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To study the efficacy and safety of upadacitinib in children with refractory inflammatory bowel disease (IBD). Clinical data of eight children with refractory IBD treated with upadacitinib at the Children's Hospital, Zhejiang University School of Medicine, from December 1, 2023 to May 20, 2025 were retrospectively collected to evaluate efficacy and safety. Eight children were included [Crohn's disease (CD), n=5; ulcerative colitis (UC), n=3]; seven were male and one was female. At baseline, six patients had mild disease activity (CD, n=4; UC, n=2) and two had severe activity (CD, n=1; UC, n=1). In UC, all three patients achieved clinical remission by week 2; the week-8 steroid-free clinical remission rate was 67% (2/3). In CD, after 4 weeks of upadacitinib, one patient achieved a clinical response and two switched to other drugs due to persistent activity; the week-12 clinical response rate was 20% (1/5). After 12 weeks, one additional patient switched therapy due to disease activity, and two received combination therapy with thalidomide and exclusive enteral nutrition, respectively. The median follow-up duration was 28 weeks. Overall, five patients achieved clinical remission; among CD, two achieved clinical remission at weeks 14 and 20, respectively. Among eight patients, one episode of norovirus infection occurred, complicated by hypovolemic shock. Upadacitinib can induce a rapid response in children with refractory UC. In refractory CD, combining upadacitinib with agents of different mechanisms may increase the clinical remission rate. Multicenter studies with larger samples are required for confirmation, and vigilance for infection during treatment is warranted. 目的: 分析乌帕替尼治疗难治性炎症性肠病(inflammatory bowel disease, IBD)患儿的有效性及安全性。方法: 回顾性收集2023年12月1日—2025年5月20日在浙江大学医学院附属儿童医院接受乌帕替尼治疗的8例难治性炎症性肠病(inflammatory bowel disease, IBD)患儿的临床资料,评估乌帕替尼对难治性IBD患儿的有效性及安全性。结果: 8例患儿中,克罗恩病(Crohn's disease, CD)5例,溃疡性结肠炎(ulcerative colitis, UC)3例。男7例,女1例。在基线水平,疾病轻度活动期6例(CD 4例,UC 2例),重度活动期2例(CD 1例,UC 1例)。3例UC患儿经乌帕替尼治疗2周均获得临床缓解,8周无激素临床缓解率67%(2/3);5例CD患儿经乌帕替尼治疗4周后,1例获得临床应答,2例因疾病活动更换为其他药物,12周临床应答率为20%(1/5),12周后1例因疾病活动更换药物,另2例分别联合沙利度胺及全肠内营养治疗。随访时长中位数为28周。最终5例患儿获得临床缓解,2例CD患儿分别于第14周及第20周获临床缓解。8例患儿共发生1例次诺如病毒感染,合并低血容量性休克。结论: 乌帕替尼对难治性UC患儿可快速起效,对难治性CD患儿,乌帕替尼联合不同作用机制的药物治疗可增加临床缓解率,仍需要多中心、大样本的研究进一步证实,治疗过程中需警惕感染的风险。.
Based on the teaching practice of the course of Selected Readings of Acupuncture and Moxibustion Medical Classics, this paper explores the introduction of Huangdi Mingtang Jing Jijiao into the teaching process to assist students in constructing a framework of the early academic origins of acupuncture, deepening their understanding of theories related to acupoints (especially the tracing and differentiation of acupoint indications), training their ability to read classical acupuncture medical texts, and cultivating rigorous academic thinking. Through the combination of guided classroom lectures, group thematic discussions, and textual collation practice, students are able not only to master the core knowledge of the course, but also to experience a research-oriented learning process of "from literature to clinic, from theory to practice" in tasks such as organizing acupoint indications. This approach effectively bridges the theoretical gap between ancient and modern times, and enhances the comprehensive quality of students majoring in acupuncture and tuina. 本文立足于《针灸医籍选读》课程教学实践,探讨将《黄帝明堂经辑校》一书引入教学环节,帮助学生构建早期针灸学术源流框架、深化对腧穴相关理论的理解(尤其是腧穴主治病症的溯源与甄别)、训练针灸古典医籍研读能力以及培养严谨学术思维的具体应用路径与初步成效。通过课堂引导式讲授、分组专题研讨以及文本校勘实践等多种教学方式的结合,学生不仅能够掌握本课程的核心知识,更能在诸如腧穴主治病症的梳理过程中,体验“从文献到临床、从理论到实践”的研究性学习过程,有效弥合了古今理论隔阂,提升针灸推拿学专业学生的综合素养。.
To observe the effect of acupotomy on cartilage degeneration in rabbits with knee osteoarthritis (KOA) from the perspectives of force line and knee joint stress distribution, and to explore the biomechanical mechanism of acupotomy for KOA. A total of 24 male New Zealand rabbits were randomly divided into a normal group, a model group, an electroacupuncture (EA) group and an acupotomy group, 6 rabbits in each group. Except for the normal group, in the other 3 groups, the left hind limbs were immobilized for 6 weeks to establish KOA model using the modified Videman method. In the EA group, EA was applied at left "Neixiyan" (EX-LE4), "Dubi" (ST35), "Zusanli" (ST36) and "Yanglingquan" (GB34), with disperse-dense wave, in frequency of 2 Hz/100 Hz and current of 3 mA, 20 min each time, once every other day. In the acupotomy group, acupotomy intervention was delivered at the insertion site of the vastus medialis tendon, insertion site of the vastus lateralis tendon, etc. of the left knee joint, once a week. The interventions lasted for 3 weeks in the EA group and the acupotomy group. Before and after intervention, the Lequesne MG score was observed. After intervention, the force line angle of knee joint was measured by magnetic resonance imaging (MRI); the stress distribution of the knee joint was observed by the three-dimensional finite element analysis; the morphology of knee joint cartilage was observed by HE staining; the protein expression of type Ⅱ collagen (Col-Ⅱ) and Aggrecan in the knee joint cartilage was detected by Western blot. Compared with the normal group, the Lequesne MG score was increased (P<0.01), the knee joint force line angle was increased (P<0.05), and the protein expression of Col-Ⅱ and Aggrecan in the knee joint cartilage was decreased (P<0.01) in the model group. Compared with the model group, the Lequesne MG scores were decreased (P<0.05, P<0.01), the knee joint force line angles were decreased (P<0.05), and the protein expression of Col-Ⅱ and Aggrecan in the knee joint cartilage was increased (P<0.05) in the EA group and the acupotomy group. All the above indexes in the acupotomy group were superior to those in the EA group (P<0.05). Severe cartilage damage, disordered arrangement of chondrocytes and unclear tide line could be observed in the model group. Compared with the model group, the damage of knee joint cartilage was alleviated, the arrangement of chondrocytes was more orderly and the tide line was clearer in the EA group and the acupotomy group, and the improvement of knee joint cartilage damage in the acupotomy group was superior to that in the EA group. Compared with the normal group, in the model group, the peak Von Mises stress and peak contact stress, the stress value and the contact stress value in the high-stress areas of the knee joint were increased (P<0.05), the stress was more concentrated. Compared with the model group, in the EA group and the acupotomy group, the above stress-related indexes were decreased (P<0.05), the stress concentration was relieved, and those in the acupotomy group were superior to the EA group (P<0.05). Compared with the normal group, in the model group, the ratios and the contact areas of the medial and lateral condyles of the meniscus, femoral cartilage and tibial cartilage were significantly deviated. Those in the EA group and the acupotomy group tended to be more similar to the normal group, and those in the acupotomy group were superior to the EA group. Acupotomy can correct the knee joint force line, improve the stress concentration, delay cartilage degeneration of the knee joint, thereby improving the symptoms of KOA. 目的:从力线、膝关节应力分布角度观察针刀对膝关节骨关节炎(KOA)兔软骨退化的影响,探讨针刀干预KOA的生物力学机制。 方法:将24只雄性新西兰兔随机分为正常组、模型组、电针组、针刀组,每组6只。除正常组外,其余3组兔左后肢采用改良Videman法制动6周以建立KOA模型。电针组予电针左侧“内膝眼”“犊鼻”“足三里”“阳陵泉”,疏密波,频率2 Hz/100 Hz,电流3 mA,每次20 min,隔日1次;针刀组于左侧膝关节股内侧肌肌腱止点、股外侧肌肌腱止点等处行针刀干预,每周1次,均干预3周。干预前后,观察各组兔奎森功能障碍指数(Lequesne MG)评分。干预后,采用磁共振成像(MRI)观察各组兔膝关节力线角度;采用三维有限元分析法观察各组兔膝关节应力分布情况;采用HE染色法观察各组兔膝关节软骨形态学;采用Western blot法检测各组兔膝关节软骨Ⅱ型胶原蛋白(Col-Ⅱ)和聚集蛋白聚糖(Aggrecan)蛋白表达。 结果:与正常组比较,模型组Lequesne MG评分升高(P<0.01),膝关节力线角度增大(P<0.05),膝关节软骨Col-Ⅱ、Aggrecan蛋白表达降低(P<0.01);与模型组比较,电针组与针刀组Lequesne MG评分降低(P<0.05,P<0.01),膝关节力线角度减小(P<0.05),膝关节软骨Col-Ⅱ、Aggrecan蛋白表达增高(P<0.05);针刀组上述指标均优于电针组(P<0.05)。模型组膝关节软骨损伤严重,软骨细胞排列紊乱,潮线不清晰;与模型组比较,针刀组、电针组膝关节软骨表面损伤均减轻,软骨细胞排列更为有序,潮线更为清晰,针刀组膝关节软骨损伤改善程度优于电针组。与正常组比较,模型组Von Mises应力峰值、接触应力峰值及高应力区域应力值、高应力区域接触应力值升高(P<0.05),应力分布更集中。与模型组比较,针刀组与电针组上述各项应力指标均降低(P<0.05),应力集中程度减轻,且针刀组优于电针组(P<0.05)。与正常组比较,模型组半月板、股骨软骨、胫骨软骨内外侧髁比值及内外侧接触面积均显著偏离;针刀组与电针组半月板、股骨软骨、胫骨软骨内外侧髁比值和内外侧接触面积均接近正常水平,且针刀组优于电针组。 结论:针刀干预可纠正膝关节力线,改善膝关节应力集中现象,延缓软骨退化,从而改善KOA症状。.
