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Membrane endothelial protein C receptor expression in renal tissue of pediatric lupus nephritis patients 膜内皮蛋白C受体在儿童狼疮性肾炎患者肾组织中的表达
IF 0.3 Pub Date : 2019-04-01 DOI: 10.21608/EJPA.2019.41537
M. Ibrahim, S. El-Sayed, R. Said, M. Ismail, N. Ahmed, N. Radwan
Background: Lupus nephritis (LN) is more common and more severe is pediatric systemic lupus erythematosus (pSLE). Endothelial protein C receptor (EPCR) is an inducer of anti-apoptotic pathways in endothelial cells. Recent studies have taken elevated anti-injury biomarkers as EPCR into consideration regarding their roles to antagonize LN. Objectives: to evaluate the membrane expression of endothelial protein C receptor (mEPCR) in the renal microvasculature in pediatric patients with LN. Methods: This study was conducted on 25 patients with pSLE following up at the Allergy and Immunology Clinic, Children’s Hospital, Ain Shams University. The 25 patients have LN proved by a previous renal biopsy. Medical history, clinical examination and routine laboratory investigations for assessment of disease activity were done for all patients. Paraffin blocks of patients’ renal biopsies were subjected to immunohistochemistry staining for the frequency of mEPCR. Results: mEPCR was mainly expressed in the endothelium of the peritubular capillaries. Our results showed that an equal number of patients had nil and mild marker expression (8 patients each, 32%) while 9 patients (36%) showed moderate/strong marker expression. We found that 9 out of 10 (90%) of patients with class II had nil/mild marker expression, 5 patients out of 9 (55.5%) with class III had mild/moderate marker expression, while 5 patients 0ut of 6 (83.3%) with class IV and V had moderate/strong marker expression. We only found a significant statistical difference between the different degrees of mEPCR expression regarding 24 hours urinary proteins. No statistical significance was found between the different degrees of mEPCR expression and different immuno-suppressive therapy dose/kg or renal outcome using the renal British Isles Lupus Assessment Group (BILAG) score; in spite that most of the patients who got improved had nil/mild marker expression. Conclusion: mEPCR -bearing a statistically significant difference in relation to different LN classes- showed more expression in the more aggressive classes; a finding which might suggest a contribution of the endothelium of the renal parenchyma to the pathophysiology of more progressive LN. Hence the tissue marker might emerge as a potential new therapeutic target in the search for more selective treatment for SLE. Keywords: p SLE, mEPCR, renal biopsy, immunohistochemistry, BILAG, lupus nephritis
背景:狼疮性肾炎(LN)更常见,更严重的是儿童系统性红斑狼疮(pSLE)。内皮蛋白C受体(EPCR)是内皮细胞中抗凋亡途径的诱导剂。最近的研究已经将升高的抗损伤生物标志物作为EPCR考虑到它们对抗LN的作用。目的:评价内皮蛋白C受体(mEPCR)在LN患儿肾微血管中的膜表达。方法:本研究在艾因沙姆斯大学儿童医院过敏和免疫学诊所对25名pSLE患者进行随访。25例患者既往肾活检证实为LN。对所有患者进行病史、临床检查和常规实验室调查,以评估疾病活动性。对患者肾活检的石蜡块进行mEPCR频率的免疫组织化学染色。结果:mEPCR主要表达于管周毛细血管内皮。我们的结果显示,同样数量的患者无和轻度标记物表达(各8例,32%),而9例患者(36%)显示中度/强烈标记物表达。我们发现,10名II类患者中有9名(90%)无/轻度标志物表达,9名III类患者中5名(55.5%)有轻度/中度标志物表达;而6名IV和V类患者中,5名(83.3%)有中度/强烈标志物表达。我们只发现在24小时尿蛋白中mEPCR表达的不同程度之间存在显著的统计学差异。使用肾脏不列颠群岛狼疮评估组(BILAG)评分,mEPCR表达的不同程度与不同的免疫抑制治疗剂量/kg或肾脏结果之间没有统计学意义;尽管大多数好转的患者的标志物表达为零/轻度。结论:mEPCR在不同LN类别中具有统计学意义的差异,在更具攻击性的类别中表现出更多的表达;这一发现可能表明肾实质内皮对进展性LN的病理生理学有贡献。因此,该组织标志物可能成为寻找更选择性治疗SLE的潜在新治疗靶点。关键词:p SLE,mEPCR,肾活检,免疫组织化学,BILAG,狼疮性肾炎
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引用次数: 0
Selections from international journals 国际期刊精选
IF 0.3 Pub Date : 2019-04-01 DOI: 10.21608/EJPA.2019.41540
N. Heshmat
