Results 31 to 40 of about 11,766 (177)

Residues Arg568 and Phe592 contribute to an antigenic surface for anti-ADAMTS13 antibodies in the spacer domain

Haematologica, 2011
Background The majority of patients diagnosed with thrombotic thrombocytopenic purpura have autoantibodies directed towards the spacer domain of ADAMTS13.Design and Methods In this study we explored the epitope specificity and immunoglobulin class and ...
Wouter Pos, Nicoletta Sorvillo, Rob Fijnheer, Hendrik B. Feys, Paul H.P Kaijen, Gestur Vidarsson, Jan Voorberg   +6 more
doaj   +1 more source

Contrasting Approaches in the Implementation of GRADE Methodology in Guidelines for Haemophilia and Von Willebrand Disease

Haemophilia, EarlyView.
ABSTRACT Introduction The 2024 ISTH clinical practice guideline (CPG) for treatment of congenital haemophilia, the NBDF‐McMaster Guideline on Care Models for Haemophilia Management, and ASH ISTH NBDF WFH guidelines on the diagnosis and management of VWD all utilised GRADE methodology.
Mark W. Skinner, Manuela Albisetti, Jesús Ardila, Jan Astermark, Jan Blatny, Manuel Carcao, Pratima Chowdary, Nathan T. Connell, Miguel Crato, Yesim Dargaud, Roseline d'Oiron, Amy L. Dunn, Miguel A. Escobar, Carmen Escuriola‐Ettingshausen, Emna Gouider, Annie Harroche, Cedric Hermans, Victor Jimenez‐Yuste, Radoslaw Kaczmarek, Gili Kenet, Liane Khoo, Robert Klamroth, Florian Langer, David Lillicrap, Johnny Mahlangu, Christoph Male, Tadashi Matsushita, Sandrine Meunier, Wolfgang Miesbach, Beatrice Nolan, Johannes Oldenburg, Brian O'Mahony, Margareth Ozelo, Glenn F. Pierce, Gloria Ramos, Michael Recht, Olivia Romero‐Lux, Dawn Rotellini, Rita C. Santoro, Tammuella C. Singleton, Alok Srivastava, Sophie Susen, Kate Talks, Huyen Tran, Leonard A. Valentino, Jerzy Windyga, Renchi Yang, Maria Elisa Mancuso, also on behalf of AICE, AHAD‐AP, AHCDC, ATHN, CLAHT, EHC, French CRH & MHEMO, HTCCNC, NBDF, PEDNET, UKHCDO, WFH   +59 more
wiley   +1 more source

Pediatric thrombotic thrombocytopenic purpura [PDF]

European Journal of Haematology, 2018
AbstractChild‐onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)‐cleavage protease.
Bérangère S. Joly, Paul Coppo, Agnès Veyradier   +2 more
openaire   +2 more sources

Seventh Åland Island Meeting on von Willebrand Disease

Haemophilia, EarlyView.
ABSTRACT Introduction The seventh Åland Island Meeting on von Willebrand Disease (VWD) was held on the Åland archipelago in Finland, from 26 to 28 September 2024. Aim The meeting brought together experts in the field of VWD from around the world to share the latest advances and knowledge in VWD.
Riitta Lassila, Ruben Bierings, Nathan T. Connell, Fernando F. Corrales‐Medina, Augusto B. Federici, Veronica H. Flood, Floor Heubel‐Moenen, Jill M. Johnsen, Geoffrey Kuppens, Davide Matino, Carolyn Millar, Ingmar Schoen, Anish Sharda, Robert F. Sidonio Jr, Alok Srivastava, Timea Szanto, Angela C. Weyand, Erik Berntorp   +17 more
wiley   +1 more source

Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report

Journal of Medical Case Reports, 2018
Background Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and ...
Valter Romão de Souza, Ana Beatriz Cavalcante de Oliveira, Ana Maria Vanderlei, Amanda Queiroz da Mota Silveira Aroucha, Bruna Pontes Duarte, Aureli Nunes Machado, Lívia Netto Chaer, Cláudia Wanderley de Barros Correia, Maria da Conceição de Barros Correia, Manuela Freire Hazin Costa   +9 more
doaj   +1 more source

Beyond the Kidney and Lung: Cutaneous and Mucosal Clues to Human Hantavirus Disease

International Journal of Dermatology, EarlyView.
Rodent exposure and aerosolized excreta can lead to hantavirus infection with systemic endothelial injury and capillary leak, resulting in thrombocytopenia and syndromes such as HPS/HCPS and HFRS/NE. Early dermatologic and mucosal findings, including flushing, conjunctival injection, petechiae/purpura, and mucosal bleeding, may provide important clues ...
Giuseppe Gallo, Pietro Quaglino, Simone Ribero   +2 more
wiley   +1 more source

Catastrophic Thrombotic Thrombocytopenic Purpura Accompanying Recurrent Acute Pancreatitis Attacks and Splenic Vein Rupture

Turkish Journal of Nephrology, 2019
Acute pancreatitis can be encountered as a rare complication of thrombotic thrombocytopenic purpura and it has been associated with recurrent thrombotic thrombocytopenic purpura.
Mesudiye BULUT, Ebru GÖK OĞUZ, Müge EREK, Didem TURGUT, Nihal ÖZKAYAR, Serhan PİŞKİNPAŞA, Fatih DEDE   +6 more
doaj  

Using plasma exchange to successfully manage thyrotoxicosis in a patient with possible antithyroid drug-related thrombotic thrombocytopenic purpura

Endocrine Regulations, 2017
Objective. Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever, and renal insufficiency.
Tazegul G, Ogut TS, Bozoglan H, Dogan O, Yilmaz N, Ulas T, Salim O, Sari R, Altunbas HA, Balci MK   +9 more
doaj   +1 more source

Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura

Haematologica, 2010
Background Type G immunoglobulins against ADAMTS13 are the primary cause of acquired (idiopathic) thrombotic thrombocytopenic purpura. However, the domains of ADAMTS13 which the type G anti-ADAMT13 immunoglobulins target have not been investigated in a ...
X. Long Zheng, Haifeng M. Wu, Dezhi Shang, Erica Falls, Christopher G. Skipwith, Spero R. Cataland, Charles L. Bennett, Hau C. Kwaan   +7 more
doaj   +1 more source

Comparative 3‐Year Allograft Outcomes for Recipients of Kidneys From SARS‐CoV‐2 NAT‐Positive Donors

Transplant Infectious Disease, EarlyView.
In this retrospective single‐center study of 220 kidney transplants from SARS‐CoV‐2 NAT+, NAT+ with COVID as cause of death (CoV‐COD) and NATneg donors, we found no significant difference in rejection or kidney pathologies at 1 year, and death, graft loss, or eGFR at 3 years by CoV‐donor status.
Christine E. Koval, Mohamed Eltemamy, Nabin K. Shrestha, Leal Herlitz, Emilio D. Poggio, Alvin C. Wee   +5 more
wiley   +1 more source