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??Abstract??Objecive??To compare the efficacy and safety of 10-day sequential treatment??including rabeprazole?? amoxicillin and clavulanate for oral suspension??clarithromycin?? and 7-day traditional trigeminy therapy ??including omeprazole?? amoxicillin and clarithromycin?? for the eradication of helicobacter pylori??HP??in children. Methods??From Oct.2006 to Aug.2009??123 children with gastropathy with Hp infection??in Luoyang Locomotive Hospital of Henan??were divided into two groups.The control group ??41?? was treated with traditional trigeminy therapy for 7 days??while the treatment group??82??was treated with 10-day sequential treatment.Cure rates of the ulcer healing and Hp eradication were assessed after treatment course of 7-10 days??while the symptom scores and the side effect were evaluated and investigated after the treatment. Results The healing rate of ulcer in control group was 40%??2/5????which was 55.5%??5/9?? in the treatment group??the difference being statistically significant??P??0.05??. The Hp eradication rate was 92.68%??76/82??and 70.73%??29/41??in treatment group and control group?? respectively .There was significant statistical difference??P??0.05??. The total symptom score of treatment group before treatment was??56.89±4.32???? and after treatment it was ??14.96±2.13????while that of control group before treatment was ??58.75 ± 5.12?? and ??21.42 ± 3.78?? after treatment??P??0.05 or P??0.01????the change of treatment group being more significant??P??0.01??. The side effect rate of treatment group was lower than that of control group ??6.1% VS 17.1%??respectirely??P??0.05??. Conclusion??The 10-day sequential treatment??including rabeprazole??amoxicillin and clavulanate for oral suspension and clarithromycin??is much more efficient with lower side effect than 7-day traditional trigeminy therapy for Hp eradication in children with gastropathy with Hp infection.     

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目的探讨小儿异位胃黏膜症合并急腹症的临床特点。方法回顾分析了2000-12—2005-12中国医科大学第二临床学院收治的55例经病理证实存在异位胃黏膜的急腹症患儿,对其临床资料进行分类汇总和分析。结果男44例,女11例;年龄3个月至13岁,平均4·5岁。临床诊断下消化道出血31例,急性肠套叠13例,急性肠梗阻5例,急性腹膜炎6例;手术切除肠管标本经病理检查证实均有异位胃黏膜。其中Meckel憩室40例,肠重复畸形11例,肠壁正常4例。结论异位胃黏膜可能是小儿急腹症的病因之一;急腹症患儿ECT检查阴性也应开腹探查,以免遗漏异位胃黏膜。     

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??Objective??To detect pathogenic genes of short stature with unknown etiology by a targeted next generation sequencing panel to analyze the correlation between genotypes and clinical phenotypes. Methods??A total of 77 children diagnosed with unexplained short stature were enrolled for the study. These children were treated in Ruijin Hospital of Shanghai Jiao Tong University from 2007 to 2015. To search for genetic variation in 187 candidate genes which were associated with growth?? we constructed a targeted next generation sequencing panel encompassing the coding regions of 187 genes. According to ACMG Guidelines??the sites of variation were determined. Sanger sequencing was used to verify the suspected pathogenic genes variation. The relationship between genotype and clinical phenotype was analyzed. Results??Including 5 pathogenic variants?? one likely pathogenic variant and one variant of uncertain significance?? we identified 7 heterozygous variants of 7 cases in 77 cases of short stature with unknown etiology. A pathogenic variant p.D2407fs of ACAN gene was found in a case with advanced bone age. There were 3 reported pathogenic variants?? including p.A72G?? p.I282V and p.P491S of PTPN11 gene?? which were diagnosed as Noonan syndrome. A case carrying known pathogenic variant COL2A1??p.R904C?? was diagnosed as Stickler syndrome. We still got one likely pathogenic variant COMP??p.D401N???? which could cause multiple epiphyseal dysplasia. There was a familial short stature of delayed bone age carrying a variant??p.S289Y?? of uncertain significance??in which the genotype was in accordance with the clinical phenotype. Conclusion??The ACAN gene defection is associated with the idiopathic short stature with advanced bone age. The likely pathogenic variant COMP??p.D401N?? may cause multiple epiphyseal dysplasia. The newly-found heterozygous varians??p.S289Y?? of GHSR gene may result in short stature??which needs further function verification.     

