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Cerebrovascular diseases (CVDs), comprising predominantly ischemic stroke and chronic cerebral hypoperfusion (CCH), are a significant threat to global health, often leading to disability and mortality. Remote ischemic conditioning (RIC) has emerged as a promising, non-pharmacological strategy to combat CVDs by leveraging the body's innate defense mechanisms. This review delves into the neuroprotective mechanisms of RIC, categorizing its effects during the acute and chronic phases of stroke recovery. It also explores the synergistic potential of RIC when combined with other therapeutic strategies, such as pharmacological treatments and physical exercise. Additionally, this review discusses the pathways through which peripheral transmission can confer central neuroprotection. This review concludes by addressing the challenges regarding and future directions for RIC, emphasizing the need for standardized protocols, biomarker identification, and expanded clinical trials to fully realize its therapeutic potential. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is a multifaceted autoimmune disorder characterized by dysregulated immune responses and autoantibody production, which affects multiple organs and varies in clinical presentation and disease severity. The development of SLE is intricate, encompassing dysregulation within the immune system, a collapse of immunological tolerance, genetic susceptibilities to the disease, and a variety of environmental factors that can act as triggers. This review provides a comprehensive discussion of the pathogenesis and treatment strategies of SLE and focuses on the progress and status of traditional and emerging treatment strategies for SLE. Traditional treatment strategies for SLE have mainly employed non-specific approaches, including cytotoxic and immunosuppressive drugs, antimalarials, glucocorticoids, and NSAIDs. These strategies are effective in mitigating the effects of the disease, but they are not a complete cure and are often accompanied by adverse reactions. Emerging targeted therapeutic drugs, on the other hand, aim to control and treat SLE by targeting B and T cells, inhibiting their activation and function, as well as the abnormal activation of the immune system. A deeper understanding of the pathogenesis of SLE and the exploration of new targeted treatment strategies are essential to advance the treatment of this complex autoimmune disease. [ABSTRACT FROM AUTHOR]

Charge management, which lowers charging costs for fleets and prevents straining the electrical grid, is critical to the successful deployment of medium- and heavy-duty battery-electric trucks (MHD BETs). This study introduces an energy demand and cost management framework that optimizes depot charging for MHD BETs by combining an energy consumption machine learning model and a linear program optimization model. The framework considers key factors impacting real-world MHD BET operations, including vehicle and charger configurations, duty cycles, use cases, geographic and climate conditions, operation schedules, and utilities' time-of-use (TOU) rates and demand charges. The framework was applied to a hypothetical fleet of 100 MHD BETs in California under three different utilities for 365 days, with results compared to unmanaged charging. The optimized charging solution avoided more than 90% of on-peak charging, reduced fleet charging peak load by 64–75%, and lowered fleet energy variable costs by 54–64%. This study concluded that the proposed charge management framework significantly reduces energy costs and peak loads for MHD BET fleets while making recommendations for fleet electrification infrastructure planning and the design of utility TOU rates and demand charges. [ABSTRACT FROM AUTHOR]

Introduction: Postoperative cognitive dysfunction (POCD) is a common neurological issue following cardiopulmonary bypass (CPB)-assisted heart surgery. Remote ischemic preconditioning (RIPC) increases the tolerance of vital organs to ischemia/reperfusion injury, leading to reduced brain injury biomarkers and improved cognitive control. However, the exact mechanisms underlying RIPC's neuroprotective effects remain unclear. This systematic review aimed to explore the hypothesis that RIPC lowers neurocognitive dysfunction in patients undergoing CPB surgery. Method: All relevant studies were searched in PubMed, ScienceDirect, EBSCOhost, Google Scholar, Semantic Scholar, Scopus, and Cochrane Library database. Assessment of study quality was carried out by two independent reviewers individually using the Cochrane Risk of Bias (RoB-2) tool. Meta-analysis was performed using a fixed-effect model due to low heterogeneity among studies, except for those with substantial heterogeneity. Results: A total of five studies with 1,843 participants were included in the meta-analysis. RIPC was not associated with reduced incidence of postoperative cognitive dysfunction (five RCTs, odds ratio [OR: ] 0.79, 95% confidence interval [CI]: 0.56-1.11) nor its improvement (three RCTs, OR: 0.80, 95% CI: 0.50-1.27). In addition, the analysis of the effect of RIPC on specific cognitive function tests found that pooled SMD for RAVLT 1-3 and RAVLT LT were -0.07 (95% CI: -0.25,012) and -0.04 (95% CI: -0.25-0.12), respectively, and for VFT semantic and phonetic were -0.15 (95% CI: -0.33-0.04) and 0.11 (95% CI: -0.40-0.62), respectively. Conclusion: The effect of RIPC on cognitive performance in CABG patients remained insignificant. Results from previous studies were unable to justify the use of RIPC as a neuroprotective agent in CABG patients. [ABSTRACT FROM AUTHOR]

