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中华实用儿科临床杂志
中华实用儿科临床杂志

郭学鹏

半月刊

2095-428X

syqk@xxmu.edu.cn

0373-3029144，3831456

453003

河南省新乡市新乡医学院

中华实用儿科临床杂志/Journal Journal of Applied Clinical PediatricsCSCD北大核心CSTPCD
查看更多>>本刊创刊20年来，严格遵守国家的出版法规及条例，执行出版编辑规范，始终坚持突出实用为主，理论联系实践，注重基础与临床相结合，报道儿科领域新的科研成果、新理论、新技术、新进展，为促进我国儿科医学领域的学术交流服务为办刊宗旨。2005年本刊的总被引频次1417，在18种妇、儿科医学类期刊中列第4位，在1606种中国科技论文统计源期刊中列第79位；影响因子0.659，在18种妇、儿科医学类期刊中列第6位，在1606种中国科技论文统计源期刊中列第221位；即年指标0.155,在8种儿科学类期刊中仅次于《中华儿科杂志》，位居第2位，其他学术期刊指标均较去年明显提高。目前已连续四次进入北京大学图书馆主编的《中文核心期刊要目总览》、列入中国科技论文统计源期刊（中国科技核心期刊），2003年被俄罗斯《文摘杂志》收录,2005年又被美国《化学文摘》收录。2004年被评为河南省优秀期刊。我刊已被中国生物医学文献数据库（CBMdisc）、Quick全文资料管理系统（FTME）、中文科技期刊数据库、中国学术期刊（光盘版）、万方数据库和美国《化学文摘》、俄罗斯《文摘杂志》、《中国医学文摘：儿科学分册》等多种权威性数据库与文摘期刊作为固定收录对象。
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    训练免疫及其对儿童健康的影响

    韩鹏申昆玲李建华
    81-84页
    查看更多>>摘要：训练免疫是近10余年新出现的免疫学术语，指固有免疫系统对再次刺激所产生的记忆免疫。训练免疫能够非特异性增强机体的免疫防御功能，也参与过敏性炎症和自身免疫性疾病的发生。目前，越来越多的研究开始关注训练免疫在疾病预防、治疗和发生发展等方面的作用。现就训练免疫的定义、机制、诱导剂及其对儿童健康的影响进行阐述，以提高儿科医师对训练免疫的认识。 Trained immunity has been a novel term in immunology for over a decade， referring to the memory immunity produced by the innate immune system upon re-stimulation。Non-specific training of the immune system enhances the immune defense function and is also involved in allergic inflammation and autoimmune diseases。An increasing number of researchers are focusing on the role of immune training in the prevention， treatment， onset and progression of diseases。This review explains the definition， mechanism， and inducers of immune training and its impact on children′s health and disease， in order to enhance pediatricians′ comprehension of trained immunity。

    儿童训练免疫非特异记忆免疫

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    国内当前百日咳治疗的抗菌药物选择之我见

    姚开虎孟庆红史伟袁林...
    85-88页
    查看更多>>摘要：长期以来，大环内酯类抗菌药物一直是百日咳抗菌治疗的首选药物。但是，近十年来，国内临床分离的百日咳鲍特菌常对大环内酯类抗菌药物耐药，与推荐首选大环内酯类治疗已呈明显矛盾。因此作者建议国内当前百日咳抗菌治疗首选甲氧苄啶-磺胺甲唑(TMP-SMZ)，根据年龄和体重确定给药剂量，并坚持用药14 d；如不能使用TMP-SMZ，可尝试足量、足疗程选用β-内酰胺类药物，其效果需谨慎评估；还应积极评估喹诺酮类、四环素类等其他抗菌药物清除体内百日咳鲍特菌的作用，以治疗成人百日咳或应对将来新出现的耐药菌。 For a long time， macrolides have been the first choice for the antibacterial treatment for pertussis。However， in the past decade， resistance to macrolide antimicrobials has been common in clinically isolated Bordetella pertussis in China， which is in contradiction with the recommended macrolide treatment。Therefore， Trimethoprim-Sulfamethoxazole (TMP-SMZ) is suggested as the first choice for antibacterial treatment for pertussis in China， with a dosage determined according to age and body weight， lasting 14 days。If TMP-SMZ cannot be used， full-dose and full-course β-lactam antimicrobials may be used， of which the effects should be assessed carefully。The impact of other antibacterial drugs， such as quinolones and tetracyclines， on the elimination of Bordetella pertussis should also be evaluated as soon as possible to treat adult pertussis and potential cases caused by drug-resistant bacteria in future。

    百日咳百日咳鲍特菌耐药性抗菌药物治疗

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    ptxP1与 ptxP3型红霉素耐药百日咳鲍特菌感染患儿临床表现的比较研究

