Cystic Fibrosis

Common Name(s)

Cystic Fibrosis

Cystic fibrosis (CF) is an inherited condition that causes mucus to build up and clog some of the organs in the body, particularly in the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria to get stuck in the airways, which causes inflammation and infections. These infections can cause chronic coughing, and wheezing. Over time, mucus buildup and infections can lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. Mucus can also block the digestive tract and pancreas, leading to digestive problems. CF is caused by mutations in the CFTR gene and is inherited in an autosomal recessive fashion.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Cystic Fibrosis" for support, advocacy or research.

Boomer Esiason Foundation

The Boomer Esiason Foundation is a dynamic partnership of leaders in the medical and business communities joining with a committed core of volunteers to heighten awareness, education and quality of life for those affected by cystic fibrosis, while providing financial support to research aimed at finding a cure.

Last Updated: 15 Feb 2013

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Center for Jewish Genetics

The Center is dedicated to gathering and disseminating knowledge about Jewish genetic disorders and hereditary cancers. Its mission is to educate and serve health care professionals, clergy and the Jewish community.

Last Updated: 26 Dec 2012

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Cochrane Collaboration

The Cochrane Collaboration is an international network of healthcare professionals, researchers and consumers preparing, maintaining, and disseminating high quality systematic reviews of randomised control trials. This review group focuses on genetic disorders such as cystic fibrosis (CF), haemoglobinopathies, coagulopathies and inborn errors of metabolism.

Last Updated: 12 May 2015

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Cystic Fibrosis Foundation

Our mission is to assure the development of the means to cure and control Cystic Fibrosis and to improve the quality of life for those with the disease.

Last Updated: 19 Mar 2013

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Cystic Fibrosis Research, Inc.

Cystic Fibrosis Research, Inc. exists to fund research, to provide educational and personal support and to spread awareness of cystic fibrosis, a life-threatening genetic disease.

Last Updated: 5 Mar 2013

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General Support Organizations

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How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Cystic Fibrosis" for support, advocacy or research.

Boomer Esiason Foundation

The Boomer Esiason Foundation is a dynamic partnership of leaders in the medical and business communities joining with a committed core of volunteers to heighten awareness, education and quality of life for those affected by cystic fibrosis, while providing financial support to research aimed at finding a cure.

http://www.esiason.org

Last Updated: 15 Feb 2013

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Center for Jewish Genetics

The Center is dedicated to gathering and disseminating knowledge about Jewish genetic disorders and hereditary cancers. Its mission is to educate and serve health care professionals, clergy and the Jewish community.

http://www.jewishgenetics.org

Last Updated: 26 Dec 2012

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Cochrane Collaboration

The Cochrane Collaboration is an international network of healthcare professionals, researchers and consumers preparing, maintaining, and disseminating high quality systematic reviews of randomised control trials. This review group focuses on genetic disorders such as cystic fibrosis (CF), haemoglobinopathies, coagulopathies and inborn errors of metabolism.

http://cfgd.cochrane.org

Last Updated: 12 May 2015

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Cystic Fibrosis Foundation

Our mission is to assure the development of the means to cure and control Cystic Fibrosis and to improve the quality of life for those with the disease.

http://www.cff.org

Last Updated: 19 Mar 2013

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Cystic Fibrosis Research, Inc.

Cystic Fibrosis Research, Inc. exists to fund research, to provide educational and personal support and to spread awareness of cystic fibrosis, a life-threatening genetic disease.

http://www.cfri.org

Last Updated: 5 Mar 2013

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General Support Organizations

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General Resources

CF in the Classroom (English and Spanish)

A guide/manuel for parents, teachers, school administrators, and the healthcare team.

Updated 19 Oct 2012

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CFRI newsletter

Newsletter issued 3 times a year, addressing CF education, research, inspiration, and new information on medications and therapies.

Updated 19 Oct 2012

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Cystic Fibrosis" returned 5474 free, full-text research articles on human participants. First 3 results:

Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.
 

