Assessing liver and iron markers in steady state paediatric SCD patients to ascertain the hepatic consequences of haemotransfusion: A case-control study in Ghana.

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Abstract

Background and Aims: Between 200,000 and 300,000 children with sickle cell disease are born in Africa every year, with 75–80% of these children living in sub-Saharan Africa. In newborns with sickle cell disease (SCD), significant iron accumulations may develop because of their increased risk of requiring multiple blood transfusions. Limited information is available regarding liver enzyme levels in Ghanaian children with sickle cell disease. This study aimed to assess the hepatic effects of haemotransfusion in paediatric sickle cell disease patients in steady state by measuring liver and iron markers. Methods: This case-control research enrolled 129 children with SCD and 60 children without SCD from the Asokwa children hospital sickle cell clinic and child welfare clinic. Participants' information 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 to evaluate serum ferritin, serum iron, total iron binding capacity (TIBC), alanine transaminase (ALT), aspartate aminotransaminase (AST) and gamma-glutamyl transferase (GGT) levels. Statistical significance was considered at p <0.05. Result: Significant elevated serum iron, total iron binding capacity, ALT and GGT levels were observed in children with SCD than 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). Elevated concentration of serum iron and transferrin saturation were also observed in children with SCD who last received haemotransfusion between 0-6 months ago than children who received transfusion between 7-12 months ago ( p < 0.01), and more than or equal to 13 months ago ( p < 0.05). Similarly, children with SCD who had haemotransfusion history of 0-6 months ago had significantly increased levels of ferritin concentration and ALT concentration than more than or equal to 13 months ago. In a linear regression prediction model, an increase in the number of frequency of haemotransfusion among children with SCD resulted in 2.6 umol/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 sickle cell disease in children, especially those with history of transfusion should be routinely monitored for elevated iron stores and liver enzymes for early interventions and managements.

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