Immunology Bieber T. Atopic dermatitis. N Engl J Med. 2008; 358: 1483-1494 View in Article Scopus (1419) PubMed Crossref Google Scholar Davidson W.F. Leung D.Y.M. Beck L.A. Berin C.M. Boguniewicz M. Busse W.W. et al. Report from the National Institute of Allergy and Infectious Diseases workshop on “Atopic dermatitis and the atopic march: Mechanisms and
0 Comments Dudeck A. Koberle M. Goldmann O. Meyer N. Dudeck J. Lemmens S. et al. Mast cells as protectors of health. J Allergy Clin Immunol. 2019; 144: S4-S18 IgE and mast cells in allergic disease. Nat Med. 2019; 18: 693-704 Cahill K.N. Katz H.R. Cui J. Lai J. Kazani S. Crosby-Thompson A. et al. KIT
0 Comments Chinn I.K. Eckstein O.S. Peckham-Gregory E.C. Goldberg B.R. Forbes L.R. Nicholas S.K. et al. Genetic and mechanistic diversity in pediatric hemophagocytic lymphohistiocytosis. Blood. 2019; 132: 89-100 Bousfiha A. Jeddane L. Picard C. Ailal F. Bobby Gaspar H. Al-Herz W. et al. The 2019 IUIS phenotypic classification for primary immunodeficiencies. J Clin Immunol. 2019; 38:
0 Comments Background Mast cells (MCs) have a profound impact on allergic asthma. Under such conditions, MCs undergo degranulation, resulting in the release of exceptionally large amounts of MC-restricted proteases. However, the role of these proteases in asthma is only partially understood. Objectives We sought to test our hypothesis that MC proteases can influence the functionality
0 Comments The α-gal epitope and the anti-Gal antibody in xenotransplantation and in cancer immunotherapy. Immunol Cell Biol. 2005; 83: 674-686 Koike C. Uddin M. Wildman D.E. Gray E.A. Trucco M. Starzl T.E. et al. Functionally important glycosyltransferase gain and loss during catarrhine primate emergence. Proc Natl Acad Sci U S A. 2007; 104: 559-564 Commins
0 Comments Anderson G. Lane P.J.L. Jenkinson E.J. Generating intrathymic microenvironments to establish T-cell tolerance. Nat Rev Immunol. 2007; 7: 954-963 Thymus organogenesis and molecular mechanisms of thymic epithelial cell differentiation. Semin Immunol. 2000; 12: 421-428 Nehls M. Pfeifer D. Schorpp M. Hedrich H. Boehm T. New member of the winged-helix protein family disrupted in mouse
0 Comments Arican O. Aral M. Sasmaz S. Ciragil P. Serum levels of TNF-alpha, IFN-gamma, IL-6, IL-8, IL-12, IL-17, and IL-18 in patients with active psoriasis and correlation with disease severity. Mediators Inflamm. 2005; 2005: 273-279 Johansen C. Funding A.T. Otkjaer K. Kragballe K. Jensen U.B. Madsen M. et al. Protein expression of TNF-alpha in psoriatic
0 Comments T cells are the predominant, but not the only lymphocyte subpopulation within skin. Apart from a few reports on B cells, 1 Nihal M. Mikkola D. Wood G.S. Detection of clonally restricted immunoglobulin heavy chain gene rearrangements in normal and lesional skin: analysis of the B cell component of the skin-associated lymphoid tissue and
0 Comments Background Currently, there are no approved therapies to treat congenital athymia, a condition of immune deficiency resulting in high early mortality due to infection and immune dysregulation. Multiple syndromic conditions, such as complete DiGeorge syndrome, 22q11.2 deletion syndrome, CHARGE (coloboma, heart defects, choanal atresia, growth or mental retardation, genital hypoplasia, and ear anomalies and/or
0 Comments The airway epithelium plays a central role in the initiation and perpetuation of type 2 (T2) inflammation in eosinophilic chronic rhinosinusitis (CRS) with nasal polyps (CRSwNP) and aspirin-exacerbated respiratory disease (AERD). T2 cytokine–driven airway epithelial remodeling leads to overexpression of proinflammatory products, loss of protective mucosal factors, and dysregulated antiviral programs that sustain tissue
0 Comments Mast cells (MCs) are considered the main effectors in allergic reactions and well known for their contribution to the pathogenesis of various inflammatory diseases, urticaria, and mastocytosis. To study their functions in vitro, human primary MCs are isolated directly from several tissues or differentiated from hematopoietic progenitors. However, these techniques bear several disadvantages and challenges