Muscular atrophy is among the systematic decline in organ functions in aging, while defective thermogenic fat functionality precedes these anomalies. The potential crosstalk between adipose tissue and muscle during aging is poorly understood. In this study, it is showed that UCP1 knockout (KO) mice characterized deteriorated brown adipose tissue (BAT) function in aging, yet their glucose homeostasis is sustained and energy expenditure is increased, possibly compensated by improved inguinal adipose tissue (iWAT) and muscle functionality compared to age-matched WT mice. To understand the potential crosstalk, RNA-seq and metabolomic analysis were performed on adipose tissue and muscle in aging mice and revealed that creatine levels are increased both in iWAT and muscle of UCP1 KO mice. Interestingly, molecular analysis and metabolite tracing revealed that creatine biosynthesis is increased in iWAT while creatine uptake is increased in muscle in UCP1 KO mice, suggesting creatine transportation from iWAT to muscle. Importantly, creatine analog β-GPA abolished the differences in muscle functions between aging WT and UCP1 KO mice, while UCP1 inhibitor α-CD improved muscle glycolytic function and glucose metabolism in aging mice. Overall, these results suggested that iWAT and skeletal muscle compensate for declined BAT function during aging via creatine metabolism to sustain metabolic homeostasis., (© 2024 The Author(s). Advanced Science published by Wiley‐VCH GmbH.)

The Nouabalé-Ndoki National Park (NNNP) in Republic of Congo has become a beacon for conservation in Central Africa. This manuscript documents the arrival of primatologists, the establishment of field stations and major discoveries in primate behavior and ecology. Field stations were strategically established to study primate behavior in a variety of different contexts from stationary platforms to forest follows of habituated groups. The implementation of new technologies and analyses have also been a hallmark of research at Ndoki. Scientists are shaping a new era in primatology at NNNP by building on past successes and promoting the next generation of Congolese conservationists to address environmental challenges. Results have proven crucial in discussions with government and industry and led to conservation gains such as the inclusion of the intact forests of the Goualougo and Djéké Triangles into the NNNP. The research stations have also become essential for developing a long-term certified sustainable international gorilla tourism program. Despite the many advancements for conservation such as increased protection of forests, development of internationally recognized protocols and large-scale capacity building initiatives, there are reasons for considerable concern in the near- and long-term for primates and their forest habitats in the Ndoki landscape. To address these concerns, we emphasize the long history of forming partnerships with local communities. We also discuss shared overlap featuring multicultural and environmental use of forest resources that is likely to be crucial in championing the conservation of the Ndoki forests for the next 25 years and beyond., (© 2024. The Author(s), under exclusive licence to Japan Monkey Centre.)

Autoimmune inflammation is caused by the loss of tolerance to specific self-antigens and can result in organ-specific or systemic disorders. Systemic autoimmune diseases affect a significant portion of the population with an increasing rate of incidence, which means that is essential to have effective therapies to control these chronic disorders. Unfortunately, several patients with systemic autoimmune diseases do not respond at all or just partially respond to available conventional synthetic disease-modifying antirheumatic drugs and targeted therapies. However, during the past few years, some new medications have been approved and can be used in real-life clinical settings. Meanwhile, several new candidates appeared and can offer promising novel treatment options in the future. Here, we summarize the newly available medications and the most encouraging drug candidates in the treatment of systemic lupus erythematosus, rheumatoid arthritis, Sjögren's disease, systemic sclerosis, systemic vasculitis, and autoimmune myositis. [ABSTRACT FROM AUTHOR]

