CYSTIC-FIBROSIS

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Abstract
It appears that cystic fibrosis is transmitted as an autosomal recessive trait possibly by single mutant allele, although no single lesion has been proved to provide a unifying hypothesis for the pathophysiology of this syndrome. The pathophysiology in humans consists of: an exocrine gland dysfunction, failure of mucociliary transport, development of chronic respiratory infection, presence of a factor that disrupts normal ciliary pattern and alterations of transport mechanisms. The manifestation of cystic fibrosis involves the respiratory, gastrointestinal, genitourinary systems and sweat glands; a pattern of presentation is variable. Diagnosis of cystic fibrosis involves a positive sweat test evaluation of pancreatic function and detection of defective genes. Because of the multiple system involvement, a comprehensive care program should integrate pulmonary and gastrointestinal therapy. The complications, treatment and psychosocial aspects of cystic fibrosis were discussed.

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