Upper Limb Compartment Syndrome-An Extremely Rare Life-Threatening Complication of Cutaneous Anthrax.

(1) Background: Cutaneous anthrax is a disease caused by a Gram-positive bacillus, spore-forming Bacillus anthracis (BA). Cutaneous anthrax accounts for 95% of all anthrax cases, with mortality between 10-40% in untreated forms. The most feared complication, which can be life-threatening and is rarely encountered and described in the literature, is compartment syndrome. (2) Methods: We report a series of six cases of cutaneous anthrax from the same endemic area. In two of the cases, the disease was complicated by compartment syndrome. The systematic review was conducted according to systematic review guidelines, and the PubMed, Google Scholar, and Web of Science databases were searched for publications from 1 January 2008 to 31 December 2023. The keywords used were: "cutaneous anthrax" and "compartment syndrome by cutaneous anthrax". (3) Results: For compartment syndrome, emergency surgical intervention for decompression was required, along with another three surgeries, with hospitalization between 21 and 23 days. In the systematic review, among the 37 articles, 29 did not contain cases focusing on compartment syndrome of the thoracic limb in cutaneous anthrax. The results were included in a Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) flow diagram. (4) Conclusions: Early recognition of the characteristic cutaneous lesions and compartment syndrome with early initiation of antibiotics and urgent surgical treatment is the lifesaving solution.

Exploring the gut-brain Axis: Potential therapeutic impact of Psychobiotics on mental health.

One of the most challenging and controversial issues in microbiome research is related to gut microbial metabolism and neuropsychological disorders. Psychobiotics affect human behavior and central nervous system processes via the gut-brain axis, involving neuronal, immune, and metabolic pathways. They have therapeutic potential in the treatment of several neurodegenerative and neurodevelopmental disorders such as depression, anxiety, autism, attention deficit hyperactivity disorder, Alzheimer's disease, Parkinson's disease, schizophrenia, Huntington's disease, anorexia nervosa, and multiple sclerosis. However, the mechanisms underlying the interaction between psychobiotics and the abovementioned diseases need further exploration. This review focuses on the relationship between gut microbiota and its impact on neurological and neurodegenerative disorders, examining the potential of psychobiotics as a preventive and therapeutic approach, summarising recent research on the gut-brain axis and the potential beneficial effects of psychobiotics, highlighting the need for further research and investigation in this area.

Personalized Decisional Algorithms for Soft Tissue Defect Reconstruction after Abdominoperineal Resection for Low-Lying Rectal Cancers.

BACKGROUND: Abdominoperineal resection (APR)-the standard surgical procedure for low-lying rectal cancer (LRC)-leads to significant perineal defects, posing considerable reconstruction challenges that, in selected cases, necessitate the use of plastic surgery techniques (flaps). PURPOSE: To develop valuable decision algorithms for choosing the appropriate surgical plan for the reconstruction of perineal defects. METHODS: Our study included 245 LRC cases treated using APR. Guided by the few available publications in the field, we have designed several personalized decisional algorithms for managing perineal defects considering the following factors: preoperative radiotherapy, intraoperative position, surgical technique, perineal defect volume, and quality of tissues and perforators. The algorithms have been improved continuously during the entire period of our study based on the immediate and remote outcomes. RESULTS: In 239 patients following APR, the direct closing procedure was performed versus 6 cases in which we used various types of flaps for perineal reconstruction. Perineal incisional hernia occurred in 12 patients (5.02%) with direct perineal wound closure versus in none of those reconstructed using flaps. CONCLUSION: The reduced rate of postoperative complications suggests the efficiency of the proposed decisional algorithms; however, more extended studies are required to categorize them as evidence-based management guide tools.

The rise in popularity of central bank digital currencies. A systematic review.

