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Intravenous immunoglobulins in liver transplant patients: Perspectives of clinical immune modulation 被引量:7
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作者 Arno Kornberg 《World Journal of Hepatology》 CAS 2015年第11期1494-1508,共15页
Shortage of appropriate donor grafts is the foremost current problem in organ transplantation. As a logical consequence, waiting times have extended and pretransplant mortality rates were significantly increasing. The... Shortage of appropriate donor grafts is the foremost current problem in organ transplantation. As a logical consequence, waiting times have extended and pretransplant mortality rates were significantly increasing. The implementation of a priority-based liver allocation system using the model of end-stage liverdisease(MELD) score helped to reduce waiting list mortality in liver transplantation(LT). However, due to an escalating organ scarcity, pre-LT MELD scores have significantly increased and liver recipients became more complex in recent years. This has finally led to posttransplant decreasing survival rates, attributed mainly to elevated rates of infectious and immunologic complications. To meet this challenging development, an increasing number of extended criteria donor grafts are currently accepted, which may, however, aggravate the patients' infectious and immunologic risk profiles. The administration of intravenous immunoglobulins(IVIg) is an established treatment in patients with immune deficiencies and other antibody-mediated diseases. In addition, IVIg was shown to be useful in treatment of several disorders caused by deterioration of the cellular immune system. It proved to be effective in preventing hyperacute rejection in highly sensitized kidney and heart transplants. In the liver transplant setting, the administration of specific Ig against hepatitis B virus is current standard in post-LT antiviral prophylaxis. The mechanisms of action of IVIg are complex and not fully understood. However, there is increasing experimental and clinical evidence that IVIg has an immuno-balancing impact by a combination of immuno-supporting and immuno-suppressive properties. It may be suggested that, especially in the context of a worsening organ shortage with all resulting clinical implications, liver transplant patients should benefit from immuno-regulatory capabilities of IVIg. In this review, perspectives of immune modulation by IVIg and impact on outcome in liver transplant patients are described. 展开更多
关键词 INTRAVENOUS IMMUNOGLOBULINS Immunemodulation hyperimmunoglobulin Model of end-stageliver disease LIVER TRANSPLANTATION
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常染色体显性及隐性遗传高免疫球蛋白E综合征临床特征及基因分析 被引量:3
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作者 王珊 刘盈 +5 位作者 徐子刚 王召阳 焦磊 梁源 徐哲 马琳 《罕见病研究》 2022年第3期268-277,共10页
