1.Influencing factors and clinical treatment of severe complications after unilateral pneumonectomy in treating tuberculous destroyed lung
Xiao LI ; Ning WANG ; Lei BAO ; Zhiqiang WU ; Gang LI ; Cong CAI ; Yijie SONG ; Dan LI ; Banggui WU ; Liangshuang JIANG ; Xiaojun YAO
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery 2025;32(05):626-633
Objective To evaluate the surgical efficacy of unilateral pneumonectomy for the treatment of tuberculous destroyed lung, analyze the causes of severe postoperative complications, and explore clinical management strategies. Methods A retrospective analysis was conducted on the clinical data of patients with tuberculous destroyed lung who underwent unilateral pneumonectomy at the Public Health Clinical Center of Chengdu from 2017 to 2023. Postoperative severe complications were statistically analyzed. Patients were divided into a non-severe complication group and a severe-complication group, and the causes, management, and outcomes of complications were analyzed. Results A total of 134 patients were included, comprising 69 males and 65 females, with a mean age of 17-73 (40.43±12.69) years. There were 93 patients undergoing left pneumonectomy and 41 patients undergoing right pneumonectomy. Preoperative sputum smear was positive in 35 patients, all of which converted to negative postoperatively. There were 58 patients with hemoptysis preoperatively, and none experienced hemoptysis postoperatively. Postoperative incisional infection occurred in 8 (5.97%) patients, and postoperative pulmonary infection in 26 (19.40%) patients. Severe postoperative complications occurred in 17 (12.69%) patients, including empyema in 9 (6.72%) patients, bronchopleural fistula with empyema in 1 (0.75%) patient, severe pneumonia in 3 (2.24%) patients, postpneumonectomy syndrome in 1 (0.75%) patient, chylothorax in 1 (0.75%) patient, ketoacidosis in 1 (0.75%) patient, and heart failure with severe pneumonia in 1 (0.75%) patient. Perioperative mortality occurred in 2 (1.49%) patients, both of whom underwent right pneumonectomy. Multivariate logistic regression analysis revealed that a history of ipsilateral thoracic surgery, concomitant Aspergillus infection, and greater blood loss were independent risk factors for severe complications following unilateral pneumonectomy for tuberculous destroyed lung (P<0.05). Conclusion Unilateral pneumonectomy for patients with tuberculous destroyed lung can significantly improve the clinical cure rate, sputum conversion rate, and hemoptysis cessation rate. However, there is a certain risk of severe perioperative complications and mortality, requiring thorough perioperative management and appropriate management of postoperative complications.
2.Wendantang Regulates Energy Metabolism in Treatment of Myocardial Ischemia via SIRT3/PGC-1α Pathway
Xinjun ZHANG ; Zhiqiang XIAO ; Jia LU ; Wenliang DUN ; Ning GU
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(2):1-8
ObjectiveTo investigate the mechanism by which Wendantang regulates the silent information regulator 3 (SIRT3)/peroxisome proliferator-activated receptor-gamma coactivator-1α (PGC-1α) pathway to influence energy metabolism and thereby prevent and treat myocardial ischemia (MI) in a rat model of hyperlipidemia (HL). MethodsThirty SD rats were randomly assigned into five groups: control, model, low-dose (3.702 g·kg-1·d-1) Wendantang, high-dose (7.404 g·kg-1·d-1) Wendantang, and positive control (trimetazidine, 0.006 g·kg-1·d-1), with six rats in each group. The control group was fed normally, while the other groups were fed with a high-fat diet for six weeks for the modeling of HL. Subsequently, the drug intervention groups were administrated with corresponding drugs by gavage, and the control and model groups received an equivalent volume of normal saline for 14 days. One hour after the last gavage, the other groups except the control group were injected intraperitoneally with posterior pituitary hormone (30 U·kg-1) to induce MI. Electrocardiography (ECG) was employed to detect changes in the electrocardiogram. Hematoxylin-eosin staining was performed to observe cardiac pathological changes. Enzyme-linked immunosorbent assay was employed to measure the serum levels of cardiac troponin I(cTnI), myoglobin (MYO), and creatine kinase-MB (CK-MB). Colorimetry was used to determine the levels of total cholesterol (TC) and triglycerides (TG) in the serum and