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??Objective To explore the epidemiological features of NV and RV infection and to investigate the clinical features and prognosis of NV infection. Methods Stool specimens??without pus??mucus or blood?? were collected from children less than 3 years old with acute diarrhea within 7 days admitted from November 2012 to October 2013 in Beijing Children’s Hospital??Capital Medical University. Stool specimens were screened for NV and RV using immunochromatographic test??ICG??. History was investigated and the severity was evaluated by Vesikari scale. Follow-ups were conducted via phone calls. The epidemiological features?? clinical manifestations and prognosis of children infected with NV were analyzed and compared with those infected with RV. Results Among the total 942 samples??RV was detected in 329??34.9%?? samples??while NV was detected in 137??14.5%?? samples. Rate of co-infection was 9.7%??91/942??. The seasonal distribution of RV detection showed a peak of incidence from September to February the following year??but NV detection showed sporadic distribution. The times and duration of diarrhea and vomiting were similar in two kinds of infection??but fever??vomiting??severe diarrhea??with fatigue??sleepiness or lethargy?? were significantly more common in RV infection??P??0.05??. NV infection had a higher incidence of respiratory symptoms??such as cough and rhinorrhea. Mean Vesikari severity score was ??8.98±2.94?? in NV infection??remarkably lower than??10.45±3.04??in RV infection??P??0.05??. Most patients recovered in??7 days??but NV infection was likely to have longer duration. Conclusion RV is still the leading cause of acute viral diarrhea??and NV is also a significant cause. NV infection occurs year-round??while RV infection shows a peak in autumn and winter. Fever??vomiting and severe diarrhea with dehydration are more common in RV infection. NV infection has a higher rate of respiratory symptoms and longer recovery period.     

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??Objective??To analyze the clinical characteristics??pathological features and treatment responses of 6 pediatric patients with C3 glomerulonephritis??C3GN????in order to improve the understanding and treatment of this disease for pediatricians. Methods??Analyze the clinical manifestations??pathological features??therapies??prognosis of patients who were diagnosed with C3 glomerulonephritis from September??2010 to June??2016 retrospectively. Results??Clinical characteristics and laboratory examination??2 patients’ first symptom was hematuria??4 patients’ first symptom was hematuria and proteinuria??3 patients presented as acute nephritic syndrome??one presented as nephrotic syndrome. All the patients showed that the level of serum complement C3 was reduced??while sernm complement C4 was normal. Pathological character??6 patients showed strong positive complement C3 deposition under immunofluorescence. Lightmicroscopy showed mesangial proliferative glomerulonephritis in 5 cases??1 case was diagnosed as endocapillary proliferative glomerulonephritisin??and 3 patients presented electrondense depositionin under electron microscope. Treatment and prognosis??after conventional treatment??2 patients who were with crescent were treated with glucocorticoid .After a follow-up from 6 months to 42 months ??the prognosis was pretty good. Conclusion??Children with C3GN are usually presented with hematuria and ??or??proteinuria??characterized by strong positive C3 deposition. Lightmicroscopy always shows mesangial proliferative glomerulonephritis. Electron microscope show electron dense deposition??and short-term prognosis is pretty good.     

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目的 通过比较小于胎龄儿(SGA)和足月正常新生儿脐血Ghrelin水平的变化,分析脐血Ghrelin与新生儿出生体重、身长、头围、BMI和胎盘重量、胎盘体积的关系,以及脐血Ghrelin水平与IGF-1、胰岛素、胰岛素敏感性和血糖的关系.探讨脐血Ghrelin水平与胎儿发育和代谢轴之间,尤其是Ghrelin与IGF-1的相互关系.方法 2006年12月至2007年3月选择中山大学附属一院新生儿78例,SGA组22例为观察组,足月适于胎龄儿(AGA)42例为正常对照组.采用酶联免疫反应法(ELISA)对新生儿脐血Ghrelin、胰岛素、IGF-1进行测定分析,测量新生儿净体重、身长、头围、胎盘体积、胎盘重量,计算体重指数(BMI)和胰岛素敏感指数(ISI).结果 (1)SGA组脐血Ghrelin水平(38.74±4.90 ng/mL)明显高于正常对照组(23.37±8.78 ng/mL),其差异有统计学意义(P＜0.05).(2)脐血Ghrelin水平与新生儿出生体重、身长、头围、BMI、胎盘重量、胎盘体积呈负相关,均有统计学意义(P＜0.05).(3)脐血Ghrelin水平与IGF-1、胰岛素、ISI呈负相关均有统计学意义(P＜0.05).与血糖无相关性(r=0.081,P=0.454).(4)通过偏相关分析,控制出生体重、身长、胎盘重量、BMI、胰岛素后,Ghrelin与IGF-1有明显负相关性,有统计学意义.(5)剖宫产新生儿脐血Ghrelin水平与顺产儿无明显差异(P=0.956);男性与女性新生儿脐血Ghrelin水平无明显差异(P=0.385).结论 新生儿脐血Ghrelin水平是反映胎儿的宫内发育及营养状态的指标之一.并以负反馈形式参与胎儿宫内发育和代谢轴的调控过程.小于胎龄儿出生时可能存在胰岛素抵抗.小于胎龄儿高Ghrelin并没有刺激胰岛素释放,提示小于胎龄儿可能存在Ghrelin受体或受体后信号传导通路的抵抗.     

