Fibrosis quística en el adulto: experiencia de un centro de referencia nacional

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 patien...

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Autores principales:	Fernández,Patricia, Labarca,Gonzalo
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2012
Materias:	Bronchiectasis Cystic fibrosis Hemoptysis Pseudomonas aeruginosa
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872012000700002
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spelling	oai:scielo:S0034-988720120007000022012-10-22Fibrosis quística en el adulto: experiencia de un centro de referencia nacionalFernández,PatriciaLabarca,Gonzalo Bronchiectasis Cystic fibrosis Hemoptysis Pseudomonas aeruginosa 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 AF508. Among clinical characteristics, lung involvement, mainly bronchiectasis, was found in 93%. The mean forced expiratory volume in the first second (FEV,) 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 AF508 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.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.140 n.7 20122012-07-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872012000700002es10.4067/S0034-98872012000700002
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Bronchiectasis Cystic fibrosis Hemoptysis Pseudomonas aeruginosa
spellingShingle	Bronchiectasis Cystic fibrosis Hemoptysis Pseudomonas aeruginosa Fernández,Patricia Labarca,Gonzalo Fibrosis quística en el adulto: experiencia de un centro de referencia nacional
description	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 AF508. Among clinical characteristics, lung involvement, mainly bronchiectasis, was found in 93%. The mean forced expiratory volume in the first second (FEV,) 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 AF508 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.
author	Fernández,Patricia Labarca,Gonzalo
author_facet	Fernández,Patricia Labarca,Gonzalo
author_sort	Fernández,Patricia
title	Fibrosis quística en el adulto: experiencia de un centro de referencia nacional
title_short	Fibrosis quística en el adulto: experiencia de un centro de referencia nacional
title_full	Fibrosis quística en el adulto: experiencia de un centro de referencia nacional
title_fullStr	Fibrosis quística en el adulto: experiencia de un centro de referencia nacional
title_full_unstemmed	Fibrosis quística en el adulto: experiencia de un centro de referencia nacional
title_sort	fibrosis quística en el adulto: experiencia de un centro de referencia nacional
publisher	Sociedad Médica de Santiago
publishDate	2012
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872012000700002
work_keys_str_mv	AT fernandezpatricia fibrosisquisticaeneladultoexperienciadeuncentrodereferencianacional AT labarcagonzalo fibrosisquisticaeneladultoexperienciadeuncentrodereferencianacional
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Fibrosis quística en el adulto: experiencia de un centro de referencia nacional

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