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首页> 外文期刊>Allergy >Hyper‐IgE in the allergy clinic––when is it primary immunodeficiency?
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Hyper‐IgE in the allergy clinic––when is it primary immunodeficiency?

机译：过敏诊所中的超IgE - 初级免疫缺陷什么时候？

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摘要

Abstract The 2017 International Union of Immunological Societies ( IUIS ) classification recognizes 3 hyper‐IgE syndromes ( HIES ), including the prototypic Job's syndrome (autosomal dominant STAT 3‐loss of function) and autosomal recessive PGM 3 and SPINK 5 syndromes. Early diagnosis of PID can direct life‐saving or transformational interventions; however, it remains challenging owing to the rarity of these conditions. This can result in diagnostic delay and worsen prognosis. Within increasing access to “clinical‐exome” testing, clinicians need to be aware of the implication and rationale for genetic testing, including the benefits and limitations of current therapies. Extreme elevation of serum IgE has been associated with a growing number of PID syndromes including the novel CARD 11 and ZNF 341 deficiencies. Variable elevations in IgE are associated with defects in innate, humoral, cellular and combined immunodeficiency syndromes. Barrier compromise can closely phenocopy these conditions. The aim of this article was to update readers on recent developments at this important interface between allergy and immunodeficiency, highlighting key clinical scenarios which should draw attention to possible immunodeficiency associated with extreme elevation of IgE, and outline initial laboratory assessment and management.
机译：摘要2017年国际免疫科学协会（IUIS）分类识别3 Hyper-IgE综合征（HIES），包括原型作业综合征（常染色体占优势统计3次损失）和常染色体隐性PGM 3和Spink 5综合征。 PID的早期诊断可以直接救生或转型干预措施;然而，由于这些条件的罕见，它仍然挑战。这可能导致诊断延迟和恶化预后。在增加对“临床 - 外壳”测试的进入范围内，临床医生需要了解遗传测试的含义和理由，包括当前疗法的益处和局限性。血清IgE的极端升高与越来越多的PID综合征有关，包括新型卡11和ZNF 341缺陷。 IgE中的可变升高与先天性，体液，细胞和组合免疫缺陷综合征的缺陷有关。屏障妥协可以密切地对这些条件感染。本文的目的是更新读者在过敏和免疫缺陷之间的这种重要界面上的最新发展，突出应注意应注意与IGE极端高度相关的可能免疫缺陷，以及概述初始实验室评估和管理。

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  • 来源
    《Allergy》 |2018年第11期|共15页
  • 作者

    Ponsford Mark J.; Klocperk Adam; Pulvirenti Federica; Dalm Virgil A. S. H.; Milota Tomas; Cinetto Francesco; Chovancova Zita; Rial Manuel J.; Sediva Anna; Litzman Jiri; Agostini Carlo; Hagen Martin; Quinti Isabella; Jolles Stephen;

  • 作者单位

    Immunodeficiency Centre for WalesCardiff UK;

    Department of ImmunologyCharles University and Motol University HospitalPrague Czech Republic;

    Department of Molecular MedicineSapienza University of RomeRome Italy;

    Department of Internal MedicineErasmus MCRotterdam The Netherlands;

    Department of ImmunologyCharles University and Motol University HospitalPrague Czech Republic;

    Department of MedicineTreviso HospitalPadova Italy;

    Department of Clinical Immunology and AllergologySt. Anne's University Hospital in BrnoCzech;

    Department of AllergyUniversity Hospital Jiménez Díaz FoundationMadrid Spain;

    Department of ImmunologyCharles University and Motol University HospitalPrague Czech Republic;

    Department of Clinical Immunology and AllergologySt. Anne's University Hospital in BrnoCzech;

    Department of MedicineTreviso HospitalPadova Italy;

    Department of Internal MedicineErasmus MCRotterdam The Netherlands;

    Department of Molecular MedicineSapienza University of RomeRome Italy;

    Immunodeficiency Centre for WalesCardiff UK;

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  • 正文语种 eng
  • 中图分类 医学免疫学;
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