Stress response and adaptation mechanisms in Kluyveromyces marxianus.

Kluyveromyces marxianus is a non-Saccharomyces yeast that has gained importance due to its great potential to be used in the food and biotechnology industries. In general, K. marxianus is a known yeast for its ability to assimilate hexoses and pentoses; even this yeast can grow in disaccharides such as sucrose and lactose and polysaccharides such as agave fructans. Otherwise, K. marxianus is an excellent microorganism to produce metabolites of biotechnological interest, such as enzymes, ethanol, aroma compounds, organic acids, and single-cell proteins. However, several studies highlighted the metabolic trait variations among the K. marxianus strains, suggesting genetic diversity within the species that determines its metabolic functions; this diversity can be attributed to its high adaptation capacity against stressful environments. The outstanding metabolic characteristics of K. marxianus have motivated this yeast to be a study model to evaluate its easy adaptability to several environments. This chapter will discuss overview characteristics and applications of K. marxianus and recent insights into the stress response and adaptation mechanisms used by this non-Saccharomyces yeast.

Impact of follow-up adherence on disease activity in childhood-onset systemic lupus erythematosus (cSLE).

BACKGROUND/PURPOSE: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, with a potential for significant disease damage, morbidity, and mortality. In comparison to the adult population, childhood-onset SLE (cSLE) tends to be more aggressive given the higher preponderance of renal and neuropsychiatric disease and increased disease activity. There is a paucity of literature examining relationship between disease activity, rheumatology follow-up visits, and health care utilization. The objective of this study is to determine whether adherence with outpatient clinic visits would affect disease activity in patients with childhood-onset systemic lupus erythematosus (cSLE). METHODS: 41 children <18 years of age at time of diagnosis with SLE who met Systemic Lupus International Collaborative Clinics (SLICC) criteria and not evaluated in clinic within the previous 120-day period were identified as eligible for inclusion. Patients were continuously searched between December 2021 and July 2022 for eligibility evaluation. Through retrospective chart review, we assessed disease activity (SLE Disease Activity Index) at the last clinic visit. The patients were stratified into two cohorts of lower and higher disease activity, with SLE disease activity index (SLEDAI) ≤ 3 and SLEDAI ≥ 4, respectively. Descriptive statistics and Willcox Rank Sum (numerical variables) and Fisher's test (categorical variables) were used to compare these two groups. RESULTS: Clinical, epidemiological, and serological data were compared between the two groups, with observed statistically significant differences to include current use of high dose prednisone associated with higher SLEDAI scores (p = 0.019). In nonparametric analysis, time to follow-up (p < 0.001), hospitalizations (p = 0.017), and Emergency Department visits (ED) (p < 0.001) were found to be associated with higher SLEDAI scores. CONCLUSION: Our findings suggest that cSLE patients with higher disease activity are at risk for increased health care utilization with respect to ED visits as well as hospitalizations in the setting of follow-up nonadherence. While further studies are required to enhance our understanding of this association, this links the importance of disease-related outcome and routine outpatient visits in this particularly vulnerable patient population.

Distinguishing immune activation and inflammatory signatures of multisystem inflammatory syndrome in children (MIS-C) versus hemophagocytic lymphohistiocytosis (HLH).

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a potentially life-threatening sequela of severe acute respiratory syndrome coronavirus 2 infection characterized by hyperinflammation and multiorgan dysfunction. Although hyperinflammation is a prominent manifestation of MIS-C, there is limited understanding of how the inflammatory state of MIS-C differs from that of well-characterized hyperinflammatory syndromes such as hemophagocytic lymphohistiocytosis (HLH). OBJECTIVES: We sought to compare the qualitative and quantitative inflammatory profile differences between patients with MIS-C, coronavirus disease 2019, and HLH. METHODS: Clinical data abstraction from patient charts, T-cell immunophenotyping, and multiplex cytokine and chemokine profiling were performed for patients with MIS-C, patients with coronavirus disease 2019, and patients with HLH. RESULTS: We found that both patients with MIS-C and patients with HLH showed robust T-cell activation, markers of senescence, and exhaustion along with elevated TH1 and proinflammatory cytokines such as IFN-γ, C-X-C motif chemokine ligand 9, and C-X-C motif chemokine ligand 10. In comparison, the amplitude of T-cell activation and the levels of cytokines/chemokines were higher in patients with HLH when compared with patients with MIS-C. Distinguishing inflammatory features of MIS-C included elevation in TH2 inflammatory cytokines such as IL-4 and IL-13 and cytokine mediators of angiogenesis, vascular injury, and tissue repair such as vascular endothelial growth factor A and platelet-derived growth factor. Immune activation and hypercytokinemia in MIS-C resolved at follow-up. In addition, when these immune parameters were correlated with clinical parameters, CD8+ T-cell activation correlated with cardiac dysfunction parameters such as B-type natriuretic peptide and troponin and inversely correlated with platelet count. CONCLUSIONS: Overall, this study characterizes unique and overlapping immunologic features that help to define the hyperinflammation associated with MIS-C versus HLH.

