[Patients with cystic fibrosis managed at the cystic fibrosis units of Madrid: cross-sectional study of 387 subjects].
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.
García Hernández
Gloria
G
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
Antelo
Carmen
C
Maiz
Luis
L
Girón
Rosa María
RM
Salcedo
Antonio
A
Martínez Gimeno
Antonio
A
Martínez
María Teresa
MT
Isabel Barrio
María
M
Prados
Concepción
C
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