Renal Glomerular and Tubular Functions among Egyptian Transfusion Dependent β-Thalassemia Patients: Relation to Renal MRI

Abstract

eta thalassemia major is an inherited disease resulting from reduction or total lack of beta globin chains. Patients with this disease need repeated blood transfusion for survival. Repeated blood transfusions are inevitably associated with iron overload that leads to multiple organ dysfunctions namely heart, liver and endocrine glands. Historically, renal involvement has not been a commonly recognized complication in patients with β-thalassemia major (β-TM). Some published studies demonstrated proteinuria, aminoaciduria, low urine osmolarity, and excess secretion of the proximal tubule damage markers in pediatric patients with thalassemia. Serum Cys C and urinary β2-M are promising biomarkers for monitoring glomerular and tubular dysfunction. Magnetic resonance imaging (MRI) gradient echo (T2*), the reciprocal of T2* (known as R*), has been developed to quantify tissue iron in the liver, the heart as well as the kidneys. Therefore, we assessed levels of serum Cys C and urinary β2-M to albumin ratio as markers of renal functions (glomerular and tubular) among transfusion dependent β-thalassemia patients and their relation to iron overload and renal MRI (R2*). This study included 50 β-TM patients without symptomatic renal disease (25 males and 25 females) recruited from the regular attendants of the Pediatric Hematology Clinic,