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International Guideline on the Diagnosis and Management of Pediatric Patients With Hereditary Angioedema. 儿童遗传性血管性水肿的国际诊断和治疗指南。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-30 DOI: 10.1111/all.70207
Henriette Farkas,Inmaculada Martinez-Saguer,Konrad Bork,Anastasios E Germenis,Anete S Grumach,Hanga Réka Horváth,Andrea Luczay,Andrea Zanichelli,Markus Magerl,Stephen Betschel,Emel Aygören-Pürsün,Jonathan A Bernstein,Isabelle Boccon-Gibod,Teresa Caballero,Mauro Cancian,Sandra Christiansen,Danny M Cohn,Francisco Contreras,Sansanee Craig,Camelia Isaic,Ankur Jindal,Constance H Katelaris,Hilary J Longhurst,Andrew MacGinnitie,Jonny Peter,Grzegorz Porebski,Avner Reshef,Dinh Van Nguyen,Bruce Zuraw,Anthony J Castaldo,Henrik Balle Boysen,Timothy Craig,
Hereditary angioedema (HAE) with C1 inhibitor deficiency is a rare disease characterized by unpredictable episodes of tissue swelling (angioedema), which, in most cases, occur first under the age of 18 years, and entail a significant burden of disease not only for the patients but also for their families. Clinical symptoms of HAE are not specific, which may cause difficulties in differential diagnosis. Additionally, if not appropriately treated, HAE attacks can be life-threatening. The international HAE guidelines published so far have focused mainly on adults. A guideline that refers to the age-specific characteristics of pediatric patients, both in terms of diagnosis and management, was therefore needed. The International Steering Committee and Taskforce developed recommendations and provided evidence-based grading based on expert opinion and strength of evidence. Recommendations were presented to, discussed, and electronically voted by healthcare professionals during the 14th C1 Inhibitor Deficiency and Angioedema Workshop in Budapest, Hungary, 2025. This international guideline will ensure early diagnosis, standardized and up-to-date treatment, and promote the availability of effective therapies for all pediatric patients affected with this rare disease. It also draws attention to the importance of establishing HAE centers and registries, which solicit specialist care and research of the disease.
遗传性血管性水肿(HAE)伴C1抑制剂缺乏症是一种罕见的疾病,其特征是不可预测的组织肿胀(血管性水肿)发作,在大多数情况下,首先发生在18岁以下,不仅对患者而且对其家庭都带来了重大的疾病负担。HAE的临床症状不特异性,这可能会给鉴别诊断带来困难。此外,如果治疗不当,HAE发作可能危及生命。迄今为止发表的国际HAE指南主要针对成人。因此,在诊断和管理方面,需要制定一项涉及儿科患者年龄特异性特征的指南。国际指导委员会和工作组根据专家意见和证据力度提出了建议并提供了基于证据的分级。在2025年匈牙利布达佩斯举行的第14届C1抑制剂缺乏和血管性水肿研讨会上,医疗保健专业人员提出了建议,并进行了讨论和电子投票。该国际指南将确保早期诊断、标准化和最新治疗,并促进所有患有这种罕见疾病的儿科患者获得有效治疗。它还提请注意建立HAE中心和登记的重要性,这些中心和登记要求对该疾病进行专家护理和研究。
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引用次数: 0
Decoding Resistin-Like Molecule Beta (RELMβ): A Key Modulator in Food Allergy. 解码抵抗素样分子β (RELMβ):食物过敏的关键调节因子。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-28 DOI: 10.1111/all.70238
Beatriz Moya,Carmen Riggioni,Beatriz Cabanillas
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引用次数: 0
Algorithms in Allergy: Molecular Allergology in the Context of Animal Allergy. 变态反应的算法:动物变态反应的分子变态反应学。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-26 DOI: 10.1111/all.70234
Christiane Hilger,Bulent E Sekerel,Marianne van Hage,Wolfgang Hemmer,Jon R Konradsen
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引用次数: 0
