Results 71 to 80 of about 2,214,075 (211)
Pediatric Investigation, EarlyView.This study elucidates the critical pathogenic mechanisms of pediatric Epstein–Barr virus‐associated hemophagocytic lymphohistiocytosis (EBV‐HLH), where EBV infection triggers a cytokine storm leading to immune cell hyperactivation and tissue damage. Dynamic plasma biomarkers (EBV‐DNA, ferritin, and interferon‐γ) effectively predict treatment response ...
Feifei Liu +9 more
wiley +1 more source
Approach to genetic analysis in the diagnosis of hereditary autoinflammatory syndromes. [PDF]
, 2005 Contains fulltext : 50710.pdf (Publisher’s version ) (Open Access)OBJECTIVE: Hereditary autoinflammatory syndromes are characterized by recurrent episodes of fever and inflammation.
K. Yoshimura +13 more
core +2 more sources
Precision immunomodulation for pediatric hemophagocytic lymphohistiocytosis in intensive care
Pediatric Investigation, EarlyView.This review presents a bedside framework for recognizing pediatric hemophagocytic lymphohistiocytosis and cytokine storm, stabilizing organ dysfunction, identifying the underlying phenotype, selecting targeted immunomodulation, considering extracorporeal adjuncts, and reassessing response within 24–72 h. ABSTRACT Hemophagocytic lymphohistiocytosis (HLH)
Weerapong Lilitwat, Prakreeti Bhandari
wiley +1 more source
Allergy, EarlyView.IL‐10 producing CCR6+Th‐cells are central memory T‐cells that express ROR‐γt and differentiate to Th17‐cells via an autocrine loop of STAT3‐activating cytokines (preTh17). STAT3‐deficient AD‐HIES patients lack Th17‐ and Tfh17‐cells but retain preTh17‐ and Th1/17‐cells.
Giorgia Moschetti +18 more
wiley +1 more source
Elucidation of the Pathogenesis of Autoinflammatory Diseases Using iPS Cells
, 2021 Autoinflammatory diseases are a disease entity caused by the dysregulation of innate immune cells. Typical autoinflammatory diseases are monogenic disorders and often very rare.
Megumu K. Saito
core +1 more source
Clinical and Laboratory Parameters of Autoinflammatory Disorders in Single Tertiary Care Center
Iranian Journal of Allergy, Asthma and Immunology, 2022 Autoinflammatory diseases (AIDs) are disorders with an inborn error of innate immunity, characterized by recurrent episodes of fever and inflammatory attacks.
Roya Sherkat +6 more
doaj
Induced pluripotent stem cells representing Nakajo-Nishimura syndrome
Inflammation and Regeneration, 2019 Nakajo-Nishimura syndrome is a proteasome-associated autoinflammatory syndrome with a distinct homozygous mutation in the PSMB8 gene encoding an inducible β5i subunit of the immunoproteasome.
Nobuo Kanazawa +2 more
doaj +1 more source
Allergy, EarlyView.Skin tape strip lipidomic analysis revealed preexisting skin barrier lipid abnormalities at day 0 that were further exacerbated after 6 weeks of CPI treatment. Significant changes in skin (but not plasma) proinflammatory cytokines were identified in future ircAE patients after 6 weeks of CPI immunotherapy.
Taras Lyubchenko +9 more
wiley +1 more source
A genetic and historical perspective on the origins of keratitis fugax hereditaria
Acta Ophthalmologica, EarlyView.Abstract Purpose To investigate the genetic and genealogical background of keratitis fugax hereditaria (KFH), a periodic corneal disease caused by the heterozygous pathogenic variant c.61G>C in the NLRP3 gene. KFH is characterized by recurrent unilateral autoinflammatory attacks alternating between the eyes and permanent corneal opacities.
Annamari T. Immonen +7 more
wiley +1 more source
British Journal of Haematology, EarlyView.Summary While the majority of myeloid neoplasms are sporadic, the increasing application of germline genetic testing has led the World Health Organization to designate ‘Myeloid malignancies with germline predisposition’ as a distinct clinical entity, carrying major implications for clinical care and research.
Elena Vuelta +9 more
wiley +1 more source