Results 71 to 80 of about 22,345 (254)

Hypofibrinogenemia is an independent predictor of hemophagocytic lymphohistiocytosis in children with sepsis

Scientific Reports, 2023
Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening condition in children with sepsis. We herein aimed to identify clinical and laboratory predictors of HLH in children with sepsis.
Xin Luo, Chentao Zhou, Cunwei Ji, Chunmin Lu, Yasha Luo, Zhenhui Chen, Tianhua Zhong, Ruoting Ye, Liwei Zeng, Mingyong Luo   +9 more
doaj   +1 more source

Inherited Hemophagocytic Lymphohistiocytosis (HLH)

, 2010
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal immune disorder characterized by uncontrolled lymphocyte- and macrophage-activation.
Pachlopnik Schmid, Jana; https://orcid.org/, de Saint Basile, G   +1 more
core   +1 more source

ALFA-1/C9orf72 regulates lipid metabolism through HLH-30/TFEB.

, 2020
(A) Representative images of Nile Red staining in N2, alfa-1(ok3062), HLH-30::GFP-expressing wild-type and HLH-30::GFP-expressing alfa-1(ok3062) mutant C. elegans after 1 day of starvation. Enlarged images of boxed areas are shown in each panel.
Yon Ju Ji (3359057), Tao Zhang (43681), Janet Ugolino (3359054), Jiou Wang (267956), Jiayin Lu (716730), Dohoon Kim (251220)   +5 more
core   +1 more source

Humanized immune system animal models and their recent applications

Animal Models and Experimental Medicine, EarlyView.
In this review, we provide an updated and comprehensive overview of the current state of humanized immune system animal model research. We discuss the variety of techniques used to produce humanized mice with their respective strengths and weaknesses, and evaluate the advantages and limitations of these models, including issues with incomplete immune ...
Nicolas Skuli, Rudra B. Amin, A’ishah Bakayoko, Marisa Kruidenier, Sahara Aqui, Sarah J. Skuli, Terence P. Gade   +6 more
wiley   +1 more source

Laboratory and imaging risk factors for mortality in children with primary hemophagocytic lymphohistiocytosis

Frontiers in Oncology
ObjectivesPrimary hemophagocytic lymphohistiocytosis (p-HLH), a genetic disorder characterized by hyperinflammation, is associated with high mortality in pediatric hematology. This study investigates laboratory and imaging risk factors for mortality in p-
Jia Huang, Jia Huang, Sipei Xu, Rui Tang, Yan Huang, Wei Li, Chundan Gong, Sijie Gao, Hailun Peng, Li Xiao, Wei Ma   +10 more
doaj   +1 more source

Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice

Frontiers in Immunology, 2013
Primary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease of hyperinflammation resulting from immune dysregulation due to inherited defects in the cytolytic machinery of NK and T cells.
Birthe eJessen, Tamara eKögl, Fernando E. Sepulveda, Fernando E. Sepulveda, Genevieve ede Saint Basile, Genevieve ede Saint Basile, Genevieve ede Saint Basile, Peter eAichele, Stephan eEhl   +8 more
doaj   +1 more source

A Multifaceted Interplay Among Hemophagocytosis, Interleukin‐18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases

Arthritis &Rheumatology, EarlyView.
Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller, Charlotte Girard, Harmen J. G. van de Werken, Stefan J. Erkeland, Dwin G. B. Grashof, Laurie Vaillant, Renske J. H. den Dekker, Iris van Zuijen, Sharon Veenbergen, Fanny Coffin, Eric Bindels, Yvan Jamilloux, Katerina Laskari, Vassili Soumelis, Bruno Fautrel, Cem Gabay, Peter D. Katsikis   +16 more
wiley   +1 more source

Etiologies and long-term outcome of pediatric hemophagocytic lymphohistiocytosis and macrophage activation syndrome in Taiwan: a single-center retrospective study

Frontiers in Immunology
Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening hyperinflammatory conditions. Primary HLH is caused by genetic mutations associated with defective cytotoxicity, while secondary HLH is triggered by ...
Ching-Yu Wang, Jyh-Hong Lee, Ni-Chung Lee, Ya-Chiao Hu, Hsiu-Hao Chang, Li-Chieh Wang, Yu-Tsan Lin, Yao-Hsu Yang, Bor-Luen Chiang, Bor-Luen Chiang, Hsin-Hui Yu   +10 more
doaj   +1 more source

Recurrent parvovirus B19 viremia resulting in two episodes of hemophagocytic lymphohistiocytosis

Virology Journal, 2022
Background Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with uncontrolled activation of lymphocytes and macrophages. Besides a primary (genetic) form, HLH can also be triggered by malignant, autoimmune and infectious diseases.
Hans Martin Orth, Andre Fuchs, Nadine Lübke, Björn-Erik Ole Jensen, Tom Luedde   +4 more
doaj   +1 more source

Regulation of HLH-2/E2A during Caenorhabditis elegans gonadogenesis

, 2021
Organisms are comprised of many cells with multiple distinct cell types, each of which must be decided precisely to ensure proper formation of a functional organism. In C. elegans, the basic helix-loop helix transcription factor HLH-2 is required for the
Benavidez, Justin M.
core   +1 more source