Results 61 to 70 of about 24,724 (214)

NBAS, a gene involved in cytotoxic degranulation, is recurrently mutated in pediatric hemophagocytic lymphohistiocytosis

Journal of Hematology & Oncology, 2022
Hemophagocytic lymphohistiocytosis (HLH), particularly primary HLH (pHLH), is a rare, life-threatening disease. Germline genetic deficiency of 12 known HLH genes impairs cytotoxic degranulation in natural killer (NK) cells or cytotoxic T lymphocytes ...
Xiaoman Bi, Qing Zhang, Lei Chen, Dan Liu, Yueying Li, Xiaoxi Zhao, Ya Zhang, Liping Zhang, Jingkun Liu, Chaoyi Wu, Zhigang Li, Yunze Zhao, Honghao Ma, Gang Huang, Xin Liu, Qian-fei Wang, Rui Zhang   +16 more
doaj   +1 more source

Long‐term outcomes of haploidentical hematopoietic stem cell transplantation with antithymocyte globulin–based myeloablative conditioning in pediatric refractory or relapsed non‐Hodgkin lymphoma

Pediatric Investigation, EarlyView.
Single‐center retrospective data showed that ATG‐based myeloablative haploidentical hematopoietic stem cell transplantation provides durable remission and favorable long‐term survival in children with refractory/relapsed non‐Hodgkin lymphoma, achieving high engraftment rates, low relapse incidence, and acceptable toxicity.
Chenguang Jia, Jie Zheng, Meng Yuan, Hongbo He, Yibo Fan, Bin Wang, Guanghua Zhu, Jun Yang, Yanhui Luo, Lixiao Cai, Pan Zhang, Yanlong Duan, Maoquan Qin   +12 more
wiley   +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

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

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

hlh-30 was necessary for survival during starvation and upon refeeding, and starvation promotes HLH-30 nuclear localization.

, 2019
(A) Schematic depicting starvation and refeeding assay. Gravid adults were bleached with alkaline hypochlorite to obtain eggs, which were transferred to buffered salt solution (M9). Upon hatching, larvae arrest development at the L1 stage.
Abhinav Diwan (759026), Haiyan Liu (51889), Rohan Khopkar (6697076), Ali Javaheri (4615927), Scot J. Matkovich (421129), Xiucui Ma (759024), John T. Murphy (226645), Brian M. Egan (6697070), Kartik Mani (6697079), Alexander Boyko (28471), Daniel Ory (2278774), Xuntian Jiang (2031358), Clara Oh (5567483), Samuel Ang (6697073), Alex Shaver (6697067), Kerry Kornfeld (226655), Sandeep Kumar (145759), Travis Mazer (136384)   +17 more
core   +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

Schematized hlh-4 functions.

, 2018
(A) Overall logic of hlh-4/terminal selector function. “0” indicates gene/feature not expressed; “1” indicates expressed (this binary scheme is a simplification).
Saeed Tavazoie (7618), Oliver Hobert (70540), Neda Masoudi (541180), Leesun Ryu (651729), Lori Glenwinkel (4729329), Kyuhyung Kim (651731)   +5 more
core   +1 more source

Self‐Assembling Gemini‐Type Amphiphiles From Helix‐Loop‐Helix Peptides

Small, EarlyView.
Peptide particles are formed through self‐assembly of amphiphilic helix‐loop‐helix peptides. Depending on the preparation method, two different particle morphologies can be obtained: nanospheres or nanodiscs with α‐helical or β‐sheet structure of the monomers, respectively.
Thomas Heim, Charlotte Helm, Morten Mosbach, Ernest Ronald Curticean, Vanessa Reusche, Irene Wacker, Rasmus R. Schröder, Franziska Thomas   +7 more
wiley   +1 more source