Clinical and Mucosal Transcriptomic Profiling of Adult Food Protein-Induced Enterocolitis Syndrome. 成人食物蛋白诱导的小肠结肠炎综合征的临床和粘膜转录组学分析。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-26 DOI: 10.1111/all.70233
Sho Watanabe,Ayako Sato,Hiroto Seki,Mariko Negi,Tsunehito Yauchi,Kiwako Yamamoto-Hanada,Tatsuki Fukuie,Yukihiro Ohya,Ichiro Nomura
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引用次数: 0
Elevated Tryptase and Hereditary Alpha Tryptasemia in Drug Hypersensitivity and Desensitization. 药物过敏和脱敏的胰蛋白酶升高和遗传性α -胰蛋白酶血症。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-25 DOI: 10.1111/all.70226
Marina Labella,Kathleen Marquis,Olivia McWhorter,Kylie Marie Besz,Donna Marie Lynch,Mariana Castells
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引用次数: 0
Early Risk Assessment and Recognition of Allergies in Children: Rationale, Methodology, and Proposed Algorithms. 儿童过敏的早期风险评估和识别:基本原理、方法和提出的算法。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-25 DOI: 10.1111/all.70224
E Hamelmann,B Schaub,K Beyer,K Blümchen,M Gerstlauer,M V Kopp,L Lange,S Lau,N Maison,H Ott,S M Schmidt,T Spindler,C Traidl-Hoffmann,C Vogelberg
BACKGROUNDAtopic diseases-including atopic dermatitis (AD), food allergy (FA), allergic rhinitis (AR), and asthma-are the most common chronic conditions in childhood and adolescence, affecting up to 30% of the global population. In Germany alone, more than 2.1 million children and adolescents are affected. These conditions frequently coexist and share common genetic, environmental, dietary, and microbial risk factors.METHODSA comprehensive literature review was conducted, and a multidisciplinary Task Force of the German Society for Pediatric Allergology and Environmental Medicine (GPA) and the German Society for Allergology and Clinical Immunology (DGAKI) developed consensus-based algorithms for early risk assessment and recognition of allergies in children in already existing preventive medical check-ups. The approach emphasizes stepwise risk assessment, including family history, environmental exposures, and early clinical signs such as recurrent wheezing.RESULTSFor children identified as at risk for or with early clinical signs of atopy, targeted diagnostic steps are recommended that follow the national/international guidelines for the management of the suspected atopic disease. This may include general and specific recommendations for nutrition, measurement of specific sensitization and selected biomarkers, if indicated and recommended by the guidelines. Routine allergy testing in asymptomatic children is not recommended. The algorithms are designed to be embedded into routine pediatric check-ups, enabling systematic and early identification of children at increased risk or with early clinical signs of atopic diseases. Early recognition and management can reduce disease severity, improve quality of life, and decrease healthcare costs.CONCLUSIONStructured programs for early risk assessment and recognition of allergies in children are currently lacking but can provide substantial clinical and economic benefits. Integration into routine pediatric preventive examinations, supported by standardization, interdisciplinary collaboration, and sustainable funding, offers a promising strategy to improve long-term outcomes for affected children and their families.
