Pediatr Radiol. 2022 Nov 14. doi: 10.1007/s00247-022-05539-9. Online ahead of print.

ABSTRACT

Cystic fibrosis (CF) is one of the most common inherited and life-shortening pulmonary diseases in the Caucasian population. With the widespread introduction of newborn screening and the development of modulator therapy, tremendous advances have been made in recent years both in diagnosis and therapy. Since paediatric CF patients tend to be younger and have lower morbidity, the type of imaging modality that should be used to monitor the disease is often debated. Computed tomography (CT) is sensitive to many pulmonary pathologies, but radiation exposure limits its use, especially in children and adolescents. Conventional pulmonary magnetic resonance imaging (MRI) is a valid alternative to CT and, in most cases, provides sufficient information to guide treatment. Given the expected widespread availability of sequences with ultra-short echo times, there will be even fewer reasons to perform CT for follow-up of patients with CF. This review aims to provide an overview of the process and results of monitoring CF with MRI, particularly for centres not specialising in the disease.

PMID:36372855 | DOI:10.1007/s00247-022-05539-9

J Cyst Fibros. 2022 Nov 10:S1569-1993(22)00689-0. doi: 10.1016/j.jcf.2022.09.012. Online ahead of print.

ABSTRACT

BACKGROUND: The aim of this study was to record the current status of newborn bloodspot screening (NBS) for CF across Europe and assess performance.

METHODS: Survey of representatives of NBS for CF programmes across Europe. Performance was assessed through a framework developed in a previous exercise.

RESULTS: In 2022, we identified 22 national and 34 regional programmes in Europe. Barriers to establishing NBS included cost and political inertia. Performance was assessed from 2019 data reported by 21 national and 21 regional programmes. All programmes employed different protocols, with IRT-DNA the most common strategy. Six national and 11 regional programmes did not use DNA analysis.

CONCLUSIONS: Integrating DNA analysis into the NBS protocol improves PPV, but at the expense of increased carrier and CFSPID recognition. Some programmes employ strategies to mitigate these outcomes. Programmes should constantly strive to improve performance but large datasets are needed to assess outcomes reliably.

PMID:36372700 | DOI:10.1016/j.jcf.2022.09.012

Rev Mal Respir. 2022 Nov 10:S0761-8425(22)00346-1. doi: 10.1016/j.rmr.2022.10.005. Online ahead of print.

ABSTRACT

Lung transplantation (LTx) is the last-resort treatment for end-stage respiratory insufficiency, whatever its origin, and represents a steadily expanding field of endeavor. Major developments have been impelled over the years by painstaking efforts at LTx centers to improve donor and recipient selection, and multifaceted attempts have been made to meet the challenges raised by surgical management, perioperative care, and long-term medical complications. The number of procedures has increased, leading to improved post-LTx prognosis. One consequence of these multiple developments has been a pruning away of contraindications over time, which has, in some ways, complicated the patient selection process. With these considerations in mind, the Francophone Pulmonology Society (Société de Pneumology de Langue Française [SPLF]) has set up a task force to produce up-to-date working guidelines designed to assist pulmonologists in managing end-stage respiratory insufficiency, determining which patients may be eligible for LTx, and appropriately timing LTx-center referral. The task force has examined the most recent literature and evaluated the risk factors that continue to limit patient survival after LTx. Ideally, the objectives of LTx are to prolong life while improving quality of life. The guidelines developed by the task force apply to a limited resource and are consistent with the ethical principles described below.

PMID:36372607 | DOI:10.1016/j.rmr.2022.10.005

J Chromatogr B Analyt Technol Biomed Life Sci. 2022 Nov 3;1213:123518. doi: 10.1016/j.jchromb.2022.123518. Online ahead of print.

