【摘要】 目的 探讨血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura，TTP）患者血管内皮损伤程度，以及不同类型TTP之间血管内皮损伤差异性。 方法 纳入2005年4月-2010年12月特发性TTP患者17例（A组），继发性TTP患者15例（B组），骨髓移植相关TTP患者2例（C组），疑似TTP患者11例（D组），共45例；另选取健康体检志愿者为对照组10例（E组）。采用双夹心酶联免疫吸附试验测定血管性血友病因子前肽（von Willebrand factor propeptide，vWFpp）水平。 结果 vWFpp水平为其与正常混合血浆的比值， A组为2.2，B组为2.34，C组为2.795，D组为1.72，E组为1.08。A、B、C、D组患者vWFpp水平与E组比较，差异有统计学意义（Plt;0.05），A、B、C、D组间比较，差异无统计学意义（Pgt;0.05）。 结论 TTP患者vWFpp水平明显增高，提示血管内皮损伤明显，但vWFpp水平不能用于鉴别TTP类型。【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.
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.
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.
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.
Objective To investigate the clinical outcomes of total knee arthroplasty (TKA) by using the condylar constrained knee prosthesis in the treatment of destructive hemophilic arthritis. Methods Between September 2007 and July 2015, 8 cases (8 knees) of destructive hemophilic arthritis accepted TKA by using condylar constrained knee prosthesis. All patients were male, aged 22 to 56 years, with an average age of 35 years. The disease course of hemophilia A was 3-30 years (mean, 17.3 years). Preoperative range of motion (ROM) was (68.1±32.6)°; the flexion deformity was (14.38±16.13)°. Six patients had valgus of 7-35° (mean, 17.3°), of whom, one had fixed dislocation of patella; and one had varus of 15°. Hospital for Special Surgery (HSS) knee score was 52.5±12.9. Pre-operative X-ray film examination demonstrated narrowing of the knee gap and cystic degeneration of articular cartilage and subchondral bone. Results All patients achieved primary wound healing, and were followed up 1-9 years (mean, 5 years). Tense blister with common peroneal nerve damage and extension penetrating into distal tibial cortex occurred in 1 case respectively, which were cured corresponding treatment. At last follow-up, the knee ROM and the flexion deformity were significantly improved to (98.1±8.9)° and (0.63±1.77)° (t=–2.527,P=0.036;t=2.396,P=0.047). At 2 weeks after operation and last follow-up, the HSS scores were significantly increased to 77.3±11.0 and 85.0±9.0 (P<0.05). X-ray film showed that lower extremity alignment returned to normal in patients with varus and valgus. Conclusion Good curative effect can be get by using condylar constrained knee prosthesis in TKA for the treatment of destructive hemophilic arthritis.
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.
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.