The objective was to evaluate the clinical efficacy and safety of telitacicept in the treatment of pediatric IgA vasculitis nephritis (IgAVN). A retrospective analysis was conducted on clinical data from patients aged 3–18 years with IgAVN treated with telitacicept at the Department of Pediatrics, The First Affiliated Hospital of Henan University of Chinese Medicine, between August 2023 and August 2024. Laboratory parameters were assessed at baseline and at 1, 3, 6, and 12 months posttreatment to determine therapeutic efficacy and safety. A total of 16 patients were enrolled (7 males [43.75

RATIONALE:Lipoprotein glomerulopathy (LPG) is a rare genetic kidney disorder. Here, we report a boy and his mother with LPG. PATIENT CONCERNS:A 6-year-old boy was admitted to our hospital with a history of 6 months of experiencing foamy urine without apparent cause. DIAGNOSES:Urinalysis revealed 3+ protein and 2+ occult blood. A 24-hour urinary protein quantification measured 1110 mg. Other laboratory tests revealed that the level of serum albumin was 43.6 g/L, triglycerides 4.31 mmol/L were elevated, and high-density lipoprotein cholesterol 0.71 mmol/L were reduced, whereas total cholesterol and low-density lipoprotein cholesterol levels were normal. Renal biopsy revealed glomerular capillary loop expansion with lipoprotein thrombi on light microscopy, variable-sized vacuoles within the capillary loops on electron microscopy, positive Oil Red O staining, and positive immunofluorescence staining for ApoE. The mother of the patient had a history of uremia 5 years ago. Genetic testing confirmed a deletion of 9 nucleotides (CAAGCTGCG) in exon 4 of the ApoE gene at positions c.480-488 of the boy and his mother, resulting in a deletion of 3 amino acids (Lys143-Arg145del) in the ApoE amino acid sequence at positions 143-145, which was same variant as ApoE Tokyo/Maebashi. INTERVENTIONS:The boy showed significant improvement after treatment with fenofibrate and telmisartan, with urine protein turning negative after 1 week and blood lipid levels returning to normal after 4 weeks. OUTCOMES:During 1 year follow-up period, the results of urine routine examination and blood lipid profile remained within normal ranges. LESSONS:LPG is a rare and easily misdiagnosed kidney disease with no clinical characteristics. Early diagnosis by kidney biopsy and whole gene test is conducive to early detection and diagnosis, reducing missed diagnosis and misdiagnosis, and improving the long-term prognosis of patients.

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Objective: Investigating the clinical and imaging characteristics of Posterior Reversible Encephalopathy Syndrome (PRES) with renal disease among children and improving pediatrician awareness of PRES. Methods: The clinical presentation, imaging data, treatment, and prognosis of six children diagnosed with kidney disease with PRES at The First Affiliated Hospital of Henan University of Traditional Chinese Medicine between October 2016 and December 2021 were retrospectively analyzed, and relevant literature was reviewed. Results: Of six children (five boys and one girl) aged from 7 to 14 years, three had Henoch-Schönlein purpura nephritis (HSPN), two had nephrotic syndrome (NS), and one had lupus nephritis (LN). All children had a history of hormone and immunosuppressant therapy. Clinical manifestations of the six children all had convulsions and consciousness disorder. Five had moderate to severe hypertension, two had dizziness and headaches, two had nausea and vomiting, and one had visual disturbances. Cranial magnetic resonance imaging (MRI) of all six patients was characterized by reversible white matter abnormalities, primarily symmetrical. After controlling convulsions, decreasing cranial pressure, and actively controlling the primary disease, all children made a full recovery to their premorbid state. Conclusions: The main clinical manifestations of PRES include convulsions, consciousness disorder, headache, and visual disturbances. Cranial MRI is an important adjunctive test for the diagnosis of PRES. Children with renal disease treated with hormones and immunosuppressants commonly suffer from PRES. With early diagnosis and aggressive treatment, there is a good prognosis for children with PRES.

过敏性紫癜(henoch-schonlein purpura,HSP)是儿童时期常见的以IgA介导的全身性微血管变态反应性疾病. 高敏等[1]报道 14809 例HSP患儿发病率男:女=1.28:1,以学龄期儿童为主,冬季发病率最高,8 月份最低.

