Cystic fibrosis: a review of pulmonary and nutritional therapies.
In summary, there is a significant interplay between the pulmonary manifestations and nutritional status of CF patients. The advances in CF clinical care in the past 2 decades are mainly attributed to anti-infective therapy as well as aggressive nutritional management. Currently, there are multiple therapeutic agents that are in clinical trial that target either the underlying CFTR defect or the downstream effects of CFTR. The broad spectrum of therapeutic agents being studied as well as the advances in therapies that target the underlying CFTR defect are exciting, making it likely that at least one of the treatments will make a major difference in how we will treat CF in the future.
Amin
Reshma
R
Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Ratjen
Felix
F
D000890:Anti-Infective Agents / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D003920:Diabetes Mellitus / Q000503:physiopathology; D004234:Diuretics, Osmotic / Q000627:therapeutic use; D005100:Expectorants / Q000627:therapeutic use; D015316:Genetic Therapy; D006801:Humans; D007249:Inflammation / Q000378:metabolism; D008168:Lung / Q000503:physiopathology; D044342:Malnutrition / Q000503:physiopathology / Q000517:prevention & control; D008353:Mannitol / Q000627:therapeutic use; D009504:Neutrophils / Q000378:metabolism; D012141:Respiratory Tract Infections / Q000209:etiology
D000890:Anti-Infective Agents; D000893:Anti-Inflammatory Agents; C505032:CFTR protein, human; D004234:Diuretics, Osmotic; D005100:Expectorants; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D008353:Mannitol