Incorporating habitat use and life history to predict PCB residues in wild fish in an urban estuary.

Owing to the heterogenous distribution of contaminated sediments in urban estuaries, contaminant residues, such as polychlorinated biphenyls (PCBs), in fish tissue can vary widely. To investigate the relationship between PCBs in fish tissue and heterogeneity of PCBs in sediment, we developed a geospatial Biota-Sediment Accumulation Factor (BSAF) model for an urban estuary. The model predicts whole fish total PCB residues at a scale of 0.

Human well-being and natural capital indictors for Great Lakes waterfront revitalization.

Revitalization of natural capital amenities at the Great Lakes waterfront can result from sediment remediation, habitat restoration, climate resilience projects, brownfield reuse, economic redevelopment and other efforts. Practical indicators are needed to assess the socioeconomic and cultural benefits of these investments. We compiled U.

Effects of Tham Nasal Alkalinization on Airway Microbial Communities: A Pilot Study in Non-CF and CF Adults.

Objectives: In cystic fibrosis (CF), loss of CFTR-mediated bicarbonate secretion reduces the airway surface liquid (ASL) pH causing airway host defense defects. Aerosolized sodium bicarbonate can reverse these defects, but its effects are short-lived. Aerosolized tromethamine (THAM) also raises the ASL pH but its effects are much longer lasting.

Deep Lake Explorer: A web application for crowdsourcing the classification of benthic underwater video from the Laurentian Great Lakes.

Underwater video is increasingly used to study aspects of the Great Lakes benthos including the abundance of round goby and dreissenid mussels. The introduction of these two species have resulted in major ecological shifts in the Great Lakes, but the species and their impacts have heretofore been underassessed due to limitations of monitoring methods. Underwater video (UVID) can "sample" hard bottom sites where grab samplers cannot.

Amphotericin B induces epithelial voltage responses in people with cystic fibrosis.

Background: Approximately 10% of people with cystic fibrosis (CF) have mutations that result in little to no CFTR production and thus cannot benefit from CFTR modulators. We previously found that Amphotericin B (AmB), a small molecule that forms anion channels, restored HCO secretion and increased host defenses in primary cultures of CF airway epithelia. Further, AmB increased ASL pH in CFTR-null pigs, suggesting an alternative CFTR-independent approach to achieve gain-of-function.

A synoptic assessment of water quality in two Great Lakes connecting channels.

We conducted a probabilistic water quality assessment of two Great Lakes connecting channels, the St. Marys River, and the Lake Huron-Lake Erie Corridor (HEC) in 2014-2015. We compared the condition of the channels to each other and to the up- and down-river Great Lakes with data from an assessment of the Great Lakes nearshore conducted in 2015.

Randomized controlled study of aerosolized hypertonic xylitol versus hypertonic saline in hospitalized patients with pulmonary exacerbation of cystic fibrosis.

Background: Cystic fibrosis (CF) lung disease is characterized by chronic bacterial infection and recurrent pulmonary exacerbations. Xylitol is a 5-carbon sugar that can lower the airway surface salt concentration and augment innate immunity. We examined the safety and efficacy of aerosolized xylitol use for 2 weeks in subjects hospitalized with a pulmonary exacerbation of CF.

Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.

Background: Disruption of cystic fibrosis transmembrane conductance regulator (CFTR) anion channel function causes cystic fibrosis (CF), and lung disease produces most of the mortality. Loss of CFTR-mediated HCO3- secretion reduces the pH of airway surface liquid (ASL) in vitro and in neonatal humans and pigs in vivo. However, we previously found that, in older children and adults, ASL pH does not differ between CF and non-CF.

Determining preferences for ecosystem benefits in Great Lakes Areas of Concern from photographs posted to social media.

Relative valuation of potentially affected ecosystem benefits can increase the legitimacy and social acceptance of ecosystem restoration projects. As an alternative or supplement to traditional methods of deriving beneficiary preference, we downloaded from social media and classified ≈21,000 photographs taken in two Great Lakes Areas of Concern (AOC), the St. Louis River and the Milwaukee Estuary.

Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections.

Rationale: Previous work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFTR) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not reduce density of bacteria or markers of inflammation in the airway. These findings raise the possibility that infection and inflammation may progress independently of CFTR activity once cystic fibrosis lung disease is established.

Objectives: To better understand the relationship between CFTR activity, airway microbiology and inflammation, and lung function in subjects with cystic fibrosis and chronic airway infections.

A depth-adjusted ambient distribution approach for setting numeric removal targets for a Great Lakes Area of Concern beneficial use impairment: degraded benthos.

We compiled macroinvertebrate data collected from 1995 to 2014 from the St. Louis River Area of Concern (AOC) of Lake Superior. Our objective was to define depth-adjusted cutoff values for benthos condition classes to provide an analytical tool for quantifying progress toward achieving removal targets for the degraded benthos beneficial use impairment.

Repurposing tromethamine as inhaled therapy to treat CF airway disease.

In cystic fibrosis (CF), loss of CF transmembrane conductance regulator (CFTR) anion channel activity causes airway surface liquid (ASL) pH to become acidic, which impairs airway host defenses. One potential therapeutic approach is to correct the acidic pH in CF airways by aerosolizing HCO and/or nonbicarbonate pH buffers. Here, we show that raising ASL pH with inhaled HCO increased pH.

