特发性血小板减少性紫癜与巨细胞病毒、EB病毒感染相关性

目的：探讨小儿特发性血小板减少性紫癜（ITP）与巨细胞病毒、EB病毒感染的关系。方法：实验组：48例确诊断为ITP患儿,对照组：44例同期呼吸道感染患儿,应用酶联免疫吸附法（ELISA）对两组小儿外周血进行巨细胞病毒IgM抗体（HCMV-IgM）、EB病毒感染IgM抗体（EB-IgM）检测。结果：48例ITP患儿中HCMV-IgM抗体阳性者20例,阳性率为41.67%,明显高于对照组,两组之间差异有显著性（P〈0.01）;EBV-IgM抗体阳性者14例,阳性率为29.17%,明显高于正常对照组,两组之间差异有显著性（P〈0.05）。结论：1、巨细胞病毒感染是引起特发性血小板减少性紫癜的重要原因之一,且通过临床观察巨细胞病毒感染引起的ITP患儿病情重,病程长,治疗时间长,转为慢性ITP的可能性大;2、EB病毒感染可能是引起特发性血小板减少性紫癜的原因之一,并且EB病毒感染引起的特发性血小板减少性紫癜病情也偏重。     

肝细胞生长因子和基质金属蛋白酶-9在过敏性紫癜中表达的意义

目的:探讨肝细胞生长因子(HGF)和基质金属蛋白酶-9(MMP-9)在儿童过敏性紫癜(HSP)中表达的意义,探寻肾脏损害预测的敏感指标。方法:选取HSP与过敏性紫癜肾炎(HSPN)患儿各30例,并选取同期体检的健康儿童30名作为对照组,采用酶联免疫吸附试验法(ELISA)检测3组血清和尿液中的HGF及血清MMP-9含量,并采用比浊法检测24 h尿蛋白,分析HGF、MMP-9与24 h尿蛋白的相关性。结果:HSP组、HSPN组血清、尿液HGF,血清MMP-9及尿蛋白定量均高于对照组(P0.05);HSPN组中Ⅱ~Ⅳ期患者血清HGF及Ⅰ~Ⅳ期尿液HGF、Ⅲ~Ⅳ期MMP-9及Ⅰ~Ⅳ期尿蛋白定量均高于HSP组(P0.05);血清及尿液HGF与血清MMP-9无明显相关性(r=0.014,0.027,P0.05);血清HGF与尿蛋白定量无明显相关性(r=0.032,P0.05);尿液HGF、血清MMP-9与尿蛋白定量均呈正相关(r=0.412、0.302,P0.05)。结论:尿液HGF、MMP-9均参与HSP、HSPN病情的发展,尿液HGF、MMP-9水平的监测有助于评估HSPN的病情及预后,但HGF与MMP-9之间无明显相关性。     

经食道超声心动图评估特发性房颤左心房左心耳的价值

目的:研究经食道超声心动图(TEE)评估特发性房颤左心房左心耳的临床价值。方法:选择自2015年1月到2016年8月在医院接受诊治的特发性房颤患者100例纳入本次研究,阵发性房颤92例,记为阵发性房颤组;持续性房颤8例,记为持续性房颤组。另选同期在医院进行健康体检的心功能正常志愿者90例作为对照组。利用TEE对受试者进行检查,对比房颤组与对照组的左心房及左心耳参数,是否含有自发性显影(LASEC)的房颤患者的左心房及左心耳参数,利用TEE分析对房颤患者的预后情况。结果:阵发性房颤组左心房的前后径和左右径,左心耳血流最大的排空速度(Lev)均明显小于对照组,左心耳的面积变化率及最大的充盈速度(Lfv)均明显大于对照组,差异有统计学意义(P0.05)。持续性房颤组左心房的前后径和左右径均明显大于对照组,左心耳的面积变化率、Lev及Lfv均明显小于对照组,差异有统计学意义(P0.05)。阵发性房颤组左心房的前后径和左右径均明显小于持续性房颤组,左心耳的面积变化率、Lev及Lfv均明显大于持续性房颤组,差异有统计学意义(P0.05)。有LASEC者左心房的前后径和左右径均明显大于无LASEC者,左心耳的面积变化率、Lev及Lfv均明显小于无LASEC者,差异有统计学意义(P0.05)。100例房颤患者中发现34例LASEC,占34.00%,其中有18例患者合并有左心耳血栓,占18.00%。总计有66例患者接受导管射频消融疗法,占66.00%,均未在术中及术后7d内出现血栓及栓塞并发症。结论:利用TEE对特发性房颤的患者左心房及左心耳进行评估,有利于更好的辅助患者的临床治疗,值得重视。     

