Results 31 to 40 of about 43,174 (164)

Cerebral Small Vessel Disease in Immune‐Mediated Thrombotic Thrombocytopenic Purpura Patients During the Acute Phase and Disease Remission

American Journal of Hematology, EarlyView.
ABSTRACT Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a life‐threatening thrombotic microangiopathy characterized by acute neurological manifestations and long‐term sequelae related to microvascular brain injury. Cerebral small vessel disease (cSVD), detectable on MRI through lacunes, microbleeds, white matter hyperintensities, and ...
Addolorata Truma, Francesco Maria Lo Russo, Giorgio Conte, Ilaria Mancini, Andrea Artoni, Juri Alessandro Giannotta, Barbara Ferrari, Pasquale Agosti, Maria Abbattista, Matteo Gagliardi, Eleonora Piccin, Marco Stroppi, Fabio Maria Triulzi, Flora Peyvandi   +13 more
wiley   +1 more source

Complement Activation Linked to Type II Interferon Signaling in Still Disease

Arthritis &Rheumatology, EarlyView.
Objective Still disease (SD) is an autoinflammatory syndrome characterized by innate immune dysregulation. Although complement can drive inflammation, its involvement in SD remains to be defined. Thus, we aimed to assess complement activation in SD. Methods Complement was assessed using transcriptomic, proteomic, and in vitro approaches. RNA sequencing
Freya M. C. H. Huijsmans, Tabea Thalheim, Alejandra Bodelón, Greta Rogani, Lyanne J. P. M. Sijbers, Remco G. A. Erkens, Aafke de Ligt, Rianne Scholman, Aron Brinker, Gisella B. Beretta, Nienke M. Ter Haar, Thomas Vogl, Johannes Roth, Trang T. Duong, Sytze de Roock, Joost F. Swart, Deborah A. Marshall, Susanne M. Benseler, Rae S. M. Yeung, Christoph Kessel, Sebastiaan J. Vastert, Emely L. Verweyen, Jorg van Loosdregt, on behalf of the UCAN CAN‐DU/UCAN CURE Consortia and the One Child Every Child Initiative, Adam Huber, Bianca Lang, Chelsea DeCoste, Elizabeth Stringer, Suzanne Ramsey, Alan Rosenberg, Kate Neufeld, Mehul Jariwala, Tristan Kerr, Alexander Mosoiu, Alisa Rachlis, Amy Xu, Arthur Cheng, Brenleigh Jebb, Brian Feldman, Bruno Pereira, Deborah Levy, Dilan Dissanayake, Elizaveta Limenis, Evelyn Rozenblyum, Harper Cheng, Jennifer Ji Young Lee, Lynn Spiegel, Rayfel Schneider, Ronald Laxer, Ruud Verstegen, Shirley Tse, Andrea Human, David Cabral, Herman Tam, Jaime Guzman, Kim Morishita, Kristin Houghton, Lori Tucker, Mercedes Chan, Ross Petty, Tommy Gerschman, Annet van Royen‐Kerkhof, Berent Prakken, Erika Van Nieuwenhove, Marc Jansen, Nico Wulffraat, Ciarán Duffy, Nadia Luca, Roman Jurencak, Tala El Tal, Claire LeBlanc, Gaëlle Chédeville, Piya Lahiry, Rosie Scuccimarri, Sarah Campillo, Clare Hutchinson, Daniah Basodan, Dax G Rumsey, Hon Yan Ng, Jeanine McColl, Lillian Lim, Tara McGrath, Danielle Brinkman, Petra Hissink Muller, Elizabeth Legger, Wineke Armbrust, Ellen Schatorje, Esther Hoppenreijs, Elodie Boudes, Gillian Currie, Heinrike Schmeling, Muhammed Dhalla, Nicole Johnson, Paivi Miettunen, Ravneet Sran, Rebeka Stevenson, Erkan Demirkaya, Jonathan Park, Roberta Berard, Giske Biesbroek, Mariken Gruppen, Gordon Soon, Joseph Cafazzo, Liane Heale, Michelle Batthish, Tania Cellucci, Lily Lim, Maarten IJzerman, Marinka Twilt, Marleen Verkaaik, Philomine van Pelt, Sylvia Kamphuis, Michelle Kip, Nicholas Blanchette, Paul Dancey, Regina de Geus   +115 more
wiley   +1 more source

