BackgroundGeneralized myasthenia gravis (gMG) is a rare, chronic autoimmune disorder that imposes a substantial disease burden in China. As novel therapeutic options emerge, understanding patient preferences has become essential for treatment decision-making. This study aimed to assess treatment preferences and willingness to pay (WTP) among Chinese gMG patients.MethodsThis multicenter, cross-sectional survey was conducted from March to August 2025 among patients with gMG in China. A discrete choice experiment was used to quantify patient preferences by presenting hypothetical treatment scenarios defined by eight key attributes. A mixed Logit regression model was used to identify preference drivers, and WTP was estimated to reflect the monetary value assigned to changes in treatment attributes.ResultsAmong the 909 analyzed patients, the mean age was 48.9 ± 13.27 years, and 61.8% were female. Within the selected attribute sets, safety was a key determinant of patient preferences, with consistently favored for lower risks of adverse drug reactions (ADRs) on metabolic diseases, infection, myelosuppression, and liver and kidney function impairment; WTP increased as risk levels decreased. Patients preferred oral administration, followed by subcutaneous injection and intravenous infusion. Infrequent dosing, once every-6-month, weekly or daily, was preferred over 2–3 times daily. Faster onset time of action (≤2 weeks) was favored, and higher out-of-pocket costs were disfavored. Subgroup analyses revealed young patients valued once weekly dosing, subcutaneous injection, and low risk of liver and kidney function impairment than old patients. Patients with high disease burden showed similar preferences for low and moderate ADRs risk. High-income patients favored low ADRs risk, infrequent dosing, subcutaneous injection, and were less sensitive to cost than to low- and medium-income patients.ConclusionThis large-scale study was the first to investigated treatment preferences and WTP among Chinese patients with gMG. Within the selected attribute sets, patients prioritized safety, infrequent and convenient administration route, affordability, and rapid onset of action. Patients were willing to invest financially in therapies that align with these preferences. Incorporating patient preferences in clinical decision-making and reimbursement policy may improve adherence, reduce disease burden, and enhance quality of life for individuals with gMG in China.

BACKGROUND:Myasthenic crisis (MC) affects 10%-20% of myasthenia gravis patients and is life-threatening with significant economic burden, yet its long-term outcomes remain poorly understood. This prospective multicentre study aimed to evaluate 1-year clinical outcomes after MC. METHODS:This study enrolled patients with MC between December 2018 and October 2024 and prospectively followed at 1, 2, 3, 6, and 12 months post-MC. Outcome measures included Myasthenia Gravis Foundation of America (MGFA) postintervention status (PIS) classification at 1 year after MC, all-cause mortality, and complications. RESULTS:The cohort comprised 277 patients with 282 MC episodes with a follow-up duration of 29.16 ± 15.93 months. The in-hospital mortality was 5.05% (14/277), while the all-cause mortality was 15.16% (42/277). The leading causes of death were septic shock (35.7%) and multiple organ failure (21.4%). Among 247 patients with complete 1-year follow-up, 79.76% (197/247) achieved favorable outcomes. MG-ADL scores reduced from 21.3 ± 2.1 at baseline to 5.5 ± 1.5 at 6 months, and 2.8 ± 1.1 at 1 year post-MC (p < 0.01). Multivariable analysis identified older age at crisis (OR 1.03, p = 0.003), thymoma comorbidity (OR 1.84, p = 0.041), higher comorbidity burden (Charlson Comorbidity Index ≥ 3, OR 2.68, p = 0.003), and prolonged ICU stay (OR 1.03, p = 0.002) as independent predictors for minimal symptom expression non-responders. The most frequent chronic complications included infections (15.2%; predominantly fungal pathogens), gastrointestinal disorders (8.3%), and osteoporosis/fractures (5.1%). CONCLUSIONS:This is the first prospective cohort study to report clinical outcomes in MG patients at post-MC stage, highlighting the importance of disease monitoring and early intervention to improve patient-centred care.

