目的:探讨儿童黑热病继发噬血细胞综合征的临床特点及诊治策略.方法:回顾性分析了2例黑热病继发噬血细胞综合征的临床资料,复习相关文献.结果:2例患儿均有发热、脾大、3系减少、甘油三酯增高、纤维蛋白原降低、铁蛋白增高及骨髓涂片见到利什曼原虫和噬血细胞现象,黑热病rK39免疫层析试验为阳性,确诊为黑热病继发噬血细胞综合征,予足量锑剂治愈.结论:儿童黑热病继发噬血细胞综合征应早诊断、早治疗,可明确改善其远期疗效及预后.

1 病例资料患儿王某,男,9岁2月,2020年02月08日因“间断发热3天,咽痛、血小板减少2天”入院.入院前5天无明显诱因出现发热,Tmax38.0℃,自服退热药物,疗效欠佳,仍间断发热,就诊凉州区医院,查血常规:WBC 4.09×109/L,ANC 0.86×109/L, HGB 106g/L, PLT 48×109/L;CRP:21.9mg/L;骨髓穿刺示:增生明显活跃,粒系占47％,红系占30.0％,血小板少见;考虑脾功能亢进、免疫性血小板减少症.给予“利巴韦林、头孢曲松钠(罗氏芬)”等药物治疗,期间患者发热1次,Tmax38.5℃,之后未再出现发热,偶有鼻腔渗血.入院前1天血常规:WBC 1.27×109/L,ANC 0.86×109/L,HGB 106g/L,PLT 21×109/L.既往体健.入院查体:T 36.3℃,P 96次/分,R 22次/分,BP 91/58mmHg,体重24kg.神志清楚,精神稍差,睑结膜略苍白,咽部充血,扁桃体无肿大;浅表淋巴结未触及肿大.

1病例资料 患儿男,4岁,2017年7月11日因“间断发热半月”收住甘肃省妇幼保健院.近期无服用药物史,无结核等传染病接触史,无生食食物史,无食物及药物过敏史.入院体格检查:精神状态差,全身未见出血点,浅表淋巴结未触及肿大,心肺查体无异常.腹膨隆,腹壁静脉曲张,肝脾肿大,脾肋下约4cm,肝下缘平髂前上棘,质韧.双下肢未见浮肿.实验室检查:血常规:外周血白细胞总数3.7×109/L,淋巴细胞57.1％,中性粒细胞31.8％,红细胞计数3.7×1012/L,血红蛋白91g/L,血小板64×109/L.血涂片未见异常.血生化:乳酸脱氢酶2337U/L,谷丙转氨酶138.6U/L,谷草转氨酶198.4U/L;降钙素原1.61 pg/mL;C-反应蛋白12.53mg/L;血清铁蛋白＞2000 ng/mL(未稀释);血脂检查提示甘油三酯4.33mmol/L,余正常;凝血检查提示纤维蛋白原2.09g/L,余正常;EBV血清学抗体:VCA-IgM阴性,VCA-IgG阳性,EA-IgG阴性,NA-IgG阳性;血浆EBV-DNA:阴性;血清甲胎蛋白:242ng/mL;骨髓细胞形态学结果:可见噬血细胞;另可见分类不明细胞2.5％,胞体小,可见伪足及拉尾现象,疑为病毒感染.拟再次骨穿行免疫分型,家属拒绝.噬血细胞综合征易感基因检查(海斯特):PRF1、Unc13D、STX11、STXBP2;RAB27A、CHS1/LYST、AP3β1;SH2D1A、BIRC4均阴性.NK细胞活性及sCD25检查(海斯特)均正常.

