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中华小儿外科杂志
中华医学会武汉分会
中华小儿外科杂志

中华医学会武汉分会

袁继炎

月刊

0253-3006

zhxewk@263.net

027-82846835

430014

湖北省武汉市汉口胜利街155号

中华小儿外科杂志/Journal Chinese Journal of Pediatric SurgeryCSCD北大核心CSTPCD
查看更多>>1980年创刊，中华医学会主办。本刊是中国小儿外科专业的高水平学术期刊。充分报道中国小儿外科学科领域基础及临床研究的先进理论、新兴技术和前沿成果，使理论和实践相结合，以促进中国小儿外科学科的发展。本刊读者主要为从事小儿外科临床、教学、科研的工作者及外科、儿科、产科有关人员。本刊辟有述评、临床研究、实验研究、综述、会议纪要、研究报告、技术革新、病例报告等栏目。
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    二次手术治疗肺动脉吊带矫治术后气管狭窄

    杨彦亮徐惠惠吕效政王超...
    193-198页
    查看更多>>摘要：目的 探讨肺动脉吊带合并气管狭窄的治疗方法。 方法 分析2017年2月至2022年10月山东大学附属儿童医院收治的26例肺动脉吊带矫治术后因气管狭窄接受二次手术治疗的患儿资料。其中男14例，女12例；中位年龄为31.3个月；中位体重为13.7 kg；两次手术间隔中位时间为12.5个月。1级狭窄3例，2级狭窄15例，3级狭窄8例。合并右位心3例，合并左肺动脉狭窄或闭锁3例，合并迷走左锁骨下动脉畸形及Kommerell憩室1例。患儿均采用Slide气管成形术加宽气道。术后定期复查电子支气管镜。非正态分布数据采用Wilcoxon符号秩检验（配对样本）。 结果 26例患儿气管狭窄段长度中位数为4 cm，范围为2.5~6.6 cm。本研究病例无手术死亡，无住院期间死亡。术后呼吸机辅助中位通气时间为19.1 h。术后并发症包括吻合口漏合并胸骨哆开1例，声带麻痹3例，气管软化1例，乳糜胸1例，脑损伤2例。术后中位随访时间为7.0个月。活动耐力略差3例，其余患儿无呼吸道症状。3例行左肺动脉加宽术的患儿术后定期复查心脏彩色多普勒超声检查提示左肺动脉血流通畅。3例声带麻痹患儿术后3个月症状消失，2例脑损伤患儿均康复治疗中。 结论 当肺动脉吊带合并气管狭窄，气管最窄处宽度小于正常宽度的40%，或为正常宽度的40%~60%且临床症状明显时，应一期手术加宽气管。 Objective To explore the treatment of pulmonary artery sling (PAS) plus tracheal stenosis (TS). Methods From February 2017 to October 2022, the relevant clinical data were reviewed for 26 children undergoing secondary surgery for tracheal stenosis after pulmonary artery sling correction. There were 14 boys and 12 girls with a median age of 31.3 months and a median weight of 13.7 kg. The median interval between two surgeries was 12.5 months. Clinical grades of stenosis were Ⅰ (n=3), Ⅱ (n=15) and Ⅲ (n=8). Concurrent conditions included right-sided heart (n=3) and left pulmonary artery stenosis/occlusion (n=3). One child had combined left subclavian artery malformation and Kommerell diverticulum with vagus. Slide tracheoplasty was employed for widening airway. Regular postoperative reviews were conducted by electronic bronchoscopy. Wilcoxon's signed rank test (paired sample) was performed for abnormal distribution data. Results Median length of tracheal stenosis segment was 4(2.5-6.6) cm. There was no intraoperative or in-hospital mortality. Median period for postoperative ventilator assisted ventilation was 19.1 hours. Postoperative complications included anastomotic leakage plus sternal opening (n=1), vocal cord paralysis (n=3), tracheal softening (n=1), chylothorax (n=1) and brain injury (n=2). Median postoperative follow-up period was 7.0 months. Three cases had slightly poor activity endurance while the remainders were free of respiratory symptoms. Three cases of left pulmonary artery widening underwent regular echocardiographic follow-ups. There was unobstructed blood flow of left pulmonary artery. Three children of vocal cord paralysis resolved at 3 months postoperatively while two cases of brain injury underwent rehabilitation. Conclusions When pulmonary artery sling co-exists with tracheal stenosis, the width of the narrowest part of trachea is less than 40% of normal width or between 40% and 60% of normal width and clinical symptoms are obvious, trachea should be widened with one-stage surgery.

