Cystic fibrosis (CF) is characterized by progressive airway obstruction. Although it has been postulated that this is due in part to mucus hypersecretion, there are no published data showing an increase in the gel-forming mucins MUC5AC or MUC5B in CF secretions. We used confocal microscopy to assess the amount of mucin-like glycoprotein and DNA in CF sputum and found more mucin in bronchitis sp...

Infants with positive CF newborn screening (NBS) results are called to a CF Centre for verification. Those, in whom the sweat test is elevated, undergo further medical procedures. The aim of our study was to evaluate the applicability of Nanoduct - a new system measuring sweat conductivity and giving immediate results in a CF NBS protocol. Measurements with Nanoduct were compared with the class...

BACKGROUND Of the 2007 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) mutations, 202 have been assigned disease liability. California's racially diverse population, along with CFTR sequencing as part of newborn screening model, provides the opportunity to examine the phenotypes of children with uncategorized mutations to help inform disease liability and penetrance. METHODS We con...

Although sweat conductivity values are well matched with chloride concentrations for cystic fibrosis (CF) diagnosis, sweat conductivity is not accepted as a definitive diagnostic tool but only a screening method. The aim of this study was to compare the sweat chloride measurements and sweat conductivity values of our patients, and to determine cut-off values of conductivity for making or exclud...

We report results from the analysis of 21 nearby galaxy clusters, 11 with cooling flow (CF) and 10 without cooling flow, observed with BeppoSAX. The temperature profiles of both CF and non-CF systems are characterized by an isothermal core extending out to ∼ 0.2 r180; beyond this radius both CF and non-CF cluster profiles rapidly decline. Our results differ from those derived by other authors w...

The atmospheric chemistry of several gases used in industrial applications, C(4)F(9)OC(2)H(5) (HFE-7200), C(4)F(9)OCH(3) (HFE-7100), C(3)F(7)OCH(3) (HFE-7000) and C(3)F(7)CH(2)OH, has been studied. The discharge flow technique coupled with mass-spectrometric detection has been used to study the kinetics of their reactions with OH radicals as a function of temperature. The infrared spectra of th...