Post-intervention Status in Patients With Refractory Myasthenia Gravis Treated With Eculizumab During REGAIN and Its Open-Label Extension

OBJECTIVE: To evaluate whether eculizumab helps patients with anti-acetylcholine receptor-positive (AChR+) refractory generalized myasthenia gravis (gMG) achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifestations (MM), we assessed patients' status throughout REGAIN (Safety and Efficacy of Eculizumab in AChR+ Refractory Generalized Myasthenia Gravis) and its open-label extension. METHODS: Patients who completed the REGAIN randomized controlled trial and continued into the open-label extension were included in this tertiary endpoint analysis. Patients were assessed for the MGFA post-intervention status of improved, unchanged, worse, MM, and pharmacologic remission at defined time points during REGAIN and through week 130 of the open-label study. RESULTS: A total of 117 patients completed REGAIN and continued into the open-label study (eculizumab/eculizumab: 56; placebo/eculizumab: 61). At week 26 of REGAIN, more eculizumab-treated patients than placebo-treated patients achieved a status of improved (60.7% vs 41.7%) or MM (25.0% vs 13.3%; common OR: 2.3; 95% CI: 1.1-4.5). After 130 weeks of eculizumab treatment, 88.0% of patients achieved improved status and 57.3% of patients achieved MM status. The safety profile of eculizumab was consistent with its known profile and no new safety signals were detected. CONCLUSION: Eculizumab led to rapid and sustained achievement of MM in patients with AChR+ refractory gMG. These findings support the use of eculizumab in this previously difficult-to-treat patient population. CLINICALTRIALSGOV IDENTIFIER: REGAIN, NCT01997229; REGAIN open-label extension, NCT02301624. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that, after 26 weeks of eculizumab treatment, 25.0% of adults with AChR+ refractory gMG achieved MM, compared with 13.3% who received placebo

Minimal Symptom Expression' in Patients With Acetylcholine Receptor Antibody-Positive Refractory Generalized Myasthenia Gravis Treated With Eculizumab

The efficacy and tolerability of eculizumab were assessed in REGAIN, a 26-week, phase 3, randomized, double-blind, placebo-controlled study in anti-acetylcholine receptor antibody-positive (AChR+) refractory generalized myasthenia gravis (gMG), and its open-label extension

Central Sensitization and Associated Factors in Adolescents With Joint Hypermobility and Dysautonomia

Background: Postural Orthostatic Tachycardia Syndrome (POTS) is a disorder of the autonomic nervous system that has high association with chronic pain syndromes such as fibromyalgia, migraine disorders, and chronic abdominal pain in adolescents with the diagnosis. Many of these disorders are characterized as central sensitization disorders, or pathological pain memory mediated by neural plasticity. Ehlers Danlos Syndrome Type 3 (EDS-3), also called joint hypermobility syndrome (JHS) is a genetic disorder of the connective tissue that causes joint laxity and is also highly associated with chronic pain syndromes as well as POTS. Methods: This study proposed to characterize POTS as a disorder of central sensitization. The hypothesis, presented within the proposed theoretical model, demonstrates that JHS leads to chronic pain that results in central sensitization and autonomic nervous system dysfunction (POTS). Other factors that were evaluated were anxiety and function. A sample size of 40 adolescents between the ages of 12 and 19 years were recruited from the cardiology and pain clinics at Children’s National Medical Center. Analysis of data utilizing Wilcoxon, Chi square, Pearson correlation, and logistic regression tests were completed using SAS 9.3. Results: In comparison to those without POTS, there were no significant associations found between having the diagnosis of POTS and any other variable studied in the model. JHS had a stronger correlation with anxiety, central sensitization, both subjectively, and objectively with hyperalgesia on Aδ sensory nerve fiber when compared to those without JHS. Subjective central sensitization was highly correlated with anxiety, function, age, and female gender. Function and central sensitization had a significant association even when removing anxiety as a covariate. Conclusions: These findings suggest that joint hypermobility may be a factor that contributes to the development of central sensitization in individuals with chronic pain. Dysautonomia is likely not a disorder of central sensitization, but rather a variable related to joint hypermobility and chronic pain in ways yet to be discovered. As previously discussed in other literature, anxiety has strong associations with central sensitization and functional disability in chronic pain syndromes, and when treated effectively may increase function in those that suffer with these disorders

