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        find Keyword "血友病" 21 results
        • 全髖解脫術綜合治療血友病一例

          Release date:2016-09-07 02:37 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
        • 血友病性關節炎誤診手術分

          報道6例血友病性關節炎因誤診而行手術治療。誤診原因為病史詢問不詳,遺漏重要的既往史、家族史等;對血友病的骨關節改變缺乏認識;術前常規化驗結果正常疑診血友病者,應行凝血酶原消耗糾正試驗,有條件時,可動態測定血中凝血因子活性。避免術中、術后嚴重并發癥出現,并提出誤診手術后采取的有效措施。

          Release date:2016-09-01 11:14 Export PDF Favorites Scan
        • 慢性呼吸衰竭COPD 患者血清維生素E 及血管性血友病因子水平測定及意義

          目的 探討慢性呼吸衰竭COPD 患者血清維生素E( VE) 和血管性血友病因子( vWF)水平和臨床意義。方法 熒光分光光度計測定50 例慢性呼吸衰竭COPD 患者的血清VE 水平, 免疫比濁法測定其血清vWF水平, 與20 例正常志愿者比較。結果 與正常對照組比較, 慢性呼吸衰竭的COPD 患者血清VE 水平顯著降低[ ( 31. 79 ±11. 17) μmol /L 比( 68. 36 ±21. 03) μmol /L, P lt;0. 05] ,而血清vWF 水平明顯增高[ ( 81. 79 ±21. 06) U/L比( 41. 98 ±11. 64) U/L, P lt;0. 05] 。結論 慢性呼吸衰竭COPD 患者血氧分壓降低, 機體缺氧導致氧自由基清除能力降低, 對血管內皮細胞造成潛在損傷。

          Release date:2016-08-30 11:53 Export PDF Favorites Scan
        • The Role of Ⅷ Factor and Von Willebrand Factor in Acute Exacerbation of Chronic Obstructive Pulmonary Disease Complicated with Deep Venous Thrombosis

          ObjectiveTo investigate the prevalence and risk factors of deep venous thrombosis (DVT) in patients with acute exacerbation of chronic obstructive pulmonary disease (COPD). MethodsOne hundred and eight patients with acute exacerbation of COPD admitted between March 2009 and September 2010 were consecutively included.On admission,color Doppler ultrasound examination of lower extremities was performed for diagnosing DVT in all cases.The patients with DVT were compared with those without DVT in demographics,symptoms,physical signs,risk factors and laboratory examination including Ⅷ factor and von Willebrand factor (VWF). ResultsAmong 108 patients with acute exacerbation of COPD,DVT was detected in 11 cases (10.1%).In the COPD patients with DVT,the duration of hospitalization was longer (P<0.001) and the mechanical ventilation requirement increased (P=0.024) compared those without DVT.Other indicators for more possibility of DVT were immobility over 3 days (P=0.001),pneumonia as concomitance (P=0.004),type Ⅱ respiratory failure (P=0.011),and current smoking (P=0.002).The plasma leukocytes,D-dimer and Ⅷ factor levels were significantly higher in the COPD patients with DVT than those without DVT (P=0.005;P<0.001;P=0.009). ConclusionThe incidence of DVT in patients with acute exacerbation of COPD is 10.1%.The prevalence of DVT is higher in distal extremities than that in proximal,especially the intermuscular veins.The patients with acute exacerbation of COPD have a higher risk of DVT when immobilized over 3 days,complicated by pneumonia or type Ⅱ respiratory failure,and having a high levels of plasma leukocytes,D-dimer and Ⅷ factor.

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        • 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
        • 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
        • Plasma von Willebrand Factor Propeptide Level in Patients with Thrombotic Thrombocytopenic Purpura

