Hemophilia is associated with osteoporosis and increased prevalence of low bone mineral density (BMD). Many clinical risk factors have been reported, such as hemophilic arthropathy, reduced physical activity, decreased sun exposure, hepatitis C virus infections, vitamin D deficiency, and low body mass index (BMI). There is no data on the prevalence of vitamin D deficiency and low BMD in hemophilia patients in Taiwan or Asia. To the best of our knowledge, no previous studies have reported the prevalence of sarcopenia and correlation with osteoporosis in hemophilia adult patients. The purpose of this study will evaluate the prevalence of vitamin D deficiency, low BMD, sarcopenia and body composition in a cohort of patients with hemophilia in Taiwan.
Hemophilia is associated with osteoporosis and increased prevalence of low bone mineral density (BMD). Soucek et al. reported sarcopenia and low trabecular BMD at the radius in the boys with hemophilia by CT.The severity of hemophilic arthropathy associated with lower BMD, muscle atrophy and obesity has been shown by some studies. This is a single-center, cross-sectional, observational study. All participants will sign informed consent that fully disclosed the risks and benefits of study participation. The study will be performed at the Hemophilia Care and Research Center. Sixty adult patients (age ≥ 20) with hemophilia and 20 healthy subjects will be enrolled for scanning of the lean tissue, appendicular skeletal muscle mass (ASM), fat mass, lumbar spine and hip BMD by dual-energy X-ray absorptiometry (DXA) and assess serum vitamin D level. We will collect other clinically relevant information, including age, body mass index (BMI), hemophilia type and severity, inhibitor titer, Pettersson score by x-ray, HJHS, handgrip strength, history of HCV or HIV infection and history of fracture.
Study Type
OBSERVATIONAL
Enrollment
80
Hemophilia care and research center
Taipei, Taiwan
Vitamin D
serum vitamin D
Time frame: one day
Bone marrow density
Bone marrow density of lumbar spine and bilateral hip by dual X-ray absorptiometry (DXA)
Time frame: one day
Body composition
using whole-body DXA (GE Lunar iDXA, GE Healthcare Lunar, Madison, Wisconsin, U.S.A.)
Time frame: one day
Pettersson score
Bilateral shoulder, elbow, hip, knee, ankle joints score by x-ray. The Pettersson score ranges from 0 (normal) to 13 (most severe) and is according to the following eight radiographic features of joints: osteoporosis, osteophytes, narrowing of joint space, subchondral irregularity, subchondral cyst formation, erosion of joint margins, and bone remodelling.
Time frame: one day
Hemophilia Joint Health Score
Functional and structured score of bilateral elbow, knee and ankle. The score for each joint is the sum of several elements including swelling (0-3), duration of swelling (0-1), muscle atrophy (0-2), crepitus of motion (0-2), flexion loss and extension loss (0-6), joint pain (0-2) and strength (0-4). A score of 20 may indicate the most severe damage/impairment in the evaluated joint and a score of zero corresponds to no identifiable joint impairment.
Time frame: one day
Handgrip strength
Handgrip strength (kg) will be measured using the same standard calibrated hand dynamometers (Baseline® Hand Dynamometer - Digital LCD Gauge, Fabrication Enterprises Inc. White plains, New York, USA). We will calculate the mean of three maximal grip-strength measurement in each hand.
Time frame: one day
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