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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >X-Linked Agammaglobulinaemia: Outcomes in the modern era
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X-Linked Agammaglobulinaemia: Outcomes in the modern era

机译：X-Linked Agammaglobulinemia：现代时代的结果

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Abstract Colonel Ogden Bruton reported X-Linked Agammaglobulinaemia in 1952 and treated the child with replacement immunoglobulin therapy. Over 60years later, the treatment for XLA has largely remained unchanged. Replacement immunoglobulin lacks the isotypes IgA and IgM, leading to concerns that patients continue to experience recurrent sinopulmonary tract infections and be at increased risk of bronchiectasis. There is potential hope of earlier diagnosis with newborn screening, and a potential cure for these patients, in the form of gene therapy. However, it is first necessary to evaluate current management and outcomes to aid decisions regarding further research and clinical trials. This article reviews current management and outcomes of XLA, whilst identifying gaps in our knowledge base that may need answering before we proceed with novel diagnostic methods and treatment for XLA. Highlights ? Current therapy is likely to be limited in its ability to prevent complications. ? Of particular concerns is the development of bronchiectasis. ? Rates of bronchiectasis are significant in XLA despite current optimal therapy. ? Novel management options are possible, notably newborn screening and gene therapy. ? More data are needed to fully evaluate current outcomes in XLA.
机译：Abstract ogden Bruton在1952年报道了X-Linked Agammaglobulina血症，并用替代免疫球蛋白疗法治疗儿童。以后60年以后，XLA的治疗在很大程度上保持不变。替代免疫球蛋白缺乏同种型IgA和IgM，导致患者持续经历复发性的中间肺部感染，并且处于支气管扩张的风险增加。潜在的诊断潜在的诊断与新生儿筛查，以及这些患者的潜在治疗，以基因治疗的形式。但是，首先是有必要评估当前的管理和结果，以帮助有关进一步研究和临床试验的决策。本文审查了XLA的当前管理和结果，同时在我们继续回答的知识库中识别差距，在我们继续进行新的诊断方法和XLA治疗之前。强调 ？目前的疗法可能受到预防并发症的能力的限制。还特别令人担忧是支气管扩张的发展。还尽管有目前的最佳疗法，XLA中的支气管扩张率显着。还新的管理选择是可能的，特别是新生儿筛查和基因治疗。还需要更多数据来完全评估XLA中的当前结果。

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