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...

One hundred and eighty eight isolates of Haemophilus influenzae and 187 isolates of H parainfluenzae from patients with cystic fibrosis, patients with respiratory infections but without cystic fibrosis, and patients with neither cystic fibrosis nor respiratory infections were biotyped. Biotype I of H influenzae were found significantly more often in patients with cystic fibrosis compared with t...

BACKGROUND Cystic fibrosis is an autosomal recessive disorder characterized by chronic progressive multisystem involvement. AH1N1 virus infections caused classic influenza symptoms in the majority of cystic fibrosis patients while others experienced severe outcomes. CASE PRESENTATION We report a case of late incidental cystic fibrosis diagnosis in a young Caucasian man suffering from respirat...

Goodchild, M. C., Nelson, R., and Anderson, C. M. (1973). Archives ofDisease in Childhood, 48, 684. Cystic fibrosis and coeliac disease: coexistence in two children. Two children, unrelated, in whom cystic fibrosis had been diagnosed at the ages of 14 weeks and 10 months, respectively, were found subsequently to suffer from coeliac disease as well. Both children are responding well to dietary g...

Goodchild, M. C., Nelson, R., and Anderson, C. M. (1973). Archives ofDisease in Childhood, 48, 684. Cystic fibrosis and coeliac disease: coexistence in two children. Two children, unrelated, in whom cystic fibrosis had been diagnosed at the ages of 14 weeks and 10 months, respectively, were found subsequently to suffer from coeliac disease as well. Both children are responding well to dietary g...

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...

REFERENCES 1 Jones AM, Govan JR, Doherty CJ, et al. Spread of a multiresistant strain of Pseudomonas aeruginosa in an adult cystic fibrosis clinic. Lancet 2001; 358: 557–558. 2 Cheng K, Smyth RL, Govan JR, et al. Spread of b-lactam-resistant Pseudomonas aeruginosa in a cystic fibrosis clinic. Lancet 1996; 348: 639–642. 3 Armstrong DS, Nixon GM, Carzino R, et al. Detection of a widespread clone ...

OBJECTIVES Volumetric capnography provides the standard CO2 elimination by the volume expired per respiratory cycle and is a measure to assess pulmonary involvement. Thus, the objective of this study was to evaluate the respiratory dynamics of healthy control subjects and those with cystic fibrosis in a submaximal exercise protocol for six minutes on the treadmill, using volumetric capnography ...