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??Objective??To investigate the value of serum intestinal fatty acid binding protein??I-FABP?? and serum amyloid A??SAA?? in the diagnosis of necrotizing enterocolitis??NEC??in the newborn. Methods??Fifty-six preterm infants with a confirmed diagnosis of NEC from October 2014 to October 2015 were recruited as case group??stage??26 cases??stage??/??30 cases??. Thirty children diagnosed with non-digestive diseases in the same period were recruited as the control group. Serum levels of I-FABP and SAA were determined by enzyme-linked immunosorbent assay.The diagnostic value of I-FABP and SAA for severe NEC was assessed using the receiver operating characteristic??ROC??curve. Results??Stage??/?? cases in the case group had significantly higher serum I-FABP levels and SAA levels than the control group and Stage??cases??P??0.05??. The area under the ROC curve for serum I-FABP was 0.80??95%CI??0.69-0.92????with the optimal cut-off point of 21.8 μg/L. Under this cut-off point??the sensitivity and specificity were 70.0%and 81.0%??respectively. The area under the ROC curve for SAA was 0.76??95%CI??0.63-0.89????with the optimal cut-off point of 1657.8 μg/L. Under this cut-off point??the sensitivity and specificity were 67.0% and 80.0%??respectively. Conclusion??In newborn infants with NEC??serum I-FABP and SAA l can be used as biomarkers for the diagnosis of severe NEC.     

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??Abstract??Objective To study the effectiveness of the antiepileptic lamotrigine ??LTG?? and the relationship between the plasma concentration and oral dosage?? co-medication and patients age. Methods Totally 111 epileptic patients were divided into 2 groups?? LTG monotherapy group ??n=58?? and LTG??VPA ??Valproate??VPA?? group ??n=53??. LTG??VPA group was divided into three subunits in terms of plasma concentration of VPA. LTG monotherapy group was divided into two subunits?? older ??7??18 y?? n=40?? and younger ??3????7 y?? n=18?? group. The dosage was then increased to target dose gradually. Blood specimens were collected more than one month after LTG target dose or when effective maintenance dosage was achieved. The serum concentrations of LTG and VPA were determined by high performance liquid chromatography. Results The effect of the LTG was 82.9%.VPA combination enhanced LTG blood concentration. The ratio of serum level and dose of LTG did not vary with the range of the serum concentration of VPA 0.28??0.62mol/L.In younger group the relativity between blood concentration of LTG and LTG maintaining dose didn’t exist. However?? there was a positive correlation in the older group. Conclusions When we prescribe LTG??we should consider epilepsy type?? co-medication and individual difference as a whole.Therefore TDM??therapeutic drug monitoring??TDM??is an essential tool to control seizure and avoid side effects.     