    气管狭窄迷走左肺动脉手术治疗

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    婴儿心脏手术体外循环与体外膜肺氧合切换的探讨

    钱晓亮陈月梁维杰宋书波...
    199-202页
    查看更多>>摘要：目的 探讨先天性心脏病患儿手术后体外循环（extracorporeal circulation，ECC）停机困难转为体外膜肺氧合（extracorporeal membrane oxygenation，ECMO）辅助治疗的优化方案的效果。 方法 回顾性分析阜外华中心血管病医院2020年1月至2021年12月收治的12例复杂先天性心脏病患儿临床资料，其中男8例，女4例；中位年龄3.1个月，年龄范围在1~8个月；身高为（65.43±8.56）cm，体重为（3.24±0.35）kg。患儿均ECC心脏手术后停机困难，切换为ECMO继续辅助治疗，运用ECC内血液动态预充至ECMO系统，切换完成后在ECMO系统中行超滤治疗，观察系统切换及超滤过程中的血流动力学及血气等指标变化。两组比较采用独立样本t检验。 结果 患儿术中ECC转流时间为（245.00±12.08）min，阻断时间为（105.23±13.06）min，ECMO准备时间为（8.34±1.16）min，系统切换时间为（5.12±1.46）min，置换期间动脉压维持在（35.23±5.34）mmHg（1 mmHg=0.133 kPa）。与置换前相比，置换后超滤前红细胞比容、血红蛋白、胶体渗透压均有所降低，分别为（26.6±0.2）%、（93±2）g/L、（23.4±0.5）mmHg比（23.6±0.4）%、（85±4）g/L、（21.3±0.6）mmHg，差异有统计学意义（P<0.05）；与置换后超滤前相比，超滤后红细胞比容、血红蛋白、胶体渗透压包被蛋白均有所升高，（23.6±0.4）%、（85±4）g/L、（21.3±0.6）mmHg比（30.5±0.6）%、（102±6）g/L、（26.4±0.3）mmHg，pH值、血乳酸、碱剩余均有所降低，分别为（7.37±0.03）、（6.3±0.6）mmol/L、（-2.06±0.16）mmol/L比（7.32±0.04）、（3.9±0.4）mmol/L、（-3.47±0.26）mmol/L，差异有统计学意义（P<0.05）。 结论 婴儿心脏手术ECC切换ECMO过程中，运用ECC中的血液动态预充ECMO系统结合改良超滤技术的优化处理方案方便快捷、安全有效。 Objective To explore the effectiveness of an optimized protocol for converting postoperative difficulty in extracorporeal circulation (ECC) into extracorporeal membrane oxygenation (ECMO) as an adjuvant therapy in children with congenital heart disease (CHD). Methods From January 2020 to December 2021, retrospective analysis was performed for 12 children with complex CHD. There were 8 boys and 4 girls with a median age of 3.1(1-8) months. Body height was (65.43±8.56) cm and body weight (3.24±0.35) kg. After ECC cardiac procedures, all of them had difficulty weaning off the machine and switched into ECMO for auxiliary treatment, using dynamic pre-charging of blood within ECC. After switching, ultrafiltration was performed in ECMO system. The changes of hemodynamics and blood gas parameters were observed during system switching and ultrafiltration. Two groups were compared by independent sample t-test. Results Intraoperative ECC time was (245.00±12.08) min, blocking time (105.23±13.06) min, ECMO preparation time (8.34±1.16) min and system switching time (5.12±1.46) min. During replacement, arterial pressure was maintained at (35.23±5.34) mmHg (1 mmHg=0.133 kPa). As compared with pre-replacement, hematocrit, hemoglobin and colloid osmotic pressure pre-hemoconcentration decreased from (26.6±0.2)%, (93±2) g/L, (23.4±0.5) mmHg to (23.6±0.4)%, (85±4) g/L and (21.3±0.6) mmHg. There were statistically significant differences (P<0.05). Before and after hemoconcentration, hematocrit, hemoglobin and colloidal osmotic pressure spiked by (23.6±0.4)%, (85±4) g/L, (21.3±0.6) mmHg to (30.5±0.6)%, (102±6) g/L and (26.4±0.3) mmHg. And pH value, blood lactate and alkaline surplus declined from (7.37±0.03), (6.3±0.6) mmol/L, (-2.06±0.16) mmol/L to (7.32±0.04), (3.9±0.4) mmol/L and (-3.47±0.26) mmol/L. The differences were statistically significant (P<0.05). Conclusions While switching from ECC into ECMO during infantile cardiac surgery, an optimized protocol of combining blood dynamic pre-filling ECMO system in ECC with modified hemoconcentration technology is convenient, fast, safe and effective.

    心脏病体外膜肺氧合体外循环婴儿

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    心下型完全性肺静脉异位引流外科治疗的中远期疗效及危险因素分析

