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The source of HLA molecules on platelets: Does platelets adsorb soluble HLA molecules from their environment? HLA分子在血小板上的来源:血小板是否从其环境中吸附可溶性HLA分子?
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-10-10 DOI: 10.18502/ijpho.v9i4.1572
T. Dargahi, F. Yari, N. Rezaei
Background: The origin and function of human leukocyte antigen (HLA) class I molecules on platelets are still highly arguable. Given the differences in the results of the previous studies in this regard, the lack of research in recent years, and the clinical importance of HLA class I molecules, the absorption capacity of platelets for soluble HLA class I molecules was studied in this investigation. Materials and Methods: In this experimental study, HLA-A2 antigen was purified from a B cell precursor leukemia cell line (Nalm-6) by cell membrane protein solubilization and usage of HLA-A2 affinity column. Platelet concentrates (PCs) were received from Tehran Blood Transfusion Center. Eighteen bags of HLA-A2-negative PCs were prepared randomly and treated with various concentrations of the purified HLA antigen (100, 500, and 1000 ng/ml) for 48 to 72 hours. Subsequently, the HLA-A2 levels were evaluated on platelets by flow cytometery technique. Data were evaluated using repeated measure ANOVA.P-values less than 0.05 were considered significant. Results: The results of this study showed that the purified protein was an HLA molecule (HLA-A2).  After the treatment of platelets and HLA molecules, platelets inability was shown for the attracting of HLA molecules. This finding was true in both media of RPMI and plasma. The differences between the case (HLA-treated platelets) and control (untreated platelets) were not significant (p-values> 0.05). Conclusion: Platelets were unable to significantly adsorb exogenous HLA antigens from their environment. Further studies are needed to unravel the nature and origin of HLA molecules on platelets.
背景:人类白细胞抗原(HLA) I类分子在血小板上的起源和功能仍然有很大的争议。鉴于以往在这方面的研究结果存在差异,加之近年来研究的缺失,以及HLA I类分子在临床中的重要性,本研究对血小板对可溶性HLA I类分子的吸收能力进行了研究。材料与方法:本实验采用细胞膜蛋白增溶法,利用HLA-A2亲和柱,从B细胞前体白血病细胞株(Nalm-6)中纯化HLA-A2抗原。从德黑兰输血中心收到血小板浓缩物(PCs)。随机制备HLA- a2阴性PCs 18袋,分别用不同浓度纯化HLA抗原(100、500、1000 ng/ml)处理48 ~ 72小时。随后,用流式细胞术评估血小板HLA-A2水平。采用重复测量方差分析对数据进行评价。p值小于0.05被认为是显著的。结果:本研究结果表明,纯化蛋白为HLA- a2分子。经血小板和HLA分子处理后,显示血小板无法吸引HLA分子。这一发现在RPMI和血浆介质中都是正确的。病例组(hla处理过的血小板)与对照组(未处理过的血小板)的差异无统计学意义(p值0.05)。结论:血小板不能明显吸附环境中的外源HLA抗原。需要进一步的研究来揭示血小板上HLA分子的性质和起源。
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引用次数: 0
Evaluation of Twenty Four Discriminant Indices for Differentiating Beta-Thalassemia Trait from Iron Deficiency Anemia in Egyptians 埃及人β -地中海贫血与缺铁性贫血鉴别指标24项评价
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1163
S. Pessar
Background: Many Red Blood Cell (RBC) indices have been developed based on mathematical formulae to discriminate beta-thalassemia trait (βTT) from iron deficiency anemia (IDA). The latter two conditions represent the most common causes of microcytic hypochromic anemia in Egypt. This study aimed To evaluate the diagnostic reliability of 24 published discriminant indices for differentiating βTT from IDA in Egyptians. Materials and Methods: A cross sectional study included a total of 166 subjects (108 IDA & 58 βTT) aged 1-18 years were recruited from Hematology laboratory of  Pediatric Hospital, Ain Shams University, Cairo, Egypt. A full diagnostic algorithm was performed using complete blood count, hemoglobin electrophoresis by High Performance Liquid Chromatography (HPLC), iron profile and PCR detection of 22 mutations common for βTT. Twenty-four formulas were applied and their performance characteristics were calculated for each index. Results: The highest accuracy (True positive + True negative/ All cases) & Youden's Index (Sensitivity+Specificity-100) were for Red Cell Distribution Width (RDWI) and Hameed index closely followed by Keikhaei index while the least performance was for RDW-SD, RDW-CV and Shine & Lal indices. Conclusion The superiority of an index over another in distinguishing βTT from IDA allowed only partially better selection of cases warranting further confirmatory molecular studies. None of the studied formulae provided a surrogate test for Hb electrophoresis as mass screening.