Quality of life measures and physical activity in childhood systemic lupus erythematosus.

Childhood systemic lupus erythematosus (cSLE) is a life-long disease with significant morbidity and mortality, and with associated significant impact on health-related quality of life (HRQOL). Previous literature supports that physical activity has positive impact on HRQOL in patients with chronic diseases, including cSLE. We sought to describe the physical activity of our patients with cSLE and determine the relationship between physical activity, SLE activity, treatment modalities and HRQOL in cSLE. Children ≤18 years of age with cSLE and their parents were enrolled and completed corresponding child and parent Simple Measure of Impact of Lupus Erythematosus in Youngsters© reports (cSMILEY© and pSMILEY©, respectively), and the Physical Activity Questionnaire for Children (PAQ-C) or Adolescents (PAQ-A). Through retrospective chart review, we assessed the SLE Disease Activity Index (SLEDAI) using the SLEDAI-2K assessment tool. Descriptive statistics as well as Pearson's correlation coefficients were performed with the data obtained. Forty-four children and their parents were enrolled; clinical data, SMILEY© and PAQ-C or PAQ-A scores of cSLE subjects were evaluated. The most frequently reported physical activity modality was walking (61.3%), with mean frequency of 3.7 ± 1.8 days a week, and a median of 3.5 days a week. Although there was no correlation noted between treatment modalities and PAQ-C/PAQ-A, there was weak correlation between SLEDAI and PAQ-C/PAQ-A (Pearson correlation= 0.2, ρ = 0.1, p = 0.9, n = 44). There was a weak correlation between SMILEY total score and PAQ [cSMILEY© and PAQ-C/PAQ-A combined cohorts (Pearson correlation = 0.2, ρ = 0.3, p = 0.07, n = 44), and modest correlation between pSMILEY© scores and PAQ-C/PAQ-A combined cohorts (Pearson correlation = 0.3, ρ = 0.3, p = 0.05, n = 44)]. Our study emphasizes the need for larger samples to understand the prognostic value of activity levels and the extent to which increasing physical activity might be linked to improvements in HRQOL in this vulnerable population.

Incidence and risk factors for mortality in patients treated with combined ceftaroline for Gram-positive infective endocarditis.

BACKGROUND: Ceftaroline is a fifth-generation cephalosporin and represents an alternative in the treatment of infective endocarditis (IE). The main objective of this study was to describe the incidence of in-hospital and 42-day mortality in patients with IE treated with ceftaroline. METHODS: An observational retrospective study included adult patients with IE admitted during a 3.5-year period (January 2018-June 2021) and treated with ceftaroline in a single center. All cases were definite or possible IE according to the modified Duke criteria. RESULTS: Seventy cases were analyzed. The mean age was 67.35 ± 16.62 (16-89) and 39 (55.7%) were males. The mean number of days of treatment with ceftaroline was 21.26 ± 16.17 (1-75). Overall mortality at 42 days was 30%, 20.7% in the first line, and 36.6% in rescue therapy. Predictors of 42 days-mortality were increased Charlson comorbidity index (CCI) (OR of 1.7 per 1 point increment, 95% CI 1.2-2.4, P 0.001), presence of methicillin-resistance (OR 6.8, 95% CI 1.3-36.8, P 0.026) and evidence of septic shock (OR 8.6 95% CI 1.7-44.2, P 0.01). Predictors of 42 days of therapeutic failure were the increase in the CCI (OR of 1.6 per 1 point increment, 95% CI 1.3-2.1, P 0.000) and septic shock (OR 4.5 95% CI 1.1-18 P 0.036). Adverse effects were described in 6/70 (8.6%) of the patients, precipitating in 4/70 (5.7%) the definitive withdrawal of the antibiotic. CONCLUSIONS: The incidence of in-hospital and 42 day-mortality of IE patients treated with ceftaroline remains similar to literature data. Increased CCI, septic shock, and methicillin resistance are associated with poor prognosis.