The well-proven benefits of influenza immunization can now be made available to persons with a history of egg allergy. Individuals with diagnosed or suspected egg allergy who need an influenza vaccination should be evaluated by an allergist/immunologist for evaluation of egg allergy and for administration of the 2010-2011 trivalent influenza vaccine (TIV) if clinically indicated. Studies have suggested that influenza vaccines can be administered to patients with a history of anaphylaxis to egg without adverse effects. However, such studies are limited in number, and reactions to influenza vaccines in egg allergic persons can occur. Caution is warranted in patients with a history of anaphylaxis or where the severity of their clinical reactivity is uncertain, particularly when the ovalbumin content of the vaccine is unknown. Therefore, consultation with an allergist experienced in food allergy and anaphylaxis is strongly recommended. For the 2010-2011 influenza season, the routine practice of skin testing to the TIV is no longer recommended. Both the 2-dose (10%, 90%) and single-dose methods are appropriate for administering influenza vaccine to egg allergic individuals. Egg allergic individuals can receive TIV without prior skin testing to the vaccine, with the vaccine being administered via a 2-step graded challenge: first administer 10% of the age-appropriate dose, with a 30-minute observation after administration for symptom development. If no symptoms develop, the remaining 90% can be administered, with a 30minute observation for symptom development. The same TIV product brand should be used for booster vaccinations if possible, but it is not necessary to use the same lot. Egg allergic individuals can receive TIV without prior skin testing to the vaccine as a single, age-appropriate dose without use of graded challenge. Individuals should be observed for 30 minutes after injection for evidence of a systemic reaction. The same TIV product brand should be used for booster vaccinations, but the same lot is not necessary.
有鸡蛋过敏史的人现在可以获得流感免疫接种的充分证明的好处。确诊或疑似对鸡蛋过敏的人需要接种流感疫苗,应由过敏专科医生/免疫学家评估鸡蛋过敏情况,并在临床指征时接种2010-2011年三价流感疫苗(TIV)。研究表明,流感疫苗可用于有鸡蛋过敏史的患者,而不会产生不良反应。然而,这样的研究数量有限,而且对鸡蛋过敏的人可能会对流感疫苗产生反应。有过敏史或临床反应严重程度不确定的患者应谨慎使用,特别是在疫苗卵清蛋白含量未知的情况下。因此,强烈建议咨询在食物过敏和过敏反应方面经验丰富的过敏专科医生。在2010-2011年流感季节,不再推荐对TIV进行皮肤测试的常规做法。2剂法(10%、90%)和单剂法均适用于对鸡蛋过敏者接种流感疫苗。鸡蛋过敏的个体可以在没有事先对疫苗进行皮肤试验的情况下接种TIV,疫苗通过两步分级接种:首先接种适合年龄剂量的10%,注射后30分钟观察症状发展。如果没有出现症状,则可给予剩余90%的药物,并观察30分钟以观察症状的出现。如果可能的话,应使用相同的TIV产品品牌进行加强接种,但没有必要使用相同的批次。鸡蛋过敏的个体可以在没有事先皮肤试验的情况下接种TIV疫苗,作为单一的,适合年龄的剂量,不使用分级激发。个体应在注射后观察30分钟,以确定是否有全身反应。加强疫苗接种应使用相同的TIV产品品牌,但没有必要使用相同的批号。
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引用次数: 0
Editorial 社论
IF 0.3 Pub Date : 2019-04-01 DOI: 10.21608/ejpa.2019.41530
Y. El-Gamal
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引用次数: 0
Double negative alpha beta T cells in pediatric hemophagocytic syndromes 双阴性α - β T细胞在儿童噬血细胞综合征中的作用
IF 0.3 Pub Date : 2019-04-01 DOI: 10.21608/EJPA.2019.41533
E. Hossny, Rasha H. El-Owaidy, H. M. Afifi, H. Ahmad
Introduction: Autoimmune lymphoproliferative syndrome (ALPS) and hemophagocytic lymphohistiocytosis (HLH) share clinical and laboratory features including lymphadenopathy, splenomegaly, and pancytopenia. We sought to measure αβ double negative T cells (αβ DNT) in a group of patients with established diagnosis of HLH in relation to disease activity and severity. Methods: We conducted a follow-up, controlled study that comprised 25 patients with HLH and 25 healthy matched controls. Patients were subjected to clinical evaluation and flowcytometric measurement of αβ DNT Cells at presentation and 9 weeks after start of HLH induction treatment. Results: In 17 (68%) patients, infection was the trigger of HLH while the cause was malignancy in three (12%), and rheumatological disorders in two patients (8%). At enrollment, 15 patients (60%) had αβ DNT cells levels [median (IQR): 1.71 (1.25-2.12)] that were significantly higher than the control values [median (IQR): 0.7 (0.4-0.8)] (p<0.001). The αβ DNT counts of patients were also higher at enrollment as compared to values at the end of week 9 [median (IQR): 0.76 (0.45-1.17)]; p=0.018. Survivors (n=8) and non-survivors (n=17) had comparable levels of αβ DNT cells at enrollment (p=0.861). αβ DNT cell count correlated positively with ALT (p=0.019) and negatively with CD4/CD8 ratios (p=0.023). Conclusion: Elevated αβ DNT cell counts might be related to the HLH process and this implies that mild elevation can exist in HLH and are not specific to ALPS. Wider scale studies with longer periods of follow up are needed to validate the results and properly outline the correlation between both medical conditions. Keywords: Hemophagocytic lymphohistiocytosis, Double negative T cells, mortality, ALPS
