Qualitative understanding of experiences of people with cystic fibrosis in a treatment discontinuation trial: The QUEST study.

Background: As people with cystic fibrosis (PWCF) live longer due to the breakthrough drug elexacaftor-tezacaftor-ivacaftor (ETI), they have questioned whether other CF therapies could be safely discontinued. SIMPLIFY was the first prospective, randomized trial to evaluate non-inferiority of discontinuing versus continuing two therapies. The QUEST (Qualitative Understanding of Experiences in the SIMPLIFY Trial) study was conducted to understand experiences of PWCF enrolled in SIMPLIFY, including why they joined, perceptions of randomization, decision-making around study withdrawal, and considerations for future discontinuation studies.

Associations Between Chronic Rhinosinusitis and the Development of Non-Cystic Fibrosis Bronchiectasis.

Background: Studies have shown an association between chronic rhinosinusitis (CRS) and non-cystic fibrosis (CF) bronchiectasis.

Objective: We aimed to determine whether CRS increases the risk of developing non-CF bronchiectasis.

Methods: A retrospective analysis was conducted utilizing electronic medical records from an academic center.

Food security during nuclear winter: a preliminary agricultural sector analysis for Aotearoa New Zealand.

Aim: We aimed to estimate the current dietary energy content of food exports for Aotearoa New Zealand and food security during "nuclear winter" scenarios following a nuclear war.

Methods: From published sources we estimated dietary energy available from the major domains of food exports, with adjustments for wastage. The impacts on food production in New Zealand during three nuclear winter scenarios were based on those published in Nature Food in 2022 and from an earlier New Zealand Planning Council study.

A patient with untreated preeclampsia and peripartum cardiomyopathy.

The pathophysiologic process of peripartum cardiomyopathy, a rare and potentially life-threatening condition, is not completely understood. One theory is a possible link between the development of preeclampsia and peripartum cardiomyopathy. These two conditions have been linked to significant mortality in peripartum or postpartum patients.

The potential human health hazard of nitrates in drinking water: a media discourse analysis in a high-income country.

Background: Recent studies linking low levels of nitrate in drinking water to colorectal cancer have raised public concerns over nitrate contamination. The aim of this study was to analyze the media discourse on the potential human health hazard of nitrates in drinking water in a high-income country with a large livestock industry: New Zealand (NZ).

Methods: Searches of media sources ("major newspapers") held by the Factiva database for the NZ setting in the five-year period 17 December 2016 to 20 December 2021.

Beyond muddy waters: Three Waters reforms required to future-proof water service delivery and protect public health in Aotearoa New Zealand.

A 2016 drinking water-related campylobacteriosis outbreak in Aotearoa New Zealand made much of an entire town sick leading to reforms colloquially called "Three Waters", which aims to improve the management and delivery of waste, storm and drinking water systems. Public discourse on the Three Waters reforms has been dominated by anti-co-governance rhetoric, concerns around privatisation and loss of local control and alternative less comprehensive reform models. This debate has drowned out the fundamental problem statement justifying the reforms, that is, the management of drinking water resources is currently: 1) demonstrably inadequate to protect public health and promote health equity; and 2) economically inefficient.

Telehealth and CFTR modulators: Accelerating innovative models of cystic fibrosis care.

Better health and longer survival for many people with cystic fibrosis (PwCF) compels the continued evolution of the CF care model. Designed to deliver specialized care for a complex chronic condition, the model is organized around interdisciplinary healthcare teams at dedicated care centers. Introduction of CFTR modulators and the COVID-19 pandemic have catalyzed the model's evolution.

Looking toward the future: Approaching care of the aging CF patient.

Caring for people with cystic fibrosis has changed considerably since the first description of the disorder and continues to evolve in the era of highly effective modulator therapy. These new treatment advancements are resulting in improved health outcomes in an ever-growing adult population with improved long-term survival. This study explores potential comorbidities and mental health implications associated with increased longevity and survivorship.

Adult-Onset Still's Disease Presenting as Macrophage-Activation Syndrome With Critical Illness in the Third Trimester of Pregnancy: A Case Report.

Unlabelled: We report a case of a young woman with adult-onset Still's disease presenting as macrophage-activation syndrome complicated by shock and respiratory failure during the third trimester of pregnancy.

Case Summary: A previously healthy 26-year-old woman at 35 weeks of gestation presented with 1 week of constitutional symptoms and was found to be febrile, tachycardic, and hypotensive. She delivered a healthy neonate by cesarean section.

Prevalence of Bronchiectasis in Patients with Chronic Rhinosinusitis in a Tertiary Care Center.