Alendronate after denosumab discontinuation in women previously exposed to bisphosphonates was not effective in preventing the risk of spontaneous multiple vertebral fractures: two case reports.

At denosumab discontinuation, an antiresorptive agent is indicated to reduce the high bone turnover, the rapid bone loss, and the risk of spontaneous vertebral fractures. We report two cases of postmenopausal women, previously exposed to bisphosphonates, treated with alendronate at denosumab discontinuation. Alendronate was ineffective to avoid spontaneous clinical vertebral fractures. They presented three and nine spontaneous vertebral fractures 8 and 12 months after denosumab discontinuation, respectively. Ineffectiveness of alendronate was attributed to insufficient control of the rebound as assessed by B-crosslaps measures in the first case, and partially to the high risk of fractures in the later. In both situations, the increased fracture risk may have favoured these new fractures. It is urgent to define effective therapeutic strategies to avoid spontaneous vertebral fractures after denosumab discontinuation.

Is a brief self-report version of the Columbia severity scale useful for screening suicidal ideation in Chilean adolescents?

BACKGROUND: Given the high rates of suicide in the adolescent population and the reluctance of this population to seek help, developing proactive and effective strategies to timely detect individuals at high risk for suicide in non-clinical contexts is a worldwide recognized need. A series of brief self-report questionnaires have been developed for this purpose, however there are few studies providing evidence on their capability to accurately classify suicidal risk levels in specific populations. One of the instruments frequently used to evaluate suicide risk is the Columbia-Suicide Severity Rating Scales (C-SSRS). The goal of this study is to provide psychometric evidence about the accuracy of the Suicidal Ideation subscale (SI) of the C-SSRS to classify suicidal risk levels in a sample of Chilean adolescents using Item Response Theory (IRT). METHODS AND MATERIALS: Through the two parameter logistic model (2-PLM), we analyzed the capability of a self-report questionnaire addressing suicidal ideation (SI) to differentiate and classify participants according to their SI severity levels. We tested two main parameters: difficulty (localization) and discriminating power of 6 items extracted and adapted from the Columbia-Suicide Severity Rating Scales (C-SSRS). We administered this questionnaire to a general sample of 1645 adolescents aged 13 to 18. RESULTS: Our results show that the items differentiate symptoms addressing suicidal thoughts according to their severity, providing an accurate classification of the SI risk level. CONCLUSIONS: These findings support the usage of the C-SSRS in Chilean adolescents. Further research is needed to test its predictive value in different populations.

The number of biopsied trophectoderm cells may affect pregnancy outcomes.

OBJECTIVE: To study if the number of trophectoderm (TE) biopsied cells has an impact on implantation rates. DESIGN: A retrospective cohort study in a single-center study. SETTING: In vitro fertilization center. PATIENTS: Patients who underwent PGT-A from January 2013 to March 2016. In total, 482 vitrified/warmed single embryo transfers were included. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Clinical pregnancies rate, implantation rate. RESULTS: Overall, clinical pregnancies per embryo transfer were higher when a regular TE were biopsied compared to larger size biopsy cells (66% (175/267) vs 53% (115/215) (p < 0.005) respectively). Pregnancy rates were also analyzed according to embryo morphology at the moment of embryo biopsy, when a good-quality embryo was transferred the clinical outcome was 75% (81/108) in group 1 and 61% (60/99) in group 2 (p < 0.05). Data was also stratified by age in patients ≤ 35 years and > 35 years. The clinical pregnancy was 67% (51/76) in women ≤ 35 years and 65% (124/191) in women > 35 years when a regular size biopsy was performed. These results significantly reduced when a larger size biopsy was performed 54% (49/91) and 53% (66/124), respectively (p < 0.05). Further investigation indicated that miscarriage rate was similar between these groups (4% (7/182) in group 1 and 5% (6/121) in group 2). CONCLUSIONS: These findings underscore that when a large amount of TE cells are biopsied, it may negatively affect implantation rates, but once implanted, the embryos have the same chance to miscarry or reach term.

