肾小球疾病患者血浆纤维蛋白原浓度及其临床意义

目的　探讨肾小球疾病患者血浆纤维蛋白原 (FIB)浓度及其临床意义。方法　经肾活检证实的肾小球疾病患者 333例 ,其中非肾病范围蛋白尿 177例 (A组 ) ;肾病范围蛋白尿 15 6例 (B组 ) ,比较两组FIB与白蛋白 (ALB)、尿蛋白排泄量 (U pro)、总胆固醇 (TC)及甘油三酯 (TG)浓度间的相关性。 结果　A组FIB与上述指标无明显相关 (P >0 .0 5 ) ;B组FIB与ALB负相关 (r =- 0 .5 7,P <0 .0 1) ,与U pro、TC及TG正相关 (r分别为 0 .376 ,0 .4 95和 0 .19,P <0 .0 1,<0 .0 1和 =0 .0 17)。FIB >8.0 g/L者易发生血栓、栓塞。结论　尿蛋白排泄量越大、白蛋白浓度越低则FIB和血脂浓度越高 ,FIB >8.0 g/L者有诱因 (如脱水 )存在时易形成血栓     

血管痉挛性心绞痛患者嗜酸性粒细胞计数及纤维蛋白原测定的临床意义

目的研究血管痉挛性心绞痛（VAP）患者嗜酸性粒细胞（EOS）计数和血浆纤维蛋白原（Fbg）水平的变化。方法收集疑为冠状动脉疾病（CAD）或电生理研究提示心律失常而行诊断性心导管术的住院患者共93例，根据相关诊断标准将其分3组，VAP患者39例，稳定性心绞痛（SAP）患者35例，对照组19例。再根据临床症状的严重度，将VAP组分为轻度VAP组（22例）和重度VAP组（17例）。采集血样分别测定各组患者的WBC计数及分类计数、Fbg、C反应蛋白（CRP）。结果除SAP组高密度脂蛋白胆固醇（HDL-C）明显低于对照组外（P〈0．01），其他冠心病危险因子、体温、WBC总数、CRP等在VAP、SAP及对照组间无统计学意义（P〉0．05）。而重度VAP组EOS计数、血浆Fbg均明显高于其他3组（P〈0．01，P〈0．05）。VAP患者经药物治疗后，EOS计数明显低于治疗前（P〈0．01）。结论EOS计数和血浆Fbg水平可预示VAP患者病情严重度，提示冠状动脉痉挛可能引致EOS和Fbg水平升高。     

血管痉挛性心绞痛患者嗜酸性粒细胞计数及纤维蛋白原测定的临床意义

目的研究血管痉挛性心绞痛(VAP)患者嗜酸性粒细胞(EOS)计数和血浆纤维蛋白原(Fbg)水平的变化。方法收集疑为冠状动脉疾病(CAD)或电生理研究提示心律失常而行诊断性心导管术的住院患者共93例,根据相关诊断标准将其分3组,VAP患者39例,稳定性心绞痛(SAP)患者35例,对照组19例。再根据临床症状的严重度,将VAP组分为轻度VAP组(22例)和重度VAP组(17例)。采集血样分别测定各组患者的WBC计数及分类计数、Fbg、C反应蛋白(CRP)。结果除SAP组高密度脂蛋白胆固醇(HDLC)明显低于对照组外(P<0.01),其他冠心病危险因子、体温、WBC总数、CRP等在VAP、SAP及对照组间无统计学意义(P>0.05)。而重度VAP组EOS计数、血浆Fbg均明显高于其他3组(P<0.01,P<0.05)。VAP患者经药物治疗后,EOS计数明显低于治疗前(P<0.01)。结论EOS计数和血浆Fbg水平可预示VAP患者病情严重度,提示冠状动脉痉挛可能引致EOS和Fbg水平升高。     