ObjectiveTo construct Omaha system⁃based nursing intervention program for caregiver burden in primary caregivers of parkinson's disease patients undergoing deep brain stimulation(DBS).MethodsThe initial draft of Omaha system-based nursing intervention program for caregiver burden in primary caregivers of parkinson's disease patients undergoing DBS was developed through literature review.Expert consultations were conducted.The program was revised based on experts' feedback.ResultsTwo rounds of expert consultations were performed.The valid questionnaire response rate for both rounds of expert consultations was 100%.The expert authority coefficients were 0.88 and 0.87,respectively.In the second round of consultations,the importance scores for each item in the problem classification of the Omaha system⁃based nursing intervention program for caregiver burden in primary caregivers of parkinson's disease patients undergoing DBS ranged from 4.00 to 4.90.The coefficient of variation(CV) ranged 0.06 to 0.24.For the intervention categories,the importance scores ranged from 4.10 to 4.70,and CV ranged 0.10 to 0.22.The final Omaha system-based nursing intervention program for caregiver burden in primary caregivers of parkinson's disease patients undergoing DBS consisted of 3 parts,namely problem classification,intervention categories,and intervention measures.The problem classification table included 4 first⁃level items,17 second⁃level items,and 31 third⁃level items.The intervention categories table included 4 first⁃level items and 7 second⁃level items.The intervention measures table comprised 7 intervention strategies and 22 intervention details.ConclusionsThe Omaha system⁃based nursing intervention program for caregiver burden in primary caregivers of parkinson's disease patients undergoing DBS demonstrated scientific rigor,clinical specificity,and sustainability.

Impending Myasthenic Crisis (MC) is defined as a rapid worsening of myasthenia gravis (MG) that can progress to respiratory failure within days to weeks. The clinical data regarding the outcome and peripheral immune profile is limited. This multicenter cohort enrolled 37 patients with impending MC who were given timely rescue therapies from six university hospitals (n = 272). The transformation rate in the impending MC cohort to manifest MC was 24.32 % (9/37). Compared with those who have transformed to MC, the untransformed patients manifested a shorter hospital stay (13.98 ± 15.22 vs. 22.00 ± 16.03 days, P = 0.004), a lower baseline MG Quality of Life 15 (QOL-15) (23.42 ± 14.32 vs. 38.44 ± 13.56, P = 0.006), and shorter disease duration (29.30 ± 46.05 vs. 88.00 ± 71.43 months, P = 0.003). Peripheral CD4+ T profiling revealed an elevated Th17 proportion in impending crisis compared with healthy controls (22.33 ± 6.58 vs. 15.06 ± 4.35, P = 0.0006). Impending MC can be effectively prevented from transforming into manifest MC with rescue therapies.

Myasthenia gravis (MG) is a rare neuromuscular disorder that causes muscle weakness and fatigue. This review evaluated the current evidence on the efficacy, tolerance and adherence of rehabilitative interventions. Based on the PRISMA guidelines, 445 articles were identified from major scientific databases. After applying the inclusion and exclusion criteria, 6 studies were included in the final analysis. Two primary rehabilitative strategies - physical and respiratory training - were identified. The results of the systematic review and meta-analysis showed that both interventions improved functional capacity and enhanced the quality of life, with good tolerance and high adherence. This review summarizes the effects of rehabilitative interventions on improving 6-min walk distance (6MWD), quality of life (MG-QOL15), and respiratory function (FEV1 and FVC) in MG patients. One study was rated as high quality (OCEBM level 1b), and five as moderate quality (OCEBM level 2b). Five findings were established and rated as Grade B, according to the OCEBM recommendations. This review demonstrated the potential of structured rehabilitation programs to improve functioning and quality of life in patients with MG, while highlighting the need for further research to optimize and standardize these interventions.

