Attitudes toward and preparedness for lung transplantation among individuals with cystic fibrosis in the era of highly effective modulators.

Background: Outcomes for individuals with cystic fibrosis (CF) have improved due to highly effective modulator therapy (HEMT). However, lung transplant (LTx) remains an important treatment for people with advanced lung disease. This study assessed attitudes and knowledge about LTx in the HEMT era.

A national postgraduate nurse practitioner and physician assistant fellowship in cystic fibrosis: An innovative approach to the provider shortage in complex and rare disease.

Cystic fibrosis (CF) is a complex life-limiting genetic condition that affects the respiratory, digestive, reproductive system, and sweat glands. Advances in treatment have led to improved survival and quality of life. Today, most persons with CF live to adulthood but require highly specialized care at accredited CF Care Centers.

Seroprevalence of SARS-CoV-2 IgG in people with cystic fibrosis.

Background: When the first known US case of COVID-19 (Coronavirus Disease 2019) was reported in early 2020, little was known about the impact of this novel virus on the cystic fibrosis community. As the majority of individuals with CF have chronic lung disease, this population was initially considered to be at high risk for severe disease as infection with a multitude of viruses has proven to cause pulmonary exacerbation. SARS-CoV-2 virus has proven challenging to study given the multiple disease manifestations, range of severity, and wave-like phenomenon that varies geographically.

A longitudinal analysis of respiratory symptoms in people with cystic fibrosis with advanced lung disease on and off ETI.

People with CF (PwCF), particularly those with advanced lung disease (ALD), experience frequent respiratory symptoms. A major CF breakthrough was the approval of elexacaftor/tezacaftor/ivacaftor (ETI) in 2019, which has been shown to improve symptoms and lung function in the CF population, and decrease pulmonary exacerbations. The purpose of this study was to analyze longitudinal changes in respiratory symptoms over 24 months in ETI-treated and untreated PwCF with ALD Symptoms were measured among CF adults with ppFEV < 40% (N = 48, 24 ETI-treated, 24 untreated) using the CFRSD-CRISS and the CFQ-R [respiratory].

Predicting Early Versus Late In-Hospital Mortality in the Trauma Population.

This study aimed to evaluate non-survivors who were admitted to a level I trauma center but later died, in terms of predicting who would expire early vs late. This is a single-center study of Trauma Registry data, from July 3, 2016, to February 24, 2022. The inclusion criteria were based upon age (≥18 years) and in-hospital mortality.

The Algorithm Examining the Risk of Massive Transfusion (ALERT) Score Accurately Predicts Massive Transfusion at the Scene of Injury and on Arrival to the Trauma Bay: A Retrospective Analysis.

Background: Massive transfusion (MT) is required to resuscitate traumatically injured patients with complex derangements. Scoring systems for MT typically require laboratory values and radiological imaging that may delay the prediction of MT.

Study Design: The Trauma ALgorithm Examining the Risk of massive Transfusion (Trauma ALERT) study was an observational cohort study.

Apparent Reversal of a Successful Peripheral Neural Blockade With Intravenous Lipid Emulsion After Treatment for Local Anesthetic Systemic Toxicity: A Case Report.

The use of local anesthetics for improved pain management is well established. However, significant morbidity may be caused by local anesthetic systemic toxicity (LAST) from inadvertent intravascular injection or excessive dosing of local anesthetics. Despite incomplete understanding of the mechanism of action of intravenous lipid emulsions (ILE), their use has become a first-line therapy for treating LAST.

Incorporating patient and caregiver feedback into lung transplant referral guidelines for individuals with cystic fibrosis-Preliminary findings from a novel paradigm.

Background: Lung transplantation is a common therapeutic option for individuals with cystic fibrosis (CF) and advanced lung disease, yet many individuals with CF are not appropriately referred for evaluation. The present study sought to enhance CF transplant referral guidelines by integrating patient-centered input to identify possible psychosocial barriers contributing to suboptimal referral for appropriate CF transplant candidates.

