J Infect. 2023 Feb 21:S0163-4453(23)00114-7. doi: 10.1016/j.jinf.2023.02.027. Online ahead of print.

NO ABSTRACT

PMID:36822406 | DOI:10.1016/j.jinf.2023.02.027

Arch Gerontol Geriatr. 2023 Feb 17;109:104964. doi: 10.1016/j.archger.2023.104964. Online ahead of print.

ABSTRACT

INTRODUCTION: Sarcopenia prevalence varies according to differences in diagnostic criteria used. In order to overcome this issue, the European Working Group on Sarcopenia in Older People-2 (EWGSOP2) published a consensus to increase harmonization for sarcopenia diagnosis. This study aimed to determine the prevalence and risk factors across the different sarcopenia diagnosis criteria recommended by EWGSOP2 and to analyze its agreement.

METHODS: A total of 699 community-dwelling older adults (median-age: 72, 60% female) were recruited in this cross-sectional study. Sarcopenia prevalence was obtained by different combinations of muscle strength (handgrip strength or 5-times sit-to-stand) and muscle quantity (appendicular skeletal mass or skeletal muscle index) as recommended by the EWGSOP2. Cohen's Kappa coefficient was calculated to analyze agreement among the four sarcopenia diagnostic criteria and logistic regressions were performed to identify risks associated to health-related outcomes for each diagnostic criterion.

RESULTS: Sarcopenia prevalence varied from 2.1% to 11.6%, depending on the diagnostic criteria used. Weak-to-moderate agreements (κ-range: 0.13-0.66) were observed among the four sarcopenia diagnosis criteria. There was scarce overlap in sarcopenic people when different diagnostic criteria were used leading to up to 10.4% of underdiagnosis. Sarcopenia defined by 5-times sit-to-stand was more associated with health-related outcomes compared to handgrip strength.

CONCLUSIONS: Sarcopenia prevalence rates vary considerably depending on the diagnostic criteria used. These criteria should not be used in an interchangeable way due to their weak agreement. Sarcopenia diagnosis criteria defined by 5-times sit-to-stand could be more suitable in Spanish community-dwelling older adults due their associations with health-related outcomes.

PMID:36821872 | DOI:10.1016/j.archger.2023.104964

J Bras Pneumol. 2023 Feb 20;49(2):e20220312. doi: 10.36416/1806-3756/e20220312. eCollection 2023.

ABSTRACT

OBJECTIVE: To evaluate the effectiveness of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) and to characterize its safety profile in cystic fibrosis (CF) patients in a real-world clinical setting.

METHODS: This was a prospective observational study carried out in a CF referral center in Portugal involving adult CF patients who started treatment with ELX/TEZ/IVA. Clinical characteristics of the patients were collected, and effectiveness and safety data were evaluated.

RESULTS: Of the 56 patients followed in the center at the time of the study, 28 were eligible for ELX/TEZ/IVA treatment in accordance with the Portuguese National Authority for Medicines and Health Products at the time of the study. Of these, 24 met the follow-up time requirement to be included in the clinical effectiveness analysis. The mean follow-up time was 167.3 ± 96.4 days. Adverse events were generally mild and self-limited. Significant improvements in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations were observed. No significant differences in outcomes between F508del homozygous and heterozygous patients were found. The effectiveness of this new CFTR modulator combination also applied to patients with advanced lung disease.

CONCLUSIONS: Treatment with ELX/TEZ/IVA showed effective improvement in real-world clinical practice, namely in lung function, BMI, sweat chloride concentration, and number of pulmonary exacerbations, with no safety concerns.

PMID:36820745 | DOI:10.36416/1806-3756/e20220312

ERJ Open Res. 2023 Feb 20;9(1):00274-2022. doi: 10.1183/23120541.00274-2022. eCollection 2023 Jan.

ABSTRACT

BACKGROUND: Persistence of respiratory symptoms, particularly breathlessness, after acute coronavirus disease 2019 (COVID-19) infection has emerged as a significant clinical problem. We aimed to characterise and identify risk factors for patients with persistent breathlessness following COVID-19 hospitalisation.

