1.Evaluation of Pulmonary Air-Blood Barrier Damage in Ulcerative Colitis Inflammatory Cancer Transformation Model Mice:Based on the "Lung-Intestine Correlation" Theory
Huiyan XU ; Haimei ZHANG ; Xinyu ZHAN ; Fanwu WU ; Yongsen JIA ; Chenxi WU ; Lingyu KONG ; Xin YAN
Journal of Traditional Chinese Medicine 2026;67(7):776-783
ObjectiveTo dynamically observe and evaluate the damage to the pulmonary air-blood barrier in mice during the inflammatory cancer transformation process of ulcerative colitis (UC) based on the "lung-intestine correlation" theory. MethodsSixty-five C57BL/6 mice were divided into a normal group (n=25) and a model group (n=40) using a random number table. Azoxymethane/dextran sodium sulfate (DSS) method was used to establish a mouse model of UC inflammation cancer transformation in the modeling group. According to the tissue collection time points at 5, 8, 11, 13, and 15 weeks, the normal group mice were randomly divided into the normal 5w, 8w, 11w, 13w, and 15w groups. The model group mice, 10 mice of which died after the first cycle of DSS administration, were randomly divided into model 5w, 8w, 11w, 13w, and 15w groups. During the experiment, the general condition of the mice was observed daily, and their body weight was measured weekly. At the corresponding tissue collection time points, the colon length of each group was measured. Histopathology of mouse lung and colon tissues was examined using HE staining. Immunofluorescence was used to detect changes in the positive expression of tight junction protein (ZO-1), vascular endothelial cadherin (VE-cadherin), and cytoskeletal protein (F-actin) in lung and colon tissues. RT-PCR was used to detect the mRNA expression of apoptosis regulatory proteins B-cell lymphoma-2 (Bcl-2), BCL2-associated X protein (Bax), and Cysteine aspartic acid protease-3 (Caspase-3) in lung tissues. Western Blot was employed to measure protein levels of ZO-1, VE-cadherin, and F-actin in lung tissues. ResultsCompared to the normal group at the same time point, the mice in the model group at each time point generally had poorer conditions, with weight loss and shortened colon length (P<0.05 or P<0.01). In the model 5w group, there was significant inflammatory cell infiltration in the colon tissue; in the model 8w group, there was mild atypical hyperplasia; in the model 11w group, the crypt structure was disordered, and moderate to severe atypical hyperplasia occurred; in the model 13w and 15w groups, tumors appeared. Pulmonary interstitial lesions, inflammation, vasculitis, and fibrosis were observed at all stages of UC inflammation cancer transformation. The protein levels of ZO-1, VE-cadherin, and F-actin, as well as Bcl-2 mRNA expression in lung tissue decreased during the acute inflammatory recovery period, atypical hyperplasia period, and canceration period, while the expressions of Bax and Caspase-3 mRNA increased; the expressions of ZO-1, VE-cadherin, and F-actin proteins in colon tissue decreased during the acute inflammatory recovery period, atypical hyperplasia period, and canceration period (P<0.01 or P<0.05). Compared to the model 5w group, the ZO-1 and F-actin protein levels and Bcl-2 mRNA expression in lung tissue in the other model groups increased in the atypical hyperplasia period and canceration period, while the expressions of Bax and Caspase-3 mRNA decreased; the expression of ZO-1 protein in colon tissue increased in the canceration period, and the expression of VE-cadherin protein decreased in the atypical hyperplasia period (P<0.01 or P<0.05). ConclusionIn the process of "inflammatory response-atypical hyperplasia-carcinogenesis" in UC inflammatory cancer transformation mice, there were damage to air-blood barrier.
