Ivacaftor was associated with improvements in lung function at 2 weeks that were sustained through 48 weeks and substantial improvements were also observed in the risk of pulmonary exacerbations, patient-reported respiratory symptoms, weight, and concentration of sweat chloride.
American journal of respiratory and critical careā¦
1 May 2011
TLDR
Significant research efforts have resulted in a paradigm shift away from identifying noninfectious lung injury after HSCT solely as an idiopathic clinical syndrome and toward understanding IPS as a process involving aspects of both the adaptive and the innate immune response.
It is proposed that DYX1C1 is a newly identified dynein axonemal assembly factor (DNAAF4), which interacts with the cytoplasmic ODA and IDA assembly factor DNAAF2 (KTU) and is associated with dyslexia and neuronal migration in the developing neocortex.
It is shown that IL-8 promotes bacterial killing by neutrophils through CX CR1 but not CXCR2, which represents a new pathophysiologic mechanism in cystic fibrosis and other chronic lung diseases.
In a mouse model of a lethal congenital lung disease caused by a lack of surfactant protein B (SP-B), twice weekly local application of an aerosol of modified SP-B mRNA to the lung restored 71% of the wild-type SP- B expression, and treated mice survived until the predetermined end of the study after 28 days.
Recent major extensions of the Human Phenotype Ontology for neurology, nephrology, immunology, pulmonology, newborn screening, and other areas are presented and new efforts to harmonize computational definitions of phenotypic abnormalities across the HPO and multiple phenotype ontologies used for animal models of disease are presented.
Linkage analysis for asthma identified four chromosomal regions that could to be linked to asthma: chromosome 2 (at marker D2S2298, P = 0.007), chromosome 6 (around D6S291, lowest P =0.008), chromosome 9 (proximal to D9S1784), and chromosome 12 (D12S351, P=0.010).
For some pulmonary conditions surfactant replacement therapy is on the horizon, but for the majority much more needs to be learnt about the pathophysiological role the observed surfACTant abnormalities may have.
Pulmonary blockade of CXCR2 by intra-airway delivery of small-molecule antagonists inhibited NET formation and improved lung function in vivo without affecting neutrophil recruitment, proteolytic activity or antibacterial host defense.