ATP, malondialdehyde (MDA), and superoxide dismutase (SOD) in the myocardial tissue. Western blot was employed to determine the protein levels of SIRT3, PGC-1α, adenosine monophosphate-activated protein kinase (AMPK), and phosphorylated AMPK (p-AMPK) in the myocardial tissue. Real-time PCR was employed to measure the mRNA levels of SIRT3, PGC-1α, and AMPKα in the myocardial tissue. ResultsCompared with the control group, the model group showed significant J-point deviation and elevation in the ECG image, increased heart rate, disarrangement of myocardial fibers with unclear boundaries, elevated levels of CK-MB, cTnI, MYO, TC, and TG (P<0.05, P<0.01), declined levels of SOD and ATP (P<0.01), down-regulated mRNA levels of SIRT3, PGC-1α, and AMPK (P<0.05), and down-regulated protein levels of SIRT3, PGC-1α, and p-AMPK (P<0.05). Compared with the model group, the low-dose and high-dose Wendantang groups and the trimetazidine group showed inhibited J-point deviation and elevation in the ECG image, slowed heart rate, reduced inflammatory cell infiltration, alleviated disarrangement of myocardial fibers, declined levels of CK-MB, cTnI, MYO, TC, and TG (P<0.05, P<0.01), elevated level of SOD (P<0.01), up-regulated mRNA levels of SIRT3, PGC-1α, and AMPK (P<0.05, P<0.01) and up-regulated protein levels of SIRT3, PGC-1α, and p-AMPK (P<0.05, P<0.01). ConclusionWendantang can effectively intervene in HL-associated MI in rats by reducing oxidative stress in myocardial cells, alleviating lipid metabolism disorders, and improving myocardial energy metabolism via the SIRT3/PGC-1α signaling pathway.
3.Meta-Analysis and Trial Sequential Analysis of the Efficacy of TCM in the Treatment of Coronary Heart Disease and TG-Lowering Level Based on the Theory of"Spleen Failing to Disperse Essence and Combined Phlegm and Stasis"Combined with Western Medicine
Zhiqiang XIAO ; Xinjun ZHANG ; Ning GU
World Science and Technology-Modernization of Traditional Chinese Medicine 2025;27(5):1312-1325
Objective To analyze the efficacy and safety of the traditional Chinese medicine(TCM)in the treatment of treating coronary heart disease and their effects on TG levels of patients with dyslipidemia based on the theory of"Spleen failing to disperse essence and combined phlegm and stasis"combined with Western medicine.Methods Eight different electronic databases were searched to include randomized controlled trials(RCTs)of the TCM based on the theory of"Spleen failing to disperse essence and combined phlegm and stasis"for CHD complicated with dyslipidemias published from inception to November 2024.Literature screening and data extraction were performed based on the inclusion and exclusion criteria.Meta-analysis was performed by RevMan 5.4.The GRADE system and TSA 0.9.5.10 Beta were adopted to evaluate the certainty and conclusiveness of the evidence.Results A total of 30 RCTs involving 2798 subjects were included.There were 1402 subjects in the experimental group and 1396 subjects in the control group.Meta-analysis showed that the group of Huatan Quyu therapy was effective in improving the clinical efficacy(RR=1.26,95%CI[1.19,1.33],P<0.00001),angina symptoms(RR=1.25,95%CI[1.14,1.37],P<0.00001),electrocardiogram efficacy(RR=1.27,95%CI[1.17,1.39],P<0.00001),and TCM symptom efficacy(RR=1.32,95%CI[1.23,1.42],P<0.00001).Additionally,TG levels(WMD=-0.44,95%CI[-0.52,-0.36],P<0.00001)and hs-CRP levels(WMD=-2.80,95%CI[-3.15,-2.45],P<0.00001)decreased.Concerning hemorheology,Huatan Quyu therapy effectively reduced WBV(WMD=-1.34,95%CI[-1.65,-1.02],P<0.00001)、PV(WMD=-0.44,95%CI[-0.45,-0.43],P<0.00001)and FIB(WMD=-0.46,95%CI[-0.75,-0.18],P=0.001).In terms of safety,19 studies reported the adverse reactions in detail,but the severity was mild and symptoms disappeared after treatment.TSA showed that the cumulative Z-curve of the clinical efficacy crossed the traditional threshold and TSA threshold,which further affirmed the clinical efficacy.The GRADE graded the evidence of the above outcome indicators as moderate or low.Conclusion TCM based on the theory of"Spleen failing to disperse essence and combined phlegm and stasis"may be effective and safe in the treatment of patients with CHD and lowering TG levels in dyslipidemic patients.Since the quality of the literature included in this study was generally low,there is an urgent need for more RCTs with high quality,low bias risk and sufficient sample size to validate the above conclusions.
