[Mucoviscidosis: Background, risk factors, diagnosis strategy, treatment principles].
Known as a systemic disease, genetically conditioned, mucoviscidosis or cystic fibrosis brings together chronic obstructive bronchopathy, manifested since early childhood, chronic bronchial infection especially with pseudomonas or staphylococcus aureus, pancreatic impairment and a high clorum ratio in sweats. Cystic fibrosis transmembrane conductance regulator is inducing the disease, 1 of 20 caucasians being carriers of this marker. Positive diagnosis is suggested when respiratory and digestive symptoms meet fertility disfunction and "salty kiss" due to sodium--clorure disorders and confirmed by chest X-ray, spirogram, bacteriologic exam of sputum, genetic analysis and positive tests of the pancreatic enzymes and sweat's test. As regards therapeutical solutions, besides antibiotics required in infectious exacerbation, corticotherapy, kinesitherapy and alpha dornasis given in aerosols to improve bronchial mucosa's clearance, also substitutes of pancreatic enzymes and hypercaloric diet are to be considered. In the last two decades, lung transplant appeared to be as an alternative to improve poor prognosis of this disease. Recent studies reported that life expectancy in cystic fibrosis will increase from 28 years in the '90s to 40 years in the next 20 years.
Trofor
Antigona
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Disciplina de Pneumoftiziologie, Facutatea de Medicină Stomatologică, Universitatea de Medicină şi Farmacie Gr.T. Popa Iaşi.
D003550:Cystic Fibrosis / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D006801:Humans; D011379:Prognosis; D012307:Risk Factors; D013183:Sputum / Q000737:chemistry; D013542:Sweat / Q000737:chemistry