Clinical exome analysis and targeted gene repair of the c.1354dupT variant in iPSC lines from patients with PROM1-related retinopathies exhibiting diverse phenotypes.

BACKGROUND: Inherited retinal dystrophies (IRD) are one of the main causes of incurable blindness worldwide. IRD are caused by mutations in genes that encode essential proteins for the retina, leading to photoreceptor degeneration and loss of visual function. IRD generates an enormous global financial burden due to the lack of understanding of a significant part of its pathophysiology, molecular diagnosis, and the near absence of non-palliative treatment options. Patient-derived induced pluripotent stem cells (iPSC) for IRD seem to be an excellent option for addressing these questions, serving as exceptional tools for in-depth studies of IRD pathophysiology and testing new therapeutic approaches. METHODS: From a cohort of 8 patients with PROM1-related IRD, we identified 3 patients carrying the same variant (c.1354dupT) but expressing three different IRD phenotypes: Cone and rod dystrophy (CORD), Retinitis pigmentosa (RP), and Stargardt disease type 4 (STGD4). These three target patients, along with one healthy relative from each, underwent comprehensive ophthalmic examinations and their genetic panel study was expanded through clinical exome sequencing (CES). Subsequently, non-integrative patient-derived iPSC were generated and fully characterized. Correction of the c.1354dupT mutation was performed using CRISPR/Cas9, and the genetic restoration of the PROM1 gene was confirmed through flow cytometry and western blotting in the patient-derived iPSC lines. RESULTS: CES revealed that 2 target patients with the c.1354dupT mutation presented monoallelic variants in genes associated with the complement system or photoreceptor differentiation and peroxisome biogenesis disorders, respectively. The pluripotency and functionality of the patient-derived iPSC lines were confirmed, and the correction of the target mutation fully restored the capability of encoding Prominin-1 (CD133) in the genetically repaired patient-derived iPSC lines. CONCLUSIONS: The c.1354dupT mutation in the PROM1 gene is associated to three distinct AR phenotypes of IRD. This pleotropic effect might be related to the influence of monoallelic variants in other genes associated with retinal dystrophies. However, further evidence needs to be provided. Future experiments should include gene-edited patient-derived iPSC due to its potential as disease modelling tools to elucidate this matter in question.

Extended and Long-term Cancer Survivorship of Childhood Survivors: A Scoping Review of Nursing Evidence With Bibliometric Analysis.

BACKGROUND: With the increasing survival rates of childhood cancer, nurses' familiarity with published evidence has become crucial to care for this population and their families throughout the survival process. OBJECTIVE: To systematically identify and conduct a bibliometric analysis of nursing-related evidence concerning extended and long-term survival of childhood survivors. METHODS: A scoping review was conducted using bibliometric analysis with searches performed in the PubMed, CINAHL, SCOPUS, and Web of Science databases. A total of 300 studies on childhood cancer survival within the nursing field were included. RESULTS: The first study on this topic was published in 1975. American and Chinese researchers lead study publications, primarily publishing in nursing journals such as Cancer Nursing. Quantitative designs were prevalent, and the majority of the studies focused on physical late effects, overall quality of life, and survivor follow-up care. CONCLUSIONS: This study has allowed us to map and synthesize the bibliometric evidence pertaining to the extended and long-term survivorship of childhood cancer survivors in the nursing field. Consequently, it identifies gaps in knowledge, research trends, and areas necessitating further exploration. IMPLICATIONS FOR PRACTICE: The evidence presented in this review can facilitate academic and clinical discussions, offering a comprehensive synthesis of the published knowledge. More research needs to be developed on the topic, particularly in Central and Latin America, Africa, Southern and Eastern Europe, and in some regions of Asia. Furthermore, the scope of studies should extend beyond late effects and quality of life, encompassing the experience of surviving childhood cancer, including psychosocial and spiritual dimensions.

Methodological and strategic insights for online survey studies: an analysis based on the CHERRIES checklist.

The use of online surveys has become a valuable and widely employed tool in health research. However, the use of such instruments necessitates methodological rigor and optimization in their design to achieve the best response rates. Drawing upon relevant literature and the international CHERRIES guidelines for the development of online surveys, this article addresses methodological aspects related to ethical considerations and data protection (with reference to the Association of Internet Research's online ethics guide), study design and validation, recruitment, data collection processes, and data management and analysis. In conclusion, given the context of overexposure to online surveys, which can influence recruitment and response rates, strategies for their maximization are provided, encompassing both static and dynamic aspects of survey design.

