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Cystic Fibrosis (CF)

A common hereditary disease in which exocrine (secretory) glands produce abnormally thick mucus. This mucus can cause problems in digestion, breathing, and body cooling.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

Cystic Fibrosis

Cystic fibrosis (SIS-tik fi-BRO-sis), or CF, is an inherited disease of the secretory (see-KREH-tor-ee) glands. Secretory glands include glands that make mucus and sweat.

"Inherited" means the disease is passed from parents to children through genes. People who have CF inherit two faulty genes for the disease - one from each parent. The parents likely don't have the disease themselves.

CF mainly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.

Overview

Mucus is a substance made by tissues that line some organs and body cavities, such as the lungs and nose. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected.

If you have CF, your mucus becomes thick and sticky. It builds up in your lungs and blocks your airways. (Airways are tubes that carry air in and out of your lungs.)

The... Read more about Cystic Fibrosis

What works? Research summarized

Evidence reviews

The use of regular vitamin A preparations for children and adults with cystic fibrosis

Cystic fibrosis can lead to certain vitamins, such as vitamin A, not being properly absorbed by the body. This can result in problems caused by vitamin deficiency. A lack of vitamin A (vitamin A deficiency) can cause specific problems such as eye and skin problems. It can also be associated with poorer general and respiratory health. Therefore people with cystic fibrosis are usually given regular vitamin A preparations from a very young age. However, too much vitamin A can also cause respiratory and bone problems. The review aimed to show whether giving vitamin A regularly to people with cystic fibrosis is beneficial or not. However, the authors did not find any relevant trials to include in the review. They are therefore unable to draw any conclusions regarding the routine administration of vitamin A supplements and recommend that until further evidence is available, local guidelines are followed regarding this practice.

Pneumococcal vaccines for children and adults with cystic fibrosis

Cystic fibrosis is an inherited disease which causes mucus in the lungs to be more sticky than normal, leading to frequent lung infections and lung damage. Pneumococcus is a type of bacteria that can cause infection throughout the body, including in the urine, blood stream, fluid around the brain and in the lungs. These infections can be very serious and may lead to severe illness and death. There are two types of vaccine that can be used to help protect people against some forms of pneumococcus bacteria. One vaccine is used in children under two years of age and the other is used in older children and adults. This review aimed to find trials that compared giving the vaccine against no vaccine or an inactive (placebo) form to assess how useful vaccines against pneumococcus bacteria could be in reducing serious illness and death in people with cystic fibrosis. No trials were identified that assessed these vaccines in people with cystic fibrosis. The evidence to support the routine use of vaccines against pneumococcal infection is not strong; however, many countries are now recommending all children have the vaccine as part of their childhood immunisation programmes.

Antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis

Stenotrophomonas maltophilia is a bacteria which is resistant to several antibiotics. Over the last 10 years this bacteria has increasingly been found in the lungs of people with cystic fibrosis. Chronic infection with Stenotrophomonas maltophilia has been found to be linked to pulmonary infections. However, at present, it is unclear if people with cystic fibrosis should be treated for this bacteria when it is grown in their lungs. The purpose of this review is to determine if treatment with different antibiotic combinations for Stenotrophomonas maltophilia will improve lung function or decrease the frequency of hospital admission in people with cystic fibrosis. We also want to review the effect of treatment of chronic Stenotrophomonas maltophilia infection in order to clear this bacteria from the lungs of a person with cystic fibrosis. We did not find any randomized controlled trials which we could include in the review. We did find one trial of antibiotic treatment for pulmonary exacerbations which included patients with Stenotrophomonas maltophilia, but the patients in the trial also had infections caused by other bacteria and we were not able to obtain data separately for the different causes of infection. Randomized controlled trials are needed to inform clinicians as to whether they should treat Stenotrophomonas maltophilia infection in people with cystic fibrosis. In the meantime, clinicians should use their clinical judgement when considering this question.

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Summaries for consumers

The use of regular vitamin A preparations for children and adults with cystic fibrosis

Cystic fibrosis can lead to certain vitamins, such as vitamin A, not being properly absorbed by the body. This can result in problems caused by vitamin deficiency. A lack of vitamin A (vitamin A deficiency) can cause specific problems such as eye and skin problems. It can also be associated with poorer general and respiratory health. Therefore people with cystic fibrosis are usually given regular vitamin A preparations from a very young age. However, too much vitamin A can also cause respiratory and bone problems. The review aimed to show whether giving vitamin A regularly to people with cystic fibrosis is beneficial or not. However, the authors did not find any relevant trials to include in the review. They are therefore unable to draw any conclusions regarding the routine administration of vitamin A supplements and recommend that until further evidence is available, local guidelines are followed regarding this practice.

