Results 51 to 60 of about 2,994 (188)

Inborn errors of immunity in children with neuroinflammation

Developmental Medicine &Child Neurology, EarlyView.
Abstract Inborn errors of immunity (IEIs), an expanding group of monogenic disorders with diverse clinical manifestations, are increasingly recognized to include neuroinflammatory disease. Examples of diseases included under this umbrella are Aicardi–Goutières syndrome, deficiency of adenosine deaminase 2, familial haemophagocytic lymphohistiocytosis ...
Eppie M Yiu, Dilan Dissanayake, Jessie Cunningham, Helen Branson, Ori Scott, E Ann Yeh   +5 more
wiley   +1 more source

PROTEOLYTIC PROCESSING OF VON WILLEBRAND FACTOR BY ADAMTS13 AND LEUKOCYTE PROTEASES-rev1-MJHID-2013

Mediterranean Journal of Hematology and Infectious Diseases, 2014
ADAMTS13 is a 190 kDa zinc protease encoded by a gene located on chromosome 9q34.   This protease specifically hydrolyzes von Willebrand factor (VWF) multimers, thus causing VWF size reduction.
Raimondo De Cristofaro
doaj  

Thrombotic Microangiopathy in Pregnancy: Current Understanding and Management Strategies

Kidney International Reports
Thrombotic microangiopathy (TMA) represents a heterogeneous group of disorders characterized by microvascular thrombosis and end-organ damage. Pregnancy-associated thrombotic microangiopathy (p-TMA) has emerged as a distinct clinical entity with unique ...
Manuel Urra, Shannon Lyons, Corina Gabriela Teodosiu, Richard Burwick, Anuja Java   +4 more
doaj   +1 more source

Diagnosis and Treatment of Thrombotic Thrombocytopenic Purpura Developed During Gestation: Literature Review and Case Report

Клиническая онкогематология, 2014
This paper presents a literature review on state of art in issues of etiology, pathogenesis, clinical features and diagnosis of thrombotic thrombocytopenic purpura (ТТP or Moschcowitz’s disease).
V. V. Voitsekhovskii, L. B. Filatov, A. V. Pivnik, P. V. Avdonin, T. V. Esenina, A. G. Sudakov   +5 more
doaj   +1 more source

Epidemiology, treatment patterns, clinical outcomes, and disease burden among patients with immune‐mediated thrombotic thrombocytopenic purpura in the United States

Research and Practice in Thrombosis and Haemostasis, 2022
Background Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a life‐threatening thrombotic microangiopathy. Due to its rarity, epidemiology and real‐world outcomes data are scarce.
Ayoade Adeyemi, Francesca Razakariasa, Alexandra Chiorean, Rui dePassos Sousa   +3 more
doaj   +1 more source

Phase IIB, Randomized, Double‐Blind, Placebo‐Controlled Clinical Trial of Intravenous Defibrotide for the Prevention and Treatment of Respiratory Distress and Cytokine Release Syndrome in COVID‐19

European Journal of Haematology, EarlyView.
ABSTRACT Introduction Endothelial dysfunction is key in COVID‐19 pathogenesis. This randomized, double‐blind phase IIb trial investigated continuous intravenous infusion of defibrotide in patients hospitalized with SARS‐CoV‐2 infection and respiratory failure.
Ruben Jara Rubio, Antonio José Martinez‐Mellado, Jonathan Kiwitt‐Cárdenas, José G. Clavel, Bartolomé García‐Perez, Pedro Castro, Maribel Diaz‐Ricart, Andrés Carrillo‐Alcaraz, Amparo López‐Bernús, Enrique Bernal Morell, Aychel Roura, Sonia Marín, Francisco‐José Ruiz‐López, Elena Solana‐Martinez, Domingo Martinez‐Baño, Carlos L. Albacete Moreno, Enriqueta Andreu Soler, Adrián Tellez Santoyo, Sara Fernández Méndez, Jose A. Noguera Velasco, Iria Cebreiros Lopez, Andrés Parrilla, Alberto Espuny‐Miró, David García‐Bernal, Miguel Blanquer, Andrés Sánchez‐Salinas, Francisca Iniesta, Cristina Hernandez Garcia, Manuel Muro, Alfredo Minguela Puras, Antonio Moreno‐Docón, Alberto M. Torres‐Cantero, Maria Munoz Garcia, Manuel Serrano, Massimo Iacobelli, Carmelo Carlo‐Stella, Lee‐Jen Wei, Paul G. Richardson, José M. Moraleda   +38 more
wiley   +1 more source

The Complement System in Post‐Transplant Kidney Injury

Organ Medicine, EarlyView.
Mengsi Hu
wiley   +1 more source

Third Allogeneic Stem Cell Transplantation in Children: A Multicenter Analysis From the Spanish GETH‐TC Pediatric Group

European Journal of Haematology, EarlyView.
ABSTRACT Third allogeneic hematopoietic stem cell transplantation (HSCT3) is rarely performed in pediatric patients and is associated with high toxicity and mortality. Data on outcomes in this setting remain scarce. We conducted a retrospective multicenter study within the Spanish GETH‐TC Pediatric group including 29 children and adolescents who ...
Luisa Sisinni, Sara Vinagre Enríquez, Marta Villalon Herrera, Mercedes Plaza Fornieles, Iván López Torija, Andrea Urtasun Erburu, Laura Alonso García, Alexandra Regueiro García, Maria Trabazo del Castillo, Eduardo J. Bardón‐Cancho, Odelaisy León‐Triana, Carla Miñarro Chacón, José Luís Fuster Soler, Antonio Pérez‐Martínez, Pascual Balsalobre López, Marta Gonzalez‐Vicent, Cristina Díaz de Heredia, on behalf of the GETH‐TC Pediatric Working Group   +17 more
wiley   +1 more source

Cancer-related thrombotic microangiopathies: case report and narrative review

Italian Journal of Medicine, 2018
Thrombotic manifestations are common in cancer patients and increase morbidity and mortality. They can affect large or small, venous or arterial vessels. Thrombosis of large arteries and veins is much more studied than that of small vessels. Nevertheless,
Micaela La Regina, Daniele Bertoli, Tiziana Ciarambino, Rossella Petacchi, Ugo Giannoni, Francesco Orlandini   +5 more
doaj   +1 more source

Early Acute Kidney Injury in Allogeneic Hematopoietic Cell Transplantation: Incidence, Severity, and Associated Factors in a 634‐Recipient Cohort

European Journal of Haematology, EarlyView.
ABSTRACT Background Acute kidney injury (AKI) is a frequent and clinically relevant complication in allogeneic hematopoietic cell transplantation (HCT) recipients, particularly during the early post‐HCT period. Although AKI has been extensively reported in long‐term follow‐up studies, data focusing on the initial hospitalization phase remain limited ...
Luiz Carlos da Costa‐Junior, Rita de Cássia Barbosa da Silva Tavares, Marina Izu, Simone Cunha Maradei, Maria Claudia Rodrigues Moreira, Marta Colares Nogueira, Décio Lerner, Fabrício Guimarães Bino, Patrícia Moriel, Teresa de Souza Fernandez, Paulo Caleb Júnior Lima Santos   +10 more
wiley   +1 more source