目的总结常染色体显性及隐性遗传高免疫球蛋白E综合征(HIES)患儿的临床特征及基因分析。方法收集于2013年1月至2021年12月在首都医科大学附属北京儿童医院皮肤科收治的经临床及基因共同确诊为HIES患儿,归纳一般资料并分析其临床特征和... 目的总结常染色体显性及隐性遗传高免疫球蛋白E综合征(HIES)患儿的临床特征及基因分析。方法收集于2013年1月至2021年12月在首都医科大学附属北京儿童医院皮肤科收治的经临床及基因共同确诊为HIES患儿,归纳一般资料并分析其临床特征和基因型特点,比较常染色体显性遗传HIES(AD⁃HIES)和常染色体隐性遗传HIES(AR⁃HIES)临床特点的异同。结果共收集确诊HIES患儿7例,其中AD⁃HIES 3例,AR⁃HIES 4例。男4例,女3例。所有患儿均存在反复发作的顽固性湿疹样皮疹、反复皮肤及肺部感染,以及血清IgE和嗜酸性粒细胞水平升高。AD⁃HIES与AR⁃HIES的不同点主要包括:3例AD⁃HIES患儿皮肤感染均以细菌感染为主要表现(如脓肿、脓疱疮),而4例AR⁃HIES患儿皮肤均以严重的病毒感染为主要表现(如寻常疣、传染性软疣);3例AD⁃HIES患儿均存在不同种类的肺部实质改变(如肺大疱、肺囊肿、肺不张),而4例AR⁃HIES患儿肺部均无类似改变;AR⁃HIES患儿中3/4存在过敏性疾病(包括哮喘、食物过敏等),而AD⁃HIES患儿均无过敏性疾病报道;免疫系统外表现方面,AD⁃HIES更易见鼻梁扁平、高腭弓等特殊面容,AR⁃HIES中有肿瘤发生情况而AD⁃HIES尚未见。AD⁃HIES主要由STAT3基因突变引起,AR⁃HIES主要由DOCK8基因突变引起,其中两例患儿分别存在DOCK8基因的c.1798⁃2A>T和c.874G>A为新发突变位点。结论对于临床表现为顽固性湿疹样皮疹、反复感染,伴血清IgE水平显著升高的患儿,需寻找早期HIES疑诊线索,尽早进行基因检测明确诊断及分类,以更好地长期管理,改善生存预后。 展开更多
关键词 高免疫球蛋白E综合征 STAT3基因 DOCK8基因 湿疹 原发性免疫缺陷病
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小儿高免疫球蛋白A血症及其相关疾病(附90例临床分析)
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作者 冯学斌 张秋业 杨锡强 《滨州医学院学报》 1989年第2期53-56,96-97,共6页
90例高IgA血症中,单纯高IgA血症56例,高IgA+IgG血症29例,高IgA+IgM血症5例。男:女为1.65:1,且以7岁以上儿童多见。其相关疾病为急/慢性肾小球肾炎(53.3%)、风湿热/风湿性心脏病(18.9%)、过敏性紫癜(6.7%)、其他自身免疫病(5.6%)、急... 90例高IgA血症中,单纯高IgA血症56例,高IgA+IgG血症29例,高IgA+IgM血症5例。男:女为1.65:1,且以7岁以上儿童多见。其相关疾病为急/慢性肾小球肾炎(53.3%)、风湿热/风湿性心脏病(18.9%)、过敏性紫癜(6.7%)、其他自身免疫病(5.6%)、急/慢性感染性疾病(12.2%)和其它(3.3%)。63.3%的病例血清IgA在2~3g/L。IgA增高与血沉、ASO、ClC的增高和C_3的变化有关联,部分患者外周血淋巴细胞计数降低,淋转率降低,这均提示高IgA血症可能是一种非特异性的体液免疫反应过高的表现。其机理推测可能与机体潜在的调节性T细胞功能失常致多克隆B细胞活化有关。 展开更多
关键词 高免疫球蛋白A血症 相关疾病 儿童
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Current Approach to Treatment of Juvenile Idiopathic Arthritis: Case Report of Hiperimmunglobulin E Syndrome Developed Juvenile Idiopathic Arthritis
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作者 Tuba Tülay Koca Aydin Arslan 《Open Journal of Rheumatology and Autoimmune Diseases》 2015年第3期62-67,共6页
Introduction: Juvenile idiopathic arthritis (JIA) represents a heterogeneous group of childhood chronic arthritic conditions. The pathogenesis of JIA remains incompletely understood. This disease can lead to a signifi... Introduction: Juvenile idiopathic arthritis (JIA) represents a heterogeneous group of childhood chronic arthritic conditions. The pathogenesis of JIA remains incompletely understood. This disease can lead to a significant morbidity including joint deformity, growth impairment and a persistence of active arthritis into adulthood. The past two decades have witnessed significant advances in treatment and improved outcomes for affected children. With the current use of biologics, more target-specific, better tolerated, safer and more effective treatments have become possible. However, continuing, comprehensive follow-up is needed to characterize the long-term effects of such treatments. Hyperimmunoglobulin E syndrome (hyper-IgE, or Job’s syndrome) is a rare immune deficiency characterized by high IgE levels, atopic chronic eczema, tendency towards re-current pyogenic infection, neutrophil chemotaxis disorder and varying T-cell function impairment. Case Report: The case of a 17-year-old male patient with hyper-IgE who develops the oligoarticular subtype of JIA over a period of four years is discussed. The course of JIA is unfavorable, causing severe deformity of numerous joints (left elbow, right 3rd metacarpophalangeal, left knee, right ankle) and a fungal infection scar on the left eye. Blood tests show an ESR of 89 mm/h, rheumatoid factor (RF) 8.3 IU/mL (0 - 20) and positive antinuclear antibody (ANA). To improve gait, corrective surgery is performed on the right ankle, followed by rehabilitation and physical therapy. Conclusion: Developments in the near future will be crucial for understanding JIA pathophysiology and improving treatment. 展开更多
