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 compare the effect of two hemostatic drugs, tranexamic acid and hemocoagulase, on postoperative bleeding in adult patients with Kashin-Beck disease undergoing total knee arthroplasty.Methods:A retrospective analysis was conducted to select 80 adult patients with Kashin-Beck disease who underwent unilateral primary total knee arthroplasty at Linyou County Hospital from January 2021 to March 2023. According to the inclusion and exclusion criteria, 19 cases with chronic anemia, long-term oral anticoagulants implanted with cardiac stents, and hematological diseases were excluded. Among the remaining 61 adult patients, 24 cases with preoperative intravenous application combined with intraoperative local application of tranexamic acid in articular cavity were selected as the experimental group, and 37 cases with preoperative intravenous application combined with intraoperative local application of hemocoagulase in articular cavity were selected as the control group. The preoperative general condition, postoperative drainage volume, total perioperative blood loss, occult blood loss, and hemoglobin levels at different time points before and after surgery were compared between the two groups of patients.Results:There was no statistically significant difference in age, gender distribution, body mass index, preoperative hemoglobin level, preoperative prothrombin time, and preoperative activated partial thromboplastin time between the two groups of patients ( P > 0.05). The total perioperative blood loss [(1 027.78 ± 472.71) ml], drainage volume 48 h after surgery [(336.67 ± 112.74) ml] in the experimental group were all lower than those in the control group [(1 390.39 ± 454.01), (498.65 ± 187.57) ml], and the differences were statistically significant ( P < 0.05). The hemoglobin levels on the second and fifth day after surgery were significantly higher than those in the control group, and the differences were statistically significant ( P < 0.05). Conclusion:For total knee arthroplasty for adult patients with Kashin-Beck disease, preoperative intravenous application combined with intraoperative local application of tranexamic acid in articular cavity is superior to hemocoagulase, which can effectively reduce total perioperative blood loss and postoperative drainage volume, and is worthy of clinical promotion.

Objective:To compare the effect of two hemostatic drugs, tranexamic acid and hemocoagulase, on postoperative bleeding in adult patients with Kashin-Beck disease undergoing total knee arthroplasty.Methods:A retrospective analysis was conducted to select 80 adult patients with Kashin-Beck disease who underwent unilateral primary total knee arthroplasty at Linyou County Hospital from January 2021 to March 2023. According to the inclusion and exclusion criteria, 19 cases with chronic anemia, long-term oral anticoagulants implanted with cardiac stents, and hematological diseases were excluded. Among the remaining 61 adult patients, 24 cases with preoperative intravenous application combined with intraoperative local application of tranexamic acid in articular cavity were selected as the experimental group, and 37 cases with preoperative intravenous application combined with intraoperative local application of hemocoagulase in articular cavity were selected as the control group. The preoperative general condition, postoperative drainage volume, total perioperative blood loss, occult blood loss, and hemoglobin levels at different time points before and after surgery were compared between the two groups of patients.Results:There was no statistically significant difference in age, gender distribution, body mass index, preoperative hemoglobin level, preoperative prothrombin time, and preoperative activated partial thromboplastin time between the two groups of patients ( P > 0.05). The total perioperative blood loss [(1 027.78 ± 472.71) ml], drainage volume 48 h after surgery [(336.67 ± 112.74) ml] in the experimental group were all lower than those in the control group [(1 390.39 ± 454.01), (498.65 ± 187.57) ml], and the differences were statistically significant ( P < 0.05). The hemoglobin levels on the second and fifth day after surgery were significantly higher than those in the control group, and the differences were statistically significant ( P < 0.05). Conclusion:For total knee arthroplasty for adult patients with Kashin-Beck disease, preoperative intravenous application combined with intraoperative local application of tranexamic acid in articular cavity is superior to hemocoagulase, which can effectively reduce total perioperative blood loss and postoperative drainage volume, and is worthy of clinical promotion.

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.