Results 81 to 90 of about 2,283,992 (229)

Current Therapeutic Options for the Main Monogenic Autoinflammatory Diseases and PFAPA Syndrome: Evidence-Based Approach and Proposal of a Practical Guide

Frontiers in Immunology, 2020
Monogenic autoinflammatory diseases are rare conditions caused by genetic abnormalities affecting the innate immunity. Previous therapeutic strategies had been mainly based on results from retrospective studies and physicians' experience. However, during
Alessandra Soriano, Marco Soriano, Gerard Espinosa, Raffaele Manna, Giacomo Emmi, Luca Cantarini, José Hernández-Rodríguez   +6 more
doaj   +1 more source

Juvenile Gangrenous Vasculitis of the Scrotum or Scrotum Lipschütz Ulcers

JEADV Clinical Practice, EarlyView.
ABSTRACT Lipschütz genital ulcers are traditionally described in adolescent females and often linked to acute viral infections. Their male counterpart remains poorly characterised. We report a 59‐year‐old man with sudden onset of unilateral necrotic ulcers on the scrotum and inguinal region following a brief febrile episode and mild pharyngitis ...
Francesco Bellinato, Paolo Gisondi, Giampiero Girolomoni   +2 more
wiley   +1 more source

Molecular Biomarkers in Meniere's Disease: A Scoping Review of Current Evidence

Otolaryngology–Head and Neck Surgery, EarlyView.
Abstract Objective Meniere's disease is a complex chronic inner ear condition that is characterized by vertigo, tinnitus, aural fullness, and progressive hearing loss. Currently, diagnostic strategies remain symptom‐driven, and treatments focus on management of discrete episodes rather than targeting underlying pathophysiology.
Hamza Kamran, Zaharadeen Jimoh, Tariq Issa, Nahla A. Gomaa   +3 more
wiley   +1 more source

A clinical review of autoinflammatory diseases and Behcet's disease: Classification, pathogenesis and treatment [PDF]

, 2020
Behcet’s disease is a rheumatic disease with oral aphthae, genital aphthae, arthritis and vasculitis. Studies about its pathogenesis have increased and is thought to be one of the autoinflammatory diseases in recent years.
Aksakal, Sengul
core   +1 more source

Dynamic early biomarkers predict outcomes in pediatric Epstein–Barr virus‐associated hemophagocytic lymphohistiocytosis

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, Qing Zhang, Sitong Chen, Yunze Zhao, Dong Wang, Hongyun Lian, Honghao Ma, Tianyou Wang, Rui Zhang, Zhigang Li   +9 more
wiley   +1 more source

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

Therapeutic Properties and Underlying Mechanisms of Traditional Chinese Medicine in Managing Rheumatoid Arthritis: An Integrative Review of Clinical and Molecular Findings

Phytotherapy Research, EarlyView.
The translational research framework integrating TCM for RA. The figure outlines a stepwise framework for the modernization and clinical translation of TCM in RA. Part 1 highlights the inheritance and development of TCM theory, including classical medical texts, traditional herbal knowledge, and syndrome differentiation–based treatment principles. Part
Jianting Wen, Jian Liu, Lei Wan, Fanfan Wang, Yang Li   +4 more
wiley   +1 more source

Autosomal Dominant Hyper‐IgE Syndrome Patients Retain IL10‐Producing preTh17‐Cells That Are Activated by Opportunistic Pathogens and Support IgE Production

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, Chiara Vasco, Francesca Clemente, Paola Larghi, Sara Maioli, Edoardo Scarpa, Elena Carelli, Nadia Pulvirenti, Maria Lucia Sarnicola, Mariacristina Crosti, Manal Bel Imam, Willem van de Veen, Loris Rizzello, Sergio Abrignani, Lucia A. Baselli, Rosa Maria Dellepiane, Maria Carrabba, Giovanna Fabio, Jens Geginat   +18 more
wiley   +1 more source

Dysfunctional immunoproteasomes in autoinflammatory diseases [PDF]

, 2016
Recent progress in DNA sequencing technology has made it possible to identify specific genetic mutations in familial disorders. For example, autoinflammatory syndromes are caused by mutations in gene coding for immunoproteasomes.
有持, 秀喜, Yasutomo, Koji, 60214/profile-ja.html, 50454757, 30333511, 60033/profile-ja.html, キタムラ, アキコ, ササキ, ユキ, Arimochi, Hideki, Sasaki, Yuki, 321, Kitamura, Akiko, 1521, 30311822, 676, 北村, 明子, 10448318, アリモチ, ヒデキ, 佐々木, 由紀, 1616, 273670/profile-ja.html, 安友, 康二, ヤストモ, コウジ   +22 more
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Development of the autoinflammatory diseases damage index (ADDI) [PDF]

, 2016
OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently,
Ravelli, A, Uziel, Y., Lachmann, HJ, Simon, A, Ombrello, A.K., Ter Haar, N.M., De Jesus, A.A., De Benedetti, F, Russo, R, Annink, KV, Brogan, PA, Ozen, S., Cattalini, M, Durrant, KL, Hachulla, E., Herlin, T., Ozen, S, Koné-Paut, I, Hoffman, HM, Della Casa Alberighi, O, Dedeoglu, F, Al-Mayouf, S.M., Dolezalova, P., Trachana, M, Papadopoulou-Alataki, E, Dolezalova, P, Frenkel, J., Haar, N.M.R. van der, Frenkel, J, Benseler, SM, Hentgen, V, Hofer, M, Jansson, AF, Benedetti, F. De, Hachulla, E, Quartier, P, Nikishina, I., Ter Haar, NM, Kuemmerle-Deschner, J.B., Cochino, A.V., Simon, A., Benseler, S.M., Kallinich, T, Gallizzi, R., Amaryan, G, Hofer, M., Gattorno, M, Kone-Paut, I., Ravelli, A., De Benedetti, F., Martini, A, Kuemmerle-Deschner, JB, Laxer, RM, De Jesus, AA, Kozlova, A, Demirkaya, E, Della Casa Alberighi, O., Kozlova, A., Hoffman, H.M., Lachmann, H.J., Cochino, AV, Insalaco, A, Anton, J., Cantarini, L., Goldbach-Mansky, R., Papadopoulou-Alataki, E., Fabio, G., Hentgen, V., Amaryan, G., Herlin, T, Rigante, D., Gattorno, M., Quartier, P., Barron, K.S., Gallizzi, R, Annink, K.V., Jansson, A.F., Laxer, R.M., Nielsen, S, Martini, A., Goldbach-Mansky, R, Alberighi, O. Della Casa, Nielsen, S., Insalaco, A., Kallinich, T., Nikishina, I, Cantarini, L, Brogan, P.A., Al-Mayouf, SM, Fabio, G, Trachana, M., Durrant, K.L., Anton, J, Ombrello, AK, Barron, KS, Demirkaya, E., Uziel, Y, Dedeoglu, F., Rigante, D, Russo, R., Jesus, A.A. De, Rigante, Donato, Cattalini, M.   +102 more
core   +1 more source