    郭孟杨汪丙松袁林李振...
    89-93页
    查看更多>>摘要：目的 明确临床百日咳鲍特菌分离株红霉素耐药状况及其ptxP1与ptxP3型构成，比较2种基因型菌株导致的百日咳患儿在临床表现方面的差异。 方法 横断面研究。收集2019年1月至2022年12月北京儿童医院及芜湖市第一人民医院采用细菌分离培养确诊的百日咳病例，采用玻片血清凝集试验鉴定疑似百日咳鲍特菌菌落，使用E试验和K－B纸片法检测分离菌株对红霉素的敏感性。通过聚合酶链式反应扩增ptxP基因并测序，确定其基因型。应用t检验、Mann-Whitney U检验、χ2检验及Fisher′s精确概率法进行统计学分析。 结果 共鉴定出百日咳鲍特菌192株，其中红霉素耐药188株(97。9%)。耐药株中，ptxP1型占30。3%(57/188)，ptxP3型占69。7%(131/188)。在<1岁患儿中，ptxP3型感染患儿发作性咳嗽的发生率较ptxP1型更高(57。1%比29。4%，P<0。05)，ptxP3型较ptxP1型感染患儿出现呼吸暂停或窒息(23。8%比17。6%)、咳嗽后呕吐(44。4%比32。4%)、咳嗽末吸气性啸音(72。0%比50。0%)和并发肺炎/支气管炎(85。7%比73。5%)的概率更高，但差异均无统计学意义(均P>0。05)。在≥1岁患儿中，ptxP1型感染患儿的白细胞值高于ptxP3型感染者[13。5(9。9，24。5)×109/L比10。3(7。0，16。4)×109/L，P<0。05]，ptxP1型较ptxP3型感染患儿出现合并其他病原体感染的概率更高(17。4%比4。4%，P>0。05)。 结论 ptxP3型红霉素耐药百日咳鲍特菌已成为百日咳主要的致病型别；ptxP3型红霉素耐药株比ptxP1型红霉素耐药株引起的婴儿百日咳表现更为典型明显，出现重症的可能性更高。 Objective To determine the erythromycin resistance of Bordetella pertussis isolates and their ptxP1 and ptxP3 phenotypic composition and compare clinical manifestations of children with pertussis caused by the two types of strains。 Methods This was a cross-sectional study， the pertussis cases diagnosed using bacterial culture from January 2019 to December 2022 in Beijing Children′s Hospital and the First People′s Hospital of Wuhu were collected。Any suspected Bordetella pertussis colonies were identified by the slide agglutination test。The susceptibility of isolates to erythromycin was detected by the E-test and K-B test。The ptxP gene was amplified by polymerase chain reaction and sequenced to determine its genotype。t-test， Mann-Whitney U-test， Chi-square test and Fisher′s exact test were use to statistical analysis。 Results A total of 192 strains of Bordetella pertussis were identified， including 188 (97。9%) erythromycin-resistant strains。Among the 188 strains， 30。3%(57/188) belonged to the ptxP1 genotype and 69。7%(131/188) belonged to the ptxP3 genotype。In children aged below 1 year old， the incidence of paroxysmal cough caused by infection with the ptxP3 strain was higher than that with the ptxP1 strain (57。1% vs。29。4%， P<0。05)， and children infected with theptxP3 strain were more likely to develop apnea or asphyxia (23。8% vs。17。6%)， post-tussive vomiting (44。4% vs。32。4%)， whooping cough (72。0% vs。50。0%) and pneumonia or bronchitis (85。7% vs。73。5%) compared to those infected with the ptxP1 strain， but the differences were not statistically significant(all P>0。05)。 In children aged 1 year old and above， the white blood cell count of children infected with theptxP1 strain was higher than that of infections with the ptxP3 strain [13。5(9。9， 24。5)×109/L， 10。3 (7。0， 16。4)×109/L， P<0。05]， and children infected with theptxP1 strain were more likely to contract other pathogen infections than those infected with the ptxP3 strain (17。4% vs。4。4%， P>0。05)。 Conclusions ptxP3 erythromycin-resistant Bordetella pertussis has become the main pathogen of pertussis。Infants with pertussis caused by the ptxP3 erythromycin-resistant strain show more significant manifestations and a higher possibility of severe symptoms than those infected with the ptxP1 erythromycin-resistant strain。

    百日咳临床表现红霉素耐药性基因型

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    毛细管电泳用于高苯丙氨酸血症基因诊断的临床应用研究