Author(s): Claire E Wainwright, J Stuart Elborn, Bonnie W Ramsey

Journal: N. Engl. J. Med.. 2015 Oct;373(18):1783-4.

 

Last Updated: 29 Oct 2015

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Cystic Fibrosis Transmembrane Conductance Regulator (CFTR): CLOSED AND OPEN STATE CHANNEL MODELS.
 

Author(s): Valentina Corradi, Paola Vergani, D Peter Tieleman

Journal: J. Biol. Chem.. 2015 Sep;290(38):22891-906.

 

The cystic fibrosis transmembrane conductance regulator (CFTR) is a member of the ATP-binding cassette (ABC) transporter superfamily. CFTR controls the flow of anions through the apical membrane of epithelia. Dysfunctional CFTR causes the common lethal genetic disease cystic fibrosis. ...

Last Updated: 19 Sep 2015

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Effect of ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation: patient-reported outcomes in the STRIVE randomized, controlled trial.
 

Author(s): Alexandra Quittner, Ellison Suthoff, Regina Rendas-Baum, Martha S Bayliss, Isabelle Sermet-Gaudelus, Brenda Castiglione, Montserrat Vera-Llonch

Journal:

 

Cystic fibrosis (CF) is an inherited, rare autosomal recessive disease that results in chronically debilitating morbidities and high premature mortality. We evaluated how ivacaftor treatment affected CF symptoms, functioning, and well-being, as measured by the Cystic Fibrosis Questionnaire-Revised ...

Last Updated: 11 Jul 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Cystic Fibrosis" returned 581 free, full-text review articles on human participants. First 3 results:

Physiological impact of abnormal lipoxin A₄ production on cystic fibrosis airway epithelium and therapeutic potential.
 

Author(s): Gerard Higgins, Fiona Ringholz, Paul Buchanan, Paul McNally, Valérie Urbach

Journal: Biomed Res Int. 2015 ;2015():781087.

 

Lipoxin A4 has been described as a major signal for the resolution of inflammation and is abnormally produced in the lungs of patients with cystic fibrosis (CF). In CF, the loss of chloride transport caused by the mutation in the cystic fibrosis transmembrane conductance regulator ...

Last Updated: 13 Apr 2015

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Year in review 2014: Interstitial lung disease, physiology, sleep and ventilation, acute respiratory distress syndrome, cystic fibrosis, bronchiectasis and rare lung disease.
 

Author(s): Toby M Maher, Amanda Piper, Yuanlin Song, Marcos I Restrepo, Neil D Eves

Journal: Respirology. 2015 Jul;20(5):834-45.

 

Last Updated: 15 Jun 2015

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Therapeutic options for hydrating airway mucus in cystic fibrosis.
 

Author(s): Bernadett E Tildy, Duncan F Rogers

Journal: Pharmacology. 2015 ;95(3-4):117-32.

 

In cystic fibrosis (CF), genetic mutations in the CF transmembrane conductance regulator (CFTR) gene cause reduced chloride efflux from ciliated airway epithelial cells. This results in a reduction in periciliary liquid (PCL) depth of the airway surface liquid due to associated reduced ...

Last Updated: 13 May 2015

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Ivacaftor (Kalydeco) and Insulin in Cystic Fibrosis (CF)
 

Status: Recruiting

Condition Summary: Cystic Fibrosis Related Diabetes; Cystic Fibrosis

 

Last Updated: 3 Aug 2015

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Metabolic Efficiency of Combined Pancreatic Islet and Lung Transplant for the Treatment of End-Stage Cystic Fibrosis
 

Status: Recruiting

Condition Summary: Cystic Fibrosis; Diabetes Related Cystic Fibrosis

 

Last Updated: 2 Mar 2015

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Role of Glucagon In Glucose Control in Cystic Fibrosis Related Diabetes
 

Status: Recruiting

Condition Summary: Cystic Fibrosis; Cystic Fibrosis Related Diabetes

 

Last Updated: 13 Oct 2015

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