Abstract
The present study aimed to investigate the association of Nε-carboxymethyllysine (CML) with laboratory parameters and βS haplotypes in pediatric sickle cell anemia (SCA) patients with or without hydroxyurea (HU) therapy. We included 55 children with SCA (SCAtotal), where 27 were on HU treatment (SCA-HU+) and 28 without HU treatment (SCA-HU−). Laboratory characteristics were determined using electronic methods while CML was measured using competitive ELISA. βS haplotypes were determined by RFLP-PCR. Significant increases in MCV and MCH and significant decreases in leukocytes, eosinophils, basophils, atypical lymphocytes, lymphocytes, and monocytes were found in SCA-HU+ compared to SCA-HU−. SCA-HU+ presented significant reduction in aspartate transaminase and lactate dehydrogenase and increase in creatinine levels compared to SCA-HU−. CML levels were significantly higher in both SCA-HU+ and SCA-HU− compared to the healthy control. In addition, a negative correlation was found between CML and alanine transaminase in SCA-HU+ and SCAtotal (p < 0.01). A significant association was found between CML levels and βS haplotypes. The results suggest that CML has a role to play in SCA complications, independent of HU therapy.
Highlights
Sickle cell anemia (SCA) is a monogenic hematological disorder caused by substitution GAG>GTG at the 6th position of the beta globin gene (HBB) located in chromosome 11 [1]
This can be explained by factors such as fetal hemoglobin (HbF) levels, coexistence of alpha (α) thalassemia, haplotypes associated with the βS globin gene, oxidative stress, features intrinsic to the red blood cell (RBC), and extracellular environment [4, 5]
They were divided into two groups: the SCA-HU+ group composed of children with HU therapy (15–25 mg/kg/day) and the SCA-HU- group composed of children without HU therapy
Summary
Sickle cell anemia (SCA) is a monogenic hematological disorder caused by substitution GAG>GTG at the 6th position of the beta globin gene (HBB) located in chromosome 11 [1]. SCA patients present a wide variability in clinical manifestations regarding the functions of vital organs as well as frequency and severity of vasoocclusive crises [2, 3]. This can be explained by factors such as fetal hemoglobin (HbF) levels, coexistence of alpha (α) thalassemia, haplotypes associated with the βS globin gene, oxidative stress, features intrinsic to the red blood cell (RBC), and extracellular environment [4, 5]. SCA-HU+ N = 27, M (25th–75th per). SCA-HUN = 28, M (25th–75th per) p value∗. Senegal (SEN), Cameroon (CAM), Bantu or Central African Disease Markers
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