Background: Quad bikes are popular recreational, four-wheeled bikes in the Middle East. Injury prevention programs targeting quad bike crashes in the United Arab Emirates (UAE) need evidence about the risk factors and behaviours associated with these crashes in the target population. This is a protocol for a study aiming to investigate quad bike rider behaviours and to assess the risk factors associated with related injuries in the UAE. Methods: This is a cross-sectional observational study aiming to describe a seasonal sport in a desert environment. With an estimated sample size of 451, the survey will follow a three-stage, location-based sampling strategy using the line-transect method. A sampling frame of desert locations with high injury incidences was developed, using Dubai ambulance injury records. Further expansion of the sampling frame was participatory, involving police, enthusiasts, emergency responders and gas station employees. The data collection will be limited to the winter months in fifteen high-injury desert locations across three major Emirates in the UAE. Trained researchers will observe the riders directly in the desert to note their riding habits, followed by a researcher-administered interview on riding and injury history. The interviews will be administered in Arabic and English using Qualtrics software on handheld tablets with offline and online entry mode. In addition, paper-based entry with the same format will be used as a contingency in busy quad bike locations. Conclusion: The objective of this study protocol is to develop a comprehensive survey that will furnish substantial evidence for the formulation of effective injury prevention strategies. To enhance the credibility of the recorded riding behaviors, field observations will be employed. The uniqueness of this study lies in its innovative sampling strategy, custom-tailored to accommodate the highly mobile and transient population of desert bikers in the UAE. [ABSTRACT FROM AUTHOR]

Wound care in horses, particularly chronic non-healing lesions, pose a major medical challenge for equine practitioners and can result in considerable financial loss as well as missed training goals for owners. The lower equine limb is particularly susceptible to damage due to the lack of protective muscling and superficial synovial and tendinous structures. This paper presents the treatment of a non-healing distal leg wound in a horse by topical application of a Chinese herbal medicine decoction using a traditional Chinese veterinary medicine (TCVM) approach. The patient had sustained damage to the palmar aspect of the flexor tendons on the left thoracic limb caused by jumping off a transport vehicle. This injury was initially treated by cleaning the area, topical erythromycin antibiotic ointment and intramuscular injection of penicillin for 3 days. Over a 2-month period the initial trauma progressed to a chronic non-healing wound. Medical management of the wound was then changed to topical application of a Chinese herbal decoction which was applied 3 times daily. Over a 29-day period, this treatment encouraged normalization of the wound healing process with epithelization, tissue remodeling and normal scar formation. The clinical results achieved in this horse suggest topical treatment with Chinese herbal medicine can decrease complications and speed the healing process of chronic wounds. The use of the topical herbal formula in this case was convenient, simple to use, and cost effective. [ABSTRACT FROM AUTHOR]

Background: H Syndrome is a rare genetic condition caused by biallelic pathogenic variants in the SLC29A3 gene. It is characterized by a wide range of clinical manifestations, many of which are related to the immune-rheumatological field. These include scleroderma-like skin changes, deforming arthritis, and enlarged lymph nodes. The condition also features cardiac and endocrine defects, as well as hearing loss, for which the immune pathogenesis appears less clear. Immunomodulatory medications have been shown to improve many symptoms in recent experiences. Case presentation: A 21-year-old girl was referred to our institute after being diagnosed with H syndrome. Her medical history was characterized by the development of finger and toe deformities, which developed since the first years of life and progressively worsened with clinodactyly. At 6 years of age, she was diagnosed with diabetes mellitus without typical autoantibodies and with bilateral sensorineural hearing loss. She also complained of frequent episodes of lymphadenopathy, sometimes with colliquation and growth retardation due to pancreatic insufficiency. It wasn't until the genetic diagnosis of H syndrome that the continual increase in acute phase reactants was noticed, suggesting that an immunological pathogenesis may be the source of her problems. During her visit to our institute, she reported serious pain in both feet and hands and difficulty walking due to knee arthritis and muscle contractures. Conventional therapy with steroid injection in affected joints and methotrexate only led to partial improvement. After a thorough assessment of her inflammatory profile showing a high interferon score, the girl received treatment with baricitinib. Furthermore, based on recent data showing that SLC29A3 deficiency results in interferon production because of Toll-like Receptor 7 activation in lysosomes, hydroxychloroquine was also added. The combination of the two drugs resulted for the first time in a rapid and persistent normalization of inflammatory markers, paralleled by a dramatic improvement in symptoms. Conclusions: We describe the results of inhibiting IFN inflammation in H syndrome and discuss how JAK inhibitors and antimalarials might represent a mechanistically based treatment for this orphan drug disorder. [ABSTRACT FROM AUTHOR]