Central bank digital currencies (CBDCs) have been growing in popularity since 2018, as worldwide countries explore their impact and implementation options. This article analyzes the state of research around central bank digital currencies and the evolving landscape of CBDCs, and explores emerging areas of research and trends by using the PRISMA method and VOSviewer, with the goal of showing the main opportunities and challenges related to them. AMSTAR, DistillerSR, Eppi-Reviewer, ROBIS, and SRDR were the screening and quality evaluation tools employed for study eligibility criteria, design screening and content selection, text analysis data extraction, methodological quality predictors, and reliable and reproducible evidence assessment. A total of 1024 articles on central bank digital currencies were identified in Scopus and the Web of Science, out of which 747 have been included in the review (documents which were not in English language and not categorized as journal articles were excluded). Through an analysis of the relevant literature, the study categorizes CBDC research into positive, negative and neutral research, with a particular focus on sustainability issues, and conducts a keyword co-occurrence analysis using VOSviewer, following a narrowing down of the relevant articles to be included in the study by applying the PRISMA framework. This generates an overall view for experts and researchers who can use the main analyzed features of CBDCs and adapt them accordingly, taking into account relevant macroeconomic characteristics. The study highlights the need to continue interdisciplinary research, by adapting the research and CBDC characteristics to keep up with the latest technologies and with the shift towards green finance, and explores the elaborate relationship between finance, technology and sustainability.

Polyphenols-Enrichment of Vienna Sausages Using Microcapsules Containing Acidic Aqueous Extract of Boletus edulis Mushrooms.

Polyphenols are ubiquitous by-products in many plant foods. Their intake has been linked to health benefits like the reduced incidence of cardiovascular disease, diabetes, and cancer. These bioactive compounds can be successfully extracted from Boletus edulis mushrooms with acidic water. However, such extract could influence the sensory or textural properties of the product to be enriched; this inconvenience can be avoided by microencapsulating it using spray drying. In this study, the Vienna sausages were reformulated by replacing 2% of the cured meat with microcapsules containing an acidic aqueous extract of Boletus edulis mushrooms and by replacing ice flakes, an ingredient that represents 22.9% of the manufacturing recipe, with ice cubes from the same extract aiming to obtain a polyphenol enriched product. The results showed a higher content of polyphenols in sausages with extract (VSe; 568.92 µg/g) and microcapsules (VSm; 523.03 µg/g) than in the control ones (455.41 µg/g), with significant differences for 2,4-dihydroxybenzoic acid, protocatechuic acid, and 1-O-galloyl-ß-D-glucose. However, because of the oxidative stress caused to the microcapsules by the extract's spray drying, VSm had the highest oxidation state. PV and TBARS levels varied with storage time in all formulations, but given the short period tested, they were well below the allowed/recommended limit. The extract, as such, negatively affected the appearance, odor, and taste of Vienna sausages. The microcapsules, instead, determined an increase in their acceptance rate among consumers; they also prevented moisture loss and color changes during storage. In conclusion, microcapsules are more suitable for use as a polyphenol enrichment ingredient in Vienna sausages than the extract.

Gratitude, Job Resources, and Job Crafting: A Two-Wave Longitudinal Study on a Sample of Romanian Employees / [La gratitud, los recursos del puesto de trabajo y la adaptación del puesto al empleado: un estudio en dos momentos con una muestra de empleados rumanos]

In this two-wave study, we tested whether there would be positive and reciprocal relationships between employees’ gratitude and the job resources they perceive at work, as well as between gratitude and job crafting behaviours. Moreover, we explored whether job crafting could mediate the relationship between gratitude and job resources. The participants were 275 Romanian employees. No evidence for reciprocal relationships was found. Results showed that gratitude at T1 predicted more job resources at T2 (three months later), but job resources did not predict employees’ gratitude over time. One dimension of job crafting (increasing challenging job demands) at T1 positively predicted employees’ gratitude at T2, but the prospective effect of gratitude on job crafting was not significant (except for a marginally significant effect on increasing structural job resources). Job crafting did not mediate the longitudinal relationship between employees’ gratitude and job resources. These findings are discussed in relation to previous literature.(AU)

