Pentraxin-3与急性冠状动脉综合征及冠状动脉病变的关系

目的了解急性冠状动脉综合征患者炎性指标pentraxin-3的变化,并了解其与冠状动脉病变之间的关系。方法回顾性研究从2003年7月到2005年9月期间85例急性冠状动脉综合征患者及25例健康体检者,检测其血浆pentraxin-3水平,并进行比较。所有急性冠状动脉综合征患者行选择性冠状动脉造影,了解pentraxin-3与冠状动脉病变之间的关系。结果急性冠状动脉综合征患者血浆pentraxin-3水平,明显高于健康体检者（P〈0.01）,且急性心肌梗死组高于不稳定性心绞痛组（P〈0.01）。A型病变、B型病变、C型病变三种病变类型患者血浆pen-traxin-3差异无显著性。结论作为炎性指标,pentraxin-3与冠心病发生急性冠状动脉综合征密切相关,但与冠状动脉病变类型无明确相关。     

血浆脂联素水平与2型糖尿病及其代谢综合征的关系

目的了解2型糖尿病及其合并代谢综合征患者中血浆脂联素水平的状况。方法对2型糖尿病合并代谢综合征组49例、2型糖尿病非代谢综合征组54例、正常对照组35例,测定其血浆脂联素水平,同时测定糖尿病患者的血糖、血压、血脂、C肽水平,比较3组间血浆脂联素平均水平、低脂联素血症发生率,分析其影响因素。结果糖尿病合并代谢综合征组、糖尿病非代谢综合征组及正常对照组血浆脂联素水平分别为(4.14±3.08)μg/ml、(6.65±4.55)μg/ml和(16.37±3.03)μg/ml,组间均有显著差异(P<0.05);3组中低脂联素血症发生率分别为69.4%、44.4%及0%,均有显著差异(P<0.05)。在糖尿病患者中,血浆脂联素与收缩压呈负相关(P<0.001)。结论2型糖尿病患者血浆脂联素水平明显降低,合并代谢综合征者降得更低,提示血浆脂联素与代谢综合征的发生发展可能有密切关系。     

老年肾病综合征患者血浆miRNA的异常表达与临床意义

目的探讨老年肾病综合征患者血浆miRNA的异常表达及与肾病综合征病理分型和治疗的关系。方法选取老年肾病综合征患者作为研究组(n=60),另选取同时期进行健康体检的老年人作为对照组(n=15),利用实时荧光定量PCR检测两组血浆中miR-181a、miR-141、miR-192的表达水平以及治疗后三者的变化。结果研究组血浆中miR-181a、miR-141、miR-192明显高于对照组(P<0.01)。血浆miRNA诊断老年肾病综合征效能较高(AUC>0.9);经过治疗的老年肾病综合征患者,血浆miRNA恢复到了正常的水平。结论血浆中游离的miRNA能作为诊断老年肾病综合征的一种新的血浆标志物,且能作为监测老年肾病综合征治疗疗效的一种有效的血浆标志物。     

急性冠脉综合征患者血浆D-二聚体和vWf的变化及临床意义

目的探讨急性冠脉综合征（ACS）患者血浆止血标志物的变化及意义。方法分别对30例急性心肌梗死（AMI）、30例不稳定型心绞痛（UAP）、20例稳定型心绞痛（SAP）患者和16例健康人清晨空腹采血3毫升,以ELISA方法检测血浆D-二聚体和血管性假血友病因子（vWf）的水平。结果AMI患者血浆D-二聚体和vWf水平显著高于UAP患者（P〈0.05）,UAP患者血浆D-二聚体和vWf水平显著高于SAP患者和健康对照组（P〈0.05）。结论急性冠脉综合征患者存在显著的凝血纤溶系统激活和内皮损伤。     

