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[Cystic fibrosis in adults]
I Durieu1, G Bellon, D Vital Durand
1Service de Médecine interne, Centre Hospitalier Lyon-Sud, Pierre-Bénite.
Presse Medicale (Paris, France : 1983)
|December 16, 1995
Summary
Cystic fibrosis (CF) patients reaching adulthood often experience chronic pulmonary infections, particularly Pseudomonas aeruginosa, and significant pancreatic insufficiency. Management focuses on airway clearance, antibiotics, and enzyme replacement therapy.
Area of Science:
- Pulmonology
- Genetics
- Internal Medicine
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs.
- An increasing number of CF patients are surviving into adulthood.
Purpose of the Study:
- To describe the clinical characteristics and current status of adult CF patients.
- To analyze the long-term outcomes and complications in this growing population.
Main Methods:
- Retrospective analysis of 61 adult CF patients (age > 18).
- Evaluation of clinical presentation at diagnosis and current health status.
- Assessment of pulmonary, digestive, and other organ system involvement.
Main Results:
- Most patients diagnosed before age 10, with 37% carrying the delta F508 mutation.
- High prevalence of chronic pulmonary infections (95%) and Pseudomonas aeruginosa (62%), associated with poorer outcomes.
- Significant rates of chronic respiratory insufficiency (25%), pancreatic insufficiency (75%), and diabetes mellitus (6 patients).
Conclusions:
- Adult CF patients face significant challenges, including chronic infections and organ dysfunction.
- Treatment strategies involve daily bronchial drainage, tailored antibiotics, and pancreatic enzyme substitution.
- Despite complications, some women experience normal pregnancies, though male fertility is often impaired.