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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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    重症儿童高密度脂蛋白动态发展轨迹与临床结局的相关性研究

    付建垒张学鹏张庚熊怀钰...
    161-169页
    查看更多>>摘要:目的 分析重症儿童高密度脂蛋白(HDL)纵向、动态发展轨迹特征,并探讨不同轨迹与临床结局之间的关系。 方法 回顾性队列研究。回顾性纳入2015年1月1日至2020年10月1日收住四川大学华西医院儿科重症监护室(PICU)的患儿,利用组基轨迹模型(GBTM)分析入PICU后0~6 d的HDL动态发展轨迹,并建立不同HDL轨迹组,住院死亡率使用例数(%)描述,采用χ2检验或Fisher′s确切概率检验比较组间差异,PICU住院时间用M(Q1,Q3)描述,采用Kruskal Wallis统计检验比较组间差异。利用Logistic回归分析以及多元线性回归分析明确HDL轨迹组与临床结局之间的关系。主要临床结局指标为住院死亡率,次要结局指标为PICU住院时间。 结果 共纳入4 384例重症儿童,GBTM分析产生了6个HDL轨迹组。1组(758例),最低水平HDL组;2组(1 413例),较低水平HDL组;3组(74例),低-高水平HDL组;4组(621例),中等水平HDL组;5组(1 371例),较高水平HDL组;6组(147例),最高水平HDL组。Logistic回归分析,以1组作为参照组,2~6组的住院死亡率明显降低,其比值比(OR)及95%可信区间(CI)分别为OR:0。475,95%CI:0。352~0。641,P<0。001;OR:0。093,95%CI:0。013~0。679,P=0。019;OR:0。322,95%CI:0。208~0。479,P<0。001;OR:0。263,95%CI:0。185~0。374,P<0。001;OR:0。142,95%CI:0。044~0。454,P=0。001。利用多元线性回归分析,以1组为参照组,4~6组PICU住院时间明显缩短,β值及95%CI分别为β:-4。332,95%CI:-5。238~-3。426,P<0。001;β:-3。053,95%CI:-3。809~-2。297,P<0。001;β:-6。281,95%CI:-7。842~-4。721,P<0。001。 结论 PICU 0~6 d内HDL动态变化轨迹与死亡率具有相关性:持续低水平轨迹与高死亡率相关,而HDL持续高水平及由低水平逐渐上升至高水平的HDL轨迹呈现较低的死亡率。提示HDL轨迹建模有望成为预测重症患儿病情以及预后的指标。 Objective To characterize the longitudinal and dynamic high-density lipoprotein (HDL) trajectories in critically ill children and explore their correlation with clinical outcomes。 Methods Retrospective cohort study。All critically ill children admitted to the Pediatric Intensive Care Unit (PICU) of West China Hospital, Sichuan University from January 1, 2015 to October 1, 2020 were included in this retrospective study。Group-based trajectory modeling (GBTM) was applied to characterize the HDL trajectories in days 0-6 post-PICU admission and develop HDL trajectory groups。The in-hospital mortality rate was reported as frequency (%) and then compared by the Chi-square test or Fisher′s exact test between HDL trajectory groups。The length of stay (LOS) in the PICU was described by M(Q1, Q3), and its difference between HDL trajectory groups was evaluated by the Kruskal Wallis test。Logistic regression and multiple linear regression were used to determine the correlation between HDL trajectories and clinical outcomes。The primary outcome was in-hospital mortality rate, and the secondary outcome was LOS in the PICU。 Results A total of 4 384 critically ill children were ultimately enrolled in the study, and 6 HDL trajectory groups were developed based on GBTM analyses: group 1 (758 cases), the lowest HDL group group 2 (1 413 cases), the low HDL group group 3 (74 cases), the low-to-high HDL group group 4 (621 cases), the medium HDL group group 5 (1 371 cases), the high HDL group and group 6 (147 cases), the highest HDL group。Logistic regression analysis showed that compared with critically ill children in group 1, those belonging to groups 2, 3, 4, 5, and 6 were at lower risks of in-hospital mortality with odds ratio (OR): 0。475, 95%confidence interval (CI): 0。352-0。641, P<0。001 OR: 0。093, 95%CI: 0。013-0。679, P=0。019 OR: 0。322, 95%CI: 0。208-0。479, P<0。001 OR: 0。263, 95%CI: 0。185-0。374, P<0。001, andOR: 0。142, 95%CI: 0。044-0。454, P=0。001, respectively。Multiple linear regression analysis revealed that compared with critically ill children in group 1, those belonging to groups 4, 5, and 6 had the trend of shorter LOS in PICU, and the β value and 95%CI were β: -4。332, 95%CI: -5。238- -3。426, P<0。001 β: -3。053, 95%CI: -3。809--2。297, P<0。001 β: -6。281, 95%CI: -7。842--4。721, P<0。001, respectively。 Conclusions The dynamic HDL trajectories during 0-6 days after PICU admission are associated with in-hospital mortality rate of critically ill children。The HDL trajectory at a persistently low level is associated with higher mortality, while the HDL trajectory at a persistently high level or with the trend from a low level rising to a high level shows a lower risk of mortality。It is suggested that the HDL trajectory model may become an indicator to predict the condition and prognosis of critically ill children。

