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Objective To determine the accuracy of energy prediction equations when compared with measured resting energy expenditure (REE) in children with sickle cell anemia. To develop a modified equation that more accurately estimates the energy needs of children with sickle cell anemia and to cross-validate these on a different set of patients (test patients). Design REE was measured in children using indirect calorimetry and compared with predicted values using the Harris-Benedict and the Food and Agriculture Organization/ World Health Organization/ United Nations University equations (WHO). Subjects/Setting Eighteen patients participated in the original sample that compared predicted with measured energy expenditure. The modified equations were developed using the original 18 patients. A test population of 20 different patients was used to validate the modified equations. Statistical analysis Wilcoxon signed-rank test was performed to compare measured with predicted REE. The correlation analysis method and multiple linear regression method were used to develop 2 modified versions for the Harris-Benedict and WHO prediction equations. Results When compared with the mean predicted REE using the Harris-Benedict and WHO equations, the mean measured REE was 14% and 12% greater than both ( P =.005 and P=. 014, respectively). Two modified equations were developed from the Harris-Benedict and WHO equations. Based on the data from the test patients, the mean measured REE was 15% greater than the mean predicted REE based on the Harris-Benedict and WHO equations ( P =.0001 for both). When the modified Harris-Benedict and WHO equations were used, there was almost no difference in the mean measured REE and the mean predicted REE (mean difference using Harris-Benedict=14, P= .9273; mean difference using WHO=−13, P =.6215). Conclusion Both energy prediction equations underestimated REE in children with sickle cell anemia. The 2 modified versions of the energy prediction equations that we propose predicted the energy needs of these children much more accurately; however, the modified equations need to be validated through application to other children with sickle cell anemia. J Am Diet Assoc. 2002;102:956–961 .

Osteopetrosis, a rare condition caused by a failure of osteo-clasts to resorb bone, results in dense and deformed bones, growth failure, anemia, hypoplastic dentition, chronic infection, blindness, and massive splenomegaly. Children with infantile osteopetrosis have disease-related complications that affect nutritional status. Altered calcium and phosphorus intake has been reported in these patients, but previous studies did not include an assessment of dietary adequacy or nutrition-related complications. A prospective study of six children with severe infantile osteopetrosis was conducted to identify specific nutrition-related problems and effective nutrition intervention strategies. Patients were monitored by a registered dietitian while they participated in a clinical research protocol at St Jude Children's Research Hospital. Dietary records for each patient were obtained at set intervals during the 4-month study period. Because most patients lacked adequate dentition, they had difficulty obtaining adequate nutrition through normal oral intake. Three children required nutrition supplementation (enteral feedings for one and oral supplements for two). We conclude that oral and enteral nutrition support can provide nutrients needed for improved growth and response to treatment in patients with osteopetrosis. J Am Diet Assoc. 1996; 96: 172-175 .

Children with sickle cell disease (SCD) have decreased height and weight when compared to their healthy peers, but exact reasons for poor growth have not been established. Researchers have reported that children with SCD have resting energy expenditure (REE) that is 18% above healthy controls and 13% above energy prediction equations. Although there are no energy prediction equations specific to patients with SCD, the Harris-Benedict (HB) equations are commonly used to predict the energy needs of a wide range of people, and the validity of these equations has been established in healthy children. In order to examine the energy needs of children with SCD, we compared the HB equations to measured energy expenditure in 21 children with SCD. Their ages ranged from 5–17 years (mean 11.5 ± 3.5, median 13); 12 were female. We measured REE by indirect calorimetry using a CPX-MAX-D Cardiopulmonary Gas Exchange System (Medical Graphics), in a supine position, after an overnight fast The patients had a mean ideal body weight of 94.7 ± 7.3%. Their mean fat free body mass (bioelectrical impedance, RJL Systems) of 85.6 ± 7.8%, compared to an estimated 80% in normal children of the same age. Four patients were below the 5 th percentile for height and 6 for weight, and 7 patients had triceps skinfold thickness (Lange calipers) ⩽ 5 th percentile for age. The measured REE (1384 ±342) was significantly higher than the predicted (1221 ± 265). The ratio of measured REE to predicted REE was 1.14 (p=0.001). We conclude that energy prediction equations underestimate the REE of patients with SCD by 14%. Although patients in this study were smaller and leaner than the general population, their energy needs were higher than predicted. Their elevated REE may be due to the higher percentage of lean body mass and/or manifestations of SCD, such as chronic anemia. This increased energy requirement, in the absence of adequate energy intake may contribute to the poor growth of children with SCD.