Watershed disturbances can have broad, long-lasting impacts that result in a range of streamflow response. Increasing disturbance regimes, particularly from wildfire, is a growing concern for watershed management. The influence of watershed disturbances on rainfall-runoff patterns has proved challenging to isolate from undisturbed streamflow variability due to the role of hydrologic controls that vary through time, including water year type, seasonality, and antecedent precipitation. To better assess the influence of watershed disturbance on rainfall-runoff event patterns we developed the Rainfall-Runoff Event Detection and Identification (RREDI) toolkit. The RREDI toolkit is a novel time-series event separation method that automates the pairing and attribution of precipitation and streamflow events, leveraging and building on existing event separation methods. A rainfall-runoff event dataset of 5042 events was generated by the RREDI toolkit from a collection of nine western US study watersheds spanning a range of streamflow regimes, watershed properties, and burn characteristics. Through analyzing the rainfall-runoff event dataset, we found that water year type and season were significant controls on rainfall-runoff metrics. The significance of antecedent precipitation was variable between watersheds, indicating a more complex relationship for this control. The watershed-specific permutations of significant controls resulted in unique significant condition group trends in the rainfall storm depth and peak runoff relationship in two contrasting watersheds. In general, for each of the significant condition groups post-fire peak runoff was higher than undisturbed peak runoff except during winter in snow-dominated watersheds. Consideration of the time-varying hydrologic controls, particularly water year type and season, were identified as important when untangling the influence of wildfire on the rainfall-runoff patterns. The RREDI toolkit can be further applied to investigate the influence of other watershed disturbances and controls to increase understanding of rainfall-runoff patterns across the landscape. [ABSTRACT FROM AUTHOR]

STING-associated vasculopathy with onset in infancy (SAVI) is a rare type Ι interferonopathy caused by gain of function mutations in an encoding stimulator of interferon genes (STING) protein 1. SAVI is characterized by neonatal or infantile-onset systemic inflammation, mainly affecting peripheral cutaneous blood vessels, skin, and lungs. The main disease manifestations include recurrent febrile episodes, cough, dyspnea, and failure to thrive, in association with progressive interstitial lung disease, polyarthritis, and cold-induced red violet plaques or papules on fingers, knees, toes, heels, nasal tip, and ears that can lead to distal ulcerations, skin necrosis, tissue loss, and autoamputation. For the management of SAVI, JAK inhibitors can be a valuable therapeutic intervention that hampers disease progression, while conventional immunosuppressive treatments have shown minimal efficacy. This review aims to describe the underlying pathophysiologic mechanisms of SAVI, highlighting the main clinical manifestations and discussing the current treatment approaches. [ABSTRACT FROM AUTHOR]

Background: Among children or adolescents with obesity, 40-70.5% will remain obese as adults according to their paediatric body mass index (BMI). The recommended management involves changes in their nutritional habits (diet, physical activity, and sedentary lifestyle). Motivational interviewing (MI), a patient-centred consultation, has proven its worth in many fields where acting on behaviours is essential. Aim: To investigate the use and outcomes of MI in the management of children and adolescents who are overweight and obese. Design & setting: A systematic review evaluated MI in the management of children and adolescents who are overweight and obese. Method: PubMed, Web of Science, Cochrane Library, and CISMeF were searched between January 2022 and March 2022 for following terms: 'motivational interviewing', 'overweight or obesity', 'children or adolescent' to identify randomised controlled trials (RCTs). Inclusion criteria were interventions involving MI in children or adolescents who were commonly (polygenically) overweight or obese. Exclusion criteria were: studies before 1991; and articles not written in English or French. The first stage of the selection process was carried out by reading the titles and abstracts. A second stage was carried out by reading the complete studies. A secondary inclusion of articles was carried out following the reading of bibliographic references, mainly from systematic reviews and meta-analyses. The data were summarised in synthetic tables based on the Population, Intervention, Comparison, Outcomes, and Study (PICOS) tool. Results: From 444 articles the review identified 26 RCTs. Statistically significant results were found for all criteria (anthropometric and behavourial) in both children and adolescents. Quality of life and depression scores were also improved. Parental presence in the interview appeared to be essential for children, whereas for adolescents, the supportive involvement of parents outside of the interviews seemed more appropriate. The frequency and duration of the interventions played a major role in obtaining results, as did the number of people involved, and the diversity of the places where they are taken care of. Conclusion: MI seems promising for children and adolescents with overweight or obesity, within the framework of a comprehensive, multiprofessional, family management, carried out over a long period with regular consultations. [ABSTRACT FROM AUTHOR]

