title: Long-term major joint outcomes in young adults with haemophilia: interim data from the HGDS.
abstract: A study of major joint outcomes, specifically range of motion and synovitis, was conducted with data from a subset of adolescents enrolled in the prospective Hemophilia Growth and Development Study (HGDS). Clinical observations were carried out over a 7-year period from 1989 to 1996. A secondary aim was to gain insight into factors that might influence decisions regarding maintaining or discontinuing prophylaxis during early adulthood. Twenty-nine participants (median age 17.4 at entry) were included. Median follow-up was 7 years (range: 4.8-7.7). Range of motion (ROM) and synovitis in six major joints (knees, elbows and ankles), were evaluated by physical examination every 6-12 months. At the baseline observation, 73.6% of joints showed no ROM abnormalities or synovitis, and all joints were normal in 11 patients. Of the 11 participants, 54.5% developed abnormalities and 28.1% of normal joints at baseline became abnormal during the follow-up. Ankles were the most severely affected and had persistent progression during late adolescence and adulthood. Elbows and knees did not show progression after the first few years of the follow-up. The progression of haemophilic arthropathy in adolescents and young adults varies from individual to individual and also in the site of affected joints. In view of this, the decision regarding discontinuation of prophylaxis in patients with haemophilia should be individualized.
authors.affiliation: Division of Hematology/Oncology, Childrens Hospital Los Angeles, Los Angeles, CA, USA.
authors.forename: Y
authors.initials: Y
authors.lastname: Su
authors.forename: W-Y
authors.initials: WY
authors.lastname: Wong
authors.forename: A
authors.initials: A
authors.lastname: Lail
authors.forename: S M
authors.initials: SM
authors.lastname: Donfield
authors.forename: S
authors.initials: S
authors.lastname: Konzal
authors.forename: E
authors.initials: E
authors.lastname: Gomperts
mesh_terms: D000293:Adolescent; D000328:Adult; D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D002648:Child; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D007592:Joint Diseases / Q000209:etiology* / Q000517:prevention & control; D008137:Longitudinal Studies; D011446:Prospective Studies; D016059:Range of Motion, Articular / Q000502:physiology; D013585:Synovitis / Q000517:prevention & control / Q000523:psychology*; D016896:Treatment Outcome
chemical_list: D001779:Blood Coagulation Factors