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.

Validation of a predictive survival model in Italian patients with cystic fibrosis / R. Buzzetti, G. Alicandro, L. Minicucci, S. Notarnicola, M.L. Furnari, G. Giordano, V. Lucidi, E. Montemitro, V. Raia, G. Magazzú, G. Vieni, S. Quattrucci, A. Ferrazza, R. Gagliardini, N. Cirilli, D. Salvatore, C. Colombo. - In: JOURNAL OF CYSTIC FIBROSIS. - ISSN 1569-1993. - 11:1(2012), pp. 24-29.

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

G. Alicandro;C. Colombo
2012

Abstract

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.
Cystic fibrosis; Survival models; Adolescent; Chi-Square Distribution; Cystic Fibrosis; Female; Forced Expiratory Volume; Humans; Logistic Models; Male; Prognosis; Pseudomonas Infections; Pseudomonas aeruginosa; Survival Analysis; Young Adult; Pulmonary and Respiratory Medicine; Pediatrics, Perinatology and Child Health
Settore MED/38 - Pediatria Generale e Specialistica
2012
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/289945
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