Intramedullary multiple hematomas in siblings with congenital alpha-2-plasmin inhibitor deficiency: orthopedic surgery with protection by tranexamic acid.
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.
Takahashi
Y
Y
Department of Pediatrics, Nara Medical College, Kashihara, Japan.
Tanaka
T
T
Nakajima
N
N
Yoshioka
A
A
Fukui
H
H
Miyauchi
Y
Y
Mii
Y
Y
Tamai
S
S
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*
D000979:alpha-2-Antiplasmin; D014148:Tranexamic Acid