Update on antineutrophil cytoplasmic autoantibody vasculitis in children.

PURPOSE OF REVIEW: Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is often organ- or life-threatening in children and impacts them during important periods of psychosocial and physical development. This review covers recent advances in the pathophysiology, diagnosis, management, and outcomes of AAV in children and highlights the ongoing need for funding and increased research collaboration. RECENT FINDINGS: Recent work has improved our understanding of AAV disease pathogenesis, potentially identifying new biomarkers and therapeutic targets. Collaborative clinical studies have also highlighted the variable manifestations in children and identified potential factors associated with poorer outcomes. Consensus-based treatment guidelines are also appearing, but clinical trials are still essential to better understanding treatment efficacy and safety in children affected by AAV. New, validated outcome measures, including those that are patient-reported, will facilitate these much-needed clinical trials in pediatric AAV. SUMMARY: There is a continued need for more rigorous study in pediatric AAV, however, there is certainly excitement with the increase in recent research relevant to the pediatric population.

A new perspective on balancing life domains: work-nonwork balance crafting.

BACKGROUND: Self-initiated and proactive changes in working conditions through crafting are essential for shaping work and improving work-related well-being. Recently, the research stream of job crafting has been extended to other life domains. The present paper aims to study a novel crafting concept-work-nonwork balance crafting-investigating the role of its antecedents and identifying relevant outcomes. Work-nonwork balance crafting is defined as individuals' unofficial techniques and activities to shape their work-nonwork balance, here considering their life domain boundary preferences. METHODS: In the study, 1,060 employees in three European countries (Austria, Germany and Switzerland) were surveyed in a longitudinal three-wave study with three-month intervals. We explored the influences of job/home demands and resources as antecedents of work-nonwork balance crafting. Important constructs for employee health and well-being (i.e., work engagement, work-related burnout, mental well-being and detachment from work) were investigated as outcomes. RESULTS: The findings suggest that resources and demands in the context of work or home are key antecedents of work-nonwork balance crafting. Work-nonwork balance crafting was also predictive for important employee health and well-being outcomes over three months, mainly in a positive and health-promoting way. CONCLUSION: This study provides insights into the antecedents of proactive efforts to balance the complex interplay of life domains. By studying work-nonwork balance crafting, we provide a new perspective on crafting beyond job crafting, which may help maintain or improve employees' mental health and well-being.

The Impact of Altitude at Birth on Perinatal Respiratory Support for Neonates with Trisomy 21.

OBJECTIVE: Both high altitude and trisomy 21 (T21) status can negatively impact respiratory outcomes. The objective of this study was to examine the association between altitude and perinatal respiratory support in neonates with T21 compared with those without T21. STUDY DESIGN: This retrospective cohort study used the United States all-county natality files that included live, singleton, in-hospital births from 2015 to 2019. Descriptive statistics for neonates with and without the primary outcome of sustained assisted ventilation (>6 hours) were compared using t-tests and Chi-squared analyses. Multivariable logistic regression was used to determine the association between respiratory support and the presence of T21, and included an interaction term to determine whether the association between respiratory support and the presence of T21 was modified by elevation at delivery. RESULTS: A total of 17,939,006 neonates, 4,059 (0.02%) with T21 and 17,934,947 (99.98%) without, were included in the study. The odds of requiring sustained respiratory support following delivery were 5.95 (95% confidence interval [CI]: 5.31, 6.66), 4.06 (95% CI: 2.39, 6.89), 2.36 (95% CI: 1.64, 3.40), and 5.04 (95% CI: 1.54, 16.54) times as high for neonates with T21 than without T21 when born at low, medium, high, and very high elevations, respectively. The odds of requiring immediate ventilation support following delivery were 5.01 (95% CI: 4.59, 5.46), 5.90 (95% CI: 4.16, 8.36), 2.86 (95% CI: 2.15, 3.80), and 12.08 (95% CI: 6.78, 21.51) times as high for neonates with T21 than without T21 when born at low, medium, high, and very high elevation, respectively. CONCLUSION: Neonates with T21 have increased odds of requiring respiratory support following delivery when compared with neonates without T21 at all categories of altitude. However, the odds ratios did not increase monotonically with altitude which indicates additional research is critical in understanding the effects of altitude on neonates with T21. KEY POINTS: · Neonates with T21 have an increased need for perinatal respiratory support at all altitudes.. · The odds of needing perinatal respiratory support did not increase monotonically with elevation.. · Additional research is critical to understanding the effects of altitude on neonates with T21..