Methods: As a component of developing the Cystic Fibrosis Foundation (CFF) Lung Transplant Referral Consensus Guidelines, we convened a focus group of lung transplant recipients with CF and two spouses of CF recipients.

Bioorthogonal non-canonical amino acid tagging reveals translationally active subpopulations of the cystic fibrosis lung microbiota.

Culture-independent studies of cystic fibrosis lung microbiota have provided few mechanistic insights into the polymicrobial basis of disease. Deciphering the specific contributions of individual taxa to CF pathogenesis requires comprehensive understanding of their ecophysiology at the site of infection. We hypothesize that only a subset of CF microbiota are translationally active and that these activities vary between subjects.

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.

Disruption of Cross-Feeding Inhibits Pathogen Growth in the Sputa of Patients with Cystic Fibrosis.

A critical limitation in the management of chronic polymicrobial infections is the lack of correlation between antibiotic susceptibility testing (AST) and patient responses to therapy. Underlying this disconnect is our inability to accurately recapitulate the environment and complex polymicrobial communities However, emerging evidence suggests that, if modeled and tested accurately, interspecies relationships can be exploited by conventional antibiotics predicted to be ineffective by standard AST. As an example, under conditions where relies on cocolonizing organisms for nutrients (i.

Effects of Signal Disruption Depends on the Substrate Preference of the Lactonase.

Many bacteria produce and use extracellular signaling molecules such as acyl homoserine lactones (AHLs) to communicate and coordinate behavior in a cell-density dependent manner, a communication system called quorum sensing (QS). This system regulates behaviors including but not limited to virulence and biofilm formation. We focused on , a human opportunistic pathogen that is involved in acute and chronic lung infections and which disproportionately affects people with cystic fibrosis.

Risk factors for neo-osteogenesis in cystic fibrosis and non-cystic fibrosis chronic rhinosinusitis.

Background: The purpose of this retrospective review was to determine how patient-related factors and culture data affect neo-osteogenesis in patients with chronic rhinosinusitis (CRS) and patients with cystic fibrosis (CF) with CRS.

Methods: Information from a database associated with a large tertiary medical center was used to assess adult patients with CF CRS and non-CF CRS (total, n = 102; CF CRS, n = 31; non-CF CRS, n = 71). Radiologic evidence of neo-osteogenesis was measured using the Global Osteitis Scoring Scale (GOSS), and mucosal disease was assessed using the Lund-Mackay score (LMS) by 2 independent reviewers who were blinded to the patient's disease state.

Prevalence and factors associated with overweight and obesity in adults with cystic fibrosis: A single-center analysis.

Background: The relation between malnutrition and pulmonary death in patients with cystic fibrosis (CF) has resulted in intensive nutritional intervention over the last few decades, leading to a significant decline in underweight and the emergence of overweight/obesity as a potential new problem.

Methods: We performed a cross-sectional database analysis of 484 adults with CF seen at the University of Minnesota CF Center between January 2015-January 2017, to determine the prevalence and pulmonary/cardiovascular risk factors associated with overweight and obesity in this population.

Results: Mean age was 35.

Case report of synchronous post-lung transplant colon cancers in the era of colorectal cancer screening recommendations in cystic fibrosis: screening "too early" before it's too late.

Background: The increasing life expectancy of individuals with Cystic Fibrosis (CF) is likely to be associated with new age-related challenges, colorectal cancer (CRC) most notably; recent consensus recommendations for CRC screening published in 2018 represent an important early step in addressing the emerging awareness of CF as a gastrointestinal cancer syndrome. These recommendations, however, need to be further refined based on more systematic data. We discuss an illustrative first-ever case of synchronous CRC arising in a post-lung transplant individual with CF within the recommended surveillance interval after a well-documented prior normal colonoscopy.

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines.

Objective: Provide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF.

Methods: The CF Foundation organized a multidisciplinary committee to develop CF Lung Transplant Referral Consensus Guidelines. Three workgroups were formed: timing for transplant referral; modifiable barriers to transplant; and transition to transplant care.