Use of Online Communities among People with Type 2 Diabetes: A Scoping Review.

PURPOSE OF REVIEW: People with diabetes require continuous self-monitoring and face numerous decisions in their day-to-day lives. Therefore, on many occasions, they need more support than that provided by health professionals. In this context, peer support in online diabetes communities could be a useful tool. The purpose of the review is to describe, analyze and synthesize the available evidence on the use and health out-comes of online communities for people with type 2 diabetes mellitus. A scoping review was conducted in accordance with the Joanna Briggs Institute guidelines. Searches were performed PubMed, Web of Science, CINHAL, Scopus and Cochrane databases. RECENT FINDINGS: From 1821 identified documents, 6 articles were included. These studies explored the characteristics of diabetes online communities and the population features. Besides, the results were classified according to whether they were clinical, psychosocial, or addressed people's experiences with the online community. The analysis underscores their value in facilitating communication, improving diabetes management, and enhancing psychosocial well-being. Future investigations should prioritize longitudinal assessments to elucidate the sustained impact of community engagement and optimize user participation for enhanced patient outcomes. The growing relevance of new technologies has led to a significant number of individuals with chronic illnesses seeking peer support. Online health communities have emerged as virtual spaces where individuals with shared health interests interact and form relationships. Within these digital spaces, individuals can engage in peer interaction, observe behaviors, and mutually benefit, potentially leading to improved attitudes toward the disease.

Osteocondroma de calcáneo solitario con crecimiento después de la madurez esquelética. Reporte de un caso y revisión bibliográfica / Solitary calcaneal osteochondroma with growth after skeletal maturity. Case report and literature review

El osteocondroma es el tumor óseo benigno más frecuente, representa el 35% de los tumores óseos benignos y el 8% de los tumores óseos. Está formado por una exostosis de hueso trabecular recubierta de cartílago que afecta la región metafisaria de los huesos largos, a partir del cartílago de crecimiento, alrededor de la rodilla, en el hombro o la muñeca, y es extremadamente raro en las falanges de la mano o el pie. Crece durante la niñez y la adolescencia hasta alcanzar la madurez esquelética; por lo tanto, su crecimiento en un adulto sugiere la transformación maligna en condrosarcoma, aunque también existen reportes sin malignización. Además, el calcáneo es un hueso corto, por lo que, desde el punto de vista fisiopatológico, sería inesperada la aparición de este tipo de lesión. El objetivo de este artículo es presentar un caso de osteocondroma de calcáneo solitario con crecimiento después de la madurez esquelética, su diagnóstico y tratamiento, así como una revisión bibliográfica. Esta presentación de caso es importante, dados la localización infrecuente y el potencial de transformación maligna del osteocondroma, y la escasa bibliografía sobre el tema. Nivel de Evidencia: IV

Osteochondroma is the most common benign bone tumor, accounting for 35% of benign bone tumors and 8% of bone tumors. It is formed by an exostosis of trabecular bone covered with cartilage that affects the metaphyseal region of long bones, from the growth plate, around the knee, in the shoulder or wrist, and it is extremely rare in the phalanges of the hand or foot. It grows during childhood and adolescence until skeletal maturity. Therefore, its growth in an adult suggests a malignant transformation into chondrosarcoma, although there are also reports without malignancy. Furthermore, the calcaneus is a short bone, so the appearance of this type of injury would be pathophysiologically unexpected. Our objective is to present a case of solitary calcaneal osteochondroma with growth after skeletal maturity, its diagnosis and treatment, as well as a review of the literature. Its relevance stems from the scarcity of literature, its uncommon location, and the possibility of malignancy. Level of Evidence: IV

Riesgo de mortalidad en infecciones invasoras por Fusarium spp en niños con enfermedades hemato-oncológicas: una revisión sistemática de la literatura / Risk of mortality in invasive infections by Fusarium spp in children with hematooncological diseases: a literature systematic review