遗传性低纤维蛋白原血症家系复合杂合基因缺陷的研究

目的 对1个遗传性低纤维蛋白原血症家系进行临床表型诊断和基因分析,并探讨该病的分子发病机制.方法 采集该家系3代共7人外周血,检测活化部分凝血活酶时间(APTT)、凝血酶原时间(PT)、凝血酶时间(TT)、蕲蛇酶时间(RT)、抗凝血酶活性(AT∶A)、蛋白C活性(PC∶A)和蛋白S活性(PS∶A),纤维蛋白原(Fg)抗原和活性分别用免疫比浊法和Clauss法测定;采用十二烷基磺酸钠-聚丙烯酰胺凝胶电泳(SDS-PAGE)对先证者及其父母的血浆纤维蛋白原进行半定量检测及肽链相对分子质量分析;使用自动校正凝血酶生成仪测定先证者的凝血酶生成;应用血栓弹力图仪检测血液凝固的动态过程和纤维蛋白形成过程的动力学变化.抽提先证者及家系成员外周血基因组DNA,采用PCR扩增Fg的3个基因(FGA、FGB和FGG)所有外显子及其侧翼序列,DNA直接测序进行基因分析.结果 先证者凝血功能检查显示Fg抗原为0.68 g/L,活性为0.48 g/L; TT延长为29.2 s,RT延长为75.8 s.SDS-PAGE结果显示,3条肽链相对分子质量正常,但含量明显减低;先证者凝血酶生成峰值为249.93 nmol/L,凝血酶生成潜力为1007.0 nmol·L-1·min;全血凝固的动态过程异常,凝血综合指数(CI)为-8.6,显示全血低凝状态.表型诊断为遗传性低纤维蛋白原血症.基因分析发现先证者纤维蛋白原Aa链存在g.3175A＞C突变导致的Gln143Pro突变,以及g·4642delC 突变导致的438位苏氨酸后编码26个异常氨基酸提前出现终止密码子;前者来源于母亲,后者来源于父亲.结论纤维蛋白原Aa链Gln143Pro和g.4642delC复合杂合突变是导致该家系先证者低纤维蛋白原血症的原因.     

Treatment of congenital fibrinogen disorders

Background: The goal of the coagulation pathway is the conversion of fibrinogen to fibrin and formation of an insoluble clot. Although relatively rare, congenital fibrinogen disorders are interesting and pose several challenges that can serve as paradigms for many diseases. An impressive body of knowledge has accumulated recently, particularly thanks to international collaborative clinical and genetic studies allowing the molecular characterization of these disorders. However, apart from the possibility of developing safer fibrinogen concentrates and the availability of prenatal diagnosis, the basic therapeutic approach has changed little. Objective: We need to better understand the clinical phenotype of patients in order to administer fibrinogen preparations or other treatments more appropriately. Methods: We discuss current therapeutic options and others that could be available in the near future. Results/conclusion: Patients with congenital fibrinogen deficiencies require better predictive tests for clinical complications and more efficient and available fibrinogen concentrates. Global hemostasis tests in combination with routine assays could help to individually tailor therapeutic protocols.     

The effect of fibrinogen concentrate and fresh frozen plasma on the outcome of patients with acute traumatic coagulopathy: A quasi-experimental study

Introduction

The debate on replacing coagulation factors and its effect on the final outcome of the patients with acute traumatic coagulopathy (ATC) in need of transfusion is still ongoing. Therefore, the present study is designed with the aim of comparing the outcome of patients with acute traumatic coagulopathies receiving fibrinogen and fresh frozen plasma (FFP).

三个遗传性异常纤维蛋白原血症家系的表型和基因型分析

目的 对3个遗传性异常纤维蛋白原血症家系进行临床表型和基因型分析.方法 检测3个家系所有成员外周血活化部分凝血活酶时间(APTT)、凝血酶原时间(PT)、凝血酶时间(TT)、靳蛇酶时间(RT)、抗凝血酶活性(AT:C)、蛋白C活性(PC:C)和蛋白S活性(PS:C),纤维蛋白原(Fg)抗原和活性分别用免疫比浊法和Clauss法测定,分别用SDS-PAGE和Western blot法检测3例先证者血浆纤维蛋白原三条肽链的相对分子质量.抽提DNA,PCR扩增Fg 3个基因FGA、FGB和FGG所有外显子及其侧翼序列,DNA测序并进行基因分析.结果 3例先证者APTT、PT以及抗凝指标(AT:C、PC:C和PS:C)都正常,而TT和RT明显延长,Fg的活性降低,分别为0.90、0.84及0.44 g/L,而抗原正常,分别为2.0、3.2及3.1 g/L;SDS-PAGE和Western blot法均未检测到异常条带.基因分析发现3例先证者各携带1个杂合错义突变,分别为FGG g.7476 G→A(γ Arg275His)、FGA g.1209 C→T(Aα Pro18Leu)和FGA g.1202 C→T(Aα Arg16Cys).结论 3例先证者遗传性异常纤维蛋白原血症分别由γ Arg275 His、Aα Pro18Leu和Aα Arg16Cys突变所致,其中Aα Pro18Leu和Aα Arg16Cys突变为国内首次报道.

