Assessing Competencies, Needs, and Satisfaction With the Transition From Pediatric to Adult Health Care in Rheumatology: Development and Validation of the Transition-KompAZ.

Objective: The aim of this study was to develop and psychometrically validate a self-report instrument to assess (1) competencies, (2) needs, and (3) satisfaction among youth transitioning from pediatric to adult rheumatology.

Methods: The Transition-KompAZ was developed in several steps with conceptual and psychometric analyses. To test its psychometric properties, the instrument was administered to adolescents and young adults (AYAs; 16-25 years) with inflammatory rheumatic diseases before (group 1) or after (group 2) transfer to adult rheumatology.

Level and correlates of physical activity among children and adolescents with juvenile idiopathic arthritis compared to controls: results from a German nationwide prospective observational cohort study.

Background: Physical active lifestyles are essential throughout growth and maturation and may offer potential preventive and therapeutic benefit in patients with juvenile idiopathic arthritis (JIA). Insufficient physical activity (PA), in contrast, can lead to aggravation of disease-related symptoms. This study aimed to i) examine PA levels in children and adolescents with JIA compared to general population controls and ii) investigate correlates of pronounced physical inactivity in order to identify risk groups for sedentary behaviour.

[Analysis of movement disorders in paediatric and adolescent rheumatology].

Rheumatic diseases in childhood and adolescence like juvenile idiopathic arthritis can cause movement disorders due to pain, swelling and limited range of motion. This article describes different possibilities and results of movement analysis for rheumatic diseases. The influence of JIA on specific movements in individual joints and complex movements such as gait is examined.

Health-related quality of life, continuity of care and patient satisfaction: long-term outcomes of former patients of the Tuebingen Transition Program (TTP) - a retrospective cohort study.

Background: A significant number of patients in pediatric rheumatology suffer from ongoing disease activity into adulthood and thus need to be transferred into adult care. Transition as a structured individual process of preparation and patient empowerment can reduce risks of adverse long-term outcomes. The aim of this study was to measure long-term transition outcomes such as health-related quality of life (HR-QoL), patient satisfaction, and continuity of care in former patients of the interdisciplinary Tuebingen Transition Program (TTP).

New variant in the IL1RN-gene (DIRA) associated with late-onset, CRMO-like presentation.

Objective: To report a chronic recurrent multifocal osteomyelitis (CRMO)-like clinical phenotype with multisystem inflammation associated with a novel gene variant in the spectrum of IL-1-mediated diseases.

Methods: A 3-year-old boy presented with recurrent episodes of fever, serositis, pancreatitis and high inflammatory markers with onset at age 13 months. At age 3 years, he started limping.

Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative.

Background: Rare autoinflammatory diseases (AIDs) including Cryopyrin-Associated Periodic Syndrome (CAPS), Tumor Necrosis Receptor-Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency Syndrome (MKD)/ Hyper-IgD Syndrome (HIDS) are genetically defined and characterized by recurrent fever episodes and inflammatory organ manifestations. Early diagnosis and early start of effective therapies control the inflammation and prevent organ damage. The PRO-KIND initiative of the German Society of Pediatric Rheumatology (GKJR) aims to harmonize the diagnosis and management of children with rheumatic diseases nationally.

Living with autoinflammatory diseases: identifying unmet needs of children, adolescents and adults.

Background: Autoinflammatory diseases (AIDs) illnesses of the innate immunity resulting in clinical signs and symptoms of systemic inflammation and loss of organ functions. While pathophysiological mechanisms are heavily studied and increasingly well understood, psychosocial needs are much less explored. The disease impact on the everyday life of patients including school and work is poorly studied.

Recommendations for collaborative paediatric research including biobanking in Europe: a Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative.

Innovative research in childhood rheumatic diseases mandates international collaborations. However, researchers struggle with significant regulatory heterogeneity; an enabling European Union (EU)-wide framework is missing. The aims of the study were to systematically review the evidence for best practice and to establish recommendations for collaborative research.

Induction of Myeloid-Derived Suppressor Cells in Cryopyrin-Associated Periodic Syndromes.

Cryopyrin-associated periodic syndromes (CAPS) are caused by mutations in the NLRP3 gene leading to overproduction of IL-1β and other NLRP3 inflammasome products. Myeloid-derived suppressor cells (MDSCs) represent a novel innate immune cell subset capable of suppressing T-cell responses. As inflammasome products were previously found to induce MDSCs, we hypothesized that NLRP3 inflammasome-dependent factors induce the generation of MDSCs in CAPS.

Early detection of sensorineural hearing loss in Muckle-Wells-syndrome.

