A5079286125- Platelet Disorders and Treatments
- Blood properties and coagulation
- Hemophilia Treatment and Research
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Blood groups and transfusion
- Cell Adhesion Molecules Research
- Blood Coagulation and Thrombosis Mechanisms
- Chronic Lymphocytic Leukemia Research
- Heparin-Induced Thrombocytopenia and Thrombosis
- Neuroscience and Neuropharmacology Research
- Blood disorders and treatments
- Autoimmune Bullous Skin Diseases
- Adipose Tissue and Metabolism
- Receptor Mechanisms and Signaling
- Skin and Cellular Biology Research
- Renal Diseases and Glomerulopathies
- Venous Thromboembolism Diagnosis and Management
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Acute Myeloid Leukemia Research
- Glycosylation and Glycoproteins Research
- Inflammatory Bowel Disease
- Chronic Myeloid Leukemia Treatments
- Ocular Diseases and Behçet’s Syndrome
- Immunodeficiency and Autoimmune Disorders
- Neurotransmitter Receptor Influence on Behavior
Hospital La Paz Institute for Health Research
2014-2025
Hospital Universitario La Paz
2016-2025
Centro de Investigaciones Biológicas Margarita Salas
1993-2008
Consejo Superior de Investigaciones Científicas
1993-2008
Centro de Investigación Biomédica en Red
2008
Instituto de Investigación de Enfermedades Raras
2008
Consejo Nacional de Investigaciones Científicas y Técnicas
1986-1994
University of Buenos Aires
1990-1994
Excessive bleeding at surgery is a feared complication in patients with inherited platelet disorders. However, very few studies have evaluated the frequency of surgical these hemorrhagic We performed worldwide, multicentric, retrospective study to assess complications surgery, preventive and therapeutic approaches adopted, their efficacy disorders: Surgery Platelet disorders And Therapeutic Approach (SPATA) study. rated outcome 829 procedures carried out 423 well-defined forms 238 function...
Despite their low platelet count some immune thrombocytopenia (ITP) patients seldom bleed, indicating the presence of factors to compensate thrombocytopenia. Moreover, ITP may have an increased risk for thrombosis. These facts suggest procoagulant mechanisms that not been clarified yet. The aim this study was identify these possible factors. utility rotational thromboelastometry (ROTEM® ) test haemostasis in also evaluated. Patients with presented a profile due amount platelet- and red...
Abstract The treatment goal for patients with immune thrombocytopaenia (ITP) is to raise platelet counts levels that minimize or stop bleeding. Thrombopoietin receptor agonists (TPO-RAs) have been successfully and extensively employed as second-line therapy ITP. However, TPO-RAs a small but significant increase in the risk of thrombosis. aim this study was elucidate mechanisms involved pro-coagulant effect take them into account when considering their use ITP concomitant diseases/conditions...
Multifactorial mechanisms leading to diminished platelet counts in immune thrombocytopaenia (ITP) might condition the ability of patients with ITP respond treatments. Examining their and features, we aimed detect singular characteristics who do not any treatment. We studied chronic primary had been without treatment, or untreated (UT-ITP), for at least six months; included were responders agonists thrombopoietin receptors (TPO-RA), showed no response first- second-line treatments (NR-ITP),...
The heterogeneous manifestations of MYH9-related disorder (MYH9-RD), characterized by macrothrombocytopenia, Döhle-like inclusion bodies in leukocytes, bleeding variable severity with, some cases, ear, eye, kidney, and liver involvement, make the diagnosis for these patients still challenging clinical practice. We collected phenotypic data analyzed genetic variants more than 3,000 with a or platelet disorder. Patients were enrolled BRIDGE-BPD ThromboGenomics Projects their samples processed...
Summary Despite the efficacy of splenectomy for chronic immune thrombocytopenia (ITP), its considerable failure rate and possible related complications prove need further research into potential predictors response. The platelet sequestration site determined by 111 In‐labelled autologous scintigraphy has been proposed to predict outcome, but without standardisation in clinical practice. Here, we conducted a single‐centre study analysing cohort splenectomised patients with ITP whom...