Abstract：

Objective To analyze the phenotype and genotype in three Chinese pedigrees with inherited dysfibrinogenemia. Methods Laboratory tests including activated partial thromboplastin time (APTT) ,plasma fibrinogen(Fg) were analyzed by Clauss and immunoturbidimetry methods, respectively. The Fg of three probands was assessed by Western blot and sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). The sequences of all the exons and exon-intron boundaries of the three Fg genes FGA, GFB and FGG were amplified by PCR and analyzed by direct sequencing. Results Three probands had normal ma Fg were reduced, but antigen levels were normal. Western blot and SDS-PAGE showed no abnormal molecular weight of Fg. The 3 heterozygous mutations of γ Arg275His, Aα Pro18Leu and Aα Arg16Cys were identified in the 3 probands, respectively. Conclusion The three probands with dysfibrinogenemia were caused by the mutations of γ Arg275His, Aα Pro18Leu and Aα Arg16Cys, respectively. Both Aα Pro18Leu and Aα Arg16Cys were first reported in Chinese population.     

急性脑梗死患者血浆D-二聚体与纤维蛋白原的变化

目的　观察急性脑梗死患者血浆中D 二聚体和纤维蛋白原的变化 ,并探讨其临床意义。方法　采集 5 3例发病 2 4h内急性脑梗死患者血液 ,测定其血浆中D 二聚体和纤维蛋白原含量 ,并与 4 0例健康对照组比较。结果　急性脑梗死患者血浆D 二聚体 ,纤维蛋白原含量明显高于对照组 (P <0 0 1) ,并与梗死灶大小及病情轻重明显相关。结论　急性脑梗死患者存在凝血纤溶系统异常 ,血浆D -二聚体及纤维蛋白原检测对脑梗死患者的诊断及病情判断、溶栓的效果评价及预后有重要的参考价值。     

纤维蛋白原Bβ链基因多态性与其含量、分子活性及脑梗死的关系

论 FgBβBcl-1等位基因A及其变异型人群是脑梗死的易感人群;FgBβ-854是Fg浓度和分子聚合活性的主要调控位点之一.     

慢性肾炎患者血液流变学、血脂和纤维蛋白原水平及其关系的研究

目的 本文观察了40例慢性肾炎患者血液流变学指标及血脂、纤维蛋白原对其的影响。 方法 检测了40例慢性肾炎患者的全血高切值、全血低切值、血浆粘度、血胆固醇、纤维蛋白原。结果 慢性肾炎患者全血高切值、全血低切值、血浆粘度较正常对照组明显升高 (p<0.005,p<0.001,p<0.005) ,血胆固醇、纤维蛋白原较正常对照组升高 (p<0.01,p<0.05) ,血胆固醇与全血高切粘度成正相关 (r=0.58,p<0.05)。纤维蛋白原与血浆粘度成正相关 (r=0.69,p<0.005)。结论 慢性肾炎患者存在高粘血症 ,血脂、纤维蛋白原在其中起着一定作用。     

胃癌患者纤维蛋白原含量与临床分期及淋巴结转移的关系

[目的]通过检测胃癌患者血浆纤维蛋白原（FIB）含量,探讨其与临床分期及淋巴结转移的关系。[方法]选取经病理确诊为胃癌的患者116例（胃癌组）及同期收治的胃良性病变25例（对照组）,采用全自动凝血分析仪测定两组的FIB浓度;化学发光仪测定癌胚抗原（CEA）、癌抗原19—9（CA19—9）、癌抗原72—4（CA72—4）、癌抗原12—5（CA12—5）水平,并进行对比分析。[结果]FIB水平胃癌组较良性病变组高,差异有统计学意义（P〈0．01）;淋巴转移组较未转移组高差异有统计学意义（P〈0．01）。FIB水平与CA12—5、CA72—4水平具有相关性（P=0．014、P=0．031）。[结论]FIB与胃癌临床分期及淋巴结转移密切相关,其含量在术前可以作为预测是否发生淋巴结转移的一个指标。     

茶多酚对血液有形成分影响的临床研究

目的 旨在观察口服茶多酚对被试者血液有形成分的影响。方法 临床病例67例,给予茶多酚（250mg/次）,每日3次口服,疗程1个月,分别于服药前和服药后30天检测被试者外周血之红细胞、血红蛋白、白细胞、血小板和纤维蛋白原等含量。结果 口服茶多酚除可轻度降低血浆纤维蛋白原外（P＞0．05）,对被试者外周血之红细胞、血红蛋白、白细胞和血小板等含量均无显著影响（P＞0．05）。结论 口服茶多酚对被试者外周血之有形成分无显著影响。     

The relationship between fibrinogen and in-hospital mortality in patients with type A acute aortic dissection

Background and purpose

Fibrinogen plays an important role in hemostasis and thrombosis and is proven to have prognostic significance in patients with cardiovascular disease. We examined the utility of fibrinogen as a prognostic indicator for patients with type A acute aortic dissection (AAD).