ABSTRACT

The triple combination modulator therapy (ETI, elexacaftor (ELX), tezacaftor (TEZ), and ivacaftor (IVA)) is a recent advancement for the care of patients with cystic fibrosis. To aid in the development of clinical pharmacokinetics studies of this treatment, we developed a liquid chromatography tandem mass spectrometry (LC-MS/MS) assay for quantifying the component compounds in human plasma and cell lysate. This assay was optimized for small volumes (10 µL), uses stably labeled isotopes of the ETI compounds as internal standards, and employs a simple methanol protein precipitation method. Chromatography was performed on an ACE Excel C18, 2.1 × 50 mm, reversed phase analytical column, using a step or bump isocratic method, with mobile phases consisting of 0.1% formic acid in water for A, and 0.1% formic acid in acetonitrile for B. Analyte and internal standard detection was conducted with ESI positive ionization tandem mass spectrometry. The precursor/product transitions (m/z) monitored were 598.0/422.0 for ELX, 521.0/449.0 for TEZ, 393.0/172.0 for IVA, 601.0/422.0 for IS-ELX, 525.0/453.0 for IS-TEZ, and 399.0/178.0 for IS-IVA, respectively. The assay has a dynamic range of 10 to 10,000 ng/mL, with a mean coefficient of determination (r2, mean ± SD) of 0.9970 ± 0.0027 (ELX), 0.9989 ± 0.0004 (TEZ), 0.9981 ± 0.0003 (IVA), regardless of specimen matrix. The mean precision values for all calibration standards ranged from 0.0 to 10.8% (ELX), 0.0 to 6.7% (TEZ), and 0.2 to 5.6% (IVA), while the accuracy for calibration standards was within the range of -5.7 to 3.5% (ELX), -3.2 to 6.0% (TEZ), and -3.8 to 5.2% (IVA). Validation results demonstrated high accuracy (≤7.3, ≤9.8, ≤10.6% deviation) and high precision (≤11.5, ≤6.3, ≤11.0% CV) for the respective ETI quality control samples. This method provides a fully validated assay for ETI quantitation for use in clinical research.

PMID:36371965 | DOI:10.1016/j.jchromb.2022.123518

Respir Med Res. 2022 Nov 2;82:100972. doi: 10.1016/j.resmer.2022.100972. Online ahead of print.

NO ABSTRACT

PMID:36371896 | DOI:10.1016/j.resmer.2022.100972

J Cyst Fibros. 2022 Nov 9:S1569-1993(22)01390-X. doi: 10.1016/j.jcf.2022.10.008. Online ahead of print.

ABSTRACT

BACKGROUND: The availability of new diagnostic algorithms for cystic fibrosis (CF), changing population demographics and programs that impact family planning decisions can influence incidence rates. Thus, previously reported incidence rates in Canada and the United States (US) may be outdated. The objectives of this study were to estimate contemporary CF incidence rates in Canada and the US and to determine if the incidence rate has changed over time.

METHOD: This population-based cohort study utilized data between 1995-2019 from the Canadian CF Registry (CCFR), Statistics Canada, US CF Foundation Patient Registry (CFFPR) data, and US Center for Disease Control (CDC) National Vital Statistics System. Incidence was estimated using the number of live CF births by year, sex, and geographic region using Poisson regression, with the number of live births used as the denominator. To account for delayed diagnoses, we imputed the proportion of diagnoses expected given historical trends, and varying rates of newborn screening (NBS) implementation by region.

RESULTS: After accounting for implementation of NBS and delayed diagnoses, the estimated incidence rate for CF in 2019 was 1:3848 (95% CI: 1:3574, 1:4143) live births in Canada compared to 1:5130 (95% CI:1:4996, 1:5267) in the US. There was substantial regional variation in incidence rates within both Canada and the US. Since 1995, incidence rates have decreased at a rate of 1.6% per year in both countries (p<0.001).

CONCLUSION: Contemporary CF incidence rates suggest CF incidence is lower than previously reported and varies widely within North America. This information is important for resource planning and for tracking how programs (e.g., genetic counselling, modulator availability etc.) may impact the incidence of CF moving forward.

PMID:36371312 | DOI:10.1016/j.jcf.2022.10.008

Clin Microbiol Infect. 2022 Nov 9:S1198-743X(22)00567-5. doi: 10.1016/j.cmi.2022.11.001. Online ahead of print.