The diagnosis of SMARCA4-altered pulmonary tumors primarily relies on the detection of protein loss by immunohistochemistry (IHC). However, the concordance between IHC and genetic testing results, as well as the clinical and biological differences caused by various mutation types, require further investigation. This study aimed to explore the clinicopathological, genomic characteristics, as well as the prognosis, of these tumors. A total of 18 consecutive cases of pulmonary tumor patients with SMARCA4 deficiency/mutation confirmed by IHC or genetic testing were retrospectively enrolled for clinical, genomic, and prognostic analysis. Based on the variant type, patients were categorized into Class 1 (protein loss or loss-of-function gene mutations, n=10) and Class 2 (missense mutation or other variants of unknown significance without protein loss, n=8) for intergroup comparison. Seventeen cases were diagnosed with non-small cell lung cancer (NSCLC) and one was diagnosed with thoracic SMARCA4-deficient undifferentiated tumor (SD-UT). Genotype-phenotype correlation analysis revealed that truncating mutations consistently led to protein loss, whereas missense mutations mostly did not. Two cases with protein loss but negative genetic testing results were identified. Class 1 alterations showed trends towards higher tumor mutational burden (TMB) (median 9.3 vs 4.7 Muts/Mb) and lower programmed cell death ligand 1 (PD-L1) expression (60.0% vs 25.0% with <1% expression) compared to Class 2, but the differences were not statistically significant (P>0.05). Two patients with co-occurring epidermal growth factor receptor (EGFR) L858R mutations showed poor initial response to third-generation EGFR-tyrosine kinase inhibitor monotherapy, but the efficacy was observed after combination with other therapies. Among stage IV patients, the median overall survival (OS) was 10.3 months for Class 1 and 19.9 months for Class 2 (P=0.967). Fourteen patients (77.8%) received immune checkpoint inhibitors (ICIs) combined with chemotherapy. Among them, 8 patients (57.1%) achieved an OS exceeding 12 months, and 7 patients (50.0%) exceeded 24 months. SMARCA4 gene mutations are not entirely consistent with IHC protein loss, necessitating combined interpretation. When SMARCA4 alterations co-exist with EGFR mutations, targeted monotherapy efficacy may be limited, and combination strategies should be considered. Classifying SMARCA4 alterations into Class 1 and Class 2 may have prognostic implications and could help predict the tumor immune microenvironment status (TMB and PD-L1). ICIs combined with chemotherapy is currently one of the main treatment options for patients with advanced SMARCA4-altered pulmonary tumors and showed potential efficacy in this study. 【中文题目:18例SMARCA4缺失或基因突变肺部肿瘤患者的临床病理与基因特征以及预后分析】 【中文摘要:背景与目的 SMARCA4变异肺部肿瘤的诊断主要依赖于免疫组化(immunohistochemistry, IHC)检测蛋白缺失,但其与基因检测结果的一致性以及不同突变类型所导致的临床与生物学差异仍有待进一步探索。本研究旨在探讨该类肿瘤的临床病理、基因特征及预后。方法 回顾性纳入18例经IHC或基因检测证实为SMARCA4缺失/基因突变的肺部肿瘤连续病例,进行临床基因特征和预后分析。根据变异类型分为1类(蛋白缺失或功能丧失性基因突变,n=10)和2类(错义突变或其他意义不明突变且蛋白非缺失,n=8),并进行组间比较。结果 17例为非小细胞肺癌(non-small cell lung cancer, NSCLC),1例为胸部SMARCA4缺失型未分化肿瘤(SMARCA4-deficient undifferentiated tumor, SD-UT)。基因型与表型分析显示:截短突变均导致蛋白缺失,错义突变多不引起缺失,2例蛋白缺失但基因检测阴性。1类较2类变异有更高的肿瘤突变负荷(tumor mutational burden, TMB)(中位数:9.3 vs 4.7 Muts/Mb)和更低的程序性死亡-配体1(programmed cell death ligand 1, PD-L1)表达(<1%者占比:60.0% vs 25.0%)趋势,但差异无统计学意义(P>0.05)。2例表皮生长因子受体(epidermal growth factor receptor, EGFR)L858R共突变患者初始第三代EGFR靶向单药疗效不佳,联合其他治疗见效。IV期患者中1类与2类变异的中位总生存期(overall survival, OS)分别为10.3和19.9个月(P=0.967)。14例(77.8%)患者接受了免疫检查点抑制剂(immune checkpoint inhibitors, ICIs)联合化疗,其中8例(57.1%)OS超12个月,7例(50.0%)超24个月。结论 SMARCA4基因突变与蛋白缺失不完全一致,需联合判读。SMARCA4变异与EGFR突变共存时,单药靶向疗效可能有限,应考虑联合策略。将SMARCA4变异区分为1类与2类,可能具有预后提示价值,并有助于预测肿瘤的免疫微环境状态(TMB及PD-L1)。ICIs联合化疗的方案可能是目前SMARCA4变异晚期肺部肿瘤患者的主要治疗选择之一,在本研究中显示出潜在疗效。
】 【中文关键词:肺肿瘤;胸部SMARCA4缺失型未分化肿瘤;SMARCA4突变;SMARCA4 (BRG1)缺失;EGFR突变;靶向治疗;免疫检查点抑制剂;预后】.