Childhood-onset systemic lupus erythematosus is an inflammatory and autoimmune condition characterized by heterogeneous multisystem involvement and a chronic course with unpredictable flares. Kidney involvement, commonly called lupus nephritis, mainly presents with immune complex-mediated glomerulonephritis and is more frequent and severe in adults. Despite a considerable improvement in long-term renal prognosis, children and adolescents with lupus nephritis still experience significant morbidity and mortality. Moreover, current literature often lacks pediatric-specific data, leading clinicians to rely exclusively on adult therapeutic approaches. This review aims to describe pediatric lupus nephritis and provide an overview of the novel perspectives on the pathogenetic mechanisms, histopathological classification, therapeutic approach, novel biomarkers, and follow-up targets in children and adolescents with lupus nephritis. [ABSTRACT FROM AUTHOR]

Background Acute kidney injury (AKI) is developing as a frequent and significant coronavirus illness [coronavirus disease 2019 (COVID-19)] sequela owing to the spread of the virus in 2019. A few trials, meanwhile, have focused on AKI managed with renal replacement therapy (AKI-RRT). Aim To evaluate the effectiveness of early renal replacement treatment in COVID-19 AKI cases. Patients and methods A total of 100 individuals with AKI and confirmed COVID-19 infection participated in this case–control research. All participants were split into two groups: group A included 50 COVID-19-confirmed diagnosis patients with AKI and group B included 50 COVID-19-confirmed diagnosis patients without AKI. Following ICU admission, we collected laboratory results, physiological parameters, drugs, therapies, and organ support (including RRT), as well as clinical events like mortality. Results Use of invasive ventilation, vasopressor, deep sedation, and RRT was significantly higher in group A. When compared with patients with AKI who did not get RRT, 14 (28%) patients who had RRT were younger, had greater creatinine and blood urea nitrogen levels, and experienced oliguria more often. Five patients had at least one session of intermittent hemodialysis, and all patients experienced continuous venovenous hemofiltration. Considering mortality, hospital mortality and 60-day mortality rate were significantly higher in group A. However, there was no substantial variation between the two groups regarding ICU mortality, 28-day mortality, and RRT mortality. Age, baseline creatinine, and effective RRT were the independent predictors linked to 60-day mortality in the multiple logistic regression analysis. Conclusion Patients with AKI who need RRT had a nonsignificantly higher death rate than COVID-19-infected patients without AKI. Age, initial creatinine, and RRT effectiveness were all independent variables linked to 60-day mortality. [ABSTRACT FROM AUTHOR]

STING-associated vasculopathy with onset in infancy (SAVI) is caused by pathogenic gain-of-function variants in the gene TMEM173 (also named stimulator of interferon genes, STING1). This report details the case of an 11-year-old girl with SAVI who presented with skin-limited symptoms and discusses the phenotype-genotype correlations of the TMEM173 variant present in our patient. Treatment of SAVI focuses on preventing the development or progression of organ damage by reducing systemic inflammation. We summarize the available treatments for this syndrome., (© 2024 Wiley Periodicals LLC.)