4.Clinical study of intracranial hypotension targeted body posture combined with pharmacotherapy in the treatment of chronic subdural hematoma
Jiayu CHEN ; Zhe WANG ; Di ZANG ; Ruizhe ZHENG ; Xiangru YE ; Zengxin QI ; Zeyu XU ; Zhiqiang LI ; Chengfeng SUN ; Liangjun SHEN ; Luoping SHENG ; Fulin XU ; Ruyong YE ; Kaiyu ZHOU ; Weijun TANG ; Yueqing HU ; Dapeng SHI ; Yuquan WANG ; Xizhen WU ; Ying WANG ; Qilin ZHANG ; Feili LIU ; Guo YU ; Yiping LU ; Yirui SUN ; Ning ZHANG ; Feng HUANG ; Xialong GU ; Han ZHANG ; Jian DING ; Yongyan BI ; Haolan DU ; Jing ZHANG ; Hailong JI ; Ding DING ; Wei ZHANG ; Xuehai WU
Chinese Journal of Surgery 2025;63(3):212-218
Objective:To compare the efficacy of body posture combined with pharmacotherapy and pharmacotherapy alone in the treatment of chronic subdural hematoma(CSDH).Methods:Firstly, retrospective case series study was conducted. Thirty cases of CSDH that had received body posture combined with pharmacotherapy at Department of Neurosurgery, Huashan Hospital Affiliated to Fudan University from December 2016 to October 2020 were studied retrospectively. Twenty-seven patients were male, and 3 patients were female. The age of patients ( M(IQR)) was 66(16) years (range:28 to 84). Nineteen patients had unilateral hematoma, and 11 patients had bilateral hematoma. All patients received pharmacotherapy and body posture therapy that was to raise their lower limbs 20 to 30 cm with leg lift pad and get abdominal compressed with customized abdominal belt in supine position. Patients were required to maintain the body posture as much as possible, with the maximum to 16 to 18 hours per day. Patients with unilateral hematoma should tilt the head to the affected side and avoid tilting it to the opposite side. For patients with bilateral hematoma, there was no need for head lateralization. Patient were treated with oral dexamethasone and atorvastatin simultaneously. The preliminary efficacy of body posture combined with pharmacotherapy was determined by hematoma improvement rate which was analyzed by Clopper-Pearson method. Then, the multi-center, prospective, randomized controlled trial had carried out in 9 medical centers from August 2020 to November 2021. The stratified block randomization method was adopted. Patients were randomized in a ratio of 1∶1 to either receive pharmacotherapy alone(the control group) or body posture combined with pharmacotherapy(the experiment group) for 3 months and followed up for 6 months. Effective treatment was defined as complete absorption of hematoma, or the hematoma volume decreased by more than 10 ml and Markwalder grading scale score had improved by more than 1 point compared to the baseline. The efficacy rate and surgery conversion rate at 3 months and recurrence at 6 months were observed. Comparison between groups was performed with paired sample t test, Mann-Whitney U test, χ2 test, corrected χ2 test, or Fisher exact probability method. Logistic regression was used to compare the effective rate and operation rate between the two groups. Results:In the respective study, 30 patients completed follow-up 13 to 353 days after treatment. At the last follow-up, the incidence of almost complete absorption or significantly absorption of hematoma (hematoma volume was significantly reduced accompanied by symptom improvement) was 93.3%. The 95% CI