Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders.
Mathieu FioreJanine-Sophie GiraudetMarie-Christine AlessiCéline FalaiseDominique DesprezRoseline d'OironSophie VoisinMarie-Françoise HurtaudHélène BoutrouxPaul SaultierCécile Lavenu-BombledGilles BagouXavier DubucsAnthony ChauvinChristophe LeroyFrancine MeckertFrançois KerbaulNicolas GiraudAmbra PühlerAna RathPublished in: Orphanet journal of rare diseases (2023)
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.
Keyphrases
- public health
- end stage renal disease
- emergency department
- healthcare
- atrial fibrillation
- newly diagnosed
- chronic kidney disease
- clinical practice
- decision making
- prognostic factors
- gene expression
- emergency medicine
- emergency medical
- coronary artery disease
- percutaneous coronary intervention
- acute coronary syndrome
- copy number
- patient reported