METHODS: PHOSP-COVID is a multicentre prospective cohort study of UK adults hospitalised for COVID-19. Clinical data were collected during hospitalisation and at a follow-up visit. Breathlessness was measured by a numeric rating scale of 0-10. We defined post-COVID-19 breathlessness as an increase in score of ≥1 compared to the pre-COVID-19 level. Multivariable logistic regression was used to identify risk factors and to develop a prediction model for post-COVID-19 breathlessness.

RESULTS: We included 1226 participants (37% female, median age 59 years, 22% mechanically ventilated). At a median 5 months after discharge, 50% reported post-COVID-19 breathlessness. Risk factors for post-COVID-19 breathlessness were socioeconomic deprivation (adjusted OR 1.67, 95% CI 1.14-2.44), pre-existing depression/anxiety (adjusted OR 1.58, 95% CI 1.06-2.35), female sex (adjusted OR 1.56, 95% CI 1.21-2.00) and admission duration (adjusted OR 1.01, 95% CI 1.00-1.02). Black ethnicity (adjusted OR 0.56, 95% CI 0.35-0.89) and older age groups (adjusted OR 0.31, 95% CI 0.14-0.66) were less likely to report post-COVID-19 breathlessness. Post-COVID-19 breathlessness was associated with worse performance on the shuttle walk test and forced vital capacity, but not with obstructive airflow limitation. The prediction model had fair discrimination (concordance statistic 0.66, 95% CI 0.63-0.69) and good calibration (calibration slope 1.00, 95% CI 0.80-1.21).

CONCLUSIONS: Post-COVID-19 breathlessness was commonly reported in this national cohort of patients hospitalised for COVID-19 and is likely to be a multifactorial problem with physical and emotional components.

PMID:36820079 | PMC:PMC9790090 | DOI:10.1183/23120541.00274-2022

Respir Med Case Rep. 2023 Jan 27;42:101816. doi: 10.1016/j.rmcr.2023.101816. eCollection 2023.

ABSTRACT

INTRODUCTION: Cystic fibrosis is known to cause serious complications, such as recurrent pulmonary infections, pancreatic insufficiency, and other symptoms related to exocrine gland dysfunction. A rare manifestation of the disease is discussed in this case of a 24-year-old female diagnosed with cystic fibrosis, a purpuric rash was documented during pulmonary infection flares. Skin biopsy shows a leukocytoclastic vasculitis eruption along with infection. Treatment options are limited and not well established. Our patient received a treatment based on colchicine 1mg per day with a total response. The patient was observed during two consecutive pulmonary infection flares separated by a few months, and a total remission without recurrence was found.

CONCLUSION: Considering its efficacy and safety, further scientific research about colchicine and vasculitis in cystic fibrosis should be aimed at in order to define a strong consensus between the disease and this treatment option.

PMID:36819890 | PMC:PMC9932180 | DOI:10.1016/j.rmcr.2023.101816

Eur J Case Rep Intern Med. 2023 Jan 17;10(1):003728. doi: 10.12890/2023_003728. eCollection 2023.

ABSTRACT

Cystic fibrosis (CF) is a common autosomal recessive disorder which is mainly found in Caucasians but has also been reported in Asian populations. CF is primarily caused by mutations in the CFTR gene which regulates the transport of chloride ions across the cell membrane. We describe the cases of two siblings with CF diagnosed with the rare missense mutation c.80G>T, which has only been referenced once in the literature and shows a possible association with classical form of CF.

LEARNING POINTS: c.80G>T is a very rare CFTR missense mutation which has not been known to be a disease-causing alteration.The mutation causes an amino acid switch from glycine to valine at position 27 in exon 2, resulting in the production of defective CFTR protein.In the homozygous state, c.80G>T seems to be associated with the classic CF phenotype.

PMID:36819652 | PMC:PMC9930882 | DOI:10.12890/2023_003728

Ann Transl Med. 2023 Jan 31;11(2):29. doi: 10.21037/atm-22-5733. Epub 2022 Dec 7.

NO ABSTRACT

PMID:36819573 | PMC:PMC9929740 | DOI:10.21037/atm-22-5733

Acta Clin Croat. 2022 Aug;61(2):342-348. doi: 10.20471/acc.2022.61.02.21.

