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营养缺乏在依赖输血的地中海贫血患者中很常见,且与铁过载相关。

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

作者信息

Goldberg Elijah K, Neogi Sushrita, Lal Ashutosh, Higa Annie, Fung Ellen

机构信息

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

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

出版信息

J Food Nutr Res (Newark). 2018;6(10):674-681. doi: 10.12691/jfnr-6-10-9. Epub 2018 Nov 21.

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.

摘要

地中海贫血患者常常缺乏关键的微量营养素。尽管摄入量与循环水平之间仍存在差异,但通过营养补充来纠正这些不足已取得了一些成效。本研究采用便利抽样法,选取了41名营养状况良好的依赖输血的地中海贫血患者,以确定营养缺乏背后的可能机制。每位受试者都完成了一份Block 2005©食物频率问卷(FFQ),通过该问卷对常量营养素和微量营养素的摄入量进行了量化。采集空腹血样以评估维生素A、C、D、E、铜、硒、锌及血液学参数。与美国医学研究所(IOM)对多种脂溶性维生素、钙和锌的建议相比,发现膳食摄入量不足。超过20%的患者还存在维生素C、D、铜、锌和γ生育酚的循环缺乏。许多饮食摄入量充足的个体循环营养素水平却不足,这表明除了微量营养素需求较高外,还存在营养物质排泄或流失的其他病因。肝脏铁浓度与维生素C(r=-0.62,p<0.001)、E(r=-0.37,p=0.03)和锌(r=-0.35,p=0.037)呈显著负相关,表明在铁过载患者中,这些营养素要么以更高的速率内源性消耗,要么被隔离在肝脏内,导致功能性营养缺乏。虽然本研究确定肝脏铁过载是地中海贫血患者常见营养缺乏的一个重要原因,但多种病因同时起作用。针对这些发现,应定期监测地中海贫血高危患者的营养状况,并通过补充或积极螯合进行预防性处理,以避免相关合并症。

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