Females with FVIII and FIX deficiency have reduced joint range of motion.
Little is known about rates of joint bleeding among females with FVIII/FIX deficiency or hemophilia carriers. In a cross-sectional study, we tested the hypothesis that females with FVIII or FIX deficiency enrolled in the Universal Data Collection (UDC) project had a reduced mean overall joint range of motion (ROM) compared with historic controls from the Normal Joint Study. Demographics, clinical characteristics, and joint ROM measurements on 303 females without a bleeding disorder and 148 females with FVIII and FIX deficiency, respectively, between the ages of 2-69 years and a body mass index (BMI) ≤ 35 were compared. Multivariate linear regression was performed with the overall joint ROM (sum of the right and left ROM measurements of five joints) as the dependent variable and FVIII or FIX activity as the independent variable adjusting for age, race, BMI, and number of joint bleeds reported over the last 6 months. As FVIII and FIX activity decreased, the mean overall joint ROM became reduced and in most cases was significantly lower than that of the controls regardless of age and clinical hemophilia severity. Further investigation of reduced joint ROM as evidence of subclinical joint bleeding in females with FVIII and FIX deficiency is warranted.
Department of Pediatrics, Division of Hematology/Oncology, Vanderbilt University Medical Center, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, Tennessee.
Robert F
RF
Sidonio
Fatima D
FD
Mili
Tengguo
T
Li
Connie H
CH
Miller
William C
WC
Hooper
Michael R
MR
DeBaun
Michael
M
Soucie
D000293:Adolescent; D000328:Adult; D000368:Aged; D015992:Body Mass Index; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003710:Demography; D005164:Factor IX / Q000378:metabolism; D005169:Factor VIII / Q000378:metabolism; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000503:physiopathology*; D002836:Hemophilia B / Q000097:blood / Q000503:physiopathology*; D006801:Humans; D007596:Joints / Q000503:physiopathology*; D008875:Middle Aged; D016059:Range of Motion, Articular; D012720:Severity of Illness Index
D005169:Factor VIII; D005164:Factor IX