    高密度脂蛋白住院死亡率儿科重症监护室重症儿童组基轨迹模型

    培门冬酶诱发儿童急性胰腺炎的危险因素及其严重程度预测分析

    赖肖蓉余莉华黄露露林丹娜...
    170-175页
    查看更多>>摘要:目的 分析急性淋巴细胞白血病(ALL)儿童接受培门冬酶治疗后发生门冬酰胺酶相关胰腺炎(AAP)的高危因素,以及评估儿童序贯器官衰竭评估(SOFA)评分、儿童急性胰腺炎(PAPS)评分、Ranson′s评分及日本儿童量表(JPN)评分对AAP严重程度的预测价值。 方法 横断面研究。收集2014年1月至2021年8月在南方医科大学珠江医院小儿血液科接受培门冬酶治疗的328例ALL患儿的临床资料及AAP患儿的临床表现、实验室检查和影像学检查。计算确诊AAP时的儿童SOFA评分、确诊48 h时的PAPS评分和Ranson′s评分、确诊72 h时的儿童JPN评分,利用受试者工作特征曲线(ROC曲线)评估其对重症AAP的预测效能。 结果 AAP发生率为6。7%(22/328),中位年龄6。62岁,最常发生于诱导缓解阶段(16/22,72。7%),3例AAP患儿再次暴露于门冬酰胺酶,其中有2例发生了第2次AAP。22例AAP患儿中,轻症AAP 16例,重症AAP 6例,其中转儿童重症监护室(PICU)治疗6例,均为重症AAP。AAP组在性别、初诊白细胞数、ALL的免疫分型、危险分层及培门冬酶单次剂量方面与非AAP组比较差异均无统计学意义(均P>0。05),AAP组的ALL诊断年龄明显大于非AAP组(t=2。385,P=0。018),AAP组的超重或肥胖例数也多于非AAP组(χ2=4。507,P=0。034)。儿童JPN评分、儿童SOFA评分、Ranson′s评分和PAPS评分预测重症AAP的ROC曲线下面积(AUC)分别为0。919、0。844、0。731和0。606,儿童JPN评分(t=4。174,P=0。001)、儿童SOFA评分(t=3。181,P=0。005)在轻症与重症AAP组间差异有统计学意义。 结论 AAP是培门冬酶联合化疗治疗ALL过程中的严重并发症,年龄较大、超重或肥胖可能是AAP的高危因素,儿童JPN评分、儿童SOFA评分对重症AAP有预测价值。 Objective To analyze the risk factors for asparaginase-associated pancreatitis (AAP) in children with acute lymphoblastic leukemia (ALL) after treatment with pegaspargase and evaluate the predictive value of pediatric sequential organ failure assessment (SOFA) score, pediatric acute pancreatitis severity (PAPS) score, Ranson′s score and pediatric Ministry of Health, Labour and Welfare of Japan (JPN) score for severe AAP。 Methods Cross-sectional study。The clinical data of 328 children with ALL who received pegaspargase treatment in the Department of Pediatric Hematology, Zhujiang Hospital, Southern Medical University from January 2014 to August 2021, as well as their clinical manifestations, laboratory examinations, and imaging examinations were collected。The SOFA score at the time of AAP diagnosis, PAPS score and Ranson′s score at 48 hours after AAP diagnosis, and JPN score at 72 hours after AAP diagnosis were calculated, and their predictive value for severe AAP was evaluated by the receiver operating characteristic (ROC) curve。 Results A total of 6。7%(22/328) of children had AAP, with the median age of 6。62 years。AAP most commonly occurred in the induced remission phase (16/22, 72。7%)。 Three AAP children were re-exposed to asparaginase, and 2 of them developed a second AAP。Among the 22 AAP children, 16 presented with mild symptoms, and 6 with severe symptoms。The 6 children with severe AAP were all transferred to the Pediatric Intensive Care Unit (PICU)。 There were no significant differences in gender, white blood cell count at first diagnosis, immunophenotype, risk stratification, and single dose of pegaspargase between the AAP and non-AAP groups。The age at diagnosis of ALL in the AAP group was significantly higher than that in the non-AAP group (t=2。385, P=0。018)。 The number of overweight or obese children in the AAP group was also higher than that in the non-AAP group (χ2=4。507, P=0。034)。 The areas under the ROC curve of children′s JPN score, SOFA score, Ranson′s score, and PAPS score in predicting severe AAP were 0。919, 0。844, 0。731, and 0。606, respectively。The JPN score (t=4。174, P=0。001) and the SOFA score (t=3。181, P=0。005) showed statistically significant differences between mild and severe AAP。 Conclusions AAP is a serious complication in the treatment of ALL with combined pegaspargase and chemotherapy。Older age and overweight or obesity may be the risk factors for AAP。Pediatric JPN and SOFA scores have predictive value for severe AAP。

