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     

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     

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     

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     

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     

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     

儿童急性淋巴细胞性白血病中枢神经系统浸润的防治

目的通过对中枢神经系统白血病(CNSL)的防治，预防和降低白血病的髓外复发机会，提高儿童急性淋巴细胞性白血病(ALL)的长期生存。 方法1999~2003年上海市新华医院对277例儿童ALL的诱导治疗期4~5次鞘内三联注射(甲氨蝶呤、阿糖胞苷、地塞米松)，巩固期后采用大剂量甲氨蝶呤(HD-MTX)24h静脉连续滴注，进入维持后，每3个月1次，总共8~9次，以后改为鞘内注射3个月1次，直至化疗结束，对于超高危ALL患儿(白细胞计数>100×109/L、Ph1染色体阳性)采用头颅照射加鞘内注射。 结果277例ALL患儿发生CNSL 9例，发生率为3.2%，9例CNSL中4例骨髓复发，5例存活，中位生存时间22.2个月。 结论CNSL的防治方法，明显降低了CNSL的发生率，使ALL患儿生存机会提高。发生CNSL的不利因素有高白细胞血症、T细胞性ALL、Ph1阳性染色体改变等。     

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     

经食管超声心动图在小儿先天性心脏病围术期的应用价值

目的 评价经食管超声心动图(TEE)对指导小儿先天性心脏病(先心病)外科手术和介入治疗的应用价值及其安全性。方法 2000年11月至2005年1月，在上海市复旦大学附属儿科医院心血管中心接受外科开胸手术或经导管介入治疗的先心病患儿中有317例进行了TEE检查，年龄2个月至17岁6个月(平均4.7岁)。采用HP/SONOS2500超声诊断仪，频率5.5~7.5MHz双平面经食管探头，全麻状态下进行。结果 与术前经胸超声心动图(TTE)比较，术前TEE检查对诊断作出修正或补充者51例(16.1%)，其中因此而修正了手术治疗方案25例(7.9%)。术后TEE检查发现有并发症或残余问题57例(18.0%)，其中8例(2.5%)因此立即再次手术。所有病例均未因TEE检查而引起并发症。结论 TEE可安全地应用于小儿先心病围术期检查，对术前诊断做出修正或补充，术后可及时发现并发症或残余问题。 Abstract Objective To evaluate the utility of transesophageal echocardiography (TEE) for surgical and interventional repairs in children with congenital heart disease (CHD) and its safety as well.Methods 317 patients with CHD underwent TEE examinations at the age of 2 months~17 years and 6 months(mean 4.7 years).HP/SONOS2500 ultrasonic instrument with transesophageal biplane probe of 5.5~7.5MHz was used to perform TEE under general anesthesia.Results Compared with preoperative transthoracic echocardiography(TTE),TEE had new findings or made revision of the diagnoses in 51 cases(16.1%) before operations,which led to the changes of surgical or interventional precedure in 25 cases(7.9%).TEE had found residual problems or evolving situations in 57 cases(18.0%) after operations,which led to a second immediate surgical intervention in 8 cases(2.5%).There were no detectable complications due to TEE in all cases.Conclusion TEE is valuable in providing meaningful preoperative emendations or additions and finding residual problems after operations. The technique is safe in children. Key words Transesophageal echocardiography (TEE);Congenital heart disease (CHD);Perioperative period;Children     

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.     