Pneumococcal vaccines for children and adults with cystic fibrosis

Cystic fibrosis is an inherited disease which causes mucus in the lungs to be more sticky than normal, leading to frequent lung infections and lung damage. Pneumococcus is a type of bacteria that can cause infection throughout the body, including in the urine, blood stream, fluid around the brain and in the lungs. These infections can be very serious and may lead to severe illness and death. There are two types of vaccine that can be used to help protect people against some forms of pneumococcus bacteria. One vaccine is used in children under two years of age and the other is used in older children and adults. This review aimed to find trials that compared giving the vaccine against no vaccine or an inactive (placebo) form to assess how useful vaccines against pneumococcus bacteria could be in reducing serious illness and death in people with cystic fibrosis. No trials were identified that assessed these vaccines in people with cystic fibrosis. The evidence to support the routine use of vaccines against pneumococcal infection is not strong; however, many countries are now recommending all children have the vaccine as part of their childhood immunisation programmes.

Antibiotic treatment for Stenotrophomonas maltophilia in people with cystic fibrosis

Stenotrophomonas maltophilia is a bacteria which is resistant to several antibiotics. Over the last 10 years this bacteria has increasingly been found in the lungs of people with cystic fibrosis. Chronic infection with Stenotrophomonas maltophilia has been found to be linked to pulmonary infections. However, at present, it is unclear if people with cystic fibrosis should be treated for this bacteria when it is grown in their lungs. The purpose of this review is to determine if treatment with different antibiotic combinations for Stenotrophomonas maltophilia will improve lung function or decrease the frequency of hospital admission in people with cystic fibrosis. We also want to review the effect of treatment of chronic Stenotrophomonas maltophilia infection in order to clear this bacteria from the lungs of a person with cystic fibrosis. We did not find any randomized controlled trials which we could include in the review. We did find one trial of antibiotic treatment for pulmonary exacerbations which included patients with Stenotrophomonas maltophilia, but the patients in the trial also had infections caused by other bacteria and we were not able to obtain data separately for the different causes of infection. Randomized controlled trials are needed to inform clinicians as to whether they should treat Stenotrophomonas maltophilia infection in people with cystic fibrosis. In the meantime, clinicians should use their clinical judgement when considering this question.

See all (161)

Terms to know

Body Fiber
In the body, fiber refers to tissue made of long threadlike cells, such as muscle fiber or nerve fiber.
Exocrine Glands
An organ that makes one or more substances, such as sweat, tears, saliva, or milk. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.
Fibrosis
Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue. Fibrosis is similar to the process of scarring.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Glands
A group of cells that secrete substances. Endocrine glands secrete hormones. Exocrine glands secrete salt, enzymes, and water.
Mucus
A thick, slippery fluid made by the membranes that line certain organs of the body, including the nose, mouth, throat, and vagina.
Phlegm
A more than normal amount of thick mucus made by the cells lining the upper airways and lungs. A buildup of phlegm may be caused by infection, irritation, or chronic lung disease, and can cause discomfort in the chest and coughing.
Reproductive System
The organs involved in producing offspring. In women, this system includes the ovaries, the fallopian tubes, the uterus, the cervix, and the vagina. In men, it includes the prostate, the testes, and the penis.
Respiratory System
The organs that are involved in breathing. These include the nose, throat, larynx, trachea, bronchi, and lungs. Also called respiratory tract.
Secretion
To form and release a substance. In the body, cells secrete substances, such as sweat that cools the body or hormones that act in other parts of the body.
Sputum
Mucus and other matter brought up from the lungs by coughing.
Sweat
Fluid that exits the body through pores in the skin usually due to physical stress and/or high temperature for the purpose of regulating body temperature and removing certain compounds from the circulation.

More about Cystic Fibrosis

Photo of a child

Also called: Mucoviscidosis, Fibrocystic disease

Other terms to know: See all 12
Body Fiber, Exocrine Glands, Fibrosis

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