    培门冬酶门冬酰胺酶相关胰腺炎高危因素评分系统严重程度

    应用血液灌流与丙种球蛋白治疗儿童过敏性紫癜合并消化道出血单中心对照研究

    周鹏杨星光赵成广杜悦...
    176-181页
    查看更多>>摘要:目的 对比血液灌流(HP)与丙种球蛋白治疗儿童过敏性紫癜伴消化道出血的临床疗效及安全性。 方法 病例对照研究。对2015年1月至2019年12月中国医科大学附属盛京医院小儿肾脏风湿免疫科诊断过敏性紫癜及消化道出血的患儿39例进行回顾性分析。根据患儿是否行HP、丙种球蛋白治疗分为HP组和丙种球蛋白组,收集患儿临床资料,并对患儿进行6个月的随访调查,了解有无消化道出血复发及肾脏损伤。应用Fisher′s精确检验、两独立样本t检验、Mann-Whitney U检验、Kruskal-Wallis H检验、方差分析等方法比较组间差异。 结果 1.HP组20例,丙种球蛋白组19例,丙种球蛋白组年龄小于HP治疗组;2。2组患儿均有明显腹痛及消化道出血,部分患儿伴血管神经性水肿、肉眼血尿等;3。实验室指标比较:2组治疗前炎症指标[白细胞(WBC),C反应蛋白(CRP)]、凝血功能指标[纤维蛋白降解产物(FDP)、D-二聚体(DD)]明显升高,但2组间差异无统计学意义(P>0。05);2组患儿治疗后WBC、CRP、FDP、DD较治疗前下降,但2组间差异无统计学意义(P>0。05);4。临床表现比较:入院3 d内HP组腹痛缓解时间明显短于丙种球蛋白组[1。00(1。00,1。00) d比2。00(1。75,6。50)d,P=0。011];2组消化道出血停止时间差异无统计学意义(P>0。05);5。住院天数比较:3 d内应用HP与4~7 d、≥10 d应用组相比住院天数明显减少[(16。89±4。99) d比(19。20±2。39) d比(34。83±8。40) d,P<0。05],丙种球蛋白组组间及组内比较差异均无统计学意义(均P>0。05);6。住院费用比较:3 d内应用HP与4~7 d、≥10 d应用组相比费用明显减少[25 554。03(22 168。61,28 527。30)元比33 619。48(32 661。18,36 971。47)元比51 290。34(34 163。04,64 772。66)元,P<0。05],应用丙种球蛋白组组间及组内比较差异均无统计学意义(均P>0。05);7。激素用量比较:2组患儿初始激素剂量、丙种球蛋白/HP治疗前剂量、丙种球蛋白/HP治疗后剂量结果差异均无统计学意义(均P>0。05),2种治疗方法安全性也无差异,丙种球蛋白与HP组内激素剂量治疗后较治疗前减少,差异有统计学意义(P<0。001)。 结论 对于伴消化道出血的重症过敏性紫癜患儿,尽早采用血液净化治疗,可以快速缓解临床症状,减少住院天数及住院费用。对于无血液净化条件及不适宜应用血液净化的病例,应用丙种球蛋白治疗为另一种选择方法。 Objective To compare the clinical efficacy and safety of hemoperfusion (HP) and gammaglobulin on the treatment of Henoch-Schönlein purpura (HSP) with gastrointestinal bleeding in children。 Methods Case-control study。A total of 39 HSP children combined with gastrointestinal bleeding diagnosed in the Department of Pediatric Nephrology, Rheumatology and Immunology, Shengjing Hospital of China Medical University from January 2015 to December 2019 were retrospectively recruited。They were divided into the HP group and the gammaglobulin group according to the therapeutic strategy。Clinical data were collected, and a 6-month follow-up survey was conducted for monitoring the relapse of gastrointestinal bleeding and the occurrence of kidney injury。The differences between groups were compared by Fisher′s exact test, two independent samples t-test, Mann-Whitney U-test, Kruskal-Wallis H-test, and One-Way ANOVA。 Results (1) There were 20 cases in the HP group and 19 cases were included in the gammaglobulin group。The gammaglobulin group was younger than the HP treatment group。(2) In addition to gastrointestinal bleeding, children in both groups had other clinical symptoms, such as abdominal pain, angioneurotic edema, and hematuria。(3)Comparison of laboratory indexes: Inflammatory indexes: white blood cell count (WBC), C-creative protein (CRP) and coagulation function indexes: fibrin degradation products (FDP), D-dimer (DD) were significantly elevated before treatment in the 2 groups, and there was no difference between the 2 groups (P>0。05) WBC, CRP and FDP, DD declined in the 2 groups after treatment compared with the former, and there was no difference between the 2 groups (P>0。05) (4) Comparison of clinical manifestations: when HP was applied with gammaglobulin in the treatment window within 3 d, the difference in the time of abdominal pain relief in the HP group was shorter than that of the gammaglobulin group [1。00(1。00, 1。00) dvs。2。00(1。75, 6。50) d, P=0。011] comparing the time of gastrointestinal bleeding stopping when HP was applied with gammaglobulin comparison, the difference in gastrointestinal bleeding cessation time was not statistically significant (P>0。05) (5) Comparison of hospitalization time: within 3 d application of HP compared with other window period hospitalization time were significantly reduced [(16。89±4。99) d than (19。20±2。39) d than (34。83±8。40) d, bothP<0。05] (6) Comparison of hospitalization costs: within 3 d application of HP compared with other window period hospitalization costs were significantly reduced [25 554。03 (22 168。61, 28 527。30) yuan than 33 619。48 (32 661。18, 36 971。47) yuan than 51 290。34 (34 163。04, 64 772。66) yuan, bothP<0。05] There were no statistically significant difference in the hospitalization time and hospitalization cost between and within the gammaglobulin group (allP>0。05) (7) Comparison of hormone dosages: the difference in the results of the initial dose of hormone use, pre-treatment dose of gammaglobulin/HP, and post-treatment dose of gammaglobulin/HP between the two groups of children was not statistically significant(allP>0。05)。 Safety profile was comparable between groups。The difference in hormone dosage before and after treatment within the gammaglobulin and HP treatment group was statistically different (P<0。001)。 Conclusions For children with severe HSP accompanied by gastrointestinal bleeding, early treatment with blood purification can rapidly relieve clinical symptoms and reduce the number of hospital days and hospitalization costs。For cases where blood purification is not available or suitable, gammaglobulin treatment is another option。

