Immune checkpoint pathways in glioblastoma: a diverse and evolving landscape.

Immune checkpoint (IC) inhibition in glioblastoma (GBM) has not shown promising results in the last decade compared to other solid tumors. Several factors contributing to the lack of immunotherapy response include the profound immunosuppressive nature of GBM, highly redundant signaling pathways underlying immune checkpoints, and the negative immunogenic impact of current standard of care on the tumor microenvironment. In this review, we will discuss various ICs in the context of GBM, their interplay with the tumor immune microenvironment, relevant pre-clinical and clinical studies, and the impact of current treatment modalities on GBM IC blockade therapy.

Similarities and differences between systemic juvenile idiopathic arthritis and adult-onset Still's disease: a multicenter Spanish study.

To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year.

Clinical characteristics and prognostic factor in juvenile dermatomyositis: data of the Spanish registry.

Background: Juvenile Dermatomyositis (JDM) is the most common chronic idiopathic inflammatory myopathy in children. The diagnosis is clinical. Baseline laboratory and complementary studies trace the phenotype of these patients.

Emerging applications of hypomethylating agents in the treatment of glioblastoma (Review).

DNA hypomethylating agents (HMAs) such as decitabine and 5-azacytidine have established roles in the treatment paradigms for myelodysplastic syndrome and acute myelogenous leukemia, where they are considered to exert their anticancer effects by restoring the expression of tumor suppressor genes. Due to their relatively favorable adverse effect profile and known ability to pass through the blood-brain barrier, applications in the treatment of glioblastoma (GBM) and other central nervous system malignancies are under active investigation. The present review examines the types of HMAs currently available, their known and less-understood antineoplastic mechanisms, and the evidence to date of their preclinical and clinical efficacy in glioblastoma and other solid malignancies.

What drives the decision to optimise biological treatment in children and youngsters with juvenile idiopathic arthritis? A discrete-choice experiment.

Objective: To analyse factors involved in the decision to optimise biologics in juvenile idiopathic arthritis.

Methods: A "discrete-choice" methodology was used. In a nominal group meeting, factors which may influence physicians' decisions to optimise biological dose were identified, together with decision nodes.

IgA deficiency and autoimmune comorbidities in Juvenile Idiopathic Arthritis.

Objectives: (1) To describe the prevalence of IgA deficiency (IgAD), uveitis, coeliac disease (CD) and thyroid disorders in a multicentric cohort of patients diagnosed with JIA and, (2) to evaluate whether patients with JIA and IgAD present other autoimmune diseases more frequently than patients with normal serum levels of IgA.

Methods: Retrospective chart review of a cohort of patients diagnosed with JIA followed at the paediatric rheumatology units of two hospitals in Madrid, Spain.

Results: A total of 193 patients were included.

Clinical characteristics and COVID-19 outcomes in a regional cohort of pediatric patients with rheumatic diseases.

Background: This study aimed to assess the baseline characteristics and clinical outcomes of coronavirus disease 2019 (COVID-19) in pediatric patients with rheumatic and musculoskeletal diseases (RMD) and identify the risk factors associated with symptomatic or severe disease defined as hospital admission, intensive care admission or death.

Methods: An observational longitudinal study was conducted during the first year of the SARS-CoV-2 pandemic (March 2020-March 2021). All pediatric patients attended at the rheumatology outpatient clinics of six tertiary referral hospitals in Madrid, Spain, with a diagnosis of RMD and COVID-19 were included.

Thromboelastography (TEG) results are predictive of ischemic and hemorrhagic complications in patients with unruptured intracranial aneurysms treated with flow diversion.

Introduction: Flow diversion is an effective treatment modality for intracranial aneurysms but is associated with ischemic and hemorrhagic complications. Patients treated with flow diversion require dual antiplatelet therapy and subsequent platelet function tests. At our institution, Thromboelastography with Platelet Mapping (TEG-PM) is the test of choice.

The incidence of venous thromboembolism following surgical resection of intracranial and intraspinal meningioma. A systematic review and retrospective study.

Introduction: Historically, the development of venous thromboembolism (VTE) including deep venous thrombosis (DVT) and pulmonary thromboembolism (PE) was cited as a higher post-operative risk for patients harboring meningiomas. However, recent literature has suggested that there may be no elevated risk for VTE among these patients. The authors perform both a retrospective review of their own cases as well as a systematic review of the literature in order to determine the frequency of the VTE and rate of post-operative hemorrhage in this patient population.

COVID-19 Infection Among Healthcare Workers: Serological Findings Supporting Routine Testing.

A growing body of evidence demonstrates that asymptomatic and pre-symptomatic transmission of SARS-CoV-2 is a major contributor to the COVID-19 pandemic. Frontline healthcare workers in COVID-19 hotspots have faced numerous challenges, including shortages of personal protective equipment (PPE) and difficulties acquiring clinical testing. The magnitude of the exposure of healthcare workers and the potential for asymptomatic transmission makes it critical to understand the incidence of infection in this population.

Epigenetic preconditioning with decitabine sensitizes glioblastoma to temozolomide via induction of MLH1.

Introduction: To improve the standard treatment paradigm for glioblastoma (GBM), efforts have been made to explore the efficacy of epigenetic agents as chemosensitizers. Recent data suggest possible synergy between decitabine (DAC), a DNA hypomethylating agent, and temozolomide (TMZ) in GBM, but the mechanism remains unclear. The objective of this study was to determine the effects of DAC on TMZ sensitization in a consecutively derived set of primary GBM cultures, with a focus on mismatch repair (MMR) proteins.

Phenotypic variability and disparities in treatment and outcomes of childhood arthritis throughout the world: an observational cohort study.

Background: To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status.

Methods: In this multinational, cross-sectional, observational cohort study, we asked international paediatric rheumatologists from specialised centres to enrol children with a diagnosis of juvenile idiopathic arthritis, according to International League of Associations for Rheumatology criteria, who were seen consecutively for a period of 6 months.

Acquired and Innate Immunity Impairment and Severe Disseminated Infection in a Patient With a NF-κB1 Deficiency.

NF-κB1 is a master regulator of both acquired and innate responses. loss-of-function mutations elicit a wide clinical phenotype with asymptomatic individuals at one end of the spectrum and patients with common variable immunodeficiency, combined immunodeficiency or autoinflammation at the other. Impairment of acquired and innate immunity and disseminated infection expands the clinical and immunological phenotype of NF-κB1 deficiency.

Pediatrician Beliefs about Juvenile Idiopathic Arthritis May Result in Referral Delays: A Spanish National Survey.

We explored, through a national survey, pediatrician beliefs and misconceptions that could interfere with early referral of patients with juvenile idiopathic arthritis. A total of 831 pediatricians participated. Approximately one-half of the respondents underestimated the incidence of the disease and thought that pain was the leading symptom of oligoarticular forms.

Unexpected relevant role of gene mosaicism in patients with primary immunodeficiency diseases.

Background: Postzygotic de novo mutations lead to the phenomenon of gene mosaicism. The 3 main types are called somatic, gonadal, and gonosomal mosaicism, which differ in terms of the body distribution of postzygotic mutations. Mosaicism has been reported occasionally in patients with primary immunodeficiency diseases (PIDs) since the early 1990s, but its real involvement has not been systematically addressed.

The Colombian Spanish version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Colombian Spanish language. The reading comprehension of the questionnaire was tested in ten JIA parents and patients.