for the incidence that analyzed by the Clopper-Pearson method was 77.9% to 99.2%. One hundred and six patients were enrolled in the multicenter study. Fifty-five patients underwent body posture combined with pharmacotherapy. The age was 74(17) years (range:26 to 92). Thirty-nine patients were males and 16 were females. Fifty-one patients underwent pharmacotherapy alone. The age was 69(12) years (range:48 to 84). Thirty-seven patients were males and 14 were females. The length of body posture recorded in diary card was (15.7±2.3) hours(range:7.6 to 19.3 hours). The efficacy rate in the body posture combined with pharmacotherapy group and pharmacotherapy alone group were 83.6% (46/55) and 56.9% (29/51), respectively at 3 months. The result of the logistic regression analysis showed that the efficacy of body posture combined with pharmacotherapy group was better than that of pharmacotherapy alone group ( OR=3.88,95% CI:1.57 to 9.58, P=0.003). Surgery rate in the body posture combined with pharmacotherapy group and pharmacotherapy alone group were 5.5% (3/55) and 21.6% (11/51) respectively. The result of Logistic regression showed that the pharmacotherapy alone group was more likely to be converted to surgery ( OR=0.21,95% CI:0.05 to 0.80, P=0.023). At the 6 months, no recurrence of cases was found in the body posture combined with pharmacotherapy group. However, the recurrence rate of pharmacotherapy alone group was 6.3% (3/48), there was no significant difference between the two groups ( P>0.05). Conclusion:The effect of body posture combined with pharmacotherapy for chronic subdural hematoma is better than that of pharmacotherapy alone.
5.The Influence of Boundary Condition Changes of Inner and Outer Walls of Osteon on Fluid Flow Characteristics:A Finite Element Study
Weilun YU ; Xiuying LIU ; Qiong WANG ; Yuan YAO ; Yubo GUO ; Ning QU ; Xiaogang WU ; Haoyu FENG ; Zhiqiang LI
Journal of Medical Biomechanics 2025;40(3):656-662
Objective To explore the characteristics of fluid flow within loaded osteons under different boundary conditions.Methods The COMSOL Multiphysics software was used to establish a three-dimensional(3D)finite element model of osteons with different boundary conditions,and the variation rules of pore pressure and flow velocity of osteons under different inner wall pulsating blood pressures and outer wall elastic constraint conditions were analyzed.Results As the pulsatile blood pressure inside the osteon increased from 0 mmHg(1 mmHg=0.133 kPa)to 300 mmHg,the peak pore pressure within the osteon correspondingly increased from 26 kPa to 68 kPa.As the elastic constraint on the outer wall of osteons changed from being completely elastic to completely constrained,the peak pore pressure within osteons increased from 15 kPa to 26 kPa,and the peak flow velocity increased from 0.04 um/s to 0.07 um/s.Conclusions This study reveals the influence laws of changes in boundary conditions such as the pulsatile blood pressure on the inner wall and the elastic constraint on the outer wall of osteons on fluid flow characteristics within loaded osteons.These findings are conducive to a deeper understanding of the mechanical response mechanisms of bone tissues in both physiological and pathological states,and provide an important theoretical basrs for further researches on bone mechanotransduction.