    过敏性紫癜消化道出血血液灌流丙种球蛋白儿童

    间接法探索早产儿和足月儿先天性肾上腺皮质增生症的筛查临界值

    郭静田国力朱之星周卓...
    182-186页
    查看更多>>摘要:目的 分析间接法建立的先天性肾上腺皮质增生症(CAH)筛查指标血17-羟基孕酮(17-OHP)在早产儿与足月儿临界值的差异及可靠性。 方法 横断面研究。回顾性分析2019年1月至2022年12月在上海市儿童医院新生儿筛查中心接受CAH筛查的210 285例新生儿的资料,其中早产儿14 312例,足月儿195 973例。采用全自动荧光分析仪测定滤纸干血片上的17-OHP水平。分析早产儿和足月儿血17-OHP水平的分布情况及统计学指标。采用Kolmogorov-Smirnov检验进行正态性分布检验。通过Box-Cox将偏态分布数据转换为近似正态分布。用四分位间距(Turkey)法剔除离群值。采用R语言编程绘制累计频率分布图,并以单边第99。5百分位数值作为筛查临界值。将筛查临界值分别与厂商说明书给定的临界值和实验室临界值进行相对偏差比较。并与参考变化值(RCV)进行比较。 结果 据实验室现有临界值得出早产儿初筛阳性率为26。76‰,确诊4例,患病率为1∶3 578;足月儿初筛阳性率为0。79‰,确诊11例,患病率为1∶17 816。采用间接法建立CAH筛查临界值:早产儿为20。35 nmol/L,足月儿为10。78 nmol/L。该临界值与厂商说明书给定的临界值和实验室临界值的相对偏差均高于RCV。依据该筛查临界值,美国疾病预防控制中心室间质量评价计划的13批65份样本的阴性、阳性符合率均达100%;回顾性分析210 285例新生儿,确诊CAH阳性患儿17-OHP水平均高于此筛查临界值。应用此筛查临界值早产儿假阳性率降低59。79%。 结论 采用间接法分别建立早产儿、足月儿CAH筛查临界值,具有可行性,可提高筛查的工作效率,为临床提供更好的诊断依据。 Objective To analyze the difference and reliability of blood 17-hydroxyprogesterone (17-OHP), an indirect screening index for congenital adrenal hyperplasia (CAH), between preterm and full-term infants。 Methods In this retrospective cross-sectional study, a total of 210 285 newborns who underwent CAH screening at the Neonatal Screening Center of Shanghai Children′s Hospital from January 2019 to December 2022 were collected, including 14 312 premature infants and 195 973 full-term infants。The concentration of 17-OHP in dried blood spots on filter paper was determined by an automatic fluorescence analyzer。The distribution of 17-OHP levels in preterm and full-term infants and its statistical index were analyzed。The Kolmogorov-Smirnov test was used for normal distribution。The skewed distribution data was converted into approximately normal distribution using Box-Cox。Outliers were eliminated by the interquartile range method。The cumulative frequency distribution map was drawn by R language programming。The 99。5th percentile value was used as the screening threshold and compared with the reference value given by the manufacturer or laboratory and with the reference change value (RCV)。 Results According to the threshold provided by the laboratory, 26。76‰ of premature infants were tested positive in preliminary screening, and 4 were confirmed with an incidence of 1∶3 578, while 0。79‰ of full-term infants were tested positive in preliminary screening, and 11 were confirmed with an incidence of 1∶17 816。The thresholds for CAH screening established indirectly were 20。35 nmol/L in preterm infants and 10。78 nmol/L in full-term infants。The relative deviations between the indirect CAH screening thresholds and the manufacturer′s or laboratory′s CAH screening thresholds were higher than the RCV, respectively。According to the indirect CAH screening thresholds, the negative and positive coincidence rates of 65 samples in 13 batches from the Centers for Disease Control and Prevention interlaboratory quality assessment program in the United States reached 100%。A retrospective analysis of 210 285 neonates showed that 17-OHP concentration was higher than the screening threshold in all CAH-positive neonates。The application of this screening threshold reduced the false positive rate of preterm infants by 59。79%。 Conclusions It is feasible to establish the CAH screening thresholds for premature and full-term infants by an indirect method, which can improve the efficiency of screening and provide better diagnostic basis for clinical practice。

    肾上腺皮质增生症,先天性间接法临界值新生儿疾病筛查早产儿

    先天性低促性腺激素性性腺功能减退男童的临床及遗传学特征分析

    焦燕华张龙江苏喆潘丽丽...
    187-192页
    查看更多>>摘要:目的 分析先天性低促性腺激素性性腺功能减退(CHH)男童的临床及遗传学特征。 方法 横断面研究,回顾性分析。收集2019年12月至2023年2月在深圳市儿童医院内分泌专科就诊,最后基因确诊为CHH的27例男性患儿的临床资料、实验室数据及基因结果。分析其临床表现、激素水平及其基因变异情况。非正态分布采用中位数表示,非正态分布数据两组间比较采用秩和检验。 结果 基因确诊为CHH男性患儿共27例,初诊年龄为0。3~16。6岁,患儿均表现为小阴茎(占100%),合并隐睾16例(占59。3%),合并小睾丸9例(占33。3%),单纯性小阴茎7例(占25。9)%,无单纯性隐睾,3例患儿合并心脏异常;基础血清黄体生成素(LH)水平中位数为0。09 IU/L,92。5%(25/27)的患儿基础LH<1。00 IU/L,促性腺激素释放激素(GnRH)兴奋后LH峰值的中位数为1。42 IU/L,96。2%(26/27)的患儿LH峰值<4。00 IU/L;血清抑制素B的中位数为41。15 μg/L;血清抗苗勒管激素(AMH)的中位数为12。62 mg/L;合并隐睾的患儿血清AMH水平明显低于非隐睾患儿(10。02 mg/L比50。50 mg/L,P<0。05);27例患儿共检出12种基因变异,其中1例为双基因变异,最常见的基因变异为CHD7基因及ANOS1基因(各7例,二者共占51。8%),其次为FGFR1基因变异3例(占11。1%)。4例患儿短期给予GnRH泵或重组人卵泡刺激素皮下注射,治疗后血清抑制素B及AMH水平较治疗前明显增加,且睾丸容积较治疗前增大。 结论 CHH是先天性疾病,不同年龄临床表现不同,儿童期主要表现为小阴茎及隐睾,部分患儿存在小睾丸,青春前期确诊较困难,基因检测对早期诊断具有重要意义。 Objective To analyze the clinical and genetic characteristics of congenital hypogonadotropic hypogonadism (CHH) in boys。 Methods Cross-sectional study。Clinical data, laboratory data and genetic results of boys who were genetically diagnosed with CHH at the Department of Endocrinology of Shenzhen Children′s Hospital from December 2019 to February 2023 were collected in this retrospective study。Their clinical manifestations, hormone levels and gene mutations were analyzed。The non-normal distribution was represented by the median。The rank sum test was used to compare the non-normal distribution data between the two groups。 Results A total of 27 boys were genetically diagnosed with CHH, with the age at first diagnosis ranging from 0。3 to 16。6 years old。All these children presented with micropenis (100%), of whom 16 were complicated with cryptorchidism (59。3%), 9 with microrchidia (33。3%), 7 with simple micropenis (25。9%), and no had simple cryptorchidism。Three children had cardiovascular dysplasia。The median of basal luteinizing hormone(LH) level was 0。09 IU/L, and 92。5%(25/27) of children had the basal LH level below 1。00 IU/L。The median of peak LH level after gonadotropin-releasing hormone(GnRH) stimulation was 1。42 IU/L, and 96。2%(26/27) of children had the peak LH level below 4。00 IU/L。The median of serum inhibin B was 41。15 μg/L, and the median of serum anti-Müllerian hormone(AMH) was 12。62 mg/L。The serum AMH level of children with cryptorchidism was significantly lower than that of children without cryptorchidism (10。02 mg/L vs。50。50 mg/L, P<0。05)。 A total of 12 gene mutations were detected in the 27 children, of which 1 was biallelic mutation。The most common gene mutations were inCHD7 and ANOS1 genes (7 children each, both accounting for 51。8%), followed by FGFR1 gene (3 children, 11。1%)。 After short-term treatment by GnRH pump or subcutaneous injection of recombinant human follicle stimulating hormone in 4 children, the levels of serum inhibin B and AMH increased significantly, and the testicular volume also increased。 Conclusions CHH is a congenital disease with different clinical manifestations at different ages。The main manifestations in childhood are micropenis and cryptorchidism, and some children have microrchidia。Its diagnosis in prepuberty is difficult, but genetic testing is of great significance for early diagnosis。

