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Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders.
Fiore, Mathieu; Giraudet, Janine-Sophie; Alessi, Marie-Christine; Falaise, Céline; Desprez, Dominique; d'Oiron, Roseline; Voisin, Sophie; Hurtaud, Marie-Françoise; Boutroux, Hélène; Saultier, Paul; Lavenu-Bombled, Cécile; Bagou, Gilles; Dubucs, Xavier; Chauvin, Anthony; Leroy, Christophe; Meckert, Francine; Kerbaul, François; Giraud, Nicolas; Pühler, Ambra; Rath, Ana.
Afiliación
  • Fiore M; Laboratoire d'hématologie, Centre de Référence des Pathologies Plaquettaires, CHU de Bordeaux, Hôpital Cardiologique, Inserm U1034 - Biologie des Maladies Cardio-Vasculaires, Pessac, France. mathieu.fiore@chu-bordeaux.fr.
  • Giraudet JS; Centre de Référence des Pathologies Plaquettaires, Pessac, France. mathieu.fiore@chu-bordeaux.fr.
  • Alessi MC; ORPHANET, INSERM US14, Plateforme Maladies Rares, 96 Rue Didot, 75014, Paris, France.
  • Falaise C; Laboratory of Hematology, Aix Marseille Univ, APHM, INSERM, INRAe, C2VN, La Timone Hospital, Marseille, France.
  • Desprez D; Reference Center of Platelet Disorders, APHM, Marseille, France.
  • d'Oiron R; Department of Pediatric Hematology, Immunology and Oncology, La Timone Children's Hospital, Marseille, France.
  • Voisin S; Reference Center of Platelet Disorders, APHM, Marseille, France.
  • Hurtaud MF; Centre de Ressources et de Compétences des Maladies Hémorragiques Constitutionnelles, CHU de Strasbourg, Strasbourg, France.
  • Boutroux H; Centre de Ressources et de Compétences des Maladies Hémorragiques Constitutionnelles, CHU du Kremlin-Bicêtre, Le-Kremlin-Bicêtre, France.
  • Saultier P; Laboratoire d'Hématologie, Centre de Référence des Pathologies Plaquettaires, CHU de Toulouse, Toulouse, France.
  • Lavenu-Bombled C; Laboratoire d'Hématologie, CHU Robert Debré, Paris, France.
  • Bagou G; Laboratoire d'Hématologie, Centre de Référence des pathologies Plaquettaires, CHU Armand Trousseau, Paris, France.
  • Dubucs X; Department of pediatric hematology, immunology and oncology, Aix Marseille Univ, APHM, INSERM, INRAe, C2VN, La Timone Children's Hospital, Marseille, France.
  • Chauvin A; Reference Center of Platelet Disorders, APHM, Marseille, France.
  • Leroy C; Service Hématologie Biologique, Centre de ressources et compétences MHEMO, CHU Bicêtre, Assistance Publique-Hôpitaux de Paris, Faculté de médecine Paris Saclay, Le Kremlin-Bicetre, France.
  • Meckert F; Anesthésiste-Réanimateur Urgentiste - SAMU-SMUR de Lyon - Hôpital Edouard-Herriot, 69437, Lyon Cedex 03, France.
  • Kerbaul F; Pôle Médecine-Urgences, CHU de Toulouse, Toulouse, France.
  • Giraud N; Président de la Commission des Référentiels de la SFMU (CREF), Chef de Service Adjoint - Service d'Accueil des Urgences/SMUR, CHU Lariboisière, Université de Paris, Paris, France.
  • Pühler A; Médecin Urgentiste - Service de Gestion des Crises Sanitaires - Département Qualité Gestion des Risques, Assistance Publique-Hôpitaux de Paris, Paris, France.
  • Rath A; Direction Opérationnelle du Prélèvement et de la Greffe de l'Agence de la Biomédecine (ABM), Saint Denis, France.
Orphanet J Rare Dis ; 18(1): 171, 2023 Jun 29.
Article en En | MEDLINE | ID: mdl-37386449
Glanzmann thrombasthenia (GT) is a genetic bleeding disorder characterised by severely reduced/absent platelet aggregation in response to multiple physiological agonists. The severity of bleeding in GT varies markedly, as does the emergency situations and complications encountered in patients. A number of emergency situations may occur in the context of GT, including spontaneous or provoked bleeding, such as surgery or childbirth. While general management principles apply in each of these settings, specific considerations are essential for the management of GT to avoid escalating minor bleeding events. These recommendations have been developed from a literature review and consensus from experts of the French Network for Inherited Platelet Disorders, the French Society of Emergency Medicine, representatives of patients' associations, and Orphanet to aid decision making and optimise clinical care by non-GT expert health professionals who encounter emergency situations in patients with GT.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trombastenia / Medicina de Emergencia Tipo de estudio: Guideline / Prognostic_studies Límite: Humans Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2023 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Trombastenia / Medicina de Emergencia Tipo de estudio: Guideline / Prognostic_studies Límite: Humans Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2023 Tipo del documento: Article País de afiliación: Francia Pais de publicación: Reino Unido