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Clinicohematological study of thrombocytosis in children. 儿童血小板增多症临床血液学研究。
Pub Date : 2014-01-29 eCollection Date: 2014-01-01 DOI: 10.1155/2014/389257
Nathiya Subramaniam, Suneel Mundkur, Pushpa Kini, Nalini Bhaskaranand, Shrikiran Aroor

Introduction. Primary thrombocytosis is very rare in children; reactive thrombocytosis is frequently observed in children with infections, anemia, and many other causes. Aims and Objectives. To identify the etiology of thrombocytosis in children and to analyze platelet indices (MPV, PDW, and PCT) in children with thrombocytosis. Study Design. A prospective observational study. Material and Methods. A total of 1000 patients with thrombocytosis (platelet > 400 × 10(9)/L) were studied over a period of 2 years. Platelet distribution width (PDW), mean platelet volume (MPV), and plateletcrit (PCT) were noted. Results. Of 1000 patients, 99.8% had secondary thrombocytosis and only two children had primary thrombocytosis (chronic myeloid leukemia and acute myelogenous leukemia, M7). The majority of the children belonged to the age group of 1month to 2 years (39.7%) and male to female ratio was 1.6 : 1. Infection with anemia (48.3%) was the most common cause of secondary thrombocytosis followed by iron deficiency alone (17.2%) and infection alone (16.2%). Respiratory infection (28.3%) was the predominant infectious cause observed. Thrombocytosis was commonly associated with IDA among all causes of anemia and severity of thrombocytosis increased with severity of anemia (P = 0.021). With increasing platelet count, there was a decrease in MPV (<0.001). Platelet count and mean PDW among children with infection and anemia were significantly higher than those among children with infection alone and anemia alone. None were observed to have thromboembolic manifestations. Conclusions. Primary thrombocytosis is extremely rare in children than secondary thrombocytosis. Infections in association with anemia are most commonly associated with reactive thrombocytosis and severity of thrombocytosis increases with severity of anemia.

介绍。原发性血小板增多症在儿童中非常罕见;反应性血小板增多症是经常观察到的儿童感染,贫血,和许多其他原因。目的和目标。目的:探讨儿童血小板增多症的病因,分析血小板指数(MPV、PDW和PCT)。研究设计。一项前瞻性观察研究。材料和方法。在2年的时间里,共研究了1000例血小板增多(血小板> 400 × 10(9)/L)患者。记录血小板分布宽度(PDW)、平均血小板体积(MPV)和血小板电积(PCT)。结果。在1000例患者中,99.8%有继发性血小板增多症,只有2名儿童有原发性血小板增多症(慢性髓性白血病和急性髓性白血病,M7)。以1月龄~ 2岁的儿童居多(39.7%),男女比例为1.6:1。感染伴贫血(48.3%)是继发性血小板增多的最常见原因,其次是缺铁(17.2%)和感染(16.2%)。呼吸道感染为主要感染原因(28.3%)。在所有贫血原因中,血小板增多通常与IDA相关,血小板增多的严重程度随着贫血的严重程度而增加(P = 0.021)。随着血小板计数的增加,MPV (
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引用次数: 24
Two cases and review of the literature: primary percutaneous angiography and antiplatelet management in patients with immune thrombocytopenic purpura. 免疫性血小板减少性紫癜的经皮血管造影及抗血小板治疗两例及文献复习。
Pub Date : 2013-12-29 DOI: 10.1155/2013/174659
Estelle Torbey, Harout Yacoub, Donald McCord, James Lafferty

We report two cases of immune thrombocytopenic purpura (ITP) associated with acute coronary artery syndrome highlighting the interventions done in every case along with the medications used during intervention and as outpatient. The first case is that of a woman with ITP exacerbation while on dual antiplatelet therapy and the second case is that of a male presenting with non-ST elevation myocardial infarction (NSTEMI) while in a thrombocytopenic crisis. In both cases antiplatelet therapy was held and thrombopoietic therapy was initiated before resuming full anticoagulation and coronary intervention. Given the paucity of data on ITP and antiplatelets treatment in the setting of acute coronary syndrome, no strict recommendations can be proposed, but antiplatelets appear to be safe acutely and in the long term in this category of patients as long as few measures are undertaken to minimize the risks of bleeding and thrombosis.