The molecular basis of quantitative fibrinogen disorders

Hereditary fibrinogen disorders include type I deficiencies (afibrinogenemia and hypofibrinogenemia, i.e. quantitative defects), with low or unmeasurable levels of immunoreactive protein; and type II deficiencies (dysfibrinogenemia and hypodysfibrinogenemia, i.e. qualitative defects), showing normal or altered antigen levels associated with reduced coagulant activity. While dysfibrinogenemias are in most cases autosomal dominant disorders, type I deficiencies are generally inherited as autosomal recessive traits. Patients affected by congenital afibrinogenemia or severe hypofibrinogenemia may experience bleeding manifestations varying from mild to severe. This review focuses on the genetic bases of type I fibrinogen deficiencies, which are invariantly represented by mutations within the three fibrinogen genes (FGA, FGB, and FGG) coding for the three polypeptide chains Aalpha, Bbeta, and gamma. From the inspection of the mutational spectrum of these disorders, some conclusions can be drawn: (i) genetic defects are scattered throughout the three fibrinogen genes, with only few sites appearing to represent relative mutational hot spots; (ii) several different types of genetic lesions and pathogenic mechanisms have been described in affected individuals (including gross deletions, point mutations causing premature termination codons, missense mutations affecting fibrinogen assembly/secretion, and uniparental isodisomy associated with a large deletion); (iii) the possibility to express recombinant fibrinogen mutants in eukaryotic cells is rapidly shedding light into the molecular mechanisms responsible for physiologic and pathologic properties of the molecule; (iv) though mutation analysis of the fibrinogen cluster does not yield precise information for predicting genotype/phenotype correlations, it still provides a valuable tool for diagnosis confirmation, identification of potential carriers, and prenatal diagnosis.     

急性脑梗死与超敏C反应蛋白及纤维蛋白原关系的临床研究

目的探讨急性脑梗死与超敏C反应蛋白（hs-CRP）及纤维蛋白原（Fib）的关系。方法入选患者为2006年8月至2008年8月87例首次脑梗死患者,年龄18-80岁,48h以内首发脑梗死,经头颅MRI／CT证实,排除脑出血。对照组来自健康体检者,共40例。两组对象血清hs-CRP浓度采用速率透射免疫比浊法测定,血浆Fib含量通过ACL-1000全自动血凝分析仪测定。结果急性脑梗死患者组血液hs-CRP及Fib水平均较健康组明显升高（P〈0．05）。两组NDS评分预后比较,hs-CRP正常组总有效率（82．7％）明显高于hs-CRP异常组（68．7％）;Fib正常组总有效率（87．2％）明显高于Fib异常组（54．1％）,差异具有显著性（P〈0．01）。结论血清hs-CRP及Fib水平数值的增高,证实其参与脑梗死急性期炎性反应,急性脑梗死患者体内存在凝血-纤溶系统异常。     

脑梗死急性期血浆纤维蛋白原水平与病情及预后相关性研究

目的探讨脑梗死急性期(ACI)患者血浆纤维蛋白原(Fib)水平的改变及其与病情和预后的关系。方法检测250例脑梗死患者(ACI组)和226名健康成人(健康对照组)的血浆Fib含量。在ACI患者入院当天和4周时进行临床神经功能缺损程度评分(NDS)。结果 ACI组血浆Fib水平异常率明显高于健康对照组(44.8%vs.9.3%,P<0.01);NDS重型患者血浆Fib含量[(5.82±1.51)mg/L]明显高于中型[(4.17±1.09)mg/L]、轻型[(4.26±1.15)mg/L]患者(均P<0.01)。血浆Fib含量升高组患者住院4周时显著进步和进步者明显低于Fib正常组(均P<0.01),而无变化和恶化者明显高于Fib正常组(均P<0.01)。结论 ACI患者血浆Fib水平均明显升高,病情重的患者升高更明显,血浆Fib含量升高的患者预后较差。     