    促性腺激素先天性低促性腺激素性性腺功能低下抑制素B抗苗勒管激素基因

    肉碱缺乏症性心肌病患儿血浆循环游离线粒体DNA定量分析及其临床意义

    聂抒林欣徐阳韩燕燕...
    193-197页
    查看更多>>摘要:目的 检测肉碱缺乏症性心肌病患儿血浆循环游离线粒体DNA(ccf-mtDNA)水平并探讨其临床意义。 方法 前瞻性病例对照研究。收集2017年7月至2022年6月吉林大学第一医院小儿心血管科病房收治的7例以心肌病为主要临床表现的原发性肉碱缺乏症(PCD)患儿(PCD组)、同期就诊的16例扩张型心肌病(DCM)患儿(DCM组)及50例健康体检儿童(健康对照组)血液样本,采用实时荧光定量PCR方法检测各组血浆ccf-mtDNA水平并比较其差异,分析PCD组中血浆ccf-mtDNA水平与血游离肉碱水平、心功能的相关性,并监测PCD组应用左旋肉碱治疗后ccf-mtDNA的水平变化。3组间比较采用Kruskal-Wallis检验,PCD组与健康对照组比较采用Mann-Whitney检验,PCD组内治疗前后比较采用配对Wilcoxon秩和检验,变量间相关分析采用Logistic回归分析。 结果 PCD组、DCM组患儿血浆ccf-mtDNA水平分别为3。69×106(1。09×106~7。26×106)拷贝/L、0。99×106(0。25×106~4。10×106)拷贝/L,明显高于健康对照组[0。09×106(0。01×106~0。35×106)拷贝/L](H=33。34、24。69,均P<0。001),且PCD组较DCM组更高(H=6。31,P<0。05)。PCD组应用左旋肉碱治疗前:血浆ccf-mtDNA水平与血游离肉碱水平、左心室射血分数呈负相关(r=-0。85、-0。82,均P<0。05),与改良Ross评分、N-末端B型利钠肽原水平呈正相关(r=0。81、0。83,均P<0。05)。PCD组应用左旋肉碱治疗后:血浆ccf-mtDNA水平逐渐下降,血游离肉碱水平、心脏功能逐渐恢复。血浆ccf-mtDNA水平自治疗第3个月[0。96×106(0。50×106~2。27×106)拷贝/L]开始,较治疗前明显下降(Z=2。24,P<0。05),在第6、9及12个月分别为0。27×106(0。18×106~0。76×106)拷贝/L、0。29×106(0。19×106~0。78×106)拷贝/L及0。16×106(0。10×106~1。06×106)拷贝/L,与健康对照组[0。09×106(0。01×106~0。35×106)拷贝/L]比较差异均无统计学意义(Z=1。23、1。09、2。12,均P>0。05)。 结论 血浆ccf-mtDNA可能是肉碱缺乏症性心肌病的致病因素之一,其水平对此疾病的心脏病情评估具有一定的临床价值。 Objective To detect the level of the circulating cell-free mitochondrial DNA (ccf-mtDNA) in plasma of children with primary carnitine deficiency (PCD)-associated cardiomyopathy and evaluate its clinical significance。 Methods In this prospective case-control study, peripheral blood samples were collected from 7 PCD patients with cardiomyopathy (PCD group), 16 dilated cardiomyopathy (DCM) patients (DCM group), and 50 healthy children (healthy control group) in the Pediatric Cardiovascular Department Ward of First Hospital of Jilin University from July 2017 to June 2022。The ccf-mtDNA levels were measured and compared between groups by the real-time fluorescence quantitative polymerase chain reaction。The correlations between plasma ccf-mtDNA level and blood free carnitine level and cardiac function in the PCD group were analyzed。The changes in the ccf-mtDNA level were monitored after L-carnitine treatment in the PCD group。The Kruskal-Wallis test was used for comparison among the three groups。The Mann-Whitney test was used for comparison between the PCD group and the control group。Changes before and after treatment in the PCD group were analyzed using the paired Wilcoxon rank sum test。The correlation between variables was evaluated by Logistic regression。 Results The plasma ccf-mtDNA levels in the PCD and DCM groups were 3。69×106 (1。09×106-7。26×106) copies/L and 0。99×106 (0。25×106-4。10×106) copies/L, respectively, which were significantly higher than that in the healthy control group[0。09×106 (0。01×106-0。35×106) copies/L](H=33。34, 24。69 all P<0。01)。 Besides, the plasma ccf-mtDNA level in the PCD group was higher than that in the DCM group (H=6。31, P<0。05)。 In the PCD group, the plasma ccf-mtDNA level was negatively correlated with the blood free carnitine level and left ventricular ejection fraction (r=-0。85, -0。82, all P<0。05) and positively correlated with the modified Ross score and the N-terminal pro B type natriuretic peptide level (r=0。81, 0。83, all P<0。05) before L-carnitine treatment。After treatment, the plasma ccf-mtDNA level decreased, and the blood free carnitine level and cardiac function recovered in the PCD group。The plasma ccf-mtDNA level declined sharply from the 3rd month[0。96×106(0。50×106-2。27×106) copies/L] after treatment (Z=2。24, P<0。05) and got to 0。27×106 (0。18×106-0。76×106) copies/L, 0。29×106(0。19×106-0。78×106) copies/L, and 0。16×106(0。10×106-1。06×106) copies/L at the 6th, 9th, and 12th months after treatment, respectively, with no statistically significant difference compared to the healthy control group[0。09×106(0。01×106-0。35×106) copies/L] (Z=1。23, 1。09, 2。12 all P>0。05)。 Conclusions Plasma ccf-mtDNA may act as one pathogenic factor of cardiomyopathy in PCD, and monitoring its level is clinically important for heart condition assessment in PCD。

