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 clini
cal 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, Di
agnosis was suspected because of pulmonary (2/3) or digestive (1/3) sy
mptoms, 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 we
re observed in 95% of patients and 62% have chronically infected sputu
m with Pseudomonas aeruginosa. These patients had lower weight and a p
oorer radiological score than patients without pseudomonas. 25% of all
patients had chronic respiratory insufficiency, 75% had pancreatic in
sufficiency and 6 patients diabetes mellitus, Thirteen patients had bi
ological cholestasis and three a liver cirrhosis with portal hypertens
ion. 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 pu
lmonary transplantation (2), and because of digestive haemorrhage (1),
Conclusion: Treatment included daily bronchial drainage, adapted antib
iotic treatment and pancreatic enzyme substitution.