Sense of coherence, off-job crafting, and mental well-being: A path of positive health development.

Our study examines the core concept of salutogenesis-sense of coherence (SOC)-in relation to off-job crafting (OJC) and mental well-being (MWB). The original salutogenic model of health mainly addresses the protective function of SOC against adversity. In our study, we focus on the recently proposed path of positive health development that captures how SOC can contribute to positive health and thriving. We present and test our theoretical assumptions about one such path, examining OJC as a possible mechanism how SOC translates into MWB. We tested our proposed model using cross-lagged panel model with three waves of panel data from Swiss and German employees (N = 2217). We compared our model to alternative nested models and conducted indirect effect analysis to test longitudinal mediation. Our hypothesized model fitted the data well and we found support for our main hypothesis that OJC partially mediates the relationship between SOC and MWB. Further, we identified positive reciprocal relationships between SOC and MWB, as well as between OJC and MWB. Overall, our study provides evidence that OJC is one mechanism underlying the recently postulated path of positive health development in the salutogenic model. For health promotion, this implies that promoting SOC and OJC may help to strengthen individual well-being and lead to positive feedback loops that foster personal development and thriving.

Being an accountant, cook, entertainer and teacher-all at the same time: Changes in employees' work and work-related well-being during the coronavirus (COVID-19) pandemic.

In March 2020, the world was hit by the coronavirus disease (COVID-19) pandemic which led to all-embracing measures to contain its spread. Most employees were forced to work from home and take care of their children because schools and daycares were closed. We present data from a research project in a large multinational organisation in the Netherlands with monthly quantitative measurements from January to May 2020 (N = 253-516), enriched with qualitative data from participants' comments before and after telework had started. Growth curve modelling showed major changes in employees' work-related well-being reflected in decreasing work engagement and increasing job satisfaction. For work-non-work balance, workload and autonomy, cubic trends over time were found, reflecting initial declines during crisis onset (March/April) and recovery in May. Participants' additional remarks exemplify that employees struggled with fulfilling different roles simultaneously, developing new routines and managing boundaries between life domains. Moderation analyses demonstrated that demographic variables shaped time trends. The diverging trends in well-being indicators raise intriguing questions and show that close monitoring and fine-grained analyses are needed to arrive at a better understanding of the impact of the crisis across time and among different groups of employees.

Relationships between recovery experiences and well-being among younger and older teachers.

PURPOSE: The study had three aims. We investigated, first, how six recovery experiences (i.e., detachment, relaxation, control, mastery, meaning, and affiliation) during off-job time suggested by the DRAMMA model (Newman et al. in J Happiness Stud 15(3):555-578. https://doi.org/10.1007/s10902-013-9435-x, 2014) are related to well-being (i.e., vitality, life satisfaction, and work ability). Second, we examined how age related to these outcomes, and third, we investigated whether age moderated the relationships between recovery experiences and well-being outcomes. METHODS: A sample of 909 Finnish teachers responded to an electronic questionnaire (78% women, average age 51 years). The data were analyzed with moderated hierarchical regression analyses. RESULTS: Detachment from work, relaxation, control, and mastery were associated with higher vitality. Detachment, relaxation, meaning, and affiliation were related to higher life satisfaction. Older age was related to lower work ability, but not to vitality or life satisfaction. Older teachers benefited more from control and mastery during off-job time than did younger teachers in terms of vitality, whereas younger teachers benefited more from relaxation in terms of all well-being outcomes. CONCLUSIONS: Detachment, relaxation, control, mastery, meaning, and affiliation during off-job time were related to higher well-being, supporting the DRAMMA model. Age moderated the relationships between control, mastery, and relaxation and vitality and life satisfaction. The role of aging in recovery from work needs further research.