    原发性肉碱缺乏症血浆循环游离线粒体DNA心肌病

    应用机器学习分析吡仑帕奈单药治疗儿童局灶性癫痫临床疗效的影响因素

    易珈沁汪洋孙丹申玉美...
    198-202页
    查看更多>>摘要:目的 分析吡仑帕奈单药治疗儿童局灶性癫痫临床疗效的影响因素,促进临床合理用药。 方法 应用回顾性研究方法分析2021年6月至2022年8月在华中科技大学同济医学院附属武汉儿童医院确诊为癫痫局灶性发作的患儿资料。以吡仑帕奈单药治疗第3、6、12个月疗效为因变量,患儿性别、起病年龄、吡仑帕奈单药治疗的初始年龄、病程、体重、体重指数(BMI)、发作频率、癫痫综合征、病因、既往史、发育迟滞、起始剂量及维持剂量、血药浓度、CYP3A4、CYP3A5基因突变情况等因素为自变量,采用Cox比例风险回归分析各项因素对吡仑帕奈单药治疗疗效的影响;采用机器学习随机森林模型及决策树模型分析影响疗效因素的重要性序列并寻找最佳分类界值切点。 结果 本研究共纳入43例患儿(男31例,女12例),起病年龄为(7。6±2。1)岁,开始使用吡仑帕奈单药治疗年龄为(7。8±2。7)岁。Cox比例风险回归分析提示患儿BMI数值越大,吡仑帕奈治疗局灶性癫痫的疗效越差(HR=0。74,95%CI:0。55~0。99,P=0。045),且通过机器学习发现患儿的BMI是影响临床疗效的显著性协变量,当BMI≥21。8 kg/m2时负向影响疗效更显著。 结论 吡仑帕奈单药治疗儿童局灶性癫痫的临床疗效与患儿的BMI值相关,单药治疗肥胖癫痫患儿可能需适当增加吡仑帕奈剂量以控制发作。 Objective To analyze influencing factors for the clinical efficacy of Perampanel monotherapy in children with focal epilepsy and promote rational clinical use of Perampanel。 Methods Children who were diagnosed with focal epilepsy and treated with Perampanel monotherapy in Wuhan Children′s Hospital, Tongji Medical College, Huazhong University of Science & Technology from June 2021 to August 2022 were included in this retrospective study。Efficacy at 3, 6 and 12 months after treatment was taken as the dependent variable。The gender, age at onset, age at initiation of Perampanel monotherapy, course of disease, weight, body mass index (BMI), seizure frequency, epilepsy syndrome, etiology, previous clinical history, developmental retardation, starting and maintenance doses, blood concentration, CYP3A4 and CYP3A5 gene mutations were taken as the independent variables。The effects of these factors on the efficacy of Perampanel monotherapy were analyzed using the Cox proportional hazards regression。The random forest and decision tree models were used to sequence the significance of the influencing factors and find the optimal cut-point for classification。 Results A total of 43 children (31 boys and 12 girls) were enrolled in this study。The average age at onset was (7。6±2。1) years, and the average age at initiation of Perampanel monotherapy was (7。8±2。7) years。The Cox proportional hazards regression analysis indicated that the greater the BMI, the worse the efficacy of Perampanel (HR=0。74, 95%CI: 0。55-0。99, P=0。045)。 Through machine learning, the BMI was found to be a significant covariate affecting the efficacy, and when BMI≥21。8 kg/m2, the negative effect was more significant。 Conclusions The clinical efficacy of Perampanel monotherapy in children with focal epilepsy is related to BMI。The dose of Perampanel for obese children with epilepsy may need to appropriately increase。

    吡仑帕奈局灶性癫痫单药治疗疗效影响因素

    狼疮性肾炎伴血栓性微血管病变的临床和病理特征及预后分析

    刘海鹏刘小微赵英荣朱峰...
    203-208页
    查看更多>>摘要:目的 探讨儿童狼疮性肾炎(LN)合并血栓性微血管病(TMA)的临床和病理特征及预后。 方法 病例对照研究。回顾性分析2008年1月至2023年1月在徐州医科大学附属徐州市儿童医院经肾活检明确诊断为LN患儿的临床和病理资料,其中46例LN患儿合并TMA(LN-TMA组)。根据年龄、性别、和病理类型进行匹配,选取92例(1∶2)无TMA的LN患儿作为对照组(LN组)。采用Kaplan-Meier法评估LN患儿的总体和肾脏存活率,采用Cox回归模型分析进展为终末期肾脏病(ESRD)的风险因素。 结果 TMA与血肌酐(Scr)、血清C3、抗C1q抗体(a-C1q)、估算肾小球滤过滤(eGFR)、毛细血管内增生、纤维素样坏死和肾组织C1q沉积呈中度相关(均r>0。5)。血清a-C1q≥20 U/mL(HR=8。724,95%CI:0。976~16。114,P=0。026)和eGFR≤60 mL/(min·1。73 m2)(HR=12。213,95%CI:1。147~25。048,P=0。038)是LN儿童发生TMA的独立危险因素。肾小球硬化(HR=7。228,95%CI:0。186~22。358,P=0。016)、TMA(HR=11。387,95%CI:3。426~42。554,P=0。009)和eGFR≤60 mL/(min·1。73 m2)(HR=3。116,95%CI:0。592~10。064,P=0。030)是LN患儿进展为ESRD的独立危险因素。LN-TMA组5年和10年肾脏生存率低于LN组(97。44%比98。28%,80。90%比87。27%)(χ2=4。918,P=0。027)。 结论 LN-TMA患儿病情危重,预后差。TMA是LN儿童进展为ESRD的独立危险因素,其发生机制可能与补体激活相关。 Objective To investigate the clinical and pathological characteristics and prognosis of children with lupus nephritis(LN)and thrombotic microangiopathy (TMA)。 Methods In this retrospective case-control study, clinical and pathological data of LN children confirmed by renal biopsy from January 2008 to January 2023 in Xuzhou Children′s Hospital, Xuzhou Medical University were analyzed。There were 46 LN children complicated with TMA (LN-TMA group)。 With matched age, sex and pathology, 92 LN children (1∶2) without TMA were selected as the control group (LN group)。 The Kaplan-Meier method was used to evaluate the overall and renal survival rates of children with LN, and the Cox regression model was used to analyze the risk factors for the progression to end-stage renal disease (ESRD)。 Results TMA was moderately associated with serum creatinine, serum C3, anti-C1q antibody (a-C1q), estimated glomerular filtration rate (eGFR), endocapillary proliferation, fibrinoid necrosis, and renal C1q deposition (all r>0。5)。 Serum a-C1q≥20 U/mL (HR=8。724, 95%CI: 0。976-16。114, P=0。026) and eGFR≤60 mL/(min·1。73 m2) (HR=12。213, 95%CI: 1。147-25。048, P=0。038) were independent risk factors for TMA in children with LN。Glomerular sclerosis (HR=7。228, 95%CI: 0。186-22。358, P=0。016), TMA (HR=11。387, 95%CI: 3。426-42。554, P=0。009) and eGFR≤60 mL/(min·1。73 m2) (HR=3。116, 95%CI: 0。592-10。064, P=0。030) were independent risk factors for developing ESRD in LN children。The 5-year and 10-year renal survival rates in the LN-TMA group were lower than those in the LN group (97。44%vs。98。28%, 80。90%vs。87。27%, χ2=4。918, P=0。027)。 Conclusions Children with LN-TMA present with severe symptoms and poor prognosis。TMA is an independent risk factor for progression to ESRD in children with LN, and the mechanism may be related to complement activation。

