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Body composition in children with sickle cell disease^1,2,3

¹ From the Department of Public Health, The Commonwealth of Massachusetts, Boston (EMB), and the Divisions of Gastroenterology and Nutrition (DAK, VAS, and BSZ) and Hematology (KO-F), The Children's Hospital of Philadelphia, Department of Pediatrics, The University of Pennsylvania, Philadelphia.

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.

Key Words: Sickle cell disease • growth • nutritional status • body composition • children • African Americans

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