J Adv Nurs. 2022 Jul 12. doi: 10.1111/jan.15358. Online ahead of print.

ABSTRACT

AIMS: To synthesize qualitative studies of subjective experience of pregnancy in people with cystic fibrosis.

DESIGN: Thematic synthesis of qualitative studies.

DATA SOURCES: We searched PUBMED, CINAHL, EMBASE. PsicINFO and Social Sciences Citation Index for qualitative studies published in English, which reported on pregnancy in people with cystic fibrosis. Searches were carried out in March 2021, updated in June 2022.

REVIEW METHODS: Studies that met the inclusion criteria were appraised for quality using the Critical Appraisal Skills Programme (CASP) for qualitative research. Data were extracted from the studies, analyse and synthesise using thematic synthesis approach.

RESULTS: Thirteen studies were included in the review and 'Walking on a wire' framework was conceptualized. We found three analytical themes: (1) desire for information related to pregnancy in cystic fibrosis, (2) factors at play in decision-making related to pregnancy for people with cystic fibrosis and (3) pregnancy experience and eight descriptive themes: (1) information topics, (2) CF healthcare team/PwCF as a provider of reproductive health information, (3) information timing, (4) barriers to information delivery, (5) barriers to decision-making and stresses relating to the process of trying to conceive, (6) environmental factors, (7) coping with challenges, and (8) moving towards parenthood.

CONCLUSION: For people with cystic fibrosis, pregnancy is a complex pathway: the amount of knowledge about cystic fibrosis and sexual and reproductive health, barriers to pregnancy and environmental factors provides the background to decision-making. Moreover, coping with pregnancy is a challenging experience, where they have to mediate between the physical and emotional implications of planning a pregnancy and the limitations imposed by the chronic health conditions.

IMPACT: Understanding the psychological experiences of people with cystic fibrosis (PwCF) will improve future research and practice. Education about sexual and reproductive health and psychosocial care programmes are necessary to help PwCF deal with the challenges related to pregnancy. Hospitals should enhance the development of specific programmes to promote the well-being of individuals with CF who are planning a pregnancy.

PMID:35819171 | DOI:10.1111/jan.15358

Nutrition. 2022 May 6;102:111725. doi: 10.1016/j.nut.2022.111725. Online ahead of print.

ABSTRACT

With increasing life expectancy of patients with cystic fibrosis (CF), gastrointestinal manifestations of the disease have been increasingly brought into focus. This was a systematic review of the PubMed database and ongoing phase III clinical trials that aimed to summarize recent (published after June 1 2016) studies reporting the effects of nutritional interventions on anthropometric measures (weight, height, and body mass index) in patients with CF. Two ongoing trials and 40 published studies (18 interventional and 22 observational) were identified. Key results supported the benefits of comprehensive, individualized nutritional plans, high-fat, high-calorie diet including high-quality carbohydrates, and enteric tube feeding (albeit the latter was derived from observational studies only). In contrast, the supplementation of probiotics, lipids, docosahexaenoic, glutathione, or antioxidant-enriched multivitamin appeared to have little effect on anthropometric measures.

PMID:35816813 | DOI:10.1016/j.nut.2022.111725

Am J Respir Crit Care Med. 2022 Jul 11. doi: 10.1164/rccm.202202-0392OC. Online ahead of print.

ABSTRACT

RATIONALE: The triple-combination regimen elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to be safe and efficacious in children aged 6 through 11 years with cystic fibrosis (CF) and at least one F508del-CFTR allele in a phase 3, open-label, single-arm study.

OBJECTIVES: To further evaluate the efficacy and safety of ELX/TEZ/IVA in children 6 through 11 years of age with CF heterozygous for F508del and a minimal function CFTR mutation (F/MF genotypes) in a randomized, double-blind, placebo-controlled phase 3b trial.

METHODS: Children were randomized to receive either ELX/TEZ/IVA (N = 60) or placebo (N = 61) during a 24-week treatment period. The dose of ELX/TEZ/IVA administered was based on weight at screening, with children <30 kg receiving ELX 100 mg once daily, TEZ 50 mg once daily, and IVA 75 mg every 12 hours, and children ≥30 kg receiving ELX 200 mg once daily, TEZ 100 mg once daily, and IVA 150 mg every 12 hours (adult dose).