背景:许多红细胞(RBC)指标基于数学公式来区分β -地中海贫血性状(βTT)和缺铁性贫血(IDA)。后两种情况是埃及小细胞性低色素贫血最常见的原因。本研究旨在评价24项已发表的埃及人βTT与IDA鉴别指标的诊断可靠性。材料与方法:横断面研究从埃及开罗艾因沙姆斯大学儿科医院血液学实验室招募了166名受试者(108名IDA和58名βTT),年龄1-18岁。利用全血细胞计数、高效液相色谱(HPLC)血红蛋白电泳、铁谱和PCR检测βTT常见的22个突变,建立完整的诊断算法。应用了24个公式,并计算了每个指标的性能特征。结果:红细胞分布宽度(RDWI)和Hameed指数准确率最高(真阳性+真阴性/所有病例),约登指数(灵敏度+特异性-100),Keikhaei指数次之,RDW-SD、RDW-CV和Shine & Lal指数准确率最低。结论某一指标在区分βTT和IDA方面的优势只允许部分更好地选择需要进一步确证性分子研究的病例。所研究的配方中没有一个提供替代试验的Hb电泳作为质量筛选。
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引用次数: 4
Seizure following acute hemolysis caused by Glucose-6-phosphate dehydrogenase Deficiency 葡萄糖-6-磷酸脱氢酶缺乏引起的急性溶血后癫痫发作
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1166
B. Darbandi, Simin Sajudi, V. Aminzadeh, Kioomars Golshekan, A. H. Rad, A. Baghersalimi
Background: Storage of platelet concentrates (PCs) at room temperature (20-24°C) limits its storage time to 5 Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common inherited enzyme deficiency of the human red blood cells . Most of G6PD deficient individuals are asymptomatic, but acute hemolytic anemia may be presented with nausea, vomiting, abdominal pain, headache, jaundice, pallor, discoloration of the urine, chills, and fever. Seizure is reported as a rare symptom, as well. The present study aimed to investigate seizure following acute hemolysis caused by Glucose-6-phosphate dehydrogenase deficiency. Material and Methods:  This analytic cross-sectional study was conducted on all consecutive patients aged 1-12 years with G6PD deficiency hospitalized for hemolysis in 17 Shahrivar children hospital, Rasht, Iran, in 2016. Demographic characteristics and other variables such as place of inhabitants, type of drinking water, history of seizure in the patients and family, cause of hemolysis, hemoglobin level and hemoglobinuria on admission, and infection history prior to hemolysis were recorded. Data were analyzed by Mann-Whitney U test and Fischer Exact Test.  P-value < 0.05 indicated statistical significance and data were assessed by SPSS (version 20). Results:  The youngest patient was one year old and the oldest was 11 years old. Most of them were males (68.9%). Out of 244 patients, 8 ones (3.3%) experienced seizure. There was a significant correlation between seizure occurrence and family history of seizure (p=0.03) as well as fava bean consumption (p=0.019) as the causes of hemolysis; but not with infection as the cause of hemolysis, hemoglobin or hemoglobinuria level on admission, types of drinking water, place of living, and gender. Methemoglobinemia was considered as the main cause of the seizure. Conclusion: Although the rate of seizure was not so high (3.3%), it seems that seizure can be a critical and potentially life-threatening complication in these patients. Environmental factors may also play a role in the pathogenesis of the seizure in these patients.
