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

Author

Listed:
  • Anna Zolin

    (Department of Clinical Sciences and Community Health, University of Milan, Milano 20133, Italy)

  • Anna Bossi

    (Department of Clinical Sciences and Community Health, University of Milan, Milano 20133, Italy)

  • Natalia Cirilli

    (Cystic Fibrosis Centre, Mother-Child Department, United Hospitals, Via Conca, 71, Torrette di Ancona I-60126, Italy)

  • Nataliya Kashirskaya

    (Laboratory of Genetic Epidemiology, Federal State Scientific Budgetary Institution «Research Centre for Medical Genetics», Moscow 115522, Russia)

  • Rita Padoan

    (Cystic Fibrosis Unit, Pediatric Department, ASST Spedali Civili Brescia, piazzale Spedali Civili, Brescia 25123, Italy)

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 (FEV 1 ) 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 FEV 1 % and BMI-SDS were low, and some deceased patients from countries with high income had high values of FEV 1 % (>60%). Evaluation of mortality in pediatric age may reflect the availability of resources for CF diagnosis and treatment in some countries.

Suggested Citation

  • Anna Zolin & Anna Bossi & Natalia Cirilli & Nataliya Kashirskaya & Rita Padoan, 2018. "Cystic Fibrosis Mortality in Childhood. Data from European Cystic Fibrosis Society Patient Registry," IJERPH, MDPI, vol. 15(9), pages 1-11, September.
    • Handle: RePEc:gam:jijerp:v:15:y:2018:i:9:p:2020-:d:170087
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    Citations

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    Cited by:

    1. Ana Villaverde-Hueso & Germán Sánchez-Díaz & Francisco J. Molina-Cabrero & Elisa Gallego & Manuel Posada de la Paz & Verónica Alonso-Ferreira, 2019. "Mortality Due to Cystic Fibrosis over a 36-Year Period in Spain: Time Trends and Geographic Variations," IJERPH, MDPI, vol. 16(1), pages 1-12, January.
    2. Marta Rachel & Stanisław Topolewicz & Andrzej Śliwczyński & Sabina Galiniak, 2020. "Managing Cystic Fibrosis in Polish Healthcare," IJERPH, MDPI, vol. 17(20), pages 1-17, October.

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