Takayasu arteritis (TA) is a chronic granulomatous inflammatory disease affecting the aorta and its branches. Paediatric TA (pTA) may present from 6 months after birth till the adolescent age group. Genetics and pathogenesis of pTA are not fully understood. Earlier studies reported monogenic mutation in NOD2, XIAP, and STAT1 genes in patients with pTA. TA, a relatively rare disease, is more common in geographical pockets, including India. We hypothesized that South Asian patients with pTA, namely, those of Indian subcontinent origin, may have clinically relevant and unique pathogenic variants involving one or more genes, especially those linked to genetically driven vasculitic illnesses, including autoinflammatory pathologies. Children with pTA fulfilling EULAR/PRINTO/PReS classification criteria and presenting with clinical symptoms to the Paediatric Rheumatology clinic of Christian Medical College, Vellore, were included. Blood samples were collected after getting informed consent from parents or guardians and assent forms from children. DNA was extracted from whole blood using the Qiagen DNA extraction kit. Initially, the common variant in Indian population, namely, ADA2 c.139G > A; p.Gly47Arg, was screened, followed by whole exome sequencing. Fourteen children were recruited for the study. Median age of patients was 11 years (4 months-14 years) with a male-to-female ratio of 4:10. Distribution of angiographic subsets by Numano's classification of included children were as follows: type 5 (n = 7), type 4 (n = 5), and type 3 (n = 2). We identified novel variants in ten different genes. This include variants in genes of classical complement pathway, namely, C2, C3, C6, C7, and C9, and other genes, namely, CYBA, SH3BP2, GUCY2C, CTC1, COL5A1, and NLPR3. Two of 14 patients have heterozygous pathogenic variants; this implies that combination of heterozygous variants in C3 and COL5A1 might lead to disease development, suggesting digenic inheritance. One patient has a homozygous variant in CYBA. None of the patients were identified to have ADA2 variants. Whole exome sequencing reveals combination of rare variants in genes C3, COL5A1, and CYBA associated with disease development in children with Takayasu Arteritis. Key Points • We identified novel variants in genes of classical complement pathway, namely, C2, C3, C6, C7, and C9, and other genes, namely, CYBA, SH3BP2, GUCY2C, CTC1, COL5A1, and NLPR3. • Two of 14 patients have heterozygous pathogenic variants in C3 and COL5A1; this may have implications in disease development, suggesting digenic inheritance. • One patient has homozygous variant in CYBA. • None of the patients were identified to have ADA2 variants., (© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Stream channel incision and deposition are common after wildfire, and after these geomorphic changes occur, they may impact runoff mechanisms and the composition of pre‐event and event water in runoff. To investigate this, we monitored discharge and electrical conductivity at six nested sites within a 15.5 km2 watershed in the northern Colorado Front Range that had burned several years prior, and then experienced large flooding and well‐documented and significant channel erosion and deposition in the following years. Over the study period, which occurs 3 years after the fire, and 2 years after the beginning of significant geomorphic changes, the watershed experienced seven precipitation events. For each hydrograph, we separate baseflow from runoff using a new method to characterize and account for the strong diurnal signal in the baseflow. Electrical conductivity is used as a tracer in a two‐component end‐member mixing analysis to separate the event hydrographs into event and pre‐event water. Correlation coefficients were computed between key variables of the hydrologic response (such as runoff ratio, volumes of event and pre‐event water) to storm and basin characteristics (including stream channel erosion/deposition, fraction of high/moderate burn severity, precipitation intensity and antecedent precipitation). The strength and significance of correlations was found to vary seasonally. In the early season, event and pre‐event volumes did not vary significantly with basin or storm characteristics. In the late season, antecedent precipitation correlated with a decrease in event runoff (R2 = 0.34) and total runoff (R2 = 0.40), increased precipitation intensity correlated with an increase in event runoff (R2 = 0.48) and local erosion correlated with an increase in pre‐event runoff (R2 = 0.60) and total runoff (R2 = 0.53). We hypothesize that the difference in correlations between early season and late season is due seasonal variations in the groundwater table gradient. These findings indicate that seasonality and postfire stream channel erosion influence the makeup of runoff response. [ABSTRACT FROM AUTHOR]

Objectives Takayasu arteritis (TAK) is a large-vessel vasculitis rarely reported in children and infants. Most articles on paediatric TAK have not focused on infants. We present the largest case series of infantile TAK, aiming to identify its demographic and clinical characteristics and compare them with existing data on older children. Methods We conducted an international multicentre retrospective cohort study. Epidemiological and clinical data were collected from patients' charts from six rheumatology centres. All patients met both the EULAR/PReS 2008 criteria and the 1990 ACR/EULAR criteria and were diagnosed with TAK at age <5 years. Results Twelve patients were included (50% female). Median age of symptom onset was 11 months, with a diagnostic delay of 4 months. The most common symptoms at presentation were hypertension, blood pressure differences between limbs, and fever. The most commonly involved arteries were the abdominal aorta and renal artery. Medications included steroids, conventional and biologic DMARDs, and other immunosuppressive therapies. Half of the patients received biologic agents, of which infliximab had the highest complete remission rate (40%). Other medications resulting in complete remission were CYC (40%) and MTX (38%). Invasive procedures were required for 58% of patients. The most common complications were cardiac (50%), stroke (42%), and serious infections (33%). No patients died. Conclusion This study presents the largest series of infantile TAK. Compared with other reported series on older children, infants with TAK have more severe disease and were more likely to receive biologic agents, develop complications, and require invasive interventions. [ABSTRACT FROM AUTHOR]