2.Effects of Combined Tongue Three-Needle Acupuncture and Acupoint Application on Lianquan (CV 23) on Swallowing Function and Surface Electromyography Signals in Patients with Dysphagia after Ischemic Stroke
Xiaoyu DENG ; Dongmei XU ; Qiong FAN ; Lei YUAN ; Wei WU ; Haimei LIU
Journal of Traditional Chinese Medicine 2025;66(15):1559-1565
ObjectiveTo observe the clinical effectiveness and potential mechanism of combined tongue three-needle acupuncture and acupoint application on Lianquan (CV 23) for patients with dysphagia after ischemic stroke. MethodsA prospective study was conducted on 160 patients with post-stroke dysphagia, who were randomly divided into a treatment group and a control group, with 80 cases in each group. The control group received conventional rehabilitation training, while the treatment group received tongue three-needle acupuncture combined with acupoint application on Lianquan (CV 23) on the basis of conventional rehabilitation training, for 4 weeks in both groups. We compared the clinical effectivenss of both groups after treatment, and assessed the swallowing function including videofluoroscopic swallowing study (VFSS), standardized swallowing assessment (SSA) and functional oral intake scale (FIOS), swallowing contrast test including hyoid maximum displacement (HmaxD), pharyngeal transit time (PTT), and upper esophageal sphincter (UES) opening, surface electromyography (sEMG) test including maximum amplitude and swallowing duration as well as swallowing quality of life questionnaire (SWAL-QOL) score of the patients in both groups before treatment, after 2 weeks and 4 weeks of treatment, respectively. ResultsThe total effective rate in treatment group was 82.50% (66/80), significantly higher than 66.25% (53/80) in control group (P<0.05). The VFSS, and FOIS scores, UES opening rate and HmaxD, sEMG maximal amplitude values, and SWAL-QOL scores were increased in both groups after 2 weeks and 4 weeks of treatment compared with the values before treatment (P<0.05), while SSA scores, PTT, and swallowing duration were decreased compared within group before treatment (P<0.05). VFSS and FOIS scores, UES opening rate and HmaxD, sEMG maximal amplitude values, and SWAL-QOL scores after 2 and 4 weeks of treatment in the treatment group were higher (P<0.05), while SSA scores, PTT, and swallowing duration were lower (P<0.05) than those in the control group at the same time. ConclusionCombined tongue three-needle acupuncture and acupoint application on Lianquan (CV 23) for patients with dysphagia after ischemic stroke can significantly improve swallowing activities, and its mechanism of action may be related to the improvement of the contraction ability and coordination of swallowing-related muscle groups.
3.Study on the Improvement of Glucose-Lipid Metabolism in Type 2 Diabetes Mellitus by Compounding Seven-Flower Granules
Yan WAN ; Haimei WU ; Feifan LIU ; Yuanmei BAI ; Lin'ao ZHANG ; Shifang LUO ; Xue WU ; Yuhuan XIE ; Chunqin KONG ; Peixin GUO
World Science and Technology-Modernization of Traditional Chinese Medicine 2025;27(5):1386-1398
Objective To investigate the ameliorative effect of Qihuakeli,a Hani formula,on glycolipid metabolism in type 2 diabetes mellitus by in vivo and in vitro experiments.Methods Rat liver mesenchymal stromal cells(BRL-3A)were inoculated in six-well plates and divided into blank,palmitic acid,fenofibrate,and Qihuakeli serum-containing 5.4,10.8,and 21.6 g/kg groups.Except for the blank group,the remaining groups were intervened with 0.2 mmol/L palmitic acid(PA)for 24 hour,and then added with drug-containing serum,and then continued to incubate for 24 hour.The proliferation rate of BRL-3A cells in each group was determined.Total cholesterol(T-CHO),low-density lipoprotein cholesterol(LDL-C),and high-density lipoprotein cholesterol(HDL-C)concentrations in the supernatant of each cell group were measured,cell culture medium was aspirated and discarded,triglyceride(TG)concentration in the cell lysate.The lipid content of the cells was determined by measuring and staining with red oil.Meanwhile,45 rats were taken and divided into blank group,model group,fenofibrate group(0.225 g/kg),Qihuakeli compound 5.4 g/kg group,and Qihuakeli compound 10.8 g/kg group,the blank group was given normal feed and the rest of the groups were given high-fat feed for 42 day.Beginning on the 43rd day,each group,except the