Josani Silva Flores1, Paula Maria Eidt Rovedder2, Bruna Ziegler3, Antônio Fernando Furlan Pinotti4, Sérgio Saldanha Menna Barreto5, Paulo de Tarso Roth Dalcin6. 1. Postgraduate Program Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Brazil. josiflores@hotmail.com. 2. Postgraduate Program Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Brazil. 3. Postgraduate Program in Ciências Pneumológicas, Hospital de Clínicas de Porto Alegre, Brazil. 4. Serviço de Cardiologia, Hospital de Clínicas de Porto Alegre, Brazil. 5. Universidade Federal do Rio Grande do Sul, Brazil. 6. Postgraduate Program Ciências Pneumológicas, Universidade Federal do Rio Grande do Sul, Brazil. Serviço de Pneumologia, Hospital de Clínicas de Porto Alegre, Brazil.
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.
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: Kathleen J Ramos; Patrick J Smith; Edward F McKone; Joseph M Pilewski; Amy Lucy; Sarah E Hempstead; Erin Tallarico; Albert Faro; Daniel B Rosenbluth; Alice L Gray; Jordan M Dunitz Journal: J Cyst Fibros Date: 2019-03-27 Impact factor: 5.482
Authors: Jaime L Rubin; Lasair O'Callaghan; Christopher Pelligra; Michael W Konstan; Alexandra Ward; Jack K Ishak; Conor Chandler; Theodore G Liou Journal: Ther Adv Respir Dis Date: 2019 Jan-Dec Impact factor: 4.031