Efficacy of a smartphone-based intervention - "Holidaily" - promoting recovery behaviour in workers after a vacation: study protocol for a randomised controlled trial.

BACKGROUND: While work-related rumination increases the risk of acute stressors developing into chronic load reactions and adverse health, mental detachment has been suggested as a way to interrupt this chain. Despite the importance of mentally detaching from work during leisure time, workers seem to struggle to disengage and, instead, experience the constant mental representation of work-related stressors, regardless of their absence. Those who struggle with work-related rumination could benefit from an easy-access intervention that fosters mental detachment by promoting recreational activities. Especially during vacations, workers appear to naturally engage in sufficient recovery activities; however, this beneficial behaviour is not sustained. The smartphone app-based intervention "Holidaily" promotes recovery behaviour and, thus, mental detachment from work with the intension of extending the beneficial effects of workers' vacations into their daily working life. METHODS: This randomised-controlled trial (RCT) evaluates the efficacy of "Holidaily". The Holidaily app is a German stand-alone program for mobile devices with either Android/iOS operating systems. The sample includes workers, who are awaiting to go on vacation and are randomly assigned to either the intervention (IG) or a waitlist-control group (CG). The IG receives two weeks pre-vacation access to Holidaily, while the CG receives access two weeks post-vacation. On a daily basis participants in the IG are provided with three options promoting recreational activities and beneficial recovery experiences. Online questionnaires are distributed to all participants at several timepoints. The primary outcome measure assesses participants' work-related rumination (Irritation Scale). A significant difference two weeks post-vacation is expected, favouring the IG. Secondary outcomes include symptoms of depression, insomnia severity, emotional exhaustion, thinking about work, recovery experiences, vacation specifics, work and personal characteristics. To help explain the intervention's effect, explorative analyses will investigate the mediation properties of the frequency of engaging in recreational activities and the moderation properties of Holidaily users' experiences. DISCUSSION: If successful, workers will maintain their recovery behaviour beyond their vacation into daily working life. Findings could, therefore, provide evidence for low-intensity interventions that could be very valuable from a public-health perspective. App-based interventions have greater reach; hence, more workers might access preventative tools to protect themselves from developing adverse health effects linked to work-related rumination. Further studies will still be needed to investigate whether the vacation phenomenon of "lots of fun quickly gone" can be defied and long-term benefits attained. TRIAL REGISTRATION: German Clinical Trials Registration DRKS00013650 . Registered retrospectively 15.01.2018.

The design and development of a hybrid off-job crafting intervention to enhance needs satisfaction, well-being and performance: a study protocol for a randomized controlled trial.

BACKGROUND: Employees dealing with job demands such as high workload and permeable work-life boundaries could benefit from bottom-up well-being strategies such as off-job crafting. We have developed a hybrid off-job crafting intervention to promote off-job crafting, a proactive pursuit to adjust one's off-job time activities to satisfy one's psychological needs. This hybrid intervention contains both on-site (two trainings) and online elements (smartphone app) to enhance employees' well-being and performance within different life domains. METHODS: The study is designed as a randomized controlled trial with an intervention group and a waitlist control group. The study population will be Finnish knowledge workers. The intervention program focuses on six psychological needs (detachment, relaxation, autonomy, mastery, meaning, and affiliation) proposed by the DRAMMA model. The intervention will consist of the following components: 1) an on-site off-job crafting training, 2) an individual off-job crafting plan for the four-week intervention period, 3) Everydaily smartphone app usage, and 4) a training session for reflection. The study outcomes are assessed with online questionnaires once at baseline, weekly during the intervention period and twice after the intervention (two-week and six-week follow-up). Moreover, during the second training session, participants will participate in a process evaluation to shed light on the mechanisms that can affect the effectiveness of the intervention. DISCUSSION: We expect that the intervention will stimulate off-job crafting behaviors, which may in turn increase well-being and performance in both non-work and work domains during and after the intervention (compared to baseline and to the control group). The intervention may provide employees with additional resources to deal with various stressors in life. Furthermore, this off-job crafting intervention could also offer performance benefits for the employers such as increased organizational citizenship behaviors among employees. TRIAL REGISTRATION: The Netherlands Trial Register (NTR): NL8219, December 9, 2019. Registered retrospectively. https://www.trialregister.nl/trial/8219.

