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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Resumen

LENTINI N, EDUARDO et al. Improving cystic fibrosis survival as a result of management in a specialized Center with 10-year follow-up. Rev. chil. pediatr. [online]. 2014, vol.85, n.3, pp.281-287. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062014000300003.

Introduction: Cystic fibrosis (CF), the most common genetic disease among whites, is a multisystem disease that requires a coordinated multidisciplinary approach to get better results regarding survival and quality of life. This is why a Cystic Fibrosis Center (CFC) was held in Mendoza, Argentina, with the assumption that the organization and management of CF, according to international standards would improve survival. Patients and Method: 106 patients with CF have been evaluated since 1975 in this Center. Patients were grouped for follow-up monitoring as the 1999, 2002, 2005 and 2008 cohort studies. The chi-square test was used to compare survival rates and the nonparametric Mann-Whitney test was used to calculate the median age of the population. Results: In 2008, survival at 17 years old was 80% compared with 45% for the same age in 1999 (p < 0,001). The median age of the total CF population increased from 4 (1985) to 12 years old (2008) (p < 0,003). Conclusions: A multidisciplinary standardized approach of this condition in a CF Center can significantly increase survival.

Palabras clave : Cystic fibrosis; Cystic Fibrosis Center; survival; mortality.

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