背景:过敏性疾病——包括特应性皮炎(AD)、食物过敏(FA)、过敏性鼻炎(AR)和哮喘——是儿童和青少年最常见的慢性疾病,影响全球30%的人口。仅在德国,就有210多万儿童和青少年受到影响。这些疾病经常共存,并具有共同的遗传、环境、饮食和微生物风险因素。方法进行全面的文献综述,由德国儿科过敏症与环境医学学会(GPA)和德国过敏症与临床免疫学学会(DGAKI)组成的多学科工作组制定基于共识的算法,用于在现有的预防性医学检查中对儿童过敏进行早期风险评估和识别。该方法强调逐步风险评估,包括家族史、环境暴露和早期临床症状,如复发性喘息。结果对于确定有特应性风险或有早期临床症状的儿童,建议采取有针对性的诊断步骤,并遵循国家/国际特应性疾病管理指南。这可能包括一般和特定的营养建议,特定致敏性的测量和选定的生物标志物,如果指南有指示和推荐的话。不建议对无症状儿童进行常规过敏试验。这些算法旨在嵌入到儿科常规检查中,能够系统和早期识别风险增加或具有特应性疾病早期临床体征的儿童。早期识别和管理可以降低疾病严重程度,改善生活质量,降低医疗费用。结论:目前缺乏儿童过敏早期风险评估和识别的结构化方案,但可以提供实质性的临床和经济效益。在标准化、跨学科合作和可持续供资的支持下,将其纳入儿科常规预防性检查是一项有希望改善受影响儿童及其家庭长期结果的战略。
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引用次数: 0
Postbiotics and Nicotinamide Utilize Distinct Mechanisms to Improve Skin Barrier Integrity, Inflammation, and Keratinocyte Differentiation. 后生物制剂和烟酰胺利用不同的机制来改善皮肤屏障完整性、炎症和角质细胞分化。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-23 DOI: 10.1111/all.70225
Yagiz Pat,Duygu Yazici,Huseyn Babayev,Sena Ardicli,Xiangting Bu,Sheri Simmons,Anthony Almada,Christine Avena,Tye Jensen,Raja Dhir,Patrick Westermann,Asuncion Garcia-Sanchez,Manru Li,Ozge Ardicli,Can Zeyneloglu,Marco Pane,Angela Amoruso,Christoph Messner,Ismail Ogulur,Yasutaka Mitamura,Mubeccel Akdis,Cezmi A Akdis
The modulation of immune responses and tissue regeneration by postbiotics is a rapidly advancing area in skin care. Here, we show that whole-cell postbiotics derived from Bifidobacterium breve, Limosilactobacillus reuteri, and Ligilactobacillus salivarius, along with nicotinamide (NAM), enhance keratinocyte growth, differentiation, and skin epithelial barrier integrity in ex vivo human skin, as determined by electrical impedance spectroscopy (EIS), multiomics, and machine learning. B. breve promoted keratinocyte differentiation and suppressed inflammatory pathways, while L. reuteri and L. salivarius primarily reduced inflammatory pathways. Although NAM downregulated keratinocyte differentiation, it exerted anti-inflammatory effects. Machine learning analyses linked EIS changes to certain genes, highlighting strain-specific mechanisms. In addition, B. breve, L. reuteri, and NAM mitigated a common skin cleanser-induced skin epithelial damage, further supporting their therapeutic potential. In conclusion, integrating skin barrier measurements with omics and machine learning enabled the dissection of essential anti-inflammatory and keratinocyte differentiation mechanisms and genes of a strengthened skin barrier.
通过后生物制剂调节免疫反应和组织再生是皮肤护理中一个快速发展的领域。通过电阻抗谱(EIS)、多组学和机器学习,研究人员发现,从短双歧杆菌、罗伊氏乳杆菌和唾液脂乳杆菌中提取的全细胞后生制剂与烟酰胺(NAM)一起,可以增强离体人体皮肤中角质细胞的生长、分化和皮肤上皮屏障的完整性。短芽孢杆菌促进角质细胞分化并抑制炎症通路,而罗伊氏乳杆菌和唾液乳杆菌主要抑制炎症通路。NAM虽然下调角质细胞分化,但具有抗炎作用。机器学习分析了EIS变化与某些基因的联系,突出了菌株特异性机制。此外,短芽胞杆菌、罗伊氏乳杆菌和NAM减轻了一种常见的皮肤清洁剂引起的皮肤上皮损伤,进一步支持了它们的治疗潜力。总之,将皮肤屏障测量与组学和机器学习相结合,可以解剖增强皮肤屏障的基本抗炎和角质细胞分化机制和基因。
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引用次数: 0
Allergic Sensitization to Inhalant Allergens in the Upper Respiratory Tract-the B Cell Side. 上呼吸道吸入性过敏原的过敏致敏- B细胞侧。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-23 DOI: 10.1111/all.70229
Ola Grimsholm,Mohammed Zghaebi,Bita Hambrecht,Tanja Kalic,Christopher C Udoye,Rudolf Manz,Barbara Bohle,Katarzyna M Sitnik,Julia Eckl-Dorna,Heimo Breiteneder
Allergic diseases are on the rise worldwide, driven by respiratory epithelial barrier dysfunction that promotes sensitization to inhalant allergens such as pollen, dust mites, pet dander, and fungal spores. These antigens trigger IgE-mediated immune responses that lead to diseases such as allergic rhinitis (AR) and asthma. B cells play a central role by producing allergen-specific IgE, presenting antigens, releasing cytokines, and forming memory B cells (MBCs). Their differentiation into IgE-secreting plasma cells (PCs) mainly relies on T cell help, germinal center (GC) reactions, and/or extrafollicular responses and class