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J Infect Dev Ctries
September 2024
Department of Pediatric Hematology and Oncology, Faculty of Medicine, Usak University, Usak, Turkiye.
Clin Rheumatol
September 2024
Syndrome Des Anticorps Anti-Phospholipides Et Autres Maladies Auto-Immunes Systémiques Rares, Sorbonne Université, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Paris, France.
Pediatr Nephrol
January 2025
Department of Physiology, Radboud University Centre, Nijmegen, the Netherlands.
Vascular endothelial cells are equipped with numerous specialized granules called Weibel-Palade bodies (WPBs). They contain a cocktail of proteins that can be rapidly secreted (3-5 min) into the vascular lumen after an appropriate stimulus such as thrombin. These proteins are ready without synthesis.
View Article and Find Full Text PDFEur J Haematol
October 2024
Fundació Institut d'Investigació Sanitària Illes Balears (IdISBa), Hospital Universitario Son Espases, Palma de Mallorca, Balearic Islands, Spain.
Thrombotic microangiopathy (TMA), characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multisystem organ dysfunction, is a life-threatening disease. Patients with TMA who do not exhibit a severe ADAMTS-13 deficiency (defined as a disintegrin-like and metalloprotease with thrombospondin type 1 motif no. 13 activity ≥10%: TMA-13n) continue to experience elevated mortality rates.
View Article and Find Full Text PDFJ Thromb Haemost
October 2024
French Reference Center for Thrombotic Microangiopathies, Paris, France; Assistance Publique - Hôpitaux de Marseille (APHM), Centre Hospitalier Universitaire (CHU) Conception, Service de médecine interne et d'immunologie clinique, Marseille, France.
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