Operated hepatocellular carcinoma in two HIV- and HCV-positive hemophilic patients

Some hemophilic patients in Japan suffer from infections with both human immunodeficiency virus (HIV) and hepatitis virus because they received contaminated nonheated blood products. Coinfection with HIV appears to accelerate the course of chronic hepatitis. Although powerful antiviral therapy was introduced as HIV treatment and the prognosis of HIV patients was dramatically improved, the risk of rapid progression of hepatitis and carcinogenesis remains for the patients. Recently, we performed surgery for hepatocellular carcinoma (HCC) in two hemophilic patients with HIV and hepatitis C virus (HCV) coinfection. Case 1 was a 52-years-old man who suffered from liver cirrhosis, hypersplenism, and hyperammonemia due to portosystemic shunt. A recent abdominal computed tomography (CT) scan had revealed a low-density area in segment VI of the liver. Splenectomy and partial resection of the liver were performed. Case 2 was a 66-year-old man who had been diagnosed with chronic hepatitis at age 50, and HIV infection at age 52 years. When his serum alpha-fetoprotein level was increased, CT scan of the liver revealed a mass in segment VIII. Subsegmentectmy of the liver was performed. Although the CD4 value in each patient was lower than 200µl, the operations were safely carried out and no major complication occurred. Because the chance of encountering HCC patients infected with HIV and HCV is increasing in Japan, we should consider the perioperative care of these patients, as well as the protection of medical workers against HIV infection.     

Factor V Leiden and hemophilia

Several inherited prothrombotic risk factors have been identified so far. Among them, the factor V (FV) Leiden mutation causes a reduced ability of activated protein C to inactivate activated FV and is the most frequent genetic predisposing factor for venous thromboembolism. However, the high prevalence of FV Leiden (up to 15%) in the Caucasian population suggests that this mutation might confer an evolutionary survival advantage. Indeed, there is mounting evidence about the role of FV Leiden in modulating the clinical phenotype of some physiological and pathological conditions, including hemophilia. The existing literature on the interaction between FV Leiden and hemophilia-related factor VIII or IX mutations is analyzed in this review focusing on the clinical effects and possible pathogenic mechanisms. In summary, current evidence suggests that this prothrombotic mutation may compensate for the low factor VIII or IX levels, resulting in more efficient thrombin generation and ensuing attenuation of clinical symptoms. On the other hand, the association of this prothrombotic mutation with other acquired or inherited thrombophilic factors might overcome the congenital bleeding tendency in hemophiliacs, thereby increasing the risk of thrombotic complications.     

A study of reported factor VIII use around the world

Summary.  The effect of replacement therapy has significantly improved the morbidity and mortality of people with haemophilia A in high income countries, a recent socio-economic development as the availability of safe concentrates has been matched by a willingness for their provision through reimbursement. In the developing world, however, this state has not been achieved, primarily because of the low visibility of haemophilia coupled with its expense, leading to inadequate treatment with its sequelae of severe pain, joint deformities, arthropathy, disabilities, and even death in childhood or early adult life. The objective of this paper was to study the reported factor VIII (FVIII) use on a country-by-country basis. Data on the reported FVIII use for 104 countries were obtained from the Marketing Research Bureau, Inc. and the World Federation of Hemophilia. The results show that FVIII use varies considerably among countries, even among the wealthiest of countries. The use of FVIII concentrate increases as economic capacity increases; in addition, consumption of FVIII has been increasing at a greater rate in high income countries. Given these trends, there probably will be a global increase in FVIII concentrates usage. Such information is critical for national healthcare agencies to determine realistic budget priorities in planning for an increased allocation of resources required to improve the treatment of patients with haemophilia A. This information is also important for pharmaceutical manufacturers to adequately plan for increased production of FVIII concentrates.     

Utilization Patterns of Coagulation Factor Consumption for Patients with Hemophilia

Hemophilia is a serious rare disease that requires continuous management and treatment for which the medicine is costly at the annual average of 100 million KRW for an individual. The aim of this study was to investigate trends in the utilization of coagulation factor (CF) used for hemophilia treatment using the National Health Insurance database from 2010 to 2013 in Korea and compare the utilization of CF with other countries. The consumption of CF per capita (IU) in Korea was not more than other countries with similar income to Korea. However, CF usage per patient IU was higher because the prevalence rate of hemophilia in Korea was lower than in other countries while the number of serious patients was much more. Therefore, it is difficult to say that the consumption of hemophilia medicine in Korea is higher than that in other countries. The consumption and cost of hemophilia medicine in Korea is likely to increase due to the increased utilization of expensive bypassing agents and the widespread use of prophylaxis for severe hemophilia. Even during the research period, it increased slightly and other countries show a similar trend. Thus, hemophilia patient management should accompany active monitoring on the health and cost outcomes of pharmaceutical treatment in the future. This study is expected to contribute to further insight into drug policies for other countries that face similar challenges with high price pharmaceuticals.     

