新生儿青紫型先天性心脏病156例的早期诊治分析

目的 了解青紫型先天性心脏病在新生儿期的临床表现、分布情况、治疗和转归。方法 1999年11月至2004年7月上海儿童医学中心对156例青紫型先天性心脏病(CHD)新生儿进行临床分析。结果 (1)青紫型CHD在新生儿期主要表现为中央性紫绀、心功能不全，个别可合并心律紊乱。(2)新生儿期青紫型CHD以完全性大动脉转位(TGA)居首位，其他依次为肺动脉闭锁(PA)、完全性肺静脉异位引流(TAPVC)和肺动脉狭窄(PS)。(3)内科治疗包括强心、利尿、扩血管药物和前列腺素E1(PGE1)的运用；156例中57例施行外科手术，其中以TGA最多。结论新生儿青紫型CHD心脏畸形复杂，病情多危重，在生后尽早明确诊断并给予适当的内外科治疗有助于提高其生存率和改善预后。 Abstract Objective To investigate the clinical manifestation,distribution,treatment and prognosis of congenital heart disease in neonatal period.Methods Analyze 156 cases of neonatal cyanotic congenital heart disease clinically.Results ① Neonatal cyanotic CHD was manifested with central cyanosis,heart failure,and arrhythmia occasionally.② D TGA stand first on the list of neonatal cyanotic CHD and the others ordinal was PA,TAPVC and PS.③ Internal medical therapy included using of cardiotonic,diuretic and prostaglandin E157 patients were given surgical operations and TGA lead the first.Conclusion For complex anomaly and critical condition in most cases,neonatal cyanotic CHD should be diagnosed as soon as possible and treated appropriately in order to improve the viability and prognosis of these neonates. Key words NeonateHeart disease;Congenital     

圆锥动脉干畸形室间隔缺损的三维超声定量研究

目的 探讨三维超声心动图(3DE)在圆锥动脉干畸形(CTD)室间隔缺损(VSD)定量研究中的准确性及其临床意义。 方法 2000年5月至2003年11月在上海儿童医学中心住院且二维超声心动图(2DE)、经胸3DE、心血管造影及手术等定量测量结果完整的CTD患儿共63例，将3DE测量结果与2DE、造影及手术测值对比。 结果 方差分析、SNK均数间两两比较及相关分析显示，3DE VSD长径测值为1.69cm(从右室面)和1.63cm(从左室面)，虽较手术测值(1.84cm)低估，但低估程度小于2DE测值(1.27cm)及造影测值(1.12cm)；3DE与手术测值的相关性(r=0.74)明显高于2DE(r=0.39)及造影测值(r=0.25)。3DE VSD短径测值与造影测值相接近，两者比较无统计学差异(P＞0.05)。3DE VSD短径测值与2DE及造影测值的相关性(r=0.45，r=0.54)高于3DE VSD长径测值与2DE及造影测值的相关性(r=0.37，r=0.44)。3DE VSD面积测值与手术测值呈良好相关(r=0.57)。 结论 3DE可作为CTD VSD定量分析的又一无创手段，能提供较2DE更准确的定量诊断。 Abstract Objective To evaluate the accuracy and clinical value of transthoracic three dimensional echocardiographic (3DE) quantitative analysis in ventricular septal defect (VSD) of conotruncal defects (CTD).Methods From May 2000 to November 2003 in Shanghai Children Medical Center,there were 63 inpatients of CTD with all quantitative findings measured by 3DE,two dimensional echocardiography (2DE),angiocardiography and surgical findings.Their measurements of VSD sizes and areas with 3DE were compared with those with 2DE,angiocardiography and surgical findings.Results From the analysis of variance,Student Newman Keuls (SNK) test and correlation analysis,the measurements of VSD long diameter (mean 1.69cm in en face view of ventricular septa from right ventricle and 1.63 cm in en face view of ventricular septa from left ventricle) with 3DE was less than the measurements of VSD diameter (mean 1.84 cm) with surgical findings,but they were better correlated with those with surgical findings (r=0.74) than the measurements of VSD diameter (mean 1.27 cm) with 2DE (r=0.39) and those (mean 1.12 cm) with angiocardiography (r=0.25).However,the measurements of VSD short diameter with 3DE was close to those with angiocardiography,and there was no significant difference (P>0.05) between them.The measurements of VSD short diameter with 3DE were better correlated with those with 2DE and angiocardiography than the measurements of VSD long diameter with 3DE.Meanwhile,the measurements of VSD area with 3DE were well correlated with the measurements of VSD diameter with surgical findings (r=0.57).Conclusion 3DE can provide more accurate quantitative diagnosis in CTD VSD than 2DE. Key words Conotruncal defects;Three dimensional echocardiography;Two dimensional echocardiography;Ventricular septal defect     

多层螺旋CT和经胸二维超声心动图检查

目的 探讨多层螺旋CT(MSCT)及联合经胸二维超声心动图(TTE)检查在先天性心脏病诊断中的意义。方法收集广东省心血管病研究所自2002年9月至2003年12月间86例儿童先天性心脏病病例，全部患儿接受了MSCT和TTE检查，12例接受心导管检查，其中69例进行了外科手术，将术前MSCT、TTE和心导管检查结果分别与手术诊断进行比较。结果 69例患儿共计有129处畸形，TTE正确诊断116处(89.9%)，MSCT正确诊断112处畸形(86.8%)，两者联合正确诊断127处畸形(98.4%)，12例心导管检查未能提供更有价值的资料。结论 MSCT对先天性心脏病诊断具有较高的价值，尤其是联合TTE可取代部分心导管检查为外科手术提供正确和充分的术前诊断。对于年龄小或重症不耐受心导管检查的患儿具有更大的意义。 Abstract Objective To evaluate the effectiveness of multislice computed tomography (MSCT) in diagnosis of congenital heart diseases in children.Methods 86 patients were involved.69 had been performed operations.The preoperative results of transthoracic two dimensional echocardiography (TTE),MSCT and angiocardiography were compared with those of surgery.Results Of the totally 129 malformations,TTE had correctly revealed 116 (90%),MSCT 112(86.8%),and both combined had correct diagnosis for 127(98.4%).Angiocardiography in 12 patients had not provided further information. Conclusion MSCT is a valuable additional diagnosis tool in congenital heart diseases,especially when combined with TTE.They might take place some of the angiocardiography before surgery. Key words Multislice computed tomography;Transthoracic two dimensional echocardiography;Congenital heart disease     