switch recombination (CSR), which makes them important therapeutic targets. The nasal mucosa, as the first point of contact for allergens, acts both as a barrier and as an immunological site. In AR, IL-13-driven goblet cell hyperplasia and overproduction of mucus compromise the integrity of the barrier. Although the nasal microbiome can influence the immune response, its role in atopy remains unclear. Local B cell activity, including extrafollicular IgE production and ectopic GCs, enhances mucosal immunity. Epithelial cells detect allergens via pattern recognition receptors (PRRs) and release alarmins (IL-25, IL-33, TSLP), which can trigger type 2 inflammation. Proteases from allergens such as house dust mites (HDM) disrupt epithelial junctions, while pollutants, smoke, microplastics, and allergen-derived metabolites further modulate immune activation. Allergens are transported to the lymph nodes by the passive flow to follicular dendritic cells (FDCs) or by active uptake by interferon regulatory factor (IRF) 4-dependent conventional type 2 DCs, which activate T follicular helper (TFH) cells to drive IgE responses. Advanced lymphoid organoids that mimic the microenvironment of GCs offer promising models for the study of allergic sensitization but require improved standardization.
过敏性疾病在世界范围内呈上升趋势,这是由呼吸道上皮屏障功能障碍引起的,它促进了对吸入性过敏原(如花粉、尘螨、宠物皮屑和真菌孢子)的致敏。这些抗原触发ige介导的免疫反应,导致过敏性鼻炎(AR)和哮喘等疾病。B细胞通过产生过敏原特异性IgE、呈递抗原、释放细胞因子和形成记忆B细胞(MBCs)发挥核心作用。它们向ige分泌浆细胞(PCs)的分化主要依赖于T细胞帮助、生发中心(GC)反应和/或滤泡外反应和类开关重组(CSR),这使它们成为重要的治疗靶点。鼻黏膜作为过敏原的第一个接触点,既是屏障又是免疫部位。在AR中,il -13驱动的杯状细胞增生和粘液的过量产生损害了屏障的完整性。虽然鼻腔微生物组可以影响免疫反应,但其在特应性反应中的作用尚不清楚。局部B细胞活性,包括滤泡外IgE产生和异位GCs,增强粘膜免疫。上皮细胞通过模式识别受体(PRRs)检测过敏原并释放警报器(IL-25、IL-33、TSLP),从而引发2型炎症。来自屋尘螨(HDM)等过敏原的蛋白酶破坏上皮连接,而污染物、烟雾、微塑料和过敏原衍生的代谢物进一步调节免疫激活。过敏原通过被动流向滤泡树突状细胞(fdc)或通过干扰素调节因子(IRF) 4依赖的传统2型树突状细胞的主动摄取被转运到淋巴结,后者激活T滤泡辅助细胞(TFH)来驱动IgE反应。模拟GCs微环境的高级淋巴样器官为研究过敏性致敏提供了有希望的模型,但需要改进标准化。
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引用次数: 0
Targeting Immunologic Pathways in Eosinophilic Granulomatosis With Polyangiitis: Translating Emerging Evidence Into Clinical Practice. 嗜酸性肉芽肿伴多血管炎的靶向免疫途径:将新出现的证据转化为临床实践。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-23 DOI: 10.1111/all.70215
Harold Wilson-Morkeh,Lior Seluk,Philipp Bosch,Carolina Aguiar,Jens Thiel,Bernhard Hellmich,Michael E Wechsler,Salman Siddiqui
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and potentially life-threatening systemic, inflammatory disease with multi-organ manifestations, variable presentation and complex pathology. Multiple interconnected immunological pathways are implicated in EGPA pathology, including a type-2 immune response driving predominantly eosinophilic inflammation, B-cell mediated autoimmunity, neutrophil activation, and the generation of pathogenic anti-neutrophil cytoplasmic antibodies, all of which can contribute to tissue/organ damage. High-dose glucocorticoids are the mainstay treatment for EGPA, but over the past two decades the development of biologic treatments targeting interleukin (IL)-5, eosinophils and B-cells has revitalized the treatment landscape. Mepolizumab, a humanized monoclonal antibody that specifically targets IL-5, and benralizumab, which targets the IL-5 receptor (IL-5Rα), are both approved for the treatment of patients with non-severe relapsing or refractory EGPA. In Phase III trials, these biologics have demonstrated favorable safety profiles and efficacy, with treatment leading to remission induction, remission maintenance, and oral glucocorticoid sparing benefits. However, as understanding of the full complexity of EGPA pathogenesis improves, new treatment targets are emerging. Consequently, understanding key pathogenic mechanisms at the patient level, enabling a more tailored treatment approach, is an important goal for future research.
