Biochemical factors relevant to kidney functions among Jordanian children with beta-thalassemia major treated with deferoxamine

Abstract

Thalassemia is one of the systematical diseases that occur worldwide and is the commonest form of hemoglobinopathy in Jordan. The most important cause of mortality and morbidity in these patients with thalassemia is organ failure related with the shortened red cell life span, rapid iron turnover and tissue deposition of excess iron. These are the major factors responsible for functional and physiological abnormalities found in various forms of thalassemia. This study aied to examine the biochemical factors related to kidney functions such as glucose, urea, creatinine, sodium and potassium levels among Jordanian children with β-thalassemia major treated with deferoxamine. Forty two patients (aged 12 to 28 years) with β-thalassemia major (20 males and 22 females) that undergo periodical blood transfusion and they are on deferoxamine (DFO) as chelating agent were involved in this study. All patients were free from hepatitis B virus (HBV), hepatitis C virus (HCV) and human immunodeficiency virus (HIV). The diagnoses of β-thalassemia major were made based on the clinical, hematological and hemoglobin electrophoresis profiles for the patients. Haemoglobin (Hb) electrophoresis for the father and mother and genetic study of the b globins genes in some disputable cases were also done. Forty controls of matched age and gender (20 males and 20 females) were also included in this study. Results showed that the significant differences (p < 0.05) appeared between the experimental and control groups over all the measured physiological variables (urea, creatinine, uric acid, sodium and potassium) except for blood glucose and chloride. It is concluded that the functional abnormalities of the kidney in patients with β-thalassemic patients can be attributed to chronic anemia, iron overload as well as to DFO toxicity and enhancement of the oxidative stress induced by excess iron deposits. These functional abnormalities would have any long-term effects on the patients. Key words: β-Thalassemia major, renal function, desferrioxamine, iron overload.