心脏X综合征患者同型半胱氨酸与血管内皮功能关系研究

目的：研究心脏X综合征患者血浆同型半胱氨酸（Hcy）与肱动脉血流介导血管扩张功能（FMD）的关系。方法：选择心脏X综合征患者30例（心脏X综合征组）,另选取与心脏X综合征患者同时期住院的其它疾病患者30例（对照组）,采用高分辨率超声技术检测FMD,采用循环酶法检测血浆Hcy水平。结果：1.心脏X综合征患者血浆同型半胱氨酸水平明显高于对照组（13.4±6.25umol/l vs. 9.78±4.3umol/l, P<0.05）;2.心脏X综合征患者肱动脉血流介导的血管扩张功能（FMD）明显低于对照组（5.21%±3.19% vs. 10.99%±4.06%, P<0.001）;3. 直线相关分析显示：心脏X综合征患者血浆同型半胱氨酸水平与肱动脉血流介导的血管扩张功能（FMD）之间存在负相关（r = -0.782,P<0.01）。结论：心脏X综合征患者血浆同型半胱氨酸水平明显升高,肱动脉血流介导的血管扩张功能（FMD）受损,且血浆同型半胱氨酸水平与FMD呈负相关,表明心脏X综合征患者血浆同型半胱氨酸可能损害肱动脉血流介导的血管扩张功能。     

急性冠脉综合征患者血浆P-选择素及血清一氧化氮水平相关性研究

目的观察血浆P-选择素(P-selectin)及血清一氧化氮(NO)水平在急性冠脉综合征(ACS)患者中的变化,探讨其与急性冠脉综合征的关系。方法用ELISA方法测定60例急性冠脉综合征患者-急性心肌梗死(AMI)、不稳定型心绞痛(UAP)各30例,30例稳定型心绞痛患者(SAP)、30例体检健康者血浆P-selectin及血清NO水平。结果⑴急性冠脉综合征患者血浆P-selectin水平较稳定型心绞痛患者及健康对照组明显升高(P<0.01),⑵急性冠脉综合征患者血清NO水平较对照组明显降低(P<0.01)。⑶血清NO与血浆P-selectin水平成负相关(P<0.01),与高密度脂蛋白胆固醇(HDL-C)水平成正相关(P<0.05)。结论血浆P-selectin及血清NO水平对急性冠脉综合征的诊断有指导意义。     

叶酸对Budd-Chiari综合征患者支架术后血浆同型半胱氨酸浓度及再狭窄的影响

目的 探讨叶酸对Budd-Chiari综合征（BCS）患者支架术后血浆同型半胱氨酸（Hcy）浓度的干预效果及其对下腔静脉或肝静脉再狭窄的影响.方法 选择接受过腔内支架置入术随访复查的56例Budd-Chiari综合征患者为研究对象,将56例患者分为叶酸组（30例）和对照组（26例）2组.高效液相色谱法分析术后1周、3个月、半年血浆Hcy水平,在相同的时间段复查彩色多普勒评估血管再狭窄情况,化学发光法测量治疗前后血浆叶酸的浓度.结果 对Budd-Chiari综合征患者应用叶酸治疗后血浆Hcy浓度明显低于治疗前,差异有显著性（P＜0.01）,术后半年30例叶酸组患者中有3例发生再狭窄,再狭窄率为10.0%;对照组常规治疗后血浆Hcy浓度较治疗前无明显变化（P＞0.05）,有10例发生了再狭窄,再狭窄率为38.5%,与叶酸组比较差异有显著性（P＜0.05）.结论 叶酸可降低Bucld-Chiari综合征患者血浆Hey浓度,血浆Hcy水平与血清叶酸水平呈负相关（r=-0.833）;叶酸有降低Budd-Chiari综合征患者支架术后下腔静脉或肝静脉早期（术后半年）再狭窄率的作用.     