MEASUREMENTS AND MAIN RESULTS: The primary endpoint was absolute change in lung clearance index2.5 (LCI2.5) from baseline through Week 24. Children given ELX/TEZ/IVA had a mean decrease in LCI2.5 of 2.29 units (95% CI, 1.97 to 2.60) compared with 0.02 units (95% CI, -0.29 to 0.34) in children given placebo (between-group treatment difference, -2.26 units; 95% CI, -2.71 to -1.81, P<0.0001). ELX/TEZ/IVA treatment also led to improvements in the secondary endpoint of sweat chloride concentration (between-group treatment difference, -51.2 mmol/L; 95% CI, -55.3 to -47.1), and in the other endpoints of percent predicted FEV1 (between-group treatment difference, 11.0 percentage points; 95%, CI 6.9 to 15.1) and Cystic Fibrosis Questionnaire-Revised respiratory domain score (between-group treatment difference, 5.5 points; 95% CI, 1.0 to 10.0) compared with placebo from baseline through Week 24. The most common adverse events in children receiving ELX/TEZ/IVA were headache and cough (30.0% and 23.3%, respectively); most adverse events were mild or moderate in severity.

CONCLUSIONS: In this first randomized, controlled study of a CFTR modulator conducted in children 6 through 11 years of age with F/MF genotypes, ELX/TEZ/IVA treatment led to significant improvements in lung function, as well as robust improvements in respiratory symptoms and CFTR function. ELX/TEZ/IVA was generally safe and well tolerated in this pediatric population with no new safety findings. Clinical trial registration available at www.

CLINICALTRIALS: gov, ID: NCT04353817.

PMID:35816621 | DOI:10.1164/rccm.202202-0392OC

Am J Respir Crit Care Med. 2022 Jul 11. doi: 10.1164/rccm.202111-2606OC. Online ahead of print.

ABSTRACT

RATIONALE: The incidence and sites of mucus accumulation, and molecular regulation of mucin gene expression, in COVID-19 lung disease have not been reported.

OBJECTIVES: Characterize incidence of mucus accumulation and the mechanisms mediating mucin hypersecretion in COVID-19 lung disease.

METHODS: Airway mucus and mucins were evaluated in COVID-19 autopsy lungs by AB-PAS and immunohistochemical staining, RNA in situ hybridization, and spatial transcriptional profiling. SARS-CoV-2-infected human bronchial epithelial (HBE) cultures were utilized to investigate mechanisms of SARS-CoV-2-induced mucin expression and synthesis and test candidate countermeasures.

MEASUREMENTS AND MAIN RESULTS: MUC5B and variably MUC5AC RNA levels were increased throughout all airway regions of COVID-19 autopsy lungs, notably in the sub-acute/chronic disease phase following SARS-CoV-2 clearance. In the distal lung, MUC5B-dominated mucus plugging was observed in 90% of COVID-19 subjects in both morphologically identified bronchioles and microcysts, and MUC5B accumulated in damaged alveolar spaces. SARS-CoV-2-infected HBE cultures exhibited peak titers 3 days post inoculation, whereas induction of MUC5B/MUC5AC peaked 7-14 days post inoculation. SARS-CoV-2 infection of HBE cultures induced expression of EGFR ligands and inflammatory cytokines (e.g., IL-1α/β) associated with mucin gene regulation. Inhibiting EGFR/IL-1R pathways, or dexamethasone administration, reduced SARS-CoV-2-induced mucin expression.

CONCLUSIONS: SARS-CoV-2 infection is associated with a high prevalence of distal airspace mucus accumulation and increased MUC5B expression in COVID-19 autopsy lungs. HBE culture studies identified roles for EGFR and IL-1R signaling in mucin gene regulation post SARS-CoV-2 infection. These data suggest that time-sensitive mucolytic agents, specific pathway inhibitors, or corticosteroid administration may be therapeutic for COVID-19 lung disease. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/).

PMID:35816430 | DOI:10.1164/rccm.202111-2606OC

J Vis Exp. 2022 Jun 22;(184). doi: 10.3791/63528.