INTRODUCCIÓN: Los estudios sobre la infección fúngica invasora (IFI) por Fusarium spp en pacientes pediátricos con patología hemato-oncológica, son escasos, correspondiendo en general a series clínicas descriptas en forma retrospectiva, lo que dificulta conocer en profundidad sus características y evolución. OBJETIVO: Analizar la evolución fatal de la IFI causada por Fusarium spp en pacientes pediátricos con patología hemato-oncológica, llevándose a cabo una revisión sistemática. MATERIAL Y MÉTODOS: La búsqueda bibliográfica se realizó con fecha 23 de marzo de 2023, en las principales bases de datos (Medline (a través de PubMed), Embase (a través de Embase-Elsevier), The Cochrane Library (a través de Wiley), Cinahl (a través de EbscoHOST), SCI-EXPANDED y Scielo (a través de la WOS) y Scopus (a través de Scopus-Elsevier) y libre (mediante el motor Google) y revisando las citas de los artículos incluidos. RESULTADOS: Se rescataron 1.341 artículos, de los cuales se descartaron 931 por diversas razones. Mediante el análisis de los textos completos, finalmente se incluyeron 11 estudios. Todos los estudios eran de nivel 4 (serie de casos). Se detectó una notoria heterogeneidad (p < 0,008) entre los mismos. La mediana de la frecuencia de muerte observada implicó a un tercio de los afectados (Md 33 %; Q1:22,7-Q4:75). CONCLUSIONES: La mortalidad por IFI por Fusarium spp fue alta en niños con patología hemato-oncológica, en especial en aquellos con neutropenia profunda y mala respuesta al tratamiento de su enfermedad de base

BACKGROUND: Studies on invasive fungal infection (IFI) by Fusarium spp in pediatric patients with hemato-oncological pathology are scarce and limited and a few series of cases described retrospectively, which makes it difficult to fully understand their characteristics and outcome. With the aim of analyzing the fatal evolution of these patients, this systematic review was carried out. METHODS: The literature search was performed up to March 23, 2023, in the main databases, as Medline (through PubMed), Embase (through Embase-Elsevier), The Cochrane Library (through Wiley), Cinahl (through EbscoHOST), SCI-EXPANDED and Scielo (through WOS) and Scopus (through Scopus-Elsevier) and free (through the Google engine) and reviewing the citations of the included articles. RESULTS: 1341 articles were retrieved, of which 931 were discarded for various reasons. By analyzing its full texts, 11 studies were finally included. It was observed that heterogeneity among them was relevant (p < 0.008). Median frequency of death involved one third of those affected (Md 33%; Q1:22,7-Q4:75). CONCLUSIONS: Mortality due to IFI due to Fusarium spp was high in children with hemato-oncological pathology, especially in those with severe neutropenia and poor response to treatment of their underlying disease.

Egg yolk lipids induce sensitization to egg white proteins in a mouse model without adjuvant and exacerbate Th2 responses to egg white in cells from allergic patients.

This study evaluates the influence of egg lipid fractions in the induction of allergic sensitization to egg white (EW) proteins, using a mouse model of orally adjuvant-free induced allergy. Egg triglycerides (TG) and phospholipids (PL), and to a higher extent the whole egg lipid fraction (EL), induced allergy to EW proteins characterized by increased EW-specific IgG1. EL also increased EW-specific IgE. The administration to mice of a mixture of EW and EL increased the intestinal expression of Il33, Il25, and Tslp, the secretion of IL-33 and IL-6, the expansion of group 2 innate lymphoid cells, the regulation of Gata3, Il4 and Il13, dendritic cell (DC) activation and expression of DC molecules that drive Th2 differentiation. TG promoted the absorption of proteins through the intestinal epithelium, enhancing local Th2 responses, while PL favoured the delivery of antigens to the Peyer's Patches. This differential modulation of the site of absorption of egg proteins determined the different behaviour of TG and PL. Egg yolk lipids also induced activation of Th2-inducing innate responses on intestinal human cells in vitro and enhanced adaptive Th2 functions through the activation of DCs in egg-allergic subjects.

Evolutionary analysis and structure modelling of the Rcs-repressor IgaA unveil a functional role of two cytoplasmic small ß-barrel (SBB) domains.

