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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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??Abstract??Objective??To detect the changes of the plasma homocysteine??p-Hcy????high-sensitivity C-reactive protein??hs-CRP????1actic dehydrogenase??LDH?? and tumor specific growth factor??TSGF?? determination in patients with acute lymphocytic leukemia??ALL?? and its clinical significance. Methods??p-Hcy??enzymatic cycling assay????hs-CRP??turbidimetry respectively????LDH??automatic analyzer?? and TSGF ??with biochemistry??before treatment and after remission were measured between ALL children and normal children in Department of Pediatrics??Zhengzhou university's First Hospital from Jan. 2009 to Jul. 2010. Results??Before chemotherapy and after complete remission??p-Hcy??hs-CRP??TSGF and LDH levels in the ALL-CR patients were significantly higher than those in the controls??P < 0.01?? .The level of p-Hcy??hs-CRP??TSGF and LDH after complete remission was lower than that before treatment??P < 0.05??.The level of p-Hcy??hs-CRP??TSGF and LDH after cancer recurrence was higher than that of normal children and after complete remission of ALL-CR children??P < 0.05??.p-Hcy level was significantly correlated with LDH??TSGF and hs-CRP level before chemotherapy of ALL-CR children??P < 0.01??. Conclusion??p-Hcy??hs-CRP??TSGF and LDH can be used as a reference marker in diagnosis??monitoring patients’condition and prognosis evaluation in acute lymphocytic leukemia of children.     

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??Abstract??Objective??To investigate the expression of Notch1 protein in the children with acute leukemia ??AL?? and to explore the relationship of Notch1 expression with immunophenotype and therapeutic effect. Methods??The Notch1 protein of bone marrow smears was examined by immunohistochemical assay in 49 newly diagnosed children with acute leukemia and 20 control cases without malignant hematologic disease. Immunophenotype of leukemic cells was performed by flow cytometry. The standard chemotherapy regimens were taken to treat the patients. Results??The expression rate of Notch1 protein in T-cell acute lymphoblastic leukemia ??T-ALL?? ??77.8%?? was much higher than that of B-cell acute lymphoblastic leukemia ??B-ALL?? ??31.0%?? P < 0.05?? as well as the control group ????15.0%?? P < 0.05??. Notch1 protein expression in acute non-lymphoblastic leukemia ??ANLL?? ??63.6%?? was significantly higher than that in the control group ??15.0%?? P < 0.05??. Additionally?? in T-ALL and ANLL groups?? there was no correlation between the expression of Notch1 protein and treatment effect. However?? in B-ALL patients with more than 6 months of follow-up?? Notch1 expression rates in the favorable group??complete remission without relapse?? and the unfavorable group ??death or relapse?? were 21.4%and 80.0% respectively ??P < 0.05??. Conclusion??Notch1 expression in childhood leukemia is immunophenotype-specific. Though the expression of Notch1 protein in B-ALL is not high?? it may be a poor prognosis factor.     

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??Expression and clinical significance of peripheral Th17 cell in children with acute leukemia.????WU Xiao-yan??JIN Run-ming??YU Hui??WANG Fang??XIA Yi. Pediatrics Department of Union Hospital??Tongji Medical School??Huazhong University of Science and Technology??Wuhan 430022??China
Abstract??Objective??To study the relationship between peripheral blood Th17 cells and the occurrence and status of disease of children with acute leukemia. Methods??From Aug. 2008 to Dec. 2009 in Pediatrics Department of Union Hospital??Tongji Medical School Huazhong University of Science and Technology?? 42 children with acute leukemia and 18 healthy children were divided into non-remission group ??Group A??n = 23????remission group ??Group B??n = 19?? and control group ??Group C??n = 18??. Concentration of IL-17 and IL-23 in the peripheral blood monocytes culture supernatant after stimulated with anti-CD3 and anti-CD28 mAb were determined with ELISA. Expression of CD4+ IL-17+ cells was determined with flow cytometry. Results??The concentrations of IL-17 and IL-23 in Group A and Group B were much lower than those in Group C??P < 0.05??. The level of IL-17 in Group A was lower than that in Group B??P < 0.05????while the difference of IL-23 concentration between Group A and Group B was not significant??P > 0.05??. The expression level of Th17 in Group A and Group B were much lower than those in Group C??P < 0.05????and the difference of Th17 expression level between Group A and Group B was significant??P < 0.05??. Conclusion??Th17 cells might play important role in the occurrence and development of children acute leukemia through secreting IL-17??and their function level can partly reflect the status of leukemia and evaluating the risks of infection in children with leukemia.     

