Cystic fibrosis in adults

Objectives: At least half cystic fibrosis patients now reach adulthood. Methods: We report a population of 61 patients above 18 years of age with the clinical pictures at time of diagnosis and the present clinical status, Results: Thirty-five males and 26 females are aged from 18 to 47 years, Mean age at time of diagnosis was 5 years and 5 months, under 10 years in 80% of patients and above 15 years in 9 patients, Diagnosis was suspected because of pulmonary (2/3) or digestive (1/3) symptoms, insufficient height and weight (1/3) or past family history of cystic fibrosis (1/3). 37% of patients are homozygotes for Delta F508 mutation, Adult patients had a normal height but half of them a body weight under 90% of expected weight. Recurrent pulmonary infections were observed in 95% of patients and 62% have chronically infected sputum with Pseudomonas aeruginosa. These patients had lower weight and a poorer radiological score than patients without pseudomonas. 25% of all patients had chronic respiratory insufficiency, 75% had pancreatic insufficiency and 6 patients diabetes mellitus, Thirteen patients had biological cholestasis and three a liver cirrhosis with portal hypertension. Four women underwent 6 normal pregnancies; semen analysis in five men revealed aspermia, Seven patients died during the last two years because of respiratory insufficiency (4), in the three months after pulmonary transplantation (2), and because of digestive haemorrhage (1), Conclusion: Treatment included daily bronchial drainage, adapted antibiotic treatment and pancreatic enzyme substitution.