title: Early lung disease in cystic fibrosis.
abstract: Lung disease in patients with cystic fibrosis is characterised by inflammation and recurrent and chronic infections leading to progressive loss in pulmonary function and respiratory failure. Early management of disease results in substantially improved pulmonary function at first testing (at roughly 6 years of age), but the annual decline in pulmonary function tests in older patients has remained unchanged showing how important the early years are in the disease process. Treatment regimens for patients with cystic fibrosis have changed from predominantly symptomatic treatment to preventive or causal (ie, treatments that address the underlying mechanisms of disease) therapeutic interventions. The infant and preschool age (2-5 years) could represent a unique period of opportunity to postpone or even prevent the onset of cystic fibrosis lung disease. We summarise the current knowledge and the methods used to characterise and quantify early lung disease. We discuss treatment strategies including new drugs that are being developed and their potential role in the treatment of early lung disease in patients with cystic fibrosis.
authors.affiliation: Division of Respiratory Medicine, Department of Paediatrics, and Programme in Physiology and Experimental Medicine, Research Institute, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
authors.forename: Hartmut
authors.initials: H
authors.lastname: Grasemann
authors.affiliation: Division of Respiratory Medicine, Department of Paediatrics, and Programme in Physiology and Experimental Medicine, Research Institute, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. Electronic address: felix.ratjen@sickkids.ca.
authors.forename: Felix
authors.initials: F
authors.lastname: Ratjen
mesh_terms: D000328:Adult; D056151:Airway Remodeling / Q000187:drug effects; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D019468:Disease Management; D018450:Disease Progression; D015507:Drugs, Investigational / Q000494:pharmacology; D061345:Early Medical Intervention / Q000379:methods; D006801:Humans; D007223:Infant; D007249:Inflammation / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D008171:Lung Diseases / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D012129:Respiratory Function Tests / Q000379:methods; D019141:Respiratory System Agents / Q000494:pharmacology*; D012141:Respiratory Tract Infections / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology
chemical_list: D000900:Anti-Bacterial Agents; D015507:Drugs, Investigational; D019141:Respiratory System Agents