Anales de pediatría : publicación oficial de la Asociación Española de Pediatría (A.E.P.)
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Practice Guideline
[Protocol for the diagnosis and follow up of patients with cystic fibrosis].
Cystic fibrosis (CF) is the most common severe recessive genetic disease in Caucasians. During the last years, new therapies and aggressive management of the lung disease have contributed significantly to the increased life expectancy in CF patients. A review and update of CF diagnosis and management of lung disease are included. ⋯ An overview with the recommendations for treatment of early onset and chronic infections due to Pseudomonas aeruginosa, Staphylococcus aureus and other uncommon pathogens is included. Furthermore, the management of other aspects of CF lung disease and complications is provided, as well as the indications for lung transplantation. This document has been prepared by the members of the CF working group of the Spanish Paediatrics Pulmonary Society to provide an update to the earlier documents published in this Journal in 1999.