title: Health-Related Quality of Life in Patients With α1 Antitrypsin Deficency: A Cross Sectional Study.
abstract: BACKGROUND
  Measures of health related quality of life (HRQoL) in patients with α1-antitrypsin deficiency (AATD) can help to determine the impact of the disease and provide an important insight into the intervention outcomes. There is few data regarding this issue in the literature. The aim of this study is to assess the relationship between HRQoL and gender, functional parameters and history of hospitalizations in patients with AATD.
  
  
  METHODS
  This is a cross-sectional study of 26 patients with severe AATD recruited in the pulmonology outpatient clinic at a tertiary care medical center. Social-demographic, clinical and functional parameters were recorded and HRQoL was assessed with the Portuguese version of the medical outcome study short form-36 (SF-36) self-administered questionnaire.
  
  
  RESULTS
  Older patients, females and patients with at least one hospitalization in the previous year due to respiratory disease had statistical lower scores in some dimensions of the SF-36 questionnaire. Superior FEV1 and higher distance mark in the 6-min walking test distance influenced positively several dimensions of the questionnaire. Higher scores in the mMRC scale influenced negatively the HRQoL.
  
  
  CONCLUSIONS
  These data suggests that older and female patients with AATD have worse HRQoL. Hospitalizations and functional markers of respiratory disease progression influenced negatively the HRQoL, suggesting that the SF-36 questionnaire could be useful as an outcome for AATD patients with lung involvement.
authors.affiliation: Pulmonology Department, Centro Hospitalar de São João, Oporto, Portugal. Electronic address: margarida.tredondo@gmail.com.
authors.forename: Margarida
authors.initials: M
authors.lastname: Torres Redondo
authors.affiliation: Oncology Department, Centro Hospitalar do Algarve, Faro, Portugal.
authors.forename: Elsa
authors.initials: E
authors.lastname: Campoa
authors.affiliation: Department of Clinical Epidemiology, Predictive Medicine and Public Health, University of Porto Medical School, Oporto, Portugal; EPIUnit, Institute of Public Health, University of Porto, Oporto, Portugal.
authors.forename: Luis
authors.initials: L
authors.lastname: Ruano
authors.affiliation: Pulmonology Department, Centro Hospitalar de São João, Oporto, Portugal.
authors.forename: Maria
authors.initials: M
authors.lastname: Sucena
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D000368:Aged; D001993:Bronchodilator Agents / Q000627:therapeutic use; D003430:Cross-Sectional Studies; D005080:Exercise Test; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008297:Male; D008875:Middle Aged; D011174:Portugal / Q000453:epidemiology; D011788:Quality of Life; D057566:Self Report; D012737:Sex Factors; D012907:Smoking / Q000453:epidemiology; D011795:Surveys and Questionnaires; D019896:alpha 1-Antitrypsin Deficiency / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics / Q000523:psychology*
chemical_list: D000305:Adrenal Cortex Hormones; D001993:Bronchodilator Agents
keywords: Calidad de vida relacionada con la salud; Cuestionario SF-36; Déficit de α1-antitripsina; Health related quality of life; SF-36 questionnaire; α1-antitrypsin deficiency