0 Comments Background Allogeneic hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis (HLH) disorders is associated with substantial morbidity and mortality. Objective The effect of conditioning regimen groups of varying intensity on outcomes after transplantation was examined to identify an optimal regimen or regimens for HLH disorders. Methods We studied 261 patients with HLH disorders transplanted between 2005
0 Comments Background Eosinophilic esophagitis (EoE) is a histologically “patchy” disease with uneven eosinophil distribution along the esophagus, posing a dilemma for histologically analyzing endoscopic biopsy samples, especially when biopsy samples are limited to only the distal esophagus. Objective We investigated whether molecular mRNA profiling of a distal esophageal biopsy sample predicts eosinophilia in the proximal
0 Comments Background B-cell affinity maturation in germinal center relies on regulated actin dynamics for cell migration and cell-to-cell communication. Activating mutations in the cytoskeletal regulator Wiskott-Aldrich syndrome protein (WASp) cause X-linked neutropenia (XLN) with reduced serum level of IgA. Objective We investigated the role of B cells in XLN pathogenesis. Methods We examined B cells
0 Comments Background Development of a diverse T-cell receptor β (TRB) repertoire is associated with immune recovery following hematopoietic cell transplantation (HCT) for severe combined immunodeficiency (SCID). High-throughput sequencing of the TRB repertoire allows evaluation of clonotype dynamics during immune reconstitution. Objectives We investigated whether longitudinal analysis of the TRB repertoire would accurately describe T-cell receptor
0 Comments Footnotes This study was supported by the Division of Emergency Medicine, Cincinnati Children’s Hospital Medical Center , Cincinnati, Ohio. The project described was supported by the National Center for Advancing Translational Sciences of the National Institutes of Health (NIH) (award no. 2UL1TR001425-05A1 ). The content is solely the responsibility of the authors and does
0 Comments Key words Autoinflammatory syndromes (ASs) cause systemic inflammation and tissue damage through aberrant activation of the innate immune system. In recent years a growing number of ASs have been attributed to constitutive activation and hyperactivation of innate immune signaling complexes known as inflammasomes. Inflammasomes are multimeric signaling complexes assembled following cytosolic detection of pathogens
0 Comments Fokkens W.J. Lund V.J. Hopkins C. Hellings P.W. Kern R. Reitsma S. et al. European position paper on rhinosinusitis and nasal polyps, 2020. Rhinology. 2020; 58: 1-464 Bachert C. Marple B. Schlosser R.J. Hopkins C. Schleimer R.P. Lambrecht B.N. et al. Adult chronic rhinosinusitis. Nat Rev Dis Primers. 2020; 6: 86 Hastan D. Fokkens
0 Comments Footnotes Supported by AbbVie Inc . The design, study conduct, analysis, and financial support for AD Up were provided by AbbVie. AbbVie participated in the interpretation of data, review, and approval of the manuscript. All authors had full access to all the data in the study and had final responsibility for the decision to
0 Comments Introduction Proteasome-associated autoinflammatory syndrome (PRAAS) represents any autoinflammatory disease that is characterized by multisystem inflammation caused by genomic alterations in various proteasome subunits. 1 Agarwal A.K. Xing C. DeMartino G.N. Mizrachi D. Hernandez M.D. Sousa A.B. et al. PSMB8 encoding the β5i proteasome subunit is mutated in joint contractures, muscle atrophy, microcytic anemia, and
0 Comments Chemical itch is transmitted to the spinal cord by various chemical mediators through C-pruriceptors expressing Mas-related GPR A3 (MrgprA3), natriuretic peptide B (Nppb), and gastrin-releasing peptide (GRP) at the peripheral level. 13 Han L. Ma C. Liu Q. Weng H.J. Cui Y. Tang Z. et al. A subpopulation of nociceptors specifically linked to itch.