To observe the immediate regulatory effect of electroacupuncture (EA) on gastric motility via the vagus nerve in mice with diabetic gastroparesis (DGP), and to explore the potential central mechanism related to vagus nerve underlying the effects of acupoints. A total of 27 male C57BL/6 mice of clean grade were selected. Of them, 12 mice were randomly divided into a control group 1 and a model group 1, 6 mice in each group. In the two groups, EA was applied at "Zusanli" (ST36), "Neiguan" (PC6) and "Zhongwan" (CV12), and the intragastric pressure-related indexes were detected. After subdiaphragmatic vagus nerve transection (sVX), the same EA stimulation was delivered, and the intragastric pressure-related indexes were detected again in the two groups. The remaining 15 mice were randomly divided into a control group 2, a model group 2, a Zusanli group, a Neiguan group and a Zhongwan group, 3 mice in each group. In the Zusanli group, the Zhongwan group and the Neiguan group, EA was applied at "Zusanli" (ST36), "Zhongwan" (CV12) and "Neiguan" (PC6), respectively, the number of activated choline acetyltransferase (ChAT) neurons in the dorsal motor nucleus of the vagus nerve (DMV) was detected by immunofluorescence staining. Except the control groups 1 and 2, in the remaining groups, diabetes model was established by intraperitoneal injection of streptozotocin (120 mg/kg) combined with high-fat diet feeding. Eight weeks into modeling, the gastric emptying rate was measured to verify the DGP model establishment. Continuous wave was adopted for all EA interventions, of a frequency of 10 Hz and a current of 1 mA. Compared with the control group 1, the fasting blood glucose was increased (P<0.001), the body weight was increased 8 weeks into modeling (P<0.01), the gastric emptying rate was decreased (P<0.001) in the model group 1. Compared with the baseline before sVX (pre-sVX), the gastric motility frequency after sVX (post-sVX) was decreased in the control group 1 and the model group 1 (P<0.001, P<0.05). Compared with pre-sVX, the gastric motility amplitude, area under the curve (AUC) and frequency post-sVX of EA at "Zusanli" (ST36) and "Neiguan" (PC6) were decreased in the control group 1 and the model group 1 (P<0.001). Compared with EA at "Neiguan" (PC6) pre-sVX, the gastric motility amplitude and AUC pre-sVX of EA at "Zusanli" (ST36) were increased in the control group 1 and the model group 1 (P<0.001). Compared with the control group 2, the number of activated ChAT neurons in the DMV was decreased in the model group 2 (P<0.001). Compared with the model group 2, the number of activated ChAT neurons in the DMV was increased in the Zusanli group and the Neiguan group (P<0.001, P<0.05). Compared with the Neiguan group, the number of activated ChAT neurons in the DMV was increased in the Zusanli group (P<0.01). EA at both "Zusanli" (ST36) and "Neiguan" (PC6) can promote gastric motility in DGP mice, and the effect of "Zusanli" (ST36) is superior to that of "Neiguan" (PC6). Its neural mechanism may be related to the regulation of gastric motility by descending ChAT neurons in the DMV through the vagus nerve. 目的:观察电针经迷走神经对糖尿病性胃轻瘫(DGP)小鼠胃运动的即刻调控作用,探讨穴位产生作用可能的迷走神经相关中枢机制。 方法:选取清洁级雄性C57BL/6小鼠27只,将其中12只小鼠随机分为对照1组、模型1组,每组6只,给予电针“足三里”“内关”“中脘”并检测胃内压相关指标,离断小鼠膈下迷走神经,再次给予上述穴位相同电刺激并检测胃内压相关指标;剩余15只小鼠随机分为对照2组、模型2组、足三里组、内关组、中脘组,每组3只,足三里组、内关组、中脘组分别予电针相应穴位,采用免疫荧光染色法观察小鼠迷走神经运动背核(DMV)中胆碱乙酰转移酶(ChAT)神经元激活数量。除对照1组和对照2组外,余组采用腹腔注射链脲佐菌素(120 mg/kg)联合高脂饲料喂养建立糖尿病模型,8周后检测小鼠胃排空率验证DGP造模情况;电针干预均采用连续波,频率10 Hz,电流1 mA。 结果:与对照1组比较,模型1组小鼠空腹血糖升高(P<0.001),造模第8周体质量升高(P<0.01),胃排空率降低(P<0.001)。与膈下迷走神经离断术前(pre-sVX)基线比较,对照1组、模型1组小鼠膈下迷走神经离断术后(post-sVX)胃运动频率下降(P<0.001,P<0.05);与pre-sVX比较,对照1组、模型1组post-sVX电针“足三里”“内关”胃运动振幅、曲线下面积、频率均下降(P<0.001);与pre-sVX电针“内关”比较,对照1组、模型1组pre-sVX电针“足三里”胃运动振幅、曲线下面积均升高(P<0.001)。与对照2组比较,模型2组小鼠DMV中ChAT神经元激活数量减少(P<0.001);与模型2组比较,足三里组、内关组小鼠DMV中ChAT神经元激活数量增加(P<0.001,P<0.05);与内关组比较,足三里组小鼠DMV中ChAT神经元激活数量增加(P<0.01)。 结论:电针“足三里”“内关”均能促进DGP小鼠胃运动,“足三里”较“内关”促进胃运动的作用更显著,其神经机制可能与DMV中ChAT神经元下行经迷走神经调控胃动力相关。.
To observe the long-term clinical efficacy and safety of fire needling in the treatment of knee osteoarthritis. Sixty patients with knee osteoarthritis were randomized into an observation group (30 cases, 4 cases dropped out, 1 case was eliminated) and a control group (30 cases, 4 cases dropped out, 2 cases were eliminated). Yanglingquan (GB34), Neixiyan (EX-LE4), Dubi (ST35), Xuehai (SP10), Liangqiu (ST34) and 1-2 ashi points on the affected side were selected in the two groups. Fire needling was applied in the observation group, while conventional filiform needling was applied in the control group. The treatments in both groups were delivered once every other day, 3 times a week for 2 consecutive weeks. The Western Ontario and McMaster Universities osteoarthritis index (WOMAC) score was observed before and after treatment, as well as at follow-ups of 6 months and 1 year after treatment completion, the clinical efficacy and safety were evaluated after treatment and at follow-ups of 6 months and 1 year after treatment completion. After treatment and at 6-month follow-up, the pain, stiffness, difficulty in daily activities scores and total scores of WOMAC were decreased compared with those before treatment in both groups (P<0.05). At 1-year follow-up, the pain, stiffness, difficulty in daily activities scores and total score of WOMAC were decreased compared with those before treatment in the observation group (P<0.05), the pain score of WOMAC was decreased compared with that before treatment in the control group (P<0.05). After treatment, the pain score, stiffness score and total score of WOMAC in the observation group were lower than those in the control group (P<0.05). At 6-month follow-up, the pain, stiffness, difficulty in daily activities scores and total score of WOMAC in the observation group were lower than those in the control group (P<0.05). At 1-year follow-up, the stiffness score, difficulty in daily activities score and total score of WOMAC in the observation group were lower than those in the control group (P<0.05). At 6-month follow-up, the total effective rate was 76.0% (19/25) in the observation group, which was higher than 41.7% (10/24) in the control group (P<0.05). At 1-year follow-up, the total effective rate was 64.0% (16/25) in the observation group, which was higher than 33.3% (8/24) in the control group (P<0.05). No acupuncture-related adverse reactions or events occurred in the two groups. Fire needling effectively improves the symptoms of KOA, and has a definite long-term clinical efficacy. 目的:观察火针疗法治疗膝骨关节炎的远期临床疗效及安全性。 方法:将60例膝骨关节炎患者随机分为观察组(30例,脱落4例、剔除1例)和对照组(30例,脱落4例、剔除2例)。两组均取患侧阳陵泉、内膝眼、犊鼻、血海、梁丘、阿是穴1~2个,观察组予火针治疗,对照组予毫针针刺治疗,均隔日1次,每周3次,连续治疗2周。分别在治疗前后及治疗结束后6个月、1年随访时观察两组患者西安大略和麦克马斯特大学骨关节炎指数(WOMAC)量表评分,并在治疗后及治疗结束后6个月、1年随访时评定两组临床疗效及安全性。 结果:治疗后及随访6个月,两组患者WOMAC量表疼痛、僵硬、日常活动难度评分及总分均较治疗前降低(P<0.05);随访1年,观察组患者WOMAC量表疼痛、僵硬、日常活动难度评分及总分均较治疗前降低(P<0.05),对照组患者WOMAC量表疼痛评分较治疗前降低(P<0.05)。治疗后,观察组患者WOMAC量表疼痛、僵硬评分及总分均低于对照组(P<0.05);随访6个月,观察组患者WOMAC量表疼痛、僵硬、日常活动难度评分及总分均低于对照组(P<0.05);随访1年,观察组患者WOMAC量表僵硬、日常活动难度评分及总分均低于对照组(P<0.05)。随访6个月,观察组总有效率为76.0%(19/25),高于对照组的41.7%(10/24,P<0.05);随访1年,观察组总有效率为64.0%(16/25),高于对照组的33.3%(8/24,P<0.05)。两组患者均未出现与针刺相关的不良反应与不良事件。 结论:火针疗法可有效改善KOA症状,远期临床疗效确切。.