En este estudio se probó en dos momentos distintos si había relaciones positivas recíprocas entre la gratitud de los empleados y los recursos que percibían en el trabajo, así como entre la gratitud y la adaptación del puesto de trabajo al empleado. También se exploró si la adaptación del puesto al empleado podría mediar la relación entre gratitud y recursos del puesto de trabajo. En el estudio participaron 275 empleados rumanos. No se demostró que hubiera relaciones recíprocas. Los resultados indican que la gratitud en T1 predecía más los recursos del puesto en T2 (tres meses después), pero estos no predecían la gratitud de los empleados a lo largo del tiempo. Una dimensión de la adaptación del puesto al empleado, endurecer las exigencias del puesto en T1 predecía en sentido positivo la gratitud de los empleados en T2, pero el efecto prospectivo de la gratitud en la adaptación del puesto a los empleados no era significativo, excepto un efecto marginalmente significativo en el aumento de los recursos estructurales del puesto. La adaptación del puesto al empleado no mediaba la relación longitudinal entre la gratitud de los empleados y los recursos del puesto de trabajo. Se comentan los resultados en relación con las publicaciones anteriores.(AU)

The Antioxidant, Antibacterial and Cell-Protective Properties of Bioactive Compounds Extracted from Rowanberry (Sorbus aucuparia L.) Fruits In Vitro.

Considering that Sorbus aucuparia fruits have been underutilized despite their tremendous potential, this study aimed to correlate the in vitro antioxidant, antibacterial and cell-protective abilities of fruit extracts derived from Sorbus aucuparia Romanian cultivars with their phytochemical composition. Therefore, following the preparation of ethanolic and carotenoid extracts, phytochemical screening was performed using UV-Vis and HPLC-DAD-ESI-MS methods. The antioxidant activity was analyzed using DPPH and FRAP tests. As the results revealed high contents of bioactive compounds (polyphenols 1.11 mg GAE/g DM, flavonoids 430.06 µg QE/g DM and carotenoids 95.68 µg/g DM) and an important antiradical action (DPPH 24.51 mg/mL and FRAP 0.016 µM TE/mL), we chose to further examine the fruits' biological properties. The antibacterial capacity was assessed employing agar well diffusion and broth microdilution techniques, with fruits displaying an intense activity against MSSA, MRSA and Enterococcus faecalis, but also E. coli and Pseudomonas aeruginosa. The cell-protective activity was analyzed on gentamicin-stressed renal cells, through MTT and Annexin V-FITC assays. Importantly, a significant increase in viability was registered on stressed cells following extract administration in low doses; nevertheless, viability was noticed to decline when exposed to elevated concentrations, potentially due to the cumulative actions of the extract and gentamicin. These findings offer novel light on the antibacterial activity of Sorbus aucuparia Romanian cultivars, as well as their cell-protective ability in renal cell injury.

Consumer perception towards electronic products from recycled components in the current geopolitical context: A structural equation modelling approach.

Russia's invasion of Ukraine had a negative impact worldwide, causing a severe energy crisis that also affected EU countries, which are now involved in combating the growing energy prices while also speeding up the deployment of renewable energy sources. This armed conflict impacted the electronic components supply chain, causing high prices and disruption for different raw materials, resulting in material shortages for electronic components and affecting electronic product (EP) manufacturing. Since the start of the geopolitical crisis due to the Russo-Ukrainian War, (dis)information has been disseminated via social media, affecting users' cognition, attitudes, and behavioral intentions. Therefore, this paper aims to assess the impact of social media usage, Russo-Ukrainian war fear, consumers' green values, perceived quality, usage enjoyment, and product image on consumers' purchase intention toward recycled electronic products. Based on the Stimulus-Organism-Response (SOR) approach, the authors propose a conceptual model highlighting the factors that enhance consumers' purchase intentions towards recycled electronic products. The model is tested empirically via quantitative-based research, with data collected from Romania, a close neighbor of the armed conflict, and assessed employing with structural equations modeling via SmartPLS 3.0. Results confirm that social media usage, consumers' green values, and the Russo-Ukrainian war fear do enhance consumers' image of recycled electronic products, thus leading to their increased purchase intention. The novelty of this paper consists in extending the SOR-based research regarding consumers' behavioral intentions toward buying recycled electronic products in the context of the Russo-Ukrainian war. The study highlights important managerial implications for both the electronic industry and retailers selling such goods.