Acute administration of ivacaftor to people with cystic fibrosis and a mutation reveals smooth muscle abnormalities.

Background: Airflow obstruction is common in cystic fibrosis (CF), yet the underlying pathogenesis remains incompletely understood. People with CF often exhibit airway hyperresponsiveness, CF transmembrane conductance regulator (CFTR) is present in airway smooth muscle (ASM), and ASM from newborn CF pigs has increased contractile tone, suggesting that loss of CFTR causes a primary defect in ASM function. We hypothesized that restoring CFTR activity would decrease smooth muscle tone in people with CF.

Sonographic evidence of abnormal tracheal cartilage ring structure in cystic fibrosis.

Objectives/hypothesis: Tracheal cartilage ring structural abnormalities have been reported in cystic fibrosis (CF) mice and pigs. Whether similar findings are present in humans with CF is unknown. We hypothesized that tracheal cartilage ring shape and size would be different in people with CF.

Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor.

Background: Chronic sinusitis is universal in cystic fibrosis (CF) and our current treatments are ineffective in reversing sinus disease. The objective of this work was to determine if increasing CF transmembrane conductance regulator (CFTR) activity by ivacaftor could treat CF sinus disease and assess its effect on primary sinus epithelial cultures.

Methods: Case report of 1 patient with long-standing chronic sinus disease and a new diagnosis of CF with a mild mutation (P205S) and a severe mutation (G551D).

Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study.

Background: Disrupted HCO3(-) transport and reduced airway surface liquid (ASL) pH in cystic fibrosis (CF) may initiate airway disease. We hypothesized that ASL pH is reduced in neonates with CF.

Methods: In neonates with and without CF, we measured pH of nasal ASL.

Enhancement of respiratory mucosal antiviral defenses by the oxidation of iodide.

Recent reports postulate that the dual oxidase (DUOX) proteins function as part of a multicomponent oxidative pathway used by the respiratory mucosa to kill bacteria. The other components include epithelial ion transporters, which mediate the secretion of the oxidizable anion thiocyanate (SCN(-)) into airway surface liquid, and lactoperoxidase (LPO), which catalyzes the H(2)O(2)-dependent oxidation of the pseudohalide SCN(-) to yield the antimicrobial molecule hypothiocyanite (OSCN(-)). We hypothesized that this oxidative host defense system is also active against respiratory viruses.

Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions.

A recently discovered enzyme system produces antibacterial hypothiocyanite (OSCN(-)) in the airway lumen by oxidizing the secreted precursor thiocyanate (SCN(-)). Airway epithelial cultures have been shown to secrete SCN(-) in a CFTR-dependent manner. Thus, reduced SCN(-) availability in the airway might contribute to the pathogenesis of cystic fibrosis (CF), a disease caused by mutations in the CFTR gene and characterized by an airway host defense defect.

Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia.

Defective transepithelial electrolyte transport is thought to initiate cystic fibrosis (CF) lung disease. Yet, how loss of CFTR affects electrolyte transport remains uncertain. CFTR⁻(/)⁻ pigs spontaneously develop lung disease resembling human CF.

Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth.

Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF). Understanding the pathogenesis of this disease has been hindered, however, by the lack of an animal model with characteristic features of CF. To overcome this problem, we recently generated pigs with mutated CFTR genes.

Patterns and density of early tracheal colonization in intensive care unit patients.

Objective: The study aimed to describe the patterns and density of early tracheal colonization among intubated patients and to correlate colonization status with levels of antimicrobial peptides and inflammatory cytokines.

Design: The was a prospective cohort study.

Setting: The study was conducted in medical and cardiovascular intensive care units of a tertiary referral hospital.

Safety assessment of inhaled xylitol in subjects with cystic fibrosis.

Background: Xylitol is a 5-carbon sugar that can lower the airway surface salt concentration, thus enhancing innate immunity. We tested the safety and tolerability of aerosolized iso-osmotic xylitol in subjects with cystic fibrosis.

Methods: In this pilot study, 6 subjects with cystic fibrosis and an FEV1>60% predicted underwent a baseline spirometry followed by exposures to aerosolized saline (10 ml) and 5% xylitol (10 ml).

Bronchoscopic assessment of airway retention time of aerosolized xylitol.

Background: Human airway surface liquid (ASL) has abundant antimicrobial peptides whose potency increases as the salt concentration decreases. Xylitol is a 5-carbon sugar that has the ability to lower ASL salt concentration, potentially enhancing innate immunity. Xylitol was detected for 8 hours in the ASL after application in airway epithelium in vitro.

Safety assessment of inhaled xylitol in mice and healthy volunteers.

Background: Xylitol is a 5-carbon sugar that can lower the airway surface salt concentration, thus enhancing innate immunity. We tested the safety and tolerability of aerosolized iso-osmotic xylitol in mice and human volunteers.

Methods: This was a prospective cohort study of C57Bl/6 mice in an animal laboratory and healthy human volunteers at the clinical research center of a university hospital.