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Fuchs GJ, Tienboon P, Linpisarn S, Nimsakul S, Leelapat P, Tovanabutra S, et al. Nutritional factors and thalassaemia major. Arch Dis Child. 1996; 74(3): 224-7.

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Article

Nutritional Deficiencies Are Common in Patients with Transfusion-Dependent Thalassemia and Associated with Iron Overload

1Children’s Hospital Oakland Research Institute, 5700 Martin Luther King Jr. Way, Oakland CA, 94609

2Department of Hematology, UCSF Benioff Children’s Hospital Oakland, 5700 Martin Luther King Jr. Way, Oakland CA, 94609

3Clinical and Translational Sciences Institute, UCSF Benioff Children’s Hospital, 5700 Martin Luther King Jr. Way, Oakland CA, 94609


Journal of Food and Nutrition Research. 2018, Vol. 6 No. 10, 674-681
DOI: 10.12691/jfnr-6-10-9
Copyright © 2018 Science and Education Publishing

Cite this paper:
Elijah K Goldberg, Sushrita Neogi, Ashutosh Lal, Annie Higa, Ellen Fung. Nutritional Deficiencies Are Common in Patients with Transfusion-Dependent Thalassemia and Associated with Iron Overload. Journal of Food and Nutrition Research. 2018; 6(10):674-681. doi: 10.12691/jfnr-6-10-9.

Correspondence to: Ellen  Fung, Children’s Hospital Oakland Research Institute, 5700 Martin Luther King Jr. Way, Oakland CA, 94609. Email: efung@mail.cho.org

Abstract

Patients with thalassemia are frequently deficient in key micronutrients. Attempts to correct these inadequacies through nutritional supplementation have been met with some success, although disparities between intake and circulating levels continue to be observed. This study employed a convenience sample of 41 well-nourished transfusion dependent patients with thalassemia to identify possible mechanisms behind nutritional deficiencies. Each subject completed a Block 2005© Food Frequency Questionnaire (FFQ), through which macro and micronutrient intake was quantified. Fasting blood was drawn to assess vitamins A, C, D, E, copper, selenium, zinc and hematologic parameters. Dietary intake was found to be inadequate compared to Institute of Medicine (IOM) recommendations for many of the fat-soluble vitamins, as well as calcium and zinc. Circulating deficiencies of vitamins C, D, copper, zinc and γ tocopherol were also present in over 20% of patients. Many individuals who consumed an adequate dietary intake had deficient levels of circulating nutrients, which suggest alternative etiologies of nutrient excretion or loss, in addition to higher micronutrient requirements. Liver iron concentration displayed a significant negative relationship with vitamins C (r=-0.62, p<0.001), E (r=-0.37, p=0.03), and zinc (r=-0.35, p=0.037), indicating that in iron-overloaded patients, these nutrients are either endogenously consumed at higher rates or sequestered within the liver, resulting in a functional nutrient deficiency. While this study identified hepatic iron overload to be a significant cause of nutritional deficits commonly observed in patients with thalassemia, multiple etiologies are simultaneously responsible. In response to these findings, nutritional status should be monitored regularly in at-risk patients with thalassemia, and prophylactically addressed with supplementation or aggressive chelation to avoid associated co-morbidities.

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