Cystic fibrosis in adults. From researcher to practitioner.
The Cystic Fibrosis Foundation currently tracks about 20,000 Americans with cystic fibrosis, an autosomal recessive genetic disease that leads to multisystem complications. With the institution of better therapeutic regimens over the past 2 decades, more patients with this disease are surviving to adulthood. Within the past decade, both clinical and basic science research in the field of cystic fibrosis has progressed at a rapid rate. The intent of this review is to introduce readers to the molecular, cellular, and systemic disorders of this disease. We discuss treatment strategies involving antibiotics, nutrition, immune-response mediators, chest physiotherapy, and sputum-active agents with respect to the airway dysfunction of cystic fibrosis. Other common complications, recent developments, transplantation, and gene therapy are also reviewed.
Marelich
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GP
Dept of Internal Medicine, UC Davis School of Medicine, Sacramento, 95817, USA.
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D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000150:complications / Q000235:genetics* / Q000276:immunology / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005081:Exercise Therapy; D005260:Female; D018014:Gene Transfer Techniques; D015316:Genetic Therapy; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D013183:Sputum / Q000187:drug effects
D000900:Anti-Bacterial Agents; C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator