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        west china medical publishers
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        find Keyword "血友病" 21 results
        • 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
        • 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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        • 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
        • 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
        • 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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        • 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
        • 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
        • 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
        • 血友病患兒合并先天性心臟病的圍手術期護理一例

          Release date:2017-11-24 10:58 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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