{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T19:10:13Z","timestamp":1787166613636,"version":"build-2736575974"},"reference-count":48,"publisher":"Public Library of Science (PLoS)","issue":"12","license":[{"start":{"date-parts":[[2024,12,5]],"date-time":"2024-12-05T00:00:00Z","timestamp":1733356800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:sec id=\"sec001\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>The duration of time a person with cystic fibrosis (pwCF) spends on the lung transplant waitlist is dependent on waitlist and post-transplant survival probabilities and can extend up to 2 years. Understanding the characteristics involved with lung transplant and survival prognoses may help guide decision making by the patient, the referring CF Center and the transplant team.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"sec002\">\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This study seeks to identify clinical predictors of lung transplant and survival of individuals with CF using 29,847 subjects from 2003\u20132014 entered in the Cystic Fibrosis Foundation Patient Registry (CFFPR).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"sec003\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Predictors significant (p \u2264 0.05) in the final logistic regression model predicting probability of lung transplant\/death were: FEV\n                      <jats:sub>1<\/jats:sub>\n                      (% predicted), BMI, age of diagnosis, age, number of pulmonary exacerbations, race, sex, CF-related diabetes (CFRD), corticosteroid use, infections with\n                      <jats:italic>B<\/jats:italic>\n                      .\n                      <jats:italic>cepacia<\/jats:italic>\n                      ,\n                      <jats:italic>P<\/jats:italic>\n                      .\n                      <jats:italic>aeruginosa<\/jats:italic>\n                      ,\n                      <jats:italic>S<\/jats:italic>\n                      .\n                      <jats:italic>aureus<\/jats:italic>\n                      , MRSA, pancreatic enzyme use, insurance status, and consecutive ibuprofen use for at least 4 years. The final Cox regression model predicting time to lung transplant identified these predictors as significant FEV\n                      <jats:sub>1<\/jats:sub>\n                      (% predicted), BMI, age of diagnosis, age, number of pulmonary exacerbations, race, sex, CF-related diabetes (CFRD), corticosteroid use, infections with\n                      <jats:italic>B<\/jats:italic>\n                      .\n                      <jats:italic>cepacia<\/jats:italic>\n                      ,\n                      <jats:italic>P<\/jats:italic>\n                      .\n                      <jats:italic>aeruginosa<\/jats:italic>\n                      ,\n                      <jats:italic>S<\/jats:italic>\n                      .\n                      <jats:italic>aureus<\/jats:italic>\n                      , MRSA, pancreatic enzyme use, and consecutive ibuprofen use for at least 4 years. The concordance indices were 0.89 and 0.92, respectively.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"sec004\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The models are translated into nomograms to simplify investigation of how various characteristics relate to lung transplant and survival prognosis individuals with CF not receiving highly effective CFTR modulator therapy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1371\/journal.pone.0292568","type":"journal-article","created":{"date-parts":[[2024,12,5]],"date-time":"2024-12-05T13:32:39Z","timestamp":1733405559000},"page":"e0292568","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":1,"title":["Modeling cystic fibrosis patient prognosis: Nomograms to predict lung transplantation and survival prior to highly effective modular therapy"],"prefix":"10.1371","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1127-7219","authenticated-orcid":true,"given":"Annalisa V.","family":"Piccorelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerry A.","family":"Nick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2024,12,5]]},"reference":[{"key":"pone.0292568.ref001","unstructured":"U.S. Cystic Fibrosis Foundation. 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Middleton","year":"2019","journal-title":"N Engl J Med"},{"key":"pone.0292568.ref004","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.1164\/rccm.201301-0153OC","article-title":"Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation","volume":"187","author":"J.C. Davies","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"issue":"18","key":"pone.0292568.ref005","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR Potentiator in Patients with Cystic Fibrosis and the G551 Mutation","volume":"365","author":"B.W. Ramsey","year":"2011","journal-title":"N Engl J Med"},{"key":"pone.0292568.ref006","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1164\/rccm.200410-1369OC","article-title":"Risk factors for death of patients with cystic fibrosis awaiting lung transplantation","volume":"173","author":"R.A. Belkin","year":"2006","journal-title":"Am. J. Respir. Crit. Care Med"},{"key":"pone.0292568.ref007","unstructured":"Health Resources and Services Administration. OPTN (Organ Procurement and Transplantation)\/SRTR (Scientific Registry of Transplant Recipients) 2017 Annual Data Report: Lung. (2018). https:\/\/srtr.transplant.hrsa.gov\/annual_reports\/2017\/Lung.aspx. 2017"},{"issue":"Part 2","key":"pone.0292568.ref008","doi-asserted-by":"crossref","first-page":"1212","DOI":"10.1111\/j.1600-6143.2006.01276.x","article-title":"Development of the New Lung Allocation System in the United States.","volume":"6","author":"T.M. Egan","year":"2006","journal-title":"American Journal of Transplantation"},{"key":"pone.0292568.ref009","unstructured":"U.S. Cystic Fibrosis Foundation. Patient Registry (2021) 2020 Annual Data Report. 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