Objective To systematically evaluate the effects of acute and long-term exercise interventions on the physical health of children with Prader-Willi syndrome (PWS). Methods A systematic review was conducted following the preferred reporting items for systematic reviews and meta-analyses of non-randomized studies of interventions guidelines.Relevant studies from 1987 to 2023 were retrieved from PubMed,Web of Science,and EBSCO databases.Eligible studies were primarily non-randomized controlled trials focusing on acute or long-term exercise interventions in the children with PWS.Outcome measures included cardiorespiratory function,muscle strength,body composition,metabolism,and inflammation. Results A total of 19 studies were included.Studies of acute exercise showed that the children with PWS had lower cardiorespiratory function and muscle strength than normal or obese children,but similar metabolic responses.Long-term exercise interventions resulted in significant body mass index reduction,increased muscle mass,improved cardiorespiratory function,and regulation of inflammation markers such as interleukin-8.In addition,exercise improved the quality of life in the children with PWS. Conclusions Exercise interventions positively impact the health of children with PWS by reducing the body mass index,increasing the muscle strength,improving the cardiorespiratory function,and regulating inflammation.Exercise intervention combined with hormone therapy and nutritional intervention can serve as an effective rehabilitation regimen for the health management of children with PWS.Future research should explore optimal exercise prescriptions and long-term effects and mechanisms. 目的 系统评估急性及长期运动干预对普拉德-威利综合征(PWS)儿童体质健康的影响。方法 遵循非随机干预研究的系统综述和Meta分析报告规范,通过检索PubMed、Web of Science、EBSCO等数据库,收集1987至2023年发表的关于运动干预PWS儿童体质健康影响的文献。纳入的文献主要为非随机对照试验,研究对象为PWS儿童,干预措施为急性或长期运动训练,结局指标包括心肺功能、肌肉力量、体成分、代谢指标及炎症反应等。对纳入的研究进行偏倚风险评估,并汇总分析运动干预的效果。结果 共纳入19篇研究,急性运动干预研究显示PWS儿童的心肺功能和肌肉力量均显著低于正常儿童或肥胖对照组,但其代谢反应与对照组相似。长期运动干预结果显示,PWS儿童的体重指数显著下降,肌肉质量增加,心肺功能和身体协调性均显著改善,且长期运动调节部分炎症标志物如白细胞介素-8的水平。此外,部分研究表明运动干预提升了PWS儿童的生活质量。结论 运动干预对PWS儿童的体质健康具有多方面的积极影响,尤其在控制体重、提高肌肉力量、改善心肺功能及调节炎症反应方面具有重要作用。结合激素治疗和营养干预,运动干预可为PWS儿童的健康管理提供有效的康复方案,未来应进一步优化运动处方,探索长期干预的效果和机制。.
To investigate the efficacy and safety of reduced-dose emicizumab (EMI) prophylaxis in infants under one year of age with severe hemophilia A (HA). Medical records of 11 infants aged ≤12 months with severe HA who received reduced-dose EMI in the Department of Hematology and Oncology, Shenzhen Children's Hospital, from July 2022 to June 2025 were retrospectively reviewed. Efficacy and safety were analyzed statistically. The median age at EMI initiation was 7.9 months (range 4.2-11.2 months). The median loading dose (first 4 weeks) and median maintenance dose were 6.2 mg/kg per 4 weeks (range 2.8-12.6 mg/kg per 4 weeks) and 3.1 mg/kg per 4 weeks (range 2.0-4.8 mg/kg per 4 weeks), respectively. The median duration of EMI prophylaxis was 15.8 months (range 1.7-36.0 months). After treatment, the median (range) annualized bleeding rate, spontaneous annualized bleeding rate, and annualized treated bleeding rate were 0.5 (0-6.2), 0 (0-0.5), and 0 (0-3.0), respectively. All decreased compared with pre-treatment, and the reduction in spontaneous annualized bleeding rate was statistically significant (P<0.05). Overall, 64% (7/11) of patients experienced no bleeding events requiring treatment. No EMI-related adverse events were observed. Reduced-dose EMI prophylaxis effectively controls bleeding in infants with hemophilia A and shows good safety. 目的: 探讨1岁以下重型血友病A(hemophilia A, HA)患儿接受减低剂量艾美赛珠单抗(emicizumab, EMI)治疗的有效性和安全性。方法: 回顾性收集2022年7月—2025年6月于深圳市儿童医院血液肿瘤科接受减低剂量EMI治疗的11例年龄≤12个月的重型HA患儿资料,对其治疗的有效性及安全性进行统计分析。结果: 11例患儿开始使用EMI的中位年龄(范围)为7.9(4.2~11.2)个月,EMI的中位负荷剂量(最初4周的剂量)(范围)和中位维持剂量(范围)分别为6.2(2.8~12.6)mg/(kg·4周)和3.1(2.0~4.8)mg/(kg·4周)。EMI预防治疗的中位时长(范围)为15.8(1.7~36.0)个月。治疗后出血指标:年化出血率、自发性年化出血率及年化治疗出血率的中位数(范围)分别为0.5(0~6.2)、0(0~0.5)和0(0~3.0),均较治疗前降低,其中自发性年化出血率在治疗前后的差异具有统计学意义(P<0.05)。64%(7/11)的患儿未发生需治疗的出血事件,所有患儿均无EMI相关不良事件。结论: 减低剂量EMI用于婴儿血友病A患者预防治疗可以有效控制出血,治疗效果显著,安全性好。.
Gastroesophageal reflux disease (GERD) in infants and young children is a common digestive disorder with diverse clinical manifestations that often overlap with physiological reflux and behavioral expressions, creating diagnostic challenges. Comprehensive searches were conducted in English- and Chinese-language databases, including PubMed, Web of Science, China National Knowledge Infrastructure, and Wanfang Data, to review the epidemiological characteristics, clinical presentations, and impacts of GERD on children and their families. Particular emphasis is placed on four principal assessment tools [Infant Gastroesophageal Reflux Questionnaire (I-GERQ), GERD Symptom Questionnaire for Infants/Young Children (GSQ-I/YC), I-GERQ Revised (I-GERQ-R), and Gastrointestinal and Gastroesophageal Reflux Scale for Infants and Toddlers (GIGER)], appraising their development background, structural characteristics, reliability, validity, and clinical application value. The aim is to support early identification, standardized assessment, and individualized management of GERD in infants and young children. Evidence indicates that the I-GERQ-R shows high specificity, good reliability and validity, and cross-cultural applicability, and is currently recommended for symptom assessment and treatment-response monitoring in GERD among infants and young children, while the GIGER scale shows potential for comprehensive screening. Future research should prioritize translation and development of Chinese versions of GERD questionnaires for infants and young children, as well as the establishment of a multidimensional assessment system. 婴幼儿胃食管反流病(gastroesophageal reflux disease, GERD)是婴幼儿期常见的消化道疾病,其临床表现多样,常与生理性反流和行为表现重叠,致使诊断存在挑战。该文通过系统检索PubMed、Web of Science、中国知网和万方数据等中英文数据库,系统综述了婴幼儿GERD的流行病学特征、临床表现及其对患儿和家庭的影响,重点评价了4种主要评估工具(I⁃GERQ、GSQ⁃I/YC、I⁃GERQ⁃R和GIGER量表)的开发背景、结构特点、信效度及临床应用价值,旨在为婴幼儿GERD的早期识别、标准化评估及个体化管理提供理论依据与实践参考。该文发现I⁃GERQ⁃R具有较高的特异性、良好的信效度和跨文化适用性,是目前推荐用于婴幼儿GERD症状评估与疗效监测的有效工具,GIGER量表在全面筛查方面展现出潜力;未来研究应致力于汉化及开发中文版本婴幼儿GERD问卷并建立多维度评估体系。.