ABSTRACT

OBJECTIVES: In cystic fibrosis (CF) there is a predisposition to bronchial colonization by potentially pathogenic microorganisms such as fungi. Our aims were to describe the dynamics of respiratory mycobiota in patients with CF and to evaluate the geographic, age and gender variability in its distribution.

METHODS: Cohort study in which 45 patients with CF from 4 hospitals of 3 Spanish cities were followed during a 1-year period, obtaining spontaneous sputum samples every 3-6 months. Fungal microbiota was characterized by ITS sequencing and Pneumocystis jirovecii was identified by nested-PCR in a total of 180 samples.

RESULTS: The presence of fungi was detected in 119 (66.11%) of the 180 samples and in 44 (97.8%) of the 45 patients: 19 were positive and 1 negative throughout all the follow-up and the remaining 25 presented alternation between positive and negative results. A total of 16 different genera were identified, with Candida spp. (50/180, 27.78%) and Pneumocystis spp. (44/180, 24.44%) being the most prevalent ones. The distribution of fungal genera was different among the evaluated centres (p<0.05), by age (non-adults 6-17 years old versus adults 18 years old and older) (p<0.05) and by gender (p<0.05).

CONCLUSIONS: There is a high prevalence of fungal respiratory microbiota in patients with CF, whose dynamics is characterized by the existence of multiple cycles of clearance and colonization, reporting the existence of geographic, age and gender variability in the distribution of fungal genera in this disease.

PMID:36371030 | DOI:10.1016/j.cmi.2022.11.001

J Sep Sci. 2022 Nov 12. doi: 10.1002/jssc.202200666. Online ahead of print.

ABSTRACT

A high-performance liquid chromatography protocol for the analysis of brevetoxins has been developed using a silica hydride-based cholesterol column. Brevetoxins are neurotoxins produced by harmful algae that have additional potential as drugs for a number of illnesses/diseases. To develop the optimum conditions, a number of different experimental approaches were tested. These include isocratic and gradient elution, different organic mobile phase components, and temperature variations. A separate protocol was developed for the compounds brevenal and brevenol, also produced by the same algae that make brevetoxins. Brevenal is a natural product under investigation as a therapy for chronic respiratory diseases, such as cystic fibrosis or asthma. The goal of this study was to provide a protocol for the analysis of these compounds that could be further developed into a validated method depending on a particular laboratory's capabilities and to highlight some of the unique features of the cholesterol stationary phase. This article is protected by copyright. All rights reserved.

PMID:36369995 | DOI:10.1002/jssc.202200666

J Cell Mol Med. 2022 Nov 11. doi: 10.1111/jcmm.17605. Online ahead of print.

ABSTRACT

This short report documented cystic fibrosis transmembrane conductance regulator (CFTR) variants in 37 patients with cystic fibrosis (CF) in the Rio Grande do Norte region of Northeast Brazil. The high-throughput sequencing technology (HTS) genetic testing provided a definitive molecular diagnosis in 31 patients (83.8%). Among them, 25 patients' carriers of the c.1521_1523delCTT variant, categorized as a class 2 mutation, can be currently treated with CFTR modulator drugs. Five children aged 2-5 years could benefit from double lumacaftor/ivacaftor therapy, and 20 patients aged >6 years could be treated with the triple-combination elexacaftor/tezacaftor/ivacaftor therapy. Thus, the identification of pathogenic variants associated with the development of this disease allows for the introduction of therapy with CFTR modulators that favour better patient management.

PMID:36369753 | DOI:10.1111/jcmm.17605

BMC Genomics. 2022 Nov 11;23(1):748. doi: 10.1186/s12864-022-08985-9.

ABSTRACT

BACKGROUND: Shotgun metagenome analysis provides a robust and verifiable method for comprehensive microbiome analysis of fungal, viral, archaeal and bacterial taxonomy, particularly with regard to visualization of read mapping location, normalization options, growth dynamics and functional gene repertoires. Current read classification tools use non-standard output formats, or do not fully show information on mapping location. As reference datasets are not perfect, portrayal of mapping information is critical for judging results effectively.

