Neonatal screening for cystic fibrosis in Cataluña, Spain: 10 years of experience

Gartner, Sílvia; Casals, Teresa; Marín, Javier López; Séculi, J. L.; Asensio, Òscar; Hernandez, Jurado; Prats, Ramón; Cobos, N.

doi:10.1016/s1569-1993(10)60034-3

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“…The median survival age has been put at about 30 years in the United Kingdom [10] and Canada [11], and at 35 years in the USA in 2004 [12], and at 22 years in France [13]. Incidence of CF cases in Spain varies from 1 in 2,810 [14] to 1 in 5,532 newborn infants [15]. These differences could be due to the observed prevalence of the main mutation F508del in Spain, that varies between 50 and 80% [16] Respiratory failure is the most common cause of death among cystic fibrosis sufferers [17].…”

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confidence: 99%

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

et al. 2008

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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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