Perceptions and Use of E-cigarettes among Young Adults with Cystic Fibrosis: An Observational Study.

While smoking prevalence has decreased among the general population, the use of electronic cigarettes (E- cigarettes) has risen significantly and can cause significant lung injury. We sought to determine if persons with cystic fibrosis (PwCF) have similar rates of E-cigarette use as compared with age-matched peers, and to understand perceptions of E-cigarette safety through a survey-based study. A total of 29 PwCF and 26 age-matched control patients participated in this study.

Integrating Primary Care into the Management of Cystic Fibrosis.

Over the last 50 years, cystic fibrosis has radically transformed from a fatal disease of infancy to a chronic disease of adulthood. By 2025 it is estimated that 70% of individuals with cystic fibrosis (iwCF) will be cared for in adult clinics. We believe the role of a dedicated primary care provider (PCP) for preventative care will be crucial for the longevity of iwCF.

Challenges to advance care planning among multidisciplinary health professionals in China.

Background: Advance care planning (ACP) is a critical component of quality end-of-life care. Little is known regarding the cultural influences on health professionals' attitudes toward the discussion of ACP in China.

Objective: To better understand attitudes toward ACP among Chinese healthcare professionals in China and the influence of cultural factors such as filial piety, or xiào.

A New Murine Model for Gastrointestinal Anthrax Infection.

The scientific community has been restricted by the lack of a practical and informative animal model of gastrointestinal infection with vegetative Bacillus anthracis. We herein report the development of a murine model of gastrointestinal anthrax infection by gavage of vegetative Sterne strain of Bacillus anthracis into the complement-deficient A/J mouse strain. Mice infected in this manner developed lethal infections in a dose-dependent manner and died 30 h-5 d following gavage.

Anthrax lethal toxin disrupts intestinal barrier function and causes systemic infections with enteric bacteria.

A variety of intestinal pathogens have virulence factors that target mitogen activated protein kinase (MAPK) signaling pathways, including Bacillus anthracis. Anthrax lethal toxin (LT) has specific proteolytic activity against the upstream regulators of MAPKs, the MAPK kinases (MKKs). Using a murine model of intoxication, we show that LT causes the dose-dependent disruption of intestinal epithelial integrity, characterized by mucosal erosion, ulceration, and bleeding.

The effects of anthrax lethal toxin on host barrier function.

The pathological actions of anthrax toxin require the activities of its edema factor (EF) and lethal factor (LF) enzyme components, which gain intracellular access via its receptor-binding component, protective antigen (PA). LF is a metalloproteinase with specificity for selected mitogen-activated protein kinase kinases (MKKs), but its activity is not directly lethal to many types of primary and transformed cells in vitro. Nevertheless, in vivo treatment of several animal species with the combination of LF and PA (termed lethal toxin or LT) leads to morbidity and mortality, suggesting that LT-dependent toxicity is mediated by cellular interactions between host cells.

Mutations in GATA2 are associated with the autosomal dominant and sporadic monocytopenia and mycobacterial infection (MonoMAC) syndrome.

The syndrome of monocytopenia, B-cell and NK-cell lymphopenia, and mycobacterial, fungal, and viral infections is associated with myelodysplasia, cytogenetic abnormalities, pulmonary alveolar proteinosis, and myeloid leukemias. Both autosomal dominant and sporadic cases occur. We identified 12 distinct mutations in GATA2 affecting 20 patients and relatives with this syndrome, including recurrent missense mutations affecting the zinc finger-2 domain (R398W and T354M), suggesting dominant interference of gene function.

Neutrophil elastase mediates pathogenic effects of anthrax lethal toxin in the murine intestinal tract.

Neutrophils isolated from BALB/c or C57BL/6 mice and treated in vitro with anthrax lethal toxin release bioactive neutrophil elastase, a proinflammatory mediator of tissue destruction. Similarly, neutrophils isolated from mice treated with anthrax lethal toxin in vivo and cultured ex vivo release greater amounts of elastase than neutrophils from vehicle-treated controls. Direct measurements from murine intestinal tissue samples demonstrate an anthrax lethal toxin-dependent increase in neutrophil elastase activity in vivo as well.