RESULTS: Our alignment-based pipeline, Wochenende, incorporates flexible quality control, trimming, mapping, various filters and normalization. Results are completely transparent and filters can be adjusted by the user. We observe stringent filtering of mismatches and use of mapping quality sharply reduces the number of false positives. Further modules allow genomic visualization and the calculation of growth rates, as well as integration and subsequent plotting of pipeline results as heatmaps or heat trees. Our novel normalization approach additionally allows calculation of absolute abundance profiles by comparison with reads assigned to the human host genome.

CONCLUSION: Wochenende has the ability to find and filter alignments to all kingdoms of life using both short and long reads, and requires only good quality reference genomes. Wochenende automatically combines multiple available modules ranging from quality control and normalization to taxonomic visualization. Wochenende is available at https://github.com/MHH-RCUG/nf_wochenende .

PMID:36368923 | DOI:10.1186/s12864-022-08985-9

Camb Q Healthc Ethics. 2022 Nov 11:1-4. doi: 10.1017/S0963180122000433. Online ahead of print.

ABSTRACT

Mallory Smith's posthumously published book, Salt in My Soul: An Unfinished Life, is an insightful and moving account of one young woman's experience living with a chronic, often invisible, illness.1 Mallory was diagnosed with cystic fibrosis (CF) at age three and began writing in a journal when she was 15. According to those close to her, Mallory wrote consistently over the span of 10 years, until shortly before she died at age 25 from complications related to a double lung transplant. Much of what Mallory included in her journal was too difficult for her to share with family and friends while she was alive, yet she hoped that her writing would one day "offer insight for people living with, or loving someone with, chronic illness." (page x) As Mallory explained, "I want to create a piece so moving that people are in disbelief. And I want it to be like handing people a pair of glasses, giving them a way of seeing something they didn't even realize they weren't seeing." (page 291) Shortly before Mallory died, she shared the password to her 2,500-page journal with her mother, along with instructions for how to select excerpts for what would become Salt in My Soul.

PMID:36366957 | DOI:10.1017/S0963180122000433

Nutr Health. 2022 Nov 10:2601060221136653. doi: 10.1177/02601060221136653. Online ahead of print.

ABSTRACT

Background: Probiotics are used by people with cystic fibrosis (CF) and other chronic diseases to manage gastrointestinal symptoms. Aim: To describe probiotic knowledge; its relationship with probiotic use, probiotic information sources and factors influencing choice in adults with CF and a general population control group. Methods: A cross-sectional questionnaire study was conducted in adults with CF (n = 205) and Controls (n = 158). Probiotic knowledge was compared between CF and Controls using a knowledge score (maximum 5) based on predefined criteria: (1a) bacteria/microorganism; (1b) live; (2a) administered; (2b) adequate dose and (3) health benefit, using independent samples t-test. Two-way analysis of variance explored knowledge scores between CF and Control and between Ever User and Never User groups. Chi-square and Fisher's exact tests compared knowledge criterion, probiotic sources and influences on probiotic choice between groups. Thematic analysis of open-text responses explored probiotic-related knowledge and influences on probiotic decision making. Results: Knowledge scores (mean ± SD) did not differ between CF (1.70 ± 1.12) and Controls (1.89 ± 0.99), p = 0.13. Probiotic use was associated with knowledge score (p < 0.001). More CF Ever Users than Never Users correctly identified criteria 1a (65% vs. 38%), 1b (16% vs. 0%), 2a (45% vs. 22%) and 3 (73% vs. 42%) (all p < 0.005). CF participants considered 'dairy yoghurt' (69%), 'live cultures' (64%) and 'fermented foods' (37%) as 'all/mostly' probiotic sources. The internet was the commonest source of probiotic-related information. Conclusion: Probiotic knowledge and use were associated in adults with CF. Understanding of probiotic characteristics and sources were limited. Education is needed to help guide patient probiotic decision making.

