title: [Cystic fibrosis in adults--clinical aspects].
abstract: Nineteen patients with cystic fibrosis were seen in the I Department of Tuberculosis and Lung Diseases during 3.5 years. There were 12 (63%) female, and 7 male, aged from 16 to 35 years (mean 23.2). Most patients were diagnosed in childhood, but 4 were diagnosed in their early adulthood. The diagnosis was confirmed by positive chloride sweat test in all cases. Molecular DNA analyses were performed in 16 cases. In 9 (56%) cases two mutations in the CFTR gene were identified. In 5 cases one mutation was identified. All patients had bronchiectases confirmed by CT. Spirometry showed lung function impairment with predominantly obstructive pattern. Mean VC was 2.57l, mean FEVI was 1.66l. In 7 (37%) cases FEVI was lower then 30% of predictive value. Hypoxemia was found in 11 (58%) cases and hypercapnia in 3 (16%) cases. Sputum cultures were positive for mucoid P. aeruginosa in 12 (63%) cases, for Staph. aureus in 16 (84%) cases. Persistent colonisation with nontuberculous mycobacteria was found in 2 (10.5%) cases. Aspergillus fumigatus was identified in sputum cultures in 2 subjects who had also positive precipitation test. Diabetes mellitus was diagnosed in 2 cases. Meconium ileus equivalent was seen in 1 case. Pneumothorax was seen in 1 case. One patient died in the endstage of the illness.
authors.affiliation: I Kliniki Gruźlicy i Chorób Płuc Instytutu, Gruźlicy i Chorób Płuc w Warszawie.
authors.forename: W
authors.initials: W
authors.lastname: Skorupa
authors.forename: M
authors.initials: M
authors.lastname: Wierzbicka
authors.forename: J
authors.initials: J
authors.lastname: Kuś
mesh_terms: D000293:Adolescent; D000328:Adult; D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000209:etiology; D002712:Chlorides / Q000032:analysis; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis*; D004247:DNA / Q000032:analysis; D005260:Female; D006801:Humans; D008297:Male; D009154:Mutation; D009161:Mycobacterium / Q000302:isolation & purification; D011550:Pseudomonas aeruginosa / Q000302:isolation & purification; D012129:Respiratory Function Tests; D013183:Sputum / Q000382:microbiology; D013211:Staphylococcus aureus / Q000302:isolation & purification; D013542:Sweat / Q000737:chemistry; D014057:Tomography, X-Ray Computed
chemical_list: D002712:Chlorides; D004247:DNA
vernacular_title: Mukowiscydoza u dorosłych--obraz kliniczny.