ABSTRACT

Fluorescence-based studies are suitable for high-throughput plate reader assays of cells in culture. They have been commonly employed for drug discovery campaigns targeting recombinant ion channel proteins overexpressed in cells such as HEK-293 cells. However, there is increasing emphasis on the use of tissue-relevant cell lines for studying the effects of small molecule interventions. The following protocol describes the adaptation of a fluorescence-based membrane potential assay for the study of ion channels endogenously expressed in epithelial cell lines. The membrane potential assay details a high-throughput assay for chloride channel activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in two commonly studied epithelial cell lines, Caco-2 and Calu-3. In addition, this paper describes a novel application of this system to measure the activity of the Epithelial Sodium Channel (ENaC) in a high-throughput format in the same epithelial cell lines. Together, these fluorescence-based assays provide a robust and flexible platform for studying small molecule modulators, targeting two epithelial channels in a relevant cellular context.

PMID:35815993 | DOI:10.3791/63528

Int Forum Allergy Rhinol. 2022 Jul 11. doi: 10.1002/alr.23060. Online ahead of print.

NO ABSTRACT

PMID:35815929 | DOI:10.1002/alr.23060

Front Pharmacol. 2022 Jun 17;13:891041. doi: 10.3389/fphar.2022.891041. eCollection 2022.

ABSTRACT

The so-called primary interface between the SNARE complex and synaptotagmin-1 (Syt1) is essential for Ca2+-triggered neurotransmitter release in neuronal synapses. The interacting residues of the primary interface are conserved across different species for synaptotagmins (Syt1, Syt2, Syt9), SNAP-25, and syntaxin-1A homologs involved in fast synchronous release. This Ca2+-independent interface forms prior to Ca2+-triggering and plays a role in synaptic vesicle priming. This primary interface is also conserved in the fusion machinery that is responsible for mucin granule membrane fusion. Ca2+-stimulated mucin secretion is mediated by the SNAREs syntaxin-3, SNAP-23, VAMP8, Syt2, and other proteins. Here, we designed and screened a series of hydrocarbon-stapled peptides consisting of SNAP-25 fragments that included some of the key residues involved in the primary interface as observed in high-resolution crystal structures. We selected a subset of four stapled peptides that were highly α-helical as assessed by circular dichroism and that inhibited both Ca2+-independent and Ca2+-triggered ensemble lipid-mixing with neuronal SNAREs and Syt1. In a single-vesicle content-mixing assay with reconstituted neuronal SNAREs and Syt1 or with reconstituted airway SNAREs and Syt2, the selected peptides also suppressed Ca2+-triggered fusion. Taken together, hydrocarbon-stapled peptides that interfere with the primary interface consequently inhibit Ca2+-triggered exocytosis. Our inhibitor screen suggests that these compounds may be useful to combat mucus hypersecretion, which is a major cause of airway obstruction in the pathophysiology of COPD, asthma, and cystic fibrosis.

PMID:35814209 | PMC:PMC9258623 | DOI:10.3389/fphar.2022.891041

Front Mol Biosci. 2022 Jun 24;9:919187. doi: 10.3389/fmolb.2022.919187. eCollection 2022.

ABSTRACT

Ferroptosis is a distinct type of programmed cell death (PCD) that depends on iron and is characterized by the accumulation of intracellular iron, exhaustion of glutathione, deactivation of glutathione peroxidase, and promotion of lipid peroxidation. Recently, accumulated investigations have demonstrated that ferroptosis is strongly correlated with the initiation and development of many lung diseases. In this review, we summarized the contribution of ferroptosis to the pathologic process of lung diseases, namely, obstructive lung diseases (chronic obstructive pulmonary disease, asthma, and cystic fibrosis), interstitial lung diseases (pulmonary fibrosis of different causes), pulmonary diseases of vascular origin (ischemia-reperfusion injury and pulmonary hypertension), pulmonary infections (bacteria, viruses, and fungi), acute lung injury, acute respiratory distress syndrome, obstructive sleep apnea, pulmonary alveolar proteinosis, and lung cancer. We also discussed the therapeutic potential of targeting ferroptosis for these lung diseases.

PMID:35813823 | PMC:PMC9263225 | DOI:10.3389/fmolb.2022.919187

Front Mol Biosci. 2022 Jun 24;9:921680. doi: 10.3389/fmolb.2022.921680. eCollection 2022.

