Validation of a predictive survival model in Italian patients with cystic fibrosis

Roberto Buzzetti, Gianfranco Alicandro, Laura Minicucci, Sara Notarnicola, Maria Lucia Furnari, Gabriella Giordano, Vincenzina Lucidi, Enza Montemitro, Valeria Raia, Giuseppe Magazzú, Giuseppe Vieni, Serena Quattrucci, Alessandro Ferrazza, Rolando Gagliardini, Natalia Cirilli, Donatello Salvatore, Carla Colombo

Research output: Contribution to journalArticlepeer-review


Background: In 2001 Liou published a 5-year survival model using CFF Registry data. Aims: To evaluate its validity in predicting survival in Italian CF patients. Methods: In a retrospective study on 945 patients, the 9 variables selected by Liou were analyzed, vital status on December 2008 recorded and observed and expected deaths compared. To develop a new model, patients were randomly divided into a derivation (n = 475) and a validation sample (n = 470). Results: A significant difference was found between observed and expected deaths based on Liou's model (62 vs 94), with a 34% reduction in mortality (p <0.05). A new model (based on FEV1, Staphylococcus aureus and Burkholderia cepacia complex infection, number of pulmonary exacerbations/year) was generated, that correctly predicted survival in the validation sample (31 observed vs 29 expected deaths, p = 0.660). Conclusions: The Liou model did not adequately predict 5-year survival in our CF population that, compared to the one in which it was originally tested, could benefit from 10. years of improvement in treatments and practice patterns. A new generated model, based on only four variables, was more accurate in predicting 5-year survival in Italian CF patients.

Original languageEnglish
Pages (from-to)24-29
Number of pages6
JournalJournal of Cystic Fibrosis
Issue number1
Publication statusPublished - Jan 2012


  • Cystic fibrosis
  • Survival models

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Pediatrics, Perinatology, and Child Health


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