环孢素A对难治性免疫性血小板减少性紫癜的辅助治疗

目的探讨环孢素A辅助治疗难治性免疫性血小板减少性紫癜(ITP)的临床疗效及对患者血小板计数和细胞因子的影响。方法选择2013年1月至2016年12月期间浙江省金华市人民医院收治的难治性ITP患者78例。按照随机数字表分为观察组39例与对照组39例。对照组患者给予利妥昔单抗治疗,观察组在对照组基础上结合环孢素A辅助治疗,两组患者疗程均为3个月。比较两组患者的疗效,治疗前后血小板计数变化,血小板恢复正常时间,治疗前后细胞因子变化及不良反应发生情况。结果观察组患者治疗总有效率(92.31%)高于对照组(69.23%),差异有统计学意义(χ2=6.6857,P0.05);两组患者治疗后血小板计数增加,观察组患者治疗后血小板计数高于对照组、血小板恢复正常时间低于对照组,差异均有统计学意义(t=20.6717、8.1189,P0.05);两组患者治疗后IFN-γ水平较治疗前降低,IL-4、IL-10水平升高,差异有统计学意义(观察组:t=22.9522、11.7500、11.9032,对照组:t=9.8537、7.2250、6.7012,P0.05),且治疗后观察组患者IFN-γ水平低于对照组,IL-4、IL-10水平高于对照组,差异有统计学意义(t=13.0096、3.8277、4.7989,P0.05);两组患者不良反应发生率比较差异无统计学意义(χ2=0.0000,P0.05)。结论环孢素A辅助治疗难治性ITP患者疗效显著,且可增加患者血小板数量,降低IFN-γ水平,增加IL-4、IL-10水平,提高患者免疫力。     

The Study of Cu and Zn Serum Levels in Idiopathic Dilated Cardiomyopathy (IDCMP) Patients and its Comparison with Healthy Volunteers

Changes in the cupper (Cu) and zinc (Zn) concentrations have been reported previously in idiopathic dilated cardiomyopathy (IDCMP). As a result of controversial results, the aim of this study was to compare the Zn and Cu concentrations and Zn/Cu ratio of IDCMP patients to healthy volunteers. In addition, the correlation of Cu and Zn levels with age has been evaluated. The study population consisted of 18 IDCMP patients and 27 healthy volunteers. IDCMP patients had normal angiography with echocardiography supporting cardiomyopathy without pericardial and valvular diseases. Exclusion criteria were renal or hepatic insufficiency, alcohol usage, and intake of supplements containing Cu or Zn within 1 week ago. Cu and Zn levels have been assayed with atomic absorption spectrophotometry. Statistical analysis was performed with SPSS 10 software with independent sample t test for comparing the level of Cu and Zn of IDCMP patients with normal subjects and Pearson correlation to determine the correlation between numeric data. P < 0.05 was considered as significant differences. There was a trend for a lower Zn level in IDCMP patients compared to healthy volunteers. (0.97 ± 0.25 mg/l vs. 1.12 ± 0.42 mg/l, respectively). The mean Cu levels of IDCMP and normal subjects were 1.33 ± 0.20 mg/l and 1.31 ± 0.23 mg/l, respectively. There was a significant difference in Zn/Cu ratio among patients based on the NYHA classification of heart failure (P = 0.003). Age was negatively correlated with Zn levels in IDCMP group (P = 0.037) and positively with Cu levels in healthy volunteers (P = 0.012). A lower Zn level in IDCMP patients compared to healthy volunteers and specially a significant difference in Zn/Cu ratio of patients based on their NYHA classification would suggest a critical role of zinc and Cu imbalance in development of IDCMP.     

特发性肺纤维化患者血清血管生成素-2、潜在转化生长因子结合蛋白2、趋化因子配体14水平与预后的关系研究

摘要 目的：研究特发性肺纤维化（IPF）患者血清血管生成素-2（Ang-2）、潜在转化生长因子结合蛋白2（LTBP2）、趋化因子配体14（CXCL14）水平与预后的关系。方法：选取2019年3月～2020年3月河北医科大学第二医院东院区呼吸内科收治的100例IPF患者作为研究对象。将其按照随访结局分成预后不良组59例及预后良好组41例。比较两组Ang-2、LTBP2、CXCL14水平、肺功能指标、实验室指标以及一般资料,Pearson相关分析血清Ang-2、LTBP2、CXCL14水平与肺功能指标的相关性,多因素Logistic回归分析IPF患者预后的影响因素,受试者工作特征（ROC）曲线分析Ang-2、LTBP2、CXCL14对IPF患者预后的预测价值。结果：预后不良组血清Ang-2、LTBP2、CXCL14水平均高于预后良好组（P＜0.05）。预后不良组第1s用力呼气容积（FVC₁）、一氧化碳弥散量占预计值百分比（DLCO%）、动脉血氧分压（PaO₂）/氧体积分数（FiO₂）均低于预后良好组,而吸烟史、肺动脉高压人数占比高于预后不良组（P＜0.05）。Pearson相关性分析显示：IPF患者Ang-2、LTBP2、CXCL14水平均和FVC₁、DLCO%呈负相关（P＜0.05）。多因素Logistic回归分析结果显示：FVC₁、DLCO%、PaO₂/FiO₂、吸烟史、肺动脉高压以及血清Ang-2、LTBP2、CXCL14水平是IPF患者预后不良的影响因素（P＜0.05）。ROC曲线分析结果显示：血清Ang-2、LTBP2、CXCL14三项联合检测预测IPF患者预后的曲线下面积高于上述三项指标单独检测。结论：IPF患者血清Ang-2、LTBP2、CXCL14水平与其预后密切相关,可作为评估其预后的辅助性指标。     