    谭建新孙云邵彬彬王彦云...
    94-97页
    查看更多>>摘要：目的 评估一种基于毛细管电泳的高苯丙氨酸血症的快速基因诊断方法的临床价值。 方法 前瞻性单中心研究。选取2021年2月至2023年2月于南京市妇幼保健院经液相串联质谱新生儿筛查检测疑似高苯丙氨酸血症的40例患儿。其中男22例，女18例；平均诊断年龄21。93 d。采用毛细管电泳技术对40例疑似高苯丙氨酸血症患儿苯丙氨酸羟化酶(PAH)基因中的85个变异位点进行检测，对未明确诊断患儿的PAH基因进一步采用Sanger测序进行检测。评估毛细管电泳技术的检出率、敏感度和特异度。 结果 在40例疑似高苯丙氨酸血症患儿中，毛细管电泳技术共检出71个PAH变异，32例患儿明确基因诊断，5例患儿仅发现1个致病变异，3例患儿未检出变异。毛细管电泳技术的检出率为80。00%，敏感度为88。75%，特异度为100%。 结论 毛细管电泳技术能够快速、高效、准确地检出PAH基因变异，且成本较低，是一种极具临床应用前景的高苯丙氨酸血症基因检测方法。 Objective To evaluate the clinical value of a capillary electrophoresis-based method for gene diagnosis of hyperphenylalaninemia。 Methods In this single-center prospective study， 40 newborns with suspected hyperphenylalaninemia detected by neonatal liquid chromatography-tandem mass spectrometry screening at Nanjing Maternity and Child Health Care Hospital from February 2021 to February 2023 were included， with 22 males， 18 females and a mean age at diagnosis of 21。93 days。Capillary electrophoresis was used to detect 85 variants of the phenylalanine hydroxylase (PAH) gene in 40 newborns with suspected hyperphenylalaninemia。The PAH gene of undiagnosed patients was further analyzed by Sanger sequencing。The detection rate， sensitivity and specificity of capillary electrophoresis were calculated。 Results Among these 40 newborns with suspected hyperphenylalaninemia， 71 PAH variants were detected by capillary electrophoresis， 32 patients were clearly diagnosed， only 1 pathogenic variant was found in 5 patients， and no pathogenic variant was found in the last 3 patients。Therefore， the detection rate， sensitivity and specificity of capillary electrophoresis for analysis of the PAH gene were 80。00%， 88。75% and 100%， respectively。 Conclusions The capillary electrophoresis-based method can rapidly， efficiently and accurately detect PAH gene variants at lower cost and is a promising gene detection method for hyperphenylalaninemia in clinical practice。

    高苯丙氨酸血症苯丙氨酸羟化酶毛细管电泳基因变异

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    携带 ABCA3突变的肺表面活性物质代谢障碍患儿的诱导多能干细胞系构建

    田智琛谢欣池婧涵陈佳...
    98-103页
    查看更多>>摘要：目的 利用携带腺苷三磷酸结合盒转运子A3(ABCA3)复合杂合突变的新生儿呼吸窘迫综合征(NRDS)患儿的外周血单核细胞(PBMCs)，建立符合研究需求的诱导多能干细胞(iPSCs)细胞系。 方法 细胞实验研究。采集患儿的外周静脉血并分离出PBMCs，体外培养，用携带重编程因子的非整合性仙台病毒载体转染PBMCs，对所产生的iPSCs细胞系进行染色体核型分析。采用免疫荧光技术、流式细胞术检测干细胞多能性标志物、验证其分化潜能，采用Sanger测序对基因突变进行分析，另外还行短串联重复序列(STR)位点分析，聚合酶链式反应(PCR)和琼脂糖凝胶电泳法检测病毒残留。 结果 对建立的iPSCs细胞系行核型分析显示为正常的二倍体46，XY核型，免疫荧光技术显示干细胞多能性标志物OCT4、SSEA4、Nanog及Sox2染色呈阳性。流式细胞术检测干细胞多能性标志物，显示TRA-1-60、SSEA-4和OCT4的表达。诱导向三胚层分化后用免疫荧光技术对外胚层(PAX-6)、中胚层(Brachyury)和内胚层(甲胎蛋白)标志物进行免疫荧光染色，结果显示均为阳性。Sanger测序显示c。3997_3998del及c。3137C>T复合杂合突变。STR位点分析结果显示其来源于患儿PBMCs，PCR和琼脂糖凝胶电泳法未检测到仙台病毒残留。 结论 本研究用ABCA3复合杂合突变患儿的PBMCs建立了iPSCs细胞系，为ABCA3基因突变所致的NRDS发病机制的研究、治疗药物筛选及细胞治疗奠定了基础。 Objective Induced pluripotent stem cells (iPSCs) cell lines were established using peripheral blood mononuclear cells (PBMCs) from a patient suffering from neonatal respiratory distress syndrome (NRDS) who carried Adenosine triphosphate-binding cassette transporter A3 (ABCA3) compound heterozygous mutations。 Methods Cell experimental research。Peripheral venous blood was collected and PBMCs were isolated and cultured in vitro。 PBMCs were transfected with non-integrated Sendai vector carrying reprogramming factors。The chromosome karyotypes of the established iPSCs were analyzed。Immunofluorescence and flow cytometry were used to detect pluripotency markers of stem cells and verify their differentiation potential。Sanger sequencing was performed to analyze gene mutations。In addition， short tandem repeat (STR) analysis was performed， polymerase chain reaction(PCR) and agarose gel electrophoresis were used to detect virus residual。 Results Karyotype analysis of established iPSCs cell lines showed normal diploid 46， XY karyotype。Immunofluorescence showed positive staining of stem cell pluripotency markers OCT4， SSEA4， Nanog and Sox2。Flow cytometry was used to detected stem cell pluripotency markers and showed expression of TRA-1-60， SSEA-4 and OCT4。After differentiation into all three germ layers， immunofluorescence was performed to detect ectoderm (Pax-6)， mesoderm (Brachyury) and endoderm alpha-fetoprotein markers， and the results showed positive staining， which confirmed that the iPSCs had the potential to differentiate。Sanger sequencing showed c。 3997_3998del and c。 3137C>T compound heterozygous mutations。STR analysis showed they originate from PBMCs， and no Sendai virus residual was detected by PCR and agarose gel electrophoresis。 Conclusions In this study， PBMCs from patient carrying ABCA3 compound heterozygous mutations was used to establish iPSCs cell lines。The research lays a foundation for the study of pathogenesis， therapeutic drug screening and cell therapy of NRDS caused by ABCA3 gene mutations。

