Results 71 to 80 of about 22,112 (212)

Fatal Chronic Varicella‐Zoster Viral Infection in a Young Man With Chediak–Higashi Syndrome

Pediatric Dermatology, EarlyView.
ABSTRACT Chediak–Higashi syndrome (CHS) is a rare autosomal recessive primary immunodeficiency characterized by partial oculocutaneous albinism, neurologic involvement, and a predisposition to severe infections. Patients are particularly susceptible to developing hemophagocytic lymphohistiocytosis (HLH), which significantly worsens prognosis. We report
Albane Badet, Clément Pruvot, Zoé Manssens, Wadih Abou‐Chahla, Sébastien Buche   +4 more
wiley   +1 more source

Presentation of hemophagocytic lymphohistiocytosis due to a novel MUNC 13–4 mutation masked by partial therapeutic immunosuppression

Pediatric Rheumatology Online Journal, 2012
Hemophagocytic lymphohistiocytosis is a potentially fatal disease characterized by excessive macrophage and lymphocyte activity. Patients can be affected following immune activation after an oncologic, autoimmune or infectious trigger. An associated gene
Garrett Jackie P-D, Fung Irene, Rupon Jeremy, Knight Andrea, Mizesko Melissa, Paessler Michelle, Orange Jordan S   +6 more
doaj   +1 more source

Adjunctive Therapeutic Plasma Exchange in Refractory Adult‐Onset Still's Disease Complicated by Secondary Macrophage Activation Syndrome: A Single‐Center Experience

Therapeutic Apheresis and Dialysis, Volume 30, Issue 3, Page 389-396, June 2026.
ABSTRACT Introduction Adult‐onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS) carries substantial mortality. The role of therapeutic plasma exchange (TPE) remains uncertain. Methods We retrospectively analyzed patients with AOSD‐MAS treated with TPE at a single‐center.
Masataka Ueda, Tomohiro Saito, Masahide Mizobuchi, Tomoki Shimomura, Saki Kawamori, Mitsuru Kawanishi, Natsuko Hattori, Jo Sugiyama, Satoshi Tsubata, Hanami Horiguchi, Kazuki Abe, Nao Iida, Yuki Kajio, Nao Oguro, Ryo Yanai, Hirokazu Honda   +15 more
wiley   +1 more source

Human Inborn Errors of Immunity: 2019 Update of the IUIS Phenotypical Classification. [PDF]

, 2020
Since 2013, the International Union of Immunological Societies (IUIS) expert committee (EC) on Inborn Errors of Immunity (IEI) has published an updated phenotypic classification of IEI, which accompanies and complements their genotypic classification ...
Ailal, Fatima, Al-Herz, Waleed, Bousfiha, Aziz, Casanova, Jean-Laurent, Chatila, Talal, Cunningham-Rundles, Charlotte, Etzioni, Amos, Franco, Jose Luis, Holland, Steven M, Jeddane, Leila, Klein, Christoph, Morio, Tomohiro, Ochs, Hans D, Oksenhendler, Eric, Picard, Capucine, Puck, Jennifer, Sullivan, Kathleen E, Tangye, Stuart G, Torgerson, Troy R   +18 more
core  

Age‐Dependent Differences in Canakinumab Safety: A Comprehensive Pharmacovigilance Analysis Using the FAERS Database

Pharmacology Research &Perspectives, Volume 14, Issue 3, June 2026.
ABSTRACT While the efficacy of canakinumab, an anti‐interleukin‐1β monoclonal antibody, is well‐established, its safety profile, particularly across different age groups, remains inadequately explored. Using the FDA Adverse Event Reporting System (FAERS) database, this study evaluated postmarketing safety by analyzing adverse event (AE) reports from ...
Youyang Wang, Ngasin Lam, Xiaoming Huang, Yang Jiao   +3 more
wiley   +1 more source

Neonatal Hemophagocytic Lymphohistiocytosis – Case Report [PDF]

, 2010
Hemophagocytic lymphohystiocytosis (HLH) represents a severe hyperinflammatory condition with the cardinal symptoms prolonged fever, hepatosplenomegaly, and cytopenias. The most prominent histopathological feature of HLH is an accumulation of activated T
Barbara Kvenić, Ika Kardum-Skelin, Irena Seili-Bekafigo, Jelena Roganović, Nives Jonjić   +4 more
core   +1 more source

Immune endotypes in tuberculosis: Keys to decoding disease complexity

Journal of Internal Medicine, Volume 299, Issue 6, Page 670-693, June 2026.
Abstract Tuberculosis (TB) remains a major global health challenge, with multi‐drug antibiotic regimens as the current standard of care. While effective at killing Mycobacterium tuberculosis, these treatments do not resolve persistent inflammation, prevent lung damage, or reverse immune dysregulation that contribute to poor outcomes and disease ...
Shamila D. Alipoor, Julia Guthrie, Lina Davies Forsman, Andrew R. Di Nardo, Susanna Brighenti   +4 more
wiley   +1 more source

Relative Exchangeable and Exchangeable Copper: Emerging New Biomarkers for Diagnosis and Therapy Monitoring in Wilson's Disease

Liver International, Volume 46, Issue 6, June 2026.
ABSTRACT Background and Aims Wilson's disease (WD) is a genetic disorder of copper metabolism in which early diagnosis remains challenging, particularly in acute liver failure (ALF). Relative exchangeable copper (REC) and exchangeable copper (CuEXC) are emerging biomarkers for diagnosis and monitoring, but data from larger cohorts are limited.
Sebastian Köhrer, Antoan Rusev, Holger Zimmer, Silke Wolf, Jessica Langel, Andrea Langel, Thomas Longerich, Patrick Michl, Moritz Niesert, Alexander Fichtner, Isabelle Mohr   +10 more
wiley   +1 more source

Anemia in Antiretroviral Naïve HIV/AIDS Patients: A Study from Eastern India [PDF]

, 2012
Background: Hematological manifestations are common throughout the course of HIV infection. Impact of anemia is the most significant among them. The present study was undertaken to evaluate the etiologies underlying anemia in HIV/AIDS. Methods This was a
Banerjee, A, Bhattacharyya, M, Ghosh, A, Ghosh, B, Pain, S, Pande, A, Saha, S   +6 more
core   +1 more source

Diagnostic biomarkers for adult haemophagocytic lymphohistiocytosis in critically ill patients (HEMICU): a prospective observational study protocol [PDF]

, 2019
INTRODUCTION: Haemophagocytic lymphohistiocytosis (HLH) in adults is characterised by toxic immune activation and a sepsis-like syndrome, leading to high numbers of undiagnosed cases and mortality rates of up to 68%.
Akyüz, Levent, Balzer, Felix, Brunkhorst, Frank M., Haefen, Clarissa von, Keh, Didier, Knaak, Cornelia, Kruppa, Jochen, Kölsch, Uwe, La Rosée, Paul, Lachmann, Gunnar, Lachmann, Nils, Meisel, Christian, Nyvlt, Peter, Paeschke, Nadine, Piper, Sophie K., Schenk, Thomas, Schuster, Friederike S., Spies, Claudia, Unterwalder, Nadine, Volk, Hans-Dieter, Vorderwülbecke, Gerald   +20 more
core   +1 more source