    赵雅淇宓亚平陈纲贾兵...
    203-207页
    查看更多>>摘要：目的 分析心下型完全性肺静脉异位引流的手术治疗效果，并探讨影响预后的相关危险因素。 方法 回顾性分析2002年1月至2017年1月在复旦大学附属儿科医院心血管中心确诊为心下型完全性肺静脉异位引流的59例患儿的临床资料，其中男41例，女18例；手术时中位年龄23（10，49）d；手术时中位体重3.5（3.2，4.1）kg。59例患儿均接受完全性肺静脉异位引流根治术，术中常规横断垂直静脉，应用Sutureless技术24例。统计分析患儿术前、术中相关资料以及肺静脉形态与手术疗效，计量资料采用独立样本t检验、Mann-Whitney U检验分析，计数资料采用χ2检验或Fisher精确检验。 结果 59例患儿体外循环时间为（109±37）min，主动脉阻断时间为（51±13）min。术后延迟关胸33例，术后早期死亡4例，中远期随访5~20年。术后未发生肺静脉狭窄（pulmonary vein stenosis，PVS）42例，发生PVS 17例；2例家属放弃再干预后死亡；再干预5例，其中2例再干预后死亡。与非PVS患儿相比，PVS患儿中采用Sutureless技术比例低，Y形共同肺静脉比例、延迟关胸比例较高、术后机械通气时间显著延长，差异有统计学意义（P<0.05）。Y形共同肺静脉组患儿术后3、6、12个月未发生PVS占比为3/8、3/8、3/8，非Y形共同肺静脉组患儿术后3、6、12个月未发生PVS占比分别为26/29、24/29、24/29，两者比较差异有统计学意义（P=0.07）。 结论 心下型完全性肺静脉异位引流根治术治疗效果总体良好，共同肺静脉形态与术后肺静脉狭窄相关。改良Sutureless技术能改善心下型完全性肺静脉异位引流的手术疗效和远期预后。 Objective To explore retrospectively the clinical efficacy of infra-cardiac total anomalous pulmonary venous drainage (TAPVD) and to examine the related prognostic factors. Methods From January 2002 to January 2017, the relevant clinical data were retrospectively reviewed for 59 children with infra-cardiac TAPVD. There were 41 boys and 18 girls with a median age of 23(10-49) days and a median weight of 3.5(3.2-4.1) kg. Radical operation of TAPVD was performed. During operation, vertical vein was transected routinely and Sutureless technique applied (n=24). Perioperative data, pulmonary vein morphology and surgical efficacy were statistically analyzed. The measurement data were analyzed by independent sample T and Mann-Whitney U tests. And counting data were analyzed by χ2 or Fisher's exact test. Results Cardiopulmonary bypass time was (109±37) min and aortic occlusion time (51±13) min. During a follow-up period of (5-20) years, there were delayed chest closure (n=33) and early mortality (n=4). Postoperative pulmonary vein stenosis (PVS) was absent (n=42) and present (n=17). Two deaths occurred after family members gave up re-intervention. Among 5 re-intervened cases, 2 children died. As compared with non-PVS counterparts, the proportion of using Sutureless technique was lower while the proportions of Y-shaped common pulmonary vein and delayed chest closure were higher in PVS children. And postoperative mechanical ventilation time became significantly prolonged with statistical significance (P<0.05). At Month 3/6/12 post-operation, the proportion of children of Y-shaped common pulmonary vein without PVS was 3/8, 3/8 and 3/8 while the proportion of children of non-Y-shaped common pulmonary vein without PVS 26/29, 24/29 and 24/29 respectively. The inter-group differences were statistically significant (P=0.07). Conclusions Modified Sutureless technique is efficacious for infra-cardiac TAPVD. And the morphology of common pulmonary vein is correlated with postoperative restenosis

    狭窄,肺静脉完全性肺静脉异位引流危险因素

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    儿童肝移植术后早期血流感染的危险因素及对预后的影响

    邹国寅董冲孙超王凯...
    208-216页
    查看更多>>摘要：目的 探讨儿童肝移植受者术后早期（3个月内）血流感染的患病率和致病菌分布情况，并分析血流感染（blood stream infection，BSI）和由多重耐药菌（multi-drug resistant organism，MDRO）引起的BSI的高危因素和预后。 方法 收集并分析2016年8月至2020年12月在天津市第一中心医院器官移植中心儿童器官移植科进行肝移植的872例患儿的临床资料，根据手术后早期BSI的发生情况以及病原菌是否为MDRO将患儿分为3组：非BSI组（765例）、非MDRO BSI组（73例）和MDRO BSI组（34例）。采用单变量分析和多元logistic回归分析，确定术后早期BSI的独立危险因素，比较各组患儿生存率和术后并发症发生率。 结果 872例患儿中，共有107例（12.3%，107/872）在术后3个月内分离出207株病原菌。207株病原菌中有25种不同的病原体，其中肺炎克雷伯杆菌（30.9%，64/207）、屎肠球菌（23.7%，49/207）占比较高。共有34例患儿中分离出MDRO 76株（36.7%，76/207），其中以耐碳青霉烯肺炎克雷伯菌（56株）为主。术前1个月内抗生素暴露、手术时长、重症监护病房停留时间、术后再次手术、术后低磷血症和低镁血症是术后早期BSI发生的独立危险因素。术前1个月抗生素暴露、腹腔引流时间、术后低磷血症和再移植是术后早期MDRO引起BSI的独立危险因素。与非BSI组相比，另外两组的术后并发症发生率明显更高，包括肝动脉血栓、胆道狭窄、巨细胞病毒感染和肺感染。与非MDRO BSI组和非BSI组相比，MDRO BSI患儿的1年和3年生存率显著降低（76.5%比94.5%和95.8%，P<0.001；73.5%比94.5%和95.4%，P<0.001）。 结论 儿童肝移植术后早期BSI的发生率仍然很高，MDRO BSI的占比较大且预后不佳。移植术后早期MDRO BSI的独立危险因素包括术前1个月内抗生素暴露、腹腔引流时间、术后低磷血症和再移植，预防儿童肝移植术后早期MDRO BSI有助于改善预后。 Objective To explore the prevalence and pathogen distribution of early (within 3 months) bloodstream infection (BSI) after pediatric liver transplantation (LT) and analyze the high-risk variables and prognosis of BSI due to multidrug-resistant organism (MDRO). Methods Between August 2016 and December 2020, the relevant clinical data were reviewed for 872 children undergoing LT at First Central Municipal Hospital. According to the postoperative occurrence of early BSI and whether or not infected bacteria belonged to MDRO, they were assigned into three groups of non-BSI group (n=765), non-MDRO BSI (n=73) and MDRO BSI (n=34). The independent risk variables of early postoperative BSI were identified by univariate and multiple Logistic regression analyses. Survival rates and postoperative complications of three groups were compared. Results Among them, 209 pathogen strains in 25 different types were isolated from 107 patients (12.3%, 107/872) within Month 3 post-LT. A large majority were Klebsiella pneumoniae (30.9%, 64/207) and Enterococcus faecium (23.7%, 49/207). Seventy-six MDRO strains were isolated from 34 recipients (36.7%, 76/207), mostly carbapenem-resistant K. pneumoniae (56 strains). Antibiotic exposure within one month pre-LT, operative duration, length of ICU stay, reoperation post-LT and hypophosphatemia/hypomagnesemia post-LT were independent risk factors for early postoperative BSI. And early postoperative MDRO BSI was associated with antibiotic exposure within one month pre-LT, abdominal drainage time, postoperative hypophosphatemia and retransplantation. As compared with non-BSI group, another two groups had significantly higher occurrences of postoperative complications, including hepatic artery thrombosis, biliary stricture, cytomegalovirus infection and pulmonary infection. The 1/3-year survival rates were significantly lower in MDRO BSI group than those in non-MDRO BSI and non-BSI groups (76.5% vs 94.5% and 95.8%, P<0.001 73.5% vs 94.5% and 95.4%,P<0.001). Conclusions Early BSI post-LT is common in children. Most cases who have MDRO BSI have a poor prognosis. Antibiotic exposure within one month pre-LT, abdominal drainage time, postoperative hypophosphatemia and retransplantation are independent risk factors for early MDRO BSI. Prevention of early MDRO BSI post-LT improves prognosis.

