急性肠系膜静脉血栓形成16例临床分析

目的　分析急性肠系膜静脉血栓形成 (ASMVT)的临床特征和诊断方法。方法　对北京友谊医院 1985年 10月至 2 0 0 2年 10月确诊的 16例ASMVT的临床资料进行分析 ,并结合文献 ,总结ASMVT的临床特征和诊断方法。结果 16例ASMVT患者 ,年龄 19～ 6 4岁 (平均 4 5 9岁 )。主要症状有腹痛 ( 16 / 16 )、腹胀 ( 16 / 16 )、呕吐 ( 10 / 16 )、发热 ( 8/ 16 ) ,主要体征有腹部膨隆 ( 16 / 16 )、腹膜刺激征( 16 / 16 )。误诊率 75 % ( 12 / 16 ) ,死亡率 4 4 % ( 7/ 16 )。结论　ASMVT的临床表现有一定的特征 ,早期彩色超声检查、诊断性腹腔穿刺、结合CT或肠系膜上动脉造影异常发现可对ASMVT做出正确的诊断 ,早期手术可以降低死亡率     

Characterization of an immunologic polymorphism (D79H) in the heavy chain of factor V.

BACKGROUND: During the study of a family with hereditary factor (F)V deficiency (FV Amersfoort, 1102 A > T in exon 7) we identified an individual with 5% FV heavy chain antigen (FV(HC)) and 50% FV light chain antigen (FV(LC)). Further testing revealed that apart from the FV Amersfoort allele a second variant FV allele was segregating in this family, which encodes for a FV molecule with a reduced affinity for mAb V-23 used in the FV heavy chain ELISA (ELISA(HC)). OBJECTIVE: Identification and characterization of the molecular basis responsible for the reduced affinity of the variant FV for mAb V-23. METHODS: Family members of the proband were screened for mutations in the exons coding for the heavy chain of FV, after which the recombinant variant FV could be generated and characterized. Next, the cases and controls of the Leiden Thrombophilia Study (LETS) were genotyped for carriership of the variant FV. RESULTS: In the variant FV allele a polymorphism in exon 3 (409G > C) was identified, which predicts the replacement of aspartic acid 79 by histidin (D79H). Introduction of this mutation in recombinant FV confirmed that it reduces the affinity for binding to mAb V-23. The substitution has no effect on FV(a) stability and Xa-cofactor activity. In Caucasians the frequency of the FV-79H allele is approximately 5%. Analysis of the LETS revealed that the FV-79H allele is not associated with FV levels (FV(LC)), activated protein C sensitivity (using an activated partial thromboplastin time-based test) or risk of venous thrombosis (OR 1.07, CI 95: 0.7-1.7). CONCLUSION: The D79H substitution in FV should be considered as a neutral polymorphism. The monoclonal antibody V-23, which has a strongly reduced affinity for FV-79H, is not suitable for application in diagnostic tests.     

Von Willebrand factor, platelets and endothelial cell interactions

Summary.  The adhesive protein von Willebrand factor (VWF) contributes to platelet function by mediating the initiation and progression of thrombus formation at sites of vascular injury. In recent years there has been considerable progress in explaining the biological properties of VWF, including the structural and functional characteristics of specific domains. The mechanism of interaction between the VWF A1 domain and glycoprotein Ibα has been elucidated in detail, bringing us closer to understanding how this adhesive bond can oppose the fluid dynamic effects of rapidly flowing blood contributing to platelet adhesion and activation. Moreover, novel findings have been obtained on the link between regulation of VWF multimer size and microvascular thrombosis. This progress in basic research has provided critical information to define with greater precision the role of VWF in vascular biology and pathology, including its possible involvement in the onset of atherosclerosis and its acute thrombotic complications.     

