cystic fibrosis - meaning and definition. What is cystic fibrosis
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What (who) is cystic fibrosis - definition

AUTOSOMAL RECESSIVE DISEASE CHARACTERIZED BY THE BUILDUP OF MUCUS
Mucoviscidosis; Cistic fibrosis; Cystic Fiborsis; Cystic fibrosis allele; Cystic fibrose; Mucoviscidose; Viscoidosis; Mucuviscoidosis; Mucoviscoidosis; Mucoviscidopsis; Fibrocystic disease of the pancreas; Cystic Fibrosis; Cyctic fibrosis; Cystic Fibrosis (CF); History of Cystic fibrosis; Gene therapy for cystic fibrosis; Treatment of cystic fibrosis
  • Health problems associated with cystic fibrosis
  • The CFTR protein is a channel protein that controls the flow of H<sub>2</sub>O and Cl<sup>−</sup> ions in and out of cells inside the lungs. When the CFTR protein is working correctly, ions freely flow in and out of the cells. However, when the CFTR protein is malfunctioning, these ions cannot flow out of the cell due to a blocked channel. This causes cystic fibrosis, characterized by the buildup of thick mucus in the lungs.
  • The location of the ''CFTR'' gene on chromosome 7
  • Respiratory infections in CF vary according to age.<br><br>Green = ''[[Pseudomonas aeruginosa]]''<br>Brown = ''[[Staphylococcus aureus]]''<br>Blue = ''[[Haemophilus influenzae]]''<br>Red = ''[[Burkholderia cepacia]]'' complex
  • [[Dorothy Hansine Andersen]] first described cystic fibrosis in 1938.
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  • Intracytoplasmic sperm injection can be used to provide fertility for men with cystic fibrosis.

cystic fibrosis         
Cystic fibrosis is a serious disease of the glands which usually affects children and can make breathing difficult.
N-UNCOUNT
cystic fibrosis         
¦ noun a hereditary disorder which affects the exocrine glands and results in the production of abnormally thick mucus, leading to the blockage of the pancreatic ducts, intestines, and bronchi.
Cystic fibrosis–related diabetes         
MEDICAL CONDITION
Cystic fibrosis-related diabetes
Cystic fibrosis-related diabetes (CFRD) is diabetes specifically caused by cystic fibrosis, a genetic condition. Cystic fibrosis related diabetes mellitus (CFRD) develops with age, and the median age at diagnosis is 21 years.

Wikipedia

Cystic fibrosis

Cystic fibrosis (CF) is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.

Cystic fibrosis is inherited in an autosomal recessive manner. It is caused by the presence of mutations in both copies of the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Those with a single working copy are carriers and otherwise mostly healthy. CFTR is involved in the production of sweat, digestive fluids, and mucus. When the CFTR is not functional, secretions which are usually thin instead become thick. The condition is diagnosed by a sweat test and genetic testing. Screening of infants at birth takes place in some areas of the world.

There is no known cure for cystic fibrosis. Lung infections are treated with antibiotics which may be given intravenously, inhaled, or by mouth. Sometimes, the antibiotic azithromycin is used long term. Inhaled hypertonic saline and salbutamol may also be useful. Lung transplantation may be an option if lung function continues to worsen. Pancreatic enzyme replacement and fat-soluble vitamin supplementation are important, especially in the young. Airway clearance techniques such as chest physiotherapy have some short-term benefit, but long-term effects are unclear. The average life expectancy is between 42 and 50 years in the developed world. Lung problems are responsible for death in 80% of people with cystic fibrosis.

CF is most common among people of Northern European ancestry, for whom it affects about 1 out of 3,000 newborns, and among which around 1 out of 25 people is a carrier. It is least common in Africans and Asians, though it does occur in all races. It was first recognized as a specific disease by Dorothy Andersen in 1938, with descriptions that fit the condition occurring at least as far back as 1595. The name "cystic fibrosis" refers to the characteristic fibrosis and cysts that form within the pancreas.

Examples of use of cystic fibrosis
1. And his youngest son suffers from cystic fibrosis.
2. One in every 2,500 babies is born with cystic fibrosis.
3. Impassioned appeal: Gordon Brown with his son Fraser, who has cystic fibrosis Read more...
4. Richard Mattingly fights a daily battle to maintain funding for cystic fibrosis research.
5. Alan Larsen, director of research at the Cystic Fibrosis Trust, said: "We welcome any advances in scientific research that may speed up the development of an effective treatment for cystic fibrosis, which affects millions across the world."