EVALUTION OF SERUM BETA-2-MICROGLOBULIN AS A MARKER OF RENAL DYSFINCTION IN PEDIATRIC PATIENTS WITH SICKLE CELL DISEASE

Abstract

Background: Sickle-cell disease is a multisystem disease, associated with episodes of acute illness and progressive organ damage, and is one of the most common severe monogenic disorders worldwide. Renal dysfunction is a frequent complication in sickle cell disease as a result of long-standing anemia and disturbed circulation through the medullar capillaries, so early detection of it is important to guard against chronic kidney disease. Beta-2-Microglobulin, a low molecular weight protein, is freely filtered in the glomerulus wherefrom it is totally reabsorbed and degraded in the renal tubules. Thus, it is a sensitive marker of the glomerular filtration capacity of the kidney. Objectives: To identify the role of serum Beta-2-Microglobulin as early predictor of renal dysfunction in patients with sickle cell disease. Patients & Methods: We measured serum B-2-Microglobulin in 67 children previously diagnosed as Sickle Cell Disease and attending to Bab Elsharia (Sayed Galal) University Hospital and Misr Children Hospital in period from November 2016 to September 2017. This group of patient was matched with control group (20 children) with the same ages who do not have Sickle Cell Disease and recruited from the same study site. Results: Although, serum B2M was within the reference range, cases had significantly lower serum B2M to the control group. Serum level of B2M was not affected by gender and our data clearly demonstrate age independency for serum concentrations of B2M. Comparing serum B2M to serum creatinine by ROC curve, showed that area under the curve of serum B2M (0.48) versus (0.05) was insignificantly higher and both were below 0.7; indicating that overall predictability of SCD of serum B2M as well as serum creatinine were not statistically significant. Conclusion: ● Renal dysfunction in SCD children may occur before the occurrence of any symptoms or complication. ● Serum B2M is not a good marker in detection of early stages of SCD nephropathy in children. ● A/C ratio and GFR are considerable methods of preliminary detection of glomerular affection in SCD children. Recommendations: ● Routine screening of SCD children with A/C ratio and GFR is mandatory for early detection of renal affection. ● Further studies of establishment of best marker used for early assessment of tubular and glomerular dysfunction in SCD children is recommended.