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find Keyword "血友病" 21 results
  • CHANGES OF BLOOD COAGULATION FACTORS INDEPENDENT OF VITAMIN K IN PATIENTS WITH BILIARY TRACT DISEASES

    Platelet aggregation test (PAgT), platelet adhesion test (PAdT), thromboplastic activity of factor Ⅷ (FⅧ∶c), antithrombin Ⅲ activity (AT-Ⅲ∶a), antithrombin Ⅲ antigen (AT-Ⅲ∶Ag), von willebrand factor (vWF) and fibrinogen (Fg) were measured in 33 patients with biliary tract diseases and 24 normal individuals. The results showed that there was no significant difference in PAgT, PAdT, AT-Ⅲ∶a and AT-Ⅲ∶Ag between the two groups (P>0.05). Fg increased more significantly in biliary tract disease than in the controls (P<0.01). FⅧ∶c increased more significantly in patients with obstructive jaundice than in that of nonjaundiced and the controls (P<0.01). The levels of vWF increased higher and higher in the sequence of patients with no jaundice, obstructive jaundice due to benign diseases and obstructive jaundice due to malignancy(P<0.01). In conclusion, Fg, FⅧ∶c and vWF increased in patients with biliary tract disease.

    Release date:2016-08-29 03:19 Export PDF Favorites Scan
  • SURGICAL TREATMENT FOR HEMOPHILIA INDUCED LESIONS OF FOOT AND ANKLE

    Objective To explore perioperative management and postoperative effectiveness of hemophilia induced lesions of the foot and ankle. Methods Between June 1998 and February 2012, 10 cases (12 feet) of hemophilia induced lesions of the foot and ankle were treated with surgery, including 9 cases (11 feet) of hemophilia A and 1 case (1 foot) of hemophilia B. Single foot was involved in 8 cases and both feet in 2 cases, including 3 left feet and 9 right feet. All were males, aged from 13-41 years (mean, 22.6 years). Disease duration was 5-84 months (mean, 32.2 months). Preoperative American Orthopaedic Foot and Ankle Society (AOFAS) score was 43.2 ± 21.1. Short Form 36 Health Survey Scale (SF-36) score was 45.4 ± 20.0. All patients were given clotting factors (2 000-3 500 U) for pre-experiment and clotting factors substitution therapy was performed perioperatively. Four cases (4 feet) underwent arthrodesis, and 7 cases (8 feet) underwent Achilles tendon lengthening/tendon transposition (1 patient underwent tendon lengthening on the left foot and arthrodesis on the right foot). Results The operation time was 65-265 minutes (mean, 141.1 minutes); 1 case had 400 mL blood loss and 200 mL autogenous blood transfusion, the other cases had less than 50 mL blood loss and no blood transfusion. Wounds healed by first intention in all patients, no postoperative infection, deep vein thrombosis, or other complications occurred. All cases were followed up 6 months to 14 years and 3 months (median, 22 months). The X-ray films at last follow-up showed the patients undergoing arthrodesis obtained complete joint fusion. AOFAS scores at postoperative 6 months and last follow-up were 78.8 ± 14.7 and 75.8 ± 14.5, respectively; SF-36 scores were 76.6 ± 13.1 and 75.5 ± 13.2, respectively; and significant differences were found when compared with preoperative scores (P lt; 0.05), but no significant difference between postoperative 6 months and last follow-up (P gt; 0.05). Conclusion For patients with hemophilia induced lesions of the foot and ankle, surgical treatment could relieve foot and ankle pain and improve the function. Clotting factors pre-experiment at preoperation and substitution therapy at perioperation can reduce the risk of severe postoperative hemorrhage.

    Release date:2016-08-31 04:06 Export PDF Favorites Scan
  • 基层医院建立血友病小儿专科护理的必要性

    针对基层医院血友病小儿护理现状及问题,在参考国外医疗机构血友病护理模式基础上,指出建立血友病小儿专科护理的必要性,借以提升其血友病评估、治疗、宣教、协调、扶持的规范化,提高血友病患儿的生存质量,减少残疾,促进血友病治疗及专科护理的更大发展。

    Release date:2016-09-08 09:16 Export PDF Favorites Scan
  • Pharmacoeconomic studies on coagulation factor Ⅷ for the treatment of hemophilia A: a systematic review

    ObjectiveTo systematically review the pharmacoeconomics research of coagulation factor Ⅷ for the treatment of hemophilia A. MethodsPubMed, EMbase, Web of Science, The Cochrane Library, CNKI, VIP and WanFang Data databases were electronically searched to collect pharmacoeconomic studies of coagulation factor Ⅷ for the treatment of hemophilia A from inception to February 2022. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies; then, qualitative systematic review was carried out from the aspects of research model, research parameters and uncertainty analysis. ResultsA total of 17 pharmacoeconomic studies were included. The overall quality of the included literature was relatively high, and most of them conformed to the basic framework of pharmacoeconomic research; however, there were still differences and deficiencies in model setting and parameter selection. Most results of the study evaluation showed that prophylaxis of coagulation factor Ⅷ had cost-effectiveness advantages over on-demand treatment. ConclusionCurrent evidence shows that the preventive treatment of coagulation factor Ⅷ may have certain cost-effectiveness advantages compared with on-demand treatment; however, the adaptability of this conclusion to China still needs to be analyzed.

