-analysis. Microb Pathog. 2022 Apr;165:105461.[Abstract][Full Text]
108. Smith S, Rowbotham NJ, Charbek E. Inhaled antibiotics for pulmonary exacerbations in cystic fibrosis. Cochrane Database Syst Rev. 2022 Aug 1;8(8):CD008319.[Abstract][Full Text]
109. Somayaji R, Parkins MD, Shah A, et al; Antimicrobial Resistance in Cystic Fibrosis International Working Group. Antimicrobial susceptibility testing (AST) and associated clinical outcomes in individuals with cystic fibrosis: a systematic review. J Cyst Fibros. 2019 Mar;18(2):236-43.[Abstract][Full Text]
110. Frost F, Shaw M, Nazareth D. Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis. Cochrane Database Syst Rev. 2019 Jun 13;6(6):CD013079.[Abstract][Full Text]
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112. Nicholson TT, Smith A, McKone EF, et al. Duration of intravenous antibiotic treatment for acute exacerbations of cystic fibrosis: a systematic review. J Cyst Fibros. 2022 Jul;21(4):562-73.[Abstract]
113. Ramos KJ, Smith PJ, McKone EF, et al. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J Cyst Fibros. 2019 May;18(3):321-33.[Abstract][Full Text]
114. Shah P, Lowery E, Chaparro C, et al. Cystic fibrosis foundation consensus statements for the care of cystic fibrosis lung transplant recipients. J Heart Lung Transplant. 2021 Jul;40(7):539-56.[Abstract][Full Text]
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116. Holmes M, Murphy V, Taylor M, et al. Intussusception in cystic fibrosis. Arch Dis Child. 1991 Jun;66(6):726-7.[Abstract][Full Text]
117. Ng C, Major G, Smyth AR. Timing of pancreatic enzyme replacement therapy (PERT) in cystic fibrosis. Cochrane Database Syst Rev. 2021 Aug 2;8(8):CD013488.[Abstract][Full Text]
118. Gilchrist FJ, Green J, Carroll W. Interventions for treating distal intestinal obstruction syndrome (DIOS) in cystic fibrosis. Cochrane Database Syst Rev. 2021 Dec 22;12(12):CD012798.[Abstract][Full Text]
119. Shidrawi RG, Murugan N, Westaby D, et al. Emergency colonoscopy for distal intestinal obstruction syndrome in cystic fibrosis patients. Gut. 2002 Aug;51(2):285-6.[Abstract][Full Text]
120. El Boghdady M, Ewalds-Kvist BM. Laparoscopic surgery in patients with cystic fibrosis: a systematic review. Asian J Endosc Surg. 2021 Jul;14(3):327-34.[Abstract]
121. Ng J, Friedmacher F, Pao C, et al. Gastroesophageal reflux disease and need for antireflux surgery in children with cystic fibrosis: a systematic review on incidence, surgical complications, and postoperative outcomes. Eur J Pediatr Surg. 2021 Feb;31(1):106-14.[Abstract][Full Text]
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