Therapeutic potential of Takeda-G-protein-receptor-5 (TGR5) agonists. Hope or hype?

The gastrointestinal tract regulates glucose and energy metabolism, and there is increasing recognition that bile acids function as key signalling molecules in these processes. For example, bile acid changes that occur after bariatric surgery have been implicated in the effects on satiety, lipid and cholesterol regulation, glucose and energy metabolism, and the gut microbiome. In recent years, Takeda-G-protein-receptor-5 (TGR5), a bile acid receptor found in widely dispersed tissues, has been the target of significant drug discovery efforts in the hope of identifying effective treatments for metabolic diseases including type 2 diabetes, obesity, atherosclerosis, fatty liver disease and cancer. Although the benefits of targeting the TGR5 receptor are potentially great, drug development work to date has identified risks that include histopathological changes, tumorigenesis, gender differences, and questions about the translation of animal data to humans. The present article reviews the noteworthy challenges that must be addressed along the path of development of a safe and effective TGR5 agonist therapy.

Glucose and lipid effects of the ileal apical sodium-dependent bile acid transporter inhibitor GSK2330672: double-blind randomized trials with type 2 diabetes subjects taking metformin.

AIMS: To investigate the pharmacodynamics, pharmacokinetics and safety/tolerability of blocking reuptake of bile acids using the inhibitor GSK2330672 (GSK672) in patients with type 2 diabetes (T2D). METHODS: Subjects with T2D taking metformin were enrolled in two studies in which they took metformin 850 mg twice daily for 2 weeks prior to and during the randomized treatment periods. In the first crossover study (n = 15), subjects received GSK672 45 mg, escalating to 90 mg, twice daily, or placebo for 7 days. The second parallel-group study (n = 75) investigated GSK672 10-90 mg twice daily, placebo or sitagliptin for 14 days. RESULTS: In both studies, GSK672 reduced circulating bile acids and increased serum 7-α-hydroxy-4-cholesten-3-one (C4), an intermediate in the hepatic synthesis of bile acids. Compared with placebo, in the parallel-group study 90 mg GSK672 twice daily reduced fasting plasma glucose [FPG; -1.21 mmol/l; 95% confidence interval (CI) -2.14, -0.28] and weighted-mean glucose area under the curve (AUC)0-24 h (-1.33 mmol/l; 95% CI -2.30, -0.36), as well as fasting and weighted-mean insulin AUC0 -24 h . GSK672 also reduced cholesterol (LDL, non-HDL and total cholesterol) and apolipoprotein B concentrations; the maximum LDL cholesterol reduction was ∼40%. There was no change in HDL cholesterol but there was a trend towards increased fasting triglyceride levels in the GSK672 groups compared with placebo. In both studies, the most common adverse events associated with GSK672 were gastrointestinal, mostly diarrhoea (22-100%), which appeared to be independent of dose. CONCLUSIONS: In subjects with T2D on metformin, GSK672 improved glucose and lipids, but there was a high incidence of gastrointestinal adverse events.

Effect of gamma irradiation on phenol content, antioxidant activity and biological activity of black maca and red maca extracts (Lepidium meyenii walp).