To observe the effects of electroacupuncture (EA) on high mobility group box-1 (HMGB1)/ receptor for advanced glycation end products (RAGE)/nuclear factor kappa-B (NF-κB) pathway-mediated neuroinflammatory response and reactive astrocyte in Parkinson's disease (PD) mice, and to explore the mechanism of EA in the prevention and treatment of PD. Thirty-six male C57BL/6 mice were randomly divided into a control group, a model group, and an EA group, with 12 mice in each group. The PD model was established by intraperitoneal injection of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) for 7 consecutive days. From the first day of model establishment, mice in the EA group received EA at "Baihui" (GV20) and bilateral "Shenshu" (BL23), with continuous wave, frequency of 2 Hz and intensity of 2 mA, 15 min each time, once daily, for 14 days. Pole test, hanging test, and gait analysis were used to assess behavioral performance. Immunofluorescence staining was used to detect tyrosine hydroxylase (TH) and glial fibrillary acidic protein (GFAP) positive cells in the substantia nigra of the midbrain. ELISA was used to detect α-synuclein (α-syn) content in the substantia nigra. Western blot was used to detect protein expression levels of TH, HMGB1, RAGE, NF-κB, phosphorylated NF-κB (p-NF-κB), GFAP, tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), and interleukin-10 (IL-10) in the substantia nigra. Real-time quantitative PCR was used to detect mRNA expression levels of HMGB1, RAGE, NF-κB, GFAP, TNF-α, IL-6, and IL-10 in the substantia nigra. Compared with the control group, the model group showed prolonged pole test time (P<0.01), decreased hanging score (P<0.01); shortened stride length and standing time (P<0.01), increased step frequency (P<0.01), and prolonged swing time (P<0.01) of bilateral forelimbs and hindlimbs; the number of TH-positive cells and TH protein expression level, as well as IL-10 protein and mRNA expression levels in the substantia nigra were decreased (P<0.01, P<0.05), while α-syn content and the number of GFAP-positive cells, protein and mRNA expression levels of HMGB1, RAGE, GFAP, TNF-α, and IL-6, as well as p-NF-κB/NF-κB and NF-κB mRNA expression were increased (P<0.05, P<0.01). Compared with the model group, the EA group showed shortened pole test time (P<0.01), increased hanging score (P<0.05); increased stride length (P<0.05, P<0.01), decreased step frequency (P<0.01), prolonged standing time (P<0.05, P<0.01), and shortened swing time (P<0.05) of bilateral forelimbs and hindlimbs; the number of TH-positive cells, TH protein level, and IL-10 protein and mRNA expression levels in the substantia nigra were increased (P<0.01, P<0.05), while α-syn content and the number of GFAP-positive cells, protein and mRNA expression levels of HMGB1, RAGE, GFAP, TNF-α, and IL-6, as well as p-NF-κB/NF-κB and NF-κB mRNA expression were decreased (P<0.05, P<0.01). EA can improve motor dysfunction in PD mice, protect dopaminergic (DA) neurons, and reduce α-syn protein aggregation, thereby exerting a neuroprotective effect. This effect may be related to inhibition of reactive astrocyte activation and the HMGB1/RAGE/NF-κB pathway, thereby reducing neuroinflammatory responses. 目的:观察电针(EA)对帕金森病(PD)小鼠高迁移率族蛋白B1(HMGB1)/晚期糖基化终末产物受体(RAGE)/核因子κB(NF-κB)通路介导的神经炎症反应及反应性星形胶质细胞的影响,探讨电针防治PD的作用机制。 方法:将36只C57BL/6雄性小鼠随机分为对照组、模型组和电针组,每组12只。采用连续7 d腹腔注射1-甲基-4-苯基-1,2,3,6-四氢吡啶(MPTP)建立PD小鼠模型。自模型制备首日起,对电针组小鼠给予电针“百会”和双侧“肾俞”干预,予连续波,频率2 Hz,强度2 mA,每次15 min,每天1次,持续14 d。采用爬杆、悬挂和步态实验检测小鼠行为表现;免疫荧光染色法检测小鼠中脑黑质区酪氨酸羟化酶(TH)和胶质纤维酸性蛋白(GFAP)阳性细胞数;ELISA法检测小鼠中脑黑质区α-突触核蛋白(α-syn)含量;Western blot法检测小鼠中脑黑质区TH、HMGB1、RAGE、NF-κB、磷酸化核因子κB(p-NF-κB)、GFAP、肿瘤坏死因子-α(TNF-α)、HYPERLINK "https://www.baidu.com/s?rsv_dl=re_dqa_generate&sa=re_dqa_generate&wd=%E7%99%BD%E7%BB%86%E8%83%9E%E4%BB%8B%E7%B4%A0-6&rsv_pq=acdba3be0001d32d&oq=IL-6%E3%80%81IL-10%E5%85%A8%E7%A7%B0&rsv_t=8e4b0e+iCop+YyyzFG6jxhsBVjIPoSlFCUVJEGqzpikXHro22omoZwOgsp4zg/pJ35WJSJo&tn=15007414_15_dg&ie=utf-8" \t "_blank" 白细胞介素-6(IL-6)、白细胞介素-10(IL-10)蛋白表达;实时荧光定量PCR法检测小鼠中脑黑质区HMGB1、RAGE、NF-κB、GFAP、TNF-α、IL-6、IL-10 mRNA表达。 结果:与对照组比较,模型组小鼠爬杆时间延长(P<0.01),悬挂得分降低(P<0.01);双侧前肢、后肢步长及站立时间缩短(P<0.01),步频增加(P<0.01),摆动时间延长(P<0.01);中脑黑质区TH阳性细胞数和TH蛋白表达、IL-10蛋白及mRNA表达均降低(P<0.01,P<0.05);中脑黑质区α-syn含量和GFAP阳性细胞数,HMGB1、RAGE、GFAP、TNF-α、IL-6蛋白及mRNA表达,p-NF-κB/NF-κB及NF-κB mRNA表达均升高(P<0.05,P<0.01)。与模型组比较,电针组小鼠爬杆时间缩短(P<0.01),悬挂得分升高(P<0.05);双侧前肢、后肢步长增加(P<0.05,P<0.01),步频减慢(P<0.01),站立时间延长(P<0.05,P<0.01),摆动时间缩短(P<0.05);中脑黑质区TH阳性细胞数与TH蛋白表达、IL-10蛋白及mRNA表达均升高(P<0.01,P<0.05);中脑黑质区α-syn含量和GFAP阳性细胞数,HMGB1、RAGE、GFAP、TNF-α、IL-6蛋白及mRNA表达,p-NF-κB/NF-κB及NF-κB mRNA表达均降低(P<0.05,P<0.01)。 结论:电针可以改善PD模型小鼠运动功能障碍,保护多巴胺(DA)能神经元,减少α-syn蛋白聚集,从而发挥神经保护作用。该作用可能与抑制反应性星形胶质细胞活化及HMGB1/RAGE/NF-κB通路的激活,进而减轻神经炎症反应有关。.
This paper reported a case of giant renal hamartoma complicated with renal venous tumor thrombus.MRI was used for diagnosis and renal function evaluation on the affected side before surgery,and the patient underwent robot-assisted laparoscopic radical resection of the left kidney and left renal venous tumor thrombus embolectomy.The operation was successfully completed,and there were no complication or tumor recurrence during the 20-month follow-up after surgery.This case,combined with the literature review,suggests that MRI has important guiding significance in the preoperative evaluation and surgical modality selection of giant renal hamartoma. 分析1例巨大肾错构瘤合并肾静脉瘤栓的病例,术前应用MRI进行诊断及患侧肾功能评估,予该患者行机器人辅助腹腔镜下左肾根治性切除术+左肾静脉瘤栓取出术。手术顺利完成,术后20个月随访期间未见并发症发生及肿瘤复发。结合文献综述,MRI在诊治巨大肾错构瘤的术前评估及手术选择上具有重要的指导意义。.
