Vitamin D Deficiency and Body Composition in the Patients With Hemophilia

Association of Vitamin D Deficiency, Sarcopenia, Body Composition, Osteoporosis and Hemophilic Arthropathy in the Patients With Hemophilia

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.

Study Overview

• Status: Completed
• Conditions: Hemophilia; Vitamin D Deficiency; Body Composition; Sarcopenia; Osteoporosis; Hemophilic Arthropathy

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.

• Study Type: Observational
• Enrollment (Actual): 80

Contacts and Locations

Study Locations

• Taiwan
  • Taipei, Taiwan, 114
    • Hemophilia care and research center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 20 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Non-Probability Sample

Study Population

60 hemophilia patients and 20 healthy subjects

Description

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

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort
Hemophilia Group; Hemophilia adult patients
Control Group; Healthy adult subjects

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Vitamin D	serum vitamin D	one day

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Bone marrow density	Bone marrow density of lumbar spine and bilateral hip by dual X-ray absorptiometry (DXA)	one day
Body composition	using whole-body DXA (GE Lunar iDXA, GE Healthcare Lunar, Madison, Wisconsin, U.S.A.)	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.	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.	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.	one day

Collaborators and Investigators

• Sponsor: Tsung-Ying Li
• Investigators: Principal Investigator: Tsung Ying Li, Dr, No.325,Sec.2, Cheng-Kung Rd. Neihu 11490, Taipei, Taiwan, R.O.C

Study record dates

Study Major Dates

• Study Start (Actual): January 1, 2021
• Primary Completion (Actual): December 31, 2023
• Study Completion (Actual): December 31, 2023

Study Registration Dates

• First Submitted: August 24, 2022
• First Submitted That Met QC Criteria: August 24, 2022
• First Posted (Actual): August 26, 2022

Study Record Updates

• Last Update Posted (Actual): August 13, 2024
• Last Update Submitted That Met QC Criteria: August 11, 2024
• Last Verified: August 1, 2024

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Metabolic Diseases; Nervous System Diseases; Neurologic Manifestations; Hematologic Diseases; Nutrition Disorders; Blood Coagulation Disorders, Inherited; Coagulation Protein Disorders; Hemorrhagic Disorders; Genetic Diseases, Inborn; Musculoskeletal Diseases; Neuromuscular Manifestations; Pathological Conditions, Anatomical; Avitaminosis; Deficiency Diseases; Malnutrition; Bone Diseases; Blood Coagulation Disorders; Muscular Atrophy; Atrophy; Bone Diseases, Metabolic; Hemophilia A; Vitamin D Deficiency; Joint Diseases; Sarcopenia; Osteoporosis
• Other Study ID Numbers: 1-108-05-001

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No