Objective: To use 16SrRNA and metagenomic sequencing methods to compare the composition of oral microbiota between children with allergic purpura（henoch-sch9nlein purpura, HSP） and healthy children, as well as HSP children treated with Yinqiao Powder, and to explore the possible mechanism of Yinqiao Powder in treatment of of Wind-Heat injured collateral HSP.Methods: A total of 20 children with newly diagnosed HSP were included.The saliva samples were collected from 7■00 to 8■00 in the morning as the pre-treatment group.After Yinqiao Powder treatment for 2 weeks, the saliva was collected again at the same time as the post-treatment group.20 cases were recruited at the same time.Healthy children served as a healthy control group.The Illumina NovaSeq sequencing platform was used to perform high-throughput sequencing of 16SrRNA in each group of saliva microorganisms.Through α-diversity and β-diversity analysis, the differences in the species composition and community structure of each group of flora were compared, and the differential flora was screened out; metagenomics was used for comparison Functional gene differences between children with HSP and healthy children.Results: Compared with the healthy control group, the richness and diversity of the salivary flora in the HSP treatment group decreased, but there was no significant difference（P＞0.05）;compared with the pre-treatment group, the salivary flora in the Yinqiao Powder treatment group Richness and diversity increased significantly（P＜0.05）.Compared with the healthy control group, there was no significant difference in the oral flora structure of the pre-treatment group（P＞0.05）;compared with the pre-treatment group, the oral flora structure of the post-treatment group was significantly different（P＜0.01）.Using the linear discriminant analysis（LDA） effect size（LEfSe） and the MetaStat method, it was shown that at the phylum level, compared with the healthy control group, the Bacteroidota in the pre-treatment group was significantly increased; compared with the pre-treatment group, the post-treatment group Bacteroidota lowered.Compared with the healthy control group, the Actinobacteriota in the pre-treatment group decreased, and compared with the pre-treatment group, the Actinobacteriota in the post-treatment group increased significantly.At the genus level, compared with the healthy control group, the genera of Porphyromonas, Capnocytophaga and Granulicatella in the group before treatment were significantly higher than those in the group after treatment.Before treatment group; compared with the healthy control group, the Veillonella genus（Veilonela） decreased in the pre-treatment group, and was significantly higher in the post-treatment group than in the pre-treatment group.There are obvious functional gene differences between children with HSP and healthy children.The functions of the oral bacterial community in children with HSP are mainly concentrated in: signal transduction, exogenous biodegradation and metabolism, metabolism of three major nutrients, antibiotic resistance, etc.,while healthy children The oral flora maintains the ecological balance with the host, and its main function is reflected in the establishment of environmental adaptive immunity and autoimmunity.Conclusion: The oral microecology of children with Wind-Heat injured type HSP has changed, and the richness and diversity of saliva flora decreased.There are obvious functional gene differences between children with HSP and healthy children.Yinqiao Powder may improve the oral microecology by changing the abundance of Porphyromonas, Veilonela, Capnocytophaga, Granulicatella and related metabolic pathways, and then achieve the therapeutic effect.

Review question / Objective This study aims to evaluate the clinical efficacy and safety of Cimetidine combined with Montelukast in treating children with Henoch-Schonlein Purpura (HSP).Condition being studied Henoch-Schonlein Purpura (HSP) is a common systemic vasculitis in children, that incidence rate is 13.5-18.0/100000.It primarily affects children under 10 years old, with no significant difference between males and females.Current treatment for HSP is mainly supportive and symptomatic, supplemented with glucocorticoid and immunosuppressive treatment when necessary.However, the efficacy of many drugs requires further verification through clinical trials.Recent studies have indicated that combining Cimetidine with Montelukast yields promising results in treating children with HSP.Therefore, this study conducted a meta-analysis to further clarify its efficacy and safety. METHODSParticipant or population Patients who were diagnosed with HSP aged 1-18 years and without limitations in gender and race. I n t e r v e n t i o n C i m e t i d i n e c o m b i n e d w i t h Montelukast.Comparator Nither conventional treatment or conventional treatment combined with Cimetidine or Montelukast.Study designs to be included Randomized Controlled Trial.

