Bone Mineral Density, Hand Grip Strength, Health Related Quality of Life, Sickle Cell Disease, Vitamin D Deficiency
Conditions
Brief summary
Suboptimal vitamin D status is well reported in sickle cell disease (SCD) patients and associated with a negative impact on health-related quality of life (HRQL). The investigators enrolled 42 SCD patients and 42 healthy controls, subjects within each group received monthly oral vitamin D3 dose according to the baseline status of vitamin D as follows: sufficient: 100,000 IU, insufficient: 150,000 IU, and deficient: 200,000 IU. The investigators assessed safety and efficacy on normalization of vitamin D level, bone mineral density (BMD), hand grip strength (HGS), and HRQL.
Interventions
DRUGVitamin D3
Subjects within SCD as well as healthy controls, received monthly oral vitamin D3 dose, for 6 months, according to the baseline status of vitamin D as follows: sufficient (\>30 ng/mL): 100,000 IU, insufficient (20-29.9 ng/mL): 150,000 IU, and deficient (\<20 ng/mL): 200,000 IU.
Sponsors
Zagazig University
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
No minimum to 18 Years
Healthy volunteers
Yes
Inclusion criteria
* children with SCD (HbSS, hemoglobin sickle beta zero (HbSβ0) thalassemia genotype), aged ≤ 18 years old, male or female study participants who were at a steady state (≥ one month from blood transfusion and ≥ 14 days from any acute sickle complication as hospitalization for Vaso occlusive crisis (VOC) or acute chest syndrome (ACS)), stable Hb level near their usual baseline and stable dose of Hydroxyurea (HÚ) mg/kg for at least 90 days prior to enrollment. * A control group of 42 healthy age and sex-matching children
Exclusion criteria
* SCD patients who are on chronic blood transfusion therapy * Comorbid chronic conditions * Use of medications known to interfere with calcium or vitamin D absorption or metabolism * Known hypercalcemia or vitamin D hypersensitivity * Use of vitamin D therapy to treat vitamin D deficiency or rickets * Urolithiasis, liver or renal impairment, and malabsorption disorders. * Obese children with body mass index (BMI) \> 85th percentile for age and sex
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Serum 25(OH)D level | up to 6 months | Serum 25(OH)D level change from baseline at 6 months |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Maximum handgrip strength (HGS) | up to 6 months | Hand grip power using a handheld dynamometer. |
| Health related quality of life (HRQL) | up to 6 months | Health related quality of life (HRQL) assessed by HRQL questionnaires, The questionnaire was divided into eight subscales: physical function, role limitations resulting from physical health, bodily pain, general health perception, vitality, social function, role limitations resulting from emotional problems, and mental health. For each subscale: higher score indicated good health and ranged from 0 to 100. |
| Serum concentrations of C reactive protein (CRP) | up to 6 months | Serum concentrations of inflammatory marker (CRP) level change from baseline |
| Serum concentrations of Erythrocyte sedimentation rate (ESR) | up to 6 months | Serum concentrations of inflammatory marker (ESR) level change from baseline |
| Bone mineral density (BMD) | up to 6 months | BMD was evaluated at the posterior-anterior spine, Z-scores were used to interpret the results, with Z-scores less than -2 standard deviation (SD) being regarded as abnormal |
| Safety measurements of serum Ca | at 3 and 6 months | serum Ca levels |
| childhood health assessment | up to 6 months | Assessed by childhood health assessment questionnaire (CHAQ), There are four potential responses to each question: without any difficulty (score 0); with some difficulty (score 1); with much difficulty (score 2); and unable to do (score 3). A summary score known as CHAQ-DI/, which varies from 0 to 3, is calculated by averaging the highest score in each domain. For a CHAQ-DI score to be considered minimally clinically significant, it must be ≥ 0.75. |
| Safety measurements of serum 25(OH)D levels | at 3 and 6 months | serum 25(OH)D levels |
| Safety reporting of any adverse events | up to 6 months | e.g. nausea, drowsiness, vomiting, loss of appetite, constipation, confusion, cardiac arrhythmias, renal failure, coma |
Countries
Egypt
Outcome results
None listed