blank group,was injected with a single intraperitoneal injection of Starting from the 43rd day,except the blank group,each group was given a one-time intraperitoneal injection of 0.25%streptozotocin(STZ)solution,and at the same time,the corresponding drugs were given by gavage for 14 day.The rats'weight gain and liver index were measured.Serum fasting blood glucose(FBG)and fasting insulin(FINS)were detected,and the insulin resistance index(ISI)was calculated.Serum free fatty acid(FFA)levels and tumor necrosis factor-α(TNF-α)in liver tissue were also detected.HE staining was used to detect pathological changes in the pancreas.Pathological changes were observed in the tissues,and islet α and β cell expression was detected by immunohistochemistry.Results Compared to the PA group,the accumulation rate of BRL-3A cells was significantly higher(P<0.01)in the 10.8 and 21.6 g/kg Qihuakeli-containing serum groups.The levels of T-CHO,LDL-C and TG in the 5.4 and 21.6 g/kg serum groups were significantly lower(P<0.05),and HDL-C levels significantly increased(P<0.05).Oil red staining results showed that lipids in the cytoplasm of the 5.4,10.8 and 21.6 g/kg.Qihuacel-containing groups significantly reduced.Compared to the model group,the body weight of the 10.8 g/kg group containing Qihuakeli granules increased significantly(P<0.05).The liver index of the 5.4 g/kg group containing Qihuakeli decreased significantly(P<0.05).The serum indices of FBG,FINS,FFA and insulin resistance of the 5 g/kg group containing Qihuakeli decreased significantly(P<0.05).In the 5.4,10.8 g/kg groups,all serum FBG,FINS,FFA and insulin resistance indices significantly reduced in the 5.4 and 10.8 g/kg Qihuakeli groups(P<0.05 or P<0.01).TNF-α levels were significantly reduced(P<0.01).HE staining showed that a small number of lymphocytes were scattered in the pancreatic ducts and perivascular area of the rats in the Qihuakeli 5.4 and 10.8 g/kg groups,the local vasodilatation was observed,the number of pancreatic islet cells and the area of islet cells significantly increased.Immunohistochemical study was further used.The results of immunohistochemistry showed that the area of pancreatic islet α-cells significantly reduced and the area of pancreatic islet β-cells significantly increased in Qihuakeli 5.4 and 10.8 g/kg groups.Conclusion Qihuakeli compound improved glucose-lipid metabolism in T2DM,probably by improving the function of pancreatic islet cells,increasing the sensitivity of insulin to blood glucose,improving insulin resistance,decreasing the secretion of insulin and glucagon,and thus lowering the level of fasting blood glucose.Meanwhile,by decreasing the content of TNF-α,inhibiting lipolysis in the body,and promoting the uptake of FFA by adipocytes,and further lowering the FFA.Thus,it regulates the levels of TG,T-CHO,HDL-C and LDL-C,improves the abnormalities of glucose and lipid metabolism,and alleviates T2DM.
4.Risk factors of hyperhomocysteinemia in military pilots
Junwei YANG ; Xiufang WU ; Chunhong PAN ; Haimei TANG ; Tao ZHAO
Journal of Navy Medicine 2025;46(6):547-551
Objective To investigate the epidemiological characteristics and risk factors of hyperhomocysteinemia(HHcy)in military aircrew.Methods A retrospective study was conducted on 506 aircrew convalescents undergoing health examination from September 2023 to April 2024.Demographic,lifestyle,and biochemical data were analyzed.The risk factors of HHcy were identified by x2 test and multivariate Logistic regression.Results The HHcy detection rate was 21.94%(111/506),with a median homocysteine(Hcy)level of 11.75(9.70-14.30)μmol/L.Multivariate analysis revealed that smoking(OR=2.093,95%CI:1.313-3.336),service in conventional fighter aircraft(OR=1.716,95%CI:1.063-2.770),elevated low-density lipoprotein cholesterol(LDL-C)(OR=2.510,95%CI:1.413-4.458),and elevated bilirubin(OR=2.360,95%CI:1.509-3.691)were independent risk factors for HHcy(all P<0.05).Conclusion There is a high prevalence of HHcy in military pilots.It is strongly associated with smoking,aircraft type,and metabolic abnormalities.It is recommended to incorporate Hcy testing into routine physical examination,implement risk factor-based stratified interventions,and establish an atherosclerotic cardiovascular diseases early warning system integrating vascular imaging and risk assessment,aiming to reduce cardiovascular risks and safeguard combat effectiveness.