Background: Myasthenic crisis (MC) is a life-threatening condition for myasthenia gravis (MG). Therapeutic plasma exchange (TPE) and intravenous immunoglobulin (IVIg) efficaciously treat patients with MC. However, not every MC responds well to rescue therapies, and the determinants for outcome with the evidence from prospective cohorts are still lacking. Objectives: To explore the risk factors for in-hospital outcomes in patients with MC. Methods: Using a national neuromuscular center-based cohort of MG with prospective follow-ups from the crisis to the post-crisis phase, we finally included 90 MC episodes from 76 independent patients who received a standard regimen of rescue therapies. Results: The mean admission age was 52.89 ± 15.72 years. With a female predominance of 63.16% (48/76) and a high proportion of thymoma-associated MG (TMG) of 63.16% (48/76), the overall in-hospital mortality was 2.63% (2/76) and the average duration for mechanical ventilation (MV) use was 17.09 ± 13.36 days (0–53 days). In contrast to the patients with anti-acetylcholine receptor (AChR) antibodies, muscle-specific tyrosine kinase (MuSK)-associated MC exhibited a shorter MV support (5.20 ± 5.07 versus 17.40 ± 13.24 days, p = 0.023), length of intensive care units (ICU) stay (6.00 ± 4.64 versus 19.16 ± 17.54 days, p = 0.046), and hospital stay (16.00 ± 4.12 versus 34.43 ± 20.48 days, p = 0.011). Thymoma [odds ratio (OR): 0.200, 95% confidence interval (CI): 0.058–0.687, p = 0.011], partial pressure of carbon dioxide (PCO 2 ) in blood gas before MV (OR: 1.238, 95% CI: 1.015–1.510, p = 0.035), and pneumonia (OR: 0.204, 95% CI: 0.049–0.841, p = 0.028) were identified as independent risk factors for prolonged MV use. TMG patients with thymoma burden exhibited a notable longer MV use (22.08 ± 17.54 versus 8.88 ± 6.79 days, p = 0.001), a prolonged hospital stay (40.40 ± 26.13 versus 23.67 ± 13.83 days, p = 0.009) compared with non-TMG. Even with complete thymoma resection (R0), TMG exhibited an unfavorable outcome versus non-TMG. Conclusion: With timely rescue therapies and prospective follow-ups, the in-hospital outcome of MCs was substantially improved. Thymoma, PCO 2 in blood gas before MV, and pneumonia were identified as independent risk factors for prolonged MV use.

PURPOSE:This study summarized characteristics and risk factors of caregiver burden in PD patients and used meta-analysis to verify the effectiveness of the intervention on caregiver burden.METHODS:Systematic review and meta-analysis were conducted.RESULTS:Forty-nine articles that involved 5387 caregivers of patients with PD were included in this study. Results of systematic review indicated that Zarit burden Inventory (ZBI) was the most used scale to measure the caregiver burden. All scales revealed caregivers of PD patients had mild to moderate caregiver burden. For the PD patients with longer disease duration, severer disease severity, more negative emotion and cognition impairment, their caregivers intended to have higher caregiver burden. The caregiver with negative emotion and who spent more time on caregiving indicated higher caregiver burden than the others. The caregiver burden was not improved after deep brain stimulation (DBS). Meta-analysis showed that cognitive behavior therapy and palliative care had no significant effect to reduce caregiver burden in PD patients' caregiver.CONCLUSION:Caregivers of PD patients experienced mild to moderate caregiver burden. Demographic factor, diseased-related factor and negative emotional factor were the risk factors of caregiver burden. Health education and care support for long-term management after DBS surgery should be provided for patients and caregivers to decrease caregiver burden.

重症肌无力危象(MC)是重症肌无力(MG)患者出现呼吸衰竭、需要机械呼吸支持的最危急状态,具有较高的在院病死率和较长的ICU住院时间.在神经重症监护室内建立"危象单元",由神经内科、重症医学科、感染科、输血科、心内科、护理部和康复医学科等多学科团队组成医疗综合体,从气道管理、快速疗法、重症肺炎的监测和预防、身心护理及综合康复等方面,为MC患者提供高效的个体化治疗.文中介绍复旦大学附属华山医院的单中心经验,以探讨MC患者管理的多学科诊疗协作模式.

目的 探讨志愿者式专业社会实践对本科护生的影响.方法 招募2018级本科三年级下学期护生志愿者22人,在学院老师的组织下,以2～3人一小组由临床老师指导,分别对12例居家神经肌病患者实行上门护理服务.每2周上门1次,共3次,每次活动2h左右.每次实践结束书写1篇反思日志.结果 实践前后护生的一般自我效能感、临床沟通能力得分比较,差异无统计学意义(均P＞0.05);反思日志提炼出"看到自己:收获与不足;看到他人:榜样的力量,敬畏生命,重视心理护理"2个主题.结论 短期专业社会实践对提高护生一般自我效能感和沟通能力的作用有限,但可有效帮助护生自我成长,提高对他人的客观认知.

The impact of Omicron infections on the clinical outcome and immune responses of myasthenia gravis (MG) remained largely unknown. From a prospective multicenter MG cohort (n = 189) with 197 myasthenic crisis (MC), we finally included 41 independent MG patients to classify into two groups: the Omicron Group (n = 13) and the Control Group (n = 28). In this matched cohort study, all-cause mortality was 7.69% (1/13) in Omicron Group and 14.29% (4/28) in Control Group. A higher proportion of elevated serum IL-6 was identified in the Omicron Group (88.89% vs 52.38%, P = 0.049). In addition, the proportions of CD3+CD8+T in lymphocytes and Tregs in CD3+CD4+ T cells were significantly elevated in the Omicron Group (both P = 0.0101). After treatment, the Omicron Group exhibited a marked improvement in MG-ADL score (P = 0.026) and MG-QoL-15 (P = 0.0357). MCs with Omicron infections were associated with elevated serum IL-6 and CD3+CD8+T response. These patients tended to present a better therapeutic response after fast-acting therapies and anti-IL-6 treatment.