Background: Whereas chronic rhinosinusitis (CRS) is associated with asthma, and vice versa, the association between CRS and other lower respiratory conditions is not well-established. Bronchiectasis is characterized by permanent damage of the airways, and as many as 45% of bronchiectasis patients have CRS, but the prevalence of bronchiectasis among CRS patients is not known.

Objective: To determine the prevalence of bronchiectasis among CRS patients and to characterize demographic and clinical features of patients with bronchiectasis and CRS.

Bronchoscopy on Intubated COVID-19 Patients is Associated with Low Infectious Risk to Operators at a High-Volume Center Using an Aerosol-minimizing Protocol.

Background: The coronavirus disease 2019 (COVID-19) pandemic raised concern for exposure to healthcare providers through aerosol generating procedures, such as bronchoalveolar lavage (BAL). Current society guidelines recommended limiting use of BAL to reduce operators' risk for infection, yet data on the infection rate for providers after BAL is sparse. Since March 2020, our institution used a modified protocol to perform over 450 BALs on intubated COVID-19 patients.

Formative evaluation of a dashboard to support coproduction of healthcare services in cystic fibrosis.

Background: Healthcare coproduction engages patients and clinicians to design and execute services, yet little is known about tools that facilitate coproduction. Our objective was to understand uptake, experiences, benefits, and limitations of a dashboard to support patient-clinician partnerships within the cystic fibrosis (CF) community.

Methods: People living with CF (PwCF) and clinicians co-designed a dashboard that displayed patient-reported and clinical data.

The Komodo dragon (Varanus komodoensis) genome and identification of innate immunity genes and clusters.

Background: We report the sequencing, assembly and analysis of the genome of the Komodo dragon (Varanus komodoensis), the largest extant lizard, with a focus on antimicrobial host-defense peptides. The Komodo dragon diet includes carrion, and a complex milieu of bacteria, including potentially pathogenic strains, has been detected in the saliva of wild dragons. They appear to be unaffected, suggesting that dragons have robust defenses against infection.

Polymyxin B Pharmacokinetics in Adult Cystic Fibrosis Patients.

Objectives: Polymyxin B pharmacokinetics (PK) in adults with cystic fibrosis (CF) are not well described. The goals of this pilot study were to identify a PK model for patients with CF receiving polymyxin B with exploration of covariate relationships of the PK parameters, to compare polymyxin B PK parameters in adults without CF, and to probe exposures associated with different dosing schemes through simulation.

Methods: Adult patients with CF treated with polymyxin B at New York-Presbyterian Hospital had PK samples measured by liquid chromatography-mass spectrometry (MS)/MS.

The relationship between sweat chloride levels and mortality in cystic fibrosis varies by individual genotype.

Rationale: The association between CFTR genotype, sweat chloride and mortality has been inconsistent, but no previous analyses have examined the association stratified by individual genotypes.

Objectives: To evaluate the genotype-specific association between sweat chloride and mortality.

Methods: The CFF Patient Registry was assessed and included all patients in the registry between 1996 and 2012 with at least one F508del allele.

Antibiotic duration and changes in FEV are not associated with time until next exacerbation in adult cystic fibrosis: a single center study.

Background: Pulmonary exacerbations (PEx) are a major driver of morbidity and mortality in cystic fibrosis and reducing their frequency by extending the time between them is an important therapeutic goal. Although treatment decisions for exacerbations are often made based on dynamic changes in lung function, it is not clear if these changes truly impact future exacerbation risk. We analyzed adults with chronic Pseudomonas aeruginosa infection to determine whether changes in FEV or duration of intravenous antibiotic therapy were associated with time to the next pulmonary exacerbation.

Aminoglycoside resistance of Pseudomonas aeruginosa in cystic fibrosis results from convergent evolution in the mexZ gene.

Rationale: Aminoglycoside (AG) resistance of Pseudomonas aeruginosa in cystic fibrosis (CF) is associated with poorer clinical outcomes and is usually due to overexpression of the efflux pump MexXY. MexXY is regulated by mexZ, one of the most commonly mutated genes in CF P. aeruginosa isolates.

Gene therapy in cystic fibrosis.

Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene and is the most common life-shortening genetic defect in Caucasians. Life expectancy in CF has improved substantially over the last 75 years because of treatments aimed at end-organ complications. Since the CFTR gene was discovered in 1989 more than 1900 mutations have been reported to cause CF and significant effort has been put forth into gene therapy to find a mutation independent "cure" for CF.

TLR15 is unique to avian and reptilian lineages and recognizes a yeast-derived agonist.

The TLRs represent a family of pattern recognition receptors critical in the induction of vertebrate immune responses. Between 10 and 13 different TLR genes can be identified in each vertebrate species, with many represented as orthologous genes in different species. The agonist specificity of orthologous TLR is also highly conserved.