Recommendations for the classification of diseases as CFTR-related disorders

Several diseases have been clinically or genetically related to cystic fibrosis (CF), but a consensus definition is lacking. Here, we present a proposal for consensus guidelines on cystic fibrosis transmembrane conductance regulator (CFTR)-related disorders (CFTR-RDs), reached after expert discussion and two dedicated workshops. A CFTR-RD may be defined as \u201ca clinical entity associated with CFTR dysfunction that does not fulfil diagnostic criteria for CF\u201d. The utility of sweat testing, mutation analysis, nasal potential difference, and/or intestinal current measurement for the differential diagnosis of CF and CFTR-RD is discussed. Algorithms which use genetic and functional diagnostic tests to distinguish CF and CFTR-Rds are presented. According to present knowledge, congenital bilateral absence of vas deferens (CBAVD), acute recurrent or chronic pancreatitis and disseminated bronchiectasis, all with CFTR dysfunction, are CFTR-RDs

A Simple Route for the Preparation of Mesoporous Nanostructures Using Block Copolymers

Poly(styrene-b-4-vinylpyridine) (PS-b-P4VP) nanostructures with multiple morphologies were fabricated by immersing PS-b-P4VP nanotubes in ethylene glycol, a nonsolvent for PS and a good solvent for P4VP, at different temperatures. Mesoporous structures were generated from uniform nanoscopic wormlike micelles due to a solvent-induced reconstruction when the spherical micellar structures were heated above the glass transition temperature of the PS block. The mesoporous nanostructures can be converted into inorganic oxide structures, like SiO(2) and TiO(2), by well-known sol-gel methods, The mesoporous inorganic oxides can be produced with tunable porosity by controlling the molecular weight of the block copolymers. Confinement also plays an important role to create the nanostructures with unusual morphologies.close282