ABSTRACT

Cystic fibrosis (CF) is a lethal hereditary disease caused by loss-of-function mutations of the chloride channel cystic fibrosis transmembrane conductance regulator (CFTR). With the development of small-molecule CFTR modulators, including correctors that facilitate protein folding and expression and potentiators that promote channel activity, about 90% of the CF patients are now receiving efficacious target therapies. G542X-CFTR, a premature termination codon (PTC) mutation, is the most common disease-associated mutation found in the remaining 10% of patients that await effective drugs to rectify the fundamental defects caused by PTC. In this study, we employed biophysical and biochemical techniques to characterize the pharmacological responses of the translational products of G542X-CFTR to a range of new CFTR modulators. Specifically, we identified two different proteins translated from the G542X-CFTR cDNA using western blotting: the C-terminus truncated protein that responds to the C1 corrector which binds to the N-terminal part of the protein and a full-length CFTR protein through the read-through process. Electrophysiological data suggest that the read-through protein, but not the C-terminus truncated one, is functional and responds well to CFTR potentiators despite a lower open probability compared to wild-type CFTR. As the expression of the read-through products can be increased synergistically with the read-through reagent G418 and C1 corrector, but not with combinations of different types of correctors, we concluded that an efficacious read-through reagent is a prerequisite for mitigating the deficits of G542X-CFTR. Moreover, the CFTR potentiators may help improve the effectiveness of future combinational therapy for patients carrying PTCs such as G542X.

PMID:35813815 | PMC:PMC9263564 | DOI:10.3389/fmolb.2022.921680

Front Pediatr. 2022 Jun 22;10:926248. doi: 10.3389/fped.2022.926248. eCollection 2022.

ABSTRACT

BACKGROUND: An exercise test combined with a multiple breath washout nitrogen test (MBWN2) may offer a comprehensive clinical evaluation of cystic fibrosis (CF) disease in children with normal spirometry. The purpose of the present study is to explore whether information derived from spirometry, MBWN2, and exercise tests can help the CF multidisciplinary team to characterize time free from hospitalization due to pulmonary exacerbation (PE) in a cohort of pediatric patients with CF.

METHODS: This prospective observational study was carried out at the Lombardia Region Reference Center for Cystic Fibrosis in Milano, Italy. In 2015, we consecutively enrolled children and adolescents aged <18 years with spirometry, MBWN2, and Godfrey exercise test performed during an outpatient visit.

RESULTS: Over a median follow-up time of 2.2 years (interquartile range [IQR], 2.01; 3.18), 28 patients aged between 13.0 and 17.4 years were included. When lung functions were outside the normal range, 50% of patients were hospitalized 4 months after the outpatient visit, and their response to exercise was abnormal (100%). Half of the individuals with normal forced expiratory volume in the first second (FEV1) and abnormal lung clearance index (LCI) experienced the first hospital admission 9 months after the clinic visit, and 84.2% presented an abnormal response to exercise. Conversely, 15.8% had abnormal exercise responses when lung functions were considered normal, with half of the adolescents hospitalized at 11 months.

CONCLUSION: Maintaining ventilation homogeneity, along with a normal ability to sustain intense work, may have a positive impact on the burden of CF disease, here conceived as time free from hospitalization due to PE.

PMID:35813385 | PMC:PMC9257036 | DOI:10.3389/fped.2022.926248

Pediatr Pulmonol. 2022 Jul 10. doi: 10.1002/ppul.26071. Online ahead of print.

ABSTRACT

The negative impact of healthcare disparities, specifically low health literacy, on clinical outcomes related to Cystic Fibrosis (CF) have been described [1]. This article is protected by copyright. All rights reserved.

PMID:35811375 | DOI:10.1002/ppul.26071

Pediatr Pulmonol. 2022 Jul 10. doi: 10.1002/ppul.26069. Online ahead of print.

ABSTRACT

OBJECTIVES: To evaluate feasibility of the Alfred Step Test Exercise Protocol (A-STEP) for the assessment of exercise capacity in adults and children with CF; in adults to test whether demographics and/or lung function correlated with exercise capacity.