丹参凝胶治疗对特应性皮炎小鼠模型皮肤屏障功能、表皮增生以及免疫功能的影响

摘要 目的：研究丹参凝胶治疗对特应性皮炎小鼠模型皮肤屏障功能、表皮增生以及免疫功能的影响。方法：30只C57BL/6小鼠被随机分为Control组、AD组和SG组,每组10只。AD组和SG组背部涂抹对二硝基氟苯建立特应性皮炎小鼠模型,SG小鼠在模型建立成功后涂抹丹参凝胶治疗3周,Control组和AD组涂抹凡士林作为对照。3周后,测量所有小鼠经皮水分丢失量( TEWL) 、皮肤厚度、脾脏指数、胸腺指数,血清IgE、IFN-γ和IL-4,脾脏树突状细胞、Th1和Th2细胞比例。结果：丹参凝胶治疗3周后,AD组和SG组小鼠TEWL、皮肤厚度、脾脏指数、胸腺指数,血清IgE、IFN-γ和IL-4含量,以及脾脏Th2细胞比例均显著高于对照组正常小鼠（P<0.05）,而脾脏树突状细胞、Th1细胞和Th1/Th2细胞比例均显著低于对照组正常小鼠（P<0.05）;与AD组小鼠相比,SG组小鼠TEWL、皮肤厚度、脾脏指数、胸腺指数,血清IgE、IFN-γ和IL-4含量,以及脾脏Th2细胞比例均显著降低（P<0.05）,而脾脏树突状细胞、Th1细胞和Th1/Th2细胞比例均显著升高（P<0.05）。结论：丹参凝胶具有保护特应性皮炎样小鼠皮肤屏障功能和抑制表皮增生的功能,并且可以影响特应性皮炎样小鼠脾脏树突状细胞和辅助性T细胞比例。     

Conformational stability and domain unfolding of the Von Willebrand factor A domains

Von Willebrand factor (VWF), a multimeric multidomain glycoprotein secreted into the blood from vascular endothelial cells, initiates platelet adhesion at sites of vascular injury. This process requires the binding of platelet glycoprotein Ib-IX-V to the A1 domain of VWF monomeric subunits under fluid shear stress. The A2 domain of VWF monomers contains a proteolytic site specific for a circulating plasma VWF metalloprotease, A Disintegrin and Metalloprotease with Thrombospondin motifs, member #13 of the ADAMTS enzyme family (ADAMTS-13), that functions to reduce adhesiveness of newly released, unusually large (UL), hyperactive forms of VWF. Shear stress assists ADAMTS-13 proteolysis of ULVWF multimers allowing ADAMTS-13 cleavage of an exposed peptide bond in the A2 domain. Shear stress may induce conformational changes in VWF, and even unfold regions of VWF monomeric subunits. We used urea as a surrogate for shear to study denaturation of the individual VWF recombinant A domains, A1, A2, and A3, and the domain triplet, A1-A2-A3. Denaturation was evaluated as a function of the urea concentration, and the intrinsic thermodynamic stability of the domains against unfolding was determined. The A1 domain unfolded in a 3-state manner through a stable intermediate. Domains A2 and A3 unfolded in a 2-state manner from native to denatured. The A1-A2-A3 triple domain unfolded in a 6-state manner through four partially folded intermediates between the native and denatured states. Urea denaturation of A1-A2-A3 was characterized by two major unfolding transitions: the first corresponding to the simultaneous complete unfolding of A2 and partial unfolding of A1 to the intermediate state; and the second corresponding to the complete unfolding of A3 followed by gradual unfolding of the intermediate state of A1 at high urea concentration. The A2 domain containing the cleavage site for ADAMTS-13 was the least stable of the three domains and was the most susceptible to unfolding. The low stability of the A2 domain is likely to be important in regulating the exposure of the A2 domain cleavage site in response to shear stress, ULVWF platelet adherence, and the attachment of ADAMTS-13 to ULVWF.