    狼疮性肾炎血栓性微血管病儿童补体激活

    单中心先天性胆总管囊肿根治术术后早期并发症影响因素分析及治疗体会

    杜青金祝郑泽兵黄露...
    209-214页
    查看更多>>摘要:目的 探讨单中心先天性胆总管囊肿(CCC)根治术术后早期并发症影响因素,为减少术后并发症提供一定临床依据及指导。 方法 病例对照研究。分析遵义医科大学附属医院2010年9月至2019年10月诊断明确并已行根治术的124例CCC患儿的临床资料,男29例,女95例,根据术后是否发生并发症(胆漏、肠肠吻合口瘘、出血、切口裂开、胆管炎、腹腔感染、胰腺炎、淋巴瘘)分为并发症组(A组)和无并发症组(B组),对2组患儿的年龄、实验室指标(术前白细胞计数、血红蛋白、丙氨酸转氨酶、前白蛋白及术后白蛋白)以及2组患儿手术方式、手术时间、术中失血量、囊肿类型、囊肿直径、肝总管直径,是否合并腹部手术史、胆泥结石、肝胆管解剖变异、胆胰合流等相关临床因素进行统计分析,并对其中的计量资料符合正态分布的行t检验,非正态分布行非参数秩和检验,多因素分析采用Logistic回归分析。 结果 124例手术病例发生并发症25例(20。16%),未发生并发症99例(79。84%),其中胆漏14例(11。29%),7例再次手术,7例保守治愈;肠肠吻合口瘘2例(1。61%),1例再次手术,1例保守治愈;出血1例(0。81%),再次手术治愈;切口裂开2例(1。61%),1例再次手术,1例保守治愈;胆管炎2例(1。61%)、腹腔感染2例(1。61%)、胰腺炎1例(0。81%)、淋巴瘘1例(0。81%)均保守治愈。其中非正态分布指标:2组年龄及白细胞计数比较,差异均无统计学意义(均P>0。05);2组失血量及囊肿直径比较,差异均有统计学意义(均P<0。05);符合正态分布指标的术后白蛋白(27。84±4。62) g/L比(32。45±3。72) g/L,2组比较差异有统计学意义(t=5。254,P<0。05)。Logistic多因素回归分析:术前贫血(OR=7。922,95%CI:1。468~42。757)、合并胆泥结石(OR=1。295,95%CI:1。075~4。359)是术后并发症发生的独立危险因素,术后白蛋白(OR=0。055,95%CI:0。012~0。244)是术后并发症发生的保护因素,差异均有统计学意义(均P<0。05)。 结论 胆总管囊肿直径越大、术中出血量越多,越增加手术风险。术前纠正贫血、术中精细操作清理肝胆管淤泥、减少出血及加强围术期白蛋白、血红蛋白的监测,可预防及减少CCC根治术后早期并发症发生。 Objective To explore the influencing factors of early postoperative complications after radical resection of congenital choledochal cyst (CCC) in a single center and provide some clinical basis and guidance for reducing postoperative complications。 Methods Case control study。Clinical data of 124 children (29 boys and 95 girls) with CCC diagnosed and radically treated at the Affiliated Hospital of Zunyi Medical University from September 2010 to October 2019 were analyzed。According to postoperative complications (bile leakage, gastrointestinal anastomotic fistula, bleeding, incision dehiscence, cholangitis, abdominal infection, pancreatitis, and lymphatic fistula), these children were divided into the complication group (group A) and non-complication group (group B)。 Age, laboratory indicators[preoperative white blood cell (WBC) count, hemoglobin, glutamic pyruvic transaminase, prealbumin, and postoperative albumin], and clinical factors, such as operation method, operation time, intraoperative blood loss, cyst type, cyst diameter, hepatic duct diameter, abdominal operation history, biliary sludge and calculus, hepatic duct anatomic variation, and pancreaticobiliary maljunction were statistically analyzed between the two groups。The t-test was performed for normal distribution of the measurement data, and the non-parametric rank sum test for non-normal distribution。Multivariate analysis was made using Logistic regression。 Results Among the 124 children, 25(20。16%) had complications, and 99(79。84%) had no complications。Bile leakage occurred in 14 children (11。29%), of whom 7 received operation again and 7 received conservative treatment。Gastrointestinal anastomotic fistula occurred in 2 children (1。61%), of whom 1 was re-operated and 1 was cured conservatively。One child (0。81%) was complicated with bleeding and cured by re-operation。Two children (1。61%) were complicated with incision dehiscence, of whom 1 was cured by re-operation and 1 was cured by conservative treatment。Cholangitis in 2 children (1。61%), abdominal infection in 2 children (1。61%), pancreatitis in 1 child (0。81%), and lymphatic fistula in 1 child (0。81%) were all conservatively cured。No significant difference was found in non-normal distribution indicators-age and WBC count-between the two groups (all P>0。05)。 Blood loss volume and cyst diameter were significantly different between the two groups (allP<0。05)。 Postoperative albumin[(27。84±4。62) g/Lvs。(32。45±3。72) g/L] meeting the normal distribution showed a statistically significant difference between the two groups (t=5。254, P<0。05)。 Logistic multivariate regression analysis suggested that preoperative anemia (OR=7。922, 95%CI: 1。468-42。757) and biliary sludge and calculus (OR=1。295, 95%CI: 1。075-4。359) were independent risk factors for postoperative complications postoperative albumin (OR=0。055, 95%CI: 0。012-0。244) was a protective factor for postoperative complications, and the differences were statistically significant (all P<0。05)。 Conclusions The larger the cyst diameter, the more the intraoperative bleeding, and the higher the risk of operation。Treating anemia before operation, clearing sludge in the hepatic duct during operation, reducing bleeding, and strengthening the monitoring of albumin and hemoglobin during the perioperative period can prevent and reduce early complications after radical resection of CCC in children。

