Nutritional Outcomes After Vitamin A Supplementation in Subjects With SCD

Children's Hospital of Philadelphia42 enrolled

Overview

This study establishes the safety and efficacy of vit A supplementation doses (3000 and 6000 IU/d) over 8 weeks in children with SCD-SS, ages 9 and older and test the impact of vit A supplementation on key functional and clinical outcomes. Additionally, vitamin A status is assessed in healthy children ages 9 and older to compare to subjects with SCD-SS.

Suboptimal vitamin A (vit A) status is prevalent in children with type SS sickle cell disease (SCD-SS) and associated with hospitalizations and poor growth and hematological status. Preliminary data in children with SCD-SS show that vit A supplementation at the dose recommended for healthy children failed to improve vit A status, resulting in no change in hospitalizations, growth or dark adaptation. This indicates an increased vit A requirement most likely due to chronic inflammation, low vit A intake and possible stool or urine loss. The dose of vit A needed to optimize vit A status in subjects with SCD-SS is unknown.

Study Type

INTERVENTIONAL

Allocation

RANDOMIZED

Purpose

TREATMENT

Masking

QUADRUPLE

Enrollment

Conditions

Sickle Cell Anemia in Children Vitamin A Deficiency in Children

Interventions

retinyl palmitateDIETARY_SUPPLEMENT

The intervention is a daily vitamin A supplement.

Eligibility

Sex: ALLMin age: 9 YearsHealthy volunteers:

Medical Language ↔ Plain English

Inclusion Criteria: * Sickle cell disease, SS genotype (subjects with sickle cell disease only) * Usual state of good health (no hospitalizations, emergency room visits, or unscheduled acute illness clinic visits for two weeks prior to screening) * Commitment to a 119-day study (subjects with sickle cell disease only), or a 4-day study (healthy volunteers only) Exclusion Criteria: * Hydroxyurea initiated within the previous 6 weeks (subjects with sickle cell disease only) * History of stroke (subjects with sickle cell disease only) * Other chronic conditions that may affect growth, dietary intake or nutritional status * Retinoic acid (topical or oral), weight loss medication and/or lipid lowering medications * Subjects with a BMI greater than 98th percentile for age and sex * Pregnant or lactating females (subjects who become pregnant during the course of the study will not continue participation) * Liver function tests \>4 x upper limit of reference range * Participation in another study with impact on vitamin A status (subjects with sickle cell disease only) * Use of multi-vitamin or commercial nutritional supplements containing vitamin A (those who are willing to discontinue these supplements, with the approval of the medical care team, will be eligible for the study after a 1 month washout period. Subjects taking nutritional products without vitamin A will be eligible) * Inability to swallow pills (subjects with sickle cell disease only)

Locations (1)

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States