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Body composition in children with sickle cell disease
Elizabeth M Barden1, Deborah A Kawchak, Kwaku Ohene-Frempong
1Department of Public Health, The Commonwealth of Massachusetts, Boston, USA.
Insights
Children with sickle cell disease (SCD) show impaired growth and delayed maturation. They exhibit significant deficits in fat-free mass and fat mass, indicating unmet nutritional needs.
Area of Science:
- Pediatrics
- Genetics
- Nutrition Science
Background:
- Sickle cell disease (SCD) is associated with impaired growth, poor nutrition, and delayed maturation in children.
- The specific body composition deficits in pediatric SCD require further elucidation.
Purpose of the Study:
- To assess growth, nutritional status, and body composition in African American children with type SS SCD.
- To compare body composition metrics between children with SCD and healthy controls.
Main Methods:
- Evaluated 36 children with SCD and 30 healthy controls (ages 5-18).
- Assessed height, weight, bone age, pubertal status, skinfold thickness, and arm circumference.
- Estimated fat-free mass (FFM) and fat mass (FM) using four methods.
Main Results:
- Children with SCD had significantly lower z scores for weight, height, arm circumference, and upper arm fat/muscle areas compared to controls.
- Skeletal maturation was significantly delayed in children with SCD.
- Children with SCD exhibited lower FM and FFM across all analyzed groups after age adjustment.
Conclusions:
- Children with SCD experience impaired growth, delayed puberty, and poor nutritional status.
- Low fat and muscle mass in children with SCD suggest inadequate energy and protein stores.
- These body composition abnormalities highlight the unmet nutritional needs in African American children with SCD.
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. Height and weight were converted to z scores by comparison with national reference data and skinfold-thickness measurements were converted to z scores by comparison with African American- specific reference data. Fat-free mass (FFM) and fat mass (FM) were estimated by using 4 methods. Prepubertal children, pubertal males, and pubertal females were analyzed separately.
Results:
Relative to the control subjects and to a national sample, children with SCD had significantly lower z scores for weight, height, arm circumference, and upper arm fat and muscle areas. Relative skeletal maturation was significantly delayed. After adjustment for age, children with SCD had significantly lower FM (prepubertal children and pubertal males only) and FFM (all 3 groups).
Conclusions:
Children with SCD have impaired growth, delayed puberty, and poor nutritional status. Low z scores for upper arm fat area indicate deficits in fat (energy) stores, and low FFM coupled with low upper arm muscle area indicate muscle wasting and low protein stores. These body-composition abnormalities suggest that the nutritional needs of the African American children with SCD were not being met.