title: [Patients with cystic fibrosis managed at the cystic fibrosis units of Madrid: cross-sectional study of 387 subjects].
abstract: OBJECTIVE
  Our objective was to describe the clinical and genetics features of patients with cystic fibrosis (CF) attended in Madrid.
  
  
  METHODS
  This was a descriptive, cross-sectional study of CF patients attended during 2001. Demographic, genetic, anthropometric, pancreatic insufficiency, diabetes mellitus, Pseudomonas aeruginosa colonization, lung function and body mass index (BMI) (z score) data were recorded and compared with the American CF Registry.
  
  
  RESULTS
  387 patients were included, most of them living in Madrid (n = 247 [63%]), 209 males (54%), with a mean age (SD) of 15.15 (10.42) years (younger than 18 years, 248, 64.24%). F508del was the most common mutation (52.8% of chromosomes) with 104 homozygous patients. Pancreatic insufficiency was present in 310 subjects (80.1%), diabetes mellitus in 30 (7.8%) and P. aeruginosa colonization in 126 (33.1%). Lung function was measured in 309 patients: mean of FEV1 and FVC predicted values (SD) was 82.5 (27.11) and 89.32 (21.89), respectively. The mean BMI z score was 0.0796 (1.18).
  
  
  CONCLUSIONS
  CF patients from Madrid have a good nutritional status, less P. aeruginosa colonization, less pancreatic insufficiency and better lung function than those of the American CF Registry. The lower prevalence of homozygous F508del in our population may explain, at least partly, our findings.
authors.affiliation: Unidad de Fibrosis Quística, Hospital Universitario 12 de Octubre, Edificio Materno-Infantil, planta 6aA, Avda. de Córdoba, s/n, 28041 Madrid, Spain. gloriagh@eresmas.net
authors.forename: Gloria
authors.initials: G
authors.lastname: García Hernández
authors.forename: Carmen
authors.initials: C
authors.lastname: Antelo
authors.forename: Luis
authors.initials: L
authors.lastname: Maiz
authors.forename: Rosa María
authors.initials: RM
authors.lastname: Girón
authors.forename: Antonio
authors.initials: A
authors.lastname: Salcedo
authors.forename: Antonio
authors.initials: A
authors.lastname: Martínez Gimeno
authors.forename: María Teresa
authors.initials: MT
authors.lastname: Martínez
authors.forename: María
authors.initials: M
authors.lastname: Isabel Barrio
authors.forename: Concepción
authors.initials: C
authors.lastname: Prados
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy*; D005260:Female; D006757:Hospital Units / Q000706:statistics & numerical data; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009154:Mutation; D012129:Respiratory Function Tests; D013030:Spain / Q000453:epidemiology