title: Successful arthroscopic treatment of pigmented villonodular synovitis of the knee in a patient with congenital deficiency of plasminogen activator inhibitor-1 and recurrent haemarthrosis.
abstract: We report the arthroscopic treatment of pigmented villonodular synovitis (PVNS) in a 13-year-old Japanese boy with congenital partial deficiency of plasminogen activator inhibitor-1 (PAI-1). He was admitted to our hospital with recurrent haemarthrosis of his right knee. Characteristic abnormalities of fibrinolysis included shortened euglobulin lysis time, low PAI-1 activity and low PAI-1 antigen levels. In addition, levels of "active PAI" in the plasma, which is a measure of total PAI bound to exogenous plasminogen activator, were very low. These parameters remained low after venous occlusion. The diagnosis of PVNS was established by synovial membrane biopsy, and arthroscopic synovectomy was performed with adjuvant administration of intravenous tranexamic acid. Subsequent bleeding episodes have been well controlled by oral administration of tranexamic acid on demand.
authors.affiliation: Department of Pediatrics, Nara Medical University Hospital, 840 Shijo-Cho, Kashihara, Nara 634-8522, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Matsui
authors.forename: Y
authors.initials: Y
authors.lastname: Takahashi
authors.forename: T
authors.initials: T
authors.lastname: Matsunaga
authors.forename: T
authors.initials: T
authors.lastname: Tanaka-Horie
authors.forename: H
authors.initials: H
authors.lastname: Minowa
authors.forename: M
authors.initials: M
authors.lastname: Sugimoto
authors.forename: R
authors.initials: R
authors.lastname: Tsukino
authors.forename: Y
authors.initials: Y
authors.lastname: Mii
authors.forename: J
authors.initials: J
authors.lastname: Giddings
authors.forename: A
authors.initials: A
authors.lastname: Yoshioka
mesh_terms: D000293:Adolescent; D001182:Arthroscopy / Q000379:methods; D005192:Family Health; D006395:Hemarthrosis / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007719:Knee Joint / Q000473:pathology* / Q000601:surgery; D008297:Male; D017395:Plasminogen Activator Inhibitor 1 / Q000172:deficiency*; D012008:Recurrence; D013586:Synovitis, Pigmented Villonodular / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D014148:Tranexamic Acid / Q000008:administration & dosage
chemical_list: D017395:Plasminogen Activator Inhibitor 1; D014148:Tranexamic Acid