目的 探讨多发性硬化（MS）患者不同疾病修正治疗（DMT）药物与严重新型冠状病毒（简称新冠病毒）感染风险的相关性。方法 选择截止2023年1月31日于泛长三角MS诊疗协作组各医疗中心接受治疗的MS患者，通过问卷形式收集2022年12月至2023年1月中国新冠病毒感染高峰期间患者的感染情况，进行回顾性和横断面分析。结果336例MS患者的平均年龄（34.14±10.21）岁，女性70.54%。治疗包括：特立氟胺86例，鞘氨醇-1-磷酸受体调节剂99例，CD20单抗37例，其他治疗43例，未接受任何治疗71例。接种过新冠病毒疫苗138例（41.07%），发生新冠病毒感染244例（72.62%）。最常见的感染症状有发热、咳嗽、疲劳；21例发生严重新冠病毒感染，其中15例经肺部CT诊断为肺炎，需急诊或住院治疗9例，1例入住ICU,2例使用呼吸机。6例在新冠病毒感染后发生MS复发。不同DMT药物对新冠病毒严重感染的风险差异无显著性（P=0.671）。未接种新冠病毒疫苗的MS患者发生严重新冠病毒感染的风险是接种疫苗的患者的2.37倍（P=0.034）；无法独立行走的MS患者发生严重新冠病毒感染的风险是能独立行走患者的3.26倍，但差异无显著性（P=0.052）。结论 MS患者感染新冠病毒后转化为严重感染及导致复发的风险较低；不同DMT药物在新冠病毒流行期间具有较高安全性；未接种新冠疫苗及无法自主行走的MS患者严重新冠病毒感染的风险增加，推荐MS患者及时接种新冠疫苗并接受规范治疗。

AbstractBackgroundNeuromyelitis optica spectrum disorder (NMOSD) is a chronic inflammatory demyelinating disease of the central nervous system with both a long course and high disability rate. It can cause different degrees of neurological impairment, resulting in the increased perception of stress in the patient. Health-related hardiness is psychological defense against stressors in cases of illness. The study was to investigate the role of health-related hardiness between disability and perceived stress in NMOSD patients and conduct health-related hardiness education for NMOSD patients to observe changes in health-related hardiness and perceived stress.MethodsThe study investigated disability status, health-related hardiness, and perceived stress in NMOSD patients. The mediating effect of health-related hardiness between disability status and perceived stress was verified. Additionally, health-related hardiness education was used as an intervention to improve the health-related hardiess. The Extended Disability Status Score (EDSS), Health-Related Hardiness Scale (HRHS), and Perceived Stress Scale (PSS) were used to assess patient disability status, health-related hardiness, and perceived stress.Results128 patients were completed the investigation of HRHS, EDSS and PSS. 69 patients were enrolled in the health-related hardiness education study. NMOSD patients exhibited high levels of perceived stress. Health-related hardiness played a mediating role between disability and perceived stress, with mediating effect accounting for 12.7% of the total effect. After patients received health-related hardiness education, the score of health-related hardiness were significantly elevated and perceived stress scores were significantly lower.ConclusionHealth-related hardiness had a partial mediating effect between disability and perceived stress in NMOSD patients. Health-related hardiness education could effectively increase health-related hardiness, as well reducing perceived stress caused by NMOSD.

ABSTRACT Objectives: This project determined current compliance with best practice recommendations for self-management in patients with multiple sclerosis (MS) and used a web-based intervention to implement strategies to improve the quality of self-management in discharged patients with MS. Methods: Guided by the JBI Evidence-based Model of Health care, this project applied the 7 phases of the JBI Evidence Implementation Framework to improve the quality of self-management in MS patients. Results: After implementation, compliance significantly improved across all criteria compared with the baseline audit. All patients were assessed to determine their ability to self-manage (Criterion 1). All health care providers were trained to facilitate self-management and behavioral change (Criterion 2). All patients had self-management goals and action plans that were set together by both health care providers and patients (Criteria 3, 5, 6, 7). All patients received education through online patient education courses and were given an education handbook (Criterion 4). The agreed action plan was documented (Criterion 8). All members of the multidisciplinary team coordinated the services required by the patients (Criterion 9) and provided appropriate support to help patients achieve their goals and solve problems through a WeChat group and the communication module on the self-management online platform (Criterion 10). Conclusion: This implementation project effectively promoted practice change by enhancing patients’ knowledge of self-management and staff awareness of delivering self-management support to MS patients.