    婴儿,新生呼吸窘迫综合征腺苷三磷酸结合盒转运子A3诱导多能干细胞外周血单核细胞

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    儿童嗜酸细胞性食管炎临床特点分析

    李婧周锦郭景房永利...
    104-108页
    查看更多>>摘要：目的 总结儿童嗜酸细胞性食管炎(EoE)的临床特点。 方法 回顾性分析2017年1月1日至2022年12月31日首都医科大学附属北京儿童医院消化科住院治疗的EoE患儿临床资料。 结果 共纳入EoE患儿18例，其中男13例，女5例，年龄11。96(4。96，12。81)岁。学龄前期患儿呕吐较为常见(4/5例)，学龄期和青春期患儿以腹痛为主(11/13例)。患儿外周血白细胞升高者占22。22%(4/18)，嗜酸性粒细胞升高者占33。33%(6/18)。55。56%(10/18)的患儿或一级亲属存在过敏史，68。75%(11/16)总IgE水平升高，66。67%(12/18)食物变应原阳性，牛奶、鸡蛋和小麦为常见的变应原。内镜下可见食管黏膜充血红斑、粗糙、糜烂、线性溃疡、环状改变、犁沟样或皱纸样改变、颗粒样改变、息肉样改变或贲门口松弛。27。78%(5/18)的患儿内镜下表现正常。病理组织学多表现为食管慢性炎症及嗜酸性粒细胞计数升高。患儿失访3例，其余15例随访时间6个月至2年。患儿均采用饮食回避治疗，9例应用糖皮质激素治疗后较快缓解，其中1例患儿停药后复发，再次激素治疗有效，另有2例消化道症状出现反复或停药后外周血嗜酸性粒细胞升高，回避饮食治疗可缓解。 结论 EoE多见于年长儿，男童多见，学龄前期以呕吐为主要症状，学龄期和青春期多表现为腹痛。部分患儿外周血白细胞和嗜酸性粒细胞升高，多数患儿食物变应原阳性；消化道内镜及病理组织学检查为诊断EoE的重要手段。饮食回避可起到一定治疗作用。糖皮质激素在EoE的治疗中发挥重要作用，但少数患儿存在激素依赖。 Objective To summarize the clinical characteristics of eosinophilic esophagitis (EoE) in children。 Methods Clinical data of children with EoE who were hospitalized in the Department of Gastroenterology， Beijing Children′s Hospital， Capital Medical University from January 1， 2017， to December 31， 2022， were retrospectively analyzed。 Results A total of 18 children with EoE were included in the study， including 13 males and 5 females， with the age of 11。96 (4。96， 12。81) years。Vomiting was more common in preschool children (4/5)， while abdominal pain was the main symptom in school-age and adolescent children (11/13)。 There were 22。22% (4/18) of the children with EoE had an increased white blood cell count， and 33。33%(6/18) had an increased eosinophil count。Allergic history in the first-degree relatives was detected in 55。56%(10/18) of the children with EoE。Total immunoglobulin E (IgE) level was elevated in 68。75% (11/16) of the children。Food-specific IgE was positive in 66。67% (12/18) of the children with EoE。Milk， eggs， and wheat were the most common allergens。Esophageal mucosal hyperemia and erythema， rough， erosion， linear ulcers， annular changes， furrow or wrinkled paper changes， granular changes， polypoid or relaxation of the cardia were seen under endoscopy， whereas 27。78% (5/18) of the children showed normal esophageal mucosa。The histopathology showed chronic inflammation of the esophagus and increased eosinophil count。Three patients were lost of follow-up， and the remaining 15 were followed up for 6-24 months。All children with EoE were treated with the elimination diet。Nine children treated with glucocorticoids experienced clinical remission in a short period of time， involving 1 case with recurrence after withdrawal and being effectively treated by hormone therapy， and 2 cases of repeated digestive system symptoms or increased eosinophil count after withdrawal and being effectively relieved by the elimination diet。 Conclusions EoE is more common in elderly children and boys。Vomiting is the main symptom in pre-school aged children， whereas abdominal pain is the main symptom in school-aged children and adolescents。Increased peripheral white blood cell count and eosinophil count can be detected in some cases， and most of them are positive for food allergen tests。Gastrointestinal endoscopy and histopathology are important in the diagnosis of EoE。Elimination diet may be effective in some patients。Glucocorticoids are of great significance in the treatment of EoE， but a few children are steroid-dependent。