法洛四联症根治术后急性肺损伤的治疗

目的 分析法洛四联症(TOF)根治术后急性肺损伤(ALI)的原因，总结治疗体会。 方法 2003年7月至2004年6月，上海第二医科大学附属新华医院上海儿童医学中心心胸外科共行TOF根治术161例，发生ALI 5例。记录5例ALI患儿术前经皮氧饱和度(SpO2)、红细胞压积比(HCT)、McGoon比值、肺动脉指数(PAI)和手术、体外循环(CPB)资料，以及ALI发生时间、ALI发生后各项治疗措施、各治疗阶段肺功能指标、计算其肺损伤分数。 结果 5例ALI患儿，年龄7~24(13.8±3.1)个月，体重7.0~9.5(8.2±0.5)kg，均在静吸复合麻醉CPB下进行，术毕给予改良超滤，平均CPB时间(67.2±5.9)min，主动脉阻断时间(43.6±2.4)min，ALI发生率为3.1%，发生时间为术后8~60h，死亡2例。5例均施行腹膜透析术(PD)，2例给予一氧化氮吸入，1例给予肺表面活性物质替代治疗。各治疗阶段肺功能指标逐步改善，至治疗后期，肺功能指标在统计学上有显著性差异(P<0.05)。 结论 TOF术后ALI治疗困难，合理机械通气、尽早维持体液平衡、改善肺通气和氧合功能有利于及时阻断低氧酸中毒导致的恶性循环。 Abstract Objective To analyse the reasons of acute lung injury (ALI) after the surgical correction of tetralogy of Fallot (TOF) and summarize the postoperative treatment skill.Methods There were 161 cases of patients with TOF who underwent corrective repair operation in the Department of Cardiovascular Surgery of Xinhua Hospital/ Shanghai Children’s Medical Center from Jul.2003 to Jun.2004,in which there were 5 patients who developed ALI after the surgical correction of TOF.The mean age was 13.8±3.06 months (7~24 months) and the mean body weight was (8.20±0.46)kg (7~9.5kg).Before operation,their mean SpO2 was (72.8±2.2)% (67%~80%),mean HCT was (49.0±5.5)%,mean McGoon ratio was (1.27±0.05) and mean PAI was (136.0±16.8)mm2/m2.The total cardiopulmonary bypass (CPB) time and aortic clamp time were (67.2±3.9) min and (43.6±2.4) min,respectively.The modified ultrafiltration was used throughout the CPB.The treatment and the blood analysis were recorded when the ALI developed.The parameters of pulmonary function and lung injury score were calculated.Results ALI developed (30.0±9.7) hrs after the operation and the incidence of ALI after the surgical correction of TOF was 3.1%.Two cases died within 48 hrs.Peritoneal dialysis (PD) was performed on all the cases.Meanwhile,2 cases were given inhaled nitric oxide (NO) and 1 case was given pulmonary surfactant (PS).The parameters of pulmonary function were improved step by step and the differences were statistically significant at the latest stage of treatment (P<0.05).Conclusion The treatment of ALI after surgical correction of TOF includes reasonable mechanical ventilation and maintaining the balance of fluid.Inhaling NO and using PS will be benefitial to improve the pulmonary function and to stop the vicious circle which results in the hypoxemia and refractory acidosis. Key wordsTetralogy of Fallot;Acute lung injury;Cardiopulmonary bypass     

新生儿血培养中凝固酶阴性葡萄球菌细胞间粘附基因A和D的检测及临床意义

目的 探讨细胞间粘附基因(ica)A和icaD在新生儿凝固酶阴性葡萄球菌(CNS)败血症中的诊断意义。方法 收集北京儿童医院新生儿病房2001年11月至2003年3月间血培养为CNS的患儿为研究入选病例。通过应用聚合酶链反应(PCR)方法检测icaA及icaD的存在。结果 在80例入选患儿中，血培养均分离出CNS。根据新生儿败血症的诊断标准，临床诊断败血症27例(33.8%)，其中早产儿7例，足月小样儿2例。在菌种的分布上，表皮葡萄球菌18例(66.7%)，溶血葡萄球菌7例(25.9%)，人葡萄球菌2例(7.4%)。对90株CNS菌株应用PCR方法检测icaA及icaD，共有8株阳性，均为表皮葡萄球菌，并且icaA和icaD同时阳性。在经PCR扩增后所产生的图谱中，icaA在814bp，icaD在282bp。在ica阳性的8例病例中，临床诊断为败血症7例，一致率为87.5%，在临床排除败血症的53例患儿中，仅有1例ica阳性(1.8%)。ica的阳性预测值为259%，阴性预测值为981%。结论 ica基因PCR检测是临床诊断CNS败血症或导管相关感染的一种潜在的实验室方法。 Abstract Objective The purpose of this study was to assess whether the person making the clinical decision may benefit from the detection of icaA and icaD genes encoding putative virulent factors.Methods From Nov.2001 to Mar.2003,we detected the icaA and icaD genes in 80 neonates with a collection of clinical isolates from blood cultures by using a simple,rapid,and reliable PCR method.Results An overall total of 80 neonates with CNS strains from blood cultures were identified.There were 27 cases(33.8%)diagnosed neonatal sepsis clinically according to the standard,of which 7 were premature,2 were small for gestational age of full term infants.The distribution of species among the clinical CNS strains,18(66.7%)cases were Staphylococcus epidermidis,which was the leading cause,and then S.hemolyticus (n=7),S.hominis (n=2).There was a statistical difference in them.Of the 90 strains,eight were positive to icaA and icaD genes,with PCR products being obtained for the icaA and icaD genes in all of these strains,a 814 bp band for the icaA gene and a 282 bp band for the icaD gene.All of them were Staphylococcus epidermidis.Among 8 cases with positive ica genes,7 were diagnosed as sepsis clinically.The coincidence rate was 87.5%.Positive predictive value of the ica genes were 25.9%，and negative predictive value were 98.1%.Conclusion Ica genes may potentiate the clinical criteria used for the diagnosis of neonatal sepsis,and may discriminate between contamination and infections. Key words Coagulase negative staphylococcus;PCR;Newborn     