X综合征患者血浆内皮素、一氧化氮浓度变化及其临床意义

目的：本研究以生化指标观察并探讨血管内皮功能与Ｘ综合征的联系。方法：采用放射免疫法、酶联免疫吸附法和分光光度法检测Ｘ综合征患者血浆内皮素、氧化型低密度脂蛋白和一氧化氮（以亚硝酸盐表示）水平，同时与冠心病患者和（或）健康人作对照分析。结果：Ｘ综合征患者血浆内皮素、一氧化氮水平较健康人升高，血浆内皮素／一氧化氮比值亦增大，而血浆氧化型低密度脂蛋白水平在正常范围。结论：Ｘ综合征患者血浆内皮素增高，血浆内皮素／一氧化氮比例可能失衡，从而导致血管舒缩状态异常。     

特利加压素联合血浆治疗肝硬化失代偿期肝肾综合征疗效观察

目的 观察特利加压素联合血浆治疗对肝硬化失代偿期肝肾综合征的疗效.方法 将肝硬化失代偿期合并有肝肾综合征的56例患者分为A、B、C3组,在内科综合治疗基础上,A组患者给予多巴胺治疗,B组患者给予特利加压素治疗,C组患者给予特利加压素联合血浆治疗,观察3组患者治疗期间临床症状、尿量、血肌酐、尿素氮、凝血酶原活动度及腹水的变化.结果 B、C组患者临床症状改善较A组明显,尿量显著增加,血肌酐、尿素氮明显下降,C组患者的肾功能改善较B组更明显,A组患者尿量、血肌酐和尿素氮下降均不明显.结论 在常规治疗的基础上,特利加压素联合血浆治疗肝硬化失代偿期肝肾综合征比单纯加用多巴胺或特利加压素的疗效更加显著.     

X综合征冠状循环内血浆内皮素浓度变化

目的　探讨X综合征患者中冠状血管内皮功能的变化。方法　采用心房起搏术,观察X综合征患者（n=8）冠状静脉窦血浆内皮素浓度水平,同时与年龄、性别相匹配的健康人（n=6）作对照。结果　X综合征组在整个试验过程中,冠状静脉窦血浆内皮素浓度明显增加（P<0.05）;并且在不同时点间的浓度均不同（P<0.05）。结论　X综合征患者冠状循环内血浆内皮素浓度增高,可能存在有内皮功能异常。     

从痰湿瘀热论治2型糖尿病代谢综合征52例

目的观察从痰、湿、瘀、热立论应用清热祛浊胶囊治疗2型糖尿病代谢综合征的疗效。方法 52例2型糖尿病代谢综合征患者,在原基础治疗基础上,加服清热祛浊胶囊5粒,每日3次。3个月后观察指标变化。结果治疗后患者的空腹血糖、餐后2 h血糖、糖化血红蛋白、三酰甘油、低密度脂蛋白胆固醇、收缩压、舒张压、体重指数明显下降(P<0.05或P<0.01);高密度脂蛋白胆固醇有所升高,但无统计学意义。治疗总有效率82.7%。结论从痰、湿、瘀、热立论组方的清热祛浊胶囊可以改善2型糖尿病代谢综合征患者的高血糖、高血压、高血脂、肥胖等多种心血管危险因子。     

血浆置换联合持续血液滤过治疗重型肝炎肝肾综合征疗效观察

目的观察血浆置换联合持续血液滤过治疗重型肝炎合并肝。肾综合征的疗效。方法应用血浆置换加持续血液滤过治疗13例重型肝炎合并肝。肾综合征患者并与单纯药物治疗进行比较。结果血浆置换联合持续血液滤过治疗组肝。肾功能及电解质恢复明显好于单纯药物治疗组，且生存率较单纯药物治疗组明显升高。结论血浆置换联合持续血液滤过是治疗重型肝炎并发肝。肾综合征的有效方法。     