The Rcs sensor system, comprising the RcsB/RcsC/RcsD and RcsF proteins, is used by bacteria of the order Enterobacterales to withstand envelope damage. In non-stress conditions, Rcs is repressed by IgaA, a membrane protein with three cytoplasmic regions (cyt-1, cyt-2 and cyt-3). How the Rcs-IgaA axis evolved within Enterobacterales has not been yet explored. Here, we report phylogenetic data supporting co-evolution of IgaA with RcsC/RcsD. Functional exchange assays showed that IgaA from Shigella and Dickeya, but not from Yersinia or the endosymbionts Photorhabdus and Sodalis, repress the Rcs system of Salmonella. IgaA from Dickeya, however, repress only partially the Rcs system despite being produced at high levels in the complementation assay. The modelled structures of these IgaA variants uncovered one periplasmic and two cytoplasmic conserved ß-rich architectures forming partially closed small ß-barrel (SBB) domains. Conserved residues map in a connector linking cytoplasmic SSB-1 and SBB-2 domains (E180-R265); a region of cyt-1 facing cyt-2 (R188-E194-D309 and T191-H326); and between cyt-2 and cyt-3 (H293-E328-R686). These structures validated early in vivo studies in Salmonella that assigned a role in function to R188, T191 and G262, and in addition revealed a previously unnoticed "hybrid" SBB-2 domain to which cyt-1 and cyt-2 contribute. IgaA variants not functional or partially functional in Salmonella lack H192-P249 and R255-D313 interactions. Among these variants, only IgaA from Dickeya conserves the helix α6 in SSB-1 that is present in IgaA from Salmonella and Shigella. RcsF and RcsD, which interact directly with IgaA, failed to show structural features linked to specific IgaA variants. Altogether, our data provide new insights into IgaA by mapping residues selected differently during evolution and involved in function. Our data also infer contrasting lifestyles of Enterobacterales bacteria as source of variability in the IgaA-RcsD/IgaA-RcsF interactions.

Effectiveness of Digital Health on the Quality of Life of Long-Term Breast Cancer Survivors: A Systematic Review.

OBJECTIVES: To identify, critically appraise, and synthesize the available evidence on the effectiveness of digital health interventions to improve the quality of life or any of its four dimensions (physical, psychological, social, and spiritual) in women survivors of breast cancer who are in the extended or permanent survival stage. DATA SOURCES: Systematic review-Four databases were searched: PubMed, CINAHL, PsycINFO, and Web of Science. CONCLUSION: The clinical evidence shows a positive relationship or association between eHealth use and improved quality of life in breast cancer survivors at extended or permanent survival stage. However, the findings point to a deficit in the assessment of the social and spiritual domains that play a fundamental role in the quality of life of survivors. IMPLICATION FOR NURSING PRACTICE: The findings found reflect implications of great value for nursing practice because these professionals are the main users of digital health tools to provide them to patients. Using these digital tools contributes to improving evidence-based practice and providing greater efficiency and effectiveness in the care of long-term cancer survivors.

Assessing the Reliability and Validity of the Spanish Version of the Actual Scope of Nursing Practice Scale.

Nursing workforce shortage is one of the main challenges for healthcare organizations and it is important to determine if nurses are fulfilling their full scope of practice. There is a questionnaire that measures the activities carried out by nurses, but there is no version available for the Spanish context. The purpose of this study was to develop a cross-cultural adaptation of the "Actual Scope of Nursing Practice" questionnaire by D'Amour et al., and to assess the psychometric characteristics of the Spanish version. An exploratory sequential research design was used. The cross-cultural adaptation was performed using translation, back-translation, review, and pre-testing. Psychometric properties were assessed to determine its construct validity and internal consistency. Among the 501 eligible nurses from the three main hospitals in the region, the first 310 nurses to respond to an online questionnaire were included in our study. The response rate was 61.9%. They were invited via email and completed the survey using the SurveyMonkey platform. The Spanish version of the questionnaire was obtained. A final scale with twenty items and two factors was confirmed with an adequate fit, with the item scores demonstrating that all items were optimally related to their respective latent construct. The alpha coefficients for the Spanish ASCOP scale were robust and revealed good internal consistency. This study showed that the Spanish version of the scale, "Scope of Nursing Practice", has a good degree of validity and reliability. This questionnaire can support nurse managers in realising nursing activities within their organisations and in promoting desirable work outcomes among nurses.