With the rapid development of biomedical research in China,biobanks have been widely established as crucial infrastructure supporting precision medicine and big data research.However,at the practical level,informed consent-central to safeguarding the rights and interests of research participants-often shows a disconnection between ethical principles and technological realities.This study first examines the Chinese translation of the term "biobank", arguing that "biological sample data bank" better reflects both the data-centric nature and governance complexity of such entities.It then systematically reviews the ethical debates surrounding various models of informed consent,including blanket consent,broad consent,dynamic consent,and meta-consent.Building on this analysis,this paper identifies key ethical elements that an effective informed consent form should include.Special attention is paid to the legitimacy and practical challenges of components such as research purpose,data collection,data use,storage duration,risk disclosure,and return of results.This study aims to provide an ethical foundation and practical guidance for improving the informed consent framework in China's biobank governance,thereby promoting a shift from mere regulatory compliance to robust ethical engagement and fostering a virtuous cycle between public trust and sustainable research development. 随着中国生物医学研究的快速发展,生物样本数据库(Biobank)广泛建立,成为支持精准医学与大数据研究的重要基础设施。然而,在实践层面,知情同意作为保障研究参与者权益的核心机制,其内容设计在伦理理念与技术现实之间存在一些脱节。本文首先对“Biobank”概念的中文译名进行辨析,提出“生物样本数据库”更能体现其数据性与治理复杂性。继而系统梳理不同知情同意模式,包括一揽子知情、广泛知情、动态知情、元知情之间的伦理分歧,并在此基础上归纳出一份有效知情同意书应当包含的关键伦理要素,重点分析研究目的、数据采集、数据使用、储存时间、风险告知与结果反馈等维度的正当性与实践困境。本文旨在为中国生物样本数据库知情同意制度的完善提供伦理依据和实践参考,促进科研治理从合规导向走向伦理充分,实现公众信任与科研可持续性的良性互动。.
To compare the differences between randomized controlled trial (RCT) evidence and physicians' clinical concerns in acupuncture treatment for eczema, and to provide a basis for optimizing clinical practice and future research. RCTs on acupuncture for eczema were systematically searched in CNKI, Wanfang, VIP, SinoMed, PubMed, EMbase, Cochrane Library, and Web of Science databases from inception to December 31, 2023. Evidence from RCTs on acupuncture treatment for eczema was collected and methodological quality was evaluated. A questionnaire on clinical concerns regarding acupuncture treatment for eczema was designed, covering population characteristics (population age, disease type, TCM syndrome), acupuncture intervention measures (types of acupuncture therapies and components of acupuncture protocols), types of control measures of concern, outcome, and selection of evaluation time points. The questionnaire was distributed nationwide among clinicians to investigate their concerns regarding acupuncture treatment for eczema. Differences and similarities between RCT evidence and physicians' clinical concerns were compared across five dimensions: study population, intervention measures, control setting, outcome, and evaluation time points. A total of 243 RCTs and 87 valid questionnaires were included. The RCTs presented certain risks of bias. The evidence was mainly concentrated on chronic eczema of spleen deficiency with dampness excess in adolescents and adults, with insufficient attention to infants, pregnant and postpartum women, and acute/subacute eczema. The intervention measures were mainly filiform needle acupuncture, fire needle, and moxibustion; however, descriptions of acupuncture parameters and treatment courses were insufficient, and there was a lack of studies on intradermal needles and auricular acupoint pressing. Control interventions were mostly glucocorticoids for external use or antihistamines, which were not entirely consistent with clinicians' concerns regarding calcineurin inhibitors, biologics, and other modern therapeutic strategies. Outcomes mainly focused on effective rate and severity scores such as skin lesions and pruritus, whereas clinicians were more concerned with multidimensional outcomes such as quality of life, psychological status, patient satisfaction, recurrence rate, and medication use. Most studies focused only on short-term efficacy at the end of treatment, lacking data on immediate efficacy and long-term follow-up, which did not align with clinical concerns about sustained efficacy and recurrence risk. There are certain differences between current RCT evidence and physicians' clinical concerns regarding acupuncture treatment for eczema. Future research should be guided by clinical questions, optimize population stratification design, refine descriptions of acupuncture intervention parameters, introduce more clinically relevant control groups, construct multidimensional outcome systems, and implement full-course efficacy evaluation to improve the quality of evidence and clinical applicability of acupuncture treatment for eczema. 目的:比较针灸治疗湿疹的随机对照试验(RCT)证据与医生临床关注之间的差异,为优化临床实践及未来研究提供依据。 方法:检索中国期刊全文数据库(CNKI)、万方数据知识服务平台(Wanfang)、维普资讯中文期刊服务平台(VIP)、中国生物医学文献数据库(SinoMed)、PubMed、EMbase、Cochrane Library及Web of Science数据库建库以来至2023年12月31日收录的有关针灸治疗湿疹的RCT文献,收集针灸治疗湿疹的RCT证据并进行方法学质量评价。设计针灸治疗湿疹的临床关注调查问卷,内容涉及人群特征(人群年龄、疾病类型、中医证型)、针灸干预措施(针灸疗法种类、针灸方案要素)、应关注的对照措施种类、结局指标及评价时点的选择等,并在全国范围的临床医生中发放,了解临床医生对针灸治疗湿疹的关注问题。围绕研究人群、干预措施、对照设置、结局指标和评价时点5个维度,比较RCT证据与临床医生关注的异同。 结果:共纳入243篇RCT和87份有效调查问卷。RCT存在一定的偏倚风险,证据多集中于青少年及成年人脾虚湿盛证的慢性湿疹,对婴幼儿、孕产妇及急性/亚急性湿疹关注不足;干预措施以毫针刺、火针和灸法为主,但针灸参数及疗程等信息描述不充分,缺乏对皮内针、耳穴贴压等便捷疗法的研究;对照措施多为外用糖皮质激素或抗组胺药物,与临床医生关注的钙调磷酸酶抑制剂、生物制剂等现代新型治疗策略不完全一致;结局侧重在有效率以及皮损、瘙痒等疾病严重程度评分,而临床医生更关注生活质量、精神心理状态、患者满意度、复发率及用药情况等多维度结局;多数研究仅关注治疗结束时的短期疗效,缺乏即刻疗效及长期随访数据,与临床对疗效持续性和复发风险的关注存在差异。 结论:当前针灸治疗湿疹的RCT证据与医生临床关注间存在一定的差异。未来研究应以临床问题为导向,优化人群分层设计,细化针灸干预参数,引入更具现实意义的对照组,构建多维度结局体系并实施全过程疗效评估,以提升针灸治疗湿疹研究的证据质量与临床适用性。.
