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?

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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 5607 free, full-text research articles on human participants. First 3 results:

Airway acidification initiates host defense abnormalities in cystic fibrosis mice.
 

Author(s): Viral S Shah, David K Meyerholz, Xiao Xiao Tang, Leah Reznikov, Mahmoud Abou Alaiwa, Sarah E Ernst, Philip H Karp, Christine L Wohlford-Lenane, Kristopher P Heilmann, Mariah R Leidinger, Patrick D Allen, Joseph Zabner, Paul B McCray, Lynda S Ostedgaard, David A Stoltz, Christoph O Randak, Michael J Welsh

Journal: Science. 2016 Jan;351(6272):503-7.

 

Cystic fibrosis (CF) is caused by mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel. In humans and pigs, the loss of CFTR impairs respiratory host defenses, causing airway infection. But CF mice are spared. We found that ...

Last Updated: 29 Jan 2016

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Epidemic spread of Pandoraea pulmonicola in a cystic fibrosis center.
 

Author(s): Nicolas Degand, Romain Lotte, Célia Decondé Le Butor, Christine Segonds, Michelle Thouverez, Agnès Ferroni, Christine Vallier, Laurent Mély, Jacqueline Carrère

Journal:

 

Pandoraea spp. are recently discovered bacteria, mainly recovered from cystic fibrosis (CF) patients, but their epidemiology and clinical significance are not well known. We describe an epidemic spread of Pandoraea pulmonicola from 2009 in our CF center, involving 6 out of 243 CF patients.

Last Updated: 26 Dec 2015

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Predicting future lung function decline in cystic fibrosis patients: Statistical methods and clinical connections.
 

Author(s): Rhonda D Szczesniak, Gary L McPhail, Dan Li, Raouf S Amin, John P Clancy

Journal: Pediatr. Pulmonol.. 2016 Feb;51(2):217-8.

 

Last Updated: 19 Jan 2016

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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 599 free, full-text review articles on human participants. First 3 results:

Cystic Fibrosis Lung Infections: Polymicrobial, Complex, and Hard to Treat.
 

Author(s): Laura M Filkins, George A O'Toole

Journal:

 

Last Updated: 1 Jan 2016

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US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis: executive summary.
 

Author(s): R Andres Floto, Kenneth N Olivier, Lisa Saiman, Charles L Daley, Jean-Louis Herrmann, Jerry A Nick, Peadar G Noone, Diana Bilton, Paul Corris, Ronald L Gibson, Sarah E Hempstead, Karsten Koetz, Kathryn A Sabadosa, Isabelle Sermet-Gaudelus, Alan R Smyth, Jakko van Ingen, Richard J Wallace, Kevin L Winthrop, Bruce C Marshall, Charles S Haworth

Journal: Thorax. 2016 Jan;71(1):88-90.

 

Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease, such as cystic fibrosis (CF). Pulmonary disease (PD) caused by NTM has emerged as a major ...

Last Updated: 18 Dec 2015

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US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of non-tuberculous mycobacteria in individuals with cystic fibrosis.
 

Author(s): R Andres Floto, Kenneth N Olivier, Lisa Saiman, Charles L Daley, Jean-Louis Herrmann, Jerry A Nick, Peadar G Noone, Diana Bilton, Paul Corris, Ronald L Gibson, Sarah E Hempstead, Karsten Koetz, Kathryn A Sabadosa, Isabelle Sermet-Gaudelus, Alan R Smyth, Jakko van Ingen, Richard J Wallace, Kevin L Winthrop, Bruce C Marshall, Charles S Haworth,

Journal: Thorax. 2016 Jan;71 Suppl 1():i1-22.

 

Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease such as cystic fibrosis (CF). Pulmonary disease caused by NTM has emerged as a major threat ...

Last Updated: 15 Dec 2015

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

 
 
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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: 10 Feb 2016

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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: 16 Mar 2016

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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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