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What is cystic fibrosis?

Cystic fibrosis is a hereditary disease of the exocrine glands, usually developing during early childhood and affecting mainly the pancreas, respiratory system, and sweat glands. It is characterized by the production of abnormally viscous mucus by the affected glands, usually resulting in chronic respiratory infections and impaired pancreatic function. Cystic fibrosis is

also called mucoviscidosis.

Cystic fibrosis affects the pancreas, which normally produces enzymes needed to break down food during digestion, and also affects the glands that secrete sweat and mucus. As a result, the child with cystic fibrosis is unable to digest fats, and the sweat becomes abnormally salty. In addition, the child's mucus becomes very thick and sticky, blocking the air passages to the lungs. Respiratory infections occur frequently, often with a cough and fever, making the lungs susceptible to pneumonia. Malnutrition is common in children with cystic fibrosis, because few nutrients are absorbed.

Cystic fibrosis is an inherited disorder, mostly of young children. Sometimes the disease is not diagnosed until adolescence or adulthood. People with cystic fibrosis secrete very thick mucus from the windpipe, or tracheobronchial glands. They also have abnormal secretions of sweat and saliva. The pancreas may release enzymes improperly, a condition called pancreatic insufficiency. The lungs have changes similar to those in emphysema.

Cystic fibrosis is an inherited disease of the mucus glands. It causes chronic, progressive damage to the respiratory system, chronic digestive system problems, and can affect other organs. The signs and symptoms of this disorder are caused by the production of abnormally thick, sticky mucus in the body's organs. Specific cells in these organs normally produce mucus and other watery secretions. In cystic fibrosis, these cells produce secretions that are thicker than normal, causing disruptions in the body's water balance and ability to handle salt. Problems with breathing are among the most serious symptoms. Mucus can obstruct the airways and cause bacterial infections in the lungs, leading to chronic coughing, wheezing, and inflammation. Over time, mucus buildup and infections lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs.

Most people with cystic fibrosis also have digestive problems. Mucus can block the ducts of the pancreas, preventing enzymes produced by that organ from reaching the intestines to help digest food. In the pancreas, thickened secretions block the normal flow of pancreatic juices, making it more difficult for the body to digest and absorb fats and fat-soluble vitamins. This can result in nutritional problems, especially in babies. Other problems related to cystic fibrosis include sinusitis, nasal polyps, esophagitis, pancreatitis, cirrhosis of the liver, rectal prolapse, diabetes and infertility, especially in males. Problems with digestion can lead to diarrhea, malnutrition, and weight loss. Some babies with cystic fibrosis have meconium ileus, a blockage of the intestine that occurs shortly after birth.

Different people have different levels of abnormalities in cystic fibrosis. Some people are very ill when they are quite young. Others can live many years with mild abnormalities. Half of males with cystic fibrosis now live to be over 30 years old. Half of females with cystic fibrosis survive beyond 28 years. Infertility, or the inability to conceive a child, is common in men with cystic fibrosis, but infrequent in women with the condition. Cystic fibrosis is the most common serious genetic disease among Caucasian children. It occurs in about 1 of every 2,500 live births. The disease almost always occurs in Caucasians, and is equally common in males and females. It is chiefly a disease of infants and children, but more adult cases are being diagnosed.

More information on cystic fibrosis

What is cystic fibrosis? - Cystic fibrosis is a hereditary disease of the exocrine glands. Cystic fibrosis affects the pancreas and the glands that secrete sweat and mucus.
What causes cystic fibrosis? - Cystic fibrosis is caused by a defect in the person's genes. The defective gene that is responsible for causing cystic fibrosis is on chromosome 7.
What're the complications of cystic fibrosis? - Most people with cystic fibrosis also have digestive problems. People with cystic fibrosis have an increased risk of lung infections.
What're the symptoms of cystic fibrosis? - The symptoms of cystic fibrosis usually develop during early childhood. Cystic fibrosis is characterized by the production of very thick and sticky mucus.
How is cystic fibrosis diagnosed? - Family history, persistent respiratory disease, or clinical evidence of pancreatic insufficiency may suggest the diagnosis of cystic fibrosis.
What's the treatment for cystic fibrosis? - Treatment for cystic fibrosis consists of the intake of digestion enzymes, nutritional supplements, percussion and postural drainage of the lungs, improved antibiotics.
What cystic fibrosis medications are available? - Cystic fibrosis medications include H2 receptor antagonists, oral corticosteroids, ibuprofen, erythromycin, amoxicillin-clavulanate, etc.
What cystic fibrosis diet is suggested? - People with cystic fibrosis usually require high-calorie diets and vitamin supplements. Most people with cystic fibrosis need to take pancreatic enzymes.
What's the gene therapy for cystic fibrosis? - Gene therapy for cystic fibrosis typically aims to supplement a defective mutant allele with a functional one.
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