Analysis of the human zona pellucida during culture: Correlation with diagnosis and the preovulatory hormonal environment

Purpose: The objective of this study was to analyze sequentially the human zona pellucida changes in an in vitro fertilization program as it relates to several variables. Methods: The zona pellucida thickness was measured daily in zygotes and cleavage-stage embryos on a Nikon inverted microscope equipped with Hoffman modulation contrast optics, using an ocular micrometer. A total of 512 embryos from 96 patients was evaluated. Results: There was a highly significant direct correlation between zona thickness and preovulatory estradiol and basal day 3 FSH levels (P < 0.02 andP < 0.0006, respectively). This relationship showed a rapid reversal following 48 hr of culture; embryos from patients with the highest FSH levels had thinner zonae prior to transfer (P < 0.0007). The zonae from patients with unexplained infertility were thicker (19.4 ± 2.7 μm) than those from patients with endometriosis (17.7 ± 2.2 μm), tubal (17.5 ± 2.4 μm), or male-factor infertility (16.4 ± 2.7 μm) (P < 0.0001) on the first day of culture. Conclusions: We hypothesize that the thickness of the human zona pellucida is influenced by the preovulatory hormonal environment and diagnosis. These factors should be considered as part of the embryo quality evaluation prior to transfer or when assessing the possibility of using assisted hatching. More studies are needed to understand the factors regulating the thickness of the human zona pellucida.     

儿童急性白血病细胞周期蛋白D3、E的表达

目的 探讨细胞周期蛋白D3、E的表达与儿童急性白血病(AL)的关系。方法 采用免疫组织化学法，对广西医科大学第一附属医院儿科2002年1月至2004年5月收治的50例初发或复发儿童AL，其中急性淋巴细胞白血病(ALL)30例(高危ALL 12例，标危ALL 18例)，急性非淋巴细胞白血病(ANLL)20例，和26例完全缓解AL(AL CR)患儿，以及23例同期住院的非恶性疾病患儿(对照组)，检测其骨髓细胞中细胞周期蛋白D3、E的表达情况。结果 AL组中细胞周期蛋白D3、E阳性率分别为54%和46%，高于AL-CR组和对照组(P<0.01)；在ALL和ANLL间差异无显著性(P>0.05)。高危ALL细胞周期蛋白D3阳性率明显高于标危ALL(P<0.01)。化疗前细胞周期蛋白D3、E表达阳性率明显高于化疗缓解后(P<0.01)。细胞周期蛋白D3、E在AL组中的表达呈正相关(r=0.298，P<0.01)。结论 细胞周期蛋白D3、E与儿童AL发病有关，它们的表达存在相关性，可作为疗效观察的指标；细胞周期蛋白D3可能与患儿不良预后有关。 Abstract Objective To investigate the relationship between the expression of cyclin D3，cyclin E and childhood acute leukemia(AL).Methods The expression of cyclin D3 and cyclin E was detected in bone marrow cells from 50 cases of newly diagnosed or relapsing AL，in whom there were 30 cases of acute lymphoblastic leukemia(ALL，including 12 cases of high risk ALL and 18 cases of standard risk ALL ) and 20 cases of acute non lymphocytic leukemia(ANLL)，26 cases of complete remission AL(AL CR) patients and 23 cases of control group(no malignant disease) using immunohistochemical assay.Results 54% AL cases were positive for cyclin D3，and 46% for cyclin E，both being remarkably higher than that of AL CR group and control group(P<0.01).There was no difference between ALL and ANLL in the expression of cyclin D3 and cyclin E(P>0.05).The positive expression of cyclin D3 was higher in high risk ALL than that in standard risk ALL(P<0.01).The expressions of cyclin D3 and cyclin E before chemotherapy were significantly higher than that after remission using chemotherapy(P<0.01).There was significantly positive correlation between the expression of cyclin D3 and cyclin E in AL (r=0.5298，P<0.01).Conclusion In childhood AL，the expression of cyclin D3 and cyclin E is quite high，which is related to the development of AL and might be index of treatment effect.There is positive relation between cyclin D3 and cyclin E.Cyclin D3 might be related to poor prognosis. Key words Acute leukemia;Cyclin;Immunohistochemistry     

