慢性肺心病患者肺动脉血栓形成相关因素分析

目的 了解慢性肺心病肺动脉血栓形成的因素。方法 对20例慢性肺心病患凝血功能指标PT、KPTT、FIB；反映纤溶指标D—二聚体；反映血小板功能的活化血小板、PAgT进行研究，并对其急性发作期、缓解期与对照组进行比较。结果 慢性肺心病急性发作期的KPTT缩短，FIB、D—二聚体、活化血小板、PAgT升高，与对照组比较有显差异，血液处于高凝状态，且在缓解期有一定改善。结论 慢性肺心病由于缺氧、肺动脉高压等存在，造成血管内皮损伤、血小板功能改变、血液高凝状态，容易引起肺动脉血栓形成，呼吸功能的改善有助于缓解血液的高凝状态。     

肝硬化患者凝血、纤溶指标及血小板变化与Child-Pugh分级的关系

[目的]探讨肝硬化患者凝血、纤溶指标、血小板计数（BPC）的变化与Child-Pugh分级及上消化道出血的关系.[方法]上海市公共卫生中心2009年2月至2011年3月90例肝硬化住院患者,按Child-Pugh分级A级15例、B级46例、C级29例,分为出血组（42例）、非出血组（48例）.均检测其凝血酶原时间（PT）、凝血因子Ⅱ、Ⅴ、Ⅶ、Ⅹ及组织纤溶酶原激活物（t-PA）、D-二聚体（D-d）、血常规,比较各组患者的相关指标.[结果]随Child-Pugh分级的增加患者PT显著延长,凝血因子Ⅱ、Ⅴ、Ⅶ、Ⅹ、BPC逐渐降低,t-PA、D-d逐渐升高.PT、凝血因子Ⅱ、Ⅶ、BPC在Child-Pugh A、B、C组间两两比较差异有显著性;Child-Pugh B、C组凝血因子Ⅴ、Ⅹ显著高与Child-Pugh A组.出血组PT、BPC 与未出血组差异有显著性;Child B、C级肝硬化患者出血率显著高与A级,而C级病死率最高,且两两比较差异均有显著性.[结论]肝硬化患者存在明显的凝血异常及纤溶亢进,且与临床病情严重程度密切相关; PT、BPC、Child-Pugh分级可作为上消化道出血的危险因素.     

Thrombosis in systemic lupus erythematosus: risk and protection

Thrombotic events occur with higher frequency in patients with systemic lupus erythematosus (SLE) than in the general population and are associated with poor outcomes. In addition to the traditional risk factors, other predisposing factors for thrombosis, such as ethnicity, disease duration, disease activity, nephritis, hypertension and, most importantly, the presence of antiphospholipid antibodies, associated or not with the antiphospholipid syndrome, have been reported in patients with SLE. On the other hand, aspirin and antimalarial medications may have a protective thrombotic effect in these patients. Strategies to prevent thrombosis occurrence should be part of the management of patients with SLE; thus, aPL antibodies should be screened in all lupus patients, especially if other risk factors are also present. Stratification of the risks of thrombotic events, smoking cessation and the use of protective medications are important elements of thrombosis prevention. To date, no laboratory test can predict a recurrent thrombotic event and no pharmacogenomics studies that could help with anticoagulation monitoring have been carried out in SLE patients. Advances in these fields may lead to a better understanding of the risk for thrombotic events and their recurrence, as well as choosing (and monitoring) the right anticoagulant agent.     

肝癌门静脉癌栓形成原因的探讨

目的：了解肝癌门静脉癌栓的发生原因;方法：应用彩超前瞻性检测９６例来源不同的肝癌和６０例对照者;结果：肝硬化肝癌者比肝硬化者门静脉逆流发生率高,同一肿块类型来源于肝硬化者门静脉逆流的发生率高;结论：门静脉高压是门静脉逆流的内因,动－门静脉分流是门静脉逆流的外因,门静脉逆流导致门静脉癌栓形成     

Thrombotic complications of myeloproliferative neoplasms: risk assessment and risk‐guided management