背景:血小板浓缩物(PCs)在室温(20-24°C)下的保存时间限制在5分钟以内。背景:葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症是人类红细胞中最常见的遗传性酶缺乏症。大多数G6PD缺乏者无症状,但急性溶血性贫血可表现为恶心、呕吐、腹痛、头痛、黄疸、脸色苍白、尿液变色、寒战和发烧。癫痫发作也是一种罕见的症状。本研究旨在探讨葡萄糖-6-磷酸脱氢酶缺乏引起的急性溶血后癫痫发作。材料与方法:本分析性横断面研究纳入2016年伊朗拉什特17家Shahrivar儿童医院所有因溶血住院的1-12岁G6PD缺乏症患者。记录人口学特征及其他变量,如居住地、饮用水类型、患者及家属癫痫发作史、溶血原因、入院时血红蛋白水平及血红蛋白尿、溶血前感染史等。数据分析采用Mann-Whitney U检验和Fischer精确检验。p值< 0.05为差异有统计学意义,采用SPSS (version 20)进行分析。结果:患者年龄最小1岁,最大11岁。其中以男性居多(68.9%)。244例患者中有8例(3.3%)发生癫痫发作。癫痫发作与家族史(p=0.03)、食用蚕豆(p=0.019)与溶血有显著相关性;但与溶血感染原因、入院时血红蛋白或血红蛋白尿水平、饮用水类型、居住地点和性别无关。高铁血红蛋白血症被认为是癫痫发作的主要原因。结论:虽然癫痫发作率不高(3.3%),但癫痫发作似乎是这类患者的一个重要且可能危及生命的并发症。环境因素也可能在这些患者癫痫发作的发病机制中起作用。
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引用次数: 0
Caring power of mothers who have a child with cancer and its predictors 患有癌症的孩子的母亲的关怀能力及其预测因素
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1167
Fatemeh Khademi, M. Rassouli, Leila Khanali Mojen, M. Heidarzadeh, Azam Shirinabadi Farahani, N. Borumandnia
Background: Attention to the family care provider needs and their caring power is essential. Since mothers are considered as the child’s main care provider, this study aimed to determine the caring power and its predictors among mothers of children with cancer. Materials and Methods: In this descriptive-correlational study, 196 mothers who had a child with cancer were selected through purposive sampling. The data were collected using two questionnaires, namely demographics questionnaire and the care power of the care providers of cancer patient questionnaire. The data were analyzed using SPSS 19 and running descriptive statistics and regression analysis. Results: The highest average score belonged to dimensions of “effective role play” (44.62 ± 5.28) and “trust” (14 ± 1.67), and the lowest ones respectively belonged to dimensions of “fatigue and resignation” (22.38 ± 6.33), “awareness” (8.46 ± 2.70), and “uncertainty” (12.38 ± 3.50). In addition, variables of educational level (p <0.001), adequacy of family income (p <0.001), and duration of illness (p0.29) were found as predictors of caring power. Conclusion: The results of this study showed that the caring power of mothers with a child with cancer is favorable. High trust and effective role-play reduced fatigue and resignation of mothers, and low awareness about the provision of care caused uncertainty affecting negatively the care power. In addition, the adequacy of family income, the high level of mother's education, and the reduction in the duration of the disease had a direct impact on care power.
