title: Clinical Outcomes and Prognostic Factors in a Cohort of Adults With Cystic Fibrosis: A 7-Year Follow-Up Study.
abstract: BACKGROUND
  Due to the heterogeneity of cystic fibrosis (CF), the longer survival observed in cohorts of adult subjects, and inter-population variations, there is a clear need to seek further information about clinical outcomes and prognostic factors in different cohorts of subjects with CF. Our objectives were to evaluate clinical outcomes and prognostic factors in a cohort of adult subjects with CF after a 7-y follow-up period and investigate longitudinal changes in clinical scores, spirometry, 6-min walk test performance, and pulmonary artery systolic pressure as assessed by Doppler echocardiography.
  
  
  METHODS
  A cohort of clinically stable subjects (≥16 y old) who were enrolled in an adult CF program in 2004-2005 underwent clinical evaluation. Outcome was classified as good (survival) or poor (survival with lung transplantation or death). In 2011-2012, survivors were re-examined.
  
  
  RESULTS
  Of 40 subjects with CF evaluated in 2004-2005, 32 (80%) survived, 2 (5%) survived with lung transplantation, and 6 (15%) died. Logistic regression analysis showed that a low percent-of-predicted FEV1 was associated with poor outcome. An FEV1 cut-off value of ≤30% and pulmonary artery systolic pressure of ≥42 mm Hg predicted poor outcome with high sensitivity, specificity, and positive and negative predictive values. Deterioration was observed in clinical scores (P = .03), FVC (P = .02), FEV1 (P < .001), distance walked in the 6-min walk test (P = .002), baseline SpO2 (P < .001), and final SpO2 (P < .001).
  
  
  CONCLUSIONS
  After 7 y of follow-up, 20% of subjects with CF had a poor outcome. Pulmonary artery systolic pressure of ≥42 mm Hg and FEV1 of ≤30% were the most significant prognostic predictors of poor outcome. Clinical and functional deterioration was observed in survivors.
authors.affiliation: Postgraduate Program Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Brazil. josiflores@hotmail.com.
authors.forename: Josani Silva
authors.initials: JS
authors.lastname: Flores
authors.affiliation: Postgraduate Program Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Brazil.
authors.forename: Paula Maria Eidt
authors.initials: PM
authors.lastname: Rovedder
authors.affiliation: Postgraduate Program in Ciências Pneumológicas, Hospital de Clínicas de Porto Alegre, Brazil.
authors.forename: Bruna
authors.initials: B
authors.lastname: Ziegler
authors.affiliation: Serviço de Cardiologia, Hospital de Clínicas de Porto Alegre, Brazil.
authors.forename: Antônio Fernando Furlan
authors.initials: AF
authors.lastname: Pinotti
authors.affiliation: Universidade Federal do Rio Grande do Sul, Brazil.
authors.forename: Sérgio Saldanha Menna
authors.initials: SS
authors.lastname: Barreto
authors.affiliation: Postgraduate Program Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Brazil. Serviço de Pneumologia, Hospital de Clínicas de Porto Alegre, Brazil.
authors.forename: Paulo de Tarso Roth
authors.initials: Pde T
authors.lastname: Dalcin
mesh_terms: D000328:Adult; D001794:Blood Pressure; D003550:Cystic Fibrosis / Q000401:mortality / Q000503:physiopathology* / Q000601:surgery; D015150:Echocardiography, Doppler; D005080:Exercise Test / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D006801:Humans; D016015:Logistic Models; D008137:Longitudinal Studies; D008168:Lung / Q000503:physiopathology; D016040:Lung Transplantation / Q000401:mortality; D008297:Male; D011237:Predictive Value of Tests; D011379:Prognosis; D011651:Pulmonary Artery / Q000000981:diagnostic imaging; D012680:Sensitivity and Specificity; D013147:Spirometry; D017741:Survivors; D016138:Walking; D055815:Young Adult
keywords: cohort studies; cystic fibrosis; lung function; prognosis; pulmonary hypertension