No improvement in suboptimal vitamin A status with a randomized, double-blind, placebo-controlled trial of vitamin A supplementation in children with sickle cell disease.

Background: Suboptimal vitamin A status is prevalent in children with type SS sickle cell disease (SCD-SS) and is associated with hospitalizations and poor growth and hematologic status. The supplemental vitamin A dose that optimizes suboptimal vitamin A status in this population is unknown.

Objective: The efficacy of Recommended Dietary Allowance (RDA) doses (based on age and sex) of vitamin A (300, 400, or 600 μg retinyl palmitate/d) or vitamin A + zinc (10 or 20 mg zinc sulfate/d) compared with placebo to optimize vitamin A status was assessed in children aged 2.

High risk of vitamin D deficiency in children with sickle cell disease.

Vitamin D is a particularly concerning nutrient for children with homozygous SS sickle cell disease (SCD-SS) due to their increased skin melanin concentrations, reduced levels of physical activity, and poor vitamin D intake. The goal of this study was to compare the vitamin D status of children with SCD-SS to healthy African-American children living in the same geographic area. Growth, dietary intake, serum 25-hydroxyvitamin D [25(OH)D], and intact parathyroid hormone (iPTH) concentrations were measured in 61 African-American subjects with SCD-SS and 89 healthy African-American control subjects age 5 to 18 years from the Philadelphia, PA, region (latitude 39.

Adequacy of dietary intake declines with age in children with sickle cell disease.

Dietary intake (24-hour recall) was evaluated prospectively over four annual visits in 97 children and adolescents (53 female), aged 1.5 to 18.7 years, with sickle cell disease, type SS.

Effects of delayed pubertal development, nutritional status, and disease severity on longitudinal patterns of growth failure in children with sickle cell disease.

Previous studies of children with sickle cell disease (SCD) reported poor growth and delayed maturation. However, the prevalence, magnitude, and correlates of suboptimal growth remain poorly understood. A prospective longitudinal study was undertaken to determine the effects of disease severity and nutritional status on growth, an indicator of childhood well-being.

Markers of bone turnover are associated with growth and development in young subjects with sickle cell anemia.

Children with sickle cell anemia (SCA) have low bone mass though bone turnover has not been well described. In this study, growth and pubertal development were assessed twice, 1 year apart, in 80 young subjects with type-SS SCA, while whole body bone mineral content (BMC) and density where measured in a subset (n = 46). Markers of bone turnover were measured at the second visit.

Plasma zinc is an insensitive predictor of zinc status: use of plasma zinc in children with sickle cell disease.

Background: This study was designed to explore the use of plasma zinc in determining zinc deficiency in children with sickle cell disease-SS (SCD-SS) as indicated by a growth response to zinc supplementation.

Methods: Fasting plasma zinc was assessed in children with SCD-SS (ages 4 to 10 years) who were randomly assigned to receive 10 mg zinc/d in cherry syrup (zinc) or cherry syrup alone (placebo) for 12 months. Evaluations for growth, dietary intake, and other biochemical parameters were made at baseline and 3, 6, and 12 months.

Bone area and bone mineral content deficits in children with sickle cell disease.

Objective: Children with sickle cell disease (SCD) experience poor growth, altered body composition, and delayed maturation. Deficits in bone mineral content (BMC) and bone area (BA) have not been well characterized. The objectives of this study were to assess whole-body BMC (WBBMC) and WBBA in children with SCD, type SS (SCD-SS), compared with healthy control subjects, adjusted for growth and body composition, and to determine the relationships of WBBMC and WBBA to bone age and hematologic parameters in children with SCD-SS.

Low vitamin D status in children with sickle cell disease.

Objectives: To examine vitamin D status in children with sickle cell disease (SCD)-SS and its relation to season and dietary intake.

Study Design: Growth, dietary intake, 25-hydroxyvitamin D (25-OHD), and parathyroid hormone levels were measured. Children with low and normal vitamin D status were compared.

Vitamin A status, hospitalizations, and other outcomes in young children with sickle cell disease.

Objective: To determine the relation of serum vitamin A status to growth, nutritional and hematologic status, and to the number of hospitalizations in children with sickle cell disease-SS (homozygous for the S allele, SCD-SS).

Study Design: Children (2-9.9 years of age) with SCD-SS were assessed for serum retinol, hemoglobin, hematocrit, reticulocyte count, height, weight, body mass index, and recalled dietary intake.

Brief report: parent perspectives of nutritional status and mealtime behaviors in children with sickle cell disease.

Objective: To qualitatively evaluate parent perspectives of eating problems, nutritional status, and the potential for nutritional intervention in children with sickle cell disease (SCD).

Methods: Twenty parents of children with SCD participated in one of three focus groups to discuss questions related to the study's objectives. Three coders rated transcripts to identify common perceptions and experiences (themes) among participants.

Vitamin B6 status of children with sickle cell disease.

Purpose: In vitro, vitamin B(6) has antisickling properties, but the effect of vitamin B status on the health of children with sickle cell disease-SS (SCD-SS) is not well described. The purpose of this study was to assess vitamin B(6) status of children with SCD-SS ages 3 to 20 years and determine its relationship to growth, dietary intake, and disease severity.

Patients And Methods: Vitamin B(6) status was assessed by serum pyridoxal 5-phosphate (PLP) concentration in subjects with SCD-SS and by urinary 4-pyridoxic acid (4-PA) concentration in other subjects with SCD-SS and healthy control children.

Body composition in children with sickle cell disease.

Background: Impaired growth, poor nutritional status, and delayed skeletal and sexual maturation are common in children with sickle cell disease (SCD), yet the nature of associated body-composition deficits has not been fully described.

Objective: The objective was to assess growth, nutritional status, and body composition in 36 African American children with type SS SCD (20 females and 16 males) and 30 healthy control children (15 females and 15 males) of similar age (5-18 y) and ethnicity.

Design: Height, weight, bone age, pubertal status, skinfold thickness, and arm circumference were assessed.

Effect of zinc supplementation on growth and body composition in children with sickle cell disease.

Background: Poor growth and delayed maturation in children with sickle cell disease (SCD) may be due, in part, to mild zinc deficiency.

Objective: The objective was to determine the effects of zinc supplementation on growth and body composition in children with SCD.

Design: Forty-two prepubertal children (20 girls and 22 boys) aged 4-10 y with SCD-SS were randomly assigned to receive 10 mg elemental Zn/d in cherry syrup (zinc group) or cherry syrup alone (control group).