    肝移植儿科学血流感染预后危险因素

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    肾恶性横纹肌样瘤免疫浸润分析及预后模型构建

    韦佶秀许琼倩陈鹏黄静茹...
    217-223页
    查看更多>>摘要：目的 基于生物信息学分析肾恶性横纹肌样瘤免疫浸润水平，鉴定与预后免疫相关基因，并构建预后模型。 方法 从TARGET数据库获取50例肾恶性横纹肌样瘤高通量测序数据和临床参数，使用estimate、CIBERSORT、ssGSEA分析免疫浸润特征及免疫浸润结果聚类亚型（高免疫浸润组和低免疫浸润组），通过亚型间的基因差异分析获取差异免疫相关基因。单因素COX、LASSO回归分析筛选预后相关免疫基因并构建预后模型。 结果 高免疫浸润组较低免疫浸润组具有较高的估计得分、免疫评分和基质核心水平，且M2巨噬细胞浸润程度最高。筛选出4个预后免疫相关基因（FGF12、REG1A、TSLP、ANGPTL7）并以此构建预后风险模型。Kaplan-Meier生存曲线和ROC评估预后模型显示出较高风险分层能力，单因素和多因素COX回归分析显示预后模型风险特征值差异有统计学意义。 结论 以预后免疫相关基因FGF12、REG1A、TSLP、ANGPTL7构建的免疫相关基因风险预后模型可信度较高，为儿童肾恶性横纹肌样瘤治疗提供新的视角。 Objective To explore immune infiltration level of malignant rhabdoid renal tumor based upon bioinformatics to identify the genes related to immune prognosis and to construct a prognostic model. Methods The high-throughput sequencing data and clinical parameters of 50 samples of malignant rhabdoid renal tumor were obtained from the database of TARGET. Immune infiltration characteristics and clustering subtypes (Immunity_High/Low) of immune infiltration result were analyzed with "estimate" R package, "CIBERSORT" R package and ssGSEA algorithm. Differentially expressed immunity genes were obtained through differential gene analysis among clustering subtypes. Univariate COX regression and LASSO regression were utilized for screening prognosis-associated immunity genes and constructing a prognostic model. Results The group of Immunity_High had higher scores of ESTIMATE, Immune and Stromal than Immunity_Low and the highest degree of infiltration of macrophages was M2. Four prognosis-associated immunity genes (FGF12, REG1A, TSLP & ANGPTL7) were screened for constructing a prognostic risk model. Kaplan-Meier survival curve and SvivalROC prognostic model showed a high risk stratification capability. Univariate and multivariate COX regression analysis revealed statistically significant difference in risk value of prognostic models. Conclusions Based upon FGF12, REG1A, TSLP and ANGPTL7, the risk prognostic model of immune-related genes is highly reliable. It may provide new therapeutic rationales for malignant rhabdoid renal tumor in children.