6.Clinical and genetic characteristics of late-onset facioscapulohumeral muscular dystrophy type 1
Fuze ZHENG ; Xinyue CHEN ; Meng YU ; Liangliang QIU ; Minghui ZENG ; Yuhua LIN ; Feng LIN ; Wei ZHANG ; Minting LIN ; Ning WANG ; Yun YUAN ; Zhiqiang WANG ; Zhaoxia WANG
Chinese Journal of Neurology 2025;58(10):1040-1047
Objective:To summarize the clinical and genetic characteristics of late-onset facioscapulohumeral muscular dystrophy type 1 (FSHD1) patients, and to compare the differences between late-onset and classic-onset FSHD1 patients.Methods:A retrospective analysis was conducted on the clinical and genetic data of genetically confirmed late-onset FSHD1 patients (age at onset30 years) between January 2007 and June 2024 from the Department of Neurology of Peking University First Hospital and the First Affiliated Hospital of Fujian Medical University. Classic-onset FSHD1 patients (10 yearsage at onset≤30 years) were matched 1∶1 according to sex and disease duration for comparison. The demographic information, the number of D4Z4 repeat units, the distal D4Z4 methylation levels, FSHD Clinical Score (CS), Clinical Severity Score (CSS), and Age-Corrected Clinical Severity Score (ACSS) of these patients were collected. Survival analysis was performed to compare the outcome of lower extremity involvement between late-onset and classic-onset FSHD1 patients. The correlation of the number of D4Z4 repeat units and D4Z4 methylation level with CS and ACSS was analyzed in late-onset FSHD1 patients.Results:A total of 61 patients with late-onset FSHD1 were enrolled, 33 (54.1%) of whom are female, with an age of 54.0 (46.0, 62.0) years and a disease duration of 14.0 (5.5, 22.5) years. Compared to classic-onset FSHD1 patients, late-onset patients exhibited significantly lower CS [7.0 (5.6, 8.4) vs 6.0 (4.4, 7.7), U=1 416.000, P=0.013], CSS [3.0 (2.8, 3.3) vs 3.0 (2.0, 4.0), U=2 352.000, P=0.010], and ACSS [189.2 (137.1, 241.3) vs 96.8 (61.3, 132.2), U=3 225.500, P0.001], and higher proportion of patients with limb girdle involvement but no facial muscle involvement [18.0% (11/61) vs 6.6% (4/61), χ2=3.725, P=0.054]. Kaplan-Meier survival analysis showed that the onset age of lower extremity involvement in late-onset patients (45 years, 95% CI 42-48 years) was significantly higher than that in classic-onset patients (24 years, 95% CI 21-27 years, χ2=61.012, P0.001). The duration from symptom onset to lower extremity involvement in late-onset patients (15 years, 95% CI 10-20 years) was significantly longer than that in classic-onset patients (8 years, 95% CI 3-13 years, χ2=9.105, P=0.003). Late-onset FSHD1 patients carried higher average distal D4Z4 methylation levels compared to those with classic-onset FSHD1 [46.68% (40.79%,52.57%) vs 41.02% (34.03%,48.00%), U=1 378.500, P=0.014]. Among late-onset FSHD1 patients, cytosine-phosphate-guanine 6 (CpG6) methylation levels were significantly negatively correlated with ACSS ( r=-0.278, P=0.025); the number of D4Z4 repeat units were significantly negatively correlated with ACSS ( r=-0.272, P=0.034);CpG6 methylation levels were significantly negatively correlated with CS ( r=-0.441, P=0.003), while no correlation was found between number of D4Z4 repeat units and CS ( r=-0.161, P=0.310). Conclusions:Compared with classic-onset FSHD1 patients, late-onset FSHD1 patients are associated with a higher degree of distal D4Z4 methylation, along with a milder muscle weakness phenotype, slower disease progression and a higher proportion of cases without facial muscle involvement. The age at onset can be used as a marker of the severity and prognosis in FSHD1.