Abstract Background Inpatients with Inflammatory Bowel Disease (IBD) are at risk for thromboembolic events, but the flaring outpatients is less understood1. Global Coagulation Assays (GCA) (Thrombin generation assay using Calibrated Automated Thrombogram [CAT] and Rotational Thromboelastometry [ROTEM]) have emerged as aiding tools in assessment of hypercoagulable states2. Methods Cross sectional, case-control study IBD outpatients, both Crohn’s (CD) Ulcerative Colitis (UC). Clinical activity...
X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection and neoplasia (XMEN) disease is caused by hemizygous loss of function (LOF) gene variants in MAGT1. MAGT1 a plasma membrane transporter (Mg2+) that plays relevant role immune responses acts as second messenger intracellular signaling, but also it involved the glycosylation proteins. Here we report two from different families XMEN disease. A de novo variant c.97_98 delinsC affecting one member family three...
Thrombopoietin receptor agonists (TPO-RA) have recently been introduced for the treatment of immune thrombocytopenia (ITP), an anti-platelet-antibodies autoimmune disease. The observation a low frequency bleeding episodes despite their suggests existence compensatory mechanism. This study aimed to evaluate effect TPO-RA on platelet function and procoagulant state in ITP patients before (ITP-bR) after responding (ITP-aR) treatment. Plasma- microparticle (MP)-associated capacity from was...
Abstract Background Tofacitinib, a small-molecule Janus kinase inhibitor (JAKi), has been shown to be effective in the treatment of ulcerative colitis (UC). Despite its benefits, higher incidence thromboembolic events reported patients treated with tofacitinib. It suggested that this effect might class and could affect other JAKi. Consequently, regulatory agencies warned about risks restricted conditions use. Therefore, it would highly relevant understand pathophysiological mechanism...
Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a genodermatosis characterized by severe cutaneous and mucosal fragility, frequently complicated multifactorial chronic anemia that responds poorly to conventional therapies. This cross-sectional study investigates the factors contributing in RDEB analyzing representative cohort, was stratified disease severity, iron status, examine their hematological parameters, cytokine profile erythropoietin (EPO)-erythroferrone (ERFE)-hepcidin axis....
Background: Tofacitinib is effective for refractory ulcerative colitis (UC), a chronic inflammatory disease of the colonic mucosa. However, its use has been associated with an increased risk thromboembolic events, prompting regulatory restrictions. Understanding pathophysiological mechanisms contributing to these potential risks critical patient safety. We aim evaluate and compare ex vivo effects tofacitinib anti-TNF on coagulation parameters platelet function. Methods: Whole blood...
Abstract Background Podocalyxin (podxl) is a heavily glycosylated transmembrane protein mainly found on the apical membrane of rat podocytes and also in endothelial, hematopoietic, tumor cells. Despite its interest no much known about transcriptional regulation podxl different Thus, we aimed at studying functional features 5'-regulatory region human Podxl gene. Results The promoter gene has been cloned structure function were analyzed. primary DNA sequence rich G+C devoid TATA or CAAT boxes....
<h3>Background:</h3> Patients with Crohn's disease have an increased risk for systemic thromboembolism. Their platelets are hyperactive and possess elevated endogenous content of CD40 ligand (CD40L), a tumour necrosis factor α family protein member. Under basal conditions after stimulation, these express more CD40L on their surface release higher amounts soluble (s)CD40L than control platelets, through mechanism that might be mediated by matrix metalloproteinases (MMPs). <h3>Objective:</h3>...
Summary Thrombocytopenia has been identified as a common complication of severe acute respiratory syndrome coronavirus‐2 (SARS‐CoV‐2) infection in the general population. In an attempt to determine impact coronavirus disease 2019 (COVID‐19) patients with immune thrombocytopenia (ITP), retrospective single‐centre study was performed. Thrombocytosis observed chronic ITP after SARS‐CoV‐2 infection, frequently needing treatment adjustment or even discontinuation therapy. Relapses and newly...
Platelets are the major source of plasma-soluble CD40L (sCD40L), an important inflammatory mediator. This study explored impact platelet-derived sCD40L on Behçet disease (BD), autoinflammatory vasculitis. We also searched for influences by platelet matrix metalloproteinases (MMP) -2 and MMP-9, implicated in several diseases, shedding from membrane. Platelet activation were studied flow cytometry aggregometry, surface expression platelet-leukocyte aggregates cytometry, ELISA, cellular CD40...