婴幼儿铅中毒脑病临床特点及预后分析

目的 探讨铅中毒脑病的临床特点及预后。方法 回顾分析衡阳市中心医院儿科2002~2003年26例铅中毒脑病患儿的临床资料及随访结果。结果 26例铅中毒脑病患儿临床均表现为频繁的抽搐伴意识障碍，中度以上贫血。伴呕吐23例、腹泻21例、发热11例、肝大5例。26例患儿治疗前血铅平均为8.486μmol/L，尿铅平均为3.598μmol/L，Hb均低于90g/L，最低55g/L。血钙均低于1.86mmol/L， 最低1.03mmol/L。脑脊液蛋白均增高，22例心肌酶谱增高，提示心肌损害明显。头颅CT/MRI仅1例脑室系统轻度扩张，全部病例经驱铅治疗后无一例死亡，1年后随访,继发性癫疒间4例，脑瘫4例。结论铅中毒脑病是铅中毒的严重类型，预后不良。对不明原因的无热惊厥者应注意及时行血铅检查，以便早期诊断，防止漏诊。 Abstract Objective Study the clinical characteristics and prognosis of lead poisoning encephalopathy.Methods We reviewed and analyzed the clinical data and the results of the 26 cases of lead poisoning encephalopathy.Results All the 26 cases had frequen convulsion,accompanied by disturbance of consciousness and above middle pitch of anemia.Among the 26 cases vomit ting was found in 23 cases,diarrhea in 21 cases,fever in 11 cases,live swelling in 5 cases.Supplemental examination:before treatment,the average of blood lead level was 8.486μmol/L，urine lead level was 3.598μmol/L,Hb was lower than 90g/ L,the lowest being 5g/L.The blood calcium was all lower than 1.86 mmol/ L,the lowest being 1.03 mmol/L.Protein of cerebrospinal fluid in all cases was over normal.Myocardial enzyme of 22 cases was over normal,suggesting myocardials injured obviously.Only 1 case was found ventricle system expanding lightly in CT/MRI.All cases had no death after treatment of driving lead.The 12 month follow up result suggested that second epilepsy was found in 4 cases and cerebral palsy in 4 cases.Conclusion The lead poisoning encephalopathy is the most serious type of the lead poisoning,and prognosis is not good. The patient of convulsion with unknown reason,and no fever,should be checked the blood lead for early diagnosis in order to prevent from leaking diagnosis. Key wordsLead poisoning;Encephalopathy;Clinical characteristics;Prognosis      

大剂量维生素C对川崎病急性期肱动脉血流介导扩张反应影响的临床观察

目的 观察大剂量维生素C(VitC)对川崎病(KD)急性期肱动脉血流介导的扩张反应(FMD)的影响，探讨VitC对KD急性期血管内皮功能障碍是否有改善作用。方法 采用高分辨率多普勒超声仪测定2001年1月至2002年12月在华中科技大学同济医学院附属同济医院儿科住院的36例KD急性期患儿(KD组)及15名健康儿童(正常对照组)肱动脉FMD，并比较KD组静脉滴注大剂量VitC(200mg/kg，最大量为4g)和安慰剂(10%葡萄糖)前后肱动脉FMD的变化。结果 KD组肱动脉FMD明显低于正常对照组(P<0.001)；KD组静滴大剂量VitC后肱动脉FMD明显增加(P<0.01)，静滴安慰剂后肱动脉FMD无明显增加(P>0.05)。结论 大剂量VitC能明显改善KD急性期肱动脉FMD，其对KD急性期血管内皮功能障碍可能有显著改善作用。 Abstract Objective To observe the effect of high dose vitamin C on flow mediated vasodilation(FMD) of brachial artery in acute phase of Kawasaki disease(KD) and investigate whether high dose vitamin C could improve vascular endothelial dysfunction in KD.Methods Using high resolution vascular ultrasound,we measured FMD of the brachial artery of 36 patients who were admitted to our hospital from Jan.2001 to Dec.2002. in acute phase of KD and 15 matched healthy subjects as controls.Studies were performed before and after intravenous infusion of high dose vitamin C (200mg/kg，max≤4g) or placebo in KD group.Results The FMD of the brachial artery in KD group was significantly lower than that in the control group (3.55±3.00% vs 10.34±4.29%，P<0.001).Intravenous infusion of vitamin C significantly increase the FMD of the brachial artery in 18 KD patients (6.97±2.93% vs 3.51±2.01%，P<0.01),while after administration of placebo in the other 18 patients,there was no significantly increased in the FMD(4.12±2.21% vs 3.75±3.00%，P>0.05).Conclusion Intravenous infusion of high dose vitamin C can increase the FMD of the brachial artery,and it can improve vascular endothelial dysfunction in KD. Key words Kawasaki disease;Vitamin C;Flow mediated vasodilation;Endothelial dysfunction     

