Abstract

Objective: To investigate the associations between nutritional status and lipid profile with biomarkers of hemolysis and inflammation in sickle cell anemia, in addition to considering gender differences. Methods: This cross-sectional study analyzed nutritional, and biochemical data of pediatric patients carrying sickle cell anemia. Results: Vitamin A, apolipoprotein B, total cholesterol, low-density lipoprotein, and non-high-density lipoprotein cholesterol were lower in boys. Hemoglobin was significantly lower, and the white blood cells and lactate dehydrogenase were higher in boys. Body mass index, vitamin A, and triglycerides were associated with hemoglobin levels, while apolipoprotein A-I was associated with white blood cells and total bilirubin. Additionally, body mass index and vitamin A were associated with lactate dehydrogenase. Vitamin A showed significant predictive power in hemoglobin alteration and lactate dehydrogenase, while apolipoprotein A-I was able to predict high white blood cells values and total bilirubin. Conclusion: This study found in a pediatric population with sickle cell anemia that body mass index, vitamin A, triglycerides, and apolipoprotein A-I were associated with biomarkers of hemolysis and inflammation. Boys exhibited the greater nutritional deficit and severity of the disease.

Highlights

  • Sickle cell anemia (SCA, MIM#603903) refers to the homozygous hereditary condition of pathogenic mutation HbS (HBB:c.20A>T; rs334; p.Glu6Val) in the HBB gene (MIM#141900, loco 11p15.4) (Rees, Williams, & Gladwin, 2010)

  • This study found in a pediatric population with sickle cell anemia that body mass index, vitamin A, triglycerides, and apolipoprotein A-I were associated with biomarkers of hemolysis and inflammation

  • Nutritional status, lipid profile, and biomarkers of SCA severity Our results showed a high prevalence of patients with a nutritional deficit or nutritional risk

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Summary

Introduction

Sickle cell anemia (SCA, MIM#603903) refers to the homozygous hereditary condition of pathogenic mutation HbS (HBB:c.20A>T; rs334; p.Glu6Val) in the HBB gene (MIM#141900, loco 11p15.4) (Rees, Williams, & Gladwin, 2010). Children with SCA often experience growth delay with a higher prevalence of weight deficit than observed in the general population (Jesus, Konstantyner, Lôbo, & Braga, 2018; Kazadi, Ngiyulu, Gini-Ehungu, Mbuyi-Muamba, & Aloni, 2017) Another characteristic frequently observed in SCA is dyslipidemia, characterized by the reduction of serum total cholesterol as well as low-density lipoprotein (LDL-C) (Adegoke, Okeniyi, & Akintunde, 2016; Teixeira et al, 2019), highdensity lipoprotein (HDL-C) (Teixeira et al, 2019) and increased triglycerides (Adegoke et al, 2016; Teixeira et al, 2019). While hemoglobin levels are low, the white blood cell count (WBC) (Mikobi et al., 2017), lactate dehydrogenase (LDH), and bilirubin levels, mainly indirect bilirubin, are increased (Aleluia et al, 2017)

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