          【摘要】 目的 探討血栓性血小板減少性紫癜(thrombotic thrombocytopenic purpura,TTP)患者血管內皮損傷程度,以及不同類型TTP之間血管內皮損傷差異性。 方法 納入2005年4月-2010年12月特發性TTP患者17例(A組),繼發性TTP患者15例(B組),骨髓移植相關TTP患者2例(C組),疑似TTP患者11例(D組),共45例;另選取健康體檢志愿者為對照組10例(E組)。采用雙夾心酶聯免疫吸附試驗測定血管性血友病因子前肽(von Willebrand factor propeptide,vWFpp)水平。 結果 vWFpp水平為其與正常混合血漿的比值, A組為2.2,B組為2.34,C組為2.795,D組為1.72,E組為1.08。A、B、C、D組患者vWFpp水平與E組比較,差異有統計學意義(Plt;0.05),A、B、C、D組間比較,差異無統計學意義(Pgt;0.05)。 結論 TTP患者vWFpp水平明顯增高,提示血管內皮損傷明顯,但vWFpp水平不能用于鑒別TTP類型。【Abstract】 Objective To explore the severity of endothelium injury in patients with thrombotic thrombocytopenic purpura (TTP) and the differences among different subtypes of TTP. Methods The clinical data of 45 patients with TTP diagnosed between April 2005 and December 2010 were retrospectively analyzed. von Willebrand factor propeptide (vWFpp) was measured by sandwich ELISA in 17 patients with idiopathic TTP (group A), 15 patients with secondary TTP (group B), 2 patients with transplantation associated TTP (group C), 11 patients with suspected TTP (group D) and 10 control healthy volunteers (group E). Results Median times of vWFpp of the five groups were 2.2, 2.34, 2.795, 1.72, and 1.08 respectively. Plasma vWFpp levels of the first four groups didn′t differ much between each other (Plt;0.05), but the differences were significant compared with the data in the control group (Pgt;0.05). Conclusions Significantly increased vWFpp level in patients with TTP indicates obvious endothelium injury. Nevertheless, it could not be used to differentiate TTP types.

          Release date:2016-09-08 09:27 Export PDF Favorites Scan
        • 血友病B患者行腸息肉切除術后消化道出血的護理一例

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        • A Preliminary Study on Treatment of Joint Bleeding in Children with Severe and Moderate Hemophilia A: Low-dose Short-course Infusion of Recombinant Human Factor Ⅷ Concentration as Secondary Prophylaxis

          【摘要】 目的 探討重組人凝血因子Ⅷ制劑小劑量短程預防性輸注能否有效減少中重度血友病A患兒關節出血問題。 方法 對2008年11月-2009年4月期間就診的13例年齡3~11歲的中重度血友病A患兒,均在為期2個月內接受重組人凝血因子Ⅷ 2次/周、間隔3 d、每次7.5~10.0 U/kg的靜脈預防性輸注,記錄治療前2個月與治療2個月時關節出血次數,以及同一關節反復發生出血的情況。 結果 治療前關節出血的發生次數為(3.77±2.13)次,治療后關節出血的發生次數為(0.46±0.87)次,治療前后比較,差異有統計學意義(Plt;0.01);治療前靶關節出血的發生率為35.7%,治療后靶關節出血的發生率為0.0%,治療前后比較,差異有統計學意義(Plt;0.01)。患兒治療成本約510~680元/(kg?2個月)。 結論 重組人凝血因子Ⅷ制劑小劑量短療程預防性輸注能有效減少中重度血友病A患兒關節出血次數,同時可有效減少靶關節出血的發生率,從而在一定程度上保護關節的功能。治療費用相對可接受。【Abstract】 Objective To evaluate the efficacy of low-dose short-course infusion of recombinant human factor Ⅷ concentration in treating joint bleeding in children with severe and moderate hemophilia A. Methods Thirteen children aged 3 to 11 years old with severe or moderate hemophilia A were included in the present study from November 2008 to April 2009. For children in the treatment group, they were treated with low-dose short-course infusion of recombinant human factor Ⅷ concentration with a dose of 7.5-10.0 U/kg twice weekly as secondary prophylaxis for two months. The incidence of joint bleeding 2 months before treatment (control group) and during the 2 months of treatment (treatment group) was observed. Moreover, the incidence of their target joint bleeding was measured in both groups. Results Children in the control group had (3.77±2.13) joint bleedings while children in the treatment group had (0.46±0.87) joint bleedings, which was obviously lower than those in the control group (Plt;0.01). Meanwhile, the incidence of target joint bleeding in the treatment group was 0%, which was obviously lower than that in the control group (35.7%) (Plt;0.01). In the treatment group, the costs of treatment were about RMB 510-680 yuan/kg every 2 months. Conclusions Treatment with low-dose short-course infusion of recombinant human factor Ⅷ concentration can effectively decrease joint bleeding in children with severe and moderate hemophilia A, and can effectively decrease the incidence of target joint bleeding. Therefore, this method may play an important role in protection of the joint function in those patients at an acceptable cost.

          Release date:2016-09-08 09:26 Export PDF Favorites Scan
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