H syndrome is a rare autosomal recessive genetic disorder characterized by the following clinical features: cutaneous hyperpigmentation, hypertrichosis, hepatosplenomegaly, heart anomalies, hearing loss, hypogonadism, short stature, hallux valgus, hyperglycemia, fixed flexion contractures of the toe joints, and the proximal interphalangeal joints. In rare cases, autoinflammatory and lymphoproliferative manifestations have also been reported. This disorder is due to loss-of-function mutations in SLC29A3 gene, which encode the equilibrative nucleoside transporter ENT3. This deficiency leads to abnormal function and proliferation of histiocytes. H syndrome is part of the R-group of histiocytosis. We report two different cases, one was diagnosed in adulthood and the other in childhood. The first case reported is a 37-year-old woman suffering from H syndrome with an autoinflammatory systemic disease that begins in adulthood (fever and diffuse organ's infiltration) and with cutaneous, articular, auditory, and endocrinological manifestations since childhood. The second case reported is a 2-year-old girl with autoinflammatory, endocrine, and cutaneous symptoms (fever, lymphadenopathy, organomegaly, growth delay, and cutaneous hyperpigmentation). Homozygous mutations in SLC29A3 confirmed the diagnosis of H syndrome in both cases. Each patient was treated with Tocilizumab with a significant improvement for lymphoproliferative, autoinflammatory, and cutaneous manifestations. Both cases were reported to show the multiple characteristics of this rare syndrome, which can be diagnosed either in childhood or in adulthood. In addition, an overview of the literature suggested Tocilizumab efficiency. [ABSTRACT FROM AUTHOR]

Background and purpose: Revascularization surgery for patients with moyamoya disease (MMD) is very complicated and has a high rate of postoperative complications. This pilot study aimed to prove the safety and efficacy of remote ischemic conditioning (RIC) in adult MMD patients undergoing revascularization surgery. Methods: A total of 44 patients with MMD were enrolled in this single-center, open-label, prospective, parallel randomized study, including 22 patients assigned to the sham group and 22 patients assigned to the RIC group. The primary outcome was the incidence of major neurologic complications during the perioperative period. Secondary outcomes were the modified Rankin Scale (mRS) score at discharge, at 90 days post-operation, and at 1 year after the operation. The outcome of safety was the incidence of adverse events associated with RIC. Blood samples were obtained to monitor the serum concentrations of cytokines (VEGF, IL-6). Results: No subjects experienced adverse events during RIC intervention, and all patients could tolerate the RIC intervention in the perioperative period. The incidence of major neurologic complications was significantly lower in the RIC group compared with the control group (18.2% vs. 54.5%, P = 0.027). The mRS score at discharge in the RIC group was also lower than the control group (0.86 ± 0.99 vs. 1.18 ± 1.22, P = 0.035). In addition, the serum IL-6 level increased significantly at 7 days after bypass surgery in the control group and the serum level of VEGF at 7 days post-operation in the RIC group. Conclusion: In conclusion, our study demonstrated the neuroprotective effect of RIC by reducing perioperative complications and improving cerebral blood flow in adult MMD patients undergoing revascularization surgery. Thus, RIC seems to be a potential treatment method for MMD. [ABSTRACT FROM AUTHOR]

Background Currently, the effects of motivational interviewing (MI) on children's behavioral changes remain obscure. Purpose This systematic review and meta-analysis examined the effects of MI on children's lifestyle behavioral changes (fruits and vegetables [F/V], dairy, sugary beverages, calories, snacks, fat intake, moderate vigorous physical activity [MVPA], and screen time). Methods Six databases (CINAHL, Cochrane, Embase, PsycINFO, PubMed, and Web of Sciences) from 2005 to 2022 were searched. Thirty-one intervention studies with a comparison group met the criteria. Random-effects models were performed to estimate the pooled effects; exploratory moderation analyses with mixed-effects models were used to explore potential intervention moderators. Results The pooled effect size was 0.10 (p =.334) on ↑F/V, 0.02 (p =.724) on ↑dairy, −0.29 (p <.001) on ↓calories, −0.16 (p =.054) on ↓sugary beverages, −0.22 (p =.002) on ↓snacks, −0.20 (p =.044) on ↓fat, 0.22 (p =.001) on ↑MVPA, and −0.06 (p =.176) on ↓screen time. The effects of MIs were moderated by ↑MI sessions regarding ↓snacks (B = −0.04, p =.010). Multicomponent and clinical programs had greater effects on dairy intake than their counterparts (0.09 vs. −0.21, p =.034; 0.12 vs. −0.14, p =.027, respectively). Similarly, interventions with a fidelity check resulted in greater dairy intake than those without a check (0.29 vs. −0.15, p =.014). A few long-term follow-up assessments revealed effects on ↓F/V (−0.18; p =.143, k = 2), ↓dairy (−0.13, p =.399, k = 4), ↓MVPA (−0.04; p =.611, k = 6), and ↑screen time (0.12; p =.242, k = 4). Conclusions Our findings support the short-term effects of MI on improving children's lifestyle behaviors. Additional investigations are needed to better sustain children's long-term behavioral changes. [ABSTRACT FROM AUTHOR]