NT-proBNP、Hcy诊断急性冠脉综合征的临床研究

目的探讨NT—proBNP、Hcy诊断急性冠脉综合征（ACS）的临床意义。方法对70例AMI患者、50例UAP患者、50例SAP患者和50例健康体检者的血浆NT—proBNP、Hey水平进行比较分析。ACS患者均行冠状动脉造影术,并对其病变程度进行评估。结果AMI组和UAP组患者血浆NT—proBNP水平显著高于SAP组及对照组（P〈0．05）,而SAP组与对照组比较差异无统计学意义（P〉0．05）;ACS组、SAP组血浆Hcy水平均高于对照组,各组间比较差异有统计学意义（P〈O．05）。随着冠脉病变支数增加,ACS患者血浆NT—proBNP、Hcy水平逐渐升高（P〈0．05）。ACS患者中,心血管事件组血浆NT—proBNP、Hcy水平明显高于无心血管事件组（p〈0．05）。结论急性冠脉综合征患者的血浆NT—proBNP、Hcy水平均与冠状动脉粥样化病变程度密切相关,是ACS心血管不良事件的预测因子。     

Complement Mutations in Diacylglycerol Kinase-ε–Associated Atypical Hemolytic Uremic Syndrome

Background and objectives

Atypical hemolytic uremic syndrome is characterized by vascular endothelial damage caused by complement dysregulation. Consistently, complement inhibition therapies are highly effective in most patients with atypical hemolytic uremic syndrome. Recently, it was shown that a significant percentage of patients with early-onset atypical hemolytic uremic syndrome carry mutations in diacylglycerol kinase-ε, an intracellular protein with no obvious role in complement. These data support an alternative, complement-independent mechanism leading to thrombotic microangiopathy that has implications for treatment of early-onset atypical hemolytic uremic syndrome. To get additional insights into this new form of atypical hemolytic uremic syndrome, the diacylglycerol kinase-ε gene in a cohort with atypical hemolytic uremic syndrome was analyzed.

Design, setting, participants, & measurements

Eighty-three patients with early-onset atypical hemolytic uremic syndrome (<2 years) enrolled in the Spanish atypical hemolytic uremic syndrome registry between 1999 and 2013 were screened for mutations in diacylglycerol kinase-ε. These patients were also fully characterized for mutations in the genes encoding factor H, membrane cofactor protein, factor I, C3, factor B, and thrombomodulin CFHRs copy number variations and rearrangements, and antifactor H antibodies.

Results

Four patients carried mutations in diacylglycerol kinase-ε, one p.H536Qfs*16 homozygote and three compound heterozygotes (p.W322*/p.P498R, two patients; p.Q248H/p.G484Gfs*10, one patient). Three patients also carried heterozygous mutations in thrombomodulin or C3. Extensive plasma infusions controlled atypical hemolytic uremic syndrome recurrences and prevented renal failure in the two patients with diacylglycerol kinase-ε and thrombomodulin mutations. A positive response to plasma infusions and complement inhibition treatment was also observed in the patient with concurrent diacylglycerol kinase-ε and C3 mutations.

Conclusions

Data suggest that complement dysregulation influences the onset and disease severity in carriers of diacylglycerol kinase-ε mutations and that treatments on the basis of plasma infusions and complement inhibition are potentially useful in patients with combined diacylglycerol kinase-ε and complement mutations. A comprehensive understanding of the genetic component predisposing to atypical hemolytic uremic syndrome is, therefore, critical to guide an effective treatment.     

