Abstract

Background: Sickle cell anemia (SCA) poses significant metabolic challenges in affected individuals, yet the relationship between resting metabolic rate (RMR) and disease severity remains underexplored, particularly in Sudanese pediatric populations.

Aim: The aim of this study is to investigate the RMR and its correlation with disease severity scores and treatment with hydroxyurea in Sudanese patients with sickle cell disease.

Materials and Methods: We conducted a case-control study on 100 individuals aged 3-18 years, with 50 having SCA and 50 forming the healthy control group. RMR was computed using the Harris-Benedict formula, the World Health Organization equation, and the Buchowski formula. Severity scores were determined using a SCA Scoring System.

Results: The mean age for the control group (9.52 ± 4.35 y) was similar to that of the SCA group, whether receiving or not receiving hydroxyl-urea (9.44 ± 4.164 and 9.68 ± 4.534, respectively (p=0.980). The resting metabolic rate (RMR) in the case group was significantly lower compared to the control group when using the Harris-Benedict and WHO equations (p = 0.004 and 0.001, respectively). A notable difference in RMR was observed between the case group (using the Buchowski formula) and the control group (using the Harris-Benedict equation) (p = 0.006), but not with the WHO equation (p = 0.054). Treatment with hydroxyl-urea was significantly associated with low severity scores among the SCA patients (p< 0.001).

Conclusions: The elevated RMR levels among Sudanese SCA patients compared to healthy subjects can be shown when calculated with the Buchowski formula but not with the other standard equations designed for healthy individuals.