METHODS: Adults and children with stable CF from two centres completed the A-STEP (a recently developed incremental maximal-effort step test). Feasibility was evaluated by: usefulness for exercise capacity assessment (measures of exercise capacity were: level reached, exercise-induced desaturation, and achievement of at least one maximal effort criteria); safety; operational factors; time to complete; floor and/or ceiling effects. We used multiple linear regression to test whether demographics and/or lung function correlated with exercise capacity.

RESULTS: 49 participants: 38 adults (18 male), percent predicted (pp) FEV1 29-109, aged 22-48 years and 11 children (6 male), ppFEV1 68-107, aged 10-15 years were included. Levels reached (mean (SD) [range]) were 10.2 (2.4) [6-15] (adults), 10.1 (2.5) [7-14] (children); desaturation (change between baseline and peak-exercise SpO2 ): was 8.4 (3.8 [0-15]% (adults), 2.0 (2.0) [0-7]% (children). Eight (21%) adults and no children desaturated <90%SpO2 . At least one criterion for maximal effort was reached by 33 (84%) adults and 10 (91%) children. There were no adverse events. The A-STEP was straightforward to use and carried out by one operator. Twenty-six (68.4%) adults and 7 (63.6%) children completed the test within the recommended 8-12 minutes. All participants completed a minimum of 6 levels, and completed the test before the final 16th level. In adults, ppFEV1 and ppFVC correlated with the level reached (r=0.55; p=<0.001 and r0.66, p=<0.0001) and desaturation (r=0.55, p=<0.001 and r=0.45, p=<0.005).

CONCLUSION: In adults and children with stable CF, the A-STEP was feasible, safe, and operationally easy to use for the assessment of exercise capacity, without floor or ceiling effects. In adults, lung function correlated with exercise capacity. This article is protected by copyright. All rights reserved.

PMID:35811327 | DOI:10.1002/ppul.26069

Respir Investig. 2022 Jul 7:S2212-5345(22)00080-6. doi: 10.1016/j.resinv.2022.06.002. Online ahead of print.

ABSTRACT

BACKGROUND: Bronchiectasis is a cause of increased morbidity of the respiratory system. Exacerbations among patients with non-CF (cystic fibrosis) bronchiectasis result in reduced pulmonary function and poor quality of life. While the role of bacteria in triggering exacerbations in patients with non- CF bronchiectasis has been well studied, little is known about viral infections in these patients. We aimed to review the evidence on the role of respiratory viruses in the exacerbations of non-CF bronchiectasis.

METHODS: Relevant literature was searched on the MEDLINE/PubMed database. Seven studies satisfied the criteria and were included in this review.

RESULTS: According to the included articles, respiratory viruses are often identified in exacerbations of patients with non-CF bronchiectasis with the most frequent being human rhinovirus and influenza viruses. When a virus is isolated during an exacerbation patients have more symptoms from the upper respiratory tract. One study showed that detection of Epstein- Barr virus among patients with non-CF bronchiectasis is correlated with faster reduction of pulmonary function and progression of the disease.

CONCLUSION: Viruses seem to have a role in the exacerbation of patients with non-CF bronchiectasis. However, the exact nature and importance of this role remain elusive. Viruses are also isolated during the stable period of the disease. Further well-designed studies are necessary to clarify this complex issue.

PMID:35811289 | DOI:10.1016/j.resinv.2022.06.002

Trends Pharmacol Sci. 2022 Jul 7:S0165-6147(22)00133-X. doi: 10.1016/j.tips.2022.06.006. Online ahead of print.

ABSTRACT

The TMEM16A Ca2+-gated Cl- channel is involved in a variety of vital physiological functions and may be targeted pharmacologically for therapeutic benefit in diseases such as hypertension, stroke, and cystic fibrosis (CF). The determination of the TMEM16A structure and high-throughput screening efforts, alongside ex vivo and in vivo animal studies and clinical investigations, are hastening our understanding of the physiology and pharmacology of this channel. Here, we offer a critical analysis of recent developments in TMEM16A pharmacology and reflect on the therapeutic opportunities provided by this target.

PMID:35811176 | DOI:10.1016/j.tips.2022.06.006

Int J Infect Dis. 2022 Jul 7:S1201-9712(22)00401-5. doi: 10.1016/j.ijid.2022.07.004. Online ahead of print.