肠易激综合征的中医虚实证型与血浆脑肠肽水平变化的相关性研究

[目的]观察肝郁气滞型、脾胃虚弱型、肝郁脾虚型肠易激综合征（IBS）患者脑肠肽的变化,探讨IBS的中医虚实证型与血浆脑肠肽水平变化的相关性。[方法]90例IBS患者分为肝气郁滞证组、肝郁脾虚证组、脾胃虚弱证组各30例,正常对照（正常）组10例,应用放射免疫法同批测定血浆血管活性肠肽（VIP）、神经肽Y（NPY）和神经降压素（NT）水平。[结果]3个证型组患者血浆VIP水平显著高于正常组（P〈0.05,〈0.01）;脾胃虚弱、肝郁脾虚证组患者VIP水平显著高于肝气郁滞证组（P〈0.05）,但脾胃虚弱证组和肝郁脾虚证组之间比较差异无统计学意义。3个证型组患者血浆NPY水平显著低于正常组（P〈0.05,〈0.01）,肝郁脾虚证组和肝气郁滞证组NPY水平明显低于脾胃虚弱证组（P〈0.01,〈0.05）,肝郁脾虚证组NPY水平低于肝气郁滞证组,但2组比较差异无统计学意义。3个证型组患者血浆NT水平显著高于正常组（P〈0.01）。3证型间比较差异无统计学意义。[结论]不同中医证型IBS脑肠肽存在不同的变化。IBS的中医病机观与现代医学所倡导的脑-肠轴学说具有相关性。     

肾上腺意外瘤中亚临床库欣综合征临床研究

目的探讨肾上腺意外瘤中亚临床库欣综合征的临床及危害性。方法2000~2005年对上海交通大学医学院附属瑞金医院的23例亚临床库欣综合征患者进行血糖、血脂、血尿皮质醇、血皮质醇昼夜节律、地塞米松抑制试验检测和观察,并与同期25例肾上腺腺瘤型库欣综合征患者的结果比较。结果(1)亚临床库欣综合征组年龄大于肾上腺腺瘤型库欣综合征组,两组肥胖、高血压、糖代谢异常及高血脂的发生率比较差异无显著性意义,两组体重指数(BMI)、腰臀比、血压、血脂、血糖及胰岛素比较差异均无显著性意义。(2)亚临床库欣综合征组血皮质醇、日平均血皮质醇、夜间皮质醇占清晨分泌量百分比及24h尿游离皮质醇均低于肾上腺腺瘤型库欣综合征组,2mg地塞米松抑制试验后血皮质醇及尿游离皮质醇亦低,两组晨8h血促肾上腺皮质激素(ACTH)比较差异无统计学意义。结论亚临床库欣综合征患者可导致肥胖、高血压、高血脂及糖代谢异常的发生,对患者造成一定的危害,且危害程度与肾上腺腺瘤型库欣综合征相近。     

Methotrexate-induced side effects are not due to differences in pharmacokinetics in children with Down syndrome and acute lymphoblastic leukemia

Background

Children with Down syndrome have an increased risk of developing acute lymphoblastic leukemia and a poor tolerance of methotrexate. This latter problem is assumed to be caused by a higher cellular sensitivity of tissues in children with Down syndrome. However, whether differences in pharmacokinetics play a role is unknown.

Design and Methods

We compared methotrexate-induced toxicity and pharmacokinetics in a retrospective case-control study between patients with acute lymphoblastic leukemia who did or did not have Down syndrome. Population pharmacokinetic models were fitted to data from all individuals simultaneously, using non-linear mixed effect modeling.

Results

Overall, 468 courses of methotrexate (1–5 g/m²) were given to 44 acute lymphoblastic leukemia patients with Down syndrome and to 87 acute lymphoblastic leukemia patients without Down syndrome. Grade 3–4 gastrointestinal toxicity was significantly more frequent in the children with Down syndrome than in those without (25.5% versus 3.9%; P=0.001). The occurrence of grade 3–4 gastrointestinal toxicity was not related to plasma methotrexate area under the curve. Methotrexate clearance was 5% lower in the acute lymphoblastic leukemia patients with Down syndrome (P=0.001); however, this small difference is probably clinically not relevant, because no significant differences in methotrexate plasma levels were detected at 24 and 48 hours.

Conclusions

We did not find evidence of differences in the pharmacokinetics of methotrexate between patients with and without Down syndrome which could explain the higher frequency of gastrointestinal toxicity and the greater need for methotrexate dose reductions in patients with Down syndrome. Hence, these problems are most likely explained by differential pharmaco-dynamic effects in the tissues between children with and without Down syndrome. Although the number of patients was limited to draw conclusions, we feel that it may be safe in children with Down syndrome to start with intermediate dosages of methotrexate (1–3 g/m²) and monitor the patients carefully.     