Background: Muckle-Wells-syndrome (MWS) is an autoinflammatory disease characterized by systemic and organ-specific inflammation due to excessive interleukin (IL)-1 release. Inner ear inflammation results in irreversible sensorineural hearing loss, if untreated. Early recognition and therapy may prevent deafness.

Dynamic knee joint function in children with juvenile idiopathic arthritis (JIA).

Background: Juvenile idiopathic arthritis (JIA) is a chronic illness with a high risk of developing long-term disability. Disease activity is currently being monitored and quantified by ACR core set. Here, joint inflammation is determined; however joint function is the crucial component for developing disability.

Treatment of Muckle-Wells syndrome: analysis of two IL-1-blocking regimens.

Objectives: Muckle-Wells syndrome (MWS) is an autoinflammatory disease characterized by excessive interleukin-1 (IL-1) release, resulting in recurrent fevers, sensorineural hearing loss, and amyloidosis. IL-1 inhibition with anakinra, an IL-1 receptor antagonist, improves clinical symptoms and inflammatory markers. Subclinical disease activity is commonly observed.

Working with Hispanic women who are deaf: recommendations from the literature.

The authors used the hermeneutics approach within social cognitive career theory to explore employment trends and issues over the past 20 years relevant to Hispanic women who are deaf. Barriers to employment were discovered including discrepancies due to gender, race/ethnicity, and severity of hearing loss. Recommendations for policymakers and professionals suggest a need for research that addresses the unique experiences of Hispanic women who are deaf within vocational rehabilitation, the use of social cognitive career theory as it relates to career development and choices, and an increase in education and training to better prepare vocational rehabilitation counselors to work with individuals from multiple minority backgrounds.

Hearing loss in Muckle-Wells syndrome.

Objective: Muckle-Wells syndrome (MWS) is an inherited autoinflammatory disease characterized by fevers, rashes, arthralgia, conjunctivitis, and sensorineural hearing loss. In MWS, NLRP3 gene mutations are associated with excessive interleukin-1 release. The aims of this study were to determine the otologic characteristics of MWS, define trajectories of hearing loss, and explore the association with distinct NLRP3 genotypes.

Progressive familial hearing loss in Muckle-Wells syndrome.

Conclusion: The age-dependent disease progression requires accelerating diagnosis of Muckle-Wells syndrome (MWS) in order to start treatment as early as possible. The most frequent, but not obligate symptoms are familial fatigue, hearing loss, and arthralgia. The design of further clinical trials should focus on hearing in order to document the long-term effect of anti-interleukin (IL)-1 drugs on hearing preservation.

The Toll-like receptor 4 agonist MRP8/14 protein complex is a sensitive indicator for disease activity and predicts relapses in systemic-onset juvenile idiopathic arthritis.

Background: Analysis of myeloid-related protein 8 and 14 complex (MRP8/14) serum concentrations is a potential new tool to support the diagnosis of systemic-onset juvenile idiopathic arthritis (SJIA) in the presence of fever of unknown origin.

Objective: To test the ability of MRP8/14 serum concentrations to monitor disease activity in patients with SJIA and stratify patients at risk of relapse.

Methods: Serum concentrations of MRP8/14 in 52 patients with SJIA were determined by a sandwich ELISA.

Impaired suppression of synovial fluid CD4+CD25- T cells from patients with juvenile idiopathic arthritis by CD4+CD25+ Treg cells.

Objective: Natural CD4+CD25+FoxP3+ Treg cells play a crucial role in maintaining immune homeostasis and controlling autoimmunity. In patients with juvenile idiopathic arthritis (JIA), inflammation occurs despite the increased total numbers of Treg cells in the synovial fluid (SF) compared to the peripheral blood (PB). This study was undertaken to investigate the phenotype of CD4+ T cells in PB and SF from JIA patients, the function of synovial Treg cells, and the sensitivity of PB and SF CD4+CD25- effector T cells to the immunoregulatory properties of Treg cells, and to study the suppression of cytokine secretion from SF effector T cells by Treg cells.

Psychosocial variables related to the adoption of video relay services among deaf or hard-of-hearing employees at the Texas School for the Deaf.

Assistive technology (AT) can help individuals with disabilities address a range of barriers and increase community and work participation, yet many devices are abandoned soon after acquisition. Video Relay Service (VRS) is a new communication technology available to people who are deaf or hard of hearing, but little is known about VRS adoption and use by intended consumers. Previous research suggests that psychosocial factors may have significant impact on adoption and use of AT, thus a nonexperimental research design was used to investigate the impact of psychosocial and demographic variables on adoption of VRS by deaf or hard-of-hearing adults.