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Investigation of some endogenous antimicrobial peptides in thalassemia 地中海贫血内源性抗菌肽的研究
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-12-31 DOI: 10.4081/THAL.2018.7744
A. M. Efendiyev, G. Azizova, A. Dadashova
The aim of this work was a comparative study of the amount of antimicrobial peptides - human neutrophil peptides - defensins (HNP), hepcidin, bactericidal/permeability-increasing protein (BPI), and endotoxin in β-thalassemia. Blood samples of 135 patients with thalassemia were investigated. All patients were divided into 3 groups. The first group included patients with heterozygous form (n=45). The second group consisted of patients with homozygous form before splenectomy (n=45). The third group included patients with homozygous form after splenectomy (n=45). The age of patients varied from 2 to 18 years. Biochemical [unconjugated and conjugated bilirubin, alkaline phosphatase, hemoglobin, ferritin, aspartate transaminase (AST), alanine transaminase (ALT), mean corpuscular volume (MCV)] and immune (IgA, IgM, IgG, phagocytic activity) parameters were defined. Obtained results suggest that increased levels of endogenous antimicrobial peptides are associated with the development of the infectious process and reflect the dynamics of changes in biochemical parameters and immune status.
本工作的目的是比较β-地中海贫血中抗菌肽-人中性粒细胞肽-防御素(HNP)、铁调素、杀菌/通透性增加蛋白(BPI)和内毒素的含量。对135例地中海贫血患者的血液样本进行了调查。将所有患者分为3组。第一组包括杂合子型患者(n=45)。第二组为脾切除术前纯合型患者(n=45)。第三组包括脾切除术后的纯合型患者(n=45)。患者年龄从2岁到18岁不等。定义了生化[非结合和结合胆红素、碱性磷酸酶、血红蛋白、铁蛋白、天冬氨酸转氨酶(AST)、丙氨酸转氨酶(ALT)、平均红细胞体积(MCV)]和免疫(IgA、IgM、IgG、吞噬活性)参数。所获得的结果表明,内源性抗菌肽水平的增加与感染过程的发展有关,并反映了生化参数和免疫状态的动态变化。
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引用次数: 0
β globin mutations in Turkish, Northern Iraqi and Albanian patients with β thalassemia major 土耳其、伊拉克北部和阿尔巴尼亚重型β地中海贫血患者的β珠蛋白突变
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-06-06 DOI: 10.4081/THAL.2018.7286
V. Hançer, T. Fışgın, Murat Büyükdoğan, C. Bozkurt, S. Lako
The mutation detection of β thalassemia is absolutely necessary for molecular diagnosis, as well as any genetic epidemiological study. The β globin gene has 3 exons and 2 introns, involved in β-thalassemic pathogenesis. The study aim of the study is to characterize the spectrum of β globin gene mutations in 136 Turkish, Northern Iraqi and Albanian pediatric β thalassemia major patients. After genomic DNA extraction from venous blood and amplification of the target DNA regions with PCR, genotyping was achieved by Sanger based DNA sequencing. The IVSI-110 G>A mutation was the most frequent allele in the Turkish and Albanian patients. In Northern Iraqi patients IVSI-1 G>A was is the most frequent. There are two mutations are firstly reported for Albania [c.*111 A>G 3’ UTR (rs63751128) and c.113 G>A (p.Trp38Ter, p.W38*) (rs35887507)] with this study. These findings may be of value for genetic counseling, premarital diagnosis, prenatal diagnosis and prevention programs.