    儿童嗜酸细胞性食管炎临床特点

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    单中心19例复发性颅外恶性生殖细胞肿瘤挽救性治疗经验及回顾性分析

    韩亚丽汤静燕潘慈张安安...
    109-113页
    查看更多>>摘要：目的 分析复发性颅外恶性生殖细胞肿瘤(MGCTs)患儿的生存率及预后因素，探索复发性颅外MGCTs患儿可行的挽救性治疗手段。 方法 回顾性研究。研究对象为2010年1月至2020年1月上海儿童医学中心收治的复发性颅外MGCTs患儿，治疗方案包括手术、化疗和放疗。采用Kaplan－Meier生存分析法及Cox风险回归对患儿进行生存率及预后因素分析。 结果 共收治172例颅外MGCTs患儿，其中21例复发，复发率为12。2%，中位复发时间为治疗结束后11个月，2例复发患儿因不符合入组标准被剔除，最终19例进入统计。19例患儿中男10例，女9例；复发年龄26个月(8～170个月)；随访时间57个月(13～122个月)。复发患儿中16例接受挽救性化疗，14例接受手术完整切除，4例患儿接受放疗。复发患儿4年总体生存(4yr-OS)率为(82。5±9。2)%(19例)，其中初诊时仅手术未行辅助化疗的患儿，复发后再治疗的4yr-OS率为(92。3±7。4)% (13例)；初诊时接受化疗的患儿，复发再治疗后4yr-OS率为(60。0±21。9)% (6例)，二者比较差异有统计学意义(P＝0。002)。单因素及Cox多因素回归分析均提示，化疗3个疗程后甲胎蛋白水平未恢复正常者明显影响最终生存率。 结论 颅外MGCTs患儿复发后通过完整手术切除、化疗、放疗等综合治疗后仍有较高的生存率，初治时仅手术未行辅助化疗者，复发再治疗后的生存率明显优于初治时已接受化疗的患儿。 Objective To study the survival and prognostic factors for the recurrent extracranial malignant germ cell tumors (MGCTs) in children， and to explore feasible salvage treatment。 Methods A retrospective study。Pediatric patients with recurrent extracranial MGCTs diagnosed in Shanghai Children′s Medical Center between January 2010 and January 2020 were retrospectively recruited。Comprehensive treatment regimens included surgery， chemotherapy and radiation。Kaplan-Meier survival analysis and Cox regression model were employed to analyze the survival and prognostic factors for children with recurrent extracranial MGCTs。 Results A total of 172 children with extracranial MGCTs were treated， including 21 (12。2%) recurrent cases。The median time of MGCT recurrence after the end of the first treatment was 11 months。Finally， 19 patients were recruited after excluding 2 non-eligible cases， including 10 boys and 9 girls with the age at recurrence of 26 (8-170) months。The follow-up time was 57 (13-122) months。Salvage chemotherapy， complete resection and radiotherapy were performed in 16， 14 and 4 patients， respectively。The 4-year overall survival (4yr-OS) rate was (82。5±9。2)%(19 cases)。 The 4yr-OS rate was significantly higher in patients managed with surgery but without adjuvant chemotherapy at the initial treatment (13 cases) than those managed with chemotherapy at the initial treatment (6 cases)[(92。3±7。4)% vs。(60。0%±21。9)%， P=0。002]。 Univariant and Cox multivariant regression analyses showed that failure to achieve the normal range of alpha fetoprotein after 3 cycles of chemotherapy significantly influenced the survival of recurrent extracranial MGCTs。 Conclusions For patients with recurrent extracranial MGCTs， comprehensive treatment approaches like complete surgical resection， chemotherapy， and radiotherapy offer a favorable survival rate。Specifically， recurrent and re-treated patients who initially received surgery alone without adjuvant chemotherapy have a higher survival rate compared to those who received chemotherapy during the initial treatment。