背景:关注家庭照护者的需求和他们的照护能力是必要的。由于母亲被认为是儿童的主要照顾者,本研究旨在确定癌症儿童母亲的照顾能力及其预测因素。材料与方法:本研究采用目的性抽样的方法,选取196名有癌症子女的母亲为研究对象。数据采用人口统计学问卷和癌症患者护理提供者护理能力问卷两份问卷进行收集。采用SPSS 19进行数据分析,并进行描述性统计和回归分析。结果:得分最高的是“有效角色扮演”维度(44.62±5.28)和“信任”维度(14±1.67),得分最低的是“疲劳与顺从”维度(22.38±6.33)、“意识”维度(8.46±2.70)和“不确定性”维度(12.38±3.50)。此外,受教育程度(p <0.001)、家庭收入充足性(p <0.001)和疾病持续时间(p≤0.29)是关怀能力的预测变量。结论:本研究结果表明,母亲对患癌儿童的照顾能力是有利的。高信任和有效的角色扮演减少了母亲的疲劳和辞职,低意识的提供照顾造成不确定性负向影响照顾力。此外,家庭收入充足、母亲受教育程度高以及疾病持续时间缩短对照料能力有直接影响。
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引用次数: 10
The role of platelet microparticles in the production of antibodies from B lymphocytes against HLA-DR antigen in vitro 血小板微粒在B淋巴细胞体外产生抗HLA-DR抗原抗体中的作用
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1164
Zahra Khayati, F. Yari
Background: Platelets can activate B cells and stimulate them for the production of antibodies. Since platelet microparticles (PMPs) originate from platelets, they may have the same virtue. In the present study, the effect of PMPs was investigated on the production of human leukocyte antigen (HLA)-specific antibody from B cells in vitro. Materials and Methods: In this experimental study, HLA-DR antigen was solubilized from the immortalized B lymphocytes (Daudi cell line) and purified using the affinity chromatography. Antigen properties were determined by the ELISA technique. PMPs were isolated from platelet concentrate bags by centrifugation. Fresh blood products were prepared from the Innovation Center of Iranian Blood Transfusion Organization (IBTO) and B lymphocytes were purified by the MACS method. B cells were exposed with PMPs and HLA-DR antigens in the culture medium. On the third day of culture, the culture supernatant was examined in terms of antibody production using the ELISA test. The results were analyzed using paired sample T-test and P-value <0.05 was considered statistically significant. Results: The specificity of the purified HLA-DR antigen was confirmed using the anti-HLA-DR antibody and ELISA technique in the presence of appropriate controls. The results showed that PMPs could stimulate the production of antibodies from B cells. The difference between the case and control was significant (P-value=0.001). Although total immunoglobulin (IgG) was higher in HLA-DR-treated wells, HLA-DR-specific antibodies were not identified by ELISA technique.  Conclusion: PMPs have the capability to induce IgG antibodies from B cells. In order to ensure the production of specific antibodies, further testing is required with high sensitivity.
背景:血小板可以激活B细胞并刺激其产生抗体。由于血小板微粒(pmp)起源于血小板,它们可能具有相同的优点。本研究探讨了pmp对B细胞产生人白细胞抗原(HLA)特异性抗体的影响。材料和方法:从永生化B淋巴细胞(Daudi细胞系)中溶解HLA-DR抗原,采用亲和层析纯化。采用酶联免疫吸附法测定抗原性质。用离心法从血小板浓缩袋中分离出pmp。从伊朗输血组织创新中心(IBTO)制备新鲜血液制品,用MACS法纯化B淋巴细胞。将B细胞暴露于pmp和HLA-DR抗原的培养基中。培养第3天,用ELISA法检测培养上清液的抗体产生情况。结果采用配对样本t检验,p值<0.05为差异有统计学意义。结果:在存在适当对照的情况下,用抗HLA-DR抗体和ELISA技术证实纯化的HLA-DR抗原的特异性。结果表明,pmp可以刺激B细胞产生抗体。病例与对照组的差异有统计学意义(p值=0.001)。虽然总免疫球蛋白(IgG)在hla - dr处理的井中较高,但ELISA技术未检测到hla - dr特异性抗体。结论:pmp具有诱导B细胞产生IgG抗体的能力。为了确保产生特异性抗体,需要进一步进行高灵敏度的检测。
{"title":"The role of platelet microparticles in the production of antibodies from B lymphocytes against HLA-DR antigen in vitro","authors":"Zahra Khayati, F. Yari","doi":"10.18502/IJPHO.V9I3.1164","DOIUrl":"https://doi.org/10.18502/IJPHO.V9I3.1164","url":null,"abstract":"Background: Platelets can activate B cells and stimulate them for the production of antibodies. Since platelet microparticles (PMPs) originate from platelets, they may have the same virtue. In the present study, the effect of PMPs was investigated on the production of human leukocyte antigen (HLA)-specific antibody from B cells in vitro. \u0000Materials and Methods: In this experimental study, HLA-DR antigen was solubilized from the immortalized B lymphocytes (Daudi cell line) and purified using the affinity