Philadelphia‐negative myeloproliferative neoplasms are considered to be acquired thrombophilic states. Thromboses, both arterial and venous (not rarely in unusual sites), are often the initial events leading to the diagnosis. After diagnosis, the yearly incidence of thrombotic events is highly variable, and ranges from approximately 1% to 10%. The identification of patients at risk who may benefit from antithrombotic therapy remains a challenge, and it is currently based on age and history of thrombotic events. However, the predictive value of these clinical characteristics is rather limited. Few prospective studies and even fewer interventional randomized studies are available, and there are no studies designed to formally validate the use of risk stratification. The implementation of laboratory parameters such as leukocytosis and/or the JAK2 V617F mutation into a scoring system may be of interest. The mechanisms at work leading to thrombosis remain largely speculative, but are likely to be complex and multifactorial, with a prominent role of cell–cell interactions, mostly owing to qualitative changes. The long‐term treatment options to prevent thrombosis are, schematically, aspirin alone as primary prevention for the low‐risk patients, and cytoreduction combined with aspirin for the other patients. In very low‐risk young essential thrombocythemia patients, abstention can even be considered. The optimal duration of anticoagulation after a thrombotic event is not established. All antithrombotic therapies should be balanced with the hemorrhagic risk, which can also be increased in these patients.     

Assessing thrombosis risk in patients with idiopathic, diabetic, and postsurgical gastroparesis

Patients with severe gastrointestinal motility disorders are often found to have intravenous access clots or deep venous thrombosis. It has previously been reported that many patients who have intravenous access thrombosis have concomitant thrombotic risk factors. In this study, the goal was to determine the underlying prevalence of hypercoagulable risk in a series of patients with documented gastroparesis. Investigators studied 62 consecutive patients (52 female; mean age, 42 y) who had symptoms of gastroparesis. All patients were evaluated for placement of a gastric neural stimulation device, or they had had one placed previously. Patients underwent a hematologic interview and standardized coagulation measures of thrombotic risk. Laboratory studies measured acquired elevations of Factor VII, Factor VIII, fibrinogen, lupus anticoagulant panel, antiphospholipid antibody panel, homocysteine (in the setting of kidney disease), and activated protein resistance. Investigators also measured congenital factors: Factor VIII (with C-reactive protein levels), antithrombin III, protein C, protein S (total and free), Factor II mutation, Factor V Leiden, methylenetetrahydrofolate reductase, and homocysteine. Fifty-five patients (89%) were found to have detectable hypercoagulable risk factors. Twentyfive of the 62 patients (40%) had a documented history of abnormal clotting, including deep venous thrombosis, intravenous access thrombosis, and pulmonary embolism. All patients with a previous history of thrombosis had detectable clotting abnormalities. Of 56 patients, 40 (71%) had hypercoagulability and did not have diabetes (P=.036), and 20 (36%) had hypercoagulability and no known history of infection. However, this value was not statistically significant when infection and hypercoagulability were compared (P=.408). A high prevalence of acquired and congenital hypercoagulable defects has been observed in patients with gastroparesis, which may predispose them to arterial and venous clots. This unique finding warrants consideration of coagulation evaluation in patients with severe gastroparesis, especially when these patients are placed in high-risk thrombophilic situations, such as hospitalization, prolonged intravenous access, and surgery.     

肝硬化上消化道出血并急性肠系膜上静脉血栓诊治体会

目的 探讨肝硬化上消化道出血患者常规应用止血及抗纤溶药物的利弊.方法 回顾分析我院2008年1月-2010年12月收治的肝硬化并肠系膜上静脉血栓形成3例的临床资料.结果 3例均因呕血、便血入院,均有乙型病毒性肝炎肝硬化病史,诊断为肝硬化上消化道出血后予止血、抗纤溶等治疗1～2周,出现发热、腹胀、腹痛,B超检查提示门静脉主干血栓伴肠系膜上静脉血栓形成.例1、例2予尿激酶溶栓,例3行经颈静脉肝内门体分流术治疗,均痊愈出院.结论 肝硬化上消化道大出血并急性肠系膜上静脉血栓临床较为少见,分析发生原因可能与过度应用止血及抗纤溶药物有关.     