    肾肿瘤肾横纹肌样瘤肿瘤免疫学浸润预后

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    儿童结肠系膜淋巴管畸形的临床特点分析

    严佳虞谢川平彭春辉庞文博...
    224-230页
    查看更多>>摘要：目的 分析儿童结肠系膜淋巴管畸形的临床特点及手术方式选择。 方法 回顾性分析2010年1月至2021年12月首都医科大学附属北京儿童医院手术治疗的44例结肠系膜淋巴管畸形患儿的临床资料，分析其囊肿特点及手术方式选择的影响因素。通过门诊或电话随访预后，包括有无残留、复发或粘连性肠梗阻等并发症。通过多因素二分类logistic回归分析探究选择开腹或腹腔镜手术的影响因素。 结果 男24例，女20例，平均就诊年龄为2.9岁。腹痛、腹胀和发热为主要临床症状。囊肿主要位于乙状结肠系膜（31.8%，14/44）和横结肠系膜（27.3%，12/44）；临床分型主要为Ⅰ型（34.1%，15/44）和Ⅱ型（45.5%，20/44）；病理类型包括大囊型（86.4%，38/44）和混合型（13.6%，6/44）；囊肿平均长径为8.5 cm，囊液性质清亮占56.8%（25/44），浑浊占34.1%（15/44）。术前主要并发症为囊内出血（43.2%，19/44）和囊肿感染（25.0%，11/44）。44例患儿行开腹手术（含腹腔镜中转开腹）31例（70.5%），腹腔镜手术13例（29.5%）；其中单纯囊肿剥除23例（52.3%），完整囊肿切除并肠切除17例（38.6%），囊肿部分切除并开窗引流4例（9.1%）。Ⅰ型结肠系膜淋巴管畸形行开腹手术（13/15比10/20，P=0.034）及完整囊肿切除并肠切除（13/15比2/20，P<0.001）的比例明显高于Ⅱ型。多因素分析表明Ⅰ型结肠系膜淋巴管畸形（OR=7.0，95%CI 1.1~45.5，P=0.034）和术前囊内出血（OR=8.3，95%CI 1.3~52.6，P=0.026）是选择开腹或腹腔镜中转开腹的主要原因。43例（97.7%）患儿获得随访，平均随访时间为4.3年，9例（20.5%，9/44）出现术后并发症（残留或复发5例，粘连性肠梗阻2例，腹壁切口疝1例，污粪1例）。 结论 结肠系膜淋巴管畸形好发部位与系膜范围相关。该病术前并发症发生率高，术前囊内出血是除临床分型外决定手术方式选择的最主要因素。 Objective To explore the clinical features and surgical approaches of colonic mesenteric lymphatic malformation in children. Methods From January 2010 to December 2021, the relevant clinical data were retrospectively reviewed for 44 operated children of colonic mesenteric lymphatic malformation. Cyst characteristics and influencing factors of surgical approaches were recorded. Follow-ups of postoperative complications (residual cyst, recurrence & adhesive ileus) were conducted at outpatient clinics or through telephone interviews. Multivariate binary Logistic regression analysis was employed for examining the influencing factors of selecting laparotomy or laparoscopy. Results There were 24 boys and 20 girls with an average age of 2.9 years. The major symptoms were abdominal pain, abdominal distention and fever. Cysts of colonic mesenteric lymphatic malformation were located at sigmoid mesocolon (31.8%) and transverse mesocolon (27.3%). Type Ⅰ (34.1%) and type Ⅱ (45.5%) dominated. The pathological types were macrocystic (86.4%) and mixed (13.6%). Average long diameter of cyst was 8.5 cm. Fluid was serous (56.8%) and turbid (34.1%). Hemorrhage (43.2%) and infection (25.0%) of cyst were the predominant preoperative complications. The procedures included laparotomy (n=31, 70.5%), including laparoscopy converting into laparotomy, and laparoscopy (n=13, 29.5%). Simple cyst excision (n=23, 52.3%), complete cyst excision with segmental bowel resection (n=17, 38.6%) and partial cyst excision & drainage (n=4, 9.1%) were performed. The percentages of laparotomy (13/15 vs 10/20, P=0.034) and complete cyst excision with segmental bowel resection (13/15 vs 2/20, P<0.001) were significantly higher performed in type I than in type Ⅱ patients. Multivariate analysis revealed that type Ⅰ classification (OR=7.0, 95%CI 1.1-45.5, P=0.034) and preoperative hemorrhage (OR=8.3, 95%CI 1.3-52.6, P=0.026) were two major factors for selecting laparotomy. Forty-three patients (97.7%) were followed up with a mean period of 4.3 years. Among 9 complicated cases (20.5%), there were residual cyst or recurrence (n=5), adhesive ileus (n=2), incisional hernia (n=1) and soiling (n=1). Conclusions The incidence of colonic mesenteric lymphatic malformation is correlated with area of mesocolon. Preoperative complications are common in children with colonic mesenteric lymphatic malformation. Preoperative hemorrhage and specific types may determine the choice of surgical approaches.