Main Challenges of Incorporating Environmental Impacts in the Economic Evaluation of Health Technology Assessment: A Scoping Review.

Health technology assessment (HTA) provides evidence-based information on healthcare technology to support decision making in many countries. Environmental impact is a relevant dimension of a health technology's value, but it has been poorly addressed in HTA processes in spite of the commitment that the health sector must have to contribute to mitigating the effects of climate change. This study aims to identify the state of the art and challenges for quantifying environmental impacts that could be incorporated into the economic evaluation (EE) of HTA. We performed a scoping review that included 22 articles grouped into four types of contribution: (1) concepts to draw up a theoretical framework, (2) HTA reports, (3) parameter designs or suitable indicators, and (4) economic or budgetary impact assessments. This review shows that evaluation of the environmental impact of HTAs is still very incipient. Small steps are being taken in EE, such as carbon footprint estimations from a life-cycle approach of technologies and the entire care pathway.

Use of Telehealth Among Cancer Survivors: A Scoping Review.

Background: Long-term cancer survivors have specific needs that are frequently neglected. Telehealth, as a new form of health care, can benefit this growing population. Objective: To identify, analyze, and synthesize the existing evidence on the use of telehealth in the care of cancer survivors after the end of treatment. Methods: A scoping review was conducted in the databases PubMed, CINAHL, COCHRANE, SCIELO, DIALNET, and LILACS and reference institutions in cancer. Results: The initial search yielded 406 publications with 59 articles meeting the eligibility criteria. There are different types of telehealth (video calls, phone calls, websites, mobile applications, and short message services) used for the care of cancer survivors. Most telehealth interventions focus on improving the physical and mental spheres of quality of life in the extended survival phase (from 1 to 3 years postdiagnosis), with only two articles (3%) on long-term cancer survivors (>5 years postdiagnosis). Survivors are satisfied with telehealth interventions, noting the importance of improving comprehensibility, personalization of the platforms, and the lack of excessive information included. Conclusions: Telehealth is a feasible modality for cancer survival care. The scarcity of interventions aimed at long-term survivors stands out, as does the general neglect of the social and spiritual spheres of quality of life. Implications for Practice: Telehealth platforms must adapt their content, format, and items to the preferences reported by the survivors.

Analysis of mental health effects among nurses working during the COVID-19 pandemic: A systematic review.

WHAT IS KNOWN ON THE SUBJECT?: Working on the frontline during the pandemic has had a negative impact on the mental health of health professionals. A significant proportion experienced anxiety, insomnia, posttraumatic stress or depression. WHAT THE PAPER ADDS TO EXISTING KNOWLEDGE?: Analysis and synthesis of the evidence of the impact of the COVID-19 pandemic on the mental health of nurses based on their work context. There exists a gap in the literature as no studies were found that analysed the effects on nurses' mental health according to the level of care they worked in (hospital-primary care-nursing home). WHAT ARE THE IMPLICATIONS FOR PRACTICE?: There is an urgent need to assess and respond to the impact of COVID-19 on the physical and mental well-being of nurses, and to monitor international policies for the improvement of nurses' working conditions. ABSTRACT: Introduction Health professionals have suffered negative consequences during the COVID-19 pandemic. No review has specifically addressed the impact of the pandemic on the mental health of nurses exclusively according to the work context. Aim To analyse the impact of the COVID-19 pandemic on the mental health of nurses who have worked in hospitals, primary care centres and social health centres. Method PubMed, CINAHL, PsychINFO and Cochrane databases were searched (Prospero number: CRD42021249513). Out of 706 papers, 31 studies (2020-2021) were included in the systematic review. A qualitative synthesis method was used to analyse the data. Results Most studies were conducted in hospitals or frontline settings. The prevalence of moderate-to-severe symptoms was for anxiety 29.55%, depression 38.79%, posttraumatic stress disorder 29.8%, and insomnia 40.66%. Discussion This review highlights the mental health effects among nurses working in acute hospital settings. It also evidences a data gap on mental health effects among nurses working in primary health care and in nursing homes. Implications for practice In the post phase of the pandemic, there is an urgent need to assess and respond to the impact on the mental well-being of nurses, and to monitor international policies for the improvement of nurses' working conditions.

Telemedicine for neurological diseases: A systematic review and meta-analysis.

