cystic fibrosis (CF)

or mucoviscidosis

Inherited 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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Fibrosis is the formation or development of excess fibrous connective tissue in an organ or tissue as a reparative or reactive process, as opposed to a formation of fibrous tissue as a normal constituent of an organ or tissue.

The term is also sometimes used to describe a normal healing process, but this usage is less common.

Types

• Cystic fibrosis of the pancreas and lungs
• Injection fibrosis, which can occur as a complication of intramuscular injections, especially in children
• Endomyocardial fibrosis
• Idiopathic pulmonary fibrosis of the lung
• Mediastinal fibrosis
• Myleofibrosis
• Retroperitoneal fibrosis
• Progressive massive fibrosis, a complication of coal workers' pneumoconiosis
• Nephrogenic systemic fibrosis

Associated conditions

• Cirrhosis can result from fibrosis of the liver
• Diffuse parenchymal lung disease
• Post-Vasectomy Pain Syndrome
• Tuberculosis (TB) can cause fibrosis of the lungs
• Sickle-cell anemia may cause enlargement and ultimately fibrosis of the spleen.
• Rheumatoid arthritis

References

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