PMID:36366805 | DOI:10.1177/02601060221136653

Vaccines (Basel). 2022 Nov 10;10(11):1899. doi: 10.3390/vaccines10111899.

ABSTRACT

BACKGROUND: Coronavirus disease 2019 (COVID-19) vaccination hesitancy is a threat as COVID-19 vaccines have reduced both viral transmission and virus-associated mortality rates, particularly in high-risk subgroups. Solid organ transplant recipients (SOTRs) are particularly vulnerable, as the underlying causes of their organ failure and the chronic immunosuppression are associated with a lower immune response to COVID-19 vaccines, and with an excessive risk of death due to SARS-CoV-2 infection. We aimed to evaluate COVID-19 vaccination hesitancy and its reasons in a population of SOTRs.

METHODS: All the SOTRs attending our post-transplant clinics were asked to fill in a vaccination status form with specific validated questions related to their willingness to receive a third vaccine dose. In the case of negative answers, the patients were encouraged to explain the reasons for their refusal. Among the SOTRs (1899), 1019 were investigated (53.7%).

RESULTS: Overall, 5.01% (51/1019) of the SOTRs raised concerns regarding the future third dose vaccination. In more detail, hesitancy rates were 3.3% (15/453), 4.2% (7/166), and 7.3% (29/400) among the investigated liver, lung, and kidney transplant recipients, respectively (p = 0.0018). The main reasons for hesitancy were fear of adverse events (30/51, 58.8%) and perceived lack of efficacy (21/51, 41.2%).

CONCLUSIONS: Full adherence to ongoing or future vaccination campaigns is crucial to prevent, or at least reduce, COVID-19-related morbidity and mortality in fragile patients. The identification of the reasons influencing COVID-19 vaccination hesitancy in these patients is very important to establish appropriate and targeted patient-doctor communication strategies, and to further implement specific vaccination campaigns.

PMID:36366406 | DOI:10.3390/vaccines10111899

Pharmaceutics. 2022 Oct 26;14(11):2297. doi: 10.3390/pharmaceutics14112297.

ABSTRACT

In recent years, we have discovered Esc(1-21) and its diastereomer (Esc peptides) as valuable candidates for the treatment of Pseudomonas lung infection, especially in patients with cystic fibrosis (CF). Furthermore, engineered poly(lactide-co-glycolide) (PLGA) nanoparticles (NPs) were revealed to be a promising pulmonary delivery system of antimicrobial peptides. However, the "ad hoc" development of novel therapeutics requires consideration of their stability, tolerability, and safety. Hence, by means of electrophysiology experiments and preclinical studies on healthy mice, we demonstrated that neither Esc peptides or Esc-peptide-loaded PLGA NPs significantly affect the integrity of the lung epithelium, nor change the global gene expression profile of lungs of treated animals compared to those of vehicle-treated animals. Noteworthy, the Esc diastereomer endowed with the highest antimicrobial activity did not provoke any pulmonary pro-inflammatory response, even at a concentration 15-fold higher than the efficacy dosage 24 h after administration in the free or encapsulated form. The therapeutic index was ≥70, and the peptide was found to remain available in the bronchoalveolar lavage of mice, after two days of incubation. Overall, these studies should open an avenue for a new up-and-coming pharmacological approach, likely based on inhalable peptide-loaded NPs, to address CF lung disease.

PMID:36365116 | DOI:10.3390/pharmaceutics14112297

Pathogens. 2022 Oct 31;11(11):1272. doi: 10.3390/pathogens11111272.

ABSTRACT

BACKGROUND: Acute kidney injury (AKI) is a common complication among SARS-CoV-2-positive patients who undergo hospitalization. Abundant evidence exists concerning the epidemiology of AKI in patients hospitalized in the ICU for COVID-19 but limited data are available about the occurrence of AKI in SARS-CoV-2-positive patients being hospitalized in a non-ICU setting.