我们报告两例与急性冠状动脉综合征相关的免疫性血小板减少性紫癜(ITP),强调在每个病例中进行的干预以及在干预期间和门诊使用的药物。第一个病例是在接受双重抗血小板治疗时ITP加重的女性,第二个病例是在血小板减少危象中表现为非st段抬高型心肌梗死(NSTEMI)的男性。在恢复全面抗凝和冠状动脉介入治疗之前,这两例患者都接受了抗血小板治疗和血小板生成治疗。由于缺乏关于急性冠脉综合征中ITP和抗血小板治疗的数据,因此不能提出严格的建议,但只要采取少量措施将出血和血栓的风险降至最低,抗血小板似乎在这类患者中是急性和长期安全的。
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引用次数: 17
Addition of multimodal therapy to standard management of steady state sickle cell disease. 在稳定状态镰状细胞病的标准管理中增加多模式治疗。
Pub Date : 2013-12-09 eCollection Date: 2013-01-01 DOI: 10.1155/2013/236374
Iheanyi Okpala, Osita Ezenwosu, Anthony Ikefuna, Augustine Duru, Barth Chukwu, Anazoeze Madu, Theresa Nwagha, Sunday Ocheni, Obike Ibegbulam, Ifeoma Emodi, Uche Anike, Charles Nonyelu, Chukwudi Anigbo, Kingsley Agu, Ifeoma Ajuba, Awele Chukwura, Ogechukwu Ugwu, Uche Ololo

Most people on folic acid to boost erythropoiesis and prophylactic antimicrobials, the standard management of steady state sickle cell disease (SCD), have unacceptable numbers of crises. The objective of this study was to evaluate the effects of adding multimodal therapy with potassium thiocyanate and omega-3 fatty acids to the standard management of steady state SCD. Pre- and post-treatment numbers of crises and other disease indices were compared in 16 HbSS individuals on folic acid and paludrine after 12 months of adding eicosapentaenoic acid 15 mg/kg/day, docosahexaenoic acid 10 mg/kg/day, and potassium thiocyanate 1-2 mL/day, each milliliter of which contained 250 mg of thiocyanate and 100 micrograms of iodine to prevent hypothyroidism: a possible side-effect due to competitive inhibition of the transport of iodide into the thyroid gland by thiocyanate. Median number of crises reduced from 3/yr to 1/yr (P < 0.0001). There was no evidence of impaired thyroid function. Plasma level of tri-iodothyronine improved (P < 0.0001). Steady state full blood count and bilirubin level did not change significantly. The findings suggest that addition of potassium thiocyanate and eicosapentaenoic and docosahexaenoic acids to standard management of steady state SCD reduces the number of crises. This observation needs to be evaluated in larger studies.

大多数人服用叶酸促进红细胞生成和预防性抗微生物药物,这是稳定状态镰状细胞病(SCD)的标准管理,有不可接受的危机数量。本研究的目的是评估在稳定状态SCD的标准治疗中加入硫氰酸钾和omega-3脂肪酸的多模式治疗的效果。预处理和后处理的危机和其他疾病在16个哈佛商学院指数比较个人叶酸和白乐君12个月后添加二十碳五烯酸15毫克/公斤/天,二十二碳六烯酸10毫克/公斤/天,和硫氰酸钾1 - 2毫升/天,每毫升含有硫氰酸250毫克和100毫克碘预防甲状腺功能减退:一个可能的副作用,由于运输的竞争性抑制碘化硫氰酸的甲状腺。危机中位数从3次/年减少到1次/年(P < 0.0001)。没有证据表明甲状腺功能受损。血浆三碘甲状腺原氨酸水平提高(P < 0.0001)。稳态全血细胞计数和胆红素水平无明显变化。研究结果表明,在稳定状态SCD的标准管理中添加硫氰酸钾、二十碳五烯酸和二十二碳六烯酸可以减少危象的发生。这一观察结果需要在更大规模的研究中进行评估。
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引用次数: 6
Assessment of regulatory T cells in childhood immune thrombocytopenic purpura. 儿童免疫性血小板减少性紫癜调节性T细胞的评估。
Pub Date : 2013-11-05 eCollection Date: 2013-01-01 DOI: 10.1155/2013/143687
Karina L M Mazzucco, Lauro M Junior, Natália E Lemos, Andréa Wieck, Annelise Pezzi, Alvaro M Laureano, Bruna Amorin, Vanessa Valim, Lucia Silla, Liane E Daudt, Paulo J C Marostica

