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Int. J. Environ. Res. Public Health 2018, 15(9), 2020; https://doi.org/10.3390/ijerph15092020

Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry

1
Department of Clinical Sciences and Community Health, University of Milan, Milano 20133, Italy
2
Cystic Fibrosis Centre, Mother-Child Department, United Hospitals, Via Conca, 71, Torrette di Ancona I-60126, Italy
3
Laboratory of Genetic Epidemiology, Federal State Scientific Budgetary Institution «Research Centre for Medical Genetics», Moscow 115522, Russia
4
Cystic Fibrosis Unit, Pediatric Department, ASST Spedali Civili Brescia, piazzale Spedali Civili, Brescia 25123, Italy
*
Author to whom correspondence should be addressed.
Received: 31 July 2018 / Revised: 29 August 2018 / Accepted: 7 September 2018 / Published: 15 September 2018
(This article belongs to the Special Issue Health Inequalities in Children)
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Abstract

Data collected in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database were used to investigate whether risk factors for death in childhood and adolescents CF patients have different impact in countries of different income. In this way, it is possible to recognize where interventions could improve the quality of care and survival in these patients. We matched deceased and alive patients by age, country, year of follow-up. Multivariable logistic models were developed. In the years of this study, the ECFSPR collected information on 24,416 patients younger than 18 years: 7830 patients were from countries with low/middle income and 16,586 from countries with high income; among these the dead are 102 and 107 (p < 0.001), respectively. The use of oxygen, forced expiratory volume in one second (FEV1) below 40% and BMI standard deviation score (SDS) below −2 represent risk factors for death. However, some patients from countries with high income remain alive even if their values of FEV1% and BMI-SDS were low, and some deceased patients from countries with high income had high values of FEV1% (>60%). Evaluation of mortality in pediatric age may reflect the availability of resources for CF diagnosis and treatment in some countries. View Full-Text
Keywords: mortality; childhood; lung disease; cystic fibrosis; registry mortality; childhood; lung disease; cystic fibrosis; registry
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Zolin, A.; Bossi, A.; Cirilli, N.; Kashirskaya, N.; Padoan, R. Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry. Int. J. Environ. Res. Public Health 2018, 15, 2020.

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