    结肠肠系膜淋巴管畸形手术治疗

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    JMJD3在新生儿坏死性小肠结肠炎肠道组织中的表达变化

    马淑蓉徐铃琪陈璐璐孙旭...
    231-236页
    查看更多>>摘要：目的 探讨组蛋白去甲基化酶含Jumonji结构域的蛋白3（Jumonji domain-containing protein D3，JMJD3）在新生儿坏死性小肠结肠炎（necrotizing enterocolitis，NEC）肠道组织中的表达情况。 方法 收集对照（先天性小肠闭锁）患儿及NEC患儿手术中切除的回肠组织进行RNA测序，筛选出差异基因。对差异基因进行组蛋白甲基化修饰酶相关基因聚类分析，筛选出目的基因赖氨酸特异性去甲基化酶6B[lysine（K）-specific demethylase 6B，KDM6B]，并采用实时荧光定量聚合酶链反应扩大样本量进行验证。蛋白免疫印迹和免疫组织化学检测JMJD3以及其底物组蛋白H3第27位赖氨酸三甲基化（trimethylation of histone H3 at lysine 27，H3K27me3）在肠道组织中的蛋白表达情况。组间比较根据数据分布特点采用t检验或Mann-Whitney U检验。 结果 RNA测序筛查出1 481条差异基因，其中643条基因上调，838条基因下调。筛查NEC肠道组织中30种组蛋白甲基化修饰酶的表达情况，其中KDM6B在NEC样本中差异表达最显著[Log2（fold change）=0.769 104，P=0.009 009]。实时荧光定量聚合酶链反应结果显示，与对照组相比，KDM6B在NEC肠道组织中高表达（P<0.05）；蛋白免疫印迹和免疫组织化学结果显示JMJD3蛋白水平在NEC肠道组织中明显增高，H3K27me3蛋白水平在NEC肠道组织中降低。 结论 在NEC肠道组织中组蛋白去甲基化酶JMJD3高表达，H3K27me3低表达。JMJD3可能通过降低H3K27me3的表达水平调控NEC的发生发展。 Objective To explore the expression of histone demethylase Jumonji domain-containing protein D3 (JMJD3) in intestinal tissue of necrotizing enterocolitis (NEC). Methods RNA-sequencing was conducted for screening out the differential genes in intestinal tissues from controls (congenital intestinal atresia) and NEC children. And cluster analysis of histone methylation modification-related genes was utilized for screening target gene lysine (K)-specific demethylase 6B (KDM6B). Quantitative real-time polymerase chain reaction (qRT-PCR) was performed for verifying the expression of KDM6B in intestinal tissues. Western blot and immunohistochemistry were utilized for detecting the protein expressions of JMJD3 and trimethylation of histone H3 at lysine 27 (H3K27me3) in intestinal tissues. Group comparisons were made by t or Mann-Whitney U test, depending on data distribution. Results RNA sequencing revealed 1481 differentially expressed genes, including 643 up-regulated and 838 down-regulated genes. Among 30 histone methylation modifying enzymes, KDM6B was the most differentially expressed in NEC intestinal tissues [Log2(fold change)=0.769104, P=0.009009]. qRT-PCR indicated a higher expression of KDM6B in NEC intestinal tissues compared to controls (P<0.05). Western blot and immunohistochemistry indicated a higher level of JMJD3 protein and a lower level of H3K27me3 protein in NEC intestinal tissues. Conclusions A high expression of histone demethylase JMJD3 and a low expression of H3K27me3 are detected in NEC intestinal tissues. JMJD3 may participate in the process of NEC through down-regulating the expression of H3K27me3.

    急腹症新生儿坏死性小肠结肠炎组蛋白甲基化组蛋白去甲基化酶JMJD3

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    新生婴儿骶尾部畸胎瘤的诊治和预后——单中心104例分析

    严文波潘伟华陈凯邬文杰...
    237-241页
    查看更多>>摘要：目的 分析总结新生婴儿骶尾部畸胎瘤诊治情况与长期预后。 方法 收集上海交通大学医学院附属新华医院小儿外科2000年9月至2021年9月间收治的104例年龄<3月龄的新生婴儿骶尾部畸胎瘤患儿的临床资料。男37例，女67例；平均孕期38.96周，平均出生体重3 369 g。均在收住院后尽早行肿瘤切除及尾骨切除术，同时对肿瘤及其边缘进行病理活检。出院后门诊随访每1次，观察肿瘤标志物甲胎蛋白等指标及影像学复检，检查患儿生长发育、智力、大小便情况等。分类变量资料采用χ2检验。 结果 104例中，良性成熟型96例（92.31%），恶性8例（7.69%），包括2例卵黄囊瘤，均无转移发生。平均瘤体直径6.93 cm。按Altman分型，Ⅰ~Ⅱ型占82.69%（86/104）。产前诊断率32.69%（34/104），以孕15~39周产前筛查检出。58例合伴其他畸形，心血管畸形占91.38%（53/58），其次为泌尿系畸形8.62%（5/58）。患儿平均住院天数24.18 d。平均随访7.84年，随访率73.08%（76/104），无死亡病例，复发1例。智力差6.58%（5/76），排便异常21.05%（16/76），排尿异常2.63%（2/76）。 结论 新生婴儿骶尾部畸胎瘤以良性为主，部分合伴畸形，早期诊断特别是产前诊断对治疗方案、评估预后有利。手术治疗长期随访预后好，复发少。 Objective To explore the diagnosis, treatment and long-term prognosis of sacrococcygeal teratoma in neonates. Methods The relevant clinical data were retrospectively reviewed for 104 neonates of sacrococcygeal teratoma aged under 3 months from September 2000 to September 2021. There were 37 boys and 67 girls with an average gestational age of 38.96 weeks and an average birth weight of 3 369 gram. Tumor and coccyx resections were performed with a pathological biopsy of tumor and its margin. After discharge, outpatient follow-ups were performed once every 3-6 months. Tumor marker (e.g. alpha-fetoprotein) and imaging re-examination were observed along with growth & development, intelligence, bowel and bladder function. Categorical data were processed by Chi-square test. Results Clinical types were benign mature (n=96, 92.31%) and malignant (n=8, 7.69%). The latter included yolk sac tumor (n=2) and none of them had metastasis. Average tumor diameter was 6.93 cm. According to the scheme of Altman classification, type Ⅰ-Ⅱ accounted for 82.69% (86/104). Prenatal diagnosis rate was 32.69% (34/104) and it was detected by prenatal screening at 15-39 weeks of gestation. And 58 cases were accompanied by other malformations, including cardiovascular malformations (91.38%, 53/58) and urinary system malformations (8.62%, 5/58). Average hospitalization stay was 24.18 days. Average follow-up time was 7.84 years with a follow-up rate of 73.08% (76/104). Without any mortality, there was only one recurrence. The outcomes were poor intelligence (6.58%, 5/76), bowel dysfunction (21.05%, 16/76) and urinary dysfunction (2.63%, 2/76). Conclusion Sacrococcygeal teratoma is mostly benign in neonates. Some cases are accompanied by malformations. Early prenatal diagnosis is beneficial for treatment planning and prognostic evaluations. Surgery has decent long-term outcomes with a low recurrence rate.