ABSTRACT

This study analyzed the characteristics of chronic rhinosinusitis patients with and without nasal polyps failing conservative treatment and undergoing functional endoscopic sinus surgery as part of their treatment for chronic inflammatory sinonasal disease. The aim of this retrospective single-institution cohort study conducted at a university hospital tertiary referral center was to evaluate the characteristics of patients with both disease phenotypes in whom conservative treatment was unsuccessful. Patients who were surgically treated with functional endoscopic sinus surgery performed by two rhinology surgeons during a one-year period (2016) were enrolled in the study. Patient data collection included demographics, risk factor exposure, diagnosis, and type of endoscopic surgical procedure performed. In total, 185 patients were included in the study. Patients with malignant disease and those with incomplete data were excluded from the study. In the group of patients with nasal polyps, mean age, male gender, presence of allergy (34.9%), asthma (21.4%), aspirin sensitivity (6.3%), cystic fibrosis (1.6%), and previous nasal surgery rates (36.5%) were significantly higher as compared with the group of patients with chronic rhinosinusitis without nasal polyps. Septal deviation was more prevalent in the chronic rhinosinusitis group (55%) as compared to the group with nasal polyps (25%). The ethmoid (17%) and maxillary sinus (13%) were most frequently involved, but most of the patients who needed surgery had involvement of multiple or all sinuses (40%). The reasons for conservative treatment failure in chronic rhinosinusitis are multifactorial, but identifying the most prevalent characteristics in patients treated surgically may be helpful in identifying patients who would benefit most from surgery.

PMID:36818937 | PMC:PMC9934050 | DOI:10.20471/acc.2022.61.02.21

iScience. 2023 Jan 23;26(2):105988. doi: 10.1016/j.isci.2023.105988. eCollection 2023 Feb 17.

ABSTRACT

Synthetic anion transporters show potential in treating life-threatening diseases like cystic fibrosis and cancer. However, with increasingly complex transporter architectures designed to control anion binding and transport, it is important to consider solubility and deliverability during transporter design. The fluorination of synthetic anion transporters has been shown to tune the transporter lipophilicity, transport rates, and binding strength. In this work, we expand on our previously reported tetrapodal (thio)urea transporters with a series of fluorinated tetrapodal anion transporters. The effects of fluorination on tuning the lipophilicity, solubility, deliverability, and anion transport selectivity of the tetrapodal scaffold were investigated using anion-binding and transport assays. The primary mode of anion transport was H+/X- cotransport, with the most fluorinated tetrathiourea (8) displaying the highest transport activity in the 8-hydroxypyrene-1,3,6-trisulfonic acid (HPTS) assay. Intriguingly, inversion of the transmembrane Cl- vs NO3 - transport selectivity compared with previously reported tripodal (thio)urea transporters was observed under a modified HPTS assay.

PMID:36818308 | PMC:PMC9932467 | DOI:10.1016/j.isci.2023.105988

Front Pediatr. 2023 Feb 2;11:1068103. doi: 10.3389/fped.2023.1068103. eCollection 2023.

ABSTRACT

OBJECTIVES: Quantitative computed tomography (QCT) offers some promising markers to quantify cystic fibrosis (CF)-lung disease. Air trapping may precede irreversible bronchiectasis; therefore, the temporal interdependencies of functional and structural lung disease need to be further investigated. We aim to quantify airway dimensions and air trapping on chest CT of school-age children with mild CF-lung disease over two years.

METHODS: Fully-automatic software analyzed 144 serial spirometer-controlled chest CT scans of 36 children (median 12.1 (10.2-13.8) years) with mild CF-lung disease (median ppFEV1 98.5 (90.8-103.3) %) at baseline, 3, 12 and 24 months. The airway wall percentage (WP5-10), bronchiectasis index (BEI), as well as severe air trapping (A3) were calculated for the total lung and separately for all lobes. Mixed linear models were calculated, considering the lobar distribution of WP5-10, BEI and A3 cross-sectionally and longitudinally.