This study was performed to determine the effects of gamma irradiation on UV spectrum on maca, total content of polyphenols, 1,1-diphenyl-2-picrylhydrazyl (DPPH) radical-scavenging activities and in vivo biological activities of red and black maca extracts (Lepidium meyenii). Adult mice of the strain Swiss aged 3 months and weighing 30-35 g in average were used to determine biological activities. Daily sperm production, effect on testosterone-induced prostate hyperplasia and forced swimming test were used to determine the effect of irradiation on biological activities of maca extracts. Irradiation did not show differences in UV spectrum but improves the amount of total polyphenols in red maca as well as in black maca extracts. In both cases, black maca extract has more content of polyphenols than red maca extract (p < 0.01). Gamma irradiation significantly increased the antioxidant capacity (p < 0.05). No difference was observed in daily sperm production when irradiated and nonirradiated maca extract were administered to mice (p > 0.05). Black maca extract but not red maca extract has more swimming endurance capacity in the forced swimming test. Irradiation of black maca extract increased the swimming time to exhaustion (p < 0.05). This is not observed with red maca extract (p > 0.05). Testosterone enanthate (TE) increased significantly the ventral prostate weight. Administration of red maca extract in animals treated with TE prevented the increase in prostate weight. Irradiation did not modify effect of red maca extract on prostate weight (p > 0.05). In conclusion, irradiation does not alter the biological activities of both black maca and red maca extracts. It prevents the presence of microorganisms in the extracts of black or red maca, but the biological activities were maintained.

[Peripartum cardiomyopathy: a case report]. / Miocardiopatía periparto: reporte de un caso.

Background: Peripartum cardiomyopathy also known as cardiomyopathy associated with pregnancy, is rarely a cause of heart failure, it affects pregnant or puerperal women in the first 5 months. Although the first case reported was in 1849, it was recognized until 1930. In 2010 the European Society of Cardiology Working Group on peripartum cardiomyopathy, defined this pathology as an idiopathic cardiomyopathy that affects pregnant women between the third trimester and five months after delivery. Characterized by a left ventricular failure with an ejection fraction of ≤45% and an end-diastolic dimension ≥2.7 cm/m2 , in absence of an identifiable cause of heart failure. Case report: We report a case of a 39-year-old patient, diagnosed with a peripartum cardiomyopathy in the early puerperium, characterized by hypertension, tachycardia, dyspnea and oxygen desaturation. The transesophageal echocardiogram reported heart failure, a hypokinetic left ventricle and a ventricular failure with an ejection fraction <40%. We could not identify an other cause to justify heart failure. Multidisciplinary management was administered successfully. Conclusion: The importance of this article relies in the fact that eripartum cardiomyopathy has a high morbidity and mortality. The impact of this pathology is unknow in our country. Here we establish and discuss the multidisciplinary management held in our hospital with this specific patient in order to improve the prognosis on future occasions.

[Salpingectomy in ovarian cancer prevention: Evidence behind the hypothesis and surgical implications]. / Salpingectomía como opción de reducción de riesgo de cáncer de ovario.

Background: Over the last decade, evidence suggests the fallopian tubes are the origin of most of the high grade ovarian serous carcinomas. This type of carcinoma represents at least 50% of all the cases of epithelial ovarian cancer. Salpingectomy may lower the risk of high grade serous carcinoma. Removing the two fallopian tubes should be considered a strategy for risk reduction in patients who decide tubal sterilization or in patients with hysterectomy for benign disease. There are ongoing protocols that evaluate the ovarian hormonal production impact after prophilactic salpingectomy. In patients with BRCA1 and BRCA2 mutations, salpingo-oophorectomy is recommended usually between 35 to 40 years of age for BRCA 1 and between 40 and 45 years of age for BRCA 2. The oopherectomy done whithin these decades has the consequences and side effects of premature menopause, some physicians have suggested doing a two step procedure: perform a salpingectomy as soon as the patient has decided to have permanent birth control, and doing the ophoorectomy at the onset of menopause. The oncological safety of this approach is still under evaluation and is not recommended outside a protocol.

GSK2374697, a long duration glucagon-like peptide-1 (GLP-1) receptor agonist, reduces postprandial circulating endogenous total GLP-1 and peptide YY in healthy subjects.