5.Visual analysis of cardiovascular disease research in pilots based on CiteSpace
Junwei YANG ; Xiufang WU ; Yujie CHEN ; Yun ZHANG ; Haimei TANG
Journal of Navy Medicine 2025;46(9):867-873
Objective To analyze the current research on cardiovascular diseases in pilots using bibliometric methods,so as to provide reference for future researches.Methods Articles indexed by China National Knowledge Infrastructure(CNKI)were selected as the research objects.The time window ranged from January 1990 to February 2024,and the keywords"pilot or flight personnel"and"cardiovascular"were selected to search for all published literatures on cardiovascular diseases in pilots.CiteSpace 6.1.R6 software was used to analyze the authors,institutions,and keywords of the included literatures,and a visual analysis graph was drawn.Results A total of 198 articles were included in this study.The journal with the most published articles was the Chinese Journal of Aerospace Medicine and Aviation Military Medicine.The author with the most published articles was Wang Lujin,followed by Zheng Jun.The institution with the most published articles was Air Force General Hospital(Air Force Medical Center).Risk factors,hypertension,and health assessment were hot keywords.The emerging word was medical appraisal.Conclusion The literatures of cardiovascular diseases among pilots are mainly published in aviation medical journals,forming a research team led by Wang Lujin and Zheng Jun.Research cooperation is mainly within the research team members,with less cooperation between research institutions.The research hotspots are risk factors of cardiovascular disease,hypertension,and health identification,and the forefront of research is the medical identification of cardiovascular diseases among pilots.With the increasing importance of cardiovascular diseases among pilots,it is necessary to strengthen cooperation among research institutions in the future,promote high-quality development in this field,and focus on strengthening scientific research in the prevention of cardiovascular diseases to maintain the physical and mental health of pilots.
6.Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody-mediated necrotizing myopathy in 10 children: clinical features and treatment outcomes
Qing WU ; Qianying LYU ; Haimei LIU ; Wanzhen GUAN ; Yinyu GONG ; Yifan LI ; Qiaoqian ZENG ; Xiaomei ZHANG ; Qijiao WEI ; Ling YANG ; Guomin LI ; Xihua LI ; Lei ZHAO ; Li SUN
Chinese Journal of Rheumatology 2025;29(7):575-582
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.
7.Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study
Huiyuan YANG ; Wanzhen GUAN ; Ling2 YANG ; Haimei LIU ; Xiaoqing3 LI ; Haiguo YU ; Meiping LU ; Jun YANG ; Xiaohui LIU ; Hongxia ZHANG ; Wei ZHANG ; Jihong XIAO ; Xiaozhong LI ; Guomin LI ; Hong CHANG ; Sheng HAO ; Yue DU ; Daliang XU ; Ling WU ; Wenjie ZHENG ; Li LIU ; Xinhui JIANG ; Shaohui ZHU ; Dongmei ZHAO ; Xuemei TANG ; Li SUN
Chinese Journal of Pediatrics 2025;63(12):1299-1305
Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.