嗜酸性肉芽肿病合并多血管炎(EGPA)是一种罕见且可能危及生命的全身性炎症性疾病,具有多器官表现、多变的表现和复杂的病理。多种相互关联的免疫途径与EGPA病理有关,包括2型免疫反应,主要驱动嗜酸性粒细胞炎症,b细胞介导的自身免疫,中性粒细胞活化和致病性抗中性粒细胞细胞质抗体的产生,所有这些都可能导致组织/器官损伤。高剂量糖皮质激素是EGPA的主要治疗方法,但在过去二十年中,针对白细胞介素(IL)-5、嗜酸性粒细胞和b细胞的生物治疗的发展使治疗领域重新焕发活力。Mepolizumab是一种特异性靶向IL-5的人源化单克隆抗体,而benralizumab是靶向IL-5受体(IL-5Rα)的,两者都被批准用于治疗非严重复发或难治性EGPA患者。在III期试验中,这些生物制剂已显示出良好的安全性和有效性,治疗可诱导缓解,维持缓解,并可节省口服糖皮质激素。然而,随着对EGPA发病机制的全面复杂性的了解的提高,新的治疗靶点正在出现。因此,了解患者水平的关键致病机制,使更有针对性的治疗方法,是未来研究的重要目标。
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引用次数: 0
An Old Story Back: Human Milk Antibodies' Protective Roles Against Allergy Development. 一个古老的故事:人乳抗体对过敏发展的保护作用。
IF 12.4 1区 医学 Q1 ALLERGY Pub Date : 2026-01-22 DOI: 10.1111/all.70218
Courtney M Jackson,Maria Carmen Collado,David C Dallas,Richard A Insel,Andrew J Macpherson,Debra J Palmer,Antti E Seppo,Valerie Verhasselt,Kirsi M Järvinen
Human milk is a rich source of immunomodulatory factors that influence the development of the infant immune system, including susceptibility to allergic diseases. Among these components, milk antibodies have been extensively studied for their role in protecting against infections; however, their potential contribution to allergy prevention may be equally important. The mechanisms of protection include allergen exclusion, enhanced and targeted antigen presentation, immune modulation via shaping of the infant gut microbiome, and direct regulation of gut immune responses. This review summarizes current evidence on the secretion of human milk antibodies, highlights what is known and what remains unclear about their role in allergy prevention and outlines the need for further research to develop antibody-based strategies for early allergy prevention.
母乳是影响婴儿免疫系统发育的免疫调节因子的丰富来源,包括对过敏性疾病的易感性。在这些成分中,牛奶抗体因其在预防感染方面的作用而被广泛研究;然而,它们对预防过敏的潜在贡献可能同样重要。保护机制包括过敏原排斥,增强和靶向抗原呈递,通过塑造婴儿肠道微生物组进行免疫调节,以及直接调节肠道免疫反应。这篇综述总结了目前关于人乳抗体分泌的证据,强调了它们在过敏预防中的已知和不清楚的作用,并概述了进一步研究开发基于抗体的早期过敏预防策略的必要性。
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引用次数: 0
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