We investigated the effects of a long-duration glucagon-like peptide-1 (GLP-1) receptor agonist, GSK2374697, on postprandial endogenous total GLP-1 and peptide YY (PYY). Two cohorts of healthy subjects, one normal/overweight and one obese, were randomized to receive GSK2374697 2 mg (n = 8 each) or placebo (n = 4 and n = 2) subcutaneously on days 1, 4 and 7. Samples for plasma endogenous GLP-1 and PYY were collected after breakfast on days -1 and 12. Weighted mean area under the curve (0-4 h) of total GLP-1 and PYY in treated subjects was reduced compared with placebo. The least squares mean difference for change from baseline was -1.24 pmol/l [95% confidence interval (CI) -2.33, -0.16] and -4.47 pmol/l (95% CI -8.74, -0.20) for total GLP-1 and PYY, respectively, in normal/overweight subjects (p < 0.05 for both), and -1.56 (95% CI -2.95, -0.16) and -3.02 (95% CI -8.58, 2.55), respectively, in obese subjects (p < 0.05 for GLP-1). In healthy subjects, GSK2374697 reduced postprandial total GLP-1 and PYY levels, suggesting feedback suppression of enteroendocrine L-cell secretion of these peptides.

Dalbavancin as consolidation therapy for infective endocarditis in patients with comorbidity. A real world experience.

OBJECTIVE: Infective endocarditis (IE) is a potentially life-threatening infection, the incidence of which has in creased in recent decades, particularly among elderly patients with comorbidity. The primary objective of this study was to evaluate the effectiveness of dalbavancin in the consolidation therapy of IE in patients with comorbidity six months after the end of treatment (EOT). METHODS: An observational and retrospective study was conducted on patients with a Charlson Comorbidity Index (CCI) ≥ 3 who were diagnosed with IE and received consolidation therapy with dalbavancin. RESULTS: Forty-eight patients were included, 58.3% were male, mean age of 76.2 years (IQR: 66-88), and a mean age adjusted CCI of 6.5 (IQR: 5-7.5). Definite IE was diagnosed in 77% of cases. The most frequently isolated microorganisms were Staphylococcus aureus (45.8%) followed by Enterococcus spp. (31.3%). Complications of IE were observed in 67.7% of cases, and cardiac surgery was performed in 27% of patients. The primary reason for using dalbavancin was outpatient parenteral antibiotic therapy in 85.4% of cases. The effectiveness at EOT was 93.8%. At six months, six IE-related deaths, four unrelated deaths, and two IE relapses were observed. The effectiveness was 77%. Adverse effects related to DBV were reported in 4.2% of cases, of which 2% were considered serious. CONCLUSIONS: Dalbavancin has proven to be an effective alternative as consolidation antibiotherapy for IE in elderly patients with comorbidity. Moreover, a very favorable safety profile with few associated adverse effects has been observed in this population.

Improving Hepatitis B Screening Prior to Rituximab: A Quality Improvement Project.

Rituximab, used in the treatment of some rheumatic and kidney diseases, can lead to hepatitis B virus (HBV) reactivation; HBV screening is recommended for those starting this medication. We aimed to improve by 50% the proportion of patients undergoing HBV screening by implementing multimodal interventions to support clinicians in this evidence-based practice. We conducted a quality improvement project from November 2020 to June 2022 at a tertiary care pediatric hospital system, including patients with rheumatic and/or kidney diseases starting rituximab. Multimodal interventions targeting clinicians included electronic health tools (dot phrase, display of screening recommendations and screening results in rituximab order sets/therapy plans), educational meetings, and e-mail/paper reminders. The primary outcome was the proportion of patients with complete HBV screening, while the secondary outcome was utilization of each laboratory component, tracked using statistical process control charts. Pre- and post-intervention data were compared using Fisher's test. One hundred eighty-two patients who had been prescribed rituximab were included, of which 98 (54%) were post-intervention. The proportions of patients undergoing complete HBV screening (6% vs. 44%; p < 0.001), HBsAg collection (60% vs. 79%; p = 0.006), anti-HBsAb collection (14% vs. 54%; p < 0.001), and total anti-HBcAb collection (8% vs. 52%; p < 0.001) were significantly higher in the post-intervention period. Improvement was sustained over 18 months, with shifts and/or data points above the control limits in all measures. Forty-five patients were HBV-non-immune. In this study, multimodal interventions including electronic health tools and education of the provider significantly increased the proportion of patients screened for HBV prior to rituximab and identified immunization opportunities.