不同分型的肝硬化门静脉血栓的临床特点

目的探讨不同分型的肝硬化门静脉血栓(PVT)的临床特点及差异。方法回顾性分析2016年8月至2019年8月昆明医科大学第二附属医院消化内科收住的肝硬化合并PVT患者107例,依据解剖因素将其分为3组:单纯门静脉主干血栓形成组(主干组,n=17)、单纯分支血栓形成组(分支组,n=35)和门静脉主干并分支血栓形成组(主干+分支组,n=55)。分别从一般临床资料、实验室指标、影像学指标、肝储备及相关评分等方面对不同PVT部位进行比较分析。检验水准取α=0.05,采用分割χ²检验时,校正为α′=0.0125。结果主干+分支组患者更易合并消化道出血,主干组次之,分支组最低,三组间比较差异有统计学意义(χ²=27.832,P<0.01),两两比较示,分支组与主干+分支组、主干组与主干+分支组差异有统计学意义(P<0.0125);主干+分支组D-二聚体水平最高,主干组次之,分支组最低,三组间比较(H=37.439,P<0.01)及两两比较差异均有统计学意义(P<0.05,P<0.01);主干+分支组血浆D-二聚体和纤维蛋白原比值(D/F)最高,主干组次之,分支组最低,三组间比较(H=33.973,P<0.01)及两两比较差异均有统计学意义(P<0.05,P<0.01)。结论血栓累及主干+分支的肝硬化PVT患者,发生消化道出血的风险最高,主干次之,分支最低。D-二聚体、D/F水平的高低可能对不同部位的肝硬化PVT诊断有一定的参考价值。     

Thromboembolic Events Among Patients with Hepatitis C Virus Infection and Cirrhosis: A Matched-Cohort Study

Introduction

Portal vein thrombosis is a known risk among patients with cirrhosis, but the incidence of other thromboembolic events among patients with liver disease is inadequately delineated. This study examined the incidence of venous and arterial thromboembolic events in patients with cirrhosis and hepatitis C virus (HCV) infection and matched comparators.

Methods

Patients diagnosed with HCV or cirrhosis of various etiologies were identified from a large medical claims database and matched by age and sex to comparator cohorts. New-onset diagnoses of venous and arterial thromboembolic events were determined. The incidence rate of each event was calculated and rate ratios computed using Poisson regression models, adjusting for baseline factors.

Results

The study included 22,733 HCV-infected patients and 68,198 comparators, and 15,158 cirrhosis patients and 45,473 comparators. The incidence of any thromboembolic event was 233.4 events per 10,000 person-years for the HCV cohort and 138.5 per 10,000 person-years for the comparators; the adjusted incidence rate ratio for any thromboembolic event was 1.62 (95% confidence interval [CI]: 1.48–1.77). For the cirrhosis patients and comparators, the crude rates of any thromboembolic event were 561.1 and 249.7 per 10,000 person-years, respectively. The adjusted incidence rate ratio was 2.28 (95% CI: 2.11–2.47). Arterial events, especially unstable angina and transient ischemic attack, were the most frequent events seen in both the HCV and cirrhosis cohorts, but venous events, especially portal vein thrombosis, showed a more pronounced elevation in patients with liver disease.

Conclusions

Patients with HCV and cirrhosis of various etiologies are at increased risk of several types of thromboembolic events. Physicians should consider this increased risk when managing patients with liver disease.     

Hypercoagulability in cirrhosis: causes and consequences1

Summary. Decreased levels of most coagulation factors and thrombocytopenia are the main haemostatic abnormalities of cirrhosis. As a consequence, this condition was, until recently, considered as the prototype acquired coagulopathy responsible for bleeding. However, recent evidence suggests that it should, rather, be regarded as a condition associated with normal or even increased thrombin generation. The bleeding events that occur in these patients should, therefore, be explained by the superimposed conditions that frequently occur in this setting. Due to elevated levels of factor VIII (procoagulant driver) in combination with decreased protein C (anticoagulant driver), which are typically found in patients with cirrhosis, a procoagulant imbalance, defined as a partial resistance to the in vitro anticoagulant action of thrombomodulin, can be demonstrated. Whether this in vitro hypercoagulability is truly representative of what occurs in vivo remains to be established. However, the hypothesis that it may have clinical consequences is attractive and deserves attention. The possible consequences that we discuss herein include whether (i) cirrhosis is a condition associated with increased risk of venous thromboembolism or portal vein thrombosis; (ii) the hypercoagulability associated with cirrhosis has any other role outside coagulation (i.e. progression of liver fibrosis); and (iii) anticoagulation should be used in cirrhosis. Although apparently provocative, considering anticoagulation as a therapeutic option in patients with cirrhosis is now supported by a rationale of increasing strength. There may be subgroups of patients who benefit from anticoagulation to treat or prevent thrombosis and to slow hepatic fibrosis. Clinical studies are warranted to explore these therapeutic options.     

