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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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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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 5089 free, full-text research articles on human participants. First 3 results:

Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis.
 

Author(s): József Maléth, Anita Balázs, Petra Pallagi, Zsolt Balla, Balázs Kui, Máté Katona, Linda Judák, István Németh, Lajos V Kemény, Zoltán Rakonczay, Viktória Venglovecz, Imre Földesi, Zoltán Pető, Áron Somorácz, Katalin Borka, Doranda Perdomo, Gergely L Lukacs, Mike A Gray, Stefania Monterisi, Manuela Zaccolo, Matthias Sendler, Julia Mayerle, Jens-Peter Kühn, Markus M Lerch, Miklós Sahin-Tóth, Péter Hegyi

Journal: Gastroenterology. 2015 Feb;148(2):427-39.e16.

 

Excessive consumption of ethanol is one of the most common causes of acute and chronic pancreatitis. Alterations to the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) also cause pancreatitis. However, little is known about the role of CFTR in the pathogenesis ...

Last Updated: 24 Jan 2015

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Identification of Inquilinus limosus in cystic fibrosis: a first report in Italy.
 

Author(s): Annunziata Gaetana Cicatiello, Dora Vita Iula, Chiara Pagliuca, Gabiria Pastore, Caterina Pagliarulo, Maria Rosaria Catania, Maria Rosaria Catania, Roberta Colicchio, Marco Picardi, Valeria Raia, Paola Salvatore

Journal: New Microbiol.. 2014 Oct;37(4):567-71.

 

Cystic fibrosis is a genetic disorder associated with a polymicrobial lung infection where classical pathogens and newly identified bacteria may interact. Inquilinus limosus is an a-proteobacterium recently isolated in the airways of cystic fibrosis patient. We report the first case ...

Last Updated: 12 Nov 2014

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Mechanisms of CFTR functional variants that impair regulated bicarbonate permeation and increase risk for pancreatitis but not for cystic fibrosis.
 

Author(s): Jessica LaRusch, Jinsei Jung, Ignacio J General, Michele D Lewis, Hyun Woo Park, Randall E Brand, Andres Gelrud, Michelle A Anderson, Peter A Banks, Darwin Conwell, Christopher Lawrence, Joseph Romagnuolo, John Baillie, Samer Alkaade, Gregory Cote, Timothy B Gardner, Stephen T Amann, Adam Slivka, Bimaljit Sandhu, Amy Aloe, Michelle L Kienholz, Dhiraj Yadav, M Michael Barmada, Ivet Bahar, Min Goo Lee, David C Whitcomb,

Journal:

 

CFTR is a dynamically regulated anion channel. Intracellular WNK1-SPAK activation causes CFTR to change permeability and conductance characteristics from a chloride-preferring to bicarbonate-preferring channel through unknown mechanisms. Two severe CFTR mutations (CFTRsev) cause complete ...

Last Updated: 18 Jul 2014

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

European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis.
 

Author(s): Martin Stern, Dominique Pougheon Bertrand, Elisabetta Bignamini, Mary Corey, Birgit Dembski, Christopher H Goss, Tanja Pressler, Gilles Rault, Laura Viviani, J Stuart Elborn, Carlo Castellani

Journal: J. Cyst. Fibros.. 2014 May;13 Suppl 1():S43-59.

 

Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled ...

Last Updated: 26 May 2014

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European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre.
 

Author(s): Steven Conway, Ian M Balfour-Lynn, Karleen De Rijcke, Pavel Drevinek, Juliet Foweraker, Trudy Havermans, Harry Heijerman, Louise Lannefors, Anders Lindblad, Milan Macek, Sue Madge, Maeve Moran, Lisa Morrison, Alison Morton, Jacquelien Noordhoek, Dorota Sands, Anneke Vertommen, Daniel Peckham

Journal: J. Cyst. Fibros.. 2014 May;13 Suppl 1():S3-22.

 

A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those ...

Last Updated: 26 May 2014

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European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.
 

Author(s): Alan R Smyth, Scott C Bell, Snezana Bojcin, Mandy Bryon, Alistair Duff, Patrick Flume, Nataliya Kashirskaya, Anne Munck, Felix Ratjen, Sarah Jane Schwarzenberg, Isabelle Sermet-Gaudelus, Kevin W Southern, Giovanni Taccetti, Gerald Ullrich, Sue Wolfe,

Journal: J. Cyst. Fibros.. 2014 May;13 Suppl 1():S23-42.

 

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for ...

Last Updated: 26 May 2014

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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: 2 Feb 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: 19 Mar 2015

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