8.Study on the Improvement of Glucose-Lipid Metabolism in Type 2 Diabetes Mellitus by Compounding Seven-Flower Granules
Yan WAN ; Haimei WU ; Feifan LIU ; Yuanmei BAI ; Lin'ao ZHANG ; Shifang LUO ; Xue WU ; Yuhuan XIE ; Chunqin KONG ; Peixin GUO
World Science and Technology-Modernization of Traditional Chinese Medicine 2025;27(5):1386-1398
Objective To investigate the ameliorative effect of Qihuakeli,a Hani formula,on glycolipid metabolism in type 2 diabetes mellitus by in vivo and in vitro experiments.Methods Rat liver mesenchymal stromal cells(BRL-3A)were inoculated in six-well plates and divided into blank,palmitic acid,fenofibrate,and Qihuakeli serum-containing 5.4,10.8,and 21.6 g/kg groups.Except for the blank group,the remaining groups were intervened with 0.2 mmol/L palmitic acid(PA)for 24 hour,and then added with drug-containing serum,and then continued to incubate for 24 hour.The proliferation rate of BRL-3A cells in each group was determined.Total cholesterol(T-CHO),low-density lipoprotein cholesterol(LDL-C),and high-density lipoprotein cholesterol(HDL-C)concentrations in the supernatant of each cell group were measured,cell culture medium was aspirated and discarded,triglyceride(TG)concentration in the cell lysate.The lipid content of the cells was determined by measuring and staining with red oil.Meanwhile,45 rats were taken and divided into blank group,model group,fenofibrate group(0.225 g/kg),Qihuakeli compound 5.4 g/kg group,and Qihuakeli compound 10.8 g/kg group,the blank group was given normal feed and the rest of the groups were given high-fat feed for 42 day.Beginning on the 43rd day,each group,except the blank group,was injected with a single intraperitoneal injection of Starting from the 43rd day,except the blank group,each group was given a one-time intraperitoneal injection of 0.25%streptozotocin(STZ)solution,and at the same time,the corresponding drugs were given by gavage for 14 day.The rats'weight gain and liver index were measured.Serum fasting blood glucose(FBG)and fasting insulin(FINS)were detected,and the insulin resistance index(ISI)was calculated.Serum free fatty acid(FFA)levels and tumor necrosis factor-α(TNF-α)in liver tissue were also detected.HE staining was used to detect pathological changes in the pancreas.Pathological changes were observed in the tissues,and islet α and β cell expression was detected by immunohistochemistry.Results Compared to the PA group,the accumulation rate of BRL-3A cells was significantly higher(P<0.01)in the 10.8 and 21.6 g/kg Qihuakeli-containing serum groups.The levels of T-CHO,LDL-C and TG in the 5.4 and 21.6 g/kg serum groups were significantly lower(P<0.05),and HDL-C levels significantly increased(P<0.05).Oil red staining results showed that lipids in the cytoplasm of the 5.4,10.8 and 21.6 g/kg.Qihuacel-containing groups significantly reduced.Compared to the model group,the body weight of the 10.8 g/kg group containing Qihuakeli granules increased significantly(P<0.05).The liver index of the 5.4 g/kg group containing Qihuakeli decreased significantly(P<0.05).The serum indices of FBG,FINS,FFA and insulin resistance of the 5 g/kg group containing Qihuakeli decreased significantly(P<0.05).In the 5.4,10.8 g/kg groups,all serum FBG,FINS,FFA and insulin resistance indices significantly reduced in the 5.4 and 10.8 g/kg Qihuakeli groups(P<0.05 or P<0.01).TNF-α levels were significantly reduced(P<0.01).HE staining showed that a small number of lymphocytes were scattered in the pancreatic ducts and perivascular area of the rats in the Qihuakeli 5.4 and 10.8 g/kg groups,the local vasodilatation was observed,the number of pancreatic islet cells and the area of islet cells significantly increased.Immunohistochemical study was further used.The results of immunohistochemistry showed that the area of pancreatic islet α-cells significantly reduced and the area of pancreatic islet β-cells significantly increased in Qihuakeli 5.4 and 10.8 g/kg groups.Conclusion Qihuakeli compound improved glucose-lipid metabolism in T2DM,probably by improving the function of pancreatic islet cells,increasing the sensitivity of insulin to blood glucose,improving insulin resistance,decreasing the secretion of insulin and glucagon,and thus lowering the level of fasting blood glucose.Meanwhile,by decreasing the content of TNF-α,inhibiting lipolysis in the body,and promoting the uptake of FFA by adipocytes,and further lowering the FFA.Thus,it regulates the levels of TG,T-CHO,HDL-C and LDL-C,improves the abnormalities of glucose and lipid metabolism,and alleviates T2DM.
