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Cystic fibrosis mortality trends in Spain among infants and young children: 1981–2004

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Abstract

This paper sought to analyse mortality trends among infants and young children who died with a diagnosis of cystic fibrosis (CF) in Spain, during the period 1981–2004. Study design: Descriptive observational study, using joinpoint regression models. Participants: Data on cystic fibrosis deaths were drawn from the National Statistics Institute (Instituto Nacional de Estadística), which collects data from all death certificates in Spain. During the period 1981–2004, overall CF mortality in Spain decreased by an annual average of 4% in both sexes. A breakdown by age showed that patients under 15 years registered a declining and those over 15 years a rising mortality rate over the study period. Mean and median age at death from CF increased with time, from a median of 4.4 years (males) and 3.8 years (females) in 1981 to 20.1 years (males) and 17.7 years (females) in 2004. The results of this study show that, as in other Western countries, CF is no longer a major cause of death in childhood, and that the challenge now lies in caring for adults who suffer from this disease. The fact that our study was descriptive meant that the reasons for the decrease in CF mortality in Spain could not be identified. Other authors have shown that this decrease is associated with improved treatment for pulmonary complications, better nutritional control and lung transplants.

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Abbreviations

CF:

Cystic fibrosis

REpIER:

Epidemiology research network for rare diseases

RD:

Rare diseases

CFTR:

Cystic fibrosis transmembrane conductance regulator gene

NSI:

National statistics institute

ICD:

International classification of diseases

APC:

Annual percentage change

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Acknowledgements

Funding This study was funded by the Health Research Fund (Fondo de Investigación Sanitaria—FIS), Instituto de Salud Carlos III (Spain): Expte: G03/123 REpIER (Epidemiology Research Network for Rare Diseases).

Author information

Authors and Affiliations

  1. Department of Epidemiology, La Rioja Regional Authority, Logroño, Spain

    Enrique Ramalle-Gomara, Milagros Perucha, María-Ángeles González & Carmen Quiñones

  2. Department of Paediatrics, Río Carrión Hospital, Palencia, Spain

    Jesús Andrés

  3. IIER-Instituto de Salud Carlos III and CIBER de Enfermedades Raras (CIBERER), Calle Sinesio Delgado, 6, 28029, Madrid, Spain

    Manuel Posada

Authors
  1. Enrique Ramalle-Gomara
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  2. Milagros Perucha
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  3. María-Ángeles González
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  4. Carmen Quiñones
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  5. Jesús Andrés
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  6. Manuel Posada
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Corresponding author

Correspondence to Manuel Posada.

Additional information

All the authors on behalf of the Rare Disease Epidemiology Research Network (Red Epidemiológica de Investigación de Enfermedades Raras—REpIER)*.

*REpIER associated (Regional co-ordinating nodes): Enrique Ramalle-Gomara, Milagros Perucha, María-Ángeles González, Carmen Quiñones, Jesús Andrés, and Manuel Posada on behalf of the Rare Disease Epidemiology Research Network (Red Epidemiológica de Investigación de Enfermedades Raras—REpIER-REpIER associated (Regional co-ordinating nodes): Francisco Javier Garcia León (Andalusia), Mario Margolles Martins (Asturias), Luis Miguel Ruiz Ceballos (Cantabria), Nieves Cardeñosa Marín (Catalonia), Belén Zorrilla Torras (Madrid), Óscar Ernesto Zurriaga Llorens (Valencian Region), Elvira Bel Prieto (Catalonia), María José Iglesias Gozalo (Aragon), Pedro Guillermo Serrano Aguilar (Canary Islands), Gonzalo Gutiérrez Ávila (Castile-La Mancha) Julián Mauro Ramos Aceitero (Extremadura), Enrique Galán Gómez (Extremadura), Carmen Martínez García (Andalusia), Carlos Lahoz Navarro (Madrid), María Luisa Martínez Frías (Madrid).

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Ramalle-Gomara, E., Perucha, M., González, MÁ. et al. Cystic fibrosis mortality trends in Spain among infants and young children: 1981–2004. Eur J Epidemiol 23, 523–529 (2008). https://doi.org/10.1007/s10654-008-9263-1

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  • DOI: https://doi.org/10.1007/s10654-008-9263-1

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