In vitro lymphocyte responses to Pseudomonas aeruginosa have been found to be impaired in cystic fibrosis patients with advanced clinical disease. The responses to Klebsiella pneumoniae, Serratia marcescens, and Proteus mirabilis were studied in a similar group of cystic fibrosis patients and normal individuals. Cystic fibrosis patients found to be unresponsive to pseudomonas were also unrespon...

Infection with Burkholderia cepacia due to social contact is well described in patients with cystic fibrosis. However, social transmission to non-cystic fibrosis individuals or chronic colonisation in non-cystic fibrosis individuals has not been described. A report of B cepacia bronchiectasis is presented where a previously healthy mother of two cystic fibrosis children colonised with B cepacia...

As cross-sectional imaging has become more common, there has been a marked increase in the incidental detection of cystic pancreatic lesions. It is estimated that more than a third of these are found in asymptomatic patients. Although knowledge of the natural history of cystic lesions is incomplete at this time, it is known that some cystic pancreatic lesions, including pseudocysts and serous c...

The Cystic Fibrosis Transmembrane Regulator (CFTR) should no longer be viewed primarily as a ‘chloride Abstract channel’ but recognized as a channel that also controls the efflux of other physiologically important anions, such as glutathione (GSH) and bicarbonate. More effective approaches to cystic fibrosis treatment may result from this reconceptualization of the CFTR by researchers and clini...

SUMMARY We report a rare case of cystic eccrine spiradenoma in the finger. A 46-year-old man presented with a cystic mass in his left index finger. Clinical assessment along with the investigation pointed toward a diagnosis of a ganglion. However, excisional biopsy of the mass revealed histopathological findings of cystic eccrine spiradenoma. Very few cases of eccrine spiradenoma have been repo...

Patients with cystic fibrosis have been found to have abnormal serum concentrations of immunoreactive trypsin and abnormal activities of pancreatic isoamylase. A study was undertaken to discover whether activity of pancreatic lipase is also altered in cystic fibrosis. Serum from 23 patients with cystic fibrosis was assayed for immunoreactive trypsin and pancreatic lipase. Median serum pancreati...

Parents of children with cystic fibrosis have been reported to have a high prevalence of increased airway reactivity, but these studies were done in a select young, healthy, symptomless population. In the present study respiratory symptoms were examined in 315 unselected parents of children with cystic fibrosis and 162 parents of children with congenital heart disease (controls). The cardinal s...

INTRODUCTION Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride loss in sweat, infertility among men due to agenesis of the vas deferens and other symptoms including liver disease. CASE PRESENTATION We descri...

Although the exact prevalence of cystic pancreatic lesions is unknown, it is estimated to be around 1% of the general population based on large scale observational imaging studies [1]. While cystic pancreatic lesions are increasingly diagnosed due to the widespread use of cross-sectional imaging modalities like computed tomography (CT) and magnetic resonance imaging (MRI), it is not known if th...

Background—Antenatal screening for cystic fibrosis has been endorsed by the US National Institutes of Health. Edinburgh is the only city in the UK with an established routine antenatal screening programme for cystic fibrosis. Aims—To report the change in numbers of infants diagnosed with cystic fibrosis born in Edinburgh after the introduction of antenatal screening for the disease. Population—...