Multicenter study. Nursing professionals' perception of training needs in pain assessment in pediatric patients with cognitive dysfunction.

INTRODUCTION: Pediatric patients with cognitive dysfunction are at greater risk of pain than typically developing children. Pain assessment in these patients is complex and could generate uncertainty in health professionals about what the key aspects are. AIM: To determine the training needs perceived by nursing professionals regarding acute pain assessment in pediatric patients with cognitive dysfunction. METHODS: A descriptive, cross-sectional, and multicenter study was performed using a survey addressed to nursing professionals who work in pediatrics during the months of August and September 2022. RESULTS: 163 responses were obtained. Most of the professionals who responded were female (92.6%, n = 151), with a mean age of 38.98 ±â€¯10.40 years. The most frequent work unit was the pediatric intensive care unit (PICU), in 36% (n = 58). Most of the participants reported not having previously received training on pain assessment in pediatric patients with cognitive disabilities (85.9%, n = 139). However, 70.4% (n = 114) considered it "very necessary" for the development of their work to receive specific training on this topic. Knowing how to assess acute pain in this population (85.3%, n = 139) and knowing the clinical and behavioral manifestations of pain in this type of patient (84.7%, n = 138) were the aspects that obtained higher scores. CONCLUSION: This research notes more than 90% of participants consider "quite necessary" and "strong necessary" to be training in pediatric cognitive dysfunction patients pain assessment. Furthermore, work experience, academic education and to be pediatric specialist obtain statistical significance data.

Successful Medical-Surgical Management of Intracardiac Thrombosis and Pulmonary Pseudoaneurysms in an Adolescent With Hughes-Stovin Syndrome.

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.

Identifying self-report measures of emotion regulation and evaluating their psychometric properties: a protocol for a systematic review.

INTRODUCTION: Successful emotion regulation (ER) is critical for psychological health. Disturbances in this ability are associated with several psychiatric disorders. There are several self-report questionnaires to assess ER. However, there are no studies synthesising the evidence on their psychometric properties. We aim to identify all available instruments addressing ER in adolescents or adults and to critically appraise, compare and summarise the quality of their psychometric properties. For this, we will use COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) criteria. METHOD AND ANALYSIS: The search process to identify eligible studies will be conducted in April 2021 including the ProQuest, PubMed, Scopus and Web of Science databases with no restriction in terms of publication date. Eligibility criteria include peer-reviewed research articles written in English or Spanish by means of patient-reported outcome measures focused on ER among participants of 13 years or older. We will assess the quality of measures according to the COSMIN Risk of Bias Checklist. The psychometric properties will be assessed by the COSMIN updated criteria for good measurement. The available evidence will be addressed by the Grading of Recommendations, Assessment, Development and Evaluations approach. Our findings will be synthesised independently for each measure, including information on their sample, theoretical model and psychometric properties when possible. ETHICS AND DISSEMINATION: Ethical approval is not required, as this study does not involve any participants or collection of primary data. Results are expected to be published in a peer-reviewed journal in the field of youth mental health and presented at relevant meetings and conferences. PROSPERO REGISTRATION NUMBER: CRD42021249498.

Severe Immediate and Delayed Hypersensitivity Reactions to Biologics in a Toddler With Systemic Juvenile Idiopathic Arthritis.

Many pediatric rheumatic diseases can be safely managed with biologic therapy. Severe allergic reactions to these medications are uncommon. We report the case of a 2-year-old male with systemic-onset juvenile idiopathic arthritis and secondary macrophage activation syndrome (MAS), whose treatment was complicated by severe allergic reactions to biologics, including drug reaction with eosinophilia and systemic symptoms (DRESS)/drug-induced hypersensitivity reaction (DIHR) likely due to anakinra, and anaphylactoid reaction to intravenous tocilizumab. These required transition to canakinumab, cyclosporine, and corticosteroids, with later development of interstitial lung disease and MAS flare needing transition from canakinumab to tofacitinib, which led to disease control. Whether lung disease is a manifestation of DRESS/DIHR to canakinumab remains unclear. High index of suspicion of hypersensitivity reactions for timely diagnosis and drug discontinuation is critical, especially in patients with active disease who might be at increased risk of these adverse events.