Total and resting energy expenditure in children with sickle cell disease,☆☆,

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Abstract

Objective: To investigate energy balance in children with sickle cell disease (SCD) as the possible cause of impaired growth and undernutrition. Study design: Growth, resting (REE), total (TEE), and activity-related (AEE) energy expenditure and dietary intake were examined in 36 African American children with SCD (20 girls and 16 boys) and 30 control subjects (15 girls and 15 boys) of similar age (mean, 11.2 years) and ethnicity. TEE was measured by means of the doubly labeled water technique and REE by indirect calorimetry. AEE was calculated as TEE minus REE. Fat free mass (FFM) was calculated from skinfold prediction equations. Results: REE was significantly increased (131 kcal/d) in children with SCD (P = .001), after adjusting for sex and FFM. Children with SCD tended to have lower TEE (214 kcal/d) than control subjects, but there was no difference after adjusting for FFM and sex (P = .57). Children with SCD had significantly (P = .025) lower AEE (268 kcal/d) but only marginally (P = .08) lower AEE after adjusting for FFM and sex. Conclusions: The elevated REE and lower AEE, in combination with poor growth status, indicate chronic energy deficiency in children with SCD. Further studies are needed to determine the best approaches to the treatment and prevention of undernutrition in children with SCD. (J Pediatr 2000;136:73-9)

Section snippets

Subjects

Subjects with SCD were recruited from among the children and adolescents from 5 to 18 years of age cared for at the Comprehensive Sickle Cell Center at the Children’s Hospital of Philadelphia. The subjects with SCD met the following criteria: homozygous HbSS-type disease (hemoglobin status confirmed by hemoglobin electrophoresis of subject and parents), no history of stroke or long-term transfusion therapy, no hospitalization or intercurrent illness within 2 weeks of the study, and no other

Subject Characteristics

A total of 36 children with SCD (16 boys and 20 girls) and 30 control children (15 boys and 15 girls) were enrolled. The final study sample was restricted to those subjects for whom REE was available. Results for 2 subjects with SCD and 2 control subjects were omitted because of REE equipment malfunction. Subjects with SCD and control subjects were similar in age, bone age, and pubertal development (Table I).

. Growth, nutritional status, and body composition characteristics (mean ± SD, range) of

DISCUSSION

This study, with a large sample size and an adequate control group, examined the relationship between SCD and multiple components of energy expenditure in a US population. Other investigators have reported that children with SCD were delayed in sexual and skeletal maturation, wasted, and short for their age1, 2, 12 and that they have elevated REE.6, 8, 9, 10, 11, 13 Our findings are consistent with earlier reports on children with SCD.6, 9, 10, 11, 13 In addition, reduced FFM and percent body

Acknowledgements

We express our deepest appreciation to the children and their families for their participation and commitment to research. Additionally, we would like to thank the staff of the Comprehensive Sickle Cell Center and the General Clinical Research Center of the Children’s Hospital of Philadelphia for their assistance with the project.

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  • Cited by (0)

    Supported in part by the Comprehensive Sickle Cell Center (NHLBI 38633-06), the General Clinical Research Center (M01RR00240), and the Nutrition Center at the Children’s Hospital of Philadelphia.

    ☆☆

    Reprint requests: Babette S. Zemel, PhD, Division of Gastroenterology and Nutrition, The Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104.

    0022-3476/2000/$12.00 + 0  9/21/102943

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