Multiple congenital coagulopathies co-expressed with Von Willebrand''s disease: the experience of Hemophilia Region III Treatment Centers over 25 years and review of the literature

The haematologist medical directors of the Hemophilia Region III Treatment Centers in the mid-Atlantic Region III of the United States identified individuals in their databases diagnosed with additional congenital coagulopathies co-expressing with Von Willebrand's disease (VWD) and its variants. Sixteen individuals from 14 unrelated families, originating from five institutions, had been evaluated comprehensively. They represented approximately 1.5% of the calculated VWD population (n=986) registered in region III at the end of year 2002. Eleven of the 16 (68.75%) had type 1 VWD; two (12.5%) had type 2A; two (12.5%) had type 2 Normandy (2N) and one (6.25%) had coexisting type 2B and type 2N VWD. The accompanying coagulopathies consisted of severe or moderate factor VIII (FVIII) deficiency (haemophilia A) in six (37.5%); severe or moderate factor IX deficiency (haemophilia B) in three (18.75%); severe or moderate factor XI deficiency in two (12.5%); severe factor VII deficiency in two (12.5%); combined factor XI and XII deficiencies in one (6.25%); qualitative platelet abnormality in one (6.25%); and one had combined type 2B and type 2N VWD (6.25%). The reversal of overt bleeding often required multiple therapeutic modalities, mandated by the specific combination of coagulation disorders. This included administration of specific purified clotting factor concentrates, cryoprecipitate, fresh frozen plasma, 1,8 deamino-d-arginine vasopressin, antifibrinolytic agents, platelet transfusions, etc. In summary, combined inherited coagulopathies coexisting with VWD are uncommon and frequently are phenotypically divergent from VWD. Successful therapy required an individualized approach, employing multiple replacement strategies, based on the laboratory definition of the specific additional coagulation defect.     

Prediction of Long‐Term Outcomes of Catheter Ablation of Persistent Atrial Fibrillation by Parameters of Preablation DC Cardioversion

Prediction of Long‐Term Outcomes of Catheter Ablation of Persistent Atrial Fibrillation. Aim: It has been demonstrated that atrial fibrillation (AF) frequently recurred after cardioversion (CV) using direct current (DC) or radiofrequency catheter ablation (RFCA) in patients with persistent (PeAF) or longstanding persistent AF (LPAF). We hypothesized that the atrial substrate impeding successful CV would also produce difficulty in catheter ablation, and therefore, the outcomes of RFCA for PeAF and LPAF could be predicted by the parameters determined at the time of DC CV. Method: From 2006 to 2009, 94 patients with PeAF and LPAF who had undergone elective DC CV before RFCA were studied. The parameters associated with DC CV, including number of shocks, cumulative energy adjusted, highest energy adjusted, with or without intravenous amiodarone use, and other clinical parameters were assessed. Result: Thirty‐two out of the 94 patients (34%) experienced AF recurrence during the follow‐up of 19.8 ± 12.3 months after RFCA. The average time to recurrence of AF after RFCA was 9.2 ± 3.2 months. Of the 62 patients, 29 patients (31%) remained sinus rhythm (SR) without antiarrhythmic drug (AAD). The patients who maintained SR had smaller body mass index (BMI) (P = 0.048), shorter duration of AF (P = 0.012), and lower prevalence of diabetes mellitus (P = 0.023) compared with patients in whom AF recurred. Total number of shocks, total energy, and highest shock energy during CV were lower (P < 0.001, P = 0.002, P = 0.048, respectively) in patients with SR during the follow‐up. The outcome in patients who used amiodarone IV prior to CV, however, was not different from that in those who did not use amiodarone IV. Conclusion: DC energy parameters for successful CV before RFCA were useful to predict the long‐term outcome after RFCA in patients with PeAF and LPAF. The presence of the atrial substrate making DC CV difficult might reflect atrial substrate that subsequently related to the recurrence of AF after RFCA in chronic AF. These DC energy parameters may be related to the chronicity or electroanatomical remodeling of AF. (J Cardiovasc Electrophysiol, Vol. 23, pp. 1165–1170, November 2012)     

Recombinant immunoblot assay reaction patterns and hepatitis C virus RNA in blood donors and non-A, non-B hepatitis patients

To establish the value of the second-generation recombinant immunoblot assay (RIBA-2) and cDNA polymerase chain reaction (cDNA PCR) for confirmation of hepatitis C virus (HCV) infection, anti-HCV reaction patterns and the presence of HCV RNA were examined in 610 blood donors and 255 non-A, non-B hepatitis patients who were positive or indeterminate in RIBA-2. Of RIBA-2-positive donors (n = 191) and patients (n = 224), 75.4 and 89.7 percent, respectively, were HCV RNA positive. The most frequently observed anti-HCV recognition patterns in HCV RNA-positive donors and patients were c22, c33c, and c100 and/or 5- 1-1 (67.3%, 57.7%) and c22, c33c (24.8%, 29.3%). Among subjects with a RIBA-2-indeterminate result, HCV RNA was detected more often in patients (n = 31) than in donors (n = 419): 67.7 and 2.1 percent, respectively. In viremic persons with single-band reactivity in RIBA-2, this reactivity was always directed against either c22 or c33c. HCV RNA was detected by cDNA PCR in none of 162 persons with only anti-c100 and/or anti-5-1-1 reactivity. Therefore, RIBA-2 reactivity against c100 in combination with 5-1-1 should not be considered positive but indeterminate. In RIBA-2-indeterminate persons, HCV RNA detection is necessary for reliable confirmation of HCV infection.     