Alcoholic liver disease

Liver injury may develop in some people who consume alcohol. The pathogenesis of liver damage in such subjects remains obscure. Major histopathologic features of alcohol-associated liver injury include steatosis, steatonecrosis, and cirrhosis. The clinical manifestations of alcoholic liver disease are nonspecific and range from asymptomatic hepatomegaly to stigmata of portal hypertension with advanced parenchymal failure. The severity of the clinical presentation and the degree of aminotransferase elevation correlate poorly with the liver histopathology, particularly in patients who continue to drink alcohol. Short-term mortality of such patients is best predicted by a composite of clinical and laboratory parameters that are influenced by alcohol consumption as well as by liver disease. Long-term prognosis is determined by residual damage to vital organs (that is, whether or not cirrhosis has developed) and whether or not the patient continues to drink. Current therapy of alcoholic liver disease includes abstinence and correction of nutritional deficiencies. Other therapies are experimental and are best utilized in the setting of controlled clinical trials.     

Clinical manifestations of the prothrombin G20210A mutation in children: a pediatric coagulation consortium study

Summary.  The prothrombin G20210A mutation is a common risk factor for thrombosis which increases the risk of deep vein thrombosis, stroke, and fetal loss. There are few publications of its clinical manifestations in children. Our objective was to determine the clinical manifestations of the prothrombin mutation in children. Via survey of pediatric hematologists, we collected data on children with thrombosis and the prothrombin mutation. Thirty-eight patients with a thrombotic event were identified as having the prothrombin mutation. Children with arterial thrombosis were younger, less than half had additional risk factors present at the time of the event, and had a high frequency of central nervous system thrombosis. Children with venous thrombosis were older, almost always had additional risk factors present, and had thrombosis occur most often in the extremities, although there were also a significant number of events in the central venous and cerebral circulation. There was a striking predilection for central nervous system events as 30% of all the events and 67% of the arterial events occurred there. In all, 14/38 children (37%) had central nervous system thrombosis. Unlike factor V Leiden and deficiencies of proteins C and S which cause venous thromboembolism, the prothrombin mutation in children is often associated with arterial thrombosis and with central nervous system events. In children with the prothrombin mutation and venous thrombosis, other risk factors are usually present. Therefore, children with arterial or venous thrombosis of any location should be evaluated for the presence of the prothrombin mutation.     

肝硬化门静脉血栓形成研究进展

门静脉血栓形成(portal vein thrombosis, PVT)是由多种病因引起门静脉及其主要分支内形成血栓,造成血流完全或部分中断,其中,肝硬化是主要原因之一。近年来,随着对该疾病认识的逐步提高,已有越来越多的研究关注于此。本文就近年来有关肝硬化PVT的研究进展做一综述。     

Hemostasis in Liver Disease: Implications of New Concepts for Perioperative Management

The hemostatic profile of patients with liver diseases is frequently profoundly different from that of healthy individuals. These complex alterations lead to abnormal results from routine laboratory tests, but because of the nature of these assays, they fail to accurately represent the patient’s hemostatic state. Nevertheless, based on abnormal laboratory coagulation values, it has long been assumed that patients with liver disease have a natural bleeding tendency and are protected from thrombosis. This assumption is false; the average patient with liver disease is actually in a state of “rebalanced hemostasis” that can relatively easily be tipped toward both bleeding and thrombosis. The new paradigm of rebalanced hemostasis has strong implications for the clinic, which are presented in this review. There is no evidence that prophylactic transfusion of plasma helps to prevent procedure-related bleeding. In addition, the presence of independent risk factors such as poor kidney status or infections should be carefully assessed before invasive procedures. Furthermore, central venous pressure plays an important role in the risk of bleeding in patients with liver diseases, so during procedures, a restrictive infusion policy should be applied. Finally, thrombosis prophylaxis should not be withheld from patients with cirrhosis or acute liver failure, and clinicians should be alert to the possibility of thrombosis occurring in these patients.     

肝硬化患者胆碱酯酶与总胆汁酸及凝血功能检测的意义

目的 探讨肝硬化患者胆碱酯酶(CHE)、总胆汁酸(TBA)和凝血功能的变化及其临床意义.方法 选取肝炎后肝硬化患者56例,健康对照30例,均检测血清CHE、TBA和血浆凝血酶原时间(PT),活化部分凝血活酶时间(APTT)、纤维蛋白原(Fbg)含量以及凝血酶时间(TT).结果 肝硬化患者CHE、TBA、PT、APTT、Fbg和TT与对照组比较差异均有统计学意义.结论 联合测定CHE、TBA和凝血功能对肝硬化患者的病情判断有重要的临床意义.     

