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Cystic fibrosis in adults: Experience of a national reference center Fibrosis quística en el adulto: Experiencia de un centro de referencia nacional
Indexado
WoS WOS:000308280100002
Scopus SCOPUS_ID:84868308077
SciELO S0034-98872012000700002
DOI 10.4067/S0034-98872012000700002
Año 2012
Tipo artículo de investigación

Citas Totales

Autores Afiliación Chile

Instituciones Chile

% Participación
Internacional

Autores
Afiliación Extranjera

Instituciones
Extranjeras


Abstract



Background: Although in most patients the diagnosis of cystic fibrosis (CF) is made during their first years of life, a proportion of patients is diagnosed during adulthood. Aim: To describe the features of adult patients diagnosed with CF. Materials and Methods: Analysis of medical records of patients diagnosed with CF in a public hospital devoted to respiratory diseases. Demographic, clinical, laboratory and microbiological data were recorded. Results: Fifty eight patients aged 25.4 +/- 6.5 years were included. In 40% of them, CF was diagnosed after 15 years of age. The most common mutation found was Delta F508. Among clinical characteristics, lung involvement, mainly bronchiectasis, was found in 93%. The mean forced expiratory volume in the first second (FEV1) was 65,7 +/- 27,1%. Fifteen patients were colonized with Pseudomonas aeruginosa. The main complication seen was hemoptysis, in 12% of patients. Five patients died, mostly due to respiratory distress associated with sepsis, while three were subjected to bilateral lung transplantation. Patients in whom the diagnosis of CF was made after 15 years of age, had lower frequency of Delta F508 mutation, were most commonly women and had a lower rate of pancreatic involvement. Conclusions: CF is a disease that is increasingly reaching adult population. CF must be suspected in adolescents and young adults who suffer chronic lung diseases such as bronchiectasis, particularly when they are colonized by Pseudomonas aeruginosa, or in patients who develop infections by uncommon organisms. (Rev Med Chile 2012; 140: 841-846).

Revista



Revista ISSN
Revista Médica De Chile 0034-9887

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Disciplinas de Investigación



WOS
Medicine, General & Internal
Scopus
Medicine (All)
SciELO
Health Sciences

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Publicaciones WoS (Ediciones: ISSHP, ISTP, AHCI, SSCI, SCI), Scopus, SciELO Chile.

Colaboración Institucional



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Autores - Afiliación



Ord. Autor Género Institución - País
1 FERNANDEZ-VASQUEZ, PATRICIA Mujer Instituto Nacional del Tórax - Chile
Inst Nacl Torax - Chile
2 LABARCA-TRUCIOS, GONZALO PATRICIO Hombre Clínica Alemana - Chile
Univ Desarrollo Santiago - Chile
Universidad del Desarrollo/Clínica Alemana de Santiago - Chile
Universidad del Desarrollo - Chile

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Financiamiento



Fuente
Sin Información

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Agradecimientos



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