自身免疫性淋巴细胞增生性综合征(ALPS)和噬血细胞性淋巴组织细胞增多症(HLH)具有共同的临床和实验室特征,包括淋巴结病、脾肿大和全血细胞减少症。我们试图在一组确诊为HLH的患者中测量αβ双阴性T细胞(αβ DNT)与疾病活动性和严重程度的关系。方法:我们进行了一项随访,对照研究,包括25例HLH患者和25例健康匹配的对照组。患者在入院时和HLH诱导治疗开始后9周进行αβ DNT细胞的临床评价和流式细胞术检测。结果:在17例(68%)患者中,感染是HLH的触发因素,3例(12%)患者的病因是恶性肿瘤,2例(8%)患者的病因是风湿病。入组时,15例(60%)患者αβ DNT细胞水平[中位数(IQR): 1.71(1.25-2.12)]显著高于对照组[中位数(IQR): 0.7 (0.4-0.8)] (p<0.001)。与第9周末相比,患者入组时αβ DNT计数也更高[中位数(IQR): 0.76 (0.45-1.17)];p = 0.018。幸存者(n=8)和非幸存者(n=17)在入组时αβ DNT细胞水平相当(p=0.861)。αβ DNT细胞计数与ALT呈正相关(p=0.019),与CD4/CD8呈负相关(p=0.023)。结论:αβ DNT细胞计数升高可能与HLH过程有关,提示HLH可能存在轻度升高,而非ALPS特异性升高。需要更大规模的研究和更长时间的随访来验证结果,并适当地概述这两种医疗状况之间的相关性。关键词:噬血细胞淋巴组织细胞增多症;双阴性T细胞;死亡率
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引用次数: 1
Test yourself in eczema in primary immunodeficiency 在原发性免疫缺陷的湿疹中测试自己
IF 0.3 Pub Date : 2019-04-01 DOI: 10.21608/EJPA.2019.41538
N. Radwan
4. Characteristic features in DOCK8 deficiency includes all EXCEPT: a) Presence of eczema b) Recurrent sinopulmonary infections c) Recurrent viral infections d) Low CD4+ cells and CD8+ e) Coarse features 5. A patient aged 3 years, presenting with eczema, recurrent sino-pulmonary infections and viral infections, neutropenia. He had normal immunoglobulin level. He had mild CD4+ lymphopenia. He had mild pulmonary stenosis which was accidentally discovered. His EBV PCR was persistently positive for the last 6 months. What is your provisional diagnosis? a) DOCK8 deficiency b) AD hyperimmunoglobulin E c) Wiscott Aldrich Syndrome d) Mammalian sterile 20-like 1(MST-1) e) ShwachmanDiamond syndrome
4.DOCK8缺乏症的特征特征包括所有特征,除了:a)湿疹的存在b)复发性窦房结感染c)复发性病毒感染d)CD4+细胞和CD8+e)粗糙特征5。一名3岁的患者,表现为湿疹、复发性中肺感染和病毒感染、中性粒细胞减少症。他的免疫球蛋白水平正常。他有轻度CD4+淋巴细胞减少症。他有轻微的肺动脉狭窄,这是意外发现的。在过去的6个月里,他的EBV PCR一直呈阳性。你的临时诊断是什么?a) DOCK8缺乏症b)AD高免疫球蛋白E c)Wiscott-Aldrich综合征d)哺乳动物不育性20样1(MST-1)E)Shwachman-Diamond综合征
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引用次数: 0
The critical level of vitamin D in childhood asthma 儿童哮喘中维生素D的临界水平
IF 0.3 Pub Date : 2018-12-01 DOI: 10.1093/qjmed/hcy200.145
R. Hassan El-Owaidy, Y. Mohammed El-Gamal, M. Aly Shabaan, M. Haggag Hassan
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引用次数: 1
Use of antibiotics for asthma attacks in Egyptian children 埃及儿童哮喘发作时抗生素的使用情况
IF 0.3 Pub Date : 2018-10-01 DOI: 10.21608/EJPA.2018.30635
N. Rifai, H. Rizk
Background : Bacterial infection plays a negligible role in pediatric acute asthma attacks. However, many patients are treated empirically with antibiotics. Objective : This study attempted to investigate the problem of antibiotic usage as a part of treatment of acute asthma among children living in Egypt. Methods : The current exploratory study included a random sample of 300 children visiting the allergy clinic, Faculty of Medicine, Cairo University from September 2016 to September 2017. A study researcher applied a validated questionnaire to the child's parents about usage of antibiotics. Results : Most families belonged to the low or middle social class. About 65.7% of children had sub-optimal asthma control, 73.7% experienced acute attacks, with over half of them (51%) receiving antibiotics; 41.8% of those who received antibiotics were self-medicated. Over 90% reused previous prescriptions. Main single reasons for resorting to self-medication were to save money and the assumption that previous prescriptions can be reused. About 45.3% expressed intention to employ self-medication in the future. Low social class was associated with significantly higher odds of resorting to self-medication (odds ratio=3.32; p=0.05). Conclusion : A remarkable antibiotic use was detected among children who had acute asthma, a large proportion of which was without medical consultation. Family social class was an independent predictor for adopting self-medication. Keywords : Asthma, attacks, antibiotics, Egyptian, children.