RESULTS: WP5-10 remained stable (P = 0.248), and BEI changed from 0.41 (0.28-0.7) to 0.54 (0.36-0.88) (P = 0.156) and A3 from 2.26% to 4.35% (P = 0.086) showing variability over two years. ppFEV1 was also stable (P = 0.276). A robust mixed linear model showed a cross-sectional, regional association between WP5-10 and A3 at each timepoint (P < 0.001). Further, BEI showed no cross-sectional, but another mixed model showed short-term longitudinal interdependencies with air trapping (P = 0.003).

CONCLUSIONS: Robust linear/beta mixed models can still reveal interdependencies in medical data with high variability that remain hidden with simpler statistical methods. We could demonstrate cross-sectional, regional interdependencies between wall thickening and air trapping. Further, we show short-term regional interdependencies between air trapping and an increase in bronchiectasis. The data indicate that regional air trapping may precede the development of bronchiectasis. Quantitative CT may capture subtle disease progression and identify regional and temporal interdependencies of distinct manifestations of CF-lung disease.

PMID:36816383 | PMC:PMC9932328 | DOI:10.3389/fped.2023.1068103

Front Pediatr. 2023 Feb 3;11:1095452. doi: 10.3389/fped.2023.1095452. eCollection 2023.

ABSTRACT

BACKGROUND: This study aims to analyze the research hotspots, evolution, and developing trends in pediatric bronchiectasis over the past 20 years using bibliometric analysis and visualization tools to identify potential new research directions.

METHODS: Publications related to bronchiectasis in children were retrieved from the Web of Science Core Collection (WoSCC) database from 2003 to 2022. Knowledge maps were performed through VOSviewer1.6.18 and CiteSpace6.1 R2.

RESULTS: A total of 2,133 publications were searched, while only 1,351 original articles written in English between 2003 and 2022 were incorporated. After removing duplicates, we finally included 1,350 articles published by 6,593 authors from 1,865 institutions in 80 countries/regions in 384 different academic journals with an average citation frequency of 24.91 times. The number of publications shows an extremely obvious binomial growth trend. The majority of publications originated from the United States, Australia, and England. The institutes in Australia, especially Charles Darwin University, published the most articles associated with pediatric bronchiectasis. In addition, Pediatric Pulmonology was the most published journal. In terms of authors, Chang AB was the most productive author, while Gangell CL had the highest average citation frequency. The five keywords that have appeared most frequently during the last two decades were "children," "cystic fibrosis," "bronchiectasis," "ct," and "pulmonary-function." According to keyword analysis, early diagnosis and intervention and optimal long-term pediatric-specific management were the most concerned topics for researchers.

CONCLUSION: This bibliometric analysis indicates that bronchiectasis in children has drawn increasing attention in the last two decades as its recognition continues to rise, providing scholars in the field with significant information on current topical issues and research frontiers.

PMID:36816374 | PMC:PMC9936077 | DOI:10.3389/fped.2023.1095452

Heliyon. 2022 Dec 30;9(2):e12601. doi: 10.1016/j.heliyon.2022.e12601. eCollection 2023 Feb.

ABSTRACT

Pseudomonas aeruginosa is an ubiquitous and opportunistic bacteria found in water, soil, plants, and immunocompromised humans. Cystic fibrosis (CF) patients are the most vulnerable population to lung colonization by these bacteria. Upon infection, choline and succinate are released from the CF lungs and are catabolized by P. aeruginosa. The bacteria accumulate inorganic polyphosphates, rather than succinate, when choline is catabolized, producing physiological and morphological changes leading to ineradicable infection. Thus, we sought to quantify the enzymes responsible for polyphosphate accumulation and to determine how choline catabolism affects energy flow and storage. Subcellular fractions showed that exo-polyphosphate phosphatase (PPX) activity resides mainly in the periplasm, and three isoenzymes of 24, 70, and 200 KD were found. The PPX activity in the periplasm of bacteria grown with choline was inhibited in an anti-competitive manner from Km 0.5 to 1 μM, and their Vmax increased from 50 to 100 nmol PO 4 ≡ /min/g of protein in succinate medium. Since PPX inhibition by choline did not explain the 3.8-fold increase in polyphosphates, we quantified the polyphosphate kinase activity, and its significant 2.4-fold increase was consistent with the accumulation. Furthermore, intracellular ATP concentration directly correlated with the energetic yield of the carbon source and was significantly higher for succinate, suggesting that the restriction of energy caused by choline catabolism may induce morphological and physiological changes to the swarm form thus facilitating their migration and tissue colonization.

