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Cystic fibrosis is an inherited disease of the mucus glands that affects many body systems. In particular, this disorder causes progressive damage to the respiratory system and chronic digestive system problems.
Mucus is a slippery substance that lubricates and protects the linings of the airways, digestive system, reproductive system, and other organs and tissues. In people with cystic fibrosis, the body produces mucus that is abnormally thick and sticky. This abnormal mucus can obstruct the airways, leading to severe problems with breathing and bacterial infections in the lungs. These infections cause chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections result in permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs.
Most people with cystic fibrosis also have digestive problems because thick, sticky mucus interferes with the function of the pancreas. The pancreas is an organ that produces insulin (a hormone that helps control blood sugar levels) and enzymes that help digest food. Mucus blocks the ducts of the pancreas, preventing these enzymes from reaching the intestines to aid digestion. Problems with digestion can lead to diarrhea, malnutrition, poor growth, and weight loss. Some babies with cystic fibrosis have meconium ileus, a blockage of the intestine that occurs shortly after birth.
Men with cystic fibrosis are often unable to father a child (infertile) because the tubes that carry sperm (the vas deferens) are blocked by mucus and do not develop properly. Infertility is also possible, though less common, in women with cystic fibrosis.
Cystic fibrosis is a common genetic disease in the Caucasian (white) population in the United States. The disease occurs in about 1 in 3,200 Caucasian newborns. Cystic fibrosis is less common in other ethnic groups, affecting 1 in 15,000 African Americans and 1 in 31,000 Asian Americans.
Mutations in the CFTR gene cause cystic fibrosis.
The CFTR gene provides instructions for making a channel that transports negatively charged particles called chloride ions into and out of cells. The flow of chloride ions helps control the movement of water in tissues and regulates the fluid consistency of mucus. Mutations in the CFTR gene disrupt the normal function of this channel, preventing the usual flow of chloride ions and water. As a result, cells that line the passageways of the lungs, pancreas, and other organs produce mucus that is abnormally thick and sticky. This mucus obstructs the airways and glands, causing the characteristic signs and symptoms of cystic fibrosis.
Other genetic and environmental factors likely influence the course of the condition. For example, mutations in genes other than CFTR might help explain why some people with cystic fibrosis are more severely affected than others. Most of these genetic changes have not been identified, however.
This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. Most often, the parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but do not show signs and symptoms of the condition.
'''Cystic fibrosis''' ('''CF'''), also called '''mucoviscidosis''', is an autosome recessive hereditary disease that affects the lungs, sweat glands and the digestive system.
Alice Martineau - Official site. Photos, forum, her music, and information about cystic fibrosis, from which she died.
The Telegraph: Alice Martineau - 'Pop singer dies after valiant battle with cystic fibrosis'. Includes details of her daily routine of oxygen, chest physiotherapy, intravenous antibiotics and taking 40 pills a day.
Hanson Music House - Supports fans with Cystic Fibrosis. Lyrics, fun, links and campaigns.
Cff.org: Special People - Salutes Cochran for her support of the Cystic Fibrosis Foundation. (April 2001)
HHCS Health Group - A full-service pharmacy, disease management and medical management company encompassing the cystic fibrosis pharmacy, medi-paws pet pharmacy, and a vitamin and supplements pharmacy program for homeopathic medicine.
Howard Hughes Medical Institute - HHMI investigators have been involved in many important recent advances, from the discovery of genes related to cystic fibrosis, obesity, and other diseases, to new insights about how human cells develop or communicate.
Genentech - Uses human genetic information to develop and manufacture pharmaceuticals such as growth hormones, tissue-plasminogen activators to dissolve blood clots, and cystic fibrosis therapeutics.
Thorpe, P. H. - Studies of Cystic Fibrosis gene therapy. From the University of Edinburgh in Scotland.
Proteome Systems Ltd. - Specializes in interpretation of 2-dimensional gels, and developing point-of-care diagnostics in the areas of cystic fibrosis, cancer, infectious disease and aging. Includes alpha-amylase test, and research programs, with contacts in USA, Japan and headquarters in Sydney, Australia.
Howard Hughes Medical Institute - Biomedical research, grants. HHMI investigators contributed to the discovery of genes related to diseases such as cystic fibrosis and obesity, and to basic understanding of cell development and communication. Scientists are employed by HHMI and conduct their work at HHMI facilities at leading research institutions nationwide. Web site has extensive news and information on recent research findings.
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