This study had the objective to assess the frequency of Tregs in children newly diagnosed with ITP and ascertain whether an association exists between Tregs and platelet counts, by means of a comparison with healthy controls. This case-control study included 19 patients newly diagnosed with ITP-whose blood samples were collected at four points in time: before any therapy and 1, 3, and 6 months after diagnosis-and 19 healthy controls. Tregs (CD4(+) CD25(+)Foxp3 T cells) were evaluated by flow cytometry. There was a statistically significant difference in platelet count between the case and control groups. There were no significant differences in Treg counts between cases and controls at any point during the course of the study and no difference in Treg counts between the chronic and nonchronic groups and no significant correlation between Tregs and platelet counts in the case and control groups. The findings of this study did not show any statistically significant correlation between Tregs and number of platelets in the case and control groups. Treg cells did not play a role in the regulation of autoimmunity in children with ITP.

本研究的目的是通过与健康对照组的比较,评估新诊断为ITP的儿童中Tregs的频率,并确定Tregs与血小板计数之间是否存在关联。这项病例对照研究包括19名新诊断为itp的患者,他们的血液样本在治疗前和诊断后1、3和6个月的四个时间点采集,以及19名健康对照。流式细胞术检测Tregs (CD4(+) CD25(+)Foxp3 T细胞)。病例组与对照组血小板计数差异有统计学意义。在研究过程中,病例和对照组之间的Treg计数在任何时候都没有显著差异,慢性组和非慢性组之间Treg计数没有差异,病例组和对照组之间Treg和血小板计数没有显著相关性。本研究结果未显示病例组和对照组Tregs和血小板数量之间有统计学意义的相关性。Treg细胞在ITP患儿自身免疫调节中不发挥作用。
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引用次数: 7
Survival and Prognostic Factors for AIDS and Non-AIDS Patients with Non-Hodgkin's Lymphoma in Bahia, Brazil: A Retrospective Cohort Study. 巴西巴伊亚艾滋病和非艾滋病非霍奇金淋巴瘤患者的生存和预后因素:一项回顾性队列研究。
Pub Date : 2013-10-29 eCollection Date: 2013-01-01 DOI: 10.1155/2013/904201
Estela Luz, Marinho Marques, Ivana Luz, Cristiani Stelitano, Eduardo Netto, Iguaracyra Araújo, Carlos Brites

Despite the benefits of HAART, HIV-infected patients are increasingly affected by different malignancies. We compared a 5-year-period survival time and prognostic factors for HIV-1-infected individuals diagnosed with non-Hodgkin lymphomas (NHL) in a nested case-control study, with non-HIV-infected individuals in Salvador, Brazil. Survival time and prognostic factors were compared to HIV-negative patients. 31 cases (versus 63 controls) had a significantly more advanced NHL at diagnosis and lower mean CD4 count (26 cells/mm(3)) than controls. Mean overall survival (OS) was 35.8 versus 75.4 months, for cases and controls, respectively (P < 0.001), while mean event-free survival time (EFS) was 34.5 months for cases, versus 68.8 for controls (P = 0.002). Higher IPI, increased LDH levels, bone marrow infiltration, lower absolute lymphocyte counts (<1,000 cells/mm(3)), and type B symptoms were associated with a shorter survival time for cases. Although patients without poorer prognostic factors at baseline had an OS comparable to controls, the mean CD4 cell count for cases was similar for patients with favorable and nonfavorable response to therapy. Our findings suggest that HIV-1 infection is significantly associated with a shorter survival time for patients with NHL, independently of other predictive factors and of disease stage.

