An international registry of patients with plasminogen deficiency (HISTORY).

An international registry of patients with plasminogen deficiency (HISTORY). Haematologica. 2020 Jan 30;: Authors: Shapiro AD, Menegatti M, Palla R, Boscarino M, Roberson C, Lanzi P, Bowen J, Nakar C, Janson IA, Peyvandi F Abstract Plasminogen deficiency is an ultra-rare multisystem disorder characterized by the development of fibrin-rich pseudomembranes on mucous membranes. Ligneous conjunctivitis, which can result in vision impairment or loss, is the most frequent symptom reported; affected systems may also include the respiratory tract, oropharynx, female reproductive tract, gingiva, middle ear, renal collecting system, skin and central nervous system. Untreated, plasminogen deficiency may result in significant reduction in quality of life and morbidity with potential life-threatening complications. Non-specific therapies are inadequate and plasminogen concentrates are not commercially available. Current understanding of plasminogen deficiency and management of disease symptoms and its progression is based on case reports/series and two small clinical trials. To date there has never been a comprehensive, international study to examine natural history or optimal therapeutic intervention; knowledge gaps include identification of contributing factors and triggers of disease manifestations, inability to predict disease course, and insufficient real-world data for use of therapeutics. We have created an international, observational study (HISTORY) in a lar...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research

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Source: Journal of Cardiovascular Medicine - Category: Cardiology Tags: Letter to the editor Source Type: research
Dynamic arterial elastance (Eadyn), the ratio between arterial pulse pressure and stroke volume changes during respiration, has been postulated as an index of the coupling between the left ventricle (LV) and the arterial system. We aimed to confirm this hypothesis using the gold-standard for defining LV contractility, afterload, and evaluating ventricular-arterial (VA) coupling and LV efficiency during different loading and contractile experimental conditions. Twelve Yorkshire healthy female pigs submitted to three consecutive stages with two opposite interventions each: changes in afterload (phenylephrine/nitroprusside), ...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
LOUISVILLE, Ky., April 6, 2020 /PRNewswire/ -- HEMA Biologics, LLC, ( " HEMA Biologics " ) today announced that the U.S. Food and Drug Administration (FDA) approved Sevenfact® [coagulation factor VIIa (recombinant)-jncw] as the first new bypassing...
Source: Drugs.com - New Drug Approvals - Category: Drugs & Pharmacology Source Type: news
Authors: Şahin DG, Akay OM, Uskudar Teke H, Andıc N, Gunduz E Abstract Chronic myeloproliferative neoplasms (MPN) are clonal disorders of hematopoietic stem/progenitor cell characterized by thrombohemorrhagic complications and a tendency to transform into acute leukemia. The pathogenesis of thrombosis in MPN is complex and results from a multifaceted interplay of clinical and disease-related factors. Rotational thromboelastometry (ROTEM) provides the complete and rapid information about all stages of the coagulation process. Here, we assess ROTEM parameters as a screening of coagulation profile in patients with M...
Source: Platelets - Category: Hematology Tags: Platelets Source Type: research
Adding clopidogrel added no efficacy but caused additional bleeding episodes.
Source: WebMD Health - Category: Consumer Health News Source Type: news
Abstract Background: Atrial fibrillation (AF) ablation under uninterrupted warfarin use is safe and recommended by experts. However, there is some controversy regarding direct-acting oral anticoagulants for the same purpose. Objective: To evaluate the safety of AF ablation under uninterrupted anticoagulation with rivaroxaban. Methods: A series of 130 patients underwent AF radiofrequency ablation under uninterrupted rivaroxaban use (RIV group) and was compared to a control group of 110 patients under uninterrupted warfarin use (WFR group) and therapeutic International Normalized Ratio (INR). We analyzed death, rates of thro...
Source: Arquivos Brasileiros de Cardiologia - Category: Cardiology Source Type: research
Abstract Background: Atrial fibrillation (AF) ablation under uninterrupted warfarin use is safe and recommended by experts. However, there is some controversy regarding direct-acting oral anticoagulants for the same purpose. Objective: To evaluate the safety of AF ablation under uninterrupted anticoagulation with rivaroxaban. Methods: A series of 130 patients underwent AF radiofrequency ablation under uninterrupted rivaroxaban use (RIV group) and was compared to a control group of 110 patients under uninterrupted warfarin use (WFR group) and therapeutic International Normalized Ratio (INR). We analyzed death, rates of thro...
Source: Arquivos Brasileiros de Cardiologia - Category: Cardiology Source Type: research
Conditions:   COVID;   Acute Coronary Syndrome;   Myocardial Infarction;   Myocarditis;   Venous Thromboembolism;   Deep Vein Thrombosis;   Pulmonary Embolism Intervention:   Sponsor:   Centre Hospitalier Universitaire de Nice Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
First new bypassing agent approved for the treatment and control of bleeding episodes in hemophilia A and B patients with inhibitors in over 2 decades Median number of infusions required to achieve bleeding control in the first 12 hours was 1 (225 mcg/k... Biopharmaceuticals, FDA HEMA Biologics, SEVENFACT, coagulation factor VIIa, hemophilia
Source: HSMN NewsFeed - Category: Pharmaceuticals Source Type: news
ConclusionVascular complications are still not negligible procedure ‐related complications during catheter ablation in the current era. CTA provides a rapid and precise diagnosis for optimal treatment strategies.This article is protected by copyright. All rights reserved.
Source: Journal of Cardiovascular Electrophysiology - Category: Cardiology Authors: Tags: ORIGINAL ARTICLE Source Type: research
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