Emotional labour and work engagement among nurses: examining perceived compassion, leadership and work ethic as stress buffers.

AIM: The study examined whether three resources, that is, compassion, transformational leadership and work ethic feasibility, buffer against the negative effects of emotional labour on work engagement. BACKGROUND: Emotional labour is a common job stressor among nurses, but little is known about whether certain personal and work resources buffer against it in relation to work engagement. Revealing buffers of emotional labour would help organizations to design tailored interventions. DESIGN: Cross-sectional online survey conducted in 2014. METHODS: Participants were 3466 Finnish nurses. Hypotheses were tested via hierarchical moderated regression analyses. RESULTS: Higher emotional labour related to lower engagement. Two interaction effects were found. First, work ethic feasibility buffered against emotional labour: the nurses who perceived work ethic feasibility as high in a situation of high emotional labour, scored higher on engagement compared with those nurses who in this stress situation perceived work ethic feasibility to be low. Second, high compassion was detrimental to engagement in the presence of high emotional labour. Transformational leadership did not act as a buffer but showed a positive relationship with engagement. CONCLUSION: Work ethic feasibility (being able to work according to high ethical standards) is an important resource in nursing as it protects an employee against the negative effects of emotional labour and as it also directly promotes engagement. However, compassion may not always be beneficial in nursing, especially if co-occurring with high job stress. Transformational leadership has potential to improve engagement in nursing although it may not operate as a stress buffer.

Patterns of daily energy management at work: relations to employee well-being and job characteristics.

PURPOSE: The present study aimed at identifying subgroups of employees with similar daily energy management strategies at work and finding out whether well-being indicators and job characteristics differ between these subgroups. METHODS: The study was conducted by electronic questionnaire among 1122 Finnish employees. First, subgroups of employees with unique and distinctive patterns of energy management strategies were identified using latent profile analysis. Second, differences in well-being indicators and job characteristics between the subgroups were investigated by means of ANCOVA. RESULTS: Four subgroups (i.e., patterns) were identified and named: Passives (n = 371), Averages (n = 390), Casuals (n = 272) and Actives (n = 89). Passives used all three (i.e., work-related, private micro-break and physical micro-break) strategies less frequently than other subgroups, whereas Actives used work-related and physical energy management strategies more frequently than other subgroups. Averages used all strategies on an average level. Casuals' use of all strategies came close to that of Actives, notably in a shared low use of private micro-break strategies. Active and Casual patterns maintained vigor and vitality. Autonomy and social support at work played a significant role in providing opportunities for the use of beneficial energy management strategies. CONCLUSIONS: Autonomy and support at work seem to support active and casual use of daily energy management, which is important in staying energized throughout the working day.

Exposure to nature versus relaxation during lunch breaks and recovery from work: development and design of an intervention study to improve workers' health, well-being, work performance and creativity.

