| |
cystic fibrosis
a disorder associated with widespread dysfunction of the exocrine glands, with accumulation of excessively thick and tenacious mucus and abnormal secretion of sweat and saliva. It is inherited as an autosomal recessive trait; both parents must be carriers. The cause is thought to be absence, insufficiency,
or abnormality of some essential hormone or enzyme. The symptoms and severity vary widely. Although cystic fibrosis is congenital,
it may not manifest itself significantly during the early weeks or months of life, or it may cause intestinal obstruction
and perforation in the newborn. Normal mucus bathes and protects internal surfaces, transports chemicals produced in one organ
through intricate small ducts to another organ, and carries bacteria, dirt, and wastes to be eliminated from the body; thus
it needs to flow easily. The mucus of cystic fibrosis, in contrast, is highly adhesive. Bacteria and other matter stick to
it, and it in turn can clog the lungs and interfere with the flow of digestive enzymes from the pancreas to the small intestine.
Patients have breathing difficulties and problems of digestion, especially of fats.
Copyright 2007. An Elsevier publication. All rights reserved.
Click here for important legal information about Dorland's Medical Dictionary.
|
