Body Composition, Hemophilia, Hemophilic Arthropathy, Osteoporosis, Sarcopenia, Vitamin D Deficiency
Conditions
Brief summary
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.
Detailed description
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.
Interventions
None listed
Sponsors
Tsung-Ying Li
Study design
Observational model
CASE_CONTROL
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
MALE
Age
20 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* Hemophilia patients
Exclusion criteria
* Severe diseases affecting bone metabolism (such as primary hyperparathyroidism, hypogonadism), * History of total hip arthroplasty * History of instrumentation in lumbar spine
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Vitamin D | one day | serum vitamin D |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Bone marrow density | one day | Bone marrow density of lumbar spine and bilateral hip by dual X-ray absorptiometry (DXA) |
| Body composition | one day | using whole-body DXA (GE Lunar iDXA, GE Healthcare Lunar, Madison, Wisconsin, U.S.A.) |
| Pettersson score | one day | 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. |
| Hemophilia Joint Health Score | one day | 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. |
| Handgrip strength | one day | 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. |
Countries
Taiwan
Outcome results
None listed