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find Keyword "血友病" 21 results
  • 慢性呼吸衰竭COPD 患者血清维生素E 及血管性血友病因子水平测定及意义

    目的 探讨慢性呼吸衰竭COPD 患者血清维生素E( VE) 和血管性血友病因子( vWF)水平和临床意义。方法 荧光分光光度计测定50 例慢性呼吸衰竭COPD 患者的血清VE 水平, 免疫比浊法测定其血清vWF水平, 与20 例正常志愿者比较。结果 与正常对照组比较, 慢性呼吸衰竭的COPD 患者血清VE 水平显著降低[ ( 31. 79 ±11. 17) μmol /L 比( 68. 36 ±21. 03) μmol /L, P lt;0. 05] ,而血清vWF 水平明显增高[ ( 81. 79 ±21. 06) U/L比( 41. 98 ±11. 64) U/L, P lt;0. 05] 。结论 慢性呼吸衰竭COPD 患者血氧分压降低, 机体缺氧导致氧自由基清除能力降低, 对血管内皮细胞造成潜在损伤。

    Release date:2016-08-30 11:53 Export PDF Favorites Scan
  • Evaluation of Clinical Data and Quality of Life of Hemophilic Children in Sichuan Province

    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.

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  • 血友病患儿合并先天性心脏病的围手术期护理一例

    Release date:2017-11-24 10:58 Export PDF Favorites Scan
  • The Effects of Sleeping-inclued Hypoxema at Different Time and Level on Pulmonary Emphysema and of Coagulation System Function in Rats with Pulmonary Emphysema

    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.

    Release date:2016-09-14 11:25 Export PDF Favorites Scan
  • A Preliminary Study on Treatment of Joint Bleeding in Children with Severe and Moderate Hemophilia A: Low-dose Short-course Infusion of Recombinant Human Factor Ⅷ Concentration as Secondary Prophylaxis

    【摘要】 目的 探讨重组人凝血因子Ⅷ制剂小剂量短程预防性输注能否有效减少中重度血友病A患儿关节出血问题。 方法 对2008年11月-2009年4月期间就诊的13例年龄3~11岁的中重度血友病A患儿,均在为期2个月内接受重组人凝血因子Ⅷ 2次/周、间隔3 d、每次7.5~10.0 U/kg的静脉预防性输注,记录治疗前2个月与治疗2个月时关节出血次数,以及同一关节反复发生出血的情况。 结果 治疗前关节出血的发生次数为(3.77±2.13)次,治疗后关节出血的发生次数为(0.46±0.87)次,治疗前后比较,差异有统计学意义(Plt;0.01);治疗前靶关节出血的发生率为35.7%,治疗后靶关节出血的发生率为0.0%,治疗前后比较,差异有统计学意义(Plt;0.01)。患儿治疗成本约510~680元/(kg?2个月)。 结论 重组人凝血因子Ⅷ制剂小剂量短疗程预防性输注能有效减少中重度血友病A患儿关节出血次数,同时可有效减少靶关节出血的发生率,从而在一定程度上保护关节的功能。治疗费用相对可接受。【Abstract】 Objective To evaluate the efficacy of low-dose short-course infusion of recombinant human factor Ⅷ concentration in treating joint bleeding in children with severe and moderate hemophilia A. Methods Thirteen children aged 3 to 11 years old with severe or moderate hemophilia A were included in the present study from November 2008 to April 2009. For children in the treatment group, they were treated with low-dose short-course infusion of recombinant human factor Ⅷ concentration with a dose of 7.5-10.0 U/kg twice weekly as secondary prophylaxis for two months. The incidence of joint bleeding 2 months before treatment (control group) and during the 2 months of treatment (treatment group) was observed. Moreover, the incidence of their target joint bleeding was measured in both groups. Results Children in the control group had (3.77±2.13) joint bleedings while children in the treatment group had (0.46±0.87) joint bleedings, which was obviously lower than those in the control group (Plt;0.01). Meanwhile, the incidence of target joint bleeding in the treatment group was 0%, which was obviously lower than that in the control group (35.7%) (Plt;0.01). In the treatment group, the costs of treatment were about RMB 510-680 yuan/kg every 2 months. Conclusions Treatment with low-dose short-course infusion of recombinant human factor Ⅷ concentration can effectively decrease joint bleeding in children with severe and moderate hemophilia A, and can effectively decrease the incidence of target joint bleeding. Therefore, this method may play an important role in protection of the joint function in those patients at an acceptable cost.

    Release date:2016-09-08 09:26 Export PDF Favorites Scan
  • 血友病的治疗进展

    血友病是一组X连锁隐形遗传性出血性疾病,主要表现为自发性出血或外伤、手术后出血,反复关节肌肉出血可以致残,危险部位出血甚至威胁生命。替代治疗是血友病唯一有效的治疗方法,同时需要康复、理疗、护理、心理关怀等综合治疗。治疗理念已从出血后按需治疗发展为预防治疗,未来的发展方向将是基因检测阻断患者出生,长效凝血因子的输注以及基因治疗等,从而使患者彻底摆脱疾病的困扰。

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  • 血友病性关节炎误诊手术分

    报道6例血友病性关节炎因误诊而行手术治疗。误诊原因为病史询问不详,遗漏重要的既往史、家族史等;对血友病的骨关节改变缺乏认识;术前常规化验结果正常疑诊血友病者,应行凝血酶原消耗纠正试验,有条件时,可动态测定血中凝血因子活性。避免术中、术后严重并发症出现,并提出误诊手术后采取的有效措施。

    Release date:2016-09-01 11:14 Export PDF Favorites Scan
  • Pharmacoeconomic studies on coagulation factor Ⅷ for the treatment of hemophilia A: a systematic review

    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.

    Release date:2023-01-16 02:58 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
  • 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
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