Succinic acid - A run-through of the latest perspectives of production from renewable biomass.

Succinic acid (SA) production is continuously rising, as its applications in diverse end-product generation are getting broader and more expansive. SA is an eco-friendly bulk product that acts as a valuable intermediate in different processes and might substitute other petrochemical-based products due to the inner capacity of microbes to biosynthesize it. Moreover, large amounts of SA can be obtained through biotechnological ways starting from renewable resources, imprinting at the same time the concept of a circular economy. In this context, the target of the present review paper is to bring an overview of SA market demands, production, biotechnological approaches, new strategies of production, and last but not least, the possible limitations and the latest perspectives in terms of natural biosynthesis of SA.

Exploring Technological, Safety and Probiotic Properties of Enterococcus Strains: Impact on Rheological Parameters in Fermented Milk.

Enterococci, known for their resilience, are commonly found in food, the environment, and the gastrointestinal tracts of humans and animals. In recent research, six strains of enterococcus were isolated from bat guano. These include Enterococcus mundtii SRBG1, Enterococcus gallinarum SRBG3, Enterococcus faecium SRBG2, Enterococcus casseliflavus EC1, and Enterococcus devriesei CAU 1344. Identification was done using 16S DNA analysis. Each strain underwent evaluation for its technological properties (such as tolerances to various NaCl concentrations and temperatures, as well as amylolytic, ß-galactosidase, lipolytic, and proteolytic activities, and EPS production) and selected probiotic properties (including safety profile, resistance to 0.3 percent bile salts and gastric juice with a pH of 2.5, lysozyme tolerance, and antibacterial and antibiofilm activities against four foodborne pathogens). The results were analyzed using Principal Component Analysis. This analysis revealed that E. mundtii SRBG1 and E. gallinarum SRBG3, followed by E. faecium SRBG2, were most closely associated with a broad range of technological characteristics and were subsequently used for fermenting skimmed milk. The rheological properties of the samples indicated a shear-thinning or non-Newtonian behavior. Furthermore, during storage of the fermented milk at 4 °C over periods of 1, 7, 14, and 21 days, there were no significant changes in bacterial count (at around 7 log10 CFU/mL) and pH when fermented with the three evaluated strains.

Fun in a Box? Loneliness and Adolescents' Problematic Smartphone Use: A Moderated Mediation Analysis of the Underlying Mechanisms.

Addictive smartphone use is one of the most concerning behaviors among adolescents. The present study investigated the indirect effects of self-esteem and boredom proneness and the moderating role of the need to belong on the link between loneliness and addictive smartphone use (ASU). Our sample included 357 adolescents aged 12 to 19 (Mage = 15.56, SD = 1.01, 57.42% males) from ten public schools in Romania. We used a moderated mediation approach, with moderation of the both second mediation paths and the direct effect path. Results suggested that the influence of loneliness on ASU was statistically significant and partially mediated both by self-esteem and boredom proneness. Adolescents' need to belong significantly moderated the positive association between boredom proneness and ASU, and the direct negative association between loneliness and ASU; however, it did not moderate the negative association between self-esteem and ASU. Thus, high levels of the need to belong also increased the influence of boredom proneness on AUS and had a marginally significant effect on the relation between loneliness and ASU. These results suggested that adolescents' need to belong, self-esteem, and boredom proneness might contribute to developing ASU. Interventions centered around countering the adverse effects of excessive technology use ought to consider group activities that facilitate social bonding to satisfy the participants' need to belong, reduce their levels of boredom, and, thus, reduce the risk of developing ASU symptoms.

Probiotic-driven advancement: Exploring the intricacies of mineral absorption in the human body.