Analysis of Carbohydrates and Glycoconjugates by Matrix‐Assisted Laser Desorption/Ionization Mass Spectrometry: An Update for 2023–2024

Mass Spectrometry Reviews, EarlyView.
ABSTRACT The use of MALDI mass spectrometry for the analysis of carbohydrates and glycoconjugates is a well‐established technique and this comprehensive review is the twelfth update of the original article published in 1999 and brings coverage of the literature to the end of 2024.
David J. Harvey
wiley   +1 more source

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome [PDF]

, 2014
Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment.
Goodship, T, Scully, M
core  

Characterization of the complications associated with plasma exchange for thrombotic thrombocytopaenic purpura and related thrombotic microangiopathic anaemias: a single institution experience. [PDF]

, 2014
Plasma exchange (PEX) is a life-saving therapeutic procedure in patients with thrombotic thrombocytopaenic purpura (TTP) and other thrombotic microangiopathic anaemias (TMAs). However, it may be associated with significant complications, exacerbating the
McGuckin, S, Collier, D, Scully, M, Webster, H, Leverett, D, Westwood, JP   +5 more
core  

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

Respiratory profile of pediatric patients undergoing hematopoietic stem cell transplantation studied by oscillometry – A TRANSPIRE cohort

Pediatric Investigation, EarlyView.
These are results from a multi‐center collaboration between four large pediatric institutions, investigating baseline respiratory system function in a cohort of children undergoing hematopoietic stem cell transplantation. This manuscript proposes respiratory oscillometry as a novel way to prospectively follow lung function in these children.
Maureen B. Parenti, Julian Allen, Lisa R. Young, Heather Boas, Jason Freedman, Christopher Towe, Jane Koo, Kasiani Myers, Jason C. Woods, Adam Lane, Jonathan Gaffin, Wai Wong, Leslie Lehmann, Fernando Urrego, Gabriel Salinas Cisneros, Matthew F. Abts, K. Scott Baker, Manuel Silva‐Carmona, Erin Doherty, Paul D. Robinson, Margaret MacMillan, Stella M. Davies, Samuel B. Goldfarb   +22 more
wiley   +1 more source

Utility of Different Scoring Systems for the Diagnosis of Thrombotic Microangiopathies

, 2023
Objective: To investigate the appropriateness of Bentley and plasmic scores and ADAMTS-13 activity to distinguish between primary thrombotic microangiopathies (TMA) syndromes and other thrombotic microangiopathies, as well as primary thrombotic ...
Yilmaz, Seda, CenelI, Ozcan, Tekinalp, Atakan   +2 more
core   +1 more source

Deficiency of high‐molecular‐weight von Willebrand factor mitigates thrombo‐complement injury in an LPS‐induced TMA‐like mouse model

VIEW, EarlyView.
Reduction of high‐molecular‐weight von Willebrand factor disrupts the platelet–complement amplification loop, attenuating microvascular thrombosis, complement deposition, endothelial injury, and organ damage in complement‐mediated thrombotic microangiopathy.
Yang Li, Chihao Zhang, Xiaoqian Xu, Sicong Wang, Xiaofan Hu, Junjie Yao, Xi Wu, Difeng Zhu, Qian Liang, Yeling Lu, Yu Liu, Guanqun Xu, Jiakuan Zhou, Qiulan Ding, Wenman Wu, Ya Li, Jing Dai, Xuefeng Wang   +17 more
wiley   +1 more source

Red lines and green lights: Gene therapy for inherited erythroid disorders beyond the haemoglobinopathies

British Journal of Haematology, EarlyView.
Gene therapy is revolutionizing treatment paradigms for haemoglobinopathies, establishing a translational framework for disorders that impact red blood cell development. In their paper, Joshi et al. describe the preclinical and early clinical landscape of gene therapies for non‐haemoglobinopathy erythroid disorders and highlight common thematic ...
Gaurav Joshi, Nandakumar Natarajan, Kathryn E. Dickerson, Richard A. Voit   +3 more
wiley   +1 more source