Testicular necrosis is a rare and severe complication of immunoglobulin A (IgA) vasculitis (IgAV). Herein, We report a case of a 10-year-old boy who was admitted to the hospital due to skin purpura and intermittent abdominal pain for 10 days and bilateral testicular pain for 2 days. Scrotal ultrasonography indicated right testicle ischemia, right epididymo-orchitis, and bilateral hydrocele of the testis. Scrotal surgical exploration revealed significant swelling and darkening of the right testicle. Conservative treatment led to improvement in his condition, and he was discharged. During 3 months of follow-up, there was no recurrence of skin purpura or pain, and the urine tests were normal. Color ultrasound indicated only partial blood flow signal to the right testicle tissue, which was slightly smaller than the left testicle. This case highlights the need for continuous attention from clinicians to the signs and symptoms of the reproductive system during the diagnosis and treatment of IgAV. Continuous monitoring with ultrasound can aid in early detection, diagnosis, and treatment of reproductive system lesions of IgA vasculitis.

Recurring episodes of fever characterize tumor necrosis factor receptor-associated periodic syndrome (TRAPS) which is autosomal dominant. The primary symptoms of patients with TRAPS include prolonged fever, abdominal pain, muscle pain, and skin rashes. The prevalence of TRAPS appeared higher in Western countries than in Asian countries. Herein, we present the case of a 13-year-old girl who experienced intermittent fever for 8 years, with episodes that occur every 2 years. The patient demonstrated periodic fever, headache, vomiting, rash, and elevated inflammatory marker levels during the disease course. A heterozygous C55Y mutation was identified via a direct DNA sequencing of her genomic DNA. This mutation is located in exon 4 of TNFRSF1A. Genetic studies of her sister and mother revealed that they possessed the C55Y heterozygous mutation without demonstrating any clinical signs, while the father did not. Further, we conducted a thorough assessment of the literature and compiled the information from the eight TRAPS case series.

全球人工智能技术持续发展并迎来巨大变革.ChatGPT已熟知人类所有已知公共知识(包括医学),使用手势、眼球运动及语音进行交互的混合现实(MR)设备Vision Pro也进入普及阶段.在教育智能化的时代基础上,我们有理由推测,基于人工智能及混合现实技术的新型教育模式正逐步成型,且正在挑战传统的教育模式.基于MR与人工智能(AI)的学生与教师、学生与学生、学生与AI、AI与教师沉浸式学习正可直接跨越空间和时间壁垒.虚拟病人模拟接诊、用药、手术全过程的全过程教育医学教育模式可极大缩短医学培训时间,医学教育正逐步走向智能化和高效化.但仍需要注意,医学教育工作者需持续学习以适应新的教学模式.总之,本文阐述了虚拟现实及人工智能对传统医学教育的挑战与变革,并且提出医学教育工作者应直面人工智能的时代潮流,尽快提升专业素质和多学科合作能力,为未来的变革做好充分准备.

目的:系统评价复方丹参注射液辅助治疗肾病综合征的疗效与安全性.方法:检索万方、维普、中国知网、CBM、百度学术、PubMed、The Cochrane Library、EMbase等数据库,时间自建库至2020年5月.依据文献的纳入及排除标准检索所需文献,并进行方法质量评价,运用软件RevMan 5.3进行Meta分析.结果:共纳入临床随机对照实验研究10篇,包括642例患者;血清白蛋白变化程度比较:治疗组优于对照组[WMD=6.57,95％ CI(4.35,8.78),P=0.00001];24小时尿蛋白定量变化程度比较:二者无差别[WMD=-1.52,95％ CI(-3.61,0.57),P=0.15];血肌酐变化程度比较:二者无差别[WMD=-21.65,95％ CI(-46.35,3.05),P=O.09];胆固醇变化程度比较:治疗组优于对照组[WMD=-0.91,95％ CI(-1.38,-0.44),P=0.0001];全血黏度变化程度比较:治疗组优于对照组[WMD=-O.7,95％ CI(-0.79,-0.62),P＜0.00001];有效率比较:治疗组优于对照组[OR=4.21,95％CI(1.62,10.94),P=0.003].结论:复方丹参注射液辅助治疗肾病综合征,可提高血清白蛋白、降低全血黏度及胆固醇,且安全性较高.