肺部肿瘤致异位ACTH综合征7例报告并文献复习

目的探讨肺部肿瘤致异位促肾上腺皮质激素（ACTH）综合征的临床特点。方法回顾性分析2000年11月～2009年12月解放军总医院内分泌科收治并确诊的7例肺部肿瘤致异位ACTH综合征患者的临床资料。结果高血压、糖尿病、低血钾、水肿为最常见的临床表现,多缺乏典型库欣综合征的体征;血ACTH和皮质醇、24 h尿游离皮质醇水平升高,皮质醇分泌均不被小剂量及大剂量地塞米松抑制试验所抑制;胸部薄层CT扫描能发现多数肺部病变;手术切除肺部病变、小细胞肺癌化疗效果较好;肾上腺切除为控制高皮质醇血症的有效手段。结论肺部肿瘤是导致异位ACTH综合征的常见病因,对于ACTH依赖性库欣综合征的患者应常规行胸部薄层CT来筛查肺部病变。     

异位ACTH综合征6例临床分析

目的提高临床医生对异位ACTH综合征的认识。方法对四川大学华西医院1996—2005年确诊的6例异位ACTH综合征患者的临床资料及诊治过程进行回顾性分析。结果副节瘤1例,神经内分泌癌1例,肺癌4例。临床特征病程15d至2个月,6例患者均有严重低血钾伴碱中毒,5例患者有高血压和双下肢水肿。Cushing综合征表现满月脸4例,淤斑和长胡须2例,多血质、痤疮、色素沉着各1例,血皮质醇(晨起8时)>1173nmol/L,大剂量地塞米松(8mg)抑制试验均不能抑制。结论对病程短,严重低血钾碱中毒,水肿明显而Cushing临床表现不明显者,血皮质醇>1000nmol/L应高度怀疑异位ACTH综合征。寻找病因,尽快手术切除肿瘤,近期预后较好。     

Renal and neurohormonal changes following simultaneous administration of systemic vasoconstrictors and dopamine or prostacyclin in cirrhotic patients with hepatorenal syndrome

Background/Aims: Intravenous ornipressin in cirrhotic patients with hepatorenal syndrome causes marked improvement of systemic hemodynamics and suppression of plasma renin and norepinephrine but only moderate improvement of renal function. This study was designed to investigate whether these beneficial effects could be enhanced by the simultaneous administration of dopamine. The renal effects of the i.v. infusion of norepinephrine plus prostacyclin in patients with hepatorenal syndrome were also assessed.Methods: Renal plasma flow, glomerula filtration rate, free water clearance, sodium excretion and the plasma levels of renin and norepinephrine were measured in baseline conditions and during the administration of ornipressin (6 IU/h) and ornipressin (6 IU/h) plus dopamine (2 μg/kg·min) in nine patients with hepatorenal syndrome. Five additional patients with hepatorenal syndrome were studied prior to and following the administration of norepinephrine (0.45±0.1 μg/kg·min) and norepinephrine (0.85±0.2 μg/kg·min) plus prostacyclin (5 ng/kg·min).Results: Despite a significant increase in arterial pressure and marked suppression of plasma renin activity during ornipressin and ornipressin plus dopamine administration, no significant improvement in renal function was observed. Norepinephrine and norepinephrine plus prostacyclin also failed to increase renal perfusion and glomerular filtration rate.Conclusions: The combined administration of systemic vasoconstrictors (ornipressin or norepinephrine) and vasodilators (dopamine or prostacyclin), at the doses used in the current study and for a short period of time, does not improve renal function in cirrhotic patients with hepatorenal syndrome. The current study does not confirm a potential role for ornipressin in the treatment of hepatorenal syndrome.