High-purity factor VIII concentrates produced without using monoclonal antibodies

Summary Manufacturers are attempting to increase the purity of FVIII concentrates. A strategy pursued by some is that of including a purification step (gel filtration, ion-exchange or affinity chromatography) that yields concentrates with an intermediate or final specific activity of 35 to 250 IU FVIII/mg of protein. The specific activity of the final product may be lower because serum albumin is added to some concentrates to stabilize FVIII. In hemophiliacs treated with these concentrates, FVIII recovery and half-life are at least as good as those for less pure concentrates. In patients with von Willebrand disease, these concentrates increase plasma levels of FVIII, but their capacity to normalize the bleeding time is not well established. The hypothesis that their reduced alloantigen load might slow the progression of human immunodeficiency virus (HIV) infection is still not validated, but a few prospective studies are now attempting to address this issue. All the concentrates undergo virucidal procedures based on pasteurization or treatment with solvent/detergent. It is well established that these virucidal methods and donor screening avoid HIV transmission. A recent large study has shown that a pasteurized concentrate carries a low risk of transmitting viral hepatitis. The assessment of safety from hepatitis of concentrates treated with solvent/detergent is based on favorable preliminary results.     

Current role for radioisotope synovectomy

Radioisotope synovectomy has been extensively used to treat patients with chronic inflammatory joint disease but has moved to a less prominent position since the introduction of new and highly effective drugs. Remaining indications are refractory synovitis, pigmented villonodular synovitis as an adjunct to surgery, and hemophilic arthropathy. The three main radioisotopes used are yttrium-90, rhenium-186, and erbium-189. Radioisotope synovectomy should be performed only by highly experienced professionals, to minimize the risk of injection-related complications. The available safety data, in particular regarding the risk of malignancy, are reassuring. The efficacy of yttrium-90 in chronic inflammatory joint disease remains controversial.     

北京市成年血友病97例口腔保健知识知晓与行为的调查

目的了解成年血友病患者口腔保健知识知晓与行为现状,为进一步开展血友病患者口腔保健教育提供依据。方法抽取于北京市血友病管理中心登记并自愿参加本次调查的全部成年血友病患者进行自设问卷调查。结果问卷调查北京市成人血友病患者97例,回收问卷97例,患者中位年龄36岁(18,68),患者整体受教育水平较低,大专及大专以上学历患者比例仅为31.96%。受调患者口腔保健知识整体知晓率为67.19%,口腔保健行为的整体正确率为41%。结论我国血友病患者口腔保健知识知晓和行为均较差,血友病患者还没有全面重视口腔保健,急需整体规范的健康指导。     

踝关节置换治疗血友病性关节炎的短期 临床疗效观察

目的：评估血友病性踝关节炎行关节置换治疗的短期疗效,讨论关节置换对患者生活质量及关节功能改善的主要方面及相关原因,积累更多的临床经验。方法：自2004年7月至2007年7月由门诊收治确诊为甲型血友病性踝关节炎患者6例8踝,均为男性,年龄23—57岁,平均41．2岁,予踝关节置换,以Kofoed和AOFAS系统评价疗效,分别于术前及术后6、12个月进行评分比较。结果：对于Kofoed评价系统,关节置换能显著改善术前、术后在疼痛、功能、活动度及总分方面的评分（P〈0．05）,除疼痛项外,其他3项又存在术后12个月和术后6个月评分的差异（P〈0．05）：在功能活动度的各项评分细类中,手术后均较术前有显著改变（P〈0．05）,而背伸功能在术后12个月的改善程度较术后6个月更加显著（R0．05）。对于AOFAS足一后踝评分系统,总分、疼痛、功能和力线方面的评分均存在手术前后的差异（P〈0．05）,但术后6个月和12个月的随访结果显示疼痛和力线没有显著改变（P〉0．05）,而总分和功能评分存在差异（P〈0．05）;在关节功能的各细类评分中,手术前后对比均有显著改善（P〈0．05）,而行走距离和屈伸活动存在6个月和12个月的随访差异（P〈0．05）。两个评分系统均显示关节置换术前后显著的疗效差异（P〈0．05）,而术后6个月及12个月随访结果提示其稳定性（P〉0．05）。结论：短期观察,关节置换术可以显著缓解血友病性踝关节炎患者的临床症状,改善关节功能,随着适应性的提高和下肢肌力的进一步改善,患者的关节功能将平稳而长久地增强,体现出良好的治疗效果。