ABSTRACT

Burkholderia cepacia complex (BCC) are non-fermenting, gram-negative bacteria known to cause high morbidity and mortality. They commonly affect patients with cystic fibrosis and are often missed in those without, despite being fatal if left untreated. We report a case of cepacia syndrome in a 42-year-old, non-cystic fibrosis, immunocompetent man who initially presented with sepsis secondary to pneumonia. Multiple isolates from blood, synovial fluid, and wound swabs grew BCC. Treatment options and management strategies remain poorly understood for BCC in general and in non-cystic fibrosis cases in specific. We were able to successfully treat the patient using a combination of intravenous and inhalational antibiotics. We herein elaborate on the disease presentation, investigations, and management strategy employed to treat this rare infection.

PMID:35811086 | DOI:10.1016/j.ijid.2022.07.004

Int J Pharm. 2022 Jul 6:121989. doi: 10.1016/j.ijpharm.2022.121989. Online ahead of print.

ABSTRACT

This study aims to investigate the effect of physicochemical properties and aerosol performance of two (2FN) and three-fluid nozzles (3FN) on the inhalable co-formulation of tobramycin and diclofenac dry powders. Combination formulations of tobramycin and diclofenac at 2:1 and 4:1 w/w ratios were prepared at a laboratory scale using a spray dryer in conjunction with a 2FN or 3FN. Powder size, morphology, solid-state characteristics, and aerodynamic and dissolution properties were characterised. The nozzle types and the formulation composition influenced the yield, particle size, solid-state properties, aerosolization behaviour and dissolution of the co-spray dried formulations. In particular, using the 2FN the co-spray dried formulation of tobramycin and diclofenac at 2:1 w/w showed smaller particle size (D50, 3.01 ± 0.06 μm), high fine particle fractions (FPF) (61.1 ± 3.6 % for tobramycin and 65.92 ± 3 for diclofenac) and faster dissolution with approx. 70% diclofenac released within 3h and approx. 90% tobramycin was released within 45 min. However, the 3FN for the co-spray dried formulation of tobramycin and diclofenac at a 2:1 w/w ratio showed a larger particle size (D50, 3.42 ± 0.02 μm), lower FPF (40.6 ± 3.4 % for tobramycin and 36.9 ± 0.84 for diclofenac) and comparative slower dissolution with approx. 60% diclofenac was released within 3h and 80% tobramycin was released within 45 min. A similar trend was observed when the tobramycin to diclofenac ratio was increased to 4:1 w/w. Overall results suggest that spray drying with 2FN showed a superior and viable approach to producing excipients-free inhalable co-spray dried formulations of tobramycin and diclofenac. However, the formulation produced using the 3FN showed higher enrichment of hydrophobic diclofenac and an ability to control the tobramycin drug release in vitro.

PMID:35809834 | DOI:10.1016/j.ijpharm.2022.121989

Int Forum Allergy Rhinol. 2022 Jul 9. doi: 10.1002/alr.23062. Online ahead of print.

NO ABSTRACT

PMID:35809000 | DOI:10.1002/alr.23062

Sensors (Basel). 2022 Jun 28;22(13):4884. doi: 10.3390/s22134884.