Decreased Risk of Renal Allograft Thrombosis Associated With Interleukin-2 Receptor Antagonists: A Report of the NAPRTCS

Graft thrombosis is the most common cause of first year graft failure in pediatric renal transplantation. The North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) database was analyzed for cases of graft failure due to thrombosis among patients transplanted from 1998 to 2004. The impact of interleukin-2 (IL-2) receptor antagonists as induction therapy was determined. There were a total of 51 graft failures due to thrombosis among the 2750 reported renal transplants (1.85%) (95% CI (1.39%, 2.41%)). This represents the most common cause of graft loss during the first year post-transplant accounting for 35% of first year losses and 18% of all graft losses. The incidence of thrombosis among patients who received IL-2 receptor antibodies was 1.07% (12/1126) compared to 2.40% (39/1624) among patients who did not (OR 0.44, 95% CI 0.23, 0.84, p = 0.014). Use of IL-2 receptor blockade was the only significant prognostic factor in a multivariate model with previously identified risk factors. Analysis of NAPRTCS data found that the use of IL-2 receptor antibodies as induction therapy is associated with a significantly decreased risk of graft failure due to thrombosis. This provocative finding requires further investigation to determine whether thrombotic failure can be decreased by this therapeutic strategy.     

Cerebral venous malformation complicated by spontaneous thrombosis

A case of spontaneous thrombosis and infarction leading to death as complications of a cerebral venous malformation in a 13-yearold boy is reported. This is the first published report of this type of complication occurring in a case of venous angioma. While the biologic behavior of cerebral venous malformations has suggested that they are benign in nature, and the results of surgical management have encouraged a conservative approach, the present case illustrates a potential complication and argues against the assumption that these malformations are completely benign in nature.     

Recurrent venous thrombosis following free flap surgery: The role of heparin-induced thrombocytopenia

Complications following free tissue transfer have been well established in the literature. Common and rare causes of free flap failure must be addressed by the treating surgeon when microvascular patency is threatened. With the evolution and prevalence of microsurgery, ‘rare’ causes of free flap failure will become increasingly frequent. A high index of suspicion must be established in patients with multiple failed operative interventions. A case of recurrent free flap failure secondary to heparin-induced thrombocytopenia is presented in a patient with a history of squamous cell carcinoma of the floor of the mouth, and a long-standing history of alcohol and tobacco consumption.     

Incremental Value of Three-Dimensional Echocardiography Over Transesophageal Multiplane Two-Dimensional Echocardiography in Qualitative and Quantitative Assessment of Cardiac Masses and Defects

In the present study, we compared three-dimensionally (3-D) reconstructed images with multiplane two-dimensional (2-D) transesophageal echocardiographic (TEE) images in 17 patients with various cardiac masses and defects. To overcome the problem of making measurements from 3-D reconstructed images, we carefully "dissected" the 3-D dataset using paraplane and anyplane 2-D sections, which were then used to obtain the maximum sizes of the cardiac masses and defects. Of the 15 vegetations and 9 abscesses detected by 3-D TEE in 7 patients, only 8 (53%) vegetations and 4 (44%) abscesses were detected by multiplane 2-D TEE (P < 0.02). Also, the exact anatomical location, shape, geometry, and extent of various cardiac masses and defects were more clearly delineated by 3-D than 2-D TEE. The maximum dimensions of cardiac masses and defects were larger by 3-D than by 2-D TEE in 17 (89%) of the 19 lesions available for comparison (P < 0.002). In addition, 3-D TEE correlated more closely than 2-D TEE when compared to surgical measurements in three patients in whom they were available. Thus, it would appear that in several instances, the exact size of the cardiac lesion could only be assessed by analysis of the 3-D volumetric dataset. Out preliminary study has demonstrated the superiority of transesophageal 3-D reconstruction over multiplane 2-D TEE in both qualitative and quantitative assessment of various cardiac mass lesions and pathological defects.     

Cavernous sinus thrombosis following odontogenic and cervicofacial infection

Summary Cavernous sinus thrombosis (CST) is rarely seen clinically as a complication of infectious processes since the discovery of penicillin. At the present time, dental abscess is an uncommon cause of CST. We now report our experiences with a 60-year-old diabetic male, who developed CST 38 days after extraction of an infected upper third molar tooth. The importance of eradicating regional cervicofacial foci of infection is stressed.