Deposition in Chronically Transfused Thalassemia Major Patients and Correlation to Cardiac and Liver Findings

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 cystatin C has been used as a biomarker for monitoring renal 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 tissue iron overload by MRI among transfusion dependent β-thalassemia patients and its relation to levels of urinary albumin excretion and serum cystatin C as markers of renal functions. This study included 50 β-TM patients (25 males and 25 females) recruited from the regular attendants of the Pediatric Hematology Clinic, Pediatric Hospital, Ain Shams University. Patients were compared with 25 age- and sex-matched healthy