儿童双表型白血病的临床特征及预后分析

目的分析儿童双免疫表型白血病的临床及生物学特征，评价儿童急性白血病双免疫表型与治疗相关因素及预后的临床重要性。方法自1998年1月1日至2003年5月31日进入XH 99治疗方案的所有新诊治的急性白血病(AL)患儿，诊断采用MICM分型诊断，治疗分别按AML XH 99、ALL XH 99危险度分类标准进行分层治疗。用流式细胞仪进行免疫表型分析，根据免疫表型结果将患儿分为4组，伴有/无髄系相关抗原表达的急性淋巴细胞白血病(My+ALL/My-ALL)以及伴有/无淋系相关抗原表达的急性髓系白血病(Ly+AML/Ly-AML)。生存分析采用Kaplan Meier方法；生存率之间的比较采用log rank检验；临床及生物学特征与治疗相关因素的分析采用χ2检验或Fisher精确概率法(双尾)。结果①174例提供免疫分型的ALL患儿中，My+ALL患儿34例，占19.54%，其与My-ALL组患儿除在B ALL组患儿达缓解时间有统计学差异外(P<0.05)，其它临床、生物学特征及治疗反应均无统计学差异(P>0.05)；两组患儿5年无事件生存(EFS)率分别为\[(61.76±8.33)%与(68.03±5.55)%\],log rank检验两组患儿5年EFS率无统计学差异(P=0.0526)。②74例提供免疫分型结果的AML患儿中，Ly+AML患儿18例，占24.32%，其与Ly-AML组患儿临床、生物学特征及治疗反应无统计学差异(P>0.05)；两组患儿5年EFS率分别为\[(39.82±13.59)%与(51.29±9.70)%\],log rank检验两组患儿5年EFS率无统计学差异(P=0.3164)。结论双免疫表型对儿童白血病预后无明显影响，可用同样现行的化疗方案治疗这部分患儿。     

髓系抗原阳性儿童急性淋巴细胞白血病临床特点及预后

目的分析髓系抗原阳性儿童急性淋巴细胞白血病(ALL)的临床特点及预后关系。 方法根据国际白血病欧洲协作组(EGIL)标准将1999—2004年上海交通大学医学院附属上海儿童医学中心收治的33例髓系抗原阳性表达的ALL (My+ALL)分为双表型、双系列型给予正规治疗。对其预后进行观察。 结果(1)My+ALL患儿，双表型26例(78.8%，26/33)，其中B系ALL伴髓系表达17例(65.4%，17/26)，T系ALL伴髓系表达6例(23.1%,6/26)，T系B系 伴髓系表达3例(11.5%,3/26)。双系列ALL患儿7例(21.2%,7/33)。(2)26例双表型ALL患儿治疗35d，缓解率80.7%。7例双系列型ALL仅1例达缓解 (14.3%)。(3)生存状态：双表型26例中20例处于缓解状态(76.9%)，双系列型7例中仅1例(14.3%)。(4)复发情况：双表型6例复发(23.1%，6/26) ，双系列7例中6例复发(85.7%)。 结论髓系抗原阳性表达在儿童ALL时不能作为预后不良的因素，但双系列白血病患儿预后差，复发率高，长期生存机会少。     

神经肽Y在惊厥性疾病患儿血浆和脑脊液中质量浓度变化及临床意义

目的观察血浆、脑脊液中神经肽Y(NPY)的质量浓度变化，探讨NPY在惊厥性疾病中的作用及临床意义。 方法选取2002 09—2003 08在陕西省人民医院儿科住院的惊厥性疾病患儿,采用放射免疫分析方法检测血浆和脑脊液中NPY的质量浓度。 结果(1)惊厥组血浆中NPY质量浓度第7天较第1天增高，差异有显著性(t=5366,P<001)，惊厥组各组第7天与第1天自身比较差异有显著性(t=3509,3480,3379,P<005)；脑炎无惊厥组第7天较第1天增高，但差异无显著性(t=1056,P=0309)；惊厥组与脑炎无惊厥组、对照组相比,差异有显著性(t=5728,P<001；t=6990,P<0001)，脑炎无惊厥组与对照组比较差异无显著性意义(t=0987,P>005)，惊厥组各组之间差异也有显著性(F=34674,P<001)。(2)惊厥组脑脊液中NPY质量浓度与脑炎无惊厥组、对照组比较差异有显著性(t=7830，5699，P<0001)，脑炎无惊厥组与对照组比较差异无显著性(t=0112，P=0911)，惊厥组各组之间差异也有显著性(F=68931，P<001)。(3)血浆和脑脊液中NPY质量浓度与惊厥发作次数有关(P<005),与性别、年龄、体重无显着相关，P均大于005;第1、7天血浆NPY质量浓度与第1天脑脊液NPY质量浓度有显着正相关性(r=0954，P<0001;r=0950，P<0001)。 结论(1)惊厥性疾病患儿血浆、脑脊液中NPY质量浓度均增高，与惊厥发作次数有关，但在惊厥发生的不同阶段质量浓度不同。(2)血浆与脑脊液中NPY质量浓度呈正相关性，血浆中的质量浓度能间接反映脑脊液质量浓度。提示动态监测NPY质量浓度可能有助于对惊厥的临床诊断、治疗及预后的判断。     