The interplay between probiotics and mineral absorption is a topic of growing interest due to its great potential for human well-being. Minerals are vital in various physiological processes, and deficiencies can lead to significant health problems. Probiotics, beneficial microorganisms residing in the gut, have recently gained attention for their ability to modulate mineral absorption and mitigate deficiencies. The aim of the present review is to investigate the intricate connection between probiotics and the absorption of key minerals such as calcium, selenium, zinc, magnesium, and potassium. However, variability in probiotic strains, and dosages, alongside the unique composition of individuals in gut microbiota, pose challenges in establishing universal guidelines. An improved understanding of these mechanisms will enable the development of targeted probiotic interventions to optimize mineral absorption and promote human health.

Food Retail Resilience Pre-, during, and Post-COVID-19: A Bibliometric Analysis and Research Agenda.

This paper aims to conduct a bibliometric mapping and systematic review of the food retail industry's resilience strategy in the context of COVID-19. Specifically, we aim to identify relevant research gaps in the industry during the pre-, during, and post-pandemic periods and highlight the differences between B2B and B2C sectors. We analyzed articles in the Scopus database from 2019 to 2022 using the PRISMA method for article selection, resulting in a total of 69 articles. We employed a VOS viewer for bibliometric mapping. Our analysis revealed that most studies focused on the impact of COVID-19, with only a few examining the pre- and post-pandemic periods critically. In the B2C context, we identified two major topics: changes in purchasing and consumption behavior, and food waste and safety. In the B2B sector, the two most recurrent subjects were retailers' strategies and supply chain management. This study provides valuable insights for policymakers by exploring industry trends and for scholars by highlighting future research agendas based on the identified topics.

Characterization of the Chemical Composition and Biological Activities of Bog Bilberry (Vaccinium uliginosum L.) Leaf Extracts Obtained via Various Extraction Techniques.

This investigation aimed to assess the chemical composition and biological activities of bog bilberry (Vaccinium uliginosum L.) leaves. Hydroethanolic extracts were obtained using four extraction techniques: one conventional (CE) and three alternative methods; ultrasound (UAE), microwave (MAE) and high-pressure (HPE) extractions. Spectrophotometric analysis was conducted to determine their chemical content, including the total phenolic content (TPC) and total flavonoid content (TFC). Furthermore, their antioxidative and antimicrobial properties were evaluated. HPLC (high performance liquid chromatography) analysis identified and quantified 17 phenolic compounds, with chlorogenic acid being the predominant compound, with the lowest level (37.36 ± 0.06 mg/g) for the bog bilberry leaf extract obtained by CE and the highest levels (e.g., HPE = 44.47 ± 0.08 mg/g) for the bog bilberry leaf extracts obtained by the alternative methods. Extracts obtained by HPE, UAE and MAE presented TPC values (135.75 ± 2.86 mg GAE/g; 130.52 ± 1.99 mg GAE/g; 119.23 ± 1.79 mg GAE/g) higher than those obtained by the CE method (113.07 ± 0.98 mg GAE/g). Regarding the TFC values, similar to TPC, the highest levels were registered in the extracts obtained by alternative methods (HPE = 43.16 ± 0.12 mg QE/g; MAE = 39.79 ± 0.41 mg QE/g and UAE = 33.89 ± 0.35 mg QE/g), while the CE extract registered the lowest level, 31.47 ± 0.28 mg QE/g. In the case of DPPH (1,1-diphenyl-2-picrylhydrazyl) antioxidant activity, the extracts from HPE, UAE and MAE exhibited the strongest radical scavenging capacities of 71.14%, 63.13% and 60.84%, respectively, whereas the CE extract registered only 55.37%. According to Microbiology Reader LogPhase 600 (BioTek), a common MIC value of 8.88 mg/mL was registered for all types of extracts against Staphylococcus aureus (Gram-positive bacteria) and Salmonella enterica (Gram-negative bacteria). Moreover, the alternative extraction methods (UAE, HPE) effectively inhibited the growth of Candida parapsilosis, in comparison to the lack of inhibition from the CE method. This study provides valuable insights into bog bilberry leaf extracts, reporting a comprehensive evaluation of their chemical composition and associated biological activities, with alternative extraction methods presenting greater potential for the recovery of phenolic compounds with increased biological activities than the conventional method.

