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.
ObjectiveTo collect and analyze the clinical data and quality of life of hemophilic children, understand the present condition of these patients in Sichuan Province, and analyze related influencing factors.
MethodWe retrospectively analyzed the clinical data of hemophilic children treated in our hospital from January 1, 2008 to May 30, 2015.
ResultsThere were 92 child patients from Sichuan Province with a median age of 9.6 years old (ranging from 3.6 to 18.0). There were 87 cases (94.6%) of hemophilia A and 5 (5.4%) of hemophilia B; the number of light cases was 4 (4.3%), of moderate cases was 67 (72.8%), and of severe cases was 21 (22.9%); eighteen (19.6) of the patients had family history. First bleeding episode occurred at a median age of 11 months (0-48 months). Mild bleeding occurred in 23 cases (25.0%), moderate bleeding in 31 cases (33.7%), and severe bleeding in 38 cases (41.3%). First bleeding site was mainly the mucous membrane of the skin, followed by the joint muscles. Fist joint bleeding occurred at a median age of 18 months (2-107 months). Forty-six patients (59.0%) had severe joint damage during the course of the disease. Intracranial hemorrhage occurred in 4 patients (4.3%), among whom 3 were cured and discharged from the hospital, and 1 had neurological sequelae. Median diagnostic age was 12 months (0-120 months). Sixty-two (67.4%) were diagnosed in a short period of time, 9 (9.8%) in a mediate period of time, and 21 (22.8%) in a long period of time. Forty (43.5%) of the patients had been given sufficient coagulation factors, while all the others had not received sufficient replacement therapy. Fifty-six (60.9%) children had received prophylactic treatment. First prophylaxis was administrated at a median age of 36 months (1-199 months), but 27 (48.2%) discontinued. The median score of the 29 retreated Disease Burden Scale was 22.7±11.6 (4-43), and among them, 11 (37.9%) could not care for themselves. Twenty-three participated in the assessment of social activity ability, among whom, 2 did not attend school, and 6 could not take part in the assessment because of school learning. Correlation analysis showed that there was no significant relationship between diagnostic timing and family history (P=0.795) or between diagnostic timing and areas they came from (P=0.495). However, significant association was found between diagnostic timing and the severity of first bleeding (r=0.392, P=0.035). Disease burden of family was significantly correlated with the number of target joints (r=0.370, P=0.048), and was not closely related with area, severity of bleeding, frequency of hemorrhage, medical insurance, or physical and social activities.
ConclusionsThe general diagnosis and treatment condition of child hemophilia in Sichuan is relatively under-developed with a high prevalence of joint damage, poor quality of life, and high disease burden to the family. Improvement in the care of hemophilia children is urgently needed.
【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.
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 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 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.