chromatography. Antigen properties were determined by the ELISA technique. PMPs were isolated from platelet concentrate bags by centrifugation. Fresh blood products were prepared from the Innovation Center of Iranian Blood Transfusion Organization (IBTO) and B lymphocytes were purified by the MACS method. B cells were exposed with PMPs and HLA-DR antigens in the culture medium. On the third day of culture, the culture supernatant was examined in terms of antibody production using the ELISA test. The results were analyzed using paired sample T-test and P-value <0.05 was considered statistically significant. \u0000Results: The specificity of the purified HLA-DR antigen was confirmed using the anti-HLA-DR antibody and ELISA technique in the presence of appropriate controls. The results showed that PMPs could stimulate the production of antibodies from B cells. The difference between the case and control was significant (P-value=0.001). Although total immunoglobulin (IgG) was higher in HLA-DR-treated wells, HLA-DR-specific antibodies were not identified by ELISA technique. \u0000 Conclusion: PMPs have the capability to induce IgG antibodies from B cells. In order to ensure the production of specific antibodies, further testing is required with high sensitivity.","PeriodicalId":44212,"journal":{"name":"Iranian Journal of Pediatric Hematology and Oncology","volume":"13 1","pages":""},"PeriodicalIF":0.3,"publicationDate":"2019-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88615993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Platelet Indices as Useful Indicators of Urinary Tract Infection 血小板指数作为尿路感染的有用指标
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1165
A. Akya, Z. Rostami-Far, R. Lorestani, S. Khazaei, A. Elahi, M. Rostamian, Bahare Andayeshgar, K. Ghadiri
Background: The changes of platelet parameters can be a useful index for rapid diagnosis of urinary tract infection (UTI), since platelet changes are routinely determined through complete blood count (CBC) test. The correlation between platelet indices, included number (PLTs), mean platelet volume (MPV) and platelet distribution width (PDW), which are the indicators of production and function of platelets, with UTI was evaluated in this study.         Materials and Methods: In this descriptive-analytical study, 97 patients with UTI (patient group) and 117 healthy people (control group). The average age for the patient and the control group was 10.84±6.68 and 11.34±7.1 years old, respectively. This study was done during 2016-2018 in Imam Reza Hospital, Kermanshah, west of Iran. The PLT, MPV, PDW, and other inflammatory indices, including white blood cell, neutrophils, lymphocytes, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) were evaluated. The diagnosis of bacteria was done using routine microbiological and biochemical methods. The platelet indices were statistically compared between the patients and the control groups (T test and Chi square test). Results: The most common isolated gram negative and gram positive bacteria were E. coli, Citrobacter, and Staphylococcus aureus, respectively. In the patient group, PLT number was significantly higher than that in the control group (p=0.0007), while difference of other indices such as MPV, PDW, neutrophils, lymphocytes, CRP, and ESR were not statistically significant between the two groups. In case of UTI with gram positive bacteria, PLT number (p=0.05) was lower but MPV (p=0.02) and PDW (p=0.045) was higher compared to the UTI with gram negative bacteria. Conclusion: The results of this study showed that the platelet number could be a useful diagnostic index for urinary tract infection. However, more studies need to be done with higher number of patients to evaluate the more details of platelet changes during UTIs.
