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

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.

Keywords: childhood; cystic fibrosis; lung disease; mortality; registry.

Figure 1

Results of the first step strategy: they are expressed as OR and 95% confidence interval (CI). All factors, among those recorded in the ECFSPR, were grouped in five classes: diagnosis (1), microbiology (2), therapies (3), complications (4), growth and lung function (5), Chr: chronic.

Figure 2

(a): alive patients from low/middle income countries (empty bullet) and from high income countries (circle bullet) by BMI and lung function. (b): deceased patients from low/middle income countries (empty bullet) and from high income countries (circle bullet) by BMI and lung function.