PMID:36816298 | PMC:PMC9929196 | DOI:10.1016/j.heliyon.2022.e12601

Pediatr Pulmonol. 2023 Feb 23. doi: 10.1002/ppul.26359. Online ahead of print.

ABSTRACT

BACKGROUND: Cepacia syndrome (CS) is an acute, necrotizing pneumonia with a high mortality rate, occurring in patients with cystic fibrosis (CF) infected with Burkholderia cepacia complex (BCC). Due to its low incidence, data on this condition are limited.

METHODS: We conducted a systematic review of the reported cases of CS by searching MEDLINE, Embase and the Cochrane Library to improve knowledge on this rare but potentially lethal condition.

RESULTS: We included 15 eligible articles, describing 18 cases (9 females) of CS. Median age at onset was 22 years (range: 10 - 60 years); median time to CS after first infection by BCC was 5 years (range: 1 - 26 years). B. cenocepacia was the most frequently reported causative agent. All patients received intravenous antibiotic treatment (most frequently including cotrimoxazole), while inhaled antibiotics were used in 5 patients (27.8%). Immunosuppressant agents were the most commonly prescribed supportive treatment (n= 7, 39%). Half of the patients died (9/18, 50%).

CONCLUSIONS: This study describes epidemiological, clinical characteristics and prognosis of CS cases reported over the last 24 years. CS is a rare yet severe complication of BCC infection in patients with CF, which occurs several years after BCC colonization and has a negative outcome in 50% of the patients. Data are too scanty to identify the most effective therapeutic approach. This article is protected by copyright. All rights reserved.

PMID:36815622 | DOI:10.1002/ppul.26359

Pediatr Pulmonol. 2023 Feb 23. doi: 10.1002/ppul.26366. Online ahead of print.

ABSTRACT

OBJECTIVE: To assess the clinical characteristics and outcomes of cystic fibrosis in Palestine by studying the quality of life (QoL)of participants.

METHOD: Thiscross-sectional study involved theapplication of Cystic Fibrosis Questionnaire-Revised (CFQ-R) to participantsattending the pediatric pulmonology clinic at Caritas Baby HospitalbetweenJanuary and May 2017. Health status was assessed by measuring pulmonary function test (FEV1 ), body mass index (BMI), age of CF diagnosis, and presence of other affected siblings or deaths in the family.

RESULTS: There were77 participants from 58 families: 46.8% (36/77) were males,and 53.3% (41/77) were females. The mean age was 10.7 years (range: 0.5-36 years). The participants were divided into three groups by age in years: group I (< 6), II (6-13), and III (≥ 14). The highest and lowest CFQ scores were for the eatingdomain in group III (55.6 ± 22.5) and the body domain in group II (14.5 ± 17.7), respectively. Mean illness severity was 69.6% (range: 33-111%).The mean BMI was 15.9 (range: 9.6-23.1). The mean age at the time of diagnosis was 4.2years(± 6.3). The study showed that1.7%of the families(1/58)had four affected siblings,and 21% (12/58) had death cases related to CF, of which58.3% (7/12) were from the Hebron district. Finally, all parameters for CF participants in West Bank, Palestine were noticeably lower than those reported in other countries.

CONCLUSIONS: This study illustrates the need fornew therapies for CF participants in Palestine to improve QoL, health status, and longevity. This article is protected by copyright. All rights reserved.

PMID:36815504 | DOI:10.1002/ppul.26366

Front Pediatr. 2023 Feb 6;10:1084313. doi: 10.3389/fped.2022.1084313. eCollection 2022.