9.Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody-mediated necrotizing myopathy in 10 children: clinical features and treatment outcomes
Qing WU ; Qianying LYU ; Haimei LIU ; Wanzhen GUAN ; Yinyu GONG ; Yifan LI ; Qiaoqian ZENG ; Xiaomei ZHANG ; Qijiao WEI ; Ling YANG ; Guomin LI ; Xihua LI ; Lei ZHAO ; Li SUN
Chinese Journal of Rheumatology 2025;29(7):575-582
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.
10.Clinical characteristics of juvenile dermatomyositis in anti-nuclear matrix protein 2 antibody-positive patients and risk factors for severity: a national multicenter retrospective study
Huiyuan YANG ; Wanzhen GUAN ; Ling2 YANG ; Haimei LIU ; Xiaoqing3 LI ; Haiguo YU ; Meiping LU ; Jun YANG ; Xiaohui LIU ; Hongxia ZHANG ; Wei ZHANG ; Jihong XIAO ; Xiaozhong LI ; Guomin LI ; Hong CHANG ; Sheng HAO ; Yue DU ; Daliang XU ; Ling WU ; Wenjie ZHENG ; Li LIU ; Xinhui JIANG ; Shaohui ZHU ; Dongmei ZHAO ; Xuemei TANG ; Li SUN
Chinese Journal of Pediatrics 2025;63(12):1299-1305
Objective:To investigate the clinical characteristics and independent risk factors of severe disease in patients with anti-nuclear matrix protein (NXP) 2 antibody-positive juvenile dermatomyositis (JDM).Methods:A retrospective cohort study was conducted, including 219 anti-NXP2 antibody-positive JDM patients admitted to 23 children′s hospitals across China from July 2011 to July 2023. Patients were classified into severe and non-severe groups based on classification criteria for severe dermatomyositis. Demographic characteristics, clinical manifestations, and laboratory parameters were compared between the 2 groups using independent sample t-test, Mann-Whitney U test, or χ2 test. Univariate and multivariate Logistic regression analyses were performed to identify risk factors for severe disease. The receiver operating characteristic curve was employed to calculate optimal cut-off values. Results:Among the 219 patients, 108 were male and 111 were female, with an age at onset of 6.3 (3.5, 9.4) years. The severe group comprised 69 patients, and the non-severe group 150 patients. The severe group had significantly higher rates of fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, as well as elevated levels of ferritin-to-albumin ratio (FAR), creatine kinase (CK), aspartate aminotransferase (AST), and lactate dehydrogenase (LDH) (all P<0.05). Multivariate analysis identified anti-Ro52 antibody positivity ( OR=13.26, 95% CI 1.37-128.29) and elevated FAR ( OR=1.90, 95% CI 1.09-2.31) as independent risk factors for severe anti-NXP2 antibody-positive JDM (both P<0.05). Receiver operating characteristic curve analysis revealed that a FAR cutoff value of 6.82 predicted severe disease with an area under the curve of 0.87 (95% CI 0.81-0.94, P<0.001), sensitivity of 0.85, and specificity of 0.70. All patients received glucocorticoid therapy, and the severe group received higher proportions of steroid pulse therapy, cyclophosphamide, mycophenolate mofetil, intravenous immunoglobulin, biologics, and adjuvant treatments compared to the non-severe group (all P<0.05). In terms of outcomes, 2 patients (2.9%) in the severe group died (due to neurological involvement and intestinal perforation, respectively), while the remaining patients achieved complete clinical response or remission. All patients in the non-severe group achieved remission. Conclusions:The primary clinical features of anti-NXP2 antibody-positive JDM included fever, heliotrope rash, subcutaneous edema, periorbital edema, anti-Ro52 antibody positivity, and elevated levels of CK, AST, LDH, and FAR. Furthermore, anti-Ro52 antibody positivity and a FAR>6.82 were identified as independent risk factors.

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