ST段抬高型心肌梗死患者纤维蛋白原和D-二聚体的变化

目的 探讨ST段抬高型心肌梗死(STEMI)患者与冠状动脉造影阴性者纤维蛋白原和D-二聚体含量的差异.方法 选取我院2005年1月至2007年12月诊断为STEMI并行直接经皮冠状动脉介入(PCI)治疗的患者100例.同时选取冠状动脉造影阴性者100例为对照组.比较2组间纤维蛋白原和D-二聚体含量.结果 2组性别、年龄、高血压史、糖尿病史和吸烟史差异无统计学意义(P均0.05).STEMI组血浆纤维蛋白原含量为(2.38±0.91)g/L,对照组为(2.65±0.68)g/L,差异有统计学意义(t=-2.34,P<0.05).D-二聚体的平方根STEMI组为(13.23±5.08)μg/L,对照组为(9.40±5.03)μg/L,差异有统计学意义(t=5.36,P<0.01).血浆D-二聚体与纤维蛋白原含量比值的平方根STEMI组为(9.11±4.13),对照组为(5.92±3.35),差异有统计学意义(t=5.99,P<0.01).结论 STEMI患者的纤维蛋白原低于冠状动脉造影阴性的对照组,D-二聚体高于对照组,提示在STEMI急性期存在急性血栓形成和继发纤溶.     

颞下颌关节紊乱病患者临床特征及MRI影像表现

目的  探讨182例颞下颌关节紊乱病患者的临床特征及MRI影像表现。方法  回顾性选取2018年9月~2021年6月于我院就诊的182例颞下颌关节紊乱病患者364只颞颌关节为研究对象,收集并分析其临床病例资料,包括一般资料、临床特征及MRI影像表现等。结果  182例颞下颌关节紊乱病患者364只关节,关节盘可复性前移162只关节,不可复性前移114只关节,侧方移位27只关节,关节盘损伤变性60只,关节盘穿孔32只,髁状突变形、破坏47例,关节囊、韧带损伤31例。患者临床特征复杂多变,但多数患者普遍表现为关节疼痛、弹响、张口受限、下颌偏斜等症状。MRI图像可见清晰的关节盘位置、形态、厚度变化和关节积液。结论  颞下颌关节紊乱病患者临床特征多样,可通过MRI精确反映颞下颌关节紊乱病进展中关节盘位置、形态及厚度变化情况,提高临床诊断准确性。     

慢性阻塞性肺疾病患者C反应蛋白与纤维蛋白原含量的变化分析

目的探讨慢性阻塞性肺疾病(COPD)急性加重期患者血清C反应蛋白(CRP)与血浆纤维蛋白原(Fib)含量的变化及其临床意义。方法分别检测30例COPD患者急性加重期、缓解期及30例健康体检者(对照组)血清CRP、血浆Fib的含量,同时进行动脉血气测定。结果 COPD患者急性加重期CRP、Fib含量较缓解期及对照组均明显升高,缓解期含量较对照组明显升高,差异有统计学意义(P<0.05或P<0.01);动脉血氧分压(PaO2)与血清CRP、血浆Fib含量均呈负相关(P<0.01),动脉血二氧化碳分压(PaCO2)与血清CRP、血浆Fib含量均呈正相关(P<0.01)。结论监测CRP、Fib的含量对于COPD患者的病情、疗效判断、预测预后有一定的临床价值。     

Patients with congenital hypothyroidism demonstrate different altered expression of plasma fibrinogen and haptoglobin polypeptide chains

OBJECTIVES: To compare the plasma protein profiles of treated and untreated congenital hypothyroidism (CH) patients with those of normal control infants. DESIGN AND METHODS: Plasma samples were subjected to two-dimensional gel electrophoresis and silver staining or lectin detection. Resolved protein spots were analyzed by using computerized densitometry software. RESULTS: The significant enhanced expression of the fibrinogen gamma-chain and reduced expression of haptoglobin beta-chain were demonstrated in the profiles of untreated CH patients using both silver staining and lectin detection methods. Lectin detection resolved only a single cluster of the haptoglobin beta-chain for the untreated patients, in contrast to five isoform clusters detected in the controls' and treated CH patients' profiles. CONCLUSIONS: Plasma from untreated CH patients demonstrated different altered expression of fibrinogen and haptoglobin polypeptide chains, which was normalized when patients were treated. Our data also suggest differences in structures of the N-glycans of haptoglobin beta-chain of the untreated CH patients.