背景:血小板参数的变化是快速诊断尿路感染(UTI)的一个有用指标,因为血小板的变化是通过常规的全血细胞计数(CBC)检测来确定的。本研究评估血小板数量(PLTs)、平均血小板体积(MPV)和血小板分布宽度(PDW)等血小板生成和功能指标与UTI的相关性。材料与方法:本研究采用描述性分析方法,选取97例尿路感染患者(患者组)和117例健康人(对照组)。患者和对照组的平均年龄分别为10.84±6.68岁和11.34±7.1岁。这项研究于2016-2018年在伊朗西部克尔曼沙的伊玛目礼萨医院完成。评估PLT、MPV、PDW及其他炎症指标,包括白细胞、中性粒细胞、淋巴细胞、红细胞沉降率(ESR)、c反应蛋白(CRP)。细菌诊断采用常规微生物学和生化方法。患者与对照组的血小板指数比较有统计学意义(T检验和卡方检验)。结果:革兰氏阴性菌和革兰氏阳性菌分别为大肠杆菌、柠檬酸杆菌和金黄色葡萄球菌。患者组PLT数量显著高于对照组(p=0.0007),而两组间MPV、PDW、中性粒细胞、淋巴细胞、CRP、ESR等指标差异均无统计学意义。与革兰氏阴性菌感染的UTI相比,革兰氏阳性菌感染的PLT数(p=0.05)较低,MPV (p=0.02)和PDW (p=0.045)较高。结论:血小板计数可作为尿路感染的诊断指标。然而,需要对更多的患者进行更多的研究,以评估尿路感染期间血小板变化的更多细节。
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引用次数: 7
The Effect of Xmn1 Gene Polymorphism on Blood Transfusion Dependency and Hemoglobin Concentration among Iranian Thalassemia Patients with IVSII-1 Mutation xm1基因多态性对伊朗地中海贫血IVSII-1突变患者输血依赖及血红蛋白浓度的影响
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1168
M. Hashemieh, A. Azarkeivan, H. Najmabadi, K. Sheibani
Background: One of the most important phenotypic modifying factors for thalassemia is the presence of Xmn1 polymorphism. This retrospective study was performed to investigate the overall prevalence of Xmn1 polymorphism among Iranian β-thalassemia patients with homozygote IVSII-1mutation and to assess the relationship between Xmn1 polymorphism with patients’ hemoglobin levels and the response to hydroxyurea (Hu) therapy. Materials and Methods: The present cross sectional study included 112 β-thalassemia patients with homozygote IVSII-1 mutation. Laboratory investigations included complete blood count and routine hematological indices were measured by Sysmex K1000 (Japan) blood auto analyzer.  To find the state of Xmn1 polymorphism, blood samples were collected from patients using EDTA containers for genomic DNA analysis. DNA extraction and amplification-refractory mutation to determine the Xmn-1 polymorphism were performed. Results: In total, 206 thalassemia patients including 112 patients diagnosed as thalassemia major and 94 patients diagnosed as thalassemia intermediate entered the study. The mean age at the start of transfusion was 5 ± 6.4 years old, and all of the patients received hydroxyurea. Twenty eight patients (14%) did not show any Xmn 1 polymorphisms (- / -), and 178 patients (86%) showed polymorphism either in one loci (- / +, 44 patients, 21.3%) or both loci   (+ / +, 134 patients, 65%).  Patients with Xmn1 polymorphism showed significantly higher age at diagnosis (p=0.002), higher age at start of transfusion (p=0.001), higher hemoglobin levels after treatment with hydroxyurea (p=0.005), and lower transfusion dependency (P=0.044). Conclusion: The presence of Xmn1 polymorphism led to a delay in onset of blood transfusions, higher hemoglobin levels, better response to hydroxyurea treatment and milder phenotypic presentation among thalassemia patients with IVSII-1 mutation.