Toxic sensation seeking? Psychological distress, cyberbullying, and the moderating effect of online disinhibition among adults.

Cyberbullying among adults is barely studied, though its consequences may be as severe as in children and adolescents. The present study investigated the links between psychological distress, cyber-perpetration, and passive cyber-bystander behavior. We also explored the moderating role of toxic disinhibition in this regard. Our sample comprised 385 adults aged 19-66 (M = 28.35, SD = 11.22, 76.62% females). The results suggested that psychological distress was significantly associated with cyberbullying perpetration and passive bystander behavior. Also, higher psychological distress significantly predicted toxic disinhibition. Further moderation analyses suggested that at high and medium levels of toxic disinhibition, psychological distress significantly predicted cyberbullying perpetration but not passive cyber-bystander behavior. Finally, we discuss our results regarding their theoretical and practical implication for cyberbullying prevention among adults.

Is it possible to predict post-adenotonsillectomy hemorrhage in children with preoperative blood tests? Single-center retrospective study.

Introduction: Post-adenotonsillectomy (PAT) bleeding, a life-threatening surgical complication, remains unpredictable despite preoperative blood tests. Every surgeon would like predictive markers for this complication of one of the most common procedures performed in pediatric ear, nose, and throat (ENT). Objective: The purpose of the study is to see whether the results of the blood tests we perform routinely preoperatively in children undergoing adenotonsillectomy (AT) (lymphocyte count and percentage, C reactive protein, fibrinogen, or coagulation variables International Normalized Ratio and activated partial thromboplastin time) can potentially predict early post-AT bleeding. Focus has been placed on the presence of relative lymphocytosis (a value of lymphocyte percentage above 55%) in the blood cell count of the patients and its possible connection to postoperative hemorrhage. Method: We conducted an observational retrospective study on 801 children undergoing adenoidectomy, tonsillectomy, or AT over a period of 6 months in our ENT department. Statistical analysis was performed to compare the data. Results: we did not find a statistically significant correlation between preoperative blood markers (coagulation or inflammatory) and early post-AT bleeding. An important blood marker in relation to PAT bleeding appears to be relative lymphocytosis. Relative lymphocytosis has a weak predictive value of early postoperative bleeding in children with AT (sensitivity of only 31.58%, but acceptable specificity of above 80%). In other words, 80% of patients without relative lymphocytosis will not bleed in the first 24 h postoperatively. Children with relative lymphocytosis may need tighter surveillance in the first 24 h after AT. Conclusions: Relative lymphocytosis has a weak predictive value of early postoperative bleeding in children with AT children.

Anti-fibrotic effects of nintedanib on lung fibroblasts derived from patients with Progressive Fibrosing Interstitial Lung Diseases (PF-ILDs).

The tyrosine kinase inhibitor nintedanib has been recently approved for the treatment of Interstitial Lung Diseases (ILDs) that manifest a progressive fibrosis phenotype other than Idiopathic pulmonary Fibrosis (IPF). Nintedanib reduces the development of lung fibrosis in various animal models resembling features of PF-ILD and in vitro, it inhibits the fibrosing phenotype of human lung fibroblasts (HLFs) isolated from patients with IPF. To get insight on the cellular and molecular mechanisms that drive the clinical efficiency of nintedanib in patients with non-IPF PF-ILD, we investigated its effects on the fibrosing functions of HLFs derived from patients with PF-hypersensitivity pneumonitis (PF-HP, n = 7), PF-sarcoidosis (n = 5) and pleuroparenchymal fibroelastosis (PPFE, n = 4). HLFs were treated with nintedanib (10 nM-1 µM) and then stimulated with PDGF-BB (25-50 ng/ml) or TGF-ß1 (1 ng/ml) for 24-72 h to assess proliferation and migration or differentiation. At nanomolar concentrations, nintedanib reduced the levels of PDGF receptor and ERK1/2 phosphorylation, the proliferation and the migration of PF-HP, PF-sarcoidosis and PPFE HLFs stimulated with PDGF-BB. Moreover, nintedanib also attenuates the myofibroblastic differentiation driven by TGF-ß1 but only when it is used at 1 µM. The drug reduced the phosphorylation of SMAD2/3 and decreased the induction of collagen, fibronectin and α-smooth muscle actin expression induced by TGF-ß1. In conclusion, our results demonstrate that nintedanib counteracts fundamental fibrosing functions of lung fibroblasts derived from patients with PF-HP, PF-sarcoidosis and PPFE, at concentrations previously reported to inhibit control and IPF HLFs. Such effects may contribute to its clinical benefit in patients suffering from these irreversible ILDs.