Improving Clinic Operational Efficiency and Utilization with RTLS.

New sources of operational data are leading to novel healthcare delivery system design and opportunities to support operational planning and decision-making. Technologies such as real time locating systems (RTLS) provide a unique view and understanding of how healthcare delivery settings behave and respond to operational design changes. In this paper RTLS data from an outpatient clinical setting is leveraged to identify the appropriate number of scheduled providers in order to improve the utilization of the clinical space while balancing the negative effects of clinic congestion.

Bad Language.

Bad language in stereochemistry-and elsewhere-can lead to sloppy thinking. In this Essay I review the history of stereochemical concepts and vocabulary in the hope that it may contribute a little to better thinking and communication.

Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial.

Rationale: Cystic fibrosis (CF) is characterized by dietary antioxidant deficiencies, which may contribute to an oxidant-antioxidant imbalance and oxidative stress.

Objectives: Evaluate the effects of an oral antioxidant-enriched multivitamin supplement on antioxidant concentrations, markers of inflammation and oxidative stress, and clinical outcomes.

Methods: In this investigator-initiated, multicenter, randomized, double-blind, controlled trial, 73 pancreatic-insufficient subjects with CF 10 years of age and older with an FEV between 40% and 100% predicted were randomized to 16 weeks of an antioxidant-enriched multivitamin or control multivitamin without antioxidant enrichment.

Pulmonary aspiration of sinus secretions in patients with cystic fibrosis.

Background: Indirect evidence suggests that sinonasal secretions are aspirated into the lungs of patients with cystic fibrosis (CF), contributing to infection, subsequent tissue damage, and decreased lung function. Our objective is to determine whether sinonasal secretions are transferred to the lungs in patients with CF-related sinus disease and healthy subjects, particularly in the recumbent position and during sleep.

Methods: We performed a prospective, controlled trial to detect pulmonary aspiration of radiolabeled albumin applied to the nasal mucosa of study subjects with chronic sinusitis related to CF and control subjects without sinus disease.

Total Pancreatectomy With Intraportal Islet Autotransplantation as a Treatment of Chronic Pancreatitis in Patients With CFTR Mutations.

Objectives: Chronic pancreatitis (CP) is an infrequent but debilitating complication associated with CFTR mutations. Total pancreatectomy with islet autotransplantation (TPIAT) is a treatment option for CP that provides pain relief and preserves β-cell mass, thereby minimizing the complication of diabetes mellitus. We compared outcomes after TPIAT for CP associated with CFTR mutations to CP without CTFR mutations.

16S rRNA gene sequencing reveals site-specific signatures of the upper and lower airways of cystic fibrosis patients.

Background: Metastasis of upper airway microbiota may have significant implications in the development of chronic lung disease. Here, we compare bacterial communities of matched sinus and lung mucus samples from cystic fibrosis (CF) subjects undergoing endoscopic surgery for treatment of chronic sinusitis.

Methods: Mucus from one maxillary sinus and expectorated sputum were collected from twelve patients.

Evidence and Role for Bacterial Mucin Degradation in Cystic Fibrosis Airway Disease.

Chronic lung infections in cystic fibrosis (CF) patients are composed of complex microbial communities that incite persistent inflammation and airway damage. Despite the high density of bacteria that colonize the lower airways, nutrient sources that sustain bacterial growth in vivo, and how those nutrients are derived, are not well characterized. In this study, we examined the possibility that mucins serve as an important carbon reservoir for the CF lung microbiota.

Colonoscopic screening shows increased early incidence and progression of adenomas in cystic fibrosis.

Background: Colorectal cancer is an emerging problem in cystic fibrosis (CF). The goal of this study was to evaluate adenoma detection by systematic colonoscopic screening and surveillance.

Methods: We analyzed prospectively collected results of colonoscopies initiated at age 40years from 88 CF patients at a single Cystic Fibrosis Center.

Intermolecular atom-atom bonds in crystals?

Some questions are raised concerning the interpretation of distances between atoms of neighbouring molecules in crystals.