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune systemic disease with a wide range of clinical symptoms, complex development processes, and uncertain prognosis. The clinical treatment of SLE is mainly based on hormones and immunosuppressants. Research on novel therapy strategies for SLE has flourished in recent years, especially the emergence of new targeted drugs and natural products that can modulate related symptoms. This review discusses the current experience including B-cell targeted drugs (belimumab, tabalumab, blisibimod, atacicept, rituximab, ofatumumab, ocrelizumab, obexelimab, and epratuzumab), T-cell targeted drugs (abatacept, dapirolizumab, and inhibitor of syk and CaMKIV), cytokines targeted drugs (anifrolumab and sifalimumab), and natural products (curcumin, oleuropein, punicalagin, sulforaphane, icariin, apigenin, and resveratrol). The aim of this paper is to combine the existing in vitro and in vivo models and clinical research results to summarize the efficacy and mechanism of natural drugs and targeted drugs in SLE for the reference and consideration of researchers. [ABSTRACT FROM AUTHOR]

Purpose. Few related factors of low bone mass in Cushing's disease (CD) have been identified so far, and relevant sufficient powered studies in CD patients are rare. On account of the scarcity of data, we performed a well-powered study to identify related factors associated with low bone mass in young CD patients. Methods. This retrospective study included 153 CD patients (33 males and 120 females, under the age of 50 for men and premenopausal women). Bone mineral density (BMD) of the left hip and lumbar spine was measured by dual energy X-ray absorptiometry (DEXA). In this study, low bone mass was defined when the Z score was −2.0 or lower. Results. Among those CD patients, low bone mass occurred in 74 patients (48.37%). Compared to patients with normal BMD, those patients with low bone mass had a higher level of serum cortisol at midnight (22.31 (17.95-29.62) vs. 17.80 (13.75-22.77), p = 0.0006), testosterone in women (2.10 (1.33–2.89) vs. 1.54 (0.97–2.05), p = 0.0012), higher portion of male (32.43% vs. 11.54%, p = 0.0016) as well as hypertension (76.12% vs. 51.67%, p = 0.0075), and lower IGF-1 index (0.59 (0.43–0.76) vs. 0.79 (0.60–1.02), p = 0.0001). The Z score was positively associated with the IGF-1 index in both the lumbar spine (r = 0.35153, p < 0.0001) and the femoral neck (r = 0.24418, p = 0.0057). The Z score in the femoral neck was negatively associated with osteocalcin (r = −0.22744, p = 0.0229). Compared to the lowest tertile of the IGF-1 index (<0.5563), the patients with the highest tertile of the IGF-1 index (≥0.7993) had a lower prevalence of low bone mass (95% CI 0.02 (0.001–0.50), p = 0.0002), even after adjusting for confounders such as age, gender, duration, BMI, hypertension, serum cortisol at midnight, PTH, and osteocalcin. Conclusions. The higher IGF-1 index was independently associated with lower prevalence of low bone mass in young CD patients, and IGF-1 might play an important role in the pathogenesis of CD-caused low bone mass. [ABSTRACT FROM AUTHOR]

Concurrent infections in hematological malignancies (HM) are major contributors to adverse clinical outcomes, including prolonged hospitalization and reduced life expectancy. Individuals diagnosed with HM are particularly susceptible to infectious pathogens due to immunosuppression, which can either be inherent to the hematological disorder or induced by specific therapeutic strategies. Over the years, the treatment paradigm for HM has witnessed a tremendous shift, from broad-spectrum treatment approaches to more specific targeted therapies. At present, the therapeutic landscape of HM is constantly evolving due to the advent of novel targeted therapies and the enhanced utilization of these agents for treatment purposes. By initiating unique molecular pathways, these agents hinder the proliferation of malignant cells, consequently affecting innate and adaptive immunity, which increases the risk of infectious complications. Due to the complexity of novel targeted therapies and their associated risks of infection, it often becomes a daunting task for physicians to maintain updated knowledge in their clinical practice. The situation is further aggravated by the fact that most of the initial clinical trials on targeted therapies provide inadequate information to determine the associated risk of infection. In such a scenario, a cumulative body of evidence is paramount in guiding clinicians regarding the infectious complications that can arise following targeted therapies. In this review, I summarize the recent knowledge on infectious complications arising in the context of targeted therapies for HM. [ABSTRACT FROM AUTHOR]