Assessing bleeding risk in 18 children with Osteogenesis imperfecta.

Keywords: Osteogenesis imperfecta; bleeding disorders; vascular disease; platelet function tests; endothelial activation markers; hemorrhage EN Osteogenesis imperfecta bleeding disorders vascular disease platelet function tests endothelial activation markers hemorrhage 785 788 4 02/17/21 20210215 NES 210215 I Osteogenesis imperfecta i (OI) is a genetic disorder of connective tissue affecting bone formation and strength, resulting in susceptibility to spontaneous fracture. Based on Elbatarny's study, OI patients were classified according to their BS.3 BS < 3 correspond to patients with no evidence of bleeding history (OI NB for OI non-bleeders) and a BS >= 3 corresponds to patients with features of bleeding (OI B for OI bleeders). PHOTO (COLOR): 1 Determination of circulating VWF antigen level (A), VWF activity (B), secreted Thrombomodulin (C) and secreted E-selectin in OI group (D), OI NB and OI B subgroups. Platelet aggregation responses to several agonists expressed as maximal amplitude (% aggregation) (MA) after 4 min in the OI group, and in the OI NB and OI B subgroups. [Extracted from the article]