β地中海贫血的突变检测对于分子诊断以及任何遗传流行病学研究都是绝对必要的。β珠蛋白基因有3个外显子和2个内含子,参与β地中海贫血的发病机制。本研究的目的是确定136名土耳其、伊拉克北部和阿尔巴尼亚儿童β地中海贫血主要患者的β珠蛋白基因突变谱。从静脉血中提取基因组DNA并用PCR扩增靶DNA区域后,通过基于Sanger的DNA测序实现基因分型。IVSI-110 G>A突变是土耳其和阿尔巴尼亚患者中最常见的等位基因。在伊拉克北部的患者中,IVSI-1 G>A是最常见的。本研究首次报道阿尔巴尼亚有两种突变[c.*111 A>G 3'UTR(rs63751128)和c.113 G>A(p.Trp38Ter,p.W38*)(rs35887507)]。这些发现可能对基因咨询、婚前诊断、产前诊断和预防计划有价值。
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引用次数: 3
National policies in ensuring access to quality and safety of drugs: A challenge or a prerequisite 确保获得高质量和安全药品的国家政策:挑战还是先决条件
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/THAL.2018.7483
M. Dehshal, M. Namini
Access to the essential medicines is an important challenge in the developing countries. To have access to the quality and affordable medicines, the pharmaceutical decision makers try different strategies. The production of generic and copy medicines is one of the strategies that if adopted based on the recognized standards and norms can be effective in raising the health status in the developing countries. However, the regulation enfeeblement has somewhat impaired the quality of generic and copy medicines and harmed the health life of consumers. Here we aim to reflect over the role of different beneficiaries including international organizations, governments, pharmaceutical companies, and NGOs in ensuring the feasible and sustainable access of citizens to the essential medicines. We also aim to highlight the importance of the patient status in the enhancement of the medical delivery.
获得基本药物是发展中国家面临的一项重要挑战。为了获得高质量和负担得起的药物,制药决策者尝试了不同的策略。仿制药和仿制药的生产是在公认标准和规范的基础上采取的战略之一,可以有效地提高发展中国家的健康状况。然而,监管不力在一定程度上损害了仿制药和仿制药的质量,损害了消费者的健康生活。在这里,我们旨在反思包括国际组织、政府、制药公司和非政府组织在内的不同受益者在确保公民可行和可持续地获得基本药物方面的作用。我们还旨在强调患者状态在加强医疗服务中的重要性。
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引用次数: 0
Optimal blood transfusion therapy in haemoglobinopathies 血红蛋白病的最佳输血治疗
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/thal.2018.7477
J. Porter
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引用次数: 1
Diagnosis of haemoglobinopathies : New scientific advances 血红蛋白病的诊断:新的科学进展
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/THAL.2018.7473
C. Harteveld
The molecular defects underlying haemoglobinopathies are both deletions and point mutations in the alpha- or beta-globin genes or gene-clusters. To detect point mutations causing alpha- or beta-thalassaemia, direct sequencing is the method of choice to detect the widest spectrum of molecular defects. The most established approach in DNA diagnostics to screen for the most common deletion defects causing alpha-thalassaemia or beta-thalassaemia is gap- PCR, Multiplex Ligation-dependent Probe Amplification (MLPA) and Sanger Sequencing technology to detect breakpoint sequences of previously uncharacterized deletions/duplications. We demonstrate the recent advances in the determination of duplications and deletions causing alpha- or beta-thalassemia, using Next Generation Sequencing, array Comparative Genome Hybridization and Target Locus Amplification. We present three cases in which the use of advanced technologies allow the diagnosis of unexpected disease genotypes.