IgA肾病(IgAN)是最常见的肾小球疾病,是导致慢性肾病的主要原因之一,"四重打击"学说为目前公认的发病机制.IgAN与肠黏膜之间存在密切关系,多种肾脏疾病均与肠道黏膜的过度免疫反应密切相关,并提出了肠-肾轴概念.黏膜微生物区系对IgAN免疫调节起重要作用.针对IgAN的治疗亦从传统的激素、免疫抑制剂等观念向分子靶向治疗发展.本文重点阐述IgAN分子机制最新研究进展、肠-肾轴机制、黏膜微生物区系以及分子靶向治疗.

致密物沉积病(dense deposit disease,DDD)是一种较罕见的肾小球疾病,既往被命名为膜增生性肾小球肾炎( membranous proliferative glomerulone-phritis,MPGN)Ⅱ型,1995年WHO肾脏病理分类将此病从MPGN中独立出来,后定义其属于C3 肾小球病(C3 glomerulopathy,C3G)范畴[1],以电镜下观察到肾小球基膜( glomerular basement membrane, GBM)内异常电子致密物沉积为诊断依据.DDD临床主要表现为持续低补体C3血症、蛋白尿和血尿.DDD临床较为少见,其患病率为( 2 ～3 )人/百万人[2] ,好发于儿童和青少年,预后较差,约半数患者会在10 a内进展为终末期肾病( end stage renal dis-ease,ESRD).河南中医药大学第一附属医院儿科医学部曾收治1例DDD患儿,给予强的松口服联合环磷酰胺( cyclophosphamide,CTX)冲击治疗及中药辨证治疗,获得临床缓解,后续连续随访9 a,现患儿病情处于完全缓解状态.由于DDD临床罕见且预后较差,现结合中外DDD 治疗相关的文献报道如下,以期为该病的诊疗提供参考.

周正教授认为儿童多发性抽动症的主要病机为痰热内扰.黄连温胆汤具有燥湿化痰之功,兼清内热之效,可异病同治运用于痰热内扰所致的诸多病证,以本方加减治疗儿童多发性抽动症亦多有良效,并附典型案例.

目的 探讨强的松联合左旋甲状腺素治疗伴甲状腺功能异常的原发性肾病综合征的临床疗效和安全性.方法 回顾性分析近6年我院原发性肾病综合征患儿的甲状腺水平、治疗、疗效及副作用.结果 46.59％的患者出现甲状腺功能异常,经强的松联合左旋甲状腺素治疗,蛋白尿转阴时间缩短,白蛋白升高,优于单用激素的患者.结论 激素联合左旋甲状腺素治疗伴甲状腺功能异常的原发性肾病综合征患儿,能降低尿蛋白,提高血浆白蛋白,优于单用激素.

目的 探讨儿童肾脏疾病合并可逆性后部脑病综合征(PRES)的临床特点.方法 回顾分析2015-2018年确诊的6例肾脏疾病合并PRES患儿的临床资料,并复习相关文献.结果 6例患儿中男4例、女2例,年龄3～14岁.原发病分别为肾病综合征2例、紫癜性肾炎2例、过敏性紫癜1例、狼疮性肾炎1例,均有激素及免疫抑制剂应用史.临床表现均为急性起病,抽搐、意识丧失、血压升高,还有精神行为异常、呕吐、头痛、视物障碍表现.影像学表现均以可逆性白质异常病变为特征,多位于半球后部白质.5例患儿恢复至发病前状态,1例死亡.结论 肾脏疾病应用激素及免疫抑制剂的患儿易合并PRES,一般预后良好,但并非完全可逆.

