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cystic fibrosis |
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cystic fibrosis (sĭs`tĭk fībrō`sĭs), inherited disorder of the exocrine glands (see gland gland, organ that manufactures chemical substances. A gland may vary from a single cell to a complex system of tubes that unite and open onto a surface through a duct. The endocrine glands, e.g. ..... Click the link for more information. ), affecting children and young people; median survival is 25 years in females and 30 years in males. It is caused by a genetic abnormality in the CF transmembrane conductance regulator (CFTR) gene that results in the disruption of chloride transfer across cell membranes. As a consequence, chloride ions build up in the cells of the lungs and other organs. Water stays inside the cells to dilute the chloride rather than being drawn out of the cells by normal chloride movement and the normal secretions of the organs thicken. Mucus in the exocrine glands becomes thick and sticky and eventually blocks the ducts of these glands (especially in the pancreas, lungs, and liver), forming cysts. The disease also causes the sweat glands to secrete excessive salt, causing heat prostration in hot weather. Symptoms, which vary according to the severity of the condition and the glands involved, include a distended abdomen; diarrhea; bulky, foul-smelling stools; and malnutrition. Medical problems include nasal polyps and sinus disease, repeated respiratory infections, infertility, liver disease, and diabetes. Diagnosis is confirmed by a sweat test or measurement of transmembrane potential. Treatment consists of dietary adjustment (low fat–high calorie) and the administration of vitamins vitamin, group of organic substances that are required in the diet of humans and animals for normal growth, maintenance of life, and normal reproduction. Vitamins act as catalysts; very often either the vitamins themselves are coenzymes , or they form integral parts Identification of the genes has also made tests for genetic screening genetic screening, testing for genetic disorders. Most commonly, prospective parents or an embryo or fetus is tested when a specific genetic disorder is suspected (e.g., Tay-Sachs or sickle cell disease ). BibliographySee P. Davis, Cystic Fibrosis (1993); M. E. Hodson and D. M. Geddes, Cystic Fibrosis (1994). cystic fibrosis (CF)or mucoviscidosisInherited metabolic disorder characterized by production of thick, sticky mucus. It is recessive (see recessiveness) and the most common inherited disorder (about 1 per 2,000 live births) in those of European ancestry. Concentrated mucous secretions in the lungs plug the bronchi, making breathing difficult, promoting infections, and producing chronic cough, recurrent pneumonia, and progressive loss of lung function, the usual cause of death. The secretions interfere with digestive enzymes and block nutrient absorption. Abnormally salty sweat is the basis for diagnosis of cystic fibrosis. Treatment includes enzyme supplements, a diet high in calories, protein, and fat, vigorous physical therapy, and antibiotics. Persons with cystic fibrosis once seldom survived beyond childhood; now more than half reach adulthood, though males are usually sterile. |
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Pharmaxis chief executive officer Dr Alan Robertson said: "The FDA decision is encouraging news for thousands of cystic fibrosis patients. Tokyo, Japan, Dec 26, 2006 - (JCN) - Shionogi announced on December 22 that it has achieved the primary objectives of Phase III clinical trials for the idiopathic pulmonary fibrosis treatment S-7701, generic name pirfenidone, which the Company is developing in Japan under a license from U. A defective gene in the bodies of cystic fibrosis patients causes the body to produce abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening infections. |
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