Cystic fibrosis - characterization of the adult population in Portugal
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| Natura: | Artículo científico |
| Lingua: | en |
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Sociedade Portuguesa de Pneumologia
2016
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| _version_ | 1876479522866462720 |
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| author | A. Silva |
| author_facet | A. Silva |
| contents | Cystic fibrosis - characterization of the adult population in Portugal A. Silva A. Amorim P. Azevedo C. Lopes F. Gamboa Medicina F508del Adulthood Cystic fibrosis Introduction: The incidence of cystic fibrosis (CF) in Portugal is estimated at 1:8000 live births, although there is a lack of accurate statistics. The average life expectancy has been steadily increasing and CF is no longer an exclusively pediatric disease. Objectives: Characterize the Portuguese adult population with the diagnosis of CF. Methods: Retrospective study based on clinical data of adult CF follow-up patients in the three specialized centers in Portugal where all of CF patients are seen, during 2012. Results: In 2012, there were 89 follow-up patients, 48 (54%) female and 15 (17%) lung transplanted. The average age was 31.3 ± 9 years. The median age at diagnosis was 13 years and 34 (38%) were diagnosed in adulthood. The most frequent mutation was F508del (54.9%). Of the 89 patients, 49 patients (56%) had pancreatic insufficiency, 7 (9%) were diabetic and 42 patients (47.7%) had a body mass index (BMI) <20 kg/m2. As to ventilatory function, the average value of the forced expiratory volume in 1 s (FEV1) was 58.45 ± 28.59%. Only one of 77 patients did not have chronic airway infection. The most commonly isolated germ was methicillin-sensitive Staphylococcus aureus in 49 patients (55%). During 2012, two patients (2.2%) died at the ages of 21 and 36 years. Discussion: This study is the first description of the Portuguese adult CF population, which is particularly important since it can give us a better understanding of the real situation. A significant percentage of these patients were diagnosed in adulthood, which highlights the need for diagnostic suspicion in a patient with chronic lung disease and atypical manifestations. 2016 artículo científico 0873-2159 https://www.redalyc.org/articulo.oa?id=169747615002 en http://www.redalyc.org/revista.oa?id=1697 Revista Portuguesa de Pneumología application/pdf Sociedade Portuguesa de Pneumologia Revista Portuguesa de Pneumología (Portugal) Num.3 Vol.22 |
| format | Artículo científico |
| id | redalyc_169747615002 |
| institution | Redalyc |
| language | en |
| publishDate | 2016 |
| publisher | Sociedade Portuguesa de Pneumologia |
| spellingShingle | Cystic fibrosis - characterization of the adult population in Portugal A. Silva Medicina F508del Adulthood Cystic fibrosis Cystic fibrosis - characterization of the adult population in Portugal A. Silva A. Amorim P. Azevedo C. Lopes F. Gamboa Medicina F508del Adulthood Cystic fibrosis Introduction: The incidence of cystic fibrosis (CF) in Portugal is estimated at 1:8000 live births, although there is a lack of accurate statistics. The average life expectancy has been steadily increasing and CF is no longer an exclusively pediatric disease. Objectives: Characterize the Portuguese adult population with the diagnosis of CF. Methods: Retrospective study based on clinical data of adult CF follow-up patients in the three specialized centers in Portugal where all of CF patients are seen, during 2012. Results: In 2012, there were 89 follow-up patients, 48 (54%) female and 15 (17%) lung transplanted. The average age was 31.3 ± 9 years. The median age at diagnosis was 13 years and 34 (38%) were diagnosed in adulthood. The most frequent mutation was F508del (54.9%). Of the 89 patients, 49 patients (56%) had pancreatic insufficiency, 7 (9%) were diabetic and 42 patients (47.7%) had a body mass index (BMI) <20 kg/m2. As to ventilatory function, the average value of the forced expiratory volume in 1 s (FEV1) was 58.45 ± 28.59%. Only one of 77 patients did not have chronic airway infection. The most commonly isolated germ was methicillin-sensitive Staphylococcus aureus in 49 patients (55%). During 2012, two patients (2.2%) died at the ages of 21 and 36 years. Discussion: This study is the first description of the Portuguese adult CF population, which is particularly important since it can give us a better understanding of the real situation. A significant percentage of these patients were diagnosed in adulthood, which highlights the need for diagnostic suspicion in a patient with chronic lung disease and atypical manifestations. 2016 artículo científico 0873-2159 https://www.redalyc.org/articulo.oa?id=169747615002 en http://www.redalyc.org/revista.oa?id=1697 Revista Portuguesa de Pneumología application/pdf Sociedade Portuguesa de Pneumologia Revista Portuguesa de Pneumología (Portugal) Num.3 Vol.22 |
| title | Cystic fibrosis - characterization of the adult population in Portugal |
| topic | Medicina F508del Adulthood Cystic fibrosis |
| url | https://www.redalyc.org/articulo.oa?id=169747615002 |