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      2. west china medical publishers
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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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        • 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
        • 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
        • 血友病的治療進展

          血友病是一組X連鎖隱形遺傳性出血性疾病,主要表現為自發性出血或外傷、手術后出血,反復關節肌肉出血可以致殘,危險部位出血甚至威脅生命。替代治療是血友病唯一有效的治療方法,同時需要康復、理療、護理、心理關懷等綜合治療。治療理念已從出血后按需治療發展為預防治療,未來的發展方向將是基因檢測阻斷患者出生,長效凝血因子的輸注以及基因治療等,從而使患者徹底擺脫疾病的困擾。

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        • 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
        • Treatment Status and Economic Burden of People with Hemophilia in Mainland China: A Systematic Review

          Objective To be aware of the treatment status and economic burden of people with hemophilia (PWH) in mainland China, so as to seek the optimal therapy for them. Methods The relevant Chinese and English databases such as CBM, CNKI, VIP, WanFang Database, PubMed, EMbase and The Cochrane Library (Issue 6 of 12, June 2011) were searched in June, 2011. The economic analyses and studies on PWH treatment and economic burden published from 1980 to 2011 were collected. Results The diagnosis and treatment of PWH in mainland China lagged behind. More than 30% of PWH did not receive or occasionally received treatment, and less than 10% received prophylactic therapy. Lots of PWHs still used FFP or cryoglobulin which were easily to cause blood-borne viral diseases. More than half of PWH families could afford a little or completely could not afford the therapy. Low dose prophylactic therapy was cost-efficient than on-demand therapy. Based on the therapy status, it was estimated that approximately RMB 53 844 yuan per year per patient should be put into practice in order to have PWH received low-dose prophylactic therapy, and to prevent 80% of bleeding. Conclusion PWH in mainland China is poor in treatment status and heavy in economic burden, so it is an optimal way to adopt comprehensive care model and low-dose prophylactic therapy in mainland China.

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        • 基層醫院建立血友病小兒專科護理的必要性

          針對基層醫院血友病小兒護理現狀及問題,在參考國外醫療機構血友病護理模式基礎上,指出建立血友病小兒專科護理的必要性,借以提升其血友病評估、治療、宣教、協調、扶持的規范化,提高血友病患兒的生存質量,減少殘疾,促進血友病治療及專科護理的更大發展。

          Release date:2016-09-08 09:16 Export PDF Favorites Scan
        • Pharmacokinetics-guided individualized management of physical activity in people with hemophilia

          Exercise has been increasingly recognized in clinical guidelines as a recommended component of rehabilitation for people with hemophilia (PWH), with evidence supporting appropriate physical activity’s multifaceted benefits. During exercise, the bleeding risk in PWH exhibits a critical correlation with circulating clotting factor activities, where higher factor concentrations demonstrably reduce hemorrhagic events. However, economic constraints limit universal access to high-dose prophylactic clotting factor replacement therapy. Through pharmacokinetics (PK) monitoring of clotting factor, clinicians can strategically tailor exercise types and frequencies, or adjust factor replacement dosages based on activity-specific demands. This individualized approach not only enhances the cost-effectiveness of clotting factor utilization, but also improves safety by mitigating bleeding risks. This article examines the feasibility and recent advancements in PK-guided individualized physical activity prescriptions for PWH, presenting evidence-based insights to inform clinical practice and future research priorities.

          Release date:2025-07-29 05:02 Export PDF Favorites Scan
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          2. 射丝袜