title: [FRENCH CYSTIC FIBROSIS EPIDEMIOLOGY AFTER A DECADE OF NEONATAL SCREENING].
abstract: Since its description in 1938, the life expectancy of cystic fibrosis patients has increased from a few months to nearly 50 years in most Western countries. This significant improvement was related to new symptomatic treatments, for nutritional and respiratory cares in specialized multidisciplinary teams. Systematic neonatal screening for the disease avoides the diagnostic delays that have very deleterious impact on the prognosis of the disease. It allows early optimal management; their nutritional benefit has been demonstrated. The French registry of cystic fibrosis shows that adult patients outnumber children. The median age of death remains under thirty years and the prognosis is very closely linked to the progression chronic respiratory insufficiency. About one hundred patients were annually treated by lung transplant
authors.forename: Isabelle
authors.initials: I
authors.lastname: Durieu
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000453:epidemiology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics*; D005602:France / Q000453:epidemiology; D005820:Genetic Testing; D006801:Humans; D007231:Infant, Newborn; D015997:Neonatal Screening; D055815:Young Adult
chemical_list: C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator