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.
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.
Objective To investigate whether the sleep-induced hypoxemia ( SIH) at different time and different level have different effects on pulmonary emphysema and coagulation systemfunction in the rats with pulmonary emphysema. Methods Thirty Wistar rats were randomly divided into three groups( n = 10 in each group) . All rats were exposed to cigarette smoke twice a day ( 30 min each time) . From29th day on, the rats in Group A ( pulmonary emphysema with short SIH) were also exposed to mixed gas of 12. 5% oxygen for 1. 5 hours during sleeping time every day ( the expose time was divided into 4 periods, 22. 5 min each) . The rats in Group B ( pulmonary emphysema with mild SIH) were also exposed to mixed gas of 15% oxygen for three hours during sleeping time every day( the expose time was divided into 4 periods, 45 min each) . The rats in Group C( pulmonary emphysema with standard SIH) were also exposed to mixed gas of 12. 5% oxygen for three hours during sleeping time every day( the expose time was divided into 4 periods,45 min each) . After continuous exposure for 56 days, the rats were sacrificed. Semi-quantitative image analytic method was employed for histopathological analysis including pathological score of lungs, mean linear intercept ( MLI) and mean alveolus number( MAN) . ATⅢ, FIB, vWF, FⅧ were measured. Results All animals in three groups manifested the histopathological features of emphysema. Pathological scores of lungs and MLI of every group were significantly different from each other( F = 21. 907, F = 18. 415, all P lt; 0. 05) , Group A [ ( 61. 90 ±4. 25) % , ( 92. 45 ±1. 78) μm] and Group B[ ( 64. 60 ±3. 95) % , ( 92. 80 ±3. 65) μm] were significantly lower than Group C[ ( 73. 30 ±3. 86) % , ( 99. 32 ±2. 81) μm, q= 8. 96, q =6. 84, q = 12. 64, q =9. 65, all P lt; 0. 05] . Levels of FIB were significantly different among three groups ( F = 20. 592, P lt; 0. 05) while FIB in Group A[ ( 189. 98 ±5. 29) mg/ dL] and Group B[ ( 182. 70 ±2. 78) mg /dL] were significantly lower than that in Group C[ ( 198. 40 ±7. 37) mg/ dL, q = 4. 86, q= 9. 07, all P lt; 0. 05] , and FIB in Group A was significantly higher than that in Group B( q = 4. 20, P lt; 0. 05) . Levels of FⅧ were significantly different from each other( F = 33. 652, P lt;0. 05) while FⅧ in Group A[ ( 232. 26 ±4. 17) % ]and Group B[ ( 242. 53 ±14. 50) % ] were significantly lower than that in Group C[ ( 303. 25 ±32. 93) % ,q= 10. 73, q = 9. 18, all P lt; 0. 05] . Conclusions Pulmonary emphysema and hypercoagulable states increases with time and severity of SIH in rats with pulmonary emphysema. The elevated activity of blood coagulation factor may be a critical role in the hypercoagulable states.
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.
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.