背景:地中海贫血最重要的表型修饰因素之一是Xmn1多态性的存在。本回顾性研究旨在调查伊朗β-地中海贫血纯合子ivsii -1突变患者中Xmn1多态性的总体流行情况,并评估Xmn1多态性与患者血红蛋白水平和羟基脲(Hu)治疗反应的关系。材料与方法:本横断面研究纳入112例纯合子IVSII-1突变的β-地中海贫血患者。实验室采用日本Sysmex K1000全自动血液分析仪检测全血细胞计数和常规血液学指标。为了发现Xmn1多态性的状态,使用EDTA容器采集患者血液样本进行基因组DNA分析。DNA提取和扩增难解突变检测Xmn-1多态性。结果:共纳入206例地中海贫血患者,其中重度地中海贫血112例,中度地中海贫血94例。输血开始时的平均年龄为5±6.4岁,所有患者均接受羟基脲治疗。28例(14%)患者未表现出任何Xmn 1多态性(- / -),178例(86%)患者在一个位点(- / +,44例,21.3%)或两个位点(+ / +,134例,65%)均表现出多态性。Xmn1多态性患者的诊断年龄(p=0.002)、输血开始年龄(p=0.001)、羟脲治疗后血红蛋白水平升高(p=0.005)、输血依赖性降低(p= 0.044)。结论:在伴有IVSII-1突变的地中海贫血患者中,Xmn1多态性的存在导致输血时间延迟、血红蛋白水平升高、对羟基脲治疗反应更好、表型表现较轻。
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引用次数: 2
The Incidence of Childhood Cancer in Iran: A systematic review and meta-analysis 伊朗儿童癌症发病率:系统回顾和荟萃分析
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1170
Soheil Hassanipour, M. Fathalipour, Hamed Delam, M. Ghorbani, E. Abdzadeh, M. Arab-Zozani, Shirin Riahi, H. Salehiniya
Background: Childhood cancer (ChC) is very rare and occurs between birth and 14 years of age. There are several reports about ChC incidence from various regions of Iran, but with conflicting results.  The present study aimed to do a systematic review to estimate the accurate incidence rate of ChC among Iranian people. Materials and Methods: This systematic review was performed based on the preferred reporting items for systematic reviews and meta-analyses (PRISMA) checklist in 2018. A literature search was conducted using international databases (Medline/PubMed, Scopus, ISI/Web of Knowledge, and Google Scholar) for English papers, and national databases (Scientific Information Database, MagIran, IranMedex, and IranDoc) for Persian papers which estimated the incidence rate of ChC in any geographical location in  Iran. The incidence rate of ChC was calculated using random-effect model. Results: Out of 157 papers in the primary searches, 12 studies were included by advanced screening and refinement. The crude incidence rate (CIR) of ChC in 0-14 years was 16.8 per 100,000 (95% CI: 9.04-24.56) for boys and 16.56 per 100,000 (95% CI: 10.51-22.62) for girls. Conclusion: The incidence of ChC in Iran is higher compared to other parts of the world. Considering this issue, holding some interventional programs on tackling potential risk factors, including air pollution, in different regions of Iran is suggested.
背景:儿童癌症(ChC)非常罕见,发生在出生至14岁之间。有几份关于伊朗不同地区ChC发病率的报告,但结果相互矛盾。本研究旨在对伊朗人群中ChC的准确发病率进行系统评价。材料和方法:本系统评价基于2018年系统评价和荟萃分析(PRISMA)清单的首选报告项目进行。文献检索使用国际数据库(Medline/PubMed、Scopus、ISI/Web of Knowledge和Google Scholar)检索英文论文,使用国家数据库(Scientific Information Database、MagIran、IranMedex和IranDoc)检索波斯语论文,这些文献估计了伊朗任何地理位置的ChC发病率。采用随机效应模型计算ChC的发病率。结果:在初步检索的157篇论文中,通过高级筛选和细化纳入了12篇研究。0 ~ 14岁男孩ChC的粗发病率(CIR)为16.8 / 10万(95% CI: 9.04 ~ 24.56),女孩ChC的粗发病率为16.56 / 10万(95% CI: 10.51 ~ 22.62)。结论:伊朗ChC发病率高于世界其他地区。考虑到这一问题,建议在伊朗不同地区举办一些干预项目,以解决潜在的风险因素,包括空气污染。
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引用次数: 10
Metastatic Melanoma (Secondary Malignancy) after Recovery from Acute Lymphoblastic Leukemia in a 10-year-old Girl: a case report 一例10岁女孩急性淋巴细胞白血病恢复后的转移性黑色素瘤(继发性恶性)报告
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-06-10 DOI: 10.18502/IJPHO.V9I3.1172
Saeed Yousofian, G. Miri-Aliabad, T. Rostami, M. Mehrazma, Pardis Nematollahi
Acute lymphoblastic leukemia is the most common malignancy in children with a 5-year survival rate, accounting for 80% of cases. Melanoma is rare in children and has been reported as a sporadically occurring secondary malignant neoplasm in children with acute lymphoblastic leukemia. This study presented a 10-year-old Iranian child with pre-B-cell acute lymphoblastic leukemia that was diagnosed at age 6. She was fully recovered after 2 years of treatment. One year and six months after cessation of treatment, she was referred  with a 1×2 cm mass in her right parietal region of scalp. Biopsy of the lesion confirmed the diagnosis of malignant melanoma. Computed tomography scan of the chest and abdomen also confirmed extensive liver metastasis which was corroborated by liver biopsy. Bone scan also revealed bone metastases. Early diagnosis and treatment of these tumors is extremely important and these patients should be closely monitored and undergo regular physical examination.