BACKGROUND: The objective of this research project is to understand and to improve workers' recovery from work stress. Although recovery during lunch breaks is the most common within-workday break, it has received only minor research attention. Therefore, we will study whether lunch breaks including a relaxation session or exposure to nature have more favorable outcomes than usually spent lunch breaks concerning: a) recovery processes, b) health, c) well-being, d) job performance and e) creativity. We approach recovery by combining the theoretical frameworks of work and environmental psychology. METHODS/DESIGN: We conduct an intervention study in a sample of 268 knowledge-workers who engage in different lunch break activities for 15-minutes per day, two weeks in a row. We randomly assign participants to three experimental conditions: 1) exposure to nature, 2) relaxation and 3) control group (lunch break spent as usual). Online questionnaires before and after the intervention assess long term changes regarding recovery processes and the major outcome variables. Before, during and after the intervention, SMS and paper-pencil questionnaires measure the same constructs four times a day with fewer items. We also measure blood pressure and collect saliva samples to map cortisol excretion across the intervention period. A timed experimental task (i.e., the Alternative Uses Task) is used to examine differences in creativity between the three groups after the intervention period. DISCUSSION: By combining the knowledge of work and environmental psychology about recovery and restorative experiences, by merging three recovery perspectives (settings, processes, and outcomes) and by using data triangulation, we produce valid results that broaden our view on mechanisms underlying recovery and enhance our understanding about their links to psychological, behavioural and physiological outcomes, resulting in a more comprehensive picture of work stress recovery in general. TRIAL REGISTRATION: ClinicalTrials.gov Protocol Registration System NCT02124837. Registered 24 April 2014.

Hybrid incompatibility emerges at the one-cell stage in interspecies Caenorhabditis embryos.

Intrinsic reproductive isolation occurs when genetic differences between populations disrupt the development of hybrid organisms, preventing gene flow and enforcing speciation. 1-4 While prior studies have examined the genetic origins of hybrid incompatibility, 5-18 the effects of incompatible factors on development remain poorly understood. Here, we investigate the mechanistic basis of hybrid incompatibility in Caenorhabditis nematodes by capitalizing on the ability of C. brenneri females to produce embryos after mating with males from several other species. Contrary to expectations, hybrid incompatibility was evident immediately after fertilization, suggesting that post-fertilization barriers to hybridization originate from physical incompatibility between sperm and oocyte-derived factors rather than from zygotic transcription, which starts after the 4-cell stage. 19-22 Sperm deliver chromatin, which expands to form a pronucleus, and a pair of centrioles, which form centrosomes that attach to the sperm-derived pronucleus and signal to establish the embryo's anterior-posterior axis. 23,24 In C. brenneri oocytes fertilized with C. elegans sperm, sperm pronuclear expansion was compromised, frequent centrosome detachment was observed, and cortical polarity was disrupted. Live imaging revealed that defective polar body extrusion contributes to defects in mitotic spindle morphology. C. brenneri oocytes fertilized with C. remanei or C. sp. 48 sperm showed similar defects, and their severity and frequency increased with phylogenetic distance. Defective expansion of the sperm-derived pronucleus and unreliable polar body extrusion immediately after fertilization generally underlie the inviability of hybrid embryos in this clade. These results indicate that physical mismatches between sperm and oocyte-derived structures may be a primary mechanism of hybrid incompatibility.

Needs-based job crafting: Validation of a new scale based on psychological needs.

We present the conceptualization and validation of the Needs-Based Job Crafting Scale (NJCS), a new assessment tool theoretically grounded in the Identity-Based Integrative Needs Model of Crafting and DRAMMA psychological needs (detachment, relaxation, autonomy, mastery, meaning, and affiliation). The article is composed of three studies. In Study 1, we develop the NJCS and test its factorial structure using a cross-sectional sample of Finnish employees (N = 578). In Study 2, we validate the factor structure and test the scale for measurement invariance across time with longitudinal samples from Finland (N = 578) and Japan (N = 228). In Study 3, we examine the convergent, criterion, and incremental validity using a sample of German and Swiss employees (N = 1,101). The results confirm a six-factor structure of the scale as defined by the detachment, relaxation, autonomy, mastery, meaning, and affiliation needs in all three samples. The NJCS showed convergent validity when correlated with the conceptually related Needs-Based Off-Job Crafting Scale (NOCS), a job crafting scale based on the job demands-resources (JD-R) model, and the Proactive Personality Scale. Further, the six job crafting dimensions explain a large amount of variance in work engagement, job satisfaction, burnout, and psychological needs satisfaction; thus, supporting criterion validity of the scale. Finally, the NJCS explains variance beyond the existing JD-R based job crafting scale in work engagement, job satisfaction, burnout, and recovery experiences; thus, supporting incremental validity of the NJCS. Together with the existing NOCS, the NJCS facilitates the examination of crafting dynamics within and across work and nonwork life domains, applying a shared theoretical framework of psychological needs. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Needs Assessment of Musculoskeletal Ultrasound Training in North American Pediatric Rheumatology Fellowship Programs.

