Validation of a predictive survival model in Italian patients with cystic fibrosis

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Evaluation of specific immune response in early P. aeruginosa infection in cystic fibrosis patients

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Incidence of filamentosus fungi in sputum of patients affected by cystic fibrosis

Introduction. Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by a mutation of the gene encoding the CF protein CFTR. Patients with CF are frequently affected by respiratory infections due to specific pathogens.The role of filamentous fungi is well established in the case of allergic bronchopulmonary aspergillosis and invasive pulmonary infections in lung transplant patients, but their involvement in lung diseases outside of these cases remain to be clarified.The aim of our study was to monitor the impact of filamentous fungi in patients with CF. Methods. In the years 2007 and 2008 and during the first half of 2009 a total of 1046 samples of sputum collected from CF patients were analyzed. Of these 236 cases were positive for fungi. Results. Filamentous fungi were isolated from 44 patients, including 29 females and 15 males.The fungi were identified as Aspergillus fumigatus (70.04% of the cases), A. terreus (2.4%),A.flavus (11.4% ), Scedosporium apiospermum (3.8%), Penicillium spp (0.84%), Aspergillus spp. (1.26%) and Exophiala dermatitidis (0.24%). Conclusions. Based on the isolates, out of 44 cases the duration in time of colonization by filamentous fungi was constant with the same species in 11 patients, intermittent with the same or occasionally another species in 14 patients and present for no more than one semester in the remaining 19 cases

A polymorphism in the 5' UTR of the DEFB1 gene is associated with the lung phenotype in F508del homozygous Italian cystic fibrosis patients

The identification of cystic fibrosis (CF) patients who are at greater risk of lung damage could be clinically valuable. Thus, we attempted to replicate previous findings and verify the possible association between three single nucleotide polymorphisms (SNPs c.-52G>A, c.-44C>G and c.-20G>A) in the 5' untranslated region (5' UTR) of the \u3b2 defensin 1 (DEFB1) gene and the CF pulmonary phenotype