0 Comments Roughly one-third of the global population is suffering from allergic hypersensitivity disorders, according to recent estimations. 1 Allergic diseases and asthma: a global public health concern and a call to action. For many patients, allergies are associated with a marked reduction in physical and mental well-being and lead to a significant loss in quality
0 Comments Background Acute increases of ≥20% + 2 ng/mL (20 + 2 rule) over basal serum tryptase (BST) is the recommended threshold supporting a clinical diagnosis of anaphylaxis. Prospective studies have demonstrated high sensitivity for this algorithm after parenteral exposure, but specificity has not been evaluated. Objective We sought to define a serum tryptase change that distinguishes
0 Comments Busse W.W. Lemanske Jr., R.F. Asthma. N Engl J Med. 2001; 344: 350-362 View in Article Scopus (1648) PubMed Crossref Google Scholar Boonpiyathad T. Sözener Z.C. Satitsuksanoa P. Akdis C.A. Immunologic mechanisms in asthma. Semin Immunol. 2019; 46101333 View in Article Scopus (87) PubMed Crossref Google Scholar Moro K. Yamada T. Tanabe M. Takeuchi
0 Comments Turvey S.E. Bonilla F.A. Junker A.K. Primary immunodeficiency diseases: a practical guide for clinicians. Postgrad Med J. 2009; 85: 660-666 Tangye S.G. Al-Herz W. Bousfiha A. Chatila T. Cunningham-Rundles C. Etzioni A. et al. Human inborn errors of immunity: 2019 update on the classification from the International Union of Immunological Societies Expert Committee. J
0 Comments Background Fixed airflow obstruction (FAO) in asthma, particularly in nonsmokers, is generally believed to be caused by airway remodeling. However, parenchymal destruction may also contribute to FAO and longitudinal decline in forced expiratory volume in 1 second (FEV1). Objectives To evaluate parenchymal destruction, we used emphysema indices, exponent D, and low-attenuation area percentage (LAA%)
0 Comments  Clinical groups  Neurology Group Reetta Kälviäinen Northern Savo Hospital District, Kuopio, Finland Valtteri Julkunen Northern Savo Hospital District, Kuopio, Finland Hilkka Soininen Northern Savo Hospital District, Kuopio, Finland Anne Remes Northern Ostrobothnia Hospital District, Oulu, Finland Mikko Hiltunen Northern Savo Hospital District, Kuopio, Finland Jukka Peltola Pirkanmaa Hospital District, Tampere, Finland Pentti Tienari Hospital District of Helsinki and Uusimaa, Helsinki, Finland
0 Comments Key words Aspirin-exacerbated respiratory disease (AERD), sometimes termed nonsteroidal anti-inflammatory drug (NSAID)-exacerbated respiratory disease (NSAID-ERD), is a triad of hypersensitivity to NSAIDs, chronic rhinosinusitis with nasal polyps (CRSwNP), and bronchial asthma. This syndrome is characterized by type 2 airway inflammation with extensive eosinophilic infiltration and ongoing mast cell activation. Symptoms of both upper and
0 Comments To the Editor: My colleagues and I read with interest the study by Chollet and Akin 1 Chollet MB, Akin C. Hereditary alpha tryptasemia is not associated with specific clinical phenotypes [e-pub ahead of print]. J Allergy Clin Immunol Accessed June 27, 2021. and note several concerns with their study design and the