目的 观察益气化瘀清热方及其拆方对嘌呤霉素氨基核苷(Puromycin aminonucleoside,PAN)损伤体外培养的小鼠足细胞表达Podocalyxin及Podocin的影响.探讨不同类中药治疗肾病的作用强度.方法 应用PAN(50 μg/mL)刺激体外培养的小鼠足细胞,形成PAN足细胞损伤模型,造模成功后的足细胞随机分为益气组、化瘀组、清热组、复方组、对照组、模型组、空白组,采用实时定量逆转录聚合酶链反应(RT-PCR)和蛋白质印迹法(Western Blot)检测各组血清对小鼠足细胞表达Podocalyxin、Podocin的影响.结果 益气化瘀清热方及其拆方均能影响造模足细胞Podocalyxin及Podocin的表达,但不同药物作用于造模足细胞的时间不同,其对Podocalyxin及Podocin表达的作用强度不同.结论 益气化瘀清热方及其拆方可能通过提高造模足细胞Podocalyxin及Podocin的表达而发挥治疗作用.

目的 评价中医阶梯治疗方案对儿童紫癜肾(血尿和蛋白尿型)的临床疗效及安全性.方法 收集100例符合标准的紫癜性肾炎住院患儿,采用中国中医科学院中央临床随机系统按2:1的比例随机分为中医重型组28例,中医轻型组39例,西医重型组13例,西医轻型组20例.中医治疗组给予雷公藤多苷片联合清热止血方加减治疗;西医对照组给予强的松片联合贝那普利、双嘧达莫及中药安慰剂治疗.治疗12周后,观察各组第2、4、8、12周临床疗效及用药安全性.结果 蛋白尿疗效:第2、4、8周末中医重型组优于西医重型组,中医轻型组仅第8周末优于西医组.尿红细胞疗效:中医轻型组在第2、8、12周末的疗效优于西医轻型组,中医重型组仅在第8周末疗效优于西医重型组.安全性:中医阶梯治疗方案不良反应发生率低.结论 中医阶梯治疗方案对血尿和蛋白尿型紫癜性肾炎患儿有效,起效时间快,对尿蛋白的疗效更优,且雷公藤多苷片在方案治疗剂量下安全,对白细胞、肝功能的影响可控.

目的 观察清热止血方联合雷公藤多苷治疗儿童紫癜性肾炎临床疗效及对血清中IgA1异常糖基化的影响.方法 选取河南中医药大学第一附属医院儿科肾脏病区2018年03月至2018年12月收治的HSPN患儿45例作为研究对象,采用随机分组,按照2∶1分为中医组30例,西医组15例.中医组给予清热止血方联合雷公藤多苷,西医组给予贝那普利联合双嘧达莫,疗程12周,随访观察临床疗效及检测血清中的IgA1异常糖基化水平.结果 经治疗患儿尿蛋白及尿红细胞均下降,在治疗4周时中医组对尿蛋白及尿红细胞的治疗总有效率优于西医组,有统计学差异(P＜0.05),对IgA1异常糖基化的下调优于西医组,有统计学差异(P＜0.05),在治疗8周及12周,两组疗效无统计学差异(P＞0.05),对IgA1异常糖基化调控无统计学差异(P＞0.05).结论 清热止血方联合雷公藤多苷能降低儿童紫癜性肾炎血尿及蛋白尿的水平,起效时间早于西医组,其作用机制可能与降低IgA1异常糖基化水平相关.

目的:观察血尿2号方联合雷公藤多苷片(TWP)治疗儿童紫癜性肾炎(HSPN)单纯血尿型的临床疗效.方法:根据HSPN的临床分型标准对符合条件的HSPN患儿124例,分为治疗组(62例)和对照组(62例),对照组采用TWP治疗,治疗组采用中药加TWP治疗,两组疗程均为3个月.结果:治疗4周:治疗组总有效率为74.2％,对照组为59.7％,治疗组与对照组差异无统计学意义(P>0.05);治疗8周:治疗组总有效率为87.1％,对照组为72.6％,治疗组效果优于对照组(P<0.05);治疗12周:治疗组总有效率为91.9％,对照组为79.0％,治疗组效果优于对照组(P<0.05).结论:采用血尿2号方联合TWP治疗儿童HSPN血尿疗效较好.