急性淋巴细胞白血病是儿童最常见的恶性肿瘤,5年生存率为80%。黑色素瘤在儿童中很少见,据报道是急性淋巴细胞白血病儿童中偶发的继发性恶性肿瘤。本研究报告了一名10岁的伊朗儿童,在6岁时被诊断患有前b细胞急性淋巴细胞白血病。经过2年的治疗,她完全康复了。停止治疗1年零6个月后,她因头皮右顶叶区1×2 cm肿块而被转诊。病灶活检证实为恶性黑色素瘤。胸部和腹部的计算机断层扫描也证实了广泛的肝转移,肝活检证实了这一点。骨扫描也显示骨转移。这些肿瘤的早期诊断和治疗非常重要,这些患者应密切监测并定期进行体检。
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引用次数: 0
Induced pluripotent stem cells (iPSCs) based approaches for hematopoietic cancer therapy 基于诱导多能干细胞(iPSCs)的造血肿瘤治疗方法
IF 0.3 Q4 PEDIATRICS Pub Date : 2019-03-10 DOI: 10.18502/IJPHO.V9I2.611
Bardia Khandany, M. Heidari, Mehri Khatami
Induced pluripotent stem cells (iPSCs) are reprogrammed from somatic cells through numerous transcription factors. Human induced pluripotent stem cell approaches are developing as a hopeful strategy to improve our knowledge of genetic association studies and the underlying molecular mechanisms.  Rapid progression in stem cell therapy and cell reprogramming provides compelling reasons for its feasibility for treating a wide range of diseases through the replacement of autologous cells. Continuous failure in embryonic stem cells (ESC) production and the dependency of iPSC on ectopic genes may be due to the inability to maintain the stability of the endogenous gene systems which are essential for creation of pluripotency state. With recent developments in the genome processing and human tissue culturing approaches as well as xenotransplantation, bioengineering, and genome editing, induced pluripotent stem cells offer the new opportunities for the study of human cancers. Most hematopoietic malignancies are originated from cells that are functionally heterogeneous and few of them are responsible for maintaining tumor state. The naming of these cancer stem cells are due to the quality characteristics of normal tissue stem cells, such as self-renewal, long term survival, and the ability to produce cells with more differentiated properties. The aim of present study was to focus on the recent progresses in the application of stem cell-based hematopoietic cancer, and to assess the benefits of treatment, opportunities, and shortcomings that can potentially help improve future efforts in experimental and clinical studies.
诱导多能干细胞(iPSCs)是由体细胞通过多种转录因子重新编程而成的。人类诱导多能干细胞方法作为一种有希望的策略正在发展,以提高我们对遗传关联研究和潜在分子机制的认识。干细胞治疗和细胞重编程的快速发展为其通过替换自体细胞治疗多种疾病的可行性提供了令人信服的理由。胚胎干细胞(ESC)产生的持续失败和iPSC对异位基因的依赖可能是由于无法维持多能状态产生所必需的内源基因系统的稳定性。随着基因组处理和人体组织培养方法以及异种移植、生物工程和基因组编辑的最新发展,诱导多能干细胞为人类癌症的研究提供了新的机会。大多数造血恶性肿瘤起源于功能异质细胞,很少有细胞负责维持肿瘤状态。这些癌症干细胞的命名是由于正常组织干细胞的质量特征,如自我更新,长期存活,以及能够产生具有更多分化特性的细胞。本研究的目的是关注干细胞造血癌应用的最新进展,并评估治疗的益处,机会和缺点,这些可能有助于改进未来的实验和临床研究。
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引用次数: 1
期刊
Iranian Journal of Pediatric Hematology and Oncology
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