单用雷贝拉唑与法莫替丁联合莫沙必利治疗胃食管反流病的临床效果研究

目的：探讨单用雷贝拉唑与法莫替丁联合莫沙必利治疗胃食管反流病的临床效果。方法选取2008年3月～2011年7月本院收治的胃食管反流病患者120例,随机分为治疗组和对照组各60例,治疗组应用雷贝拉唑进行治疗,1～4周口服雷贝拉唑20 mg,1次/d,5～12周改为隔日一次维持治疗;对照组应用法莫替丁进行治疗,1～4周用法莫替丁20 mg,2次/d,加莫沙必利5 mg,3次/d口服,5～12周维持用药则单用法莫替丁20 mg,2次/d。治疗第1、4、8、12周返院就诊,了解患者症状好转程度,第12周对原有食管炎者进行内镜检查以了解食管炎治愈情况。结果治疗组临床症状缓解率及食管炎治愈率明显高于对照组,差异有统计学意义（P＜0.05）。结论与法莫替丁加莫沙必利相比,雷贝拉唑治疗胃食管反流病具有临床症状缓解快速、完全缓解率高的优势。     

国内康复医学相关资源网站综合评估与分析

目的：探讨网络技术在康复医学资源共享领域的运用。方法：收集近五年来国内有代表性的康复医学资源网站或网络技术，按照各自属性分为学术组织类、专职机构类、特色康复医疗专业类、个体类四组，从一般普通网站共有的特征以及与康复医学资源共享相关的指标两方面进行了分析和比较。结果：所有网站在一般的总体性能指标上差异并不明显，但在与资源共享相关的指标评测中，各类网站总体差异很大。结论：网络技术在康复医学资源共享领域的应用正处于发展阶段，诸多方面尚须深人探究，但将有限的资源最大化共享对推动康复医学事业的整体发展将起到难以估量的作用。     

The inhibition of Src kinase suppresses the production of matrix metalloproteinases in from synovial fibroblasts and inhibits MAPK and STATs pathways

Background/aimThe purpose of this study was to investigate the antiarthritic potentials of the inhibition of Src kinase in vivo and in vitro settings. Materials and methodsArthritis was induced by intradermal injection of chicken type II collagen combined with incomplete Freund’s adjuvant (collagen induced arthritis [CIA] model) in Wistar albino rats. One day after the onset of arthritis, dasatinib, a potent Src kinase inhibitor, (5 mg/kg/day) was given via oral gavage. Tissue Src, Fyn, MAPK and STAT mRNA expressions were determined by real-time polymerase chain reaction. On the other hand, fibroblast like synoviocytes (FLSs) were harvested patients with rheumatoid arthritis (RA) undergoing surgical knee joint replacement. FLSs were stimulated with cytokines and dasatinib was added in different concentrations. MMP –1, –3, and –13 levels in FLSs culture were determined by ELISA.ResultsThe tissue mRNA expressions of Src, Fyn, MAPK and STATs were increased in the arthritis CIA group compared to the control group. Their mRNA expressions in the CIA + dasatinib group were decreased and similar in the control group. In in vitro setting, MMP –1, –3, and –13 expressions from FLSs induced by IL-1β and TNF-α were increased, while dasatinib suppressed their productions from FLSs.ConclusionThe present study shows that the inhibition of Src kinase has antiarthritic potentials in both in vivo and in vitro settings. Src kinase inhibition may be candidate to further research in human RA.     

徐光星教授依主次病机从“燥”论治肺癌经验

[目的] 总结徐光星教授依主次病机从燥热伤肺、气阴不足论治肺癌的经验。[方法] 通过跟诊学习，整理相关医案，查阅历代中医文献及国医大师何任教授辨治肿瘤的相关论述与治疗肺癌验案，从病因病机、辨治思路两方面，分析徐教授依主次病机，从燥热伤肺、气阴不足辨治肺癌的临床经验，并佐以两则医案验证。[结果] 徐教授认为，肺癌的形成是由于正气内虚、邪毒侵袭、燥热伤肺、痰瘀互结所致，提出以燥热伤肺、气阴不足为主次病机治疗肺癌，并传承何任教授从益气养阴治疗中晚期肺癌的基本大法，治疗或以燥热伤肺、气阴不足为主病机，清热润燥、益气养阴为主法；或以燥热伤肺、气阴不足为次病机，化痰散结、活血化瘀、清热解毒为主法，兼以清热润燥、益气养阴。所附医案一辨为肺癌后期，以燥热伤肺、气阴不足为主病机，治拟益气养阴、清热润燥、化痰散结；医案二辨为肺癌中期，以痰热壅肺为主病机，燥热伤肺、气阴不足为次病机，治拟清热化痰、养阴润燥，均药治稳好。[结论] 徐光星教授以燥热伤肺、气阴不足为主次病机论治肺癌，疗效确切，临床实用，值得学习与推广。