Isao Suzaki
Isao Suzaki
Purpose of review: Chronic rhinosinusitis (CRS) is a heterogeneous condition, so personalized treatment based on each patient's pathophysiology is essential, rather than a one-size-fits-all approach. Drug therapy for CRS ...
Paucity of gastrointestinal plasma cells in common variable immunodeficiency [0.03%]
常见变量免疫缺陷患者胃肠道浆细胞缺乏
Jan Willem N Marsden,Miangela M Laclé,Mirjam Severs et al.
Jan Willem N Marsden et al.
Purpose of review: Common variable immunodeficiency enteropathy (CVID-E) is a noninfectious complication of CVID caused by chronic inflammation of the gastrointestinal (GI) tract. Based on literature, a paucity or lack of...
Update on hereditary C1q deficiency: pathophysiology, clinical presentation, genotype and management [0.03%]
补体C1Q遗传性缺陷的最新研究进展：发病机制、临床表现、基因型和治疗管理
Helena Buso,Clément Triaille,Aisling M Flinn et al.
Helena Buso et al.
Purpose of review: C1q deficiency is a rare inborn error of immunity characterized by susceptibility to severe infections and profound immune dysregulation, with a systemic lupus erythematosus-like phenotype. The manageme...
STAT1 and STAT3 gain of function: clinically heterogenous immune regulatory disorders [0.03%]
STAT1和STAT3功能增强：异质性免疫调节障碍
Peter Olbrich,Alexandra F Freeman
Peter Olbrich
Purpose of review: The identification of STAT1 gain-of-function (GOF) in 2011 and STAT3 GOF in 2014 has advanced our understanding of the host immunity along the JAK/STAT pathway and allowed targeted treatment approaches....
Expanding the spectrum of IPEX: from new clinical findings to novel treatments [0.03%]
从新的临床发现到新型治疗方法：IPEX综合征的研究进展
Marta Voarino,Filippo Consonni,Eleonora Gambineri
Marta Voarino
Purpose of review: This review aims to provide an overview of recent research findings regarding immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, focusing on clinical and immunological nove...
New mechanisms in diisocyanate-mediated allergy/toxicity: are microRNAs in play? [0.03%]
二异氰酸酯介导过敏/毒性新机制：微小核糖核酸在起作用吗？
Chen-Chung Lin,Brandon F Law,Justin M Hettick
Chen-Chung Lin
Purpose of review: To describe recent findings of diisocyanate-mediated mechanisms in allergy and toxicology by addressing the role of microRNA (miR) in immune responses that may contribute to the development of occupatio...
Patrick Bez,Martina Ceraudo,Fabrizio Vianello et al.
Patrick Bez et al.
Purpose of review: The purpose of the review is to describe the most recent advancement in understanding of the pivotal role of autoimmune regulator ( AIRE ) gene expression in central and peripheral tolerance, and the im...
The biological basis for current treatment strategies for granulomatous disease in common variable immunodeficiency [0.03%]
常见变量免疫缺陷型肉芽肿病的当前治疗策略的生物学基础
Astrid C van Stigt,Giulia Gualtiero,Francesco Cinetto et al.
Astrid C van Stigt et al.
Purpose of review: The pathogenesis of granulomatous disease in common variable immunodeficiency (CVID) is still largely unknown, which hampers effective treatment. This review describes the current knowledge on the patho...
Hereditary angioedema due to C1-inhibitor deficiency: current therapeutic approaches [0.03%]
C1酯酶抑制剂缺乏所致遗传性血管水肿的当前治疗措施
Giulia Costanzo,Giada Sambugaro,Davide Firinu
Giulia Costanzo
Purpose of review: For decades, treatment options for hereditary angioedema (HAE) were limited by major adverse effects, insufficient efficacy, and difficult routes of administration. However, the growing body of knowledg...
Bridging the gap: neurodevelopmental disorder risks in inborn errors of immunity [0.03%]
从先天性免疫缺陷到神经发育障碍风险：建立桥梁关系
Devika Kurup,Amy M FitzPatrick,Aleksandra Badura et al.
Devika Kurup et al.
Purpose of review: The aim of this review is to examine published reports of neurodevelopmental phenotypes in patients with inborn errors of immunity (IEI). We briefly discuss potential interactions between the immune and...