Pain is a Prevalent Symptom of Adolescents at Diagnosis with Postural Orthostatic Tachycardia Syndrome

Pain is often endorsed by patients with postural orthostatic tachycardia syndrome (POTS). A retrospective chart analysis of adolescents diagnosed with PTS from January 2014-December 2015 at the pediatric cardiology clinic at Children’s National Health System were reviewed. 93% of the subjects reported pain at the time of diagnosis, with 46% of the sample reporting more than one site of pain. Since chronic pain syndromes and POTS are associated by similar symptom burden and often develop after illness or injury, both may be a product of pathology stemming from neuroinfl ammation and neuralplasticity that results in central sensitization and autonomic nervous system dysfunction.</p

sj-docx-1-rse-10.1177_07419325221135613 – Supplemental material for Momentary Affective Experiences of Teachers Serving Students With Emotional and Behavioral Disabilities in Self-Contained Settings

Supplemental material, sj-docx-1-rse-10.1177_07419325221135613 for Momentary Affective Experiences of Teachers Serving Students With Emotional and Behavioral Disabilities in Self-Contained Settings by Kristabel Stark, Elizabeth Bettini and Olivia Chi in Remedial and Special Education</p

Os processos de formação na Política Nacional de Humanização: a experiência de um curso para gestores e trabalhadores da atenção básica em saúde Formation processes within the National Humanization Politics: the experience of a course for managers and workers in primary health care

Este artigo tem como objetivo relatar a experiência de um curso de formação da Política Nacional de Humanização voltado para gestores e trabalhadores da atenção básica de um município no estado do Rio de Janeiro. O curso visou a formação de apoiadores institucionais capazes de fomentar rede no Sistema Único de Saúde (SUS), promover mudanças e consolidação nos modos de atenção e de gestão dos serviços. Como referencial metodológico, buscou-se um modo de "formação-intervenção" que fosse baseado em práticas concretas de intervenção dos trabalhadores nos processos de trabalho em saúde. O curso envolveu quarenta participantes, gestores e trabalhadores de nível médio e superior, ligados à atenção básica, oriundos da Estratégia de Saúde da Família e de Unidades de Saúde. Como resultados destacam-se ações de co-gestão no formato de reuniões com os usuários para o compartilhamento de decisões relativas ao serviço; implementação de acolhimento, com intervenções que garantam o acesso do usuário ao serviço; e de clínica ampliada, com discussões em equipe dos casos clínicos; e ações no campo da saúde do trabalhador, como efeito das discussões dos processos de trabalho nas equipes multiprofissionais.<br>This paper aims to report the experience of a training course of the National Humanization Politics facing managers and workers of primary care in a municipality in the State of Rio de Janeiro. The course aimed at training of institutional supporters that will encourage networking in the Unified Health System (SUS), advocating for change and consolidation in the modes of attention and service management. In the methodology, we sought a way of "training area" that was based on practical concrete action by employees for health work. The course involved forty participants, managers and employees of medium and higher levels are linked to primary care, resulting from the strategy of the Family Health Units and Health The results highlight comanagement actions in the form of meetings with users to share decisions on the service, implementing a host of measures to ensure the User access the service, and extended clinic, with team discussions of clinical cases, and actions in the field of occupational health, the effect of the discussions work processes in multidisciplinary teams

sj-docx-1-ecx-10.1177_00144029221146576 - Supplemental material for High-Quality Systematic Literature Reviews in Special Education: Promoting Coherence, Contextualization, Generativity, and Transparency

Supplemental material, sj-docx-1-ecx-10.1177_00144029221146576 for High-Quality Systematic Literature Reviews in Special Education: Promoting Coherence, Contextualization, Generativity, and Transparency by Michelle M. Cumming, Elizabeth Bettini and Jason C. Chow in Exceptional Children</p

sj-docx-2-ecx-10.1177_00144029221146576 - Supplemental material for High-Quality Systematic Literature Reviews in Special Education: Promoting Coherence, Contextualization, Generativity, and Transparency

Supplemental material, sj-docx-2-ecx-10.1177_00144029221146576 for High-Quality Systematic Literature Reviews in Special Education: Promoting Coherence, Contextualization, Generativity, and Transparency by Michelle M. Cumming, Elizabeth Bettini and Jason C. Chow in Exceptional Children</p