Assessment of Iron Overload in Very Young Children with Limited Thalassemia Care Resources in South China. (April 2014)

• Record Type: Journal Article
• Authors: Au, Wing-Yan; Li, Chun Fu; Fang, Jian Pei; Chen, Guang Fu; Sun, Xin; Li, Chang Gang; Zhang, Xin Hua; Wu, Xue Dong; Gao, Hong Ying; Hao, Wen Ge; Rasalkar, Darshana; Deng, Min; Mok, Sio Peng A.; Tricta, Fernando; Chu, Winnie C.W.
• Is Part Of: Hemoglobin. Volume 38:Number 2(2014)
• Page Start: 119
• Page End: 126
• Publication Date: 2014-04
• Subjects: Hemoglobinopathy -- Periodicals; Hemoglobin -- Periodicals; Hematology -- Periodicals; Thalassemia -- Periodicals; Blood -- Diseases -- Periodicals; 612.1111
• DOI: 10.3109/03630269.2014.880715
• Languages: English
• ISSNs: 0363-0269

Abstract

<abstract> <title>Abstract</title> <p>Southern China has one of the world's largest population of patients needing transfusions. Transfusion and chelation are not uniformly available and no magnetic resonance imaging (MRI) assessment data exists to date. A total of 153 young β-thalassemia major (β-TM) patients were assessed using a validated 1.5T scanner in Hong Kong, People's Republic of China (PRC). Their median age was 13 (range 7 to 30), and most patients were young (22.0% age &lt;10, 73.0% age &lt;15, 88.0% age &lt;18). Erratic health care made estimation of total transfusion and chelation exposure impossible. Despite their early age, 24.0% had severe cardiac hemosiderosis [T2*&lt;10 milliseconds (ms)], at ages as early as 8 years old. Median heart iron was 1.68 mg/g dry weight (range 0.19–7.66) and increased with age (<italic>p</italic> = 0.017), while liver iron was 22.2 mg/g dry weight (range 3.15 to 39.2). Serum ferritin levels were poor predictors of heart and liver, or pancreatic R* and pituitary R* values. Magnetic resonance imaging scans are needed to screen very young β-TM patients with immediate risk of premature cardiac death in developing nations and triage them to more intensive treatment. This is particularly important in countries with a large number of patients and limited resources. Our data suggests that in developing countries, there is no lower limit for thalassemia MRI scanning programs.</p> </abstract>