Highlights & Basics
- Cystic fibrosis (CF) is a genetic multisystem disease associated with abnormalities in salt and water transport across epithelial surfaces. Cystic fibrosis transmembrane conductance regulator (CFTR) gene variants are considered responsible for most disease.
- Carrier screening can help those at risk make informed decisions about pregnancy, while newborn screening can ensure prompt diagnosis and treatment. A positive sweat test is diagnostic; genetic testing is performed in addition to the sweat test.
- CFTR modulators are now a central component of care for most patients, improving many clinical outcomes by partially restoring CFTR function.
- Respiratory disease is treated with augmented airway clearance, antibiotics, and mucus thinners.
- Gastrointestinal disease is primarily treated with supplemental pancreatic enzymes, a high-calorie diet, and fat-soluble vitamins to support growth and nutrition. Some patients will require treatment for gastroesophageal reflux and liver disease.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Cystic fibrosis transmembrane conductance regulator (CFTR) gene variants are classified into minimal function (class I-II), residual function (class IV-VI), and gating variants (Class III; may respond to a potentiator such as ivacaftor). The schematic shows the amount of CFTR protein in the cell membrane. Impact on function is illustrated by arrows showing the movement of chloride ions (Cl-) out of the cell (solid, dashed, and no arrows indicate normal, reduced, and no Cl- flow, respectively). In practice, some variants may straddle a number of classes
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
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; Pulmonary Clinical Practice Guidelines Committee. 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]
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]
Ramos KJ, Smith PJ, McKone EF, et al; CF Lung Transplant Referral Guidelines Committee. 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]
- Clinical and Functional Translation of CFTR (CFTR2)
- Cystic Fibrosis Foundation: nutritional basics
- Cystic Fibrosis Foundation: consider tube feeding
- Cystic Fibrosis Trust (UK): diet and nutrition leaflets
- Cystic Fibrosis Foundation: drug development pipeline
- The Cystic Fibrosis Center at Stanford
- Cystic Fibrosis Foundation: a teacher's guide to cystic fibrosis
- Cystic Fibrosis Trust (UK): cystic fibrosis for teachers
- CDC: healthcare professionals - adult immunization schedule by age
- CDC: healthcare professionals - child and adolescent immunization schedule by age
- Cystic Fibrosis Foundation: managing CF - Pseudomonas
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