血红蛋白病的分子缺陷是α -或β -珠蛋白基因或基因簇的缺失和点突变。为了检测引起α -或β -地中海贫血的点突变,直接测序是检测最广泛的分子缺陷的方法。在DNA诊断中,筛选导致α -地中海贫血或β -地中海贫血的最常见缺失缺陷的最成熟方法是gap- PCR、多重连接依赖探针扩增(Multiplex lig- dependent Probe Amplification, MLPA)和Sanger测序技术,以检测先前未表征的缺失/重复的断点序列。我们展示了使用下一代测序、阵列比较基因组杂交和靶基因座扩增来确定导致α -或β -地中海贫血的重复和缺失的最新进展。我们提出了三个案例,其中使用先进技术可以诊断出意想不到的疾病基因型。
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引用次数: 1
Patient care: Unmet needs globally 患者护理:全球未满足的需求
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/thal.2018.7485
M. Angastiniotis, A. Eleftheriou
Literature demonstrates that long survival and a good quality of life are achieved where the patients’ needs for holistic care are recognised and the appropriate services are offered. The once fatal diseases of childhood have become chronic conditions of adult life. [8, 9, 10]. TIF’s mission is to promote and assist in the implementation of national programmes for the treatment of thalassaemia and other haemoglobin disorders, wherever the patients may be residing, driven by the vision of equal access to quality healthcare for every patient [1]. The purpose of this paper is to report on preliminary results of a global TIF survey that sought to examine the inequalities, which patients experience in their management by services and professionals across the world, and identify some of the reasons contributing to such inequalities. Emphasis in this investigation is given to the services that are offered from the patients’ point of view. This work derives from, and is part of TIF’s ongoing relationship with its member associations, individual patients, as well as health professional and health authorities.
文献表明,如果患者对整体护理的需求得到认可,并提供适当的服务,就可以实现长期生存和良好的生活质量。儿童时期曾经致命的疾病已成为成人生活的慢性病。[8,9,10]。该基金会的使命是促进和协助实施治疗地中海贫血和其他血红蛋白疾病的国家方案,无论患者居住在何处,其宗旨是使每一位患者都能平等获得优质医疗保健服务[b]。本文的目的是报告全球TIF调查的初步结果,该调查旨在检查患者在世界各地的服务和专业人员管理中遇到的不平等现象,并确定导致这种不平等的一些原因。这项调查的重点是从病人的角度提供的服务。这项工作来源于TIF与其成员协会、个体患者以及卫生专业人员和卫生当局的持续关系,并且是其一部分。
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引用次数: 0
Migration: The aftershocks to the provision of healthcare 移民:医疗保健的余震
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/THAL.2018.7471
S. Lobitz
Migration is the “movement of people to a new area or country in order to find work or better living conditions” (Oxford dictionary). The term “migration” summarizes forced, reluctant and voluntary migration. Voluntary migration is a comparatively constant event. But reluctant and, in particular, forced migration have been subject to substantial change during the last years. At the end of 2016, more than 17.2 million refugees (+ 5.3 million Palestinians) were on the run outside their home countries. 55% of them fled from Syria (5.5 million), Afghanistan (2.5 million) and South Sudan (1.4 million), respectively. The top hosting countries were not, in fact, the Southern and Western European or North American, but some of the poorest countries in the world. With the refugees from countries where disorders of haemoglobin are very prevalent, the number of patients in the host countries significantly increased within a very short period of time. The extraordinary circumstances required rapid rethinking and adaption and, therefore, did not only pose a big challenge but, in some countries, also a big chance to improve care for patients suffering from hemoglobinopathies. Although there are certainly several trouble spots in the world, the Middle East crisis was and still is currently the most prominent one. There is a significant prevalence of thalassemia and sickle cell disease among the Syrian and Iraqi population and since the chronically ill were presumably those who left their home countries first, there was a dramatic increase in the prevalence of thalassaemia and sickle cell disease in the host countries. Many patients fled to Western and Northern European countries where hemoglobinopathies were very rare and where the healthcare systems were unable to cope with this sudden increase in patient numbers and complications. For example, disease characteristics were much more pronounced than doctors were used to. Complications occurred that physicians only knew from textbooks. In addition, virtually all families needed significant help in psychosocial matters and many refugees were severely traumatized.