目的:探究苦参碱联合化疗药物对人急性髓系白血病HL-60细胞增殖及侵袭力的影响.方法:体外培养人急性髓系白血病HL-60细胞,苦参碱单药组以1.0 g/L苦参碱干预,三氧化二砷单药组以3μmol/L三氧化二砷处理,联合组以终浓度为1.0 g/L的苦参碱+3μmol/L三氧化二砷处理,空白组以等量生理盐水处理.MTT法检测HL-60细胞增殖情况,ranswell小室实验检测HL-60侵袭力,蛋白免疫印迹法检测HL-60细胞MMPs蛋白表达情况.结果:苦参碱单药组、三氧化二砷单药组、联合组24 h PIR分别为(18.54±2.54)％,(29.54±2.62)％,(41.45±3.72)％,48 h PIR分别为(27.46±2.55)％,(38.93±3.76)％,(45.64±3.54)％,3组内HL-60的PIR均随着培养时间的增加而增加,其中联合组24 h、48 h PIR均高于同时间点的苦参碱单药组、三氧化二砷单药组,差异有统计学意义(P<0.05).空白组、苦参碱单药组、三氧化二砷单药组、联合组侵袭细胞分别为(127.64±23.93)个、(94.24±15.23)个、(81.23±12.21)个、(75.13±10.69)个,相对侵袭指数分别为(100.00±0.00)％、(73.44±0.34)％、(62.47±0.15)％、(58.54±0.36)％;MMP-2蛋白相对表达量分别为(0.91±0.05)、(0.39±0.04)、(0.23±0.04)、(0.14±0.03);MMP-9蛋白相对表达量分别为(0.62±0.05)、(0.31±0.05)、(0.27±0.04)、(0.16±0.02).与空白组比较,苦参碱单药组、三氧化二砷单药组、联合组侵袭细胞数量显著减少,相对侵袭指数及细胞中MMP-2、MMP-9相对表达量降低,且联合组均低于苦参碱单药组、三氧化二砷单药组,差异有统计学意义(P<0.05).结论:苦参碱联合三氧化二砷通过下调MMP-2、MMP-9蛋白表达有效抑制人急性髓系白血病HL-60细胞增殖及侵袭.

目的:评价SLC22A1(rs628031、rs683369)和细胞色素P450(CYP)3A5(rs776746)基因多态性与伊马替尼治疗慢性髓性白血病的相关性.方法:以"有机阴离子转运体""细胞色素""基因多态性""伊马替尼""慢性粒细胞白血病"及"SLC22A1""CYP3A5"为中文检索词,以"SLC22A1""CYP3A5""Cytochrome""Polymorphism""Imatinib""Chronic myeloid leukemia"为英文检索词,计算机检索PubMed、Embase、The Cochrane Library、中国生物医学文献数据库、中国知网学术总库、万方数据库、中文科技期刊数据库,收集符合纳入标准的SLC22A1(rs628031、rs683369)和CYP3A5(rs776746)基因多态性与伊马替尼治疗慢性髓性白血病疗效的病例对照研究,检索时限均为从建库起至2018年1月,由两位研究者按照纳入与排除标准独立筛选文献、提取资料和评价质量后,采用Rev Man 5.3软件对SLC22A1(rs628031、rs683369)和CYP3A5(rs776746)基因多态性与主要分子学反应率(MMR)、完全细胞遗传学反应率(CCyR)的关系进行Meta分析.结果:共纳入13项病例对照研究,合计1707例患者.Meta分析结果显示,SLC22A1(rs628031)GA+AA基因型患者MMR显著低于GG基因型[OR=0.58,95％CI(0.41,0.83),P<0.01];SLC22A1(rs683369)CG+GG基因型患者MMR显著低于CC基因型[OR=0.64,95％CI(0.42,0.96),P=0.03];CYP3A5(rs776746)AG+GG基因型患者CCyR显著高于AA基因型[OR=2.43,95％CI(1.12,5.27),P=0.02].结论:SLC22A1和CYP3A5基因多态性可影响伊马替尼对慢性髓性白血病的治疗结局,二者可作为伊马替尼的潜在疗效预测指标.

Objective:To screen the markers of thrombophilia in patients with repeated embryo implantation failure(RIF)to assess the pathogenesis and risk factors for providing effective precautions and treatments.Methods:The case group comprised of 90 consecutive women with three or more previously failed IVF-embryo transfer cycles,and the control group included women with successful pregnancy after the first IVF-embryo transfer cycle.All women were tested for the polymorphism of plasma methylenetetrahydrofolate reductase(MTHFR)polymorphism(C677T),the activity of protein S & protein C and antithrombin Ⅲ,anticardiolipin antibody(ACL-IgG/IgM)and antibodies against β2 glycoprotein Ⅰ by application of sequencing technology,blood coagulation,chromogenic substrate method and enzyme-linked immunosorbent assay respectively.Results:MTHFR C677T T/T genotype in the case group was significantly higher than that of the control group(43.3％ vs.23.3％,P＜0.05),and the correlation coefficient of Cramer V was 0.574(P＜ 0.05).The positive rate of ACL-IgG/IgM and anti beta 2-GPI,the activity of protein S & protein C and antithrombin were all not significantly different between the two groups(P＞0.05).Conclusions:The T/T genotype of MTHFR C677T site may be related to the occurrence of RIF,which can be used as a screening index of thrombophilia in IVF patients in this region.