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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XH99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AMLXH99、ALLXH99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KaplanMeier方法;生存率之间的比较采用logrank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BALL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)%],logrank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)%],logrank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。     

同源盒基因HOXA9在儿童急性白血病中的表达及其临床意义

目的：研究同源盒基因HOXA9在儿童急性白血病（AL）患儿骨髓单个核细胞中的表达,并探讨其临床意义。方法：采用RT-PCR方法检测46例不同时期AL患儿HOXA9 mRNA的表达水平,以15例特发性血小板减少性紫癜（ITP）患儿作为对照。结果：46例AL患儿（52份骨髓标本）HOXA9基因阳性表达率为63%,其中急性髓细胞白血病(AML)组阳性表达率（86%）明显高于急性淋巴细胞白血病（ALL）组（35%）及对照组（13%）（P<0.05）; AML组HOXA9 mRNA表达水平明显高于ALL组及对照组（P<0.05）。HOXA9在各型儿童AML中表达不同,mRNA相对表达水平依次为：M5型＞M4型＞M1和（或）M2型,而在M3型中未检测到表达。HOXA9在AML患儿高危组中的阳性表达水平较高。AML患儿初治组HOXA9基因阳性表达率及mRNA水平明显高于缓解组和对照组（P<0.05）,而缓解组与对照组比较差异无统计学意义;未缓解组HOXA9基因表达显著高于缓解组和对照组（P<0.05）。结论：HOXA9基因高表达与AL的发生相关;AML患儿HOXA9基因表达水平明显高于ALL患儿。HOXA9基因高表达者与白血病危险程度有关,且提示预后不良。因此,HOXA9基因有望成为儿童AL诊断、治疗及判断预后的一个靶点。     

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??Abstract??Objective??To investigate the working memory profiles in children with Attention Deficit Hyperactivity Disorder ??ADHD??. Methods??We investigated 39 children with ADHD ??DSM-IV???? including ADHD combined type??ADHD-C????n = 14????ADHD inattentive type ??ADHD-I?? ??n = 14?? and ADHD hyperactive/ impulsive type??ADHD-HI?? ??n = 11???? and 19 normal controls matched for age?? sex?? handedness?? and intelligence during a working memory task. Prefrontal brain activity was measured by concentration changes of oxygenated haemoglobin ??oxyHb??. Functional near-infrared spectroscopy ??NIRS?? was used to obtain the oxyHb. Results The results of working memory task?? ??1??ADHD children had more total errors ??3.51±3.025?? and comparison errors??2.41±2.149??than controls??P < 0.05??.??2??There was no significant difference in reaction time between ADHD and control group ??P??0.05??.??3??ADHD-C patients had more reaction errors??1.71±2.016??than ADHD-HI’s??P < 0.05?? ??on comparison errors??ADHD-HI children had more than ADHD-C’s??P < 0.05????and ADHD-I children also had more than ADHD-C’s??P < 0.05??.But there was no significant difference between ADHD-I and ADHD-HI group.??4?? ADHD-HI group had longer reaction time than ADHD-I’s ??P < 0.05??.The results of NIRS?? ??1?? There was no significant difference in concentration changes of oxygenated haemoglobin ??oxyHb?? between ADHD and control group when they performed working memory task.??2??Concentration levels of oxyHb in ADHD-C decreased in the left-down prefrontal cortex area during performing reaction commission than ADHD-I???-2.46±9.62 ??×10-4 vs.??10.47±14.18??×10-4 P < 0.05??.Conclusion??There are significant deficiencies in working memory in children with ADHD??ADHD subtypes differ in working memory profile.     

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??Objective??To study the family burden of children with thalassemia and their family members in Guizhou province. Methods??Family burden scale of disease??FBS?? and basic situation questionnaire were used to investigate 114 family members of children with thalassemia aged 0??14 who were diagnosed and treated in the Department of Pediatric Hematology and Oncology??Affiliated Hospital of Guizhou Medical University??from December 2016 to April 2018.
Results??The total score of FBS of family members of children with thalassemia was ??21.53±7.89??. The standardization scores of each dimension from high to low were family economic burden ??1.389±0.330????family entertainment activities ??0.914±0.530????family relationship ??0.807±0.530????mental health of family members??0.680±0.570????daily activities of the family ??0.679±0.390?? and physical fitness of family members ??0.442±0.440??. Univariate analysis??t/F test showed that the diagnosis typing of children with thalassemia??iron overload??medical insurance and monthly family income were the main factors affecting FBS??P??0.05??. Multivariate linear regression analysis showed that the effect of diagnostic typing??medical insurance??and monthly family income on FBS was statistically significant??P??0.05??. Conclusion??The family burden of children with thalassemia is widespread. It’s time that effective measures should be take to slove the problem according to family burden situation and its influencing factors.     

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目的探讨连续性静-静脉血液透析/滤过(CVVHDF)对小儿急性肝功能衰竭的治疗作用。方法 2009年5月至2010年5月期间,采用CVVHDF辅助治疗上海交通大学附属儿童医院重症监护病房(PICU)收治的急性肝功能衰竭患儿共5例。其中男2例,女3例。年龄10个月至5岁。入院时发病时间3～14d。药物性肝损(对乙酰氨基酚)3例,严重脓毒症合并肝损1例,阑尾炎术后门静脉炎引起的肝损1例。肝性脑病分级:2级1例,3级2例,4级2例。观察治疗前后病情及肝功能指标,并观察处理并发症。结果 CVVHDF治疗开始时间为入院后(2～24h),治疗时间为(24～144h),5例患儿中治愈3例,自动出院1例,死亡1例。CVVHDF治疗后12h,丙氨酸转氨酶(ALT)从(3888.76±2373.60)U/L下降至(3284.80±1974.80)U/L,治疗后24～48h继续下降,差异有统计学意义(F=3.58,P<0.05);CVVHDF治疗后12h,血氨从(209.00±53.61)μmol/L下降至(158.80±60.93)μmol/L,差异有统计学意义,以后继续好转(F=3.75,P<0.05)。CVVHDF治疗后12...