BACKGROUND AND PURPOSE: The aim was to systematically review the effectiveness and safety of telemedicine combined with usual care (in-person visits) compared to usual care for the therapeutic management and follow-up assessment of neurological diseases. METHODS: The electronic databases MEDLINE, Embase, Web of Science and Cochrane Central Register of Controlled Trials were searched (June 2021). Randomized controlled trials (RCTs) on patients of any age with neurological diseases were considered. Two reviewers screened and abstracted data in duplicate and independently and assessed risk of bias using the Cochrane risk-of-bias tool for randomized trials (RoB 2). When possible, pooled effect estimates were calculated. RESULTS: Of a total of 3018 records initially retrieved, 25 RCTs (n = 2335) were included: 11 (n = 804) on stroke, four (n = 520) on Parkinson's disease, three (n = 110) on multiple sclerosis, two (n = 320) on epilepsy, one (n = 63) on dementia, one (n = 23) on spina bifida, one (n = 40) on migraine, one (n = 22) on cerebral palsy and one (n = 433) on brain damage. Types of telemedicine assessed were online visits (11 studies), tele-rehabilitation (seven studies), telephone calls (three), smartphone apps (two) and online computer software (two). The evidence was quite limited except for stroke. Compared to usual care alone, telemedicine plus usual care was found to improve depressive symptoms, functional status, motor function, executive function, generic quality of life, healthcare utilization and healthy lifestyle in patients in post-stroke follow-up. CONCLUSIONS: Well-designed and executed RCTs are needed to confirm our findings on stroke and to have more scientific evidence available for the other neurological diseases.

Water, environment, and socioeconomic justice in California: A multi-benefit cropland repurposing framework.

Low-income, rural frontline communities of California's Central Valley experience environmental and socioeconomic injustice, water insecurity, extremely poor air quality, and lack of fundamental infrastructure (sewage, green areas, health services), which makes them less resilient. Many communities depend financially on agriculture, while water scarcity and associated policy may trigger farmland retirement further hindering socioeconomic opportunities. Here we propose a multi-benefit framework to repurpose cropland in buffers inside and around (400-m and 1600-m buffers) 154 rural disadvantaged communities of the Central Valley to promote socioeconomic opportunities, environmental benefits, and business diversification. We estimate the potential for (1) reductions in water and pesticide use, nitrogen leaching, and nitrogen gas emissions, (2) managed aquifer recharge, and (3) economic and employment impacts associated with clean industries and solar energy. Retiring cropland within 1600-m buffers can result in reductions in water use of 2.18 km3/year, nitrate leaching into local aquifers of 105,500 t/year, greenhouse gas emissions of 2,232,000 t CO2-equivalent/year, and 5388 t pesticides/year, with accompanying losses in agricultural revenue of US$4213 million/year and employment of 25,682 positions. Buffer repurposing investments of US$27 million/year per community for ten years show potential to generate US$101 million/year per community (total US$15,578 million/year) for 30 years and 407 new jobs/year (total 62,697 jobs/year) paying 67 % more than prior farmworker jobs. In the San Joaquin Valley (southern Central Valley), where groundwater overdraft averages 2.3 km3/year, potential water use reduction is 1.8 km3/year. We have identified 99 communities with surficial soils adequate for aquifer recharge and canals/rivers within 1600 m. This demonstrates the potential of managed aquifer recharge in buffered zones to substantially reduce overdraft. The buffers framework shows that well-planned land repurposing near disadvantaged communities can create multiple benefits for farmers and industry stakeholders, while improving quality of life in disadvantaged communities and producing positive externalities for society.

Mortalidad en niños con bacteriemia por Burkholderia cepacia: una revisión sistemática / Mortality in children with bacteremia due to Burkholderia cepacia: a systematic review