OBJECTIVE: Musculoskeletal ultrasound (MSUS) is widely used in adult rheumatology practice for diagnosis of arthritis and procedural guidance; however, it is not yet common practice in pediatric rheumatology. MSUS is advantageous to the pediatric population because it lacks radiation and eliminates need for sedation. This study aims to assess interest in, access to, and barriers to MSUS training in pediatric rheumatology fellowship programs in North America. METHODS: A survey was developed by pediatric rheumatology providers with experience in medical and/or MSUS education and distributed via REDCap anonymously in March 2022 (Supplementary Material). Eligible participants included current and recently graduated (<1 year) pediatric rheumatology fellows at a North American program. Descriptive statistics and bivariate analyses using design-based Pearson chi-squared tests were performed. RESULTS: Overall response rate was 78% (88/113), and 75% reported some form of MSUS training during fellowship. Only 36% indicated their program had a formal MSUS curriculum. Of those with MSUS training, 23% reported adult-only MSUS education. Eighty-four percent felt MSUS would be beneficial to their career. Major barriers to MSUS training included lack of MSUS-trained faculty, lack of time, and lack of hands-on MSUS sessions. Those who had access to MSUS training were significantly more interested in MSUS than those without (P = 0.0036). CONCLUSION: This study demonstrates that North American pediatric rheumatology fellows have a strong interest in learning MSUS, but they face significant challenges in accessing MSUS training (lack of MSUS-trained faculty, time, and access to hands-on training). MSUS should be incorporated into fellowship curriculum; however, implementation remains a challenge.

Crafting and human energy: Needs-based crafting efforts across life domains shape employees' daily energy trajectories.

We use experience sampling methodology and adopt the integrative needs model of crafting to investigate employees' daily energy trajectories, and to test whether employees' energy can be conserved or increased throughout the day through the proactive behavioral strategy of needs-based crafting. We first examine the daily trajectories of energy and then investigate the role of employees' daily crafting efforts (at work and in their private lives) in managing their energy throughout the day. Finally, we explore the daily within-person trajectories of needs-based crafting. We tested our hypotheses on a sample of 110 employees providing data on four nonconsecutive days (resulting in 2,358 observations nested in 396 days). Continuous growth curve analyses confirmed that energy follows an inverted U-shaped pattern of increasing energy until noon, after which energy steadily decreased until bedtime. However, daily crafting efforts contributed to these change trajectories: On days when employees crafted more than average, their energy was higher, particularly in the morning and afternoon. These positive crafting effects disappeared toward the end of the day, before bedtime. Crafting followed a linear trajectory, increasing over the course of the day, suggesting that it is a proactive strategy people also engage in outside of work. This suggests that domain-spanning needs-based crafting could be an important proactive strategy to maintain higher energy throughout an entire working day, even in the afternoon, when energy normally starts to fall. Our research contributes to our understanding of the nature of energy and of the microdynamic, within-person energy effects of general crafting efforts. (PsycInfo Database Record (c) 2023 APA, all rights reserved).