Objective To investigate the relationships of various types of renal vascular lesions with the clinical,pathological,and prognostic features of lupus nephritis (LN). Method A retrospective analysis was performed on the clinical and pathological data of LN patients treated in the Peking Union Medical College Hospital over the past 10 years to explore the relationships of different vascular lesions with clinical and pathological features and analyze the effects of renal vascular lesions on kidney prognosis from multiple perspectives. Results Among 644 enrolled LN patients,females accounted for 82.6%,and LN Ⅳ/Ⅳ+Ⅴ (60.4%) was the predominant pathological type.According to the types of renal vascular lesions,the patients were classified into four groups:atherosclerosis (AS)(n=171,26.6%),thrombotic microangiopathy (TMA)(n=78,12.1%),uncomplicated immune complex deposition (ICD)(n=29,4.5%),and lupus vasculopathy (LV)(n=19,3.0%). No renal vascular lesion (NRVL)(n=347,53.9%) was taken as the control group.The average age of patients in the AS group was higher than that in the other groups (all P<0.001).The TMA (P=0.009,P=0.025) and LV groups (P=0.007,P=0.005) had lower levels of complement C3,and higher disease activity index of systemic lupus erythematosus and incidence of acute kidney injury (both P<0.001) than the NRVL and AS groups.The TMA (P<0.001),LV (P<0.001),and AS (P=0.037) groups had lower estimated glomerular filtration rate (eGFR) than the NRVL group,and the eGFR of the TMA group was lower than that of the LV,AS,and ICD groups (all P<0.001).The renal pathological activity index (AI) of each renal vascular lesion group was higher than that of the NRVL group (all P<0.001),and the AI value of the TMA and LV groups was higher than that of the AS group (both P<0.001).There was no significant difference in AI value between TMA and LV groups (P=0.675),while the proportion of fibrinoid necrosis in the LV group was higher than that in the TMA group (P=0.012).The TMA group had the highest degree of chronicity and higher chronic index than the AS (P=0.021),LV (P<0.001),ICD (P<0.001),and NRVL (P<0.001) groups.The AS group had the secondary highest degree of chronicity and higher median chronic index than the NRVL group (P=0.004).The TMA group had the lowest 1-year clinical remission rate and the highest proportion of annual eGFR decline >5 mL/(min·1.73 m2).Both TMA and AS groups showed lower long-term renal survival rates than the NRVL group (P<0.001,P=0.023) respectively. Conclusions Intrarenal vascular lesions are strongly associated with clinical severity and prognosis in LN.LV correlates with acute kidney injury and severe active renal injury,while TMA is the most severe subtype and characterized by high pathological activity,chronicity,poor treatment response,and adverse outcomes.AS,the most common type,does not affect treatment response but predicts unfavorable long-term renal survival. 目的 探讨狼疮性肾炎(LN)不同类型血管病变与患者临床、病理及预后的关系。方法 回顾性分析我院10年间LN患者的临床病理数据,探究不同血管病变与临床病理间的关系及多个角度分析肾血管病变对肾脏预后的影响。结果 共纳入644例LN患者,女性占82.6%,LN Ⅳ/Ⅳ+Ⅴ型占60.4%。根据血管病变类型分为4组:肾小动脉硬化(AS)组171例(26.6%)、血栓性微血管病(TMA)组78例(12.1%)、单纯免疫复合物沉积(ICD)组29例(4.5%)及狼疮血管病(LV)组19例(3.0%),无血管病变(NRVL)组347例(53.9%)作为对照。AS组患者平均年龄显著高于其他各组(P均<0.001)。与NRVL及AS组相比,TMA(P=0.009,P=0.025)和LV组(P=0.007,P=0.005)补体C3水平均显著降低、系统性红斑狼疮疾病活动指数及急性肾损伤的发生率均显著增加(P均<0.001)。除ICD组外,TMA(P<0.001)、LV(P<0.001)和AS组(P=0.037)估算的肾小球滤过率(eGFR)均显著低于NRVL组,其中TMA组的eGFR均显著低于LV、AS和ICD组(P均<0.001)。各肾血管病变组肾脏病理活动指数(AI)均显著高于NRVL组(P均<0.001),TMA和LV组AI值均显著高于AS组(P均<0.001)。TMA与LV组比较,AI值差异无统计学意义(P=0.675),但LV组纤维素样坏死比例显著高于TMA组(P=0.012)。慢性化程度TMA组最高,慢性指数显著高于AS(P=0.021)、LV(P<0.001)、ICD(P<0.001)和NRVL组(P<0.001);其次为AS组,中位慢性指数显著高于NRVL组(P=0.004)。TMA组1年内临床缓解率最低,年eGFR下降>5 mL/(min·1.73 m2)的比例最高。经长期随访,TMA和AS组患者肾脏存活率均显著低于NRVL组(P<0.001,P=0.023)。结论 肾内血管病变与LN临床和预后密切相关。LV与急性肾损伤的发生及严重活动性肾损伤相关;TMA最严重,存在较高的肾脏病理活动性和慢性病变,治疗反应相对较差,预后不佳;AS最常见,其存在不影响治疗反应,但与长期肾脏不良预后相关。.
Objective To understand the demand of parents in Xichang city for the sexuality education training of young children,analyze its influencing factors,and then put forward corresponding health education strategies. Methods Through cluster random sampling,8 kindergartens were selected in Xichang city,Sichuan province.The knowledge,attitude and practice scale for sexuality education of young children was used to conduct a questionnaire survey among parents of young children.The chi-square test and the multivariate Logistic regression model were used for data analysis. Results Among the 1 496 parents,the demand rates for kindergarten teacher training and expert training were 53.5% and 46.3%,respectively,and the total demand rate for training was 65.4%.The results of the multivariate Logistic regression analysis showed that higher paternal educational level (OR=1.42,95%CI=1.11-1.82,P=0.006),female parenthood (OR=1.71,95%CI=1.28-2.28,P<0.001),positive attitude (OR=1.64,95%CI=1.24-2.15,P<0.001),willingness to participate in school-based early childhood sex education with children (OR=2.44,95%CI=1.67-3.49,P<0.001),and more diverse sources of knowledge (OR=15.32,95%CI=5.49-42.79,P<0.001) were positive predictors of parents' overall demand for training.Conversely,children's prior participation in sex education-related courses at school (OR=0.57,95%CI=0.38-0.85,P=0.006) was a negative predictor of such demand. Conclusions The parents in Xichang city have high demand for the sexuality education training of young children but limited ways to knowledge sources.It is recommended that sexuality education training courses both online and offline should be developed according to the characteristics of parents' needs,and the contents,methods,and ways of health education should be optimized.Special attention should be paid to the health education for male parents,parents with low educational attainment,parents with a passive attitude,and parents who have cognitive misunderstandings about or lack knowledge of children's sexuality education.Efforts should be made to popularize sexuality education knowledge,enhance parents' ability to conduct sexuality education for young children,and improve the quality of family sexuality education through home-school cooperation,thus creating a healthy growth environment for young children. 目的 了解西昌市家长对幼儿性教育培训的需求情况,并分析其影响因素,进而提出相应的健康教育策略。方法 采用整群随机抽样方法在四川省西昌市抽取8所幼儿园,对幼儿家长使用《幼儿性教育知信行量表》进行问卷调查,采用χ2检验和多因素Logistic回归模型进行数据分析。结果 1 496名家长的幼儿园教师培训需求率为53.5%,专家培训需求率为46.3%,培训总需求率为65.4%。多因素Logistic回归分析显示,父亲文化程度高(OR=1.42,95%CI=1.11~1.82,P=0.006)、女性家长(OR=1.71,95%CI=1.28~2.28,P<0.001)、态度积极(OR=1.64,95%CI=1.24~2.15,P<0.001)、愿意和孩子一起参加学校的幼儿性教育(OR=2.44,95%CI=1.67~3.49,P<0.001)、知识来源途径数量多(OR=15.32,95%CI=5.49~42.79,P<0.001)是家长培训总需求的促进因素,孩子在学校参加过性教育相关课程情况(OR=0.57,95%CI=0.38~0.85,P=0.006)是家长培训总需求的抑制因素。结论 西昌市家长幼儿性教育培训需求较高,获取知识来源途径较少。建议针对家长需求特点结合线上线下开展性教育培训课程,优化并完善健康教育内容、方式和途径。重点加强男性家长、文化程度相对较低、态度不积极以及对幼儿性教育存在认知误区或缺乏了解的家长的健康教育,普及性教育知识,提升家长的幼儿性教育能力,通过家校合作的方式提高家庭性教育质量,为幼儿营造健康的成长环境。.
Kleine-Levin syndrome is an extremely rare sleep disorder.In addition to recurrent daytime sleepiness,symptoms such as overeating,hypersexuality,cognitive impairment,and mental and behavioral abnormalities usually occur.Hypothalamic dysfunction may be the underlying pathogenesis.Cases with headache are rare.We reported a case of Kleine-Levin syndrome with headache symptoms during narcolepsy,and his headache was characterized by both migraine and cluster headache.It is hypothesized that Kleine-Levin syndrome may have a potential common pathogenesis with functional headaches such as migraine or cluster headache,which may be related to hypothalamic dysfunction. Kleine-Levin综合征是一种极罕见的睡眠障碍疾病,除复发性日间嗜睡外,通常还可出现摄食过多、性欲亢进、认知障碍及精神行为异常等症状,下丘脑功能异常可能是其潜在的发病机制,合并头痛的病例较为罕见。本文报道1例Kleine-Levin综合征患者,发作嗜睡期间出现头痛症状,且其头痛兼具偏头痛及丛集性头痛特点,推测Kleine-Levin综合征可能与偏头痛或丛集性头痛等功能性头痛有潜在共同的发病机制,可能与下丘脑功能异常相关。.