    Release date:2023-01-16 02:58 Export PDF Favorites Scan
  • The Predictive Value of APACHEⅡ Score and von Willebrand Factor on Severity and Prognosis of Acute Respiratory Distress Syndrome

    Objective To analyze the predictive value of von Willebrand factor (vWF) and acute physiology and chronic health evaluation Ⅱ (APACHEⅡ) score on severity and prognosis of acute respiratory distress syndrome (ARDS). Methods The ARDS patients who were admitted between January 2010 and May 2012 were recruited in the study. APACHEⅡ score and plasma vWF were detected on the first day and the third day after diagnose of ARDS. The patients were divided intoasurvival group andadeath group according the prognosis. The predictive value of vWF and APACHEⅡ score on prognosis were analyzed by the receiver operating characteristic (ROC) curve. Lung injury score was calculated and its relationship with vWF and APACHEⅡ score were analyzed. Results One-hundred and twelve cases of ARDS were enrolled. There were no significant differences between the survival group and the death group in sex, age, respiration rate, blood pressure, white blood cells, procalcitonin or C-reactive protein (P > 0.05). On the first day after diagnosis of ARDS, the APACHEⅡ score and vWF level of the survival group were significantly lower than those in the death group (P < 0.05). On the third day, the APACHEⅡ score was increased but vWF level declined compared with those on the first day (P < 0.05). On the first day, lung injury score of the survival group was 1.7±0.4, significantly lower than that in the death group (2.5±0.6). On the third day, lung injury score in the survival group decreased, while lung injury score of the death group was significantly increased (P < 0.05). On the first day, vWF and APACHEⅡ score were positively correlated with lung injury score (r=0.75, P < 0.05; r=0.79, P < 0.05), respectively. On the first day, the area under the ROC curve of APACHEⅡ score and vWF were 0.87 and 0.91, respectively (P < 0.05). Conclusion APACHEⅡ score and vWF have high diagnostic value in evaluating the degree of lung injury and predicting the prognosis of patients with ARDS.

    Release date:2016-10-02 04:56 Export PDF Favorites Scan
  • 血友病性关节炎误诊手术分

    报道6例血友病性关节炎因误诊而行手术治疗。误诊原因为病史询问不详,遗漏重要的既往史、家族史等;对血友病的骨关节改变缺乏认识;术前常规化验结果正常疑诊血友病者,应行凝血酶原消耗纠正试验,有条件时,可动态测定血中凝血因子活性。避免术中、术后严重并发症出现,并提出误诊手术后采取的有效措施。

    Release date:2016-09-01 11:14 Export PDF Favorites Scan
  • Expression of von Willebrand Factor in Human Umbilical Vein Endothelial Cells Infected by Aspergillus Fumigatus Hypha

    Objective To observe the levels of von Willebrand factor ( vWF) expressed by human umbilical vein endothelial cells ( HUVECs) infected by aspergillus fumigatus ( AF) alone or treatment with cytochalasin D, N-cadherin monoclonal antibody, dexamethasone, respectively, so as to explore the mechanism of angioinvasion in invasive aspergillosis. Methods An in vitro model of HUVECs infected by AF hypha was established. The experiment included six groups, ie. a sham control group, a TNF-αgroup, an AF hypha group, a cytochalasin D group, a N-cadherin antibody group, and a dexamethasone group. Cell supernatants were collected to detect the levels of vWF at 2 h, 6 h, 12 h, and 18 h by enzyme linked immunosorbent assay ( ELISA) . Results Compared with that of vWF at 2 h, the level was higher at 18 h in the sham controlgroup and the TNF-αgroup, and higher at 6 h, 12 h, and 18 h in the other groups( P lt; 0. 05) . Compared with the sham control group, the level of vWF in each experiment group increased at 2 h, 6 h, 12 h, and 18 h except that in the N-cadherin antibody group at 2 h ( P lt; 0. 05) . The level of vWF in TNF-α group was higher than that in the AF hypha group at 2 h, but lower at 18 h. ( P lt; 0. 05) . The level of vWF was not significantly different between the cytochalasin D group and the AF hypha group at each time point. The level of vWF was lower in the N-cadherin antibody group than that in the AF hypha group at 2 h and 6 h ( P lt;0. 05) . The level of vWF was not significantly different between the dexamethasone group and the AF hypha group at each time point. Conclusion HUVECs infected by AF hypha overexpress vWF. N-cadherinmonoclonal antibody can reduce the expression of vWF, but cytochalasin D or dexamethasone has no significant effect on it.

    Release date:2016-08-30 11:53 Export PDF Favorites Scan
  • 慢性呼吸衰竭COPD 患者血清维生素E 及血管性血友病因子水平测定及意义

    目的 探讨慢性呼吸衰竭COPD 患者血清维生素E( VE) 和血管性血友病因子( vWF)水平和临床意义。方法 荧光分光光度计测定50 例慢性呼吸衰竭COPD 患者的血清VE 水平, 免疫比浊法测定其血清vWF水平, 与20 例正常志愿者比较。结果 与正常对照组比较, 慢性呼吸衰竭的COPD 患者血清VE 水平显著降低[ ( 31. 79 ±11. 17) μmol /L 比( 68. 36 ±21. 03) μmol /L, P lt;0. 05] ,而血清vWF 水平明显增高[ ( 81. 79 ±21. 06) U/L比( 41. 98 ±11. 64) U/L, P lt;0. 05] 。结论 慢性呼吸衰竭COPD 患者血氧分压降低, 机体缺氧导致氧自由基清除能力降低, 对血管内皮细胞造成潜在损伤。

    Release date:2016-08-30 11:53 Export PDF Favorites Scan
  • 全髋解脱术综合治疗血友病一例

    Release date:2016-09-07 02:37 Export PDF Favorites Scan
  • 血友病患儿合并先天性心脏病的围手术期护理一例

    Release date:2017-11-24 10:58 Export PDF Favorites Scan
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