Influence of Disease Type and Activity on Adalimumab Exposure in Children with Inflammatory Rheumatic Diseases.

Understanding adalimumab pharmacokinetics (PK) in pediatric inflammatory rheumatic diseases (PIRD) could facilitate individualized treatment strategies. This pharmacometric (PMX) analysis, utilizing prospectively collected data, aimed to develop a PMX model investigating associations between disease-specific factors and adalimumab exposure in PIRD. PK data originating from a prospective two-center study including 36 children with PIRD (weight IQR: 33-55 kg) receiving subcutaneous adalimumab (IQR: 30-40 mg biweekly; n = 28 at steady state, n = 8 after first dose, i.

Vaccination completeness in children with rheumatic diseases: A longitudinal, observational multicenter cohort study in Switzerland.

Introduction: Children with pediatric inflammatory rheumatic diseases (PRD) have an increased infection risk. Vaccinations are effective to avoid vaccine-preventable diseases. This study aimed to assess the vaccination completeness in Swiss PRD patients stratified by immunosuppressive treatment (IST).

Chronic Recurrent Multifocal Osteomyelitis in a Child's Hand: Case Report and Review.

We present a 9-year-old girl with persistent pain and swelling of the left wrist. X-ray, magnetic resonance imaging (MRI) and bone biopsy led to the diagnosis of chronic recurrent multifocal osteomyelitis (CRMO), affecting phalangeal and metacarpal bases and distal carpal bones on the ulnar side of the wrist. She was treated with non-steroidal anti-inflammatory drugs and complete remission with no long-term sequelae was achieved.

Estimating the prevalence of paediatric chronic pain in Switzerland: an online survey among paediatricians.

Background: Chronic pain is a frequent complaint in children and adolescents, with great international variation in prevalence. Paediatricians are usually the first-line contact for pain problems in children and might refer patients to other specialists or pain clinics where available. Prevalence estimates of paediatric chronic pain and paediatricians' care experiences in Switzerland are currently lacking.

Current practice of transitional care for adolescents and young adults in Swiss paediatric and adult rheumatology centres.

Background: About half of all children with rheumatic diseases need continuous medical care during adolescence and adulthood. A good transition into adult rheumatology is essential. Guidelines for a structured transition process have therefore been recommended by the European League Against Rheumatism (EULAR) and the Paediatric Rheumatology European Society (PReS).

Best Practice Recommendations for the Diagnosis and Management of Children With Pediatric Inflammatory Multisystem Syndrome Temporally Associated With SARS-CoV-2 (PIMS-TS; Multisystem Inflammatory Syndrome in Children, MIS-C) in Switzerland.

Following the spread of the coronavirus disease 2019 (COVID-19) pandemic a new disease entity emerged, defined as Pediatric Inflammatory Multisystem Syndrome temporally associated with COVID-19 (PIMS-TS), or Multisystem Inflammatory Syndrome in Children (MIS-C). In the absence of trials, evidence for treatment remains scarce. To develop best practice recommendations for the diagnosis and treatment of children with PIMS-TS in Switzerland.

Suboptimal vaccination coverage of recommended vaccines among French children with recurrent autoinflammatory fever syndromes: a study from the Juvenile Inflammatory Rheumatism cohort.

Introduction/objectives: To determine vaccination coverage among a French cohort of children with recurrent autoinflammatory fever syndromes (RFS).

Method: All RFS children aged 2 to 19 years from the Juvenile Inflammatory Rheumatism cohort and followed at the French Reference Center for Autoinflammatory Diseases, Versailles Hospital, were included in our observational study. Immunisation status at ages 2, 7 and 15 years and at the last outpatient visit was evaluated according to the standard French vaccine schedule and recommended supplementary vaccines for patients with immunosuppressive therapy.

A protein quality control pathway regulated by linear ubiquitination.

Neurodegenerative diseases are characterized by the accumulation of misfolded proteins in the brain. Insights into protein quality control mechanisms to prevent neuronal dysfunction and cell death are crucial in developing causal therapies. Here, we report that various disease-associated protein aggregates are modified by the linear ubiquitin chain assembly complex (LUBAC).

Arthritis and arthralgia as an adverse event following immunization: A systematic literature review.

Background: Arthritis and arthralgia are reported as adverse events following immunization with various vaccines.

Objective: To better understand current knowledge of arthritis and arthralgia as an adverse event following immunization.

Methods: A systematic literature review of Pubmed, Embase, and Cochrane Library was conducted.

Safety of biological agents in paediatric rheumatic diseases: A real-life multicenter retrospective study using the JIRcohorte database.

Objective: To analyse and report the incidence of side effects of biological agents in paediatric patients with inflammatory diseases using of real-life follow-up cohort.

Methods: In this international, observational, retrospective, multicentre study of children treated by biological agents and followed in the Juvenile Inflammatory Rheumatism (JIR) cohort (JIRcohorte) network, a Kaplan-Meier method was used to estimate the occurrence of adverse events. A Cox model was constructed to identify independent predictors of adverse events.

Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis.

Objective: To assess performance of the 2016 macrophage activation syndrome (MAS) classification criteria for patients with systemic juvenile idiopathic arthritis (JIA) who develop MAS while treated with biologic medications.

Methods: A systematic literature review was performed to identify patients with MAS while being treated with interleukin (IL)-1 and IL-6 blocking agents. Clinical and laboratory information was compared to a large previously compiled historical cohort.

Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA.

Amyloid-like protein aggregation is associated with neurodegeneration and other pathologies. The nature of the toxic aggregate species and their mechanism of action remain elusive. Here, we analyzed the compartment specificity of aggregate toxicity using artificial β-sheet proteins, as well as fragments of mutant huntingtin and TAR DNA binding protein-43 (TDP-43).

Complications of systemic juvenile idiopathic arthritis: risk factors and management recommendations.

Systemic juvenile idiopathic arthritis (SJIA) is an inflammatory condition characterized by fever, lymphadenopathy, arthritis, rash and serositis. Systemic inflammation has been associated with dysregulation of the innate immune system, suggesting that SJIA is an autoinflammatory disorder. IL-1 and IL-6 play a major role in the pathogenesis of SJIA, and treatment with IL-1 and IL-6 inhibitors has shown to be highly effective.

Pancreatic stone protein as a novel marker for neonatal sepsis.

Purpose: Early-onset sepsis (EOS) is one of the main causes for the admission of newborns to the neonatal intensive care unit. However, traditional infection markers are poor diagnostic markers of EOS. Pancreatic stone protein (PSP) is a promising sepsis marker in adults.