Background A growing number of studies show that integrated health care provides comprehensive and continuous care to patients with hypertension or diabetes. However, there is still no consensus about the effect of integrated health care on patients with hypertension or diabetes. The objective of this study was to verify the effectiveness of integrated health care for patients with hypertension or diabetes by using a systematic review and meta-analysis. Methods The study searched multiple English and Chinese electronic databases. The search period was from database inception to 31 October 2020. Systematic reviews and meta-analyses were conducted after assessing the risk of bias of each study. Results Sixteen studies that involved 5231 patients were included in this study. The results of the systematic review revealed that systolic blood pressure (SBP), diastolic blood pressure (DBP), body mass index (BMI) and glycosylated haemoglobin (HbA1c) are commonly used indicators for patients with hypertension or diabetes. Individual models and group- and disease-specific models are the most commonly used models of integrated health care. All the studies were from high-income and middle-income countries. Meta-analysis showed that integrated health care significantly improved SBP, DBP and HbA1c but not BMI. A comparison of interventions lasting 6 and 12 months for diabetes was conducted, and HbA1c was decreased after 12 months. The changes in SBP and DBP were statistically significant after using group- and disease-specific model but not individual models. HbA1c was significantly improved after using group- and disease-specific models and individual models. Conclusion Integrated health care is a useful tool for disease management, and individual models and group- and disease-specific models are the most commonly used models in integrated health care. Group- and disease-specific models are more effective than individual models in the disease management of hypertension patients. The duration of intervention should be considered in the disease management of patients with diabetes, and interventions longer than 12 months are recommended. The income level may affect the model of integrated health care in selecting which disease to intervene, but this point still needs support from more studies.

人工气道指将气管导管直接插入气管或经上呼吸道插入气管而所建立的气体通道[1].人工气道的建立因改变和破坏了呼吸道屏障功能,会使呼吸道感染明显增加.

Abstract Background Telemedicine has been widely used for long-term care and self-management in patients with chronic disease, but there is no consensus regarding the effect of telemedicine on chronic disease management. The aim of this study is to review and analyse the effect of telemedicine on the management of chronic diseases such as hypertension, diabetes, and rheumatoid arthritis using a systematic review and meta-analysis. Methods We performed a comprehensive literature search of the Web of Science, PubMed, MEDLINE, EMBASE, CNKI (Chinese database), VIP (Chinese database), WanFang (Chinese database), and SinoMed (Chinese database) databases from their inception until December 31, 2021. The retrieved literature was screened and assessed independently by two authors. We used the risk-of-bias assessment tool recommended by the Cochrane Handbook for Systematic Reviews of Interventions 5.0.2 for assessing literature quality and Revman 5.3 software to conduct the meta-analysis. Results Fifteen articles were included in this study. The results of the systematic review indicated that telemedicine consultation and telemonitoring are the most commonly used intervention methods. Telemedicine is helpful for improving self-management in patients with rheumatoid arthritis. The results of the meta-analysis showed patients’ index of glycosylated hemoglobin (HbA1c) improved after 12 months of intervention (MD = − 0.84; 95% CI = − 1.53, − 0.16; Z = 2.42; P = 0.02), and no significant differences in fasting blood glucose (FBG) levels were observed after 6 months of intervention (MD = − 0.35; 95% CI = − 0.75,0.06; Z = 1.69; P = 0.09). The results also showed that systolic blood pressure (MD = − 6.71; 95% CI = − 11.40, − 2.02; Z = 2.81; P = 0.005) was reduced after 6 months of intervention. Conclusion Telemedicine had a positive effect on the management of diabetes, hypertension, and rheumatoid arthritis, especially when telemedicine consultation and telemonitoring method were used. When telemedicine was used as a disease management tool for patients with diabetes, the optimal intervention time is 12 months. Telemedicine improved the systolic blood pressure in hypertensive patients while also reducing negative emotions and enhancing medication adherence in rheumatoid arthritis patients.