儿童横纹肌肉瘤8例临床分析

目的 分析儿童横纹肌肉瘤的临床特点，提高其诊治水平。方法 对中国人民解放军总医院1998年1月至2002年10月收治的8例横纹肌肉瘤患儿临床资料进行分析。结果 8例患儿中，男5例，女3例；发病年龄7个月至11岁，<5岁者3例；依据美国横纹肌肉瘤研究组(IRS)的分期标准I期2例、Ⅱ期2例、Ⅲ期1例，Ⅳ期3例；原发于头颈部4例、四肢2例，泌尿、消化系统各1例。病理类型:胚胎型7例,腺泡型1例。临床表现随原发部位不同有很大差异，基本为肿瘤组织占位、侵蚀后引起。明确诊断依靠病理，治疗以手术、化疗和放疗相结合，放疗总量40~50Gy，化疗共有3种方案:VDCA、VAC和VadrC。对Ⅰ、Ⅱ期手术彻底切除肿瘤患儿行VAC、VadrC方案交替共2年，Ⅲ、Ⅳ期患儿前14周用VDCA方案，后VAC、VadrC方案交替共2年。全部患儿2年生存率为37.5%(3/8)。结论 儿童横纹肌肉瘤是多发于头颈部，病理多表现为胚胎型的极度恶性软组织肿瘤，早期诊断，合理应用手术、化疗与放疗结合的综合治疗，是提高该肿瘤患儿生存率的关键。 Abstract Objective To analyze the clinical characteristics of the childhood rhabdomyosarcoma.Methods Clinical data was analyzed in 8 children with rhabdomyosarcoma.Results From Jan,1998 to Oct,2002,eight children with rhabdomyosarcoma had been diagnosed in our department,5 boys and 3 girls,7 months to 11 years old.Primary site was head and neck (n=4),extremities(n=2),kidney (n=1),bile duct(n=1).2,2,1,3 patients were in stage Ⅰ,Ⅱ,Ⅲ,Ⅳ respectively.Pathological data revealed embryonal rhabdomyosarcoma(n=7) and alveolar rhabdomyosarcoma(n=1).The clinical manifestation was mass in primary site or the tumor erodes.All patients had been diagnosed pathologically.The treatment included multimodal therapy with combination of surgery,chemotherapy,and radiation,whose doses were 40 to 50 Gy.Chemotherapy was given to all patients with VDCA、VAC or VadrC.The patients with pre Stage II have received chemotherapy with protocol of VAC or VadrC for two years.The protocol of VDCA has been used to the patients with Stage Ⅲand Ⅳ for 14 weeks,then the VAC or VadrC has been used until 2 years later.Two year EFS was 37.5%.Conclusion The head and neck are the most common primary site for rhabdomyosarcoma in children,with mainly histological embryonal rhabdomyosarcoma.Early diagnosis and combination therapy including surgery,chemotherapy,and radiation are key for those with rhabdomyosarcoma to get long EFS. Key words Rhabdomyosarcoma;Children;Treatment     

反复呼吸道感染儿童血清甘露聚糖结合凝集素水平及54密码子基因突变筛查

目的 了解反复呼吸道感染(RRTI)儿童血清甘露聚糖凝集素(MBL)水平及第一外显子54密码子的突变率，探讨血清MBL水平与RRTI的关系。方法 用ELISA方法检测2000~2003年在重庆医科大学附属儿童医院就诊的65例RRTI儿童和238名正常儿童血清MBL水平，测定其中11例低MBL血症的RRTI儿童的免疫学指标(IgG、IgA、IgM、C3、C4)，并用聚合酶链反应(PCR) 限制性内切酶片段长度多态性分析(RFLP)方法与55名正常儿童MBL第一外显子54密码子基因多态性进行分析。结果RRTI儿童出现低血清MBL水平频率明显多于正常儿童(χ2=6.96,P<0.05)，其MBL 54密码子突变率亦明显增高(P<0.05)，低血清MBL血症导致RRTI主要在2岁以前，血清MBL水平越低，感染频率越高，其C3、C4水平也越高。结论 儿童MBL 54密码子突变导致低MBL血症在2岁以前有反复呼吸道感染的倾向，MBL水平越低，感染机率越高。 Abstract Objective To detect the mannose binding lectin (MBL) serum levels and the mutation on code 54 of MBL in children with recurrent respiratory tract infections(RRTI),and to find the relationship between MBL serum levels and recurrent respiratory tract infections(RRTI).Methods To detect the serum MBL level of 238 normal people and 65 children with RRTI through the methods of enzyme linked immunoadsordent assay,then compare the immune data(IgG,IgA,IgM,C3,C4) of 11 children with low serum MBL level and compare the rate of the mutation on code 54 through the methods of polymerase chain reaction(PCR) restriction fragment length polymorphism (RFLP) with 55 normal children.Results The rate of low serum MBL level in the children with RRTI was higher than that in the normal population(χ2=6.96,P<0.05),the same went for the frequency of mutation of code 54 (P<0.05).RRTI caused by low serum MBL level occurred mostly among children under 2 years of age,and the rate of respiratory tract infection in the individuals with MBL level under 100μg/L was more than that in children with levels ranged from 100μg/L to 200μg/L (P<0.05),and was associated with reverse chang of serum levels of C3,C4(P<0.05).Conclusion Under 2 years of age,the children with the low serum MBL levels caused by mutation on MBL 54 show the susceptivity to IRRTI,and the low serum MBL level is parallel to high serum levels of C3，C4，and the lower the level the more possible infection. Key words Mannose binding lectin;Repeated respiratory tract infections;Gene;Polymorphism;Child     

大剂量氨甲蝶呤治疗小儿急性淋巴细胞白血病水化和碱化方法的探讨

目的 探讨进一步降低大剂量氨甲蝶呤(HD-MTX)治疗小儿急性淋巴细胞白血病(ALL)毒副反应的方法。方法 对2000年1月至2001年12月在上海儿童医学中心血液肿瘤科住院治疗的47例ALL患儿共进行的134例次HD-MTX治疗，按照两种不同的水化、碱化方法，分为全国标准治疗组和观察组。观察患儿血清中的MTX浓度、治疗效果及毒副反应的发生率。结果 (1)按WHO相关分度标准，各种毒副反应的发生率、Ⅲ度或Ⅲ度以上的毒副作用者的发生率两组比较差异有显著性意义。(2)观察组有2例次出现尿素氮的升高，标准治疗组未出现。(3)在应用HD-MTX开始后44h、68h时血清MTX浓度异常者两组相比较差异有显著性意义。结论 观察组HD-MTX的毒副反应发生率及严重程度均明显低于全国标准治疗组，且减少治疗费用,而不影响HD-MTX治疗效果。 Abstract Objective To study the ways to further reduce the toxic side effects of the HD-MTX in the treatment of childhood acute lymphoblastic leukemia.Methods The patients were divided into two groups:the testing and the treatment group.Two different ways of hydration and alkalization were used in the course of HD-MTX to evaluate the treatment results.Monitor the dynamic changes of the serum MTX level and occurrence of the toxic side effects.Results The results showed:(1)comparing the above toxic side effects occurred in both groups,significant difference could be found;(2)blood urea nitrogen(BUN) increased in 2 cases in the testing group but no one happened in the treatment group;(3)the occurrence of the serum MTX level abnormalities after 44 and 68 hours of HD-MTX treatment appeared in both groups with significant difference.Conclusion It shows the protocol for hydration and alkalization in the testing group has greater advantage than that in the treatment group in reducing the toxic side effects.This may decrease the cost of treatment,but may not influence the treatment effect of HD-MTX. Key words Methotrexate;Leukemia;Lymphoblastic;AcuteSide effect     

