High Dose Vitamin D Supplementation in Children With Sickle Cell Disease

Zagazig University75 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

Sickle Cell Disease Vitamin D Deficiency Health Related Quality of Life Hand Grip Strength Bone Mineral Density

Eligibility

Sex: ALLMax age: 18 YearsHealthy volunteers:

Medical Language ↔ Plain English

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

Outcomes

Primary Outcomes

Serum 25(OH)D level

Serum 25(OH)D level change from baseline at 6 months

Time frame: up to 6 months

Secondary Outcomes

Bone mineral density (BMD)

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

Time frame: up to 6 months

Maximum handgrip strength (HGS)

Hand grip power using a handheld dynamometer.

Time frame: up to 6 months

Health related quality of life (HRQL)

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.

Time frame: up to 6 months

Serum concentrations of C reactive protein (CRP)

Serum concentrations of inflammatory marker (CRP) level change from baseline

Time frame: up to 6 months

Serum concentrations of Erythrocyte sedimentation rate (ESR)

Serum concentrations of inflammatory marker (ESR) level change from baseline

Time frame: up to 6 months

Safety reporting of any adverse events

e.g. nausea, drowsiness, vomiting, loss of appetite, constipation, confusion, cardiac arrhythmias, renal failure, coma

Time frame: up to 6 months

Safety measurements of serum Ca

serum Ca levels

Time frame: at 3 and 6 months

childhood health assessment

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.

Time frame: up to 6 months

Safety measurements of serum 25(OH)D levels

serum 25(OH)D levels

Time frame: at 3 and 6 months

High Dose Vitamin D Supplementation in Children With Sickle Cell Disease

Overview

Conditions

Interventions

Eligibility

Locations (1)

Outcomes

Primary Outcomes

Secondary Outcomes