Therapeutic Advances in Eosinophilic Granulomatosis with Polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an eosinophilic vasculitis that affects a variety of organ systems. Historically, glucocorticoids and a variety of other immunosuppressants were used to abrogate the inflammation and tissue injury associated with EGPA. The management of EGPA has evolved greatly during the last decade with the development of novel targeted therapeutics that have resulted in significantly improved outcomes for these patients, with many more novel targeted therapies emerging.

Daily sleep in relation to subjective and physiological stress in an occupational context: Daily vigour as a mediator.

Studies on the stress-sleep relationship consistently demonstrate negative effects of stress on sleep. The reversed relation, however, has received less research attention. Also, field studies on physiological stress are scarce. The aim of this day-level diary study was to examine daily relationships between sleep quality and quantity, and subjective and physiological stress in an occupational context. Moreover, we examined daily vigour as an underlying mechanism of the sleep-stress relationship. Participants were 167 knowledge workers who filled in daily questionnaires measuring sleep quality and quantity, morning vigour and subjective afternoon stress on Tuesdays and Thursdays for 5 weeks. Physiological stress was assessed with cortisol decline from morning peak to evening, and with blood pressure in the afternoon. Multilevel path analysis results showed that better sleep quality and longer sleep hours predicted increased vigour the following morning, which in turn predicted lower subjective stress in the afternoon. Sleep quality and quantity were not related to physiological stress neither directly nor indirectly via morning vigour. On the basis of our results, sleep should be considered as a factor affecting vigour which in turn seems to lower stress.

Why so low? An unusual case of myositis in a child.

BACKGROUND: Sarcoidosis is characterized by non-caseating epithelioid granulomas in various tissues throughout the body, most commonly the lung. Non-caseating granulomas may be seen in skeletal muscle, though typically asymptomatic and under-recognized. While rare in children, there is a need to better characterize the disease and its management. Here we present a 12-year-old female with bilateral calf pain who was ultimately found to have sarcoid myositis. CASE PRESENTATION: A 12-year-old female presented to rheumatology with significantly elevated inflammatory markers and isolated lower leg pain. MRI of the distal lower extremities demonstrated extensive bilateral myositis with active inflammation, atrophy, and to a lesser extent fasciitis. This distribution of myositis in a child garnered a broad differential requiring a systematic evaluation. Ultimately, muscle biopsy revealed non-caseating granulomatous myositis with perivascular inflammation, extensive muscle fibrosis, and fatty replacement of the muscle with a CD4+ T cell predominant, lymphohistiocytic infiltrate consistent with sarcoidosis. Review of histopathology from age 6 of an extraconal mass resected from her right superior rectus muscle further confirmed the diagnosis. She had no other clinical symptoms or findings of sarcoidosis. The patient improved significantly with methotrexate and prednisone, though flared again after self-discontinuation of medications and was subsequently lost to follow-up. CONCLUSION: This is the second reported case of granulomatous myositis associated with sarcoidosis in a pediatric patient, and the first to present with a chief complaint of leg pain. Increased knowledge of pediatric sarcoid myositis within the medical community will enhance recognition of the disease, improve the evaluation of lower leg myositis, and advance outcomes for this vulnerable population.

Targeting Liver Metastases to Potentiate Immunotherapy in MS-Stable Colorectal Cancer-A Review of the Literature.

Immunotherapy has revolutionized the treatment of several cancers, including melanoma and lung cancer. However, for colorectal cancer, it is ineffective for 95% of patients with microsatellite-stable disease. Recent evidence suggests that the liver's immune microenvironment plays a pivotal role in limiting the effectiveness of immunotherapy. There is also evidence to show that targeting liver metastases with locoregional therapies, such as surgery or irradiation, could potentiate immunotherapy for these patients. This review presents evidence from preclinical studies regarding the underlying mechanisms and from clinical studies that support this approach. Furthermore, we outline potential directions for future clinical trials. This innovative strategy could potentially establish immunotherapy as an effective treatment for MS-stable colorectal cancer patients, which are currently considered resistant.