尽管HAART有好处,但感染艾滋病毒的患者越来越多地受到不同恶性肿瘤的影响。我们在巴西萨尔瓦多的一项巢式病例对照研究中,比较了诊断为非霍奇金淋巴瘤(NHL)的hiv -1感染者与非hiv感染者的5年生存时间和预后因素。比较hiv阴性患者的生存时间和预后因素。31例(对照63例)在诊断时的NHL进展明显更严重,平均CD4计数(26个细胞/毫米(3))低于对照。病例和对照组的平均总生存期(OS)分别为35.8个月和75.4个月(P < 0.001),而病例的平均无事件生存期(EFS)为34.5个月,对照组为68.8个月(P = 0.002)。IPI升高,LDH水平升高,骨髓浸润,淋巴细胞绝对计数(
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引用次数: 8
Interaction of Normal and Sickle Hemoglobins for Sodium Dodecylsulphate and Hydrogen Peroxide at pH 5.0 and 7.2. 十二烷基硫酸钠和过氧化氢在pH 5.0和7.2下正常血红蛋白和镰状血红蛋白的相互作用。
Pub Date : 2013-10-10 eCollection Date: 2013-01-01 DOI: 10.1155/2013/629640
Fortunatus C Ezebuo, Sabinus Oscar O Eze, Colin B Lukong, Ferdinand C Chilaka

Clinical manifestations of malaria primarily result from proliferation of the parasite within the hosts' erythrocytes. The malaria parasite digests hemoglobin within its digestive vacuole through a sequential metabolic process involving multiple proteases. The activities of these proteases could lead to the production of ROS which could lead to the death of the parasites due to the destruction of their membrane. The action of SDS on hemoglobins can be likened to the way malarial proteases destabilizes host hemoglobin. Hence, the study was designed to determine the binding parameters of SDS and H2O2 for normal, sickle trait carrier and sickle hemoglobins at pH 5.0 and 7.2 using UV-VIS Titration Spectrophotometry. Hb-SDS interactions were significantly different at pH 5.0 but were not at pH 7.2. Also, Hb-H2O2 interactions were statistically different at pH 5.0 and 7.2. The interactions suggest that HbA and HbS are easily destabilized than HbAS and that HbAS has more affinity for H2O2. These suggest a production of more ferryl intermediates or hydroxyl radicals. All these interactions may hinder the development of the malaria parasite at the intraerythrocytic stage and could likely account for a significant proportion of the mechanism that favours the resistance to malaria by individuals with HbAS.

疟疾的临床表现主要是由疟原虫在宿主红细胞内增殖引起的。疟原虫通过一系列涉及多种蛋白酶的连续代谢过程在其消化液泡内消化血红蛋白。这些蛋白酶的活性可导致活性氧的产生,活性氧可导致寄生虫因其膜被破坏而死亡。SDS对血红蛋白的作用可以比作疟疾蛋白酶破坏宿主血红蛋白稳定的方式。因此,本研究采用UV-VIS滴定分光光度法测定正常、镰状性状载体和镰状血红蛋白在pH 5.0和7.2条件下SDS与H2O2的结合参数。pH值为5.0时Hb-SDS相互作用差异显著,pH值为7.2时则无差异。pH 5.0和7.2时Hb-H2O2相互作用差异有统计学意义。相互作用表明HbA和HbS比HbAS更容易失稳,HbAS对H2O2更有亲和力。这表明产生了更多的铁基中间体或羟基自由基。所有这些相互作用都可能在红细胞内阶段阻碍疟疾寄生虫的发育,并可能在HbAS个体对疟疾产生抗性的机制中占很大比例。
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引用次数: 1
The modern primitives: applying new technological approaches to explore the biology of the earliest red blood cells. 现代原始人:应用新技术方法探索最早的红细胞生物学。
Pub Date : 2013-10-03 DOI: 10.1155/2013/568928
Stuart T Fraser

One of the most critical stages in mammalian embryogenesis is the independent production of the embryo's own circulating, functional red blood cells. Correspondingly, erythrocytes are the first cell type to become functionally mature during embryogenesis. Failure to achieve this invariably leads to in utero lethality. The recent application of technologies such as transcriptome analysis, flow cytometry, mutant embryo analysis, and transgenic fluorescent gene expression reporter systems has shed new light on the distinct erythroid lineages that arise early in development. Here, I will describe the similarities and differences between the distinct erythroid populations that must form for the embryo to survive. While much of the focus of this review will be the poorly understood primitive erythroid lineage, a discussion of other erythroid and hematopoietic lineages, as well as the cell types making up the different niches that give rise to these lineages, is essential for presenting an appropriate developmental context of these cells.

