Objective:To analyze the clinical characteristics and treatment outcomes of children with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibody-mediated necrotizing myopathy, and to explore early identification and management strategies to provide reference for clinical diagnosis and treatment.Methods:A retrospective analysis was conducted on the clinical data and treatment outcomes of 10 pediatric patients with anti-HMGCR antibody-mediated necrotizing myopathy admitted to the Department of Rheumatology, Children′s Hospital of Fudan University from December 2020 to December 2024. Statistical description was performed using SPSS 22.0.Results:Among the 10 patients, the male-to-female ratio was 1:4, the age of onset was (7.2±4.0) years, and the disease duration at diagnosis was (22.2±19.6) months. None had a history of statin exposure. Six patients presented with muscle weakness, and4 were diagnosed due to asymptomatic elevation of creatine kinase (CK); 4 had dermatomyositis-like rashes. All patients showed significantly elevated CK levels [median 3 291(1 969, 8 776)U/L] and underwent muscle biopsy. Histopathological findings revealed myofiber degeneration, necrosis, and regeneration in all cases, with inflammatory infiltration in 9 cases, MHC-Ⅰ positivity in all, and C5b-9 positivity in 9 cases. The median follow-up duration was (15.7±6.3) months. At the last follow-up, muscle strength was normal or nearly normal, and the CK median value had decreased to 977.5 (211.0, 3 536.0) U/L.Conclusion:For patients with suspected idiopathic inflammatory myopathy and significantly elevated CK, muscle-specific antibody testing-including anti-HMGCR-and muscle biopsy should be performed promptly regardless of the presence of skin rash, to ensure accurate diagnosis and guide treatment, thereby avoiding misdiagnosis or missed diagnosis.

Objective:To analyze the clinical characteristics and treatment outcomes of children with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibody-mediated necrotizing myopathy, and to explore early identification and management strategies to provide reference for clinical diagnosis and treatment.Methods:A retrospective analysis was conducted on the clinical data and treatment outcomes of 10 pediatric patients with anti-HMGCR antibody-mediated necrotizing myopathy admitted to the Department of Rheumatology, Children′s Hospital of Fudan University from December 2020 to December 2024. Statistical description was performed using SPSS 22.0.Results:Among the 10 patients, the male-to-female ratio was 1:4, the age of onset was (7.2±4.0) years, and the disease duration at diagnosis was (22.2±19.6) months. None had a history of statin exposure. Six patients presented with muscle weakness, and4 were diagnosed due to asymptomatic elevation of creatine kinase (CK); 4 had dermatomyositis-like rashes. All patients showed significantly elevated CK levels [median 3 291(1 969, 8 776)U/L] and underwent muscle biopsy. Histopathological findings revealed myofiber degeneration, necrosis, and regeneration in all cases, with inflammatory infiltration in 9 cases, MHC-Ⅰ positivity in all, and C5b-9 positivity in 9 cases. The median follow-up duration was (15.7±6.3) months. At the last follow-up, muscle strength was normal or nearly normal, and the CK median value had decreased to 977.5 (211.0, 3 536.0) U/L.Conclusion:For patients with suspected idiopathic inflammatory myopathy and significantly elevated CK, muscle-specific antibody testing-including anti-HMGCR-and muscle biopsy should be performed promptly regardless of the presence of skin rash, to ensure accurate diagnosis and guide treatment, thereby avoiding misdiagnosis or missed diagnosis.

Objective:To conduct reliability and validity test of Chinese versions of TeamSTEPPS medical teamwork perceptions questionnaire and TeamSTEPPS medical teamwork attitudes questionnaire so as to evaluate whether they are applicable to the current situation of medical teamwork in China.Methods:From January to April 2019, the Chinese versions of TeamSTEPPS teamwork perceptions questionnaire and TeamSTEPPS teamwork attitudes questionnaire were used to evaluate 900 emergency department workers in 9 ClassⅢGrade A hospitals in Shanghai by cluster sampling, and reliability and validity of the questionnaires were analyzed and evaluated. In this study, a total of 900 questionnaires were issued, 870 were recovered and 861 were valid, with an effective recovery rate of 96%.Results:The exploratory factor analysis of the Chinese versions of Chinese versions of TeamSTEPPS medical teamwork perceptions questionnaire and TeamSTEPPS medical teamwork attitudes questionnaire was carried out to extract the five dimensions of team structure, leadership, situation monitoring, mutual assistance and communication. The cumulative variance contribution rates were respectively 71.248% and 71.010%. In addition, a confirmatory factor analysis was performed on the questionnaires. The Chi-square degrees of freedom ratio (χ 2/ df) values were 2.870 and 2.214, normed fitting index ( NFI) values were 0.861 and 0.906, Tucker-Lewis index values were 0.896 and 0.940, incremental fit index ( IFI) values were 0.905 and 0.946, comparative fit index (CFI) values were 0.904 and 0.946, and root mean square error of approximation ( RMSEA) values were 0.066 and 0.053. The fitting values all reached the judgment standard, and the structural validity of the two questionnaires was good. The content validity indexes were good and they were respectively 0.94 and 0.95. The overall Cronbach's α coefficients of TeamSTEPPS medical teamwork perceptions questionnaire and TeamSTEPPS medical teamwork attitudes questionnaire were respectively 0.949 and 0.938, the split-half reliability was respectively 0.848 and 0.959, and the test retest reliability were respectively 0.959 and 0.964. Conclusions:The Chinese versions of TeamSTEPPS medical teamwork perceptions questionnaire and TeamSTEPPS medical teamwork attitudes questionnaire have good reliability and validity and high applicability, which can be used to measure the attitude and perceptions of medical teamwork in China.