Total and resting energy expenditure in children with sickle cell disease☆,☆☆,★
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).
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.
References (36)
- et al.
Growth patterns by age and sex in children with sickle cell disease
J Pediatr
(1984) - et al.
Growth retardation in sickle cell disease treated by nutritional support
Lancet
(1985) - et al.
Resting metabolic rate in homozygous sickle cell disease
Am J Clin Nutr
(1993) - et al.
Is there an energy deficiency in homozygous sickle cell disease?
Am J Clin Nutr
(1997) Measurement of energy expenditure in free living humans using doubly labeled water
J Nutr
(1988)- et al.
Comparative advantage of 3-day food records over 24-hour recall and 5-day food frequency validated by observation of 9- and 10-year-old girls
J Am Diet Assoc
(1994) - et al.
Mathematical ratios lead to spurious conclusions regarding age and sex-related differences in resting metabolic rate
Am J Clin Nutr
(1995) - et al.
Improved exercise performance after exchange transfusion in subjects with sickle cell anemia
Blood
(1980) - et al.
Influence of sickle hemoglobinopathies on growth and development
N Engl J Med
(1984) - et al.
Prepubertal growth and skeletal maturation in children with sickle cell disease
Pediatrics
(1986)
Nutritional factors and growth in children with sickle cell disease
Am J Dis Child
Alterations in basal nutrient metabolism increase resting energy expenditure in sickle cell disease
Am J Physiol
Growth retardation in homozygous sickle cell disease: role of calorie intake and possible gender-related differences
Am J Hematol
Nutritional status and dietary intake of children with sickle cell anemia
Am J Pediatr Hematol Oncol
Whole body protein turnover and resting metabolic rate in homozygous sickle cell disease
Clin Sci
Protein and energy metabolism in prepubertal children with sickle cell anemia
Pediatr Res
Delayed adolescent growth in homozygous sickle cell disease
Arch Dis Child
The methods of auxological anthropometry
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