哺乳动物胚胎发生过程中最关键的阶段之一是胚胎独立产生自身循环的功能性红细胞。相应地,红细胞是胚胎发育过程中第一个功能成熟的细胞类型。如果不能做到这一点,必然会导致子宫内死亡。最近,转录组分析、流式细胞术、突变胚胎分析和转基因荧光基因表达报告系统等技术的应用,使人们对发育早期出现的不同红细胞系有了新的认识。在这里,我将介绍胚胎存活所必须形成的不同红细胞群之间的异同。虽然这篇综述的重点是人们对原始红细胞系了解甚少,但讨论其他红细胞系和造血系以及构成产生这些红细胞系的不同龛位的细胞类型,对于介绍这些细胞的适当发育背景至关重要。
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引用次数: 0
Portal hypertension and myeloproliferative neoplasms: a relationship revealed. 门脉高压与骨髓增生性肿瘤的关系
Pub Date : 2013-09-16 eCollection Date: 2013-01-01 DOI: 10.1155/2013/673781
Ahmet Burak Toros, Serkan Gokcay, Guven Cetin, Muhlis Cem Ar, Yesim Karagoz, Besir Kesici

Background/Objectives. Patients with myeloproliferative neoplasms have a well-established increased risk of thrombosis. Many trials report identification of an underlying myeloproliferative neoplasm by investigation of the patients developing portal hypertensive esophagus and/or fundus variceal hemorrhage in the absence of any known etiology. This trial was designed to investigate the association between myeloproliferative neoplasms and portal hypertension and to detect the frequency of portal hypertension development in this subset of patients. Methodology. Twenty-nine patients previously diagnosed with polycythemia vera, essential thrombocytopenia, and primary myelofibrosis, who were under followup at the hematology outpatient clinic of our hospital, were included in the trial. Results. In our trial, we detected portal hypertension in 13.8% of the patients (n = 4), as a finding that was similar to those obtained in other studies performed to date. Conclusions. Considering the fact that diagnosis of myeloproliferative neoplasms usually takes a long time, treatment should be started (while, on the other hand, assessing the investigational and therapeutical choices for the complications) right after the bone marrow biopsy or cytogenetic studies required for establishing the final diagnosis have been performed.

背景/目标。骨髓增生性肿瘤患者血栓形成的风险明显增加。许多试验报告在没有任何已知病因的情况下,通过调查发生门脉高压食管和/或眼底静脉曲张出血的患者,发现潜在的骨髓增生性肿瘤。该试验旨在研究骨髓增生性肿瘤与门静脉高压症之间的关系,并检测这部分患者门静脉高压症的发生频率。方法。29例既往诊断为真性红细胞增多症、原发性血小板减少症和原发性骨髓纤维化的患者在我院血液科门诊接受随访,纳入试验。结果。在我们的试验中,我们在13.8%的患者(n = 4)中检测到门静脉高压症,这一发现与迄今为止进行的其他研究中获得的结果相似。结论。考虑到骨髓增殖性肿瘤的诊断通常需要很长时间,治疗应该在进行最终诊断所需的骨髓活检或细胞遗传学研究后立即开始(同时,另一方面,评估并发症的研究和治疗选择)。
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引用次数: 15
Waldenström macroglobulinemia: clinical and immunological aspects, natural history, cell of origin, and emerging mouse models. Waldenström巨球蛋白血症:临床和免疫学方面,自然史,细胞起源,和新兴的小鼠模型。
Pub Date : 2013-09-09 DOI: 10.1155/2013/815325
Siegfried Janz

Waldenström macroglobulinemia (WM) is a rare and currently incurable neoplasm of IgM-expressing B-lymphocytes that is characterized by the occurrence of a monoclonal IgM (mIgM) paraprotein in blood serum and the infiltration of the hematopoietic bone marrow with malignant lymphoplasmacytic cells. The symptoms of patients with WM can be attributed to the extent and tissue sites of tumor cell infiltration and the magnitude and immunological specificity of the paraprotein. WM presents fascinating clues on neoplastic B-cell development, including the recent discovery of a specific gain-of-function mutation in the MYD88 adapter protein. This not only provides an intriguing link to new findings that natural effector IgM(+)IgD(+) memory B-cells are dependent on MYD88 signaling, but also supports the hypothesis that WM derives from primitive, innate-like B-cells, such as marginal zone and B1 B-cells. Following a brief review of the clinical aspects and natural history of WM, this review discusses the thorny issue of WM's cell of origin in greater depth. Also included are emerging, genetically engineered mouse models of human WM that may enhance our understanding of the biologic and genetic underpinnings of the disease and facilitate the design and testing of new approaches to treat and prevent WM more effectively.

