Results 61 to 70 of about 61,384 (225)

Case Report: Scleroderma Renal Crisis Presenting as Thrombotic Microangiopathy

open access: yesTurkish Journal of Nephrology, 2019
Scleroderma is a systemic autoimmune disease of unknown origin characterized by excessive deposition of collagen and other connective tissue macromolecules in multiple organs. It can cause thrombotic microangiopathy.
Mehmet Can UĞUR   +6 more
doaj  

Prognostic factors in thrombotic thrombocytopenic purpura

open access: yesTürk Biyokimya Dergisi, 2022
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy with no standardized prognostic model to predict mortality. The aim of the study is to determine parameters associated with TTP-related mortality.
Yalçıner Merih, İlhan Osman
doaj   +1 more source

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

open access: yesBritish 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   +3 more
wiley   +1 more source

Renal‐vascular axis: unmasking its role in vascular endothelial growth factor‐inhibitor vascular toxicity in cancer patients

open access: yesBritish Journal of Pharmacology, EarlyView.
Abstract Vascular toxicity is a growing concern in cancer patients receiving vascular endothelial growth factor inhibitor (VEGFi) therapy, posing a significant threat to patient prognosis. While the primary mechanism of VEGFi‐induced vascular toxicity is linked to redox‐sensitive reactions that disrupt vascular tone, leading to hypertension and ...
Grace Whelan, Karla B. Neves
wiley   +1 more source

The relationship between ADAMTS13 genotype and phenotype in congenital thrombotic thrombocytopenic purpura and characterisation of ADAMTS13 mutants [PDF]

open access: yes, 2015
Congenital thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, usually involving ADAMTS13 gene defects. ADAMTS13 processes the multimeric plasma glycoprotein Von Willebrand factor making it less reactive to platelets.
Underwood, MI
core  

Inborn errors of immunity in children with neuroinflammation

open access: yesDevelopmental 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   +5 more
wiley   +1 more source

Thrombotic Microangiopathy‐Like Phenotype in Patients With Infection‐Associated Disseminated Intravascular Coagulation Treated With Thrombomodulin Alfa

open access: yesEuropean Journal of Haematology, EarlyView.
ABSTRACT Despite disseminated intravascular coagulation (DIC) and thrombotic microangiopathy (TMA) sharing features of thrombocytopenia, organ dysfunction, and bleeding, the relationship between these two conditions remains unclear. We therefore conducted a post hoc analysis of post‐marketing surveillance data from Japan to evaluate the clinical ...
Naoki Takezako   +11 more
wiley   +1 more source

Hematopoietic cell transplantation-associated thrombotic microangiopathy: a review of pathophysiology, diagnosis, and treatment [PDF]

open access: yes, 2016
Joseph Rosenthal Department of Pediatrics, City of Hope National Medical Center, Duarte, CA, USA Abstract: Transplant-associated thrombotic microangiopathy (TA-TMA) is a multifactorial disorder caused by systemic vascular endothelial injury that can be ...
Rosenthal J
core  

Expert Consensus on the Diagnosis and Monitoring of Paroxysmal Nocturnal Hemoglobinuria (PNH): An Algorithmic Approach in an Era of New Treatments

open access: yesEuropean Journal of Haematology, EarlyView.
ABSTRACT Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder caused by a defect of glycosylphosphatidyl‐anchored proteins, leading to an uncontrolled complement‐mediated hemolysis. The advent of complement inhibitors in clinical practice radically changed patients' outcomes and survival.
Bruno Fattizzo   +8 more
wiley   +1 more source

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