ABSTRACT

Respiratory tract exacerbations play a crucial role in progressive lung damage of people with cystic fibrosis, representing a major determinant in the loss of functional lung tissue, quality of life and patient survival. Detection and monitoring of respiratory tract exacerbations are challenging for clinicians, since under- and over-treatment convey several risks for the patient. Although various diagnostic and monitoring tools are available, their implementation is hampered by the current definition of respiratory tract exacerbation, which lacks objective "cut-offs" for clinical and lung function parameters. In particular, the latter shows a large variability, making the current 10% change in spirometry outcomes an unreliable threshold to detect exacerbation. Moreover, spirometry cannot be reliably performed in preschool children and new emerging tools, such as the forced oscillation technique, are still complementary and need more validation. Therefore, lung imaging is a key in providing respiratory tract exacerbation-related structural and functional information. However, imaging encompasses several diagnostic options, each with different advantages and limitations; for instance, conventional chest radiography, the most used radiological technique, may lack sensitivity and specificity in respiratory tract exacerbations diagnosis. Other methods, including computed tomography, positron emission tomography and magnetic resonance imaging, are limited by either radiation safety issues or the need for anesthesia in uncooperative patients. Finally, lung ultrasound has been proposed as a safe bedside option but it is highly operator-dependent and there is no strong evidence of its possible use during respiratory tract exacerbation. This review summarizes the clinical challenges of respiratory tract exacerbations in patients with cystic fibrosis with a special focus on imaging. Firstly, the definition of respiratory tract exacerbation is examined, while diagnostic and monitoring tools are briefly described to set the scene. This is followed by advantages and disadvantages of each imaging technique, concluding with a diagnostic imaging algorithm for disease monitoring during respiratory tract exacerbation in the cystic fibrosis patient.

PMID:36814432 | PMC:PMC9940849 | DOI:10.3389/fped.2022.1084313

PLOS Digit Health. 2023 Jan 12;2(1):e0000179. doi: 10.1371/journal.pdig.0000179. eCollection 2023 Jan.

ABSTRACT

Precise and timely referral for lung transplantation is critical for the survival of cystic fibrosis patients with terminal illness. While machine learning (ML) models have been shown to achieve significant improvement in prognostic accuracy over current referral guidelines, the external validity of these models and their resulting referral policies has not been fully investigated. Here, we studied the external validity of machine learning-based prognostic models using annual follow-up data from the UK and Canadian Cystic Fibrosis Registries. Using a state-of-the-art automated ML framework, we derived a model for predicting poor clinical outcomes in patients enrolled in the UK registry, and conducted external validation of the derived model using the Canadian Cystic Fibrosis Registry. In particular, we studied the effect of (1) natural variations in patient characteristics across populations and (2) differences in clinical practice on the external validity of ML-based prognostic scores. Overall, decrease in prognostic accuracy on the external validation set (AUCROC: 0.88, 95% CI 0.88-0.88) was observed compared to the internal validation accuracy (AUCROC: 0.91, 95% CI 0.90-0.92). Based on our ML model, analysis on feature contributions and risk strata revealed that, while external validation of ML models exhibited high precision on average, both factors (1) and (2) can undermine the external validity of ML models in patient subgroups with moderate risk for poor outcomes. A significant boost in prognostic power (F1 score) from 0.33 (95% CI 0.31-0.35) to 0.45 (95% CI 0.45-0.45) was observed in external validation when variations in these subgroups were accounted in our model. Our study highlighted the significance of external validation of ML models for cystic fibrosis prognostication. The uncovered insights on key risk factors and patient subgroups can be used to guide the cross-population adaptation of ML-based models and inspire new research on applying transfer learning methods for fine-tuning ML models to cope with regional variations in clinical care.

PMID:36812602 | DOI:10.1371/journal.pdig.0000179

J Biol Phys. 2023 Feb 22. doi: 10.1007/s10867-022-09624-w. Online ahead of print.

ABSTRACT

Calcium ([Formula: see text]), inositol trisphosphate ([Formula: see text]), and nitric oxide (NO) signaling are essential to maintain the structural integrity and physiological activity of fibroblast cells. The accumulation of excess quantity of NO for longer periods can lead to a variety of fibrotic disorders, including heart disease, penile fibrosis in Peyronie's disease (PD), and cystic fibrosis. The dynamics of these three signaling processes and their interdependence in fibroblast cells are not clearly known to date. A systems biology model is proposed using reaction-diffusion equations for calcium, [Formula: see text], and calcium-dependent NO synthesis in fibroblast cells. The finite element method (FEM) is used to examine [Formula: see text], [Formula: see text], and NO regulation and dysregulation in cells. The results throw light on the conditions that disturb the coupled [Formula: see text] and [Formula: see text] dynamics and the influence of these factors on the levels of NO concentration in the fibroblast cell. The findings suggest that changes in source inflow, buffers, and diffusion coefficient might induce an increase or reduction in nitric oxide and [Formula: see text] synthesis, resulting in fibroblast cell diseases. Furthermore, the findings provide new information regarding the size and intensity of diseases in response to changes in several factors of their dynamics, which has been linked to the development of cystic fibrosis and cancer. This knowledge could be valuable for developing novel approaches to the diagnosis of diseases and therapies for various disorders of fibroblast cells.