7.Meta-Analysis and Trial Sequential Analysis of the Efficacy of TCM in the Treatment of Coronary Heart Disease and TG-Lowering Level Based on the Theory of"Spleen Failing to Disperse Essence and Combined Phlegm and Stasis"Combined with Western Medicine
Zhiqiang XIAO ; Xinjun ZHANG ; Ning GU
World Science and Technology-Modernization of Traditional Chinese Medicine 2025;27(5):1312-1325
Objective To analyze the efficacy and safety of the traditional Chinese medicine(TCM)in the treatment of treating coronary heart disease and their effects on TG levels of patients with dyslipidemia based on the theory of"Spleen failing to disperse essence and combined phlegm and stasis"combined with Western medicine.Methods Eight different electronic databases were searched to include randomized controlled trials(RCTs)of the TCM based on the theory of"Spleen failing to disperse essence and combined phlegm and stasis"for CHD complicated with dyslipidemias published from inception to November 2024.Literature screening and data extraction were performed based on the inclusion and exclusion criteria.Meta-analysis was performed by RevMan 5.4.The GRADE system and TSA 0.9.5.10 Beta were adopted to evaluate the certainty and conclusiveness of the evidence.Results A total of 30 RCTs involving 2798 subjects were included.There were 1402 subjects in the experimental group and 1396 subjects in the control group.Meta-analysis showed that the group of Huatan Quyu therapy was effective in improving the clinical efficacy(RR=1.26,95%CI[1.19,1.33],P<0.00001),angina symptoms(RR=1.25,95%CI[1.14,1.37],P<0.00001),electrocardiogram efficacy(RR=1.27,95%CI[1.17,1.39],P<0.00001),and TCM symptom efficacy(RR=1.32,95%CI[1.23,1.42],P<0.00001).Additionally,TG levels(WMD=-0.44,95%CI[-0.52,-0.36],P<0.00001)and hs-CRP levels(WMD=-2.80,95%CI[-3.15,-2.45],P<0.00001)decreased.Concerning hemorheology,Huatan Quyu therapy effectively reduced WBV(WMD=-1.34,95%CI[-1.65,-1.02],P<0.00001)、PV(WMD=-0.44,95%CI[-0.45,-0.43],P<0.00001)and FIB(WMD=-0.46,95%CI[-0.75,-0.18],P=0.001).In terms of safety,19 studies reported the adverse reactions in detail,but the severity was mild and symptoms disappeared after treatment.TSA showed that the cumulative Z-curve of the clinical efficacy crossed the traditional threshold and TSA threshold,which further affirmed the clinical efficacy.The GRADE graded the evidence of the above outcome indicators as moderate or low.Conclusion TCM based on the theory of"Spleen failing to disperse essence and combined phlegm and stasis"may be effective and safe in the treatment of patients with CHD and lowering TG levels in dyslipidemic patients.Since the quality of the literature included in this study was generally low,there is an urgent need for more RCTs with high quality,low bias risk and sufficient sample size to validate the above conclusions.
8.The Influence of Boundary Condition Changes of Inner and Outer Walls of Osteon on Fluid Flow Characteristics:A Finite Element Study
Weilun YU ; Xiuying LIU ; Qiong WANG ; Yuan YAO ; Yubo GUO ; Ning QU ; Xiaogang WU ; Haoyu FENG ; Zhiqiang LI
Journal of Medical Biomechanics 2025;40(3):656-662
Objective To explore the characteristics of fluid flow within loaded osteons under different boundary conditions.Methods The COMSOL Multiphysics software was used to establish a three-dimensional(3D)finite element model of osteons with different boundary conditions,and the variation rules of pore pressure and flow velocity of osteons under different inner wall pulsating blood pressures and outer wall elastic constraint conditions were analyzed.Results As the pulsatile blood pressure inside the osteon increased from 0 mmHg(1 mmHg=0.133 kPa)to 300 mmHg,the peak pore pressure within the osteon correspondingly increased from 26 kPa to 68 kPa.As the elastic constraint on the outer wall of osteons changed from being completely elastic to completely constrained,the peak pore pressure within osteons increased from 15 kPa to 26 kPa,and the peak flow velocity increased from 0.04 um/s to 0.07 um/s.Conclusions This study reveals the influence laws of changes in boundary conditions such as the pulsatile blood pressure on the inner wall and the elastic constraint on the outer wall of osteons on fluid flow characteristics within loaded osteons.These findings are conducive to a deeper understanding of the mechanical response mechanisms of bone tissues in both physiological and pathological states,and provide an important theoretical basrs for further researches on bone mechanotransduction.