背景:细菌感染在儿童急性哮喘发作中起着微不足道的作用。然而,许多病人是经验性地使用抗生素治疗。目的:本研究试图调查抗生素的使用问题,作为治疗急性哮喘的一部分在埃及儿童生活。方法:本探索性研究随机抽取2016年9月至2017年9月在开罗大学医学院过敏门诊就诊的300名儿童为研究对象。一名研究人员向孩子的父母申请了一份关于抗生素使用情况的有效问卷。结果:大部分家庭属于中低阶层。约65.7%的儿童哮喘控制不佳,73.7%发生急性发作,其中超过一半(51%)接受抗生素治疗;接受抗生素治疗的患者中有41.8%是自行用药。超过90%的人重复使用以前的处方。使用自我药疗的唯一主要原因是省钱和假设以前的处方可以重复使用。约45.3%的受访者表示有意在未来使用自我药疗。社会阶层低与诉诸自我药疗的几率显著较高相关(优势比=3.32;p = 0.05)。结论:急性哮喘患儿抗生素使用情况显著,且很大一部分患儿未就诊。家庭社会阶层是采用自我药疗的独立预测因子。关键词:哮喘,发作,抗生素,埃及,儿童。
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引用次数: 0
Editorial 社论
IF 0.3 Pub Date : 2018-10-01 DOI: 10.21608/ejpa.2018.30632
Y. El-Gamal
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引用次数: 0
Is there a role for inhaled corticosteroids in early life wheeze? 吸入皮质类固醇在早期喘息中是否有作用?
IF 0.3 Pub Date : 2018-10-01 DOI: 10.21608/EJPA.2018.30633
E. Hossny
Upper respiratory tract infections account for more than 80% of wheezing episodes in young children. Most children wheeze only when they have upper respiratory tract infections, are usually non-atopic, and outgrow symptoms by 6 years of age. Yet, since preschool-age children have 6 to 10 upper respiratory tract infections each year, recurrent virus-induced wheezing is associated with considerable distress and use of health care services.
上呼吸道感染占幼儿喘息发作的80%以上。大多数儿童只有在患有上呼吸道感染时才会喘息,通常是非特应性的,到6岁时症状就会消失。然而,由于学龄前儿童每年有6至10例上呼吸道感染,复发性病毒引起的喘息与相当大的痛苦和使用保健服务有关。
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引用次数: 0
Preschool wheeze: pathogenetic factors 学龄前喘息:发病因素
IF 0.3 Pub Date : 2018-04-01 DOI: 10.21608/EJPA.2018.10372
E. Hossny
Definition and causes of wheeze Prevalence studies of recurrent wheeze in children under five have reported that one third of children in the US and Europe are affected, and rates and severity appear to be higher in developing countries. Episodic cough and wheezing is common in nonasthmatic children. Young children with multipletrigger wheeze are more likely to have asthma compared to those with episodic (viral) wheeze. Atopic features, family history, and association between wheeze and colds or other triggers such as cigarette smoke, allergens, and exercise help predicting asthma.
对五岁以下儿童复发性喘息的流行研究报告称,美国和欧洲有三分之一的儿童受到影响,发展中国家的发病率和严重程度似乎更高。发作性咳嗽和喘息在非哮喘儿童中很常见。与发作性(病毒性)喘息相比,患有多诱因喘息的幼儿更容易患哮喘。特应性特征、家族史以及喘息与感冒或其他触发因素(如吸烟、过敏原和运动)之间的联系有助于预测哮喘。
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引用次数: 1
期刊
Egyptian Journal of Pediatric Allergy and Immunology
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