Waldenström巨球蛋白血症(macroglobulinemia, WM)是一种罕见且目前无法治愈的表达IgM的b淋巴细胞肿瘤,其特征是血清中出现单克隆IgM (mIgM)副蛋白,并伴有恶性淋巴浆细胞浸润造血骨髓。WM患者的症状可归因于肿瘤细胞浸润的程度和组织部位以及副蛋白的大小和免疫特异性。WM为肿瘤b细胞的发育提供了引人入胜的线索,包括最近发现的MYD88适配器蛋白中特定的功能获得突变。这不仅为新发现提供了一个有趣的联系,即天然效应IgM(+)IgD(+)记忆b细胞依赖于MYD88信号,而且还支持了WM来自原始的,先天样b细胞,如边缘区和B1 b细胞的假设。在简要回顾WM的临床方面和自然史之后,本文将更深入地讨论WM细胞起源的棘手问题。还包括正在出现的人类WM基因工程小鼠模型,它可以增强我们对该疾病的生物学和遗传基础的理解,并促进设计和测试更有效地治疗和预防WM的新方法。
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引用次数: 24
HBsAg Positive Patient Characteristics in Hospital and Blood Donation Camps. 医院及献血营HBsAg阳性患者特征分析
Pub Date : 2013-09-04 eCollection Date: 2013-01-01 DOI: 10.1155/2013/675191
Deepti Sachan, Joy Varghese, Jensingh Joseph, Vijaya Srinivasan, Venkataraman Jayanthi, Mohamed Rela

Background. Prevention of the residual risk of transfusion transmitted hepatitis B virus infection (HBV) is mostly dependant on serological screening of blood donors for HBsAg and antibody to hepatitis B core antigen (anti-HBc Ab). This study aimed to study the prevalence of HBsAg and anti-HBc Ab and to compare the profile of blood donors attending a blood donation camp and people attending a hospital based camp. Methods. In the blood donor camp, all the blood units were screened for HBV, (HBsAg and anti-HBc), and in the hospital based camp, screening was done for HBsAg alone. Baseline demographic characteristics were noted. Results. The number of blood bank donors was 363 (47.5%) and hospital camp attendees was 402 (52.5%). Prevalence of HBsAg positivity was similar in both the groups at 1.7% and 1.9%, respectively. Anti-HBc Ab positivity (Total) was 6% among the blood donors; Overall prevalence of HBV infection in this group was 3.2%. Conclusion. Policy for checking the collected blood unit by 3 tests for anti-HBc, anti-HBsAb, and HBsAg should be reconsidered to possibly achieve the zero risk goal of transfusion transmitted HBV infection. Blood obtained from a vaccinated donor may give an added protection to the recipient.

背景。输血传播乙型肝炎病毒感染(HBV)残留风险的预防主要依赖于对献血者进行HBsAg和乙型肝炎核心抗原抗体(抗hbc抗体)的血清学筛查。本研究旨在研究HBsAg和anti-HBc Ab的流行情况,并比较参加献血营和参加医院献血营的献血者的情况。方法。在献血者阵营中,所有的血液单位都进行了HBV筛查(HBsAg和anti-HBc),而在医院阵营中,仅进行HBsAg筛查。注意到基线人口统计学特征。结果。血库献血者363人(47.5%),医院露营者402人(52.5%)。两组的HBsAg阳性率相似,分别为1.7%和1.9%。献血者中抗- hbc抗体阳性(总)占6%;该组HBV感染的总体流行率为3.2%。结论。应重新考虑对采血单位进行抗hbc、抗hbsab和HBsAg 3项检测的政策,以可能实现输血传播HBV感染的零风险目标。从接种过疫苗的献血者处获得的血液可以为接受者提供额外的保护。
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引用次数: 7
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