    儿童颅外恶性生殖细胞肿瘤,复发性

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    利妥昔单抗治疗儿童频复发/激素依赖型肾病综合征的疗效及影响因素

    窦文杰应道静曾慧勤智元昭...
    114-117页
    查看更多>>摘要：目的 探讨利妥昔单抗(RTX)治疗儿童频复发/激素依赖型肾病综合征(FRNS/SDNS)的疗效和安全性，以及影响疗效的因素。 方法 回顾性病例系列研究。收集2019年9月至2022年3月在郑州大学第一附属医院接受B细胞指导下RTX治疗(单次剂量375 mg/m2，最大500 mg，当外周血CD19＋B细胞≥0。01时追加1剂)的FRNS/SDNS患儿的临床资料，比较RTX治疗前后复发次数及激素累积剂量；Kaplan-Meier方法分析RTX治疗后无复发生存率和无FRNS/SDNS生存率。Cox比例风险回归模型分析复发的影响因素。 结果 纳入47例患儿，男35例，女12例；首次应用RTX年龄10。2(6。9，13。0)岁；既往使用1种免疫抑制剂33例，2种及以上14例；RTX治疗剂次3。0(2。0，3。0)次。RTX治疗后复发次数[0(0，0。55)次/年比1。62(1。09，2。40)次/年]和激素累积剂量[0。12(0。05，0。21) mg/(kg·d)比0。40(0。20，0。56) mg/(kg·d)]均较既往免疫抑制剂治疗时明显下降，差异均有统计学意义(Z＝－5。56、－5。54，均P<0。001)。RTX治疗后6、12、18、24个月无复发生存率分别为80。9%、72。3%、68。1%、68。1%，无FRNS/SDNS生存率分别为93。6%、89。4%、89。4%、89。4%。单因素Cox回归分析显示，既往免疫抑制剂治疗时的复发次数多是RTX治疗后复发的危险因素(P<0。05)。14例复发患儿中，6例发生在CD19＋B细胞<0。01时，RTX治疗后的复发次数显著高于CD19＋B淋巴细胞≥0。01时，差异有统计学意义(Z＝－2。84，P＝0。005)。RTX治疗及随访期间无严重不良反应发生。 结论 B细胞指导下RTX治疗儿童FRNS/SDNS有效且安全，既往免疫抑制剂治疗时的复发次数多是RTX治疗后复发的危险因素，B细胞耗竭状态时复发预示RTX治疗结局不良。 Objective To investigate the efficacy and safety of Rituximab (RTX) in the treatment of children with frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS) and to analyze the factors influencing the efficacy。 Methods Case series study。The clinical data of children with FRNS/SDNS who received B-cell-guided RTX (single dose: 375 mg/m2， maximum dose: 500 mg， one additional dose when peripheral blood CD19+ B lymphocytes ≥0。01) in the First Affiliated Hospital of Zhengzhou University from September 2019 to March 2022 were retrospectively collected。The frequency of relapse and cumulative dose of glucocorticoids before and after RTX treatment were compared。The Kaplan-Meier method was used to analyze relapse-free survival rate and FRNS/SDNS-free survival rate after RTX treatment。The influencing factors of relapse were analyzed using the Cox proportional hazards regression model。 Results Totally 47 children were enrolled， including 35 males and 12 females the age of first application of RTX was 10。2 (6。9， 13。0) years 33 children had used one type of immunosuppressant before， and 14 children had used two or more types of immunosuppressant before the dose of RTX treatment was 3。0 (2。0， 3。0)。 The frequency of relapse[0(0， 0。55) times/year vs。1。62 (1。09， 2。40) times/year] and cumulative dose of glucocorticoids[0。12 (0。05， 0。21) mg/(kg·d) vs。0。40 (0。20， 0。56) mg/(kg·d)] after RTX treatment significantly decreased compared with previous immunosuppressive treatment (Z=-5。56， -5。54， all P<0。001)。 The relapse-free survival rates at 6， 12， 18 and 24 months after treatment were 80。9%， 72。3%， 68。1% and 68。1%， respectively， and the FRNS/SDNS-free survival rates were 93。6%， 89。4%， 89。4% and 89。4%， respectively。Univariate Cox regression analysis showed that the high frequency of relapse during previous immunosuppressive therapy was a risk factor for relapse after RTX treatment (P<0。05)。 Of the 14 children who relapsed， 6 occurred in children whose CD19+ B lymphocytes<0。01， and the frequency of relapse after RTX treatment was significantly higher than those whose CD19+ B lymphocytes≥0。01 (Z=-2。84， P=0。005)。 No severe adverse reactions occurred during RTX treatment and follow-up。 Conclusions The B-cell-guided RTX is effective and safe in the treatment of FRNS/SDNS in children。The high frequency of relapse during previous immunosuppressive therapy is a risk factor for relapse after RTX treatment， and relapse in the state of B lymphocyte depletion predicts poor outcomes of RTX treatment。