Does vitamin D deficiency affect placental inflammation or infections among very low birth weight infants?

Objective: Examine the association between placental inflammation and neonatal infections, and 25OH vitamin D (25OH D) levels at birth among very low birth weight infants (VLBWI).

Study design: Serum 25OH D levels were measured in 89 VLBWI (≤1250?g) and 47 mothers on day one, and in 78 infants on day 21. Placentas were examined for maternal and fetal inflammation. Infants were divided into deficient (≤10?ng/ml) and adequate (>10?ng/ml) groups based on 25OH D levels on day 1.

Results: Mean?±?SD maternal levels of 25OH D (21?±?9?ng/ml) correlated with infants’ levels (15?±?8?ng/ml), (p?p?=?.011). Infants’ 25OH D levels rose significantly by day 21 (p?p?>?.05). Logistic regression analyses revealed no association between deficient 25OH D levels and the odds of maternal or fetal inflammation or other infections. Levels of 25OH D did not correlate with severity of placental inflammation.

Conclusions: Deficient levels of 25OH D at birth are not associated with the occurrence of placental inflammation or neonatal infections among VLBWI.     

干/髓系免疫表型对儿童T-急性淋巴细胞白血病预后影响研究

探讨干/髓系表面抗原标记对儿童T-急性淋巴细胞白血病的预后影响。方法　回顾性分析2002-01-01至2008-12-31上海交通大学医学院附属儿童医学中心收诊的57例T细胞型急性淋巴细胞白血病（ALL）和伴有骨髓转移的T细胞性淋巴母细胞型非霍奇金淋巴瘤（NHL）患儿干/髓系表面抗原表达情况,将其分为T-ALL伴有髓系表达组（T/My+ALL）和无髓系表达组（T/My-ALL）,分析其与预后的相关性。结果　T/My+ALL组31例（54.6%）和T/My-ALL组26例（45.4%）,两组细胞形态学、遗传学及临床特征差异均无统计学意义（P > 0.05）;近期疗效中,泼尼松诱导实验差异无统计学意义（P > 0.05）,19d骨髓象及第1疗程完全缓解率T/My+ALL组均较T/My-ALL组低,差异有统计学意义（P 值分别为0.002和0.006）;T/My+ALL组和T/My-ALL组的初治完全缓解率分别为90.3%和96.2%,5年无事件生存率分别为35.4%和65.7%。虽结果提示T/My+ALL组预后较T/My-ALL组差,但差异无统计学意义（P = 0.209）。结论　T-ALL/NHL伴有干/髓系表面抗原表达时预后差,但未达到统计学有效意义,目前尚不能将之作为儿童T系淋巴细胞白血病的预后因素。     

