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

Background: In 2001 Liou published a 5-year survival model using CFF Registry data.

Aims: To evaluate its validity in predicting survival in Italian CF patients.

Methods: In a retrospective study on 945 patients, the 9 variables selected by Liou were analyzed, vital status on December 2008 recorded and observed and expected deaths compared.

Two successful pregnancies in a woman affected by cystic fibrosis: case report and review of the literature.

Background: Pregnancies in patients with cystic fibrosis (CF) are subject to an increased risk of complications.

Methods: We have made a systematic review of the literature concerning pregnancies in women with CF to evaluate prognostic factors of pregnancy outcome such as lung function and nutritional status, also including and describing the case of a woman suffering from CF who had two pregnancies in her late thirties, both with a good outcome.

Results: Thirteen case series and 22 case reports involving 523 pregnancies in 401 women were extracted.

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

Background: 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.

Evaluation of a home telemonitoring service for adult patients with cystic fibrosis: a pilot study.

We have conducted a pilot trial of a home telemonitoring system involving 60 adult patients with cystic fibrosis (CF) enrolled at four Italian CF centres. Thirty randomly selected patients received a home device to perform spirometry and to measure oxygen saturation. The data were transferred to a data centre which forwarded them to the patient's CF clinic.