    利妥昔单抗肾病综合征儿童影响因素

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    胎儿胸腔积液95例妊娠结局的影响因素分析

    张慧芳董越郭晓珮姬若楠...
    118-122页
    查看更多>>摘要：目的 通过对95例胸腔积液胎儿及母体的多个因素进行分析，了解其与妊娠结局的相关性。 方法 病例系列分析。对2016年1月至2022年10月郑州大学第三附属医院收治的95例胎儿胸腔积液(FHT)患者进行回顾性分析。根据妊娠结局，分为引产组(13例)和分娩组(82例)；对引产组患者的一般资料及胎儿遗传学检查进行分析，了解其发病特点及遗传学规律；分娩组患者根据新生儿的Apgar评分分为正常组、窒息组，对2组的孕妇年龄、首诊孕周、孕妇有无并发症或合并症、胎儿胸水的位置、胎儿胸腔积液的严重程度、孕期胎儿胸腔积液的转归、有无胎儿腹水、有无胎儿水肿、胎儿有无其他结构异常、羊水量、有无宫内治疗、分娩孕周、分娩方式、新生儿性别、新生儿体重15项一般资料采用χ2检验进行差异分析；以新生儿Apgar评分为因变量，将上述单因素分析中具有统计学意义的变量纳入回归分析，建立多因素二元Logistic回归方程，探究影响妊娠结局的因素。 结果 正常组和窒息组间孕期胎儿胸腔积液的转归(消退/稳定/进展)(16/22/13例比2/18/11例，χ2＝6。994，P＝0。030)、胸腔积液严重程度(非重度/重度)(27/24例比9/22例，χ2＝4。475，P＝0。034)、有无胎儿水肿(14/37例比23/8例，χ2＝17。012，P＝0。001)、羊水量(多/不多)(21/30例比24/7例，χ2＝10。228，P＝0。001)、有无宫内治疗(19/32例比2/29例，χ2＝9。603，P＝0。002)、分娩孕周[(38。15±2。05)周比(34。83±3。14)周，t＝5。748，P＝0。001]、新生儿出生体重[(3 325。00±637。41) g比(2 714。58±837。99) g，t＝3。727，P＝0。001]7个指标差异均有统计学意义(均P<0。05)；其中合并胎儿水肿(OR＝7。070，P＝0。020)、严重的胸腔积液(OR＝6。927，P＝0。043)为新生儿Apgar评分的危险因素，宫内治疗(OR＝0。062，P＝0。027)、分娩孕周(OR＝0。577，P＝0。022)可作为新生儿Apgar评分的保护因素。 结论 FHT首诊孕周较早、合并多发结构异常，尤其是合并胎儿水肿者，遗传学检查异常的可能性大，胎儿预后较差；建议对于FHT患者产前尽早进行染色体核型、染色体微阵列分析检测、必要时进行快速全外显子测序以及一些遗传综合征的检测，避免不必要的胎儿治疗，从而提高宫内治疗后围生儿生存率；新生儿预后较差与合并水肿、严重的胸腔积液有关，而适时的宫内治疗、延长孕周可以改善妊娠结局，提高其生存率。 Objective To conjecture the correlation between fetal hydrothorax(FHT)and pregnancy outcome through the analysis of 95 fetuses with hydrothorax and their mothers。 Methods In this case series study， 95 FHT patients admitted to the Third Affiliated Hospital of Zhengzhou University from January 2016 to October 2022 were retrospectively analyzed。According to the pregnancy outcome， these patients were divided into the induced labor group (13 patients) and the delivery group (82 patients)。 General data and genetic examinations of patients in the induced labor group were analyzed to explore their pathogenesis and genetics。According to the neonatal Apgar score， patients in the delivery group were divided into the normal group and the asphyxia group。Fifteen indicators including the maternal age， gestational week at first diagnosis， maternal complications， FHT location， FHT severity， FHT outcome during pregnancy， fetal ascites， hydrops fetalis， other abnormal fetal structures， amniotic fluid volume， intrauterine treatment， gestational week of delivery， mode of delivery， sex of the newborn， and newborn birth weight in the 2 groups were comparatively analyzed by the chi-square test。With the neonatal Apgar score as the dependent variable， variables that had statistical significance during the univariate analysis were included in the regression analysis， and a multivariate binary Logistic regression equation was established to explore the factors affecting the pregnancy outcome。 Results There were significant differences in the FHT outcome during pregnancy (16/22/13 cases vs。2/18/11 cases， χ2=6。994， P=0。030)， FHT severity (27/24 cases vs。9/22 cases， χ2=4。475， P=0。034)， hydrops fetalis (14/37 cases vs。23/8 cases， χ2=17。012， P=0。001)， amniotic fluid volume (21/30 cases vs。24/7 cases， χ2=10。228， P=0。001)， intrauterine treatment (19/32 cases vs。2/29 cases， χ2=9。603， P=0。002)， gestational week of delivery[(38。15±2。05) weeks vs。(34。83±3。14) weeks， t=5。748， P=0。001]， and newborn birth weight[(3 325。00±637。41) g vs。(2 714。58±837。99) g， t=3。727， P=0。001]between the normal and asphyxia groups(all P<0。05)。 Among them， hydrops fetalis (OR=7。070， P=0。020) and severe FHT (OR=6。927， P=0。043) were risk factors for neonatal Apgar scores。Intrauterine treatment (OR=0。062， P=0。027) and gestational week of delivery(OR=0。577， P=0。022) could be used as a protective factor for neonatal Apgar scores。 Conclusions Diagnosis of FHT during the early gestational weeks and multiple fetal structural abnormalities， especially hydrops fetalis， have higher probabilities of abnormal genetic examinations， and the fetal prognosis was poor。It is recommended to carry out chromosomal karyo type and microarray tests， as well as whole exome sequencing and detection of genetic syndromes(if necessary)， to avoid unnecessary fetal treatment and improve the survival rate of perinatal infants after intrauterine treatment。The poor prognosis is related to hydrops fetalis and severe FHT however， timely intrauterine treatment and prolonging pregnancy can improve the pregnancy outcome and increase the survival rate of perinatal infants。