新生儿室间隔完整型肺动脉闭锁的治疗

Objective A retrospective study was undertaken to evaluate the outcomes of the newborns with pulmonary atresia with intact ventricular septum(PAIVS),in order to delineate strategies for the optimal management of PAIVS. Methods From July 2003 to July 2008,17 neonates with PAIVS underwent surgical treatment.The mean age at operation was(1 5.6±8.6)d and the mean weight was(3.54±0.44)kg.Initial surgical treatment included:Blalock-Taussig shunt (BT shunt)in 2 cases,closed pulmonary valvotomy and BT shunt in 1 case,right ventricular outlet tract reconstruction(RVOTR)in 2 cases,RVOTR and BT shunt in 11 cases. Results The mean duration of mechanical ventilation and intensive care unit stay were(35.5±35.1)h and(8.7±5.9)d,respectively.Two early deaths were reported inside the hospital(2/17,11.8%).Multivariable analysis demonstrated that mechanical ventilation before surgery was the risk factor for in-hospital mortality(r=1.02,P<0.01).The rest 15 cases were followed up with the average time of 19 months(2 months to 5 years)and no death was reported. Conclusions Neonates with PAIVS should be operated early.Individualized treatment strategy and regular follow-up are helpful to achieve better short-term outcome.     

Persistent increase of PRL after oral contraceptive treatment

 Background: The mechanism involved in estrogen induced hyperprolactinemia is not completely known, although one of the possible theories suggest inhibition of dopaminergic tone. Our objective was to study the mechanism implied in the increment of PRL levels as a consequence of oral contraceptive treatment and possible modifications in TSH levels. Material and methods: We performed a trial on 21 healthy women, nulliparas. We administered 35 μg of Etinil-Estradiol (EE) and 2 mg of Ciproterone Acetate (CA) for a period of 12 months. Stimulation tests with Metoclopramide and TRH were carried out before treatment, after 3, 6 and 12 months of treatment and finally 6 months after cessation of treatment. Results. Basal levels of PRL (×–₁=12.62 ng/ml) increased significantly (p<0.05) during the year of treatment (×–₁₂=17.04 ng/ml) and maintained higher levels 6 months after cessation (×–₁₈=17.53 ng/ml). Maximum values obtained in response to metoclopramide (×–₁=154.78) were significantly higher after 12 months (×–₁₂=173.29), persisting 6 months after cessation of treatment (×–₁₈=245.28). We also observed significant differences in the maximum response of TSH to metoclopramide during the same period of study (×–₆=2.45), (×–₁₂=2.76) and (×–₁₈=2.07) respectively (p<0.05). We did not find changes in PRL and TSH responses to TRH stimulation after a year of treatment with EE and CA. Conclusion: Treatment with EE (35 μg) and CA (2 mg) induces an increase in PRL levels that persist 6 months after cessation of treatment. Our results rule out the possibility that this increase in PRL is due to a decrease in dopaminergic tone or an increase in TRH sensitivity. Received: January 1999 / Accepted: 4 May 1999     

新生儿室间隔完整型肺动脉闭锁的治疗

Objective A retrospective study was undertaken to evaluate the outcomes of the newborns with pulmonary atresia with intact ventricular septum(PAIVS),in order to delineate strategies for the optimal management of PAIVS. Methods From July 2003 to July 2008,17 neonates with PAIVS underwent surgical treatment.The mean age at operation was(1 5.6±8.6)d and the mean weight was(3.54±0.44)kg.Initial surgical treatment included:Blalock-Taussig shunt (BT shunt)in 2 cases,closed pulmonary valvotomy and BT shunt in 1 case,right ventricular outlet tract reconstruction(RVOTR)in 2 cases,RVOTR and BT shunt in 11 cases. Results The mean duration of mechanical ventilation and intensive care unit stay were(35.5±35.1)h and(8.7±5.9)d,respectively.Two early deaths were reported inside the hospital(2/17,11.8%).Multivariable analysis demonstrated that mechanical ventilation before surgery was the risk factor for in-hospital mortality(r=1.02,P<0.01).The rest 15 cases were followed up with the average time of 19 months(2 months to 5 years)and no death was reported. Conclusions Neonates with PAIVS should be operated early.Individualized treatment strategy and regular follow-up are helpful to achieve better short-term outcome.     

Extra-amniotic prostaglandin E2 for midtrimester termination of pregnancy in live fetuses vs. fetal demise

This study compared the course of midtrimester termination of pregnancies with fetal demise and those with a viable fetuses by extra-amniotic prostaglandin (PG) E₂. A total of 275 women who underwent second trimester abortion with extra-amniotic PGE2₂ were divided into two groups: 95 patients (35%) with fetal demise and 180 women (65%) with a live fetuses. Extra-amniotic PGE2₂ was administered in doses of 200 μg every 2 h up to 20 doses. Bumm curettage was performed in the majority of the patients. We compared the duration and complication rate between the groups. The median induction to abortion interval was significantly shorter in the fetal demise group (13 vs. 21 h) than in the live fetus group. Mean gestational ages and complication rates were similar. Midtrimester termination of pregnancy with extra-amniotic PGE2₂ is a safe method with a low complication rate. In cases of pregnancy with fetal demise extra-amniotic PGE2₂ is associated with a significantly shorter induction to abortion interval than with a live fetus.     