ABSTRACT

Several studies have shown that patients with cystic fibrosis (CF), even at a young age, have pulmonary and cardiac abnormalities. The main complications are cardiac right ventricular (RV) systolic and/or diastolic dysfunction and pulmonary hypertension, which affects their prognosis. Exercise training (ET) is recommended in patients with CF as a therapeutic modality to improve physical fitness and health-related quality of life. However, questions remain regarding its optimal effective and safe dose and its effects on the patients' cardiac function. The study aimed to provide a wearable activity tracker (WAT)-based ET to promote physical activity in CF patients and assess its effects on cardiac morphology and function. Forty-two stable CF individuals (aged 16.8 ± 3.6 years) were randomly assigned to either the intervention (Group A) or the control group (Group B). Group A participated in a 1-year WAT-based ET program three times per week. All patients underwent a 6-min walking test (6-MWT) and an echocardiographic assessment focused mainly on RV anatomy and function at the baseline and the end of the study. RV systolic function was evaluated by measuring the tricuspid annular plane systolic excursion (TAPSE), the systolic tricuspid annular velocity (TVS'), the RV free-wall longitudinal strain (RVFWSL), and the right ventricular four-chamber longitudinal strain (RV4CSL). RV diastolic function was assessed using early (TVE) and late (TVA) diastolic transtricuspid flow velocity and their ratio TVE/A. Pulmonary artery systolic pressure (PASP) was also estimated. In Group A after ET, the 6MWT distance improved by 20.6% (p &lt; 0.05), TVA decreased by 17% (p &lt; 0.05), and TVE/A increased by 13.2% (p &lt; 0.05). Moreover, TAPSE, TVS', RVFWSL, and RV4CSL increased by 8.3% (p &lt; 0.05), 9.0% (p &lt; 0.05), 13.7% (p &lt; 0.05), and 26.7% (p &lt; 0.05), respectively, while PASP decreased by 7.6% (p &lt; 0.05). At the end of the study, there was a significant linear correlation between the number of steps and the PASP (r = -0.727, p &lt; 0.01) as well as the indices of RV systolic function in Group A. In conclusion, WAT is a valuable tool for implementing an effective ET program in CF. Furthermore, ET has a positive effect on RV systolic and diastolic function.

PMID:35808375 | DOI:10.3390/s22134884

Sensors (Basel). 2022 Jun 22;22(13):4694. doi: 10.3390/s22134694.

ABSTRACT

Pseudomonas aeruginosa is a ubiquitously distributed soil and water bacterium and is considered an opportunistic pathogen in hospitals. In cystic fibrosis patients, for example, infections with P. aeruginosa can be severe and often lead to chronic or even fatal pneumonia. Therefore, rapid detection and further identification are of major importance in hospital hygiene and infection control. This work shows the electrochemical properties of five P. aeruginosa key metabolites considering their potential use as specific signaling agents in an electrochemical sensor system. The pure solutes of pyocyanin (PYO), Pseudomonas quinolone signal (PQS), pyochelin (PCH), 2-heptyl-4-hydroxyquinoline (HHQ), and 2-heptyl-4-hydroxyquinoline N-oxide (HQNO) were analyzed by different electrochemical techniques (cyclic and square wave voltammetry) and measured using a Gamry Reference 600+ potentiostat. Screen-printed electrodes (DropSens DRP110; carbon working and counter, silver reference electrode) were used to determine signal specificities, detection limits, as well as pH dependencies of the substances. All of the compounds were electrochemically inducible with well-separated oxidation and/or reduction peaks at specific peak potentials relative to the reference electrode. Additionally, all analytes exhibited linear concentration dependency in ranges classically reported in the literature. The demonstration of these properties is a promising step toward direct multiplexed detection of P. aeruginosa in environmental and clinical samples and thus, can make a significant contribution to public health and safety.

PMID:35808191 | DOI:10.3390/s22134694

Nutrients. 2022 Jun 21;14(13):2573. doi: 10.3390/nu14132573.

ABSTRACT

Food insecurity (FI) rates among people with cystic fibrosis (CF) are significantly higher than in the general US population. This study explored the experiences and perceptions of adults and parents of children with CF surrounding FI. We recruited parents of children with CF ages 0-18 years and adults with CF ages 18 years and older from a large, accredited U.S. CF care center and the Cystic Fibrosis Foundation Community Voice to participate in a qualitative study using semi-structured telephone interviews to explore experiences and preferences related to food insecurity. Two coders independently reviewed each transcript to apply the codebook and identify any emerging codes using an ongoing, iterative process to identify central themes. We interviewed 20 participants (six adults with CF and 14 parents of children with CF) and identified five major themes: (1) FI in CF is influenced by a variety of factors, ranging from nutritional demands to competing financial barriers; (2) FI impacts CF health outcomes; (3) Open patient-provider communication around FI is vital; (4) FI screening and discussions should be normalized in CF care; (5) Comprehensive FI resources are vital. FI is an important topic that should routinely be addressed with the CF care team to destigmatize and encourage individuals to be more forthcoming about their FI status. Results from this study will inform future larger investigations on the impact of FI on CF health and aid in the design and planning of targeted interventions and advocacy efforts.

PMID:35807754 | DOI:10.3390/nu14132573