    先天性胆总管囊肿外科手术并发症影响因素治疗体会

    输尿管梗阻对新生大鼠肾盂功能及起搏细胞的影响

    任川川程光阳李晓洁王焱...
    215-220页
    查看更多>>摘要:目的 探讨新生大鼠单侧输尿管梗阻对肾盂蠕动功能及起搏细胞的影响。 方法 动物实验研究。2日龄新生SD大鼠36只,根据随机数字表法分为单侧输尿管部分梗阻(PUUO)组、单侧输尿管完全梗阻(CUUO)组和假手术组,每组12只。术后1周进行肾盂穿刺测压,测压后处死幼鼠,取左侧肾盂输尿管固定后用于组织学检测。记录并比较各组幼鼠不同灌注速度时肾盂压力、蠕动波频率、波幅等参数,同时比较各组肾盂起搏细胞(非典型平滑肌细胞和Cajal样间质细胞)的变化情况。2组间比较采用独立样本t检验,3组比较采用单因素方差分析。 结果 假手术组肾盂压力随灌注速度增加而缓慢增加;蠕动频率随灌注速度增加而快速升高,达最大值后随灌注速度增加而降低。蠕动波振幅随灌注速度增加呈现先升高后降低。PUUO组肾盂压力随灌注速度增加而快速增加,且均高于假手术组;蠕动频率较假手术组高,在40 mL/h之前的蠕动频率较为接近,但灌注速度再增加时,蠕动波频率开始下降。蠕动波振幅随灌注速度增加出现快速升高而后下降,且下降速度较假手术组快。CUUO组肾盂基础压力为12 cmH2O(1 cmH2O=0。098 kPa),但在5 mL/h的灌注速度下,肾盂压力逐渐升高,未出现平台期,直至肾盂压力达73 cmH2O时,灌注液自穿刺针旁逆行流出,肾盂压力稍下降后达到平衡,整个过程未出现规律肾盂蠕动波。3组肾盂组织免疫荧光染色分析显示,起搏细胞均位于肾盂壁的平滑肌中,假手术组阳性率最高,PUUO组次之,CUUO组最低。 结论 输尿管梗阻对肾盂蠕动功能有显著影响,其对蠕动频率、幅度及肾盂压力等产生的影响有一定规律可循;肾盂起搏细胞减少可能参与输尿管梗阻导致的肾盂蠕动功能改变,但起搏细胞如何调节肾盂输尿管蠕动仍需进一步研究。 Objective To investigate the effects of unilateral ureteral obstruction on renal pelvic peristalsis and pacemaker cells in neonatal rats。 Methods An animal experimental study。Thirty-six 2-day-old newborn SD rats were randomly divided into the partial unilateral ureteral obstruction (PUUO) group, complete unilateral ureteral obstruction (CUUO) group, and sham operation group, with 12 rats in each group。One week after surgery, all rats were subjected to renal pelvic pressure (RPP) measurement by puncture。After measurement, the rats were euthanized, and their left renal pelvis and ureter were removed and fixed for histological examination。Parameters such as RPP, peristaltic wave frequency and amplitude at different perfusion speeds were recorded and compared, and the changes in pacemaker cells (atypical smooth muscle cells and Cajal-like interstitial cells) were also compared。The independent samples t-test was used for comparison between 2 groups, and the one-way ANOVA of variance was used for comparison among 3 groups。 Results In the sham operation group, the RPP increased gradually with the increase of perfusion speed the frequency of peristaltic waves rose rapidly and then dropped after reaching the highest level with the increase of perfusion speed similarly, the amplitude of peristaltic waves first increased and then decreased as the perfusion speed increased。In the PUUO group, the RPP increased rapidly with the increase of perfusion speed, higher than that in the sham operation group the frequency of peristaltic waves was higher than that in the sham operation group, and it was relatively constant under the perfusion speed of 40 mL/h, but when the perfusion speed increased again, the frequency began to decline the amplitude of peristaltic waves increased quickly and then declined at a faster rate than the sham operation group with the increase of perfusion speed。In the CUUO group, the basic RPP was 12 cmH2O(1 cmH2O=0。098 kPa) at the perfusion speed of 5 mL/h, the RPP rose gradually, and no plateau appeared when the RPP reached 73 cmH2O, the perfusate retrograded from the side of the puncture needle, then the RPP slightly decreased and then balanced, and no regular peristaltic waves were observed in the renal pelvis throughout the whole perfusion process。Immunofluorescence staining analysis showed the pacemaker cells were all located in the smooth muscle of the renal pelvic wall。The sham operation group had the highest positive rate, followed by the PUUO group and then the CUUO group。 Conclusions Ureteral obstruction has a significant impact on the peristalsis of the renal pelvis, and its impact on the peristaltic wave frequency and amplitude and RPP can be predicted。The reduction of pacemaker cells in the renal pelvis may be involved in the changes of renal pelvic peristalsis caused by ureteral obstruction, but further research is needed on how pacemaker cells regulate the peristalsis of the renal pelvis and ureter。

    输尿管梗阻Cajal样间质细胞大鼠,新生肾盂蠕动功能