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[Cystic fibrosis in adulthood].
1Centre mucoviscidose, service de médecine interne, pavillon Giraud 1 K, 69495 Pierre-Bénite, France. isabelle.durieu@chu-lyon.fr
La Revue De Medecine Interne
|January 25, 2002
Summary
Cystic fibrosis (CF), a genetic disorder, increasingly affects adults due to longer lifespans. Diagnosis in adults involves recognizing specific symptoms like bronchiectasis and pancreatic issues, confirmed by sweat tests or genetic analysis.
Area of Science:
- Genetics and Molecular Biology
- Pulmonology
- Endocrinology
Context:
- Cystic fibrosis (CF) is an autosomal recessive genetic disorder.
- Increased life expectancy has led to a growing adult CF population.
- Adult CF patients often present with distinct complications.
Purpose:
- To describe the clinical presentation and diagnostic criteria for cystic fibrosis in adult patients.
- To highlight the shift in CF demographics towards adulthood.
- To inform healthcare providers about adult-onset CF manifestations.
Summary:
- Adult CF patients commonly exhibit diffuse bronchiectasis, chronic Pseudomonas aeruginosa colonization, and pancreatic insufficiency.
- Comorbidities include diabetes mellitus in one-third of adult cases.
- Diagnostic clues in adults include male infertility, chronic sinusitis, pancreatitis, and allergic bronchopulmonary aspergillosis.
- Confirmation relies on sweat chloride concentration or CFTR gene mutation analysis.
Impact:
- Improved recognition of adult CF can lead to earlier diagnosis and intervention.
- Understanding adult-specific complications aids in tailored patient management.
- This demographic shift necessitates adapted clinical guidelines and research focus.