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??Abstract??Objective To investigate the clinical efficacy and safety of tacrolimus ??FK506?? therapy for children with steroid-dependent and steroid-resistant nephrotic syndrome ??NS??. Methods From Jun.2007 to Dec.2011 in Guangzhou Medical College Guangzhou First Municipal People's Hospital??42 children with primary nephrotic syndrome were enrolled??in which 32 cases were steroid-dependent NS ??SDNS?? and 10 cases were steroid-resistant NS ??the SRNS??. The indicators were tested before treatment and 12 weeks later??including ??1?? the 24h urinary protein excretion??blood urea nitrogen ??BUN????serum creatinine ??Scr????plasma albumin ??Alb????blood and urine ??2-microglobulin ????2-MG??????2?? lipid testing indicators??total cholesterol ??TC????triglyceride ??TG??????3??hypercoagulable state indicators??the prothrombin time ??PT????activated partial thromboplastin time ??APTT ????the plasma concentration of fibrinogen ??Fib?? and blood D-dimer ??D-dimer??. Results Tacrolimus combined with steroid therapy had significant effects.The clinical and biochemical indicators improved significantly after treatment ??P??0.05 or P??0.01????lipids and hypercoagulability-related indicators also improved significantly??proteinuria significantly reduced.Complete remission was achieved in 29 cases??partial remission in 10 cases??no remission in 4 cases.Eleven cases of simple type NS got complete remission??and 18 cases of nephritis type NS got complete remission??partial remission in 9 cases??no remission in 4 cases.Pathological type??5 cases of minimal change disease??complete remission????5 cases of focal segmental glomerulosclerosis??3 no remission??2 partial remission????six cases of mesangial proliferative glomerulonephritis??4 complete remission??2 partial remission??.Mild gastrointestinal reaction occurred in 4 cases and diarrhea in 2 cases.Conclusion The tacrolimus and steroid combination therapy for children with steroid-dependent and steroid-resistant NS can reduce proteinuria??reduce high cholesterol and improve the hypercoagulable state??the majority of children get complete remission??steroid-resistant nephrotic syndrome also has a significant improvement in the treatment.This combined treatment is safe and effective with fewer adverse reactions.     

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目的研究异常代谢产物沉积性肝病的肝组织超微病理及组织病理改变特征,结合临床资料探讨其诊断意义。方法2005-08—2006-03,对华中科技大学附属同济医院儿科8例临床拟诊为代谢性肝病患儿进行肝组织活检,分析肝组织病理及超微病理特征。结果普通病理报告提示糖原累积病2例,尼曼-匹克病1例,代谢性肝病1例,慢性轻型普通型肝炎1例,其余3例无特别提示。超微病理报告提示糖原累积病3例,脂质沉积性肝病5例。3例以肝细胞内糖原沉积为主要特征者诊断为糖原累积病,其中,1例肝细胞内大量PAS阳性物质,脂肪变性明显,无纤维组织增生,考虑为Ⅰ型;1例脂肪变性较轻而纤维化明显,考虑为Ⅲ型;另1例电镜发现肝细胞浆内单颗粒糖原明显增加,粗面和滑面内质网扩张,Disce间隔和部分肝细胞间纤维成分增多,部分细胞脂滴增加,考虑可能为Ⅱ型或其他轻型糖原累积病。5例以肝细胞浆内多个膜包裹的电子透亮空泡和泡内或疏松或致密高电子密度物质沉积为主要特征者诊断为脂质沉积性肝病,其中1例光镜见大量典型泡沫细胞而诊断为尼曼-匹克病,其余4例分类不明。结论电镜检查肝组织超微结构对于代谢性肝沉积病的诊断较光镜检查更有帮助,可明显提高诊断率。     

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一般资料:选择2005-02—2006-06在本院住院的下呼吸道感染的患儿85例,年龄为2个月至6岁,其中男56例,女29例,病程<5d。按入院日期分为两组,对照组42例,其中支气管肺炎22例,支气管炎15例,毛细支气管炎5例;治疗组43例,其中支气管肺炎23例,支气管炎14例,毛细支气管炎6例。两组均符合《诸福棠实用儿科学》第6版关于支气管肺炎、支气管炎、毛细支气管炎的诊断标准。两组患儿均表现有发热、咳嗽、喘息、肺部听诊可闻及干性啰音和(或)湿性啰音阶榛级谀炅洹⒉〕獭⒓膊」钩伞?病情等方面差异无统计学意义(P>0.05)。方法:对照组给予抗感染、吸氧、…     

