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Autoimmune hepatitis in childhood: The role of genetic and immune factors 预览
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作者 Priscila Menezes Ferri Liu Débora Marques de Miranda +2 位作者 ElEonora DruvE TavarEs FagundEs Alexandre Rodrigues Ferreira Ana Cristina Simoes e Silva 《世界胃肠病学杂志:英文版(电子版)》 SCIE CAS 2013年第28期4455-4463,共9页
Autoimmune hepatitis (AIH) is a rare chronic inflammatory disease of the liver, which affects a group of patients who lost their immunological tolerance to antigens of the liver. It is clinically characterized by hyp... Autoimmune hepatitis (AIH) is a rare chronic inflammatory disease of the liver, which affects a group of patients who lost their immunological tolerance to antigens of the liver. It is clinically characterized by hypergammaglobulinemia, elevated liver enzymes, presence of autoantibodies and histological changes. Although being rare in children, it represents a serious cause of chronic hepatic disease that can lead to cirrhosis and hepatic failure. Clinical findings, exclusion of more common liver disorders and the detection of antibodies antinuclear antibodies, smooth muscle antibodies and anti-LKM1 are usually enough for diagnosis on clinical practice. The pathogenic mechanisms that lead to AIH remain obscure, but some research findings suggest the participation of immunologic and genetic factors. It is not yet knew the triggering factor or factors that stimulate inflammatory response. Several mechanisms proposed partially explain the immunologic findings of AIH. The knowledge of immune factors evolved might result in better markers of prognosis and response to treatment. In this review, we aim to evaluate the findings of research about genetic and immune markers and their perspectives of application in clinical practice especially in pediatric population. 展开更多
关键词 AUTOIMMUNE HEPATITIS GENETICS Clinical practice IMMUNOPHENOTYPE
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Current knowledge on esophageal atresia 预览 被引量:4
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作者 Paulo Fernando Martins Pinheiro Ana Cristina Simoes e Silva Regina Maria Pereira 《世界胃肠病学杂志:英文版》 SCIE CAS CSCD 2012年第28期3662-3672,共11页
食道的闭锁(EA ) 与或没有 tracheoesophageal 管(TEF ) 是食管的最普通的先天的异例。在以前的二十年观察的幸存的改进是 multifactorial 并且对在新生的特别护理,新生的麻醉, ventilatory 和营养的支持的进展大部分可归因,抗菌素... 食道的闭锁(EA ) 与或没有 tracheoesophageal 管(TEF ) 是食管的最普通的先天的异例。在以前的二十年观察的幸存的改进是 multifactorial 并且对在新生的特别护理,新生的麻醉, ventilatory 和营养的支持的进展大部分可归因,抗菌素,早外科的干预,外科的材料和技术。确实,死亡当前与共存被限制到那些盒子严重威胁生活的异例。EA 的诊断最通常在生活的开始的 24 h 期间被做,但是也可以胎儿地发生或可以被推迟。为 EA 和 TEF 的主要外科的修正是当严重畸形性不在时的最好的选择。没有理想的代替因为为有长差距的 EA 的病人的食管和最佳的外科的治疗仍然是争论的。主要复杂并发症在手术后的时期期间是胸的墙的吻合,胃食道的倒流,食道的 dysmotility,管复发,呼吸混乱和畸形的漏缝和狭窄。关于长期的结果和后续的数据为跟随 EA/TEF 修理的病人被限制。为复杂进化追随者 EA/TEF 修理的风险因素的决心可以断然影响长期的预测。大部分尚待关于这个条件被学习。这张手稿关于 EA 提供当前的知识的文学评论。 展开更多
关键词 食管 知识 闭锁 手术治疗 呼吸系统疾病 重症监护 营养支持 功能障碍
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