INTRODUCCIÓN: En los niños, la bacteriemia debida a Burkholderia cepacia, es considerada una complicación grave y conducente a una elevada mortalidad. Con el objetivo de conocer la mortalidad asociada a esa condición, se realizó una revisión sistemática de la literatura médica. MATERIAL Y MÉTODOS: Se aplicó una estrategia de búsqueda bibliográfica con las palabras claves: "bacteriemia por B. cepacia", "humanos", "niños" y "adolescentes", como únicos filtros. Se informan la mediana y los valores intercuartílicos de la frecuencia de la mortalidad reportada por los estudios incluidos. RESULTADOS: Se identificaron 92 estudios potencialmente útiles. De ellos, se descartaron 81, incluyéndose finalmente, 11 estudios. Se trató de descripciones retrospectivas de casos, salvo uno de ellos, que respondió a un diseño analítico caso-control. La mediana de la mortalidad reportada por esta revisión, fue 0 (Q25 = 0 y Q75 = 28,57%). INTERPRETACIÓN: Si bien la evidencia disponible es escasa y de baja calidad, sugiere que el curso clínico de esta afección no siempre resulta en una elevada mortalidad.

BACKGROUND: Bacteremia due to Burkolderia cepacia in children is considered a severe complication and associated with high mortality incidence. In order to know the level of mortality associated with it, this systematic review of the literature was carried out. METHODS: A search strategy was carried out with the keywords: "bacteremia by B cepacia and human" and "children" and "adolescents" as filters. Global frequency of mortality reported by the included studies was calculated and informed as median (Q2) and its interquartile values (Q1 and Q3). RESULTS: The search identified 92 potentially useful studies. Of these, 81 were discarded, and then remained 11 studies to be included. One out of 11 studies is an analytic case-control design. Rest are retrospective case series. Related mortality median was 0 (Q25 = 0 and Q75 = 28,57%). CONCLUSION: Although the available evidence is scarce and of low quality, it suggests that clinical course of this condition does not always lead to high mortality rates.

Caco-2 Cell Response Induced by Peptides Released after Digestion of Heat-Treated Egg White Proteins.

The heat treatment of food proteins induces structural modifications that influence their interaction with human fluids and cells. We aimed to evaluate the Caco-2 cell response induced by peptides produced after digestion of heat-treated egg white proteins. In vitro digestion of ovalbumin (OVA), ovomucoid (OM), and lysozyme (LYS), untreated or previously heated, was performed. The digestibility of proteins and the response of Caco-2 cells exposed to peptides (<10 kDa) generated during digestion were evaluated. Intact OVA and LYS persisted after the digestion of native proteins, whereas OM was completely hydrolysed. A heat treatment at 65 °C for 30 min did not alter the digestibility of OVA, whereas at 90 °C for 3 min, protein degradation was favoured. The digestibility of OM and LYS was not affected by heat treatment. Peptides derived from OVA and OM digestion induced IL-6 and IL-8 production. OVA and LYS digestion promoted the expression of Tslp, and Il6 and Il33, respectively. A heat treatment prior to OVA digestion reduced IL-6 production and Tslp expression. It was concluded that heat treatments can reduce the release of OVA-derived peptides, but not OM and LYS, with proinflammatory activity during digestion.

Analysis and Screening of Commercialized Protein Supplements for Sports Practice.

Recent years have seen a rise in the popularity of the consumption of sports-related supplements. However, the hypothesis is raised that it is necessary to analyze the quality aspects of these supplements in relation to the information provided on the label, to avoid associated risks and obtain the greatest possible benefit from their consumption. Therefore, the aim of this study has been to carry out an analysis or screening of the protein supplements that are currently marketed in Spain. We analyzed the labels of 52 protein sports supplements available both in physical stores and online. The analysis consisted of addressing three relevant aspects considering the labeling: (a) the legislative framework in which the supplements are marketed, (b) the quality of the protein, and (c) the presence of other ingredients according to the specifications of the label. In the legislative context, there do not seem to be any specific regulations to guarantee consumer protection, which can lead to unfair practices and misleading advertising. Most of the supplements analyzed to comply with the requirements of their current regulations. However, claims about their benefits that are not allowed under European legislation have been found in some of them. Regarding composition and according to label information, the supplements have been found to provide a sufficient dose of protein in terms of recommended protein intake per serving. Regarding the presence of other ingredients and according to the information on the label, most of them, except for egg supplements, contain other ingredients. Colostrum was also found in one of the supplements evaluated. The conclusions of the study reveal that, due to a lack of knowledge or misleading advertising practices, supplements are often not used properly. The information provided is essential for both professionals and consumers to avoid the risks associated with consumption, such as unintentional doping, interactions between ingredients that reduce the quality of the supplement, and consumption of supplements inappropriately, among others.