title: Intramedullary multiple hematomas in siblings with congenital alpha-2-plasmin inhibitor deficiency: orthopedic surgery with protection by tranexamic acid.
abstract: Congenital alpha 2-plasmin inhibitor deficiency is very rare, but causes bleeding problems similar to hemophilia. Three young Japanese sisters affected with congenital alpha 2-plasmin inhibitor deficiency have been reported by us earlier. Recently we encountered a particular form of intramedullary multiple hematomas in the long bones in all of them which was not reported previously. In 2 of the sisters orthopedic operations were successfully performed by using an antiplasmin reagent: tranexamic acid. In this paper we describe the characteristics of the hematomas and the hemostatic management with tranexamic acid.
authors.affiliation: Department of Pediatrics, Nara Medical College, Kashihara, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Takahashi
authors.forename: T
authors.initials: T
authors.lastname: Tanaka
authors.forename: N
authors.initials: N
authors.lastname: Nakajima
authors.forename: A
authors.initials: A
authors.lastname: Yoshioka
authors.forename: H
authors.initials: H
authors.lastname: Fukui
authors.forename: Y
authors.initials: Y
authors.lastname: Miyauchi
authors.forename: Y
authors.initials: Y
authors.lastname: Mii
authors.forename: S
authors.initials: S
authors.lastname: Tamai
mesh_terms: D000293:Adolescent; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D001847:Bone Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D002648:Child; D005260:Female; D005269:Femur; D006406:Hematoma / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D006801:Humans; D006811:Humerus; D011859:Radiography; D011884:Radius; D014148:Tranexamic Acid / Q000627:therapeutic use*; D000979:alpha-2-Antiplasmin / Q000172:deficiency*
chemical_list: D000979:alpha-2-Antiplasmin; D014148:Tranexamic Acid