荧光定量聚合酶链反应检测儿童病毒性脑炎病原体的研究

目的 探讨荧光定量聚合酶链反应(FQ PCR)检测儿童病毒性脑炎脑脊液(CSF)中的病原体的价值。方法 应用定性聚合酶链反应(PCR)对2002年1月至2004年10月保定市儿童医院收治的病毒性脑炎患儿78例的CSF标本筛选,检测最常见的引起病毒性脑炎的6种病毒,对单纯疱疹病毒(HSV)，柯萨奇病毒(CV)，埃可病毒(EV)阳性者做FQ-PCR检测，其中HSV分别在3个时点(治疗前、治疗中、出院时)检测DNA拷贝数。结果 定性聚合酶链反应阳性标本FQ PC检测100%阳性,HSV-DNA拷贝数在3个时点有统计学差异，病毒核酸拷贝数与临床表现一致。结论 FQ-PCR可作为儿童病毒性脑炎早期诊断的有效方法之一，动态观察HSV-DNA含量有助于指导治疗，估计疾病的发展和预后. Abstract Objective To discuss the value of using FQ-PCR to check pathogen in children viral encephalitis.Methods From Janurary,2002 to October,2004,PCR was used to select the children of viral encephalitis and check the six kinds of common virus which cause viral encephalitis and then we used FQ-PCR to check the children with HSV(+),CV(+)EV(+) while checking the copied number of HSVE-DNA before,during and after treatment.Results It was 100% that FQ-PCR checked the sample of PCR (+).The number of HSV DNA was different at three time points. The copied number of virus DNA was consistent with clinical symptom.Conclusion FQ-PCR is one of the effective methods to diagnose the children's viral encephalitis.Observing the number of HSV DNA is beneficial for instructing,treating and estimating the development and future disease. Key words Children’viral encephalitis;Pathogen;FQ-PCR;PCR     

新生儿感染时CD分子变化及临床意义

目的 探讨不同胎龄新生儿感染时T、B、NK细胞及中性粒细胞表面CD分子变化及临床意义。方法 2004年2~6月复旦大学儿科医院用流式细胞仪检测34例早产儿及33例足月儿CD表达。结果(1)足月感染组CD3［(76.89±2.52)%］高于足月非感染组［(64.40±5.69)%］，早产非感染组［(80.93±9.13)%］高于足月非感染组；早产非感染组CD4［(61.20±2.21)%］高于足月非感染组［(47.60±4.27)%］，早产感染组［(53.63±3.23)%］低于早产非感染组；CD8各组间无统计学差异性。(2)CD19各组间无统计学差异性。(3)足月感染组CD56CD16［(5.88±0.62)%］低于非感染组［(13.00±5.31)%］，足月非感染组高于早产非感染组［(6.13±1.25)%］。(4)足月感染组CD64［(5056.92±1255.58)分子/单位］高于足月非感染组［(2112.60±1157.21)分子/单位］，早产感染组［(4619.67±1395.99)分子/单位］高于早产非感染组［(2407.45±1247.16)分子/单位］。(5)败血症、肺炎及其它感染组CD64高于非感染组。以CD64大于3000分子/单位为阳性，CD64诊断感染灵敏度为79.6%，特异度为75.1%；以CD64大于2000分子/单位为阳性，CD64诊断感染灵敏度为88.9%，特异度为60%；CRP诊断感染灵敏度为65.3%，特异度为86%。结论早产儿细胞表面CD3、CD4、CD16CD56变化不同于足月儿，可能与早产儿免疫功能低下有关；CD64可能会成为一种新型感染诊断指标。 Abstract ObjectiveTo evaluate the change and the clinical significance of the cell CD on the surface of T,B,NK and Neutrophils cells.MethodsFrom Feb. to June 2004,50 preterms and 49 terms with infection or non infection were sturied.The level of peripherical blood CD3,CD4,CD8,CD19,CD16CD56 and CD64 were measured by flow cytometry.Results① The level of CD3 in terms(76.89±2.52)% with infection was higher than that in terms without infection(64.40±5.69)%.The level of CD3 in preterms without infection(80.93±9.13)%was significantly higher than that in terms without infection.The level of CD4 in preterms without infection(61.20±2.21)% was significantly higher than that in terms without infection(47.60±4.27)%.The level CD4 in preterms with infection (53.63±3.23)% was significantly lower than that in preterms without infection ；The level of CD8 was not different in all groups.②The level of CD19 has no difference in all groups.③The level of CD56CD16 in terms with infection(5.88±0.62)% was significantly lower than that in terms without infection(13.00±5.31)%，the level of CD56CD16in terms without infection was significantly higher than that in preterms without infection(6.13±1.25)%.④ The level of CD64 in terms with infecton (5056.92±1255.58)Molecule/Unit was higher than that in terms without infection (2112.60±1157.21)M/U.The level of CD64(4619.67±1395.99)M/U in preterms with infection was significantly higher than that in the preterms without infection(2407.45±1247.16)M/U.⑤The level of CD64 in patients with sepsis,pneumonia and other infections were higher than that in those patients without infection.If the positive standard of CD64 was over 3000 M/U,then the sensitivity was 79.6% and the specificity was 75.1%.If the positive standard of CD64 was over 2000 M/U,then the sensitivity was 88.9% and the specificity was 60%.The sensitivity of CRP was 65.3% and the specificity was 86%.Conclusion The level of CD3、CD4、CD16CD56 in preterms with or without infection differs from those in terms,which is probably due to the low immunity function of preterms.CD64 may be a kind of new diagnosis guideline. Key wordsTerms;Preterms;CD molecule;CD64     