9.Clinical and genetic characteristics of late-onset facioscapulohumeral muscular dystrophy type 1
Fuze ZHENG ; Xinyue CHEN ; Meng YU ; Liangliang QIU ; Minghui ZENG ; Yuhua LIN ; Feng LIN ; Wei ZHANG ; Minting LIN ; Ning WANG ; Yun YUAN ; Zhiqiang WANG ; Zhaoxia WANG
Chinese Journal of Neurology 2025;58(10):1040-1047
Objective:To summarize the clinical and genetic characteristics of late-onset facioscapulohumeral muscular dystrophy type 1 (FSHD1) patients, and to compare the differences between late-onset and classic-onset FSHD1 patients.Methods:A retrospective analysis was conducted on the clinical and genetic data of genetically confirmed late-onset FSHD1 patients (age at onset30 years) between January 2007 and June 2024 from the Department of Neurology of Peking University First Hospital and the First Affiliated Hospital of Fujian Medical University. Classic-onset FSHD1 patients (10 yearsage at onset≤30 years) were matched 1∶1 according to sex and disease duration for comparison. The demographic information, the number of D4Z4 repeat units, the distal D4Z4 methylation levels, FSHD Clinical Score (CS), Clinical Severity Score (CSS), and Age-Corrected Clinical Severity Score (ACSS) of these patients were collected. Survival analysis was performed to compare the outcome of lower extremity involvement between late-onset and classic-onset FSHD1 patients. The correlation of the number of D4Z4 repeat units and D4Z4 methylation level with CS and ACSS was analyzed in late-onset FSHD1 patients.Results:A total of 61 patients with late-onset FSHD1 were enrolled, 33 (54.1%) of whom are female, with an age of 54.0 (46.0, 62.0) years and a disease duration of 14.0 (5.5, 22.5) years. Compared to classic-onset FSHD1 patients, late-onset patients exhibited significantly lower CS [7.0 (5.6, 8.4) vs 6.0 (4.4, 7.7), U=1 416.000, P=0.013], CSS [3.0 (2.8, 3.3) vs 3.0 (2.0, 4.0), U=2 352.000, P=0.010], and ACSS [189.2 (137.1, 241.3) vs 96.8 (61.3, 132.2), U=3 225.500, P0.001], and higher proportion of patients with limb girdle involvement but no facial muscle involvement [18.0% (11/61) vs 6.6% (4/61), χ2=3.725, P=0.054]. Kaplan-Meier survival analysis showed that the onset age of lower extremity involvement in late-onset patients (45 years, 95% CI 42-48 years) was significantly higher than that in classic-onset patients (24 years, 95% CI 21-27 years, χ2=61.012, P0.001). The duration from symptom onset to lower extremity involvement in late-onset patients (15 years, 95% CI 10-20 years) was significantly longer than that in classic-onset patients (8 years, 95% CI 3-13 years, χ2=9.105, P=0.003). Late-onset FSHD1 patients carried higher average distal D4Z4 methylation levels compared to those with classic-onset FSHD1 [46.68% (40.79%,52.57%) vs 41.02% (34.03%,48.00%), U=1 378.500, P=0.014]. Among late-onset FSHD1 patients, cytosine-phosphate-guanine 6 (CpG6) methylation levels were significantly negatively correlated with ACSS ( r=-0.278, P=0.025); the number of D4Z4 repeat units were significantly negatively correlated with ACSS ( r=-0.272, P=0.034);CpG6 methylation levels were significantly negatively correlated with CS ( r=-0.441, P=0.003), while no correlation was found between number of D4Z4 repeat units and CS ( r=-0.161, P=0.310). Conclusions:Compared with classic-onset FSHD1 patients, late-onset FSHD1 patients are associated with a higher degree of distal D4Z4 methylation, along with a milder muscle weakness phenotype, slower disease progression and a higher proportion of cases without facial muscle involvement. The age at onset can be used as a marker of the severity and prognosis in FSHD1.