目的 系统评价遗传性易栓症(IT)与不明原因反复种植失败(RIF)的关系. 方法 计算机检索Cochrane Library、PubMed、EMbase、中国生物医学文献数据库、万方数据库等,收集符合纳入标准的研究,检索时限均为从建库至2014年5月,并追溯纳入研究的参考文献和手工检索相关会议资料.由两位研究者按照纳入与排除标准独立筛选文献、提取资料和评价质量后,采用RevMan 5.1软件进行Meta分析.病例组为不明原因RIF患者,暴露因素为IT. 结果 共纳入8个病例对照研究,共计1 290例患者,meta分析结果显示,抗凝蛋白缺陷(抗凝血酶、蛋白S、蛋白C)、凝血酶原突变(G20210A、亚甲基四氢叶酸还原酶(C677T)均与不明原因RIF无显著关联(P＞0.05);病例组与对照组因子V Leiden(FVL)突变率的差异与不明原因RIF明显关联,OR=3.09,95％CI(1.80,5.34),P＜0.01).基因关联性分析仅发现病例组与对照组间FVL突变杂合型及纯合型均与不明原因RIF显著相关,分别为OR杂合型＝2.78,95％CI(1.46,5.28),P＝0.002;OR纯合型＝9.47,95％CI(1.18,75.88),P=0.03. 结论 IT可能是不明原因RIF的独立危险因素,但FVL突变在我国发生率很低,针对我国人群,尚需进行IT与不明原因RIF关系的实验室研究,探讨IT在不明原因RIF中的致病机制.

目的 探讨血清抑制素B(INHB)与卵泡刺激素(FSH)对非阻塞性无精子症睾丸精子抽吸(TESE)成功的预测价值.方法 通过国内外主要数据库检索到符合分析要求的9篇文献,采用MetaDiSc1.4软件进行Meta分析,结果 INHB对TESE成功获取精子预测的敏感度低于FSH[0.69 (95％CI 0.64～0.73),0.75(95％CI 0.70～0.79)],特异度高于FSH[0.78(95％CI 0.75～0.82),0.66(95％CI 0.61～0.70)],INHB的受试者工作特征曲线下面积及诊断比值比均高于FSH.结论 INHB与FSH均不能独立作为TESE成功获取精子预测指标,多个指标联合检测是成为今后研究的方向.

目的 系统评价抗磷脂抗体(APA)与体外受精-胚胎移植(IVF-ET)结局之间的关系. 方法 计算机检索Cochrane Library、MEDLINE、EMbase、中国生物医学文献数据库、万方数据库等,收集符合纳入标准的研究,检索时限均为从建库至201 3年3月,并追溯纳入研究的参考文献和手工检索相关会议资料.由两位研究者按照纳入与排除标准独立筛选文献、提取资料和评价质量后,采用RevMan 5.0软件进行Meta分析. 结果 共纳入1 1 60例研究对象.结果显示:抗磷脂抗体(APA)与IVF-ET后胚胎种植失败率、临床妊娠率、活产率、流产率之间未发现明显关联(P＞0.05),合并OR值及95％CI分别为0.98,(0.78,1.24);1.01,(0.81,1.26);1.02,(0.80,1.29);1.13,(0.60,2.11). 结论 根据现有的临床证据,APA与IVF-ET结局间无显著联系,对拟行IVF-ET的妇女是否常规行APA检测尚需进一步研究,是否对仅APA阳性而无相关临床症状的患者进行治疗有待商榷,对APA的作用机制需进行深入研究.

目的:系统评价唑来膦酸(zoledronic acid,ZOL)对临床早期乳腺癌(early breast cancer,EBC)患者预后的影响.方法:计算机检索Cochrane Library、PubMed、EMbase、中国生物医学文献数据库、中国期刊全文数据库、维普和万方数据库等资源,收集符合纳入标准的研究,检索时限均为从建库至2013-03,并追溯纳入研究的参考文献和手工检索相关会议资料.由2位研究者按照纳入与排除标准独立筛选文献、提取资料和评价质量后,采用RevMan 5.0软件进行Meta分析.结果:共纳入8个随机对照研究,包括7 540例患者.Meta分析结果显示,与对照组相比,ZOL可以显著提高EBC患者3年无病生存率(OR=1.67,95％CI:1.10～2.55,P=0.02)及5年总生存率(OR=1.19,95％CI:1.02～1.40,P=0.03),显著降低5年骨转移发生率(OR=0.79,95％CI:0.63～0.98,P=0.03);低雌激素水平亚组分析中,ZOL可显著提高5年总生存率(OR=1.39,95％CI:1.08～1.80,P=0.01)及降低复发率(OR=0.66,95％CI:0.52～0.84,P＜0.01).结论:ZOL可能具有潜在的抗肿瘤效应,可提高临床EBC患者远期生存率,降低骨转移率,在低雌激素水平的临床EBC患者中效果更为明显,但尚需大样本高质量的RCT进一步验证.

