ABSTRACT Background and Aims Between 200,000 and 300,000 children with sickle cell disease (SCD) are born in Africa every year, with 75%–80% of these children living in sub‐Saharan Africa. In newborns with SCD, significant iron accumulations may develop because of their increased risk of requiring multiple blood transfusions. This study aimed to assess the hepatic effects of hemotransfusion in pediatric SCD patients in steady state by measuring liver and iron markers. Methods This case‐control research enrolled 120 children with SCD and 60 children without SCD from the Asokwa Children's Hospital Sickle Cell Clinic and Child Welfare Clinic. Participants’ sociodemographic information, history of blood transfusion, and sickle cell genotype were thoroughly documented using a structured questionnaire and patient case records. Venous blood was drawn from each participant for laboratory analysis. Statistical significance was considered at p < 0.05. Result Serum iron was lowest in HbAA (14.60 6.20–34.30 µmol/L) and highest in HbSS (24.45 7.80–51.40 µmol/L; p < 0.001). Significant elevated total iron binding capacity, ALT, and GGT levels were observed in children with SCD than in children without SCD. Children with “SS” genotype recorded the highest transfusion history and had significantly elevated levels of serum iron and transferrin saturation than those with genotype “SC” ( p < 0.05). There were significant correlations between iron markers and ALT ( p < 0.05). In a linear regression prediction model, an increase in the number of frequency of hemotransfusion among children with SCD resulted in 2.6 µmol/L increase in serum iron levels ( β = 2.6, p < 0.05), 40 ng/mL increase in ferritin levels ( β = 40, p < 0.05), and 8% increase in transferrin saturation ( β = 8, p < 0.05) among children with SCD. Conclusion Elevated iron stores and liver enzymes are associated with SCD in children, especially those with a history of transfusion, who should be routinely monitored for elevated iron stores and liver enzymes for early interventions and management.
Sah et al. (Sun,) studied this question.