母亲供髓移植治疗儿童复发非霍奇金病1例并文献复习

目的 探讨母亲供髓异基因造血干细胞移植(Allo HSCT)治疗恶性复发非霍奇金淋巴瘤的疗效和供体造血干细胞来源问题。 方法 2004年1月，第一军医大学南方医院儿科与海军总医院儿科给1例8岁男性淋巴母细胞淋巴瘤(LBL)患儿移植了母亲来源的HLA不全相合、ABO血型不合的骨髓造血干细胞。预处理选用阿糖胞苷、足叶乙苷、环磷酰胺和全身照射。预防移植物抗宿主病(GVHD)采用兔抗人T 淋巴细胞免疫球蛋白、环孢菌素A、甲氨蝶呤和CD25单克隆抗体。移植有核细胞数8.92×108/kg，单个核细胞数为1.89×108/kg，CD34细胞数为1.37×106/kg，CD3细胞数为32.9×107/kg。 结果 粒细胞绝对数>0.5×109/L的天数是移植后15d(+15d)，血小板>30×109/L的天数是+20d，+27d采用荧光原位杂交性染色体检测显示99%为供者型。+37d受者血型由O型转变为供者血型B型。患儿于+19d出现Ⅰ度急性GVHD，给予激素冲击后，口服小剂量维持治疗，渐消退，随访1年余，未发生慢性GVHD。 结论 母亲来源的骨髓造血干细胞移植对LBL有根治性治疗作用，而且在一定程度上解决造血干细胞来源问题。     

Propranolol concentrations after oral administration in term and preterm neonates

Abstract

Objective: While propranolol pharmacokinetics has been extensively studied in adults, this study reports the first evaluation of propranolol pharmacokinetics in term and preterm neonates.

Methods: Propranolol concentrations were measured in four term and 32 preterm newborns treated with oral propranolol at the dose of 0.5 or 0.25?mg/kg every 6?h by serial dried blood spots.

Results: The levels of propranolol, although with high inter-individual variability, were proportional with the administered dose. Pharmacokinetic parameters evaluated at the steady state in newborns treated with 0.5?mg/kg/6?h showed values of maximal (71.7?±?29.8?ng/mL), minimal (42.2?±?20.8?ng/mL) and average concentration (60.8?±?25.0?ng/mL), time of maximal concentration (2.6?±?0.9?h) and area under the time-concentration curve (364.7?±?150.2?ng/mL/h) similar to those observed in adults. In both dosing groups, elimination half-life was significantly longer (14.9?±?4.3 and 15.9?±?6.1?h), and apparent total body clearance (27.2?±?13.9 and 31.3?±?13.3?mL/kg/min) lower than those reported in adults, suggesting a slower metabolism in newborns. No differences were observed between newborns with different gestational age or different sex.

Conclusions: Neonates treated with propranolol-exhibited drug concentrations proportional with the dose, with significant long half-life.     

Ovarian Reserve Following Laparoscopic Ovarian Cystectomy vs Cyst Deroofing for Endometriomas

Study ObjectiveBecause laparoscopic ovarian cystectomy of endometriomas is known to adversely impact patient ovarian reserve, the search for other techniques of surgical management is ongoing. The present study was undertaken to evaluate laparoscopic cyst deroofing as a feasible alternative.Study DesignProspective, randomized clinical trial (Canadian Task Force classification I).SettingUniversity maternity hospital.PatientsWomen diagnosed with unilateral or bilateral ovarian endometriomas.InterventionsPatients were managed with either laparoscopic ovarian cystectomy or cyst deroofing.Measurements and Main ResultsA total of 122 women with endometriomas were randomized to either laparoscopic cystectomy (group 1) or laparoscopic cyst deroofing (group 2). The primary endpoint was the effect on ovarian reserve based on changes in anti-Müllerian hormone (AMH) values. At 1 month postsurgery, anti-Müllerian hormone values were significantly decreased (p < .001) from preoperative values, from 4.25 ± 0.87 ng/mL to 1.66 ± 1.02 ng/mL in group 1 and from 4.2 ± 1.69 ng/mL to 2.15 ± 1.48 ng/mL in group 2. In addition, antral follicle count and ovarian volume decreased significantly (p < .001) in both groups by 1 month postsurgery. The decreases in these 3 parameters were more significant (p < .001) in group 1 than in group 2.ConclusionLaparoscopic cyst deroofing of endometriomas appears to be a promising alternative to laparoscopic cystectomy, with less postoperative decrease in ovarian reserve; however, the higher rate of endometrioma recurrence warrants future clinical research to determine the optimal surgical management of endometriomas.