关键词 Juvenile Idiopathic Arthritis hyperimmunoglobulin E Syndrome TREATMENT
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Passive antibody therapy in emerging infectious diseases
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作者 Xiaoming Yang 《Frontiers of Medicine》 SCIE CSCD 2023年第6期1117-1134,共18页
The epidemic of corona virus disease 2019(COVID-19)caused by severe acute respiratory syndrome Coronavirus 2 and its variants of concern(VOCs)has been ongoing for over 3 years.Antibody therapies encompassing convalesc... The epidemic of corona virus disease 2019(COVID-19)caused by severe acute respiratory syndrome Coronavirus 2 and its variants of concern(VOCs)has been ongoing for over 3 years.Antibody therapies encompassing convalescent plasma,hyperimmunoglobulin,and neutralizing monoclonal antibodies(mAbs)applied in passive immunotherapy have yielded positive outcomes and played a crucial role in the early COVID-19 treatment.In this review,the development path,action mechanism,clinical research results,challenges,and safety profile associated with the use of COVID-19 convalescent plasma,hyperimmunoglobulin,and mAbs were summarized.In addition,the prospects of applying antibody therapy against VOCs was assessed,offering insights into the coping strategies for facing new infectious disease outbreaks. 展开更多
关键词 SARS-CoV-2 COVID-19 convalescent plasma hyperimmunoglobulin neutralizing monoclonal antibodies
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成人高IgE综合征五例并文献复习 被引量:2
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作者 徐娜 范洪伟 +3 位作者 赵静 王澎 宋红梅 曾学军 《中华全科医师杂志》 2017年第12期955-960,共6页
回顾性分析2011年10月至2016年10月于北京协和医院住院的5例成人高IgE综合征(HIES)患者的临床资料,总结临床特点、感染特征及预后。患者均于出生后1岁以内起病,表现为自幼反复多部位感染5例、反复皮疹5例、骨骼改变4例。反复感染的... 回顾性分析2011年10月至2016年10月于北京协和医院住院的5例成人高IgE综合征(HIES)患者的临床资料,总结临床特点、感染特征及预后。患者均于出生后1岁以内起病,表现为自幼反复多部位感染5例、反复皮疹5例、骨骼改变4例。反复感染的平均病程为19.6年,以皮肤和呼吸道感染为主,1例出现罕见的右肾脓肿,其他还有化脓性中耳炎、骨髓炎。2例皮肤脓肿患者经抗感染和脓肿引流后缓解,另2例呼吸道感染、1例皮肤溃疡患者经抗感染后缓解。提示对于反复感染的成人患者,尤其合并骨骼异常、新生儿皮疹时,需警惕HIES可能,早期识别和诊断本病有助于及早针对性抗感染和改善预后;由于HIES可累及肾脏,应常规行泌尿系筛查。 展开更多
关键词 高IGE综合征 感染
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甲羟戊酸尿症1例报道并文献复习 被引量:1
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作者 李秀珍 刘丽 +4 位作者 程静 张文 周志红 毛晓健 黄永兰 《中国实用儿科杂志》 CSCD 北大核心 2012年第1期62-65,共4页
目的 报道1例甲羟戊酸尿症(MVA)患儿的临床特点、诊断、治疗及随访,以提高对该病的认识。方法 收集2006年7月于广州市妇女儿童医疗中心就诊的1例患儿的临床资料,进行分析并长期随防,复习文献总结分析其临床特点、诊疗及预后。结果 ... 目的 报道1例甲羟戊酸尿症(MVA)患儿的临床特点、诊断、治疗及随访,以提高对该病的认识。方法 收集2006年7月于广州市妇女儿童医疗中心就诊的1例患儿的临床资料,进行分析并长期随防,复习文献总结分析其临床特点、诊疗及预后。结果 患儿男,6岁6个月,以反复发热,伴有淋巴结、肝脾大为主要临床表现,血沉增快,超敏C反应蛋白、白细胞计数、血IgA均增高,尿液气相色谱质谱联用技术(GC/MS)检测显示甲羟戊酸排泄明显增高,给予辅酶Q10、维生素C及维生素E等治疗,随诊4年8个月,发热发作渐有减少,约1~2次/月,伴有紫癜样皮疹、关节痛反复发作,淋巴结、肝脾大无进行性加重,智力轻度低下。结论 临床对不明原因反复发热患者,尤其是合并有淋巴结、肝脾大或神经系统症状时需要警惕MVA可能,进一步做尿GC/MS分析等早期诊断,以避免误诊误治。本病目前主要是对症治疗,部分患儿发热随年龄增长有减少趋势。 展开更多
关键词 甲羟戊酸尿症 甲羟戊酸酶 高IgD综合征
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