Background: Hypersecretion of viscous infected mucus plugging, mucosal inflammation, bronchoconstriction, airway instability or lung tissue damage in CF patients cause pulmonary obstruction.Although chest physiotherapy techniques are commonly used in CF respiratory diseases treatment, there are no studies evaluating the effect of the expiratory flow increase technique (French technique) on pulmonary function of CF patients.Aim: to evaluate the immediate effect of the EFIT on spirometric and cardio respiratory parameters in CF patients colonized by P aeruginosa with acute pulmonary exacerbation CAPE). Patients and Methods: A clinical, prospective study including 27 CF patients colonized by P aeruginosa at the CF center of State University of Campinas School Hospital, Brazil were studied.The patients were submitted to spirometry, SaO 2 measured by pulse oximetry, clinical examination and respiratory physiotherapy (EFIT) immediately before the treatment by intravenous antibiotics (step I).The same procedure was done two weeks later in all patients (step II).Results: 27 patients (17 females), aged 7 28 years (15±6.07)completed the study.15 patients were clinically severe by Shwachman Score.The mean values of variables from step I and step II were: oxygen saturation (92.15±6.06vs 93.74±4.56),inspiratory capacity: (62.19+18.74vs 64.44+18.66),Slow Vital Capacity (SVC) (63.96+19.90vs 66.19+25.50)without statistic significance.Conclusion: The EFIT shows no positive effects on pulmonary ventilation in CF patients with pulmonary exacerbation.

Objective To determine the prevalence of urinary incontinence (UI) in female patients (aged greater than or equal to 15 years) attending a cystic fibrosis (CF) centre, in whom stress UI could be common, as chronic coughing and sputum production are frequent symptoms associated with progressive lung disease in these patients. Patients and methods An anonymous questionnaire was completed by 176 women with CF (mean age 24.6 years, SD 5.8) during routine assessments as outpatients.Results In all, 72 patients (41%) were classified as never incontinent; occasional UI was reported in 61 women (35%). Regular UI, occurring twice or more a month for at least two consecutive months in the last year, was reported in 43 patients (24%). Regular UI was associated with increasing age and a lower mean (so) forced expiratory volume/s (of that predicted) than in women with no urinary symptoms, at 26.9 (6.5) years and 53.5(23.5)%, and 23.1(5.4) years and 65.5(23.2)%. respectively (P<0.01 and P<0.05, respectively). All incontinent women recorded stress UI: coughing, laughing and physical activity were associated with UI in 92%, 33% and 21% of the patients, respectively.Conclusion Stress UI is a common symptom in women with CF. As urine loss can be under-reported to the healthcare providers, women should be asked about incontinence as part of their routine follow-up. Pelvic floor muscle exercises are effective in treating stress UI and should be considered for those with CF and regular UI.

The following topics are discussed in this consensus document on the problems arising from molecular analysis of cystic fibrosis (CF): a) neonatal screening: the application; of DNA analysis in the neonatal screening for CF implies that same heterozygotes are going to be identified; pre-testing and post-testing informations need to be accurate and widespread the possibility of withdrawing from being tested has to be guaranteed; b) population heterozygote screening: the insufficient sensitivity of the molecular test the incomplete definition of the genotype-phenotype correlations, the high costs, the relevance of psycho-social effects, the lack of laboratory quality control programs are all ;points against a policy of a population screening of the italian population; c) carrier testing: this is at present : performed and should be offered to relatives of CF patients or heterozygotes ("cascade screening"); partners of subjects found to be positive should also be tested The test should be preferably performed before pregnancy to allow a wider variety of options; we advice against testing in children due to is purely reproductive significance; d) prenatal diagnosis: it has a well defined role for couples with a 1:4 risk; on the contrary, the offer of prenatal diagnosis to intermediate risk couples should be discouraged in view of the impossibility to obtaine conclusive results in these cases.

We studied 101 couples to determine how far their reproductive behaviour was affected by the diagnosis of cystic fibrosis (CF) on its first occurrence in the couple's progeny and by the availability of prenatal diagnosis (PD). The couples were all resident in the Veneto and Trentino regions and attending the Verona CF Centre. CF had been diagnosed in the first affected child, during the period 1 January 1980-1 July 1990, before the age of 1 year. Couples received a questionnaire regarding socio-demographic status, reproduction data, and awareness of PD. Reproductive history was divided into three phases: prior to diagnosis of CF in the first affected child; from this time until PD was made available; and after the couples had learned of PD. In phase 2 (awareness of the genetic risk but not of PD), 54 couples showed a marked decrease in reproduction, none of the few pregnancies that occurred being taken to term. When couples became aware of PD, some resumption of reproductive activity occurred and 11 per cent of the 101 couples had another child; PD was used in 65 per cent of pregnancies and the abortion rate decreased to 35 per cent. All couples who opted for PD had no children without CF.

The activity of γ-glutamyltranspeptidase and total alkaline phosphatase and its isoenzymes has been determined in 261 amniotic fluid samples taken from pregnant women with known normal outcome and in 30 amniotic fluid samples from pregnant women with a 1:4 risk for cystic fibrosis (CF). Preliminarily, 114 amniotic fluid samples were assayed in parallel in three different laboratories, and a good correlation was found even though different assays were used. From the results obtained in control amniotic fluids, normal range and CF-predictive cutoff values were established. No false-negative results were found in this study. Among the predicted affected pregnancies 7 were terminated, and 3 went to term: 1 resulting in a CF-affected child and the other 2 in healthy children. CF was confirmed in all the aborted fetuses. In 1 case the results were inconclusive. In this study numerical results obtained for samples with a 1:4 risk of CF analyzed in the three laboratories were always virtually identical.