Tips and Tricks for a Successful Literature Review as Part of Medical Career Development.

The literature review is a direct consequence of the increased volume of scientific information, becoming a necessity not only for the medical field. Such material, properly done, is of great use to any professional who wishes to keep abreast of the latest knowledge and concepts. The proposed goal is to help and guide resident doctors, doctoral students, and young researchers in understanding the concepts that are the basis of conducting a literature review and acquiring the generally accepted methodology for conducting it. The selection of information sources, accessing databases, the concept of peer-review, indexing and the impact factor are clearly presented as elements that cannot be neglected in the valorisation of scientific information sources. The structure of a literature review must consider the generally accepted format for such an article, with each chapter having its own importance. Depending on the quality and heterogeneity of the results obtained after analysing the collected data, the review can be structured narratively or systematically, the homogeneity of the results allowing the application of statistical study methods (meta-analysis). Although it seems difficult, conducting a literature review is easier and faster than developing an original study based on experimental or clinical scientific research. The literature review can be carried out in comfortable conditions, online, regardless of location and is a welcome support in the development of the professional and scientific career.

A Diagnostic Challenge in an Adolescent with Collagen VI-Related Myopathy and Emotional Disorder-Case Report.

Collagen VI-related disorders constitute a spectrum of severities from the milder Bethlem myopathy (BM) to the Ullrich congenital muscular dystrophy (UCMD), which is more severe, and an intermediate form characterized by muscle weakness that begins in infancy. Affected children are able to walk, although walking becomes increasingly difficult starting in early adulthood. They develop contractures in the ankles, elbows, knees, and spine in childhood. In some affected cases, the respiratory muscles are weakened, requiring mechanical ventilation, particularly during sleep. Individuals with collagen VI-related myopathy are at risk of restrictive lung disease and sleep-disordered breathing due to the development of scoliosis associated with neuromuscular weakness. Typical signs of respiratory failure are not always present, and some patients are unaware that their respiratory muscles have become weaker. Here, we report a case of an intermediate form of collagen VI-related myopathy confirmed by next-generation sequencing. The girl presented morning headache, irritability, and aggressiveness, and because of these main symptoms, she was referred by the neurologist for respiratory evaluation. The result of spirometry was associated with hypoventilation shown during sleep studies, indicating the necessity to initiate home non-invasive ventilation (NIV) with immediate improvement in the symptoms. Neuromuscular disorders (NMDs) have a great impact on sleep, but only very few studies evaluating sleep quality in young patients with collagen VI-related myopathy have been described. Daytime symptoms of sleep-disordered breathing may include irritability, emotional lability, and poor attentiveness, but these can be overseen by the severity of other complex medical problems in patients with collagen VI-related myopathy. We underline the importance of the close monitoring of respiratory function, sleep evaluation, and decision making to support the NIV treatment of other collagen VI-related myopathy variant-specific patients. Early recognition of sleep disturbances and initiation of respiratory support can preserve or enhance the quality of life for patients and their caregivers. Routine screening for identification of emotional distress should be instituted in the clinical practice using validated psychological measures in a multidisciplinary approach with different intervention strategies for both patient and parent when necessary.