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??Abstract??Objective The time-effect relationship in treating postural orthostatic tachycardia syndrome ??POTS?? of children with alpha receptor agonist midodrine hydrochloride was analyzed to explore the optimum course of treatment for POTS children. Methods A total of 104 cases of the POTS children were studied from 2005 to 2011 in Peking University First Hospital. A chi-square test was used to evaluate the relationship between effect and time of medication?? and the time-effect curve was constructed. Results According to the 104 cases?? the time accumulative total efficacy for 1 month?? 2 months?? 3 months?? 4 months?? 5 months and 6 months were 19.23%?? 48.08%?? 69.23%?? 73.08%?? 74.04% and 75.96%?? respectively. The time accumulative total efficacy for 3 months was higher than that for 1 month or 2 months ??P??0.05???? but there was no difference in the efficacy among 4 months?? 5 months and 6 months treatment and with 3 months treatment. Conclusion It has the best treatment efficacy when the course of treatment for POTS children with midodrine hydrochloride is 3 months?? and prolonging the duration of treatment does not significantly improve the therapeutic effect.     

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目的研究北京儿童医院脓疱疮患儿临床分离A组β溶血性链球菌(GAS)及其对抗菌药物的敏感性情况。方法收集北京儿童医院2003—2008年收治的1735例脓疱疮儿童皮损部位分离出的52株GAS菌株,采用琼脂稀释法测定9种抗菌药物的最低抑菌质量浓度(MIC)值;用聚合酶链反应方法对上述菌株进行大环内酯类抗生素耐药基因ermB、ermA和mefA检测以及emm分型情况。结果分离株对四环素及大环内酯类抗生素耐药率均为100%,MIC>256mg/L;对青霉素、头孢拉啶及氧氟沙星的敏感率高达100%。大环内酯类抗生素耐药基因ermB、ermA和mefA和阳性率分别为92.30%、7.70%和0;分离株的emm分型最常见的为emm12.0(53.85%),emm1.0(36.54%)。结论北京儿童医院收治的脓疱疮儿童GAS感染流行菌株对大环内酯类抗生素耐药率很高,主要耐药机制为ermB编码的23SrRNA甲基化酶导致靶位改变,在北京地区青霉素类和头孢菌素类抗生素仍是治疗GAS感染首选药物。     

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??Objective??To investigate the change of the platelet state and function in acute lymphocytic leukemia. Methods??The changes of platelet indices??PLT??PCT??MPV??PDW????immature platelet fraction??IPF%????immature platelet counts??IPC????granule membrane glycoprotein of platelet ??CD62p?? and PAC-1 were obtained by using automatic blood cell analyzer and whole blood flow cytometry??FCM?? respectively??in children with acute lymphocytic
leukemia??ALL????ALL in first complete remission ??ALL-CR1?? and children undergoing elective surgical procedure??control group??. Results????1??Without addition of platelet agonists ADP??expression of platelet surface activated CD62p and PAC-1 in ALL was higher than that in control group??P??0.05????while expression of platelet surface activated PAC-1 in ALL-CR1 was higher than that in control group??P??0.05????and lower than that in ALL??P??0.05??????2??With addition of platelet agonists ADP??expression of platelet surface activated CD62p and PAC-1 in ALL was lower than that in control group??P??0.05????while expression of platelet surface activated PAC-1 in ALL-CR1 was lower than that in control group??P??0.05????and higher than that in ALL??P??0.05??????3?? PLT??PCT and MPV in ALL was lower than that in control group and ALL-CR1 ??P??0.05??????4?? IPF% in ALL was higher than that in control group and ALL-CR1??P??0.05????and IPC was lower than that in control group and ALL-CR1??P??0.05??. Conclusion??The children who are newly diagnosed with acute lymphocytic leukemia have peripheral platelet reactivity and platelet function defects. Platelet indices and platelet membrane glycoprotein can be used as effective indicators to judge the effect on ALL.     

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病毒性心肌炎(VMC)较为常见,由于其诊断中缺乏特异指标,治疗中缺少特效措施,故在诊治中存在一些有争议的问题,使得临床医师无所适从。本文选择临床上经常遇到且争议较大的八大问题,通过介绍国内外文献以及个人研究结果,提出自己的观点,供临床参考。1VMC诊断中的争议1.1早搏与VMC的关系早搏与VMC的关系有不同看法,有些医生认为早搏就是心肌炎,有些认为部分频发早搏(尤其是室性早搏,以下简称室早)与VMC有关,有些认为早搏与VMC完全无关。国外心脏病专著中提出,既往无