Survivin在儿童急性白血病中的表达和P53的相关性及其临床意义

目的探讨Survivin在儿童急性白血病(AL)中的表达及其与抑癌基因P53的相关性，同时观察Survivin与急性淋巴细胞白血病(ALL)临床分型的关系。方法应用免疫组织化学链霉亲和素-生物素-过氧化物酶复合物(SABC)方法，检测华中科技大学同济医学院附属同济医院儿科2004年1~7月收治的37例儿童AL骨髓细胞Survivin和P53的表达，对照组为10例非恶性血液病且骨髓正常患儿。结果在37例AL患儿中，Survivin蛋白表达阳性率为35.14%，P53表达阳性率为32.43%，显著高于正常骨髓组织(P均<0.05)，Survivin和P53表达呈强相关性(P<0.05)，Survivin在高危(HR)ALL患儿的表达为46.15%，在标危(SR)ALL患儿的表达为0，差异有显著性意义(P<0.05)。结论(1)Survivin在儿童AL中异常表达，且与ALL的临床分型有关，提示其在判定儿童ALL预后中有意义；(2)抑癌基因Ｐ53的失活与Survivin的表达可能在AL发生中起协同作用。 Abstract Objective To investigate the expression of Survivin in pediatric acute leukemia and its relationship with expression of P53 and clinical significance.Methods The expression of Survivin and P53 proteins were detected by immunohistochemical assay in 37 acute leukemia children.The control included 10 children with normal bone marrow.Results Survivin protein expression rate was 35.14% and P53 protein expression rate was 32.43% in 37 AL children.Both of them were significantly higher than that in control group (P<0.05).The expression of Survivin and P53 being strongly related (P<0.05).The expression rate of Survivin was 46.15% in HR ALL and 0% in SR ALL,the difference was statistically significant(P<0.05).Conclusion (1)Survivin protein expression rate is abnormal and is associated with the clinical typing of ALL.It may play an important role in the prognosis of ALL.(2)Expression of Survivin and de activation of anti oncogene P53 might play synergetic roles in the process of genesis of AL. Key words Survivin;P53;Children;Acute leukemia     

地塞米松、γ-干扰素对毛细支气管炎患儿免疫调节作用的观察

目的 观察地塞米松、γ-干扰素对毛细支气管炎患儿T细胞细胞免疫和T细胞体液免疫比值(TH1/TH2)的作用。方法将60例2002年11月至2004年4月在青岛市第八人民医院儿科住院的毛细支气管炎患儿，按入院顺序分成地塞米松组和γ-干扰素组，并选择15例健康婴儿为正常对照组。检测毛细支气管炎患儿治疗前血清IgE水平及细胞因子IFN-γ、IL-4水平，然后分别用地塞米松、γ-干扰素治疗，出院时复查血清IgE、IFN-γ及IL-4水平。结果 正常对照组和毛细支气管炎组血清IgE水平及细胞因子IFN-γ、IL-4、IFN-γ/IL-4水平差异有非常显著意义(P<0.001)。γ-干扰素组和地塞米松组治疗前血清IgE和细胞因子IFN-γ、IL-4、IFN-γ/IL-4差异均无显著性意义(P>0.05),但治疗后,以上指标差异均有非常显著意义(P<0.001)。出院后随访12个月，统计喘息反复发生的例数，γ-干扰素组3例，地塞米松组10例，经卡方检验差异有显著意义(P<0.05)。结论 毛细支气管炎患儿血清IgE水平高，TH1/TH2失衡。地塞米松短期内可起到抗炎、平喘作用，止喘起效快，但TH1/TH2治疗前后无差异，反复感染、喘息反复出现、容易发展成婴幼儿哮喘。 Abstract Objective To observe the effect of dexamethasone and IFN γ on the function of TH1/TH2 in lower respiratory tract infections.Methods Sixty patients with lower respiratory tract infections were divided randomly into 2 groups:group I with dexamethasone treatment,group II with IFN γ treatment.Normal group of 15 cases was as control.Serum levels of IgE,IFN γ and IL 4 were detected before and after treatment.Results Compared with normal control,there was significant difference in the serum level of IgE,IFN γ,IL 4,IFN γ/IL 4 in group Ⅰ and group Ⅰ,P<0.001.There was no difference in the serum level of IgE,IFN γ,IL 4,IFN γ/IL 4 between group I and group Ⅱ before treatment(P>0.05).But there was significant difference after treatment(P<0.001).After being followed up for 12 months,3 cases suffered from asthma in group Ⅰ,10 cases in group Ⅱ.There were outstanding meanings by square examination,P<0.05.Conclusion In patients with lower respiratory tract infections,the serum level of IgE rises.Humoral immunity is relatively increased and cell immunity decreased.So the TH1/TH2 is out of balance.With the treatment of dexamethasone,the body’s immune system is suppressed.In short time,the infection and asthma can be controlled,but TH1/TH2 can’t change after treatment.IFN- γ can interfere the duplication of virus,increase the level of IFN-γ in body,furthermore it can strengthen the cell immune function,lower cell immunologial answer of TH2 and recover the balance of TH1/TH2 The follow up suggests IFN γ has an important meaning in preventing infant asthma. Key wordsLow respiratory tract infections;IFN dexamethasone;Cell immunity;Humoral immunity