Polymorphisms in coagulation factor genes and their impact on arterial and venous thrombosis

Arterial and venous thromboses, with their clinical manifestations such as stroke, myocardial infarction (MI), or pulmonary embolism, are the major causes of death in developed countries. Several studies in twins and siblings have shown that genetic factors contribute significantly to the development of these diseases. Since the advent of molecular genetics in medicine, it has been a focus of interest to elucidate the role of mutations in various candidate genes and their impact on hemostatic disorders such as arterial and venous thromboses. In this article, we review the current knowledge of the contribution of polymorphisms in coagulation factors to the development of thrombotic diseases. We show that in arterial thrombosis, results are controversial. Only for factor XIII 34Leu a protective effect on the development of myocardial infarction has been demonstrated in several studies. No other single polymorphism in a coagulation factor could be confirmed as a relevant risk factor, although there is evidence for a role of factor V Arg506Gln, factor VII Arg353Gln, and vWF Thr789Ala polymorphisms in patient subgroups. Further studies will be necessary to confirm the value of testing for genetic polymorphisms in arterial thrombosis. A large body of data is available on the role of factor V Arg506Gln and the prothrombin G20210A mutation in venous thrombosis. Some papers already recommend diagnosis and treatment strategies. We will discuss these recent publications on venous thrombosis in our review.     

Chronic venous abnormalities in symptomatic and asymptomatic protein C deficiency

BACKGROUND: Thrombophilia is a frequent medical condition associated with symptomatic deep vein thrombosis (DVT). Unlike other clinical risk factors associated with DVT, such as surgery, thrombophilia has not been demonstrated to be associated with asymptomatic venous thrombotic events. Our aim was to search for asymptomatic sequelae of DVT in a protein C (PC)-deficient family. METHODS: We studied 228 individuals from a large kindred with PC deficiency and performed a systematic ultrasound examination. RESULTS: Among the 203 patients without a known history of venous thrombosis we found seven patients with abnormalities indicative of prior asymptomatic thrombosis: six (7.4%) in the PC-deficient group (n = 81) and only one (0.8%) in the non-deficient group (n = 122). The relative risk for these sequelae associated with PC deficiency was 9.0 (95% CI: 1.1-73.7). CONCLUSIONS: These data suggest that chronic venous abnormalities are frequently present and that thrombotic events in asymptomatic individuals with familial PC deficiency may be underestimated.     

Mechanisms in fluid retention – towards a mutual concept

Fluid retention is a common and challenging condition in daily clinical practice. The normal fluid homoeostasis in the human body is based on accurately counter‐balanced physiological mechanisms. When compromised fluid retention occurs and is seen in pathophysiologically different conditions such as liver cirrhosis, heart and kidney failure, and in preeclampsia. These conditions may share pathophysiological mechanisms such as functional arterial underfilling, which seems to be a mutual element in cirrhosis, cardiac failure, cardiorenal and hepatorenal syndromes, and in pregnancy. However, there are also distinct differences and it is still unclear whether kidney dysfunction or arterial underfilling is the initiating factor of fluid retention or if they happen simultaneously. This review focuses on similarities and differences in water retaining conditions and points to areas where important knowledge is still needed.     

Focal areas of decreased echogenicity in the liver at the porta hepatis

Twelve patients are described in whom solitary focal hypoechoic areas in the liver were observed adjacent to the gallbladder and portal vein. These areas were all homogeneously hypoechoic relative to adjacent liver, had variable shapes but well-defined borders, and were less than 4 cm in diameter. These apparent pseudolesions were incidental findings in patients who had no evidence of significant liver disease, metastatic malignancy, or subsequent evidence of developing liver masses. In one patient, a second sonogram done 21 months after one such area was identified showed no change. In another patient, a computed tomogram done to clarify the finding was normal. Solitary hypoechoic areas in the liver at the porta hepatis fitting the above criteria should be interpreted cautiously. When there is no clinical suspicion of focal liver disease, these areas may be dismissed as insignificant or may be followed. If focal liver disease is suspected, the liver should be assessed by an additional method of examination.