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[Mucoviscidosis in adults].
1Service de Pneumologie et centre de soins de mucoviscidose, Hôpital Foch, 40, rue Worth, 92150 Suresnes. m.stern@hopital-foch.org
Presse Medicale (Paris, France : 1983)
|March 9, 2002
Summary
Cystic fibrosis, once a pediatric illness, is now increasingly seen in adults due to improved therapies. Management focuses on respiratory symptoms and Pseudomonas aeruginosa infections to enhance adult patient quality of life.
Area of Science:
- Genetics and Medicine
- Pulmonology
Context:
- Cystic fibrosis (mucoviscidosis) is an autosomal recessive genetic disorder.
- Therapeutic advancements have extended patient survival, leading to a growing adult patient population.
Purpose:
- To describe the clinical presentation of cystic fibrosis in adults.
- To outline current diagnostic approaches and therapeutic strategies for adult cystic fibrosis patients.
Summary:
- Adult cystic fibrosis primarily manifests with respiratory symptoms, including chronic bronchial Pseudomonas aeruginosa colonization, progressing to respiratory failure.
- Diagnosis involves identifying organ damage, CFTR gene mutations, and abnormal sweat tests or nasal potential difference.
- Treatment focuses on respiratory physical therapy, antibiotic management for infections, and considering lung transplantation for advanced disease.
Impact:
- Improved understanding of adult cystic fibrosis facilitates tailored treatment strategies.
- Enhanced management of respiratory complications and infections can improve patient quality of life.
- Genetic diagnosis allows for identification of milder or monosymptomatic forms.