{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,29]],"date-time":"2025-10-29T13:09:20Z","timestamp":1761743360051},"reference-count":40,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2010,6,25]],"date-time":"2010-06-25T00:00:00Z","timestamp":1277424000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2010,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Health\u2010related quality of life (HRQOL) measurements provide valuable information about the psychological and social impact of treatment on patients with cystic fibrosis (CF). This study evaluated the HRQOL of Brazilian patients with CF and assessed the changes in HRQOL domains over 1 year after dornase alfa (Pulmozyme) introduction.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods<\/jats:title><jats:p>One hundred fifty\u2010six stable patients with CF and 89 caregivers answered the Portuguese\u2010validated version of the Cystic Fibrosis Questionnaire\u2010Revised (CFQ\u2010R) at baseline (T<jats:sub>0<\/jats:sub>), and at 3 (T<jats:sub>1<\/jats:sub>), 6 (T<jats:sub>2<\/jats:sub>), 9 (T<jats:sub>3<\/jats:sub>), and 12 (T<jats:sub>4<\/jats:sub>) months of follow\u2010up. Eighteen patients were excluded because they did not fulfill the inclusion criteria. The patients were analyzed in two groups: those aged 6\u201311 years and those aged 14 years and older. ANOVA for observed repeated results and the last observation carried forward (LOCF) method for missing data were used for the statistical analysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>After 1 year of follow\u2010up, there was significant improvement in respiratory symptoms (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u20098.1; 95% confidence interval (95% CI)\u2009=\u2009[2.1;14.0]; effect size (ES)\u2009=\u20090.35; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001), Emotional Functioning (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u20095.6; 95% CI\u2009=\u2009[1.1;10.1]; ES\u2009=\u20090.31; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), Social Functioning (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u20096.0; 95% CI\u2009=\u2009[1.3;11.7]; ES\u2009=\u20090.31; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), Body Image (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u200911.9; 95% CI\u2009=\u2009[4.1;19.7]; ES\u2009=\u20090.42; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), and Treatment Burden (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u20095.3; 95% CI\u2009=\u2009[0.3;10.3]; ES\u2009=\u20090.24; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05) domains in the younger group. A significant improvement in Role Functioning (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u20096.1; 95% CI\u2009=\u2009[1.1;11.1]; ES\u2009=\u20090.40; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), Body Image (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u200912.6; 95% CI\u2009=\u2009[3.5;21.7]; ES\u2009=\u20090.46; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), and Weight (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u200911.7; 95% CI\u2009=\u2009[1.8;21.6]; ES\u2009=\u20090.40; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05) was obtained in the older group. The caregivers' CFQ\u2010R showed improvements in the Digestive Symptoms (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u20095.5; 95% CI\u2009=\u2009[1.5;9.4]; ES\u2009=\u20090.30; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), Respiratory Symptoms (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u20097.6; 95% CI\u2009=\u2009[3.9;11.4]; ES\u2009=\u20090.48; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05), and Weight (T<jats:sub>4<\/jats:sub>\u2009\u2212\u2009T<jats:sub>0<\/jats:sub>\u2009=\u200910.1; 95% CI\u2009=\u2009[1.6;18.6]; ES\u2009=\u20090.26; <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.05) domains.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The introduction of dornase alfa improved the HRQL of the patients with CF during the first year of treatment. Pediatr. Pulmonol. 2010; 45:874\u2013882. \u00a9 2010 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.21267","type":"journal-article","created":{"date-parts":[[2010,6,25]],"date-time":"2010-06-25T16:28:08Z","timestamp":1277483288000},"page":"874-882","source":"Crossref","is-referenced-by-count":15,"title":["Dornase alfa improves the health\u2010related quality of life among Brazilian patients with cystic fibrosis\u2014A one\u2010year prospective study"],"prefix":"10.1002","volume":"45","author":[{"given":"Tatiana","family":"Rozov","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Viviane Ziebell","family":"de Oliveira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Ang\u00e9lica","family":"Santana","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fab\u00edola Villac","family":"Adde","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rita Heloisa","family":"Mendes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilma Aparecida","family":"Paschoal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francisco J.","family":"Caldeira Reis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurinda Yoko Shinzato","family":"Higa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr.","given":"Antonio Carlos de Castro","family":"Toledo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00e1rcia","family":"Pahl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"the Pulmozyme Study Group","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,8,24]]},"reference":[{"volume-title":"Annual report, Vol. 2009","year":"2007","author":"Cystic Fibrosis Foundation","key":"e_1_2_6_2_2"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa043900"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200705-664OC"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.120269"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1159\/000050486"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.2165\/00128072-200305010-00004"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1513\/pats.200703-039BR"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1186\/1477-7525-4-79"},{"volume-title":"Cystic fibrosis questionnaire: a health\u2010related quality of life measure. 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