Early iron overload in beta-thalassaemia major: when to start chelation therapy?

نویسندگان

  • S Fargion
  • M T Taddei
  • V Gabutti
  • A Piga
  • A Di Palma
  • L Capra
  • G Fontanelli
  • A Avanzini
چکیده

Twenty-eight children with beta-thalassaemia major aged between 11 and 48 months were given intensive transfusions. Serum iron, transferrin saturation, serum ferritin, non-transferrin iron, and subcutaneous desferrioxamine-induced urinary iron excretion were measured. The results showed that even children with a limited number of transfusions had severe iron overload as indicated, in particular, by the raised serum ferritin levels and the high excretion rates after subcutaneous infusion of desferrioxamine. The desferrioxamine test was useful, even in very young children, in assessing response to chelation therapy thus enabling such treatment to be started early to prevent harm from iron overload.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Iron overload in Beta thalassaemia major and intermedia patients.

ABSTRACT BACKGROUND In beta thalassaemia major multiple blood transfusions, ineffective erythropoiesis and increased gastrointestinal iron absorption lead to iron overload in the body. Iron overload impairs the immune system, placing patients at greater risk of infection and illness. Iron overload can be determined by serum ferritin measurement. OBJECTIVE The aim of the present study is to...

متن کامل

Evaluation of the relationship between hepatic and cardiac iron overload with MRI T2* and carotid intima media thickness with Doppler ultrasound in beta thalassemia major patients

Background: Iron overload is caused early progression of atherosclerosis in beta thalassemia patients due to regular repeated blood transfusion. MRI T2* is a gold standard non-invasive method for detecting hepatic and cardiac iron overload. The aim of this study was the comparison of carotid intima media thickness (CIMT) in the patients and healthy control groups with Doppler ultrasound for ear...

متن کامل

Cardiac Function and Iron Chelation in Thalassemia Major and Intermedia: a Review of the Underlying Pathophysiology and Approach to Chelation Management

Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. Patients with homozygous thalassemia may have either a severe phenotype which is usually transfusion dependent or a milder form that is thalassemia intermedia. The two main factors that determine cardiac disease in homozygous β thalassemia are the high output state that results from chro...

متن کامل

Triple Drug Iron Chelation Therapy in Thalassemia Major; A Case Report

Regular packed cell transfusion in patients with thalassemia major leads to iron overload. Chelation therapy is one of the important aspects of thalassemia care as iron overload causes significant cardiac, hepatic, and endocrine dysfunction. We report a case of thalassemia major with severe iron overload causing cardiac and liver dysfunction who benefitted from triple drug chelation therapy. Tr...

متن کامل

Survival in beta-thalassaemia major in the UK: data from the UK Thalassaemia Register.

About 50% of UK patients with beta-thalassaemia major die before the age of 35 years, mainly because conventional iron-chelation therapy is too burdensome for full adherence. Patients require an individually-tailored treatment plan incorporating new, more tolerable approaches.

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Archives of disease in childhood

دوره 57 12  شماره 

صفحات  -

تاریخ انتشار 1982