AIM AND METHODS: We have carried out a retrospective study to evaluate frequency and risk factors for AKI among patients consecutively admitted at a third-level university hospital starting from February 2020 (the beginning of the first wave of the SARS-CoV-2 pandemic); all patients were hospitalized outside the ICU.

RESULTS: A total of 387 SARS-CoV-2-positive patients were included in the current study; 372 (96.1%) had SARS-CoV-2-related pneumonia. In-hospital AKI onset was recorded in 119 (30.7%) patients, mainly with AKI stage 1 (n = 74, 62.2%); eighteen (4.6%) patients reported AKI stage 3 and six (1.5%) patients had HD-dependent AKI. There were 235 (60.7%) patients with severe COVID-19, and this was more common in patients developing AKI, 94.5% (86/119) vs. 86.1% (149/268), p = 0.02. Multivariate regression model (n = 144 patients) reported an independent and significant relationship between AKI occurrence and greater levels of ferritin (p = 0.036), IL-6 (p = 0.032), and azotemia at admission (p = 0.0001). A total of 69 (17.8%) SARS-CoV-2-positive patients died and strong predictors of in-hospital death resulted from age (p &lt; 0.0001), serum ferritin (p &lt; 0.0001) and white blood cells (p &lt; 0.001). According to multivariable analysis (n = 163 patients), there was a consistent link between in-hospital death and AKI stage (1) (p = 0.021) and -stage (2) (p = 0.009). Our results support the notion that AKI occurs frequently among hospitalized COVID-19 patients even in a non-ICU setting and plays a pivotal role in the mortality of this population. Further studies are ongoing in order to clearly establish the frequency of AKI in patients with COVID-19; the mechanisms underlying kidney injury in this population are an area of active investigation. These data provide solid evidence to support close monitoring of COVID-19 patients for the development of AKI and measures taken to prevent this.

PMID:36365023 | DOI:10.3390/pathogens11111272

Nutrients. 2022 Nov 4;14(21):4666. doi: 10.3390/nu14214666.

ABSTRACT

Adequate intake of nutrients such as essential fatty acids (EFA) are critical in cystic fibrosis (CF). The clinical course of deterioration of lung function in people with CF has been shown to relate to nutrition. Independent of the higher energy consumption and malabsorption due to pancreatic insufficiency, EFA deficiency is closely associated with the risk of pulmonary infection, the most significant pathology in CF. This review will focus on the EFA deficiency identified in people with CF, as well as the limited progress made in deciphering the exact metabolic pathways that are dysfunctional in CF. Specifically, people with CF are deficient in linoleic acid, an omega 6 fatty acid, and the ratio of arachidonic acid (omega 6 metabolite) and docosahexaenoic acid (omega 3 metabolite) is increased. Analysis of the molecular pathways in bronchial cells has identified changes in the enzymes that metabolise EFA. However, fatty acid metabolism primarily occurs in the liver, with EFA metabolism in CF liver not yet investigated, indicating that further research is required. Despite limited understanding in this area, it is well known that adequate EFA concentrations are critical to normal membrane structure and function, and thus are important to consider in disease processes. Novel insights into the relationship between CF genotype and EFA phenotype will be discussed, in addition to sex differences in EFA concentrations in people with CF. Collectively, investigating the specific effects of genotype and sex on fatty acid metabolism may provide support for the management of people with CF via personalised genotype- and sex-specific nutritional therapies.

PMID:36364928 | DOI:10.3390/nu14214666

Nutrients. 2022 Nov 4;14(21):4661. doi: 10.3390/nu14214661.

ABSTRACT

BACKGROUND: The issue of vitamin metabolism in children with cystic fibrosis screen positive, inconclusive diagnosis (CFSPID) is not well known. The aim of this study was to determine the status of vitamins A, D, E, and C in the blood of a group of children with CFSPID.

MATERIAL AND METHODS: A total of 89 children were enrolled in the study (Me: 3.6 years, 52.8% boys), as follows: 28 with CFSPID, 31 with CF (cystic fibrosis), and 30 HC (healthy children). Their blood concentrations of vitamins A, D, E, and C, and their dietary intake of these vitamins were analysed in the study groups on the basis of a three-day food diary.