10.Clinical study of intracranial hypotension targeted body posture combined with pharmacotherapy in the treatment of chronic subdural hematoma
Jiayu CHEN ; Zhe WANG ; Di ZANG ; Ruizhe ZHENG ; Xiangru YE ; Zengxin QI ; Zeyu XU ; Zhiqiang LI ; Chengfeng SUN ; Liangjun SHEN ; Luoping SHENG ; Fulin XU ; Ruyong YE ; Kaiyu ZHOU ; Weijun TANG ; Yueqing HU ; Dapeng SHI ; Yuquan WANG ; Xizhen WU ; Ying WANG ; Qilin ZHANG ; Feili LIU ; Guo YU ; Yiping LU ; Yirui SUN ; Ning ZHANG ; Feng HUANG ; Xialong GU ; Han ZHANG ; Jian DING ; Yongyan BI ; Haolan DU ; Jing ZHANG ; Hailong JI ; Ding DING ; Wei ZHANG ; Xuehai WU
Chinese Journal of Surgery 2025;63(3):212-218
Objective:To compare the efficacy of body posture combined with pharmacotherapy and pharmacotherapy alone in the treatment of chronic subdural hematoma(CSDH).Methods:Firstly, retrospective case series study was conducted. Thirty cases of CSDH that had received body posture combined with pharmacotherapy at Department of Neurosurgery, Huashan Hospital Affiliated to Fudan University from December 2016 to October 2020 were studied retrospectively. Twenty-seven patients were male, and 3 patients were female. The age of patients ( M(IQR)) was 66(16) years (range:28 to 84). Nineteen patients had unilateral hematoma, and 11 patients had bilateral hematoma. All patients received pharmacotherapy and body posture therapy that was to raise their lower limbs 20 to 30 cm with leg lift pad and get abdominal compressed with customized abdominal belt in supine position. Patients were required to maintain the body posture as much as possible, with the maximum to 16 to 18 hours per day. Patients with unilateral hematoma should tilt the head to the affected side and avoid tilting it to the opposite side. For patients with bilateral hematoma, there was no need for head lateralization. Patient were treated with oral dexamethasone and atorvastatin simultaneously. The preliminary efficacy of body posture combined with pharmacotherapy was determined by hematoma improvement rate which was analyzed by Clopper-Pearson method. Then, the multi-center, prospective, randomized controlled trial had carried out in 9 medical centers from August 2020 to November 2021. The stratified block randomization method was adopted. Patients were randomized in a ratio of 1∶1 to either receive pharmacotherapy alone(the control group) or body posture combined with pharmacotherapy(the experiment group) for 3 months and followed up for 6 months. Effective treatment was defined as complete absorption of hematoma, or the hematoma volume decreased by more than 10 ml and Markwalder grading scale score had improved by more than 1 point compared to the baseline. The efficacy rate and surgery conversion rate at 3 months and recurrence at 6 months were observed. Comparison between groups was performed with paired sample t test, Mann-Whitney U test, χ2 test, corrected χ2 test, or Fisher exact probability method. Logistic regression was used to compare the effective rate and operation rate between the two groups. Results:In the respective study, 30 patients completed follow-up 13 to 353 days after treatment. At the last follow-up, the incidence of almost complete absorption or significantly absorption of hematoma (hematoma volume was significantly reduced accompanied by symptom improvement) was 93.3%. The 95% CI for the incidence that analyzed by the Clopper-Pearson method was 77.9% to 99.2%. One hundred and six patients were enrolled in the multicenter study. Fifty-five patients underwent body posture combined with pharmacotherapy. The age was 74(17) years (range:26 to 92). Thirty-nine patients were males and 16 were females. Fifty-one patients underwent pharmacotherapy alone. The age was 69(12) years (range:48 to 84). Thirty-seven patients were males and 14 were females. The length of body posture recorded in diary card was (15.7±2.3) hours(range:7.6 to 19.3 hours). The efficacy rate in the body posture combined with pharmacotherapy group and pharmacotherapy alone group were 83.6% (46/55) and 56.9% (29/51), respectively at 3 months. The result of the logistic regression analysis showed that the efficacy of body posture combined with pharmacotherapy group was better than that of pharmacotherapy alone group ( OR=3.88,95% CI:1.57 to 9.58, P=0.003). Surgery rate in the body posture combined with pharmacotherapy group and pharmacotherapy alone group were 5.5% (3/55) and 21.6% (11/51) respectively. The result of Logistic regression showed that the pharmacotherapy alone group was more likely to be converted to surgery ( OR=0.21,95% CI:0.05 to 0.80, P=0.023). At the 6 months, no recurrence of cases was found in the body posture combined with pharmacotherapy group. However, the recurrence rate of pharmacotherapy alone group was 6.3% (3/48), there was no significant difference between the two groups ( P>0.05). Conclusion:The effect of body posture combined with pharmacotherapy for chronic subdural hematoma is better than that of pharmacotherapy alone.

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