To investigate the effect of intraoperative hypothermia on short-term prognosis in neonates with necrotizing enterocolitis (NEC) undergoing surgery. Clinical data of 150 neonates with NEC who underwent surgery at Children's Hospital of Chongqing Medical University from June 2017 to August 2023 were retrospectively collected. Based on the lowest intraoperative temperature, patients were classified into a normothermia group (≥36.0℃, n=49), a mild hypothermia group (35.0-35.9℃, n=73), a moderate hypothermia group (34.0-34.9℃, n=20), and a severe hypothermia group (<34.0℃, n=8). Postoperative outcomes were analyzed across groups. Intraoperative hypothermia occurred in 101/150 (67.3%) neonates. Mild hypothermia significantly increased the risk of postoperative coagulation disorders (OR=2.170, 95%CI: 1.035-4.552, P<0.05). Postoperative hospital stay was significantly prolonged in the mild (B=12.250, 95%CI: 1.176-23.324, P<0.05) and severe hypothermia groups (B=24.510, 95%CI: 1.645-47.376, P<0.05), while the duration of central venous catheterization was significantly increased in the moderate hypothermia group (B=11.388, 95%CI: 1.625-21.15, P<0.05). Intraoperative hypothermia is common in neonates with NEC undergoing surgery, and different degrees of hypothermia are associated with distinct adverse effects on short-term prognosis. Intraoperative temperature management should be prioritized, and active warming measures should be implemented to improve outcomes. 目的: 探究新生儿坏死性小肠结肠炎(neonatal necrotizing enterocolitis, NEC)术中低体温对近期预后的影响。方法: 回顾性收集2017年6月—2023年8月重庆医科大学附属儿童医院150例NEC手术患儿的资料。按术中最低体温分为正常体温组(≥36.0℃,49例)、轻度低体温组(35.0~35.9℃,73例)、中度低体温组(34.0~34.9℃,20例)和重度低体温组(<34.0℃,8例),分析低体温对预后的影响。结果: 150例患儿中,101例(67.3%)发生术中低体温。轻度低体温显著增加了术后凝血功能障碍的发生风险(OR=2.170,95%CI:1.035~4.552,P<0.05)。轻度低体温(B=12.250,95%CI:1.176~23.324,P<0.05)和重度低体温(B=24.510,95%CI:1.645~47.376,P<0.05)均显著延长了术后住院时长,而中度低体温显著增加了中心静脉置管时长(B=11.388,95%CI:1.625~21.15,P<0.05)。结论: NEC手术患儿术中低体温发生率高,且不同程度低体温对近期预后的影响存在差异。临床需重视术中体温管理,采取主动保温措施改善预后。.
The proportion of elderly patients with advanced non-small cell lung cancer (NSCLC) is on the rise. While programmed death protein 1 (PD-1)/programmed death ligand 1 (PD-L1) inhibitors have revolutionized the management of advanced NSCLC and improved patient survival, but the elderly display prominent immunosenescence features. Age-associated thymic involution, diminished T-cell repertoire diversity, and chronic inflammation directly compromise the efficacy of immunotherapy. Furthermore, the underrepresentation of elderly patients in clinical trials and the paucity of high-quality evidence-based data jointly contribute to significant heterogeneity in their therapeutic responses to PD-1/PD-L1 inhibitors. While patients aged 65-74 years maintain significant survival benefits from immunotherapy - whether as monotherapy or combined regimens - those aged ≥75 years exhibit attenuated efficacy and heightened susceptibility to immune-related adverse events. To address these challenges, Comprehensive Geriatric Assessment (CGA) enables risk-adapted therapeutic strategies, guiding interventions such as adjusted-dose immunotherapy, selective combination therapies, and emerging approaches targeting senescence-associated immune dysfunction. Closing current evidence gaps, particularly for underrepresented octogenarians, remains imperative, as does advancing precision immunotherapy models founded on biological aging metrics rather than chronological age alone. Bridging these knowledge gaps will permit tailored treatment algorithms to optimize outcomes in this clinically complex population.
. 【中文题目:免疫衰老背景下老年晚期NSCLC患者
对PD-1/PD-L1抑制剂反应的异质性及干预策略】 【中文摘要:老年晚期非小细胞肺癌(non-small cell lung cancer, NSCLC)患者的比例逐年上升,尽管程序性死亡受体-1(programmed death protein 1, PD-1)/程序性死亡配体1(programmed death ligand 1, PD-L1)抑制剂彻底改变了晚期NSCLC的治疗格局,为患者带来生存获益,但老年人群因存在免疫衰老特征,衰老引发的胸腺退化、T细胞库多样性降低、慢性炎症削弱了免疫治疗的应答效率;加之临床试验中高龄患者入组不足、循证医学证据匮乏,导致其对PD-1/PD-L1抑制剂的治疗应答呈现显著异质性。临床研究发现,65-74岁患者从PD-1/PD-L1抑制剂单药或联合治疗中获益较明显,而≥75岁高龄人群疗效减弱,且免疫相关不良事件发生率升高。为优化老年患者治疗,基于综合老年评估结果制定个体化干预策略,主要包括低剂量免疫单药治疗、选择性联合治疗模式及靶向免疫衰老的新型疗法。未来研究应填补高龄患者临床证据空白,并推动基于免疫衰老程度和老年生理变化的精准治疗方案,以改善这一特殊人群的治疗结局。
】 【中文关键词:免疫衰老;肺肿瘤;PD-1/PD-L1抑制剂;个体化治疗】.
A 10-year-old boy was admitted with facial edema and proteinuria for two months, occurring nine months after hematopoietic stem cell transplantation. He was clinically diagnosed with nephrotic syndrome and showed no remission after four weeks of standard glucocorticoid therapy, suggesting steroid-resistant disease. Renal biopsy was consistent with membranous nephropathy. Mass spectrometry identified granular co-deposition of IgG and semaphorin 3B (Sema3B) along the glomerular basement membrane, establishing the diagnosis of Sema3B-associated membranous nephropathy. Treatment with glucocorticoids combined with tacrolimus led to a marked reduction in proteinuria, resolution of edema, and clinical stabilization over a three-month follow-up, with no recurrence. This case highlights that Sema3B-associated membranous nephropathy may occur after hematopoietic stem cell transplantation in children and underscores the importance of precise diagnostics in pediatric nephropathies. Future multicenter studies are needed to clarify disease course and genetic susceptibility, develop specific biomarkers and therapies, promote individualized treatment, and improve prognosis. 患儿,男,10岁,因造血干细胞移植后9个月,出现颜面水肿、蛋白尿2个月入院。临床诊断肾病综合征,经规范糖皮质激素治疗4周,尿蛋白未转阴,考虑激素耐药。行肾穿刺活检术,肾脏病理提示膜性肾病,质谱分析肾小球基底膜颗粒状IgG和信号蛋白 3B(semaphorin 3B, Sema 3B)共沉积,诊断为Sema 3B膜性肾病。给予糖皮质激素联合他克莫司治疗后,随访3个月,患儿尿蛋白明显减少,水肿消退,病情稳定,未出现复发。该病例提示儿童造血干细胞移植后可能出现Sema 3B膜性肾病,提示精准诊断在儿科肾病中的重要性,未来需多中心研究明确其病程和遗传易感性,开发特异性标志物和疗法,推动个体化治疗,改善患儿预后。.
This study reviews the pathological behaviors of the primary lesion cells in rheumatoid arthritis, including fibroblasts-like synoviocyte, macrophages, and T cells during the progression of the disease. It analyzes the effects of temperature on phenotypic remodeling and molecular signaling pathways of the relevant cells. After deeply exploring the action mechanism of the thermal stimuli of moxibustion on rheumatoid arthritis, it is found that during moxibustion, the different temperatures of moxibustion may influence the primary lesion cells in rheumatoid arthritis by activating different thermosensitive proteins and thermosensitive pathways, so as to alleviate or cure the disease. 总结类风湿关节炎(RA)主要病变细胞,包括成纤维样滑膜细胞、巨噬细胞和T细胞等,在疾病进展中的病理行为。分析温度对相关细胞的表型重塑和分子信号通路的影响。深入探讨艾灸热刺激在RA治疗中的作用机制,发现在艾灸过程中,不同的艾灸温度可能通过激活不同的热敏蛋白及热敏通路,对RA主要病变细胞产生影响,从而达到缓解和治疗疾病的目的。.