    畸胎瘤骶尾部畸胎瘤产前诊断手术治疗随访

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    儿童获得性隐睾8例临床分析

    沈晶徐珊朱信强张保峰...
    242-246页
    查看更多>>摘要：目的 探讨儿童获得性隐睾的临床特点，总结其诊断及治疗经验。 方法 回顾性分析杭州美中宜和妇儿医院2020年5月至2021年7月收治的获得性隐睾患儿8例，年龄为（6.9±2.3）岁；均为单侧患病。收集患儿的一般临床资料，包括年龄、合并症、既往睾丸检查等资料。本组病例诊断获得性隐睾后均采用手术治疗，术中观察患侧睾丸的解剖学特点，术前及术后利用超声测量睾丸的三维直径，计算睾丸体积、睾丸萎缩指数，采用t检验比较分析手术前后患侧与健侧睾丸的体积差异。 结果 本组患儿均合并重度尿道下裂，在尿道下裂术后临床随诊中发现获得性隐睾，其中2例为会阴型尿道下裂，6例为阴茎阴囊型尿道下裂，1例伴有46，XX性逆转综合征。本组患儿均有尿道下裂术后随访记录和儿童泌尿外科医师的睾丸体检记录，其中6例曾为正常睾丸，2例曾为回缩性睾丸。2例对侧睾丸曾因诊断先天性隐睾行睾丸下降固定术。本组患儿术前患侧睾丸体积（0.38±0.19）ml，健侧睾丸体积（0.65±0.29）ml，患侧睾丸体积较健侧均有不同程度减小（P<0.05），睾丸萎缩指数为（41.4±16.6）%。8例患儿诊断后均行睾丸下降固定术，术中发现患侧睾丸均位于腹股沟管内，未发现附睾畸形，4例鞘状突未闭合。术后3个月复查B型超声，患侧睾丸体积为（0.57±0.23）ml，较术前明显增大（P<0.05），健侧睾丸体积为（0.66±0.23）ml，与术前差异无统计学意义（P>0.05）。 结论 近端型尿道下裂以及回缩性睾丸的患儿发生获得性隐睾的风险显著增加，应密切随访睾丸位置至青春期以后。获得性隐睾可能发生睾丸体积减小，并且与先天性隐睾有相似的组织病理学改变，影响生育功能，常规睾丸下降固定术效果明确，诊断后应及时采取手术治疗。 Objective To explore the clinical characteristics of acquired undescended testis in children and to summarize the experiences of diagnosis and treatment. Methods From May 2020 to July 2021, retrospective review was performed for 8 operated children of acquired unilateral undescended testis. The diagnostic age was (6.9±2.3) years. The relevant clinical data were analyzed, including age, complications and previous testicular examinations. Anatomical characteristics of affected testis were observed during operation. The three-dimensional (3D) diameters of testicle were measured by ultrasound before and after operation and testicular volume and atrophy index calculated. Results All of them had concurrent severe hypospadias during postoperative follow-ups. The clinical types were perineal (n=2) and penoscrotal (n=6). One child was diagnosed as 46, XX reversal syndrome. Testis was normal (n=6) and retractile (n=2). Two cases of contralateral testicle underwent orchiopexy for congenital undescended testis. Preoperative testicular volume was (0.38±0.19) ml at affected side and (0.65±0.29) ml at healthy side. Testicular volume of affected side declined as compared with healthy side (P<0.05). Testicular atrophy index was (41.4±16.6)%. Orchiopexy was performed after a definite diagnosis. The testes at affected side were located in inguinal canal. No epididymal deformity was detected intraoperatively and 4 cases had open processus vaginalis. At 3 months postoperatively, testicular volume of affected side was (0.57±0.23) ml and it was significantly greater than that pre-operation (P<0.05). Testicular volume of healthy side was (0.66±0.23) ml and there was no significant difference with that at pre-operation (P>0.05). Conclusions Children with severe hypospadias and retractile testis are at a particularly elevated risk for acquired undescended testis. And testicular location should be closely followed until adolescence. Acquired undescended testis may decrease in volume and share similar histopathological features with congenital cryptorchidism. Reproductive function may be compromised. Timely surgery is recommended after a definite diagnosis.