Objective:To retrieve the best evidence-based treatment modality for a patient with Crohn's disease during pregnancy.Methods:According to the principles of patient intervention comparison outcome(PICO),studies on Crohn's disease during pregnancy were retrieved from MEDLINE,Embase,CBMdisc,Cochrane Library,CNKI,VIP and Wanfang databases.All studies were critically assessed and analyzed by meta-analysis using the RevMan 5.0 software.A treatment modality for Crohn's disease during pregnancy was established according to the preference of the patient.Results:A total of 7 randomized controlled trials,7 systematic reviews and 3 study on clinical guidelines were identified.The treatment plan was developed accordingly.Conclusion:The individualized treatment plan based on the high quality evidence and patients condition is optimal for the short-term treatment of Crohn's disease during pregnancy.However,the long-term prognostic benefits need to be confirmed by continuing follow-up.

Objective:To assess the effectiveness of intravenous hydroxyethyl starch(HES) administration for the prevention of ovarian hyperstimulation syndrome(OHSS).Methods:All randomized controlled trials (RCTs) on HES for the prevention of OHSS were retrieved from databases including Cochrane Library,MEDLINE,Embase,CBMdisc,CNKI,VIP and Wanfang databases update to Sep.2012.Information was collected by two researchers independently.The quality of included RCTs was assessed according to the Cochrane Handbook for Systematic Reviews of Interventions,and the Cochrane Collaboration′s software RevMan 5.0 was used for meta-analysis.Results:4 RCTs involving 666 patients were included.The incidence of OHSS and severe OHSS in the HES group (OR=0.18,95%CI 0.10 to 0.32,P=0.000, OR=0.15,95%CI 0.05 to 0.48,P=0.001,respectively) was significantly reducted compared to the control group.The differences of the pregnancy rate between the two groups has no statiscal significance (OR=1.02,95%CI 0.60 to 1.73,P=0.94).Conclusions:Current evidence shows that HES markedly decreases the incidence of OHSS,especially the severe OHSS,and do not affect the pregnancy rate.However,considering the limitation of the methodological quality and sample size of the included studies,more high-quality,large-scale RCTs are still needed to confirm this conclusion.

OBJECTIVE To assess the clinic effectiveness of manual lymph drainage method combined intermittent pneumatic compression for breast cancer postoperative limb rehabilitation.METHODS 120 patients after modified radical mastectomy were included.They were randomly divided into control group and treatment group.Control group receive routine nursing care,including intermittent pneumatic compression,psychological counseling,health education and functional exercise guidance,etc.Treatment group were added manual lymph drainage method.Measuring in chemotherapy cycle upper extremity edema,limb muscle,pain score,compared between groups.RESULTS Control group and treatment group lymphedema incidence were 16%,4% respectively(P<0.05).Two groups patients with shoulder joint activity and limb muscle strength is statistically significant.Two groups patients before chemotherapy cycle 3 pain score were statistically different.CONCLUSION Manual lymph drainage method combined intermittent pneumatic compression for breast cancer postoperative limb rehabilitation is effective.

OBJECTIVE To study the clinical characteristics of hepatitis-associated aplastic anemia (HAAA). A retrospective analysis was conducted among 8 cases of established HAAA in light of the clinical and laboratory findings and the patient outcomes. METHODS Serum samples from 8 patients with HAAA were tested for the antibodies to hepatitis viruses A, B, C, D, E and CMV, and 7 patients showed negative serological results while only one was positive for HBsAg. The percentage of CD4(+) cells was significantly lowered while the percentage of CD8(+) cells significantly increased to result in a lowered ratio of CD4(+)/CD8(+) cells in these HAAA patients. A shift in the Th1/Th2 and Tc1/Tc2 balance to a Th1 and Tc1 dominance was noted. The percentage of Treg cells was obviously decreased. Significant increments were found in the serum levels of interleukin-2, THF-α and interferon-γ. Three of the patients responded to immunosuppresssive treatment; one patient achieved a complete remission, two had a partial remission, and five failed to respond to the therapy and died. CONCLUSIONS HAAA may not have an obvious association with viral infections as by hepatitis virus A, B, C, D, or E, and its pathogenesis often involves abnormalities of the T cell immunity. Commonly with a poor prognosis, HAAA is associated with a high mortality rate.