RESULTS: The patients with CFSPID had significantly higher serum vitamin D (p = 0.01) and E (p = 0.04) concentrations, compared to the children with CF. None of the children with CFSPID revealed vitamin A or E deficiencies. Patients with CF had been consuming significantly higher vitamin D and E amounts (p = 0.01). The vitamin concentrations did not depend either on the pancreatic/liver function or on anthropometric parameters. In total, 32.14% of patients with CF did not cover the baseline recommended calorie intake, and 53.6% and 36% did not take the recommended vitamin E and vitamin A intake, respectively.

CONCLUSION: Children with CF and CFSPID did not fully cover the dietary recommendations for vitamin supply, but vitamin deficiency was found only in CF.

PMID:36364923 | DOI:10.3390/nu14214661

Nutrients. 2022 Oct 27;14(21):4517. doi: 10.3390/nu14214517.

ABSTRACT

Eco-concern, the distress experienced relating to climate change, is associated with mental health, yet no study has examined disordered eating related to eco-concern. This study developed and validated a 10-item scale assessing Eating-Related Eco-Concern (EREC). Participants (n = 224) completed the EREC, Climate Change Worry Scale (CCWS), and Eating Disorder Examination-Questionnaire (EDE-Q). Construct validity, convergent validity, and internal consistency were evaluated. Sex differences in EREC were evaluated using t-tests. Associations among the EREC, CCWS, and EDE-Q were evaluated using linear regression models. Sensitivity analyses were conducted in individuals below EDE-Q global score clinical cut-offs. Factor analysis suggested that all items loaded adequately onto one factor. Pearson's correlation and Bland-Altman analyses suggested strong correlation and acceptable agreement between the EREC and CCWS (r = 0.57), but weak correlation and low agreement with the EDE-Q global score (r = 0.14). The EREC had acceptable internal consistency (α = 0.88). No sex difference was observed in the EREC in the full sample; females had a significantly higher mean score than males in sensitivity analysis. The EREC was significantly positively associated with the CCWS and EDE-Q global and shape concern scores, but not in sensitivity analysis. The EREC is a brief, validated scale that can be useful to screen for eating-related eco-concern.

PMID:36364778 | DOI:10.3390/nu14214517

J Clin Med. 2022 Oct 30;11(21):6432. doi: 10.3390/jcm11216432.

ABSTRACT

(1) Background: We describe our experience with cystic fibrosis (CF) patients treated with bronchial artery embolization (BAE) for sub-massive hemoptysis to understand if early treatment of sub-massive hemoptysis can reduce the volume of any subsequent bleedings. (2) Materials: We performed a retrospective study including CF patients who underwent angiographic procedures for BAE following sub-massive hemoptysis, from March 2016 to December 2021. All patients underwent an initial chest angio-CT study. BAE was realized with microspheres or coils. (3) Results: Thirteen patients were included, subjected to at least one BAE after sub-massive hemoptysis, for a total of 19 procedures. Technical success was 94.7%; in a single case, the catheterization of the bronchial arterial feeder was not achievable and the procedure was repeated. Primary clinical success was 92.3%; secondary clinical success was 69.2%. Relative clinical success was 85%. A higher incidence of recurrent hemoptysis following treatment with coils was observed (100% of cases) compared to treatment with microspheres (54.5% of cases) χ2 = 5.43 (p &lt; 0.05). (4) Conclusions: BAE is a safe and effective method for the treatment of hemoptysis in CF patients; it should be practiced not only after massive or recurrent hemoptysis but also in patients with sub-massive bleeding to improve their life expectancy and quality of life.

PMID:36362659 | DOI:10.3390/jcm11216432

J Clin Med. 2022 Oct 26;11(21):6306. doi: 10.3390/jcm11216306.

ABSTRACT

Over recent decades, significant advances have been achieved in ameliorating clinical outcomes for patients with cystic fibrosis (CF) [...].

PMID:36362532 | DOI:10.3390/jcm11216306