移民是指“人们为了找到工作或更好的生活条件而迁移到一个新的地区或国家”(牛津词典)。“移徙”一词概括了被迫、不情愿和自愿移徙。自愿移徙是一个相对经常发生的事件。但在过去几年里,不情愿的移民,尤其是被迫移民,发生了重大变化。截至2016年底,超过1720万难民(+530万巴勒斯坦人)在本国境外逃亡。其中55%分别逃离叙利亚(550万)、阿富汗(250万)和南苏丹(140万)。事实上,最大的东道国不是南欧、西欧或北美,而是世界上一些最贫穷的国家。由于难民来自血红蛋白疾病非常普遍的国家,东道国的患者人数在很短的时间内显著增加。特殊情况需要迅速反思和适应,因此,这不仅构成了巨大的挑战,而且在一些国家,也是改善血红蛋白病患者护理的大好机会。尽管世界上肯定有几个麻烦点,但中东危机过去是,现在仍然是最突出的一个。叙利亚和伊拉克人口中地中海贫血和镰状细胞病的患病率很高,由于慢性病患者可能是最先离开本国的人,因此地中海贫血和镰刀状细胞病在东道国的患病率急剧上升。许多患者逃到西欧和北欧国家,那里的血红蛋白病非常罕见,医疗系统无法应对患者数量和并发症的突然增加。例如,疾病特征比医生习惯的要明显得多。并发症的发生是医生从课本上才知道的。此外,几乎所有家庭都需要在心理社会事务方面得到重大帮助,许多难民受到严重创伤。
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引用次数: 1
New challenges in diagnosis of haemoglobinopathies: Migration of populations 血红蛋白病诊断的新挑战:人群迁移
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/THAL.2018.7474
J. Old, A. Timbs, J. McCarthy, A. Gallienne, M. Proven, M. Rugless, H. Lopez, J. Eglinton, D. Dziedzic, Matthew Beardsall, Mohamed S.M. Khalila, S. Henderson
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引用次数: 2
Iron overload and chelation therapy in hemoglobinopathies 血红蛋白病的铁过载与螯合治疗
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/thal.2018.7478
R. Bou-Fakhredin, J. Elias, A. Taher
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引用次数: 1
TIF conference presentation in detail TIF会议详细介绍
IF 0.3 Q4 HEMATOLOGY Pub Date : 2018-04-19 DOI: 10.4081/THAL.2018.7493
Barrister Abid Waheed Shaikh
The factors determining the health behaviours may be seen in various contexts: physical, socio-economic, cultural and political. So the utilization of a health care system, public or private, formal or non-formal, may depend on socio-demographic factors, social structures, level of education, cultural beliefs and practices, gender discrimination, status of women, economic and political systems environmental conditions, and the disease pattern and health care system itself. Policy makers need to understand the drivers of health seeking behaviour of the population in an increasingly pluralistic health care system. Also a more concerted effort is required for designing behavioural health promotion campaigns through inter-sectoral collaboration focusing more on disadvantaged segments of the population. Thalassaemia is the most prevalent genetic blood disorder in Pakistan. It is estimated that there are 8-10 million Thalassaemia Minor cases in the country with a prevalence of 5-6%. It is also estimated that about 100,000 patients suffering from Thalassaemia Major exist in Pakistan and every year this number is increasing by about 6,000. Pakistan is witnessing this large increase in thalassaemic patients due to a lack of proper coordinated, nationwide efforts to contain the inherited form of anaemia, and general public awareness...
决定健康行为的因素可以在不同的背景下看到:身体、社会经济、文化和政治。因此,公共或私人、正规或非正规医疗保健系统的利用可能取决于社会人口因素、社会结构、教育水平、文化信仰和习俗、性别歧视、妇女地位、经济和政治制度环境条件以及疾病模式和医疗保健系统本身。政策制定者需要了解在日益多元化的医疗保健系统中,人们寻求健康行为的驱动因素。此外,还需要更加协调一致地努力,通过部门间合作设计行为健康促进运动,更多地关注人口中的弱势群体。地中海贫血是巴基斯坦最常见的遗传性血液病。据估计,该国有8-10万例轻度地中海贫血病例,患病率为5-6%。据估计,巴基斯坦约有10万名严重地中海贫血患者,而且这个数字每年都在增加约6000人。由于缺乏适当协调的全国性努力来控制遗传性贫血,以及公众的普遍认识,巴基斯坦的地中海贫血患者人数大幅增加。。。
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引用次数: 0
期刊
Thalassemia Reports
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