    隐睾儿童睾丸固定术

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    基于SEER数据库分析1 011例儿童睾丸非精原细胞瘤的临床病理特征及预后

    杜勇刘亮华邹兵陈志力...
    247-255页
    查看更多>>摘要：目的 分析睾丸非精原细胞瘤（nonseminomatous germ cell tumor，NSGCT）患儿的临床病理特征及预后，探讨其预后影响因素。 方法 回顾性分析SEER数据库中2004年至2019年诊断为NSGCT者（年龄≤18岁）的临床资料，采用χ2检验或Fisher确切概率法对比各组患儿病例构成比，采用Kaplan-Meier法绘制生存曲线并用Log-rank法进行检验，采用单因素和多因素COX回归风险模型分析评估影响NSGCT患儿总生存期（overall survival，OS）和癌症特异性生存期（cancer-specific survival，CSS）的相关因素。 结果 共1 011例NSGCT患儿纳入本研究，其中混合性生殖细胞肿瘤595例，卵黄囊瘤120例，胚胎性癌143例，绒毛膜癌52例，恶性畸胎瘤101例。整个队列的中位年龄为17岁（0~18岁），中位生存时间为84个月（1~191个月）。整个队列的5年OS和CSS分别为97%和97.2%；亚组生存分析显示混合性生殖细胞肿瘤、卵黄囊瘤、胚胎性癌、绒毛膜癌、恶性畸胎瘤的5年OS和CSS分别为97.2%、98.1%、100%、82.9%、97.7%和97.6%、98.1%、100%、82.9%、97.7%，其中绒毛膜癌组患儿预后最差，组间比较差异有统计学意义（P<0.05）。单因素和多因素分析结果显示TNM分期（OS：HR=3.313，P<0.001；CSS：HR=4.065，P<0.001）、肿瘤大小（OS：HR=2.124，P=0.001；CSS：HR=1.822，P=0.013）、睾丸切除术（OS：HR=0.01，P<0.001；CSS：HR=0.01，P<0.001）及淋巴结清扫（OS：HR=0.09，P=0.018；CSS：HR=0.107，P=0.028）为NSGCT患儿OS和CSS的独立影响因素。 结论 儿童NSGCT是较为罕见的恶性生殖细胞肿瘤，其中卵黄囊瘤预后较好，绒毛膜癌预后最差，根治性手术是所有患儿首选的治疗方式，高危患儿应选择根治性手术、腹腔淋巴结清扫和化疗的综合治疗，患儿总体预后较好。 Objective To explore the clinical features, outcomes and prognostic factors of pediatric children with nonseminomatous germ cell tumor (NSGCT) based upon the SEER database. Methods The database of Surveillance, Epidemiology and End Results (SEER) was retrospectively reviewed for 1011 NSGCT children aged ≤18 years from 2004 to 2019. Categorical variables were compared with chi-square or Fisher's exact test. Log-rank test with Kaplan-Meier method was applied for survival curves. Univariate and multivariate Cox regression models were employed for assessing the impact of prognostic factors on overall survival (OS) and cancer-specific survival (CSS). Results Among them, there were 595 mixed germ cell tumors, 120 yolk sac tumors, 143 embryonal carcinomas, 52 choriocarcinomas and 101 teratomas. The median age was 17(0-18) years. During a median follow-up period of 84(1-191) months, 5-year OS/CSS rate for the whole cohort was 97% and 97.2% respectively. Subgroup analysis revealed that 5-year OS and CSS rates of mixed germ cell tumor, yolk sac tumor, embryonal carcinoma, choriocarcinoma and teratoma were 97.2%, 98.1%, 100%, 82.9%, 97.7% and 97.6%, 98.1%, 100%, 82.9%, 97.7% respectively. And choriocarcinoma children had the worst prognoses (P<0.05). Univariate and multivariate analyses revealed that TNM stage (OS:HR=3.313, P<0.001 CSS:HR=4.065, P<0.001), tumor size (OS:HR=2.124, P=0.001 CSS:HR=1.822, P=0.013), radical orchiectomy (OS:HR=0.01, P<0.001 CSS:HR=0.01, P<0.001) and lymph node dissection (OS:HR=0.09, P=0.018 CSS:HR=0.107, P=0.028) were independent prognostic factors for OS and CSS. Conclusions Pediatric testicular NSGCT is a rare malignant germ cell tumor. Yolk sac tumor has a favorable prognosis while the outcomes of choriocarcinoma are the worst. Radical surgery is preferred for high-risk NSGCT children. Comprehensive treatments include radical surgery, adjuvant chemoradiotherapy and retroperitoneal lymph node dissection (RPLND). Overall outcomes are decent.

    睾丸非精原细胞瘤绒毛膜癌预后总生存期

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