    胸腔积液,胎儿胎儿水肿宫内治疗胎儿预后

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    基于Kaiser Permanente败血症风险计算器的中国新生儿早发型败血症模型的建立

    杨添添郝庆飞张静魏欣雨...
    123-127页
    查看更多>>摘要：目的 利用Kaiser Permanente败血症风险计算器和实验室指标构建并验证新生儿早发型败血症(EOS)临床预测模型。 方法 回顾性纳入2020年1月至2022年6月于郑州大学第一附属医院新生儿科住院的出生胎龄≥34周的新生儿，收集病例临床资料。基于多因素回归分析筛选预测因子，利用R软件和RStudio软件构建列线图模型。采用Hosmer-Lemeshow检验、受试者工作特征曲线、临床决策曲线(DCA曲线)评价模型。 结果 共纳入769例患儿，包括EOS组107例(其中培养证实5例，临床诊断102例)，非EOS组662例。筛选出胎龄、出生体重、体温、白细胞、C反应蛋白、降钙素原、胎膜早破≥18 h、B族链球菌感染、呼吸机应用、产前应用抗生素10个变量纳入列线图。预测模型表现出良好的区分能力(曲线下面积：0。834，95%CI：0。771～0。896)和一致性。DCA曲线分析表明当阈值概率在6%～95%时，采取相应治疗措施可以得到净收益。 结论 基于Kaiser Permanente败血症风险计算器，结合实验室检查为中国新生儿人群开发并验证了新生儿EOS的临床预测模型，供临床诊疗参考，指导抗生素的使用。 Objective To construct a Chinese neonatal model of early-onset sepsis (EOS) using the Kaiser Permanente sepsis risk calculator and laboratory indicators and validate its clinical prediction potential。 Methods Newborns with a gestational age of ≥34 weeks， who were hospitalized in the Department of Neonatology， the First Affiliated Hospital of Zhengzhou University from January 2020 to June 2022 were retrospectively recruited。Their clinical data were collected。Predictors were screened via the multivariate regression analysis， and the Nomogram model was constructed using R software and RStudio software。Hosmer-Lemeshow test， receiver operating characteristic curve， the decision curve analysis (DCA) were used to evaluate the prediction potential of the Nomogram。 Results A total of 769 patients were enrolled， including 107 patients in the EOS group (5 culture-confirmed cases and 102 clinically diagnosed cases)， and 662 cases in the non-EOS group。Ten variables were screened and introduced into the Nomogram， including the gestational age， birth weight， body temperature， white blood cell count， C-reactive protein， procalcitonin， premature rupture of membranes≥18 h， infection of Group B Streptococcus， ventilator application， and prenatal antibiotics。The predictive model showed good discrimination and consistency， with the area under the curve of 0。834 (95%CI: 0。771-0。896)。 The DCA of the prediction model showed that it was effective in clinical application within the effective threshold of 6%-95%， with a net benefit following the application of corresponding treatment measures。 Conclusions A Chinese neonatal model of EOS was created by using the Kaiser Permanente sepsis risk calculator and laboratory indicators， which has been validated effective。It provides references for clinical management and the guidance for the use of antibiotics。

    婴儿,新生早发型败血症败血症风险计算器预测模型

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