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| http://dx.doi.org/10.1016/j.thromres.2020.07.034 | DOI Listing |
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Injury induced endotheliopathy: overview, diagnosis, and management.
Curr Opin Crit Care
January 2025
Shock Trauma Center, University of Maryland School of Medicine, Baltimore, Maryland, USA.
Purpose Of Review: This review aims to examine recent advances in the understanding of injury-induced endotheliopathy and therapeutics to mitigate its development in critically injured patients.
Recent Findings: Clinical studies have clearly demonstrated that syndecan-1 ectodomains can be found in circulation after various types of trauma and injury and correlates with worse outcomes. As the mechanisms of endotheliopathy are better understood, pathologic hyperadhesive forms of von Willebrand factor, along with a relative deficiency of its cleaving enzyme, a disintegrin and metalloprotease with thrombospondin type I motifs, member 13 (ADAMTS13), have emerged as additional biomarkers.
Evaluating the potential for iodinated radiocontrast agents to interfere with ADAMTS13 activity testing via fluorescence resonance energy transfer methodology.
Am J Clin Pathol
December 2024
Special Coagulation Laboratory, Division of Hematopathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, US.
Objectives: Fluorescence resonance energy transfer (FRET)-based ADAMTS13 activity assays are critical for the diagnosis of thrombotic thrombocytopenic purpura. However, these assays are susceptible to interference. As iodide has been suggested to interfere in laboratory testing via fluorophore quenching or promotion, we aimed to determine whether iodinated contrast (Omnipaque) interferes with the ATS-13 ADAMTS13 Activity Assay 2.
View Article and Find Full Text PDFHereditary thrombotic thrombocytopenic purpura mimicking immune thrombocytopenia was revealed by miscarriage-novel compound heterozygous mutations in hTTP.
BMC Med Genomics
November 2024
Department of Hematology, Guangzhou First People's Hospital, Guangzhou, 510080, China.
We report a case of early-onset hereditary thrombotic thrombocytopenic purpura in a 16-year-old girl who suffered from thrombocytopenia and was misdiagnosed with immune thrombocytopenia for years until two failed gestations finally revealed the underlying cause. The novel compound heterozygous mutation c.2865G > A:p.
View Article and Find Full Text PDFThrombotic thrombocytopenic purpura in a patient with adult-onset Still's disease: report of a rare entity.
BMJ Case Rep
November 2024
Hematology/Oncology, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA.
Adult-onset Still's disease (AOSD) is a rare, systemic inflammatory disorder characterised by daily fever, arthritis, a salmon-pink rash and leucocytosis. Thrombotic thrombocytopenic purpura (TTP) is included in the class of thrombotic microangiopathies and manifests clinically as microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and ischaemic tissue injury secondary to microthrombi. TTP is caused by either an autoimmune, congenital or idiopathic deficiency of ADAMTS13 and carries a high mortality rate.
View Article and Find Full Text PDFLaboratory Testing for ADAMTS13 for Thrombotic Thrombocytopenia Purpura and Beyond.
Semin Thromb Hemost
October 2024
Section of Clinical Biochemistry, University of Verona, Verona, Italy.
ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), also called von Willebrand factor (VWF) cleaving protease, acts as a moderator of VWF activity. ADAMTS13 cleaves VWF multimers, thereby reducing VWF activity in blood. When ADAMTS13 is absent (e.
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