Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders
Abstract 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 patien...
| Main Authors: | , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2023-06-01
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| Series: | Orphanet Journal of Rare Diseases |
| Online Access: | https://doi.org/10.1186/s13023-023-02787-2 |
| _version_ | 1827910579305578496 |
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| author | Mathieu Fiore Janine-Sophie Giraudet Marie-Christine Alessi Céline Falaise Dominique Desprez Roseline d’Oiron Sophie Voisin Marie-Françoise Hurtaud Hélène Boutroux Paul Saultier Cécile Lavenu-Bombled Gilles Bagou Xavier Dubucs Anthony Chauvin Christophe Leroy Francine Meckert François Kerbaul Nicolas Giraud Ambra Pühler Ana Rath |
| author_facet | Mathieu Fiore Janine-Sophie Giraudet Marie-Christine Alessi Céline Falaise Dominique Desprez Roseline d’Oiron Sophie Voisin Marie-Françoise Hurtaud Hélène Boutroux Paul Saultier Cécile Lavenu-Bombled Gilles Bagou Xavier Dubucs Anthony Chauvin Christophe Leroy Francine Meckert François Kerbaul Nicolas Giraud Ambra Pühler Ana Rath |
| author_sort | Mathieu Fiore |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-03-13T01:52:45Z |
| format | Article |
| id | doaj.art-afdd1c0969c24287ad8dac6e3b9a780a |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-03-13T01:52:45Z |
| publishDate | 2023-06-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-afdd1c0969c24287ad8dac6e3b9a780a2023-07-02T11:26:41ZengBMCOrphanet Journal of Rare Diseases1750-11722023-06-011811810.1186/s13023-023-02787-2Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disordersMathieu Fiore0Janine-Sophie Giraudet1Marie-Christine Alessi2Céline Falaise3Dominique Desprez4Roseline d’Oiron5Sophie Voisin6Marie-Françoise Hurtaud7Hélène Boutroux8Paul Saultier9Cécile Lavenu-Bombled10Gilles Bagou11Xavier Dubucs12Anthony Chauvin13Christophe Leroy14Francine Meckert15François Kerbaul16Nicolas Giraud17Ambra Pühler18Ana Rath19Laboratoire d’hématologie, Centre de Référence des Pathologies Plaquettaires, CHU de Bordeaux, Hôpital Cardiologique, Inserm U1034 – Biologie des Maladies Cardio-VasculairesORPHANET, INSERM US14, Plateforme Maladies RaresLaboratory of Hematology, Aix Marseille Univ, APHM, INSERM, INRAe, C2VN, La Timone HospitalDepartment of Pediatric Hematology, Immunology and Oncology, La Timone Children’s HospitalCentre de Ressources et de Compétences des Maladies Hémorragiques Constitutionnelles, CHU de StrasbourgCentre de Ressources et de Compétences des Maladies Hémorragiques Constitutionnelles, CHU du Kremlin-BicêtreLaboratoire d’Hématologie, Centre de Référence des Pathologies Plaquettaires, CHU de ToulouseLaboratoire d’Hématologie, CHU Robert DebréLaboratoire d’Hématologie, Centre de Référence des pathologies Plaquettaires, CHU Armand TrousseauDepartment of pediatric hematology, immunology and oncology, Aix Marseille Univ, APHM, INSERM, INRAe, C2VN, La Timone Children’s HospitalService Hématologie Biologique, Centre de ressources et compétences MHEMO, CHU Bicêtre, Assistance Publique-Hôpitaux de Paris, Faculté de médecine Paris SaclayAnesthésiste-Réanimateur Urgentiste - SAMU-SMUR de Lyon - Hôpital Edouard-HerriotPôle Médecine-Urgences, CHU de ToulousePrésident de la Commission des Référentiels de la SFMU (CREF), Chef de Service Adjoint - Service d’Accueil des Urgences/SMUR, CHU Lariboisière, Université de ParisMédecin Urgentiste - Service de Gestion des Crises Sanitaires - Département Qualité Gestion des Risques, Assistance Publique-Hôpitaux de ParisDirection Opérationnelle du Prélèvement et de la Greffe de l’Agence de la Biomédecine (ABM)Direction Opérationnelle du Prélèvement et de la Greffe de l’Agence de la Biomédecine (ABM)Association Française des HémophilesORPHANET, INSERM US14, Plateforme Maladies RaresORPHANET, INSERM US14, Plateforme Maladies RaresAbstract 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.https://doi.org/10.1186/s13023-023-02787-2 |
| spellingShingle | Mathieu Fiore Janine-Sophie Giraudet Marie-Christine Alessi Céline Falaise Dominique Desprez Roseline d’Oiron Sophie Voisin Marie-Françoise Hurtaud Hélène Boutroux Paul Saultier Cécile Lavenu-Bombled Gilles Bagou Xavier Dubucs Anthony Chauvin Christophe Leroy Francine Meckert François Kerbaul Nicolas Giraud Ambra Pühler Ana Rath Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders Orphanet Journal of Rare Diseases |
| title | Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders |
| title_full | Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders |
| title_fullStr | Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders |
| title_full_unstemmed | Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders |
| title_short | Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders |
| title_sort | emergency management of patients with glanzmann thrombasthenia consensus recommendations from the french reference center for inherited platelet disorders |
| url | https://doi.org/10.1186/s13023-023-02787-2 |
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