PMID:36811722 | DOI:10.1007/s10867-022-09624-w

Int Forum Allergy Rhinol. 2023 Feb 22. doi: 10.1002/alr.23143. Online ahead of print.

NO ABSTRACT

PMID:36811323 | DOI:10.1002/alr.23143

Pediatr Rep. 2023 Jan 28;15(1):58-68. doi: 10.3390/pediatric15010008.

ABSTRACT

BACKGROUND: The pandemic of coronavirus disease 2019 (COVID-19) was undoubtedly a stressful experience for everyone. General opinion believed that children with acute or chronic illness could experience additional burden, but this is not confirmed. The aim of this study is to understand how children and adolescents already suffering from acute or chronic illness (e.g., cancer, cystic fibrosis, neuropsychiatric disorders) feel about the COVID-19 pandemic, and if the experience is significantly different between these children and children without illness.

METHODS: Children and adolescents affected by acute or chronic illness (named the "fragile group") treated at the Regina Margherita Children Hospital in Italy, were enrolled in the study by filling a questionnaire about their pandemic experiences. Also, a group of children and adolescents without acute or chronic illness (named the "low-risk group") recruited in the emergency department of the hospital, participated in the study in order to compare experiences.

RESULTS: The study group was composed of 166 children and adolescents (Median age = 12 yrs; 78% fragile group, 22% low-risk group). Participants experienced a general state of fear of the virus and of a potential infection for both themselves and their families, while feelings and thoughts that interfere with daily functioning were less frequent. The fragile group seems to be more resilient towards the pandemic situation than low-risk group and some differences on the basis of the type of illness were found in the fragile group.

CONCLUSIONS: Dedicated psychosocial intervention must be proposed in order to support fragile children and adolescents' well-being during the pandemic, also on the basis of their clinical and mental history.

PMID:36810338 | DOI:10.3390/pediatric15010008

Physiother Theory Pract. 2023 Feb 21:1-7. doi: 10.1080/09593985.2023.2182654. Online ahead of print.

ABSTRACT

BACKGROUND: Increasing physical activity in people with Cystic Fibrosis (pwCF) can positively influence their physical and mental wellbeing. Online activities provide opportunity for outpatient CF populations to enhance physical activity.

METHODS: PwCF within a large Scottish CF unit were invited to participate in a pilot study of online exercise and education sessions. Those participating shared opinions on motivation, fitness habits, types of activities enjoyed pre and during shielding, and desirable goals for online activity. Subsequently, an online activity timetable was created offering daily exercise classes. Educational presentations driven by patient request were delivered in context appropriate to health, wellbeing, and infection control needs during the pandemic and the advent of modulator therapies. Twenty-eight group exercise sessions and 12 educational sessions occurred over the six-week pilot, following which, a post-pilot questionnaire was sent to those who had participated in the sessions. Risk assessment and exercise modifications ensured safe practice and accommodation for all levels of respiratory disease.

RESULTS: Twenty-six pwCF attended one or more exercise sessions and 37 pwCF attended one or more education sessions. Group exercise and education improved time efficiency compared to in-person face-to-face delivery. The post-pilot questionnaire demonstrated increases in motivation and perceived fitness, with positive comments regarding peer support and enhanced socialization. Personal fitness goals were fully or partially achieved by 91% of participants.

CONCLUSION: Patient feedback suggested the implementation of online exercise and education sessions for pwCF was a satisfactory and convenient way to deliver exercise allowing for optimization and progression of personal goals.

PMID:36809231 | DOI:10.1080/09593985.2023.2182654