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地中海贫血症的营养问题:一项对缺乏症、与发病率的关系以及补充建议的系统评价。

Nutrition in Thalassemia: A Systematic Review of Deficiency, Relations to Morbidity, and Supplementation Recommendations.

机构信息

Children's Hospital Oakland Research Institute (CHORI).

Division of Hematology, UCSF Benioff Children's Hospital Oakland, Oakland, CA.

出版信息

J Pediatr Hematol Oncol. 2022 Jan 1;44(1):1-11. doi: 10.1097/MPH.0000000000002291.

Abstract

BACKGROUND

Reports of nutritional deficiencies in patients with thalassemia (Thal) are common. Despite its importance, however, nutritionally focused research in Thal has been limited by inadequate sample size, inconsistent methodology, a lack of control comparisons, and few interventional trials. Due to these limitations, clinicians lack evidence-based nutrition recommendations to support clinical decision-making. This systematic review summarizes observed relationships between nutrition and morbidity in Thal published in the last 3 decades.

METHODS

PubMed, Web of Science, and Embase were screened for articles pertaining to nutrition in Thal using comprehensive search terms. Studies performed in humans, written in English, and published between 1990 and 2020 were included. Over 2100 manuscripts were identified, from which 97 were included.

RESULTS

Patients with Thal were most often deficient in vitamins A, C, D, selenium, and zinc. Prevalence of nutritional deficiency was positively correlated with age and iron overload. Evidence to support the role of vitamin D and zinc for bone health was observed; zinc was also found to improve glucose metabolism.

CONCLUSIONS

Due to the risk for multinutrient deficiency, nutritional status should be assessed annually in patients with Thal with prompt nutrient replacement when deficiency is detected. Routine supplementation with vitamin D and zinc is recommended.

摘要

背景

报道称,地中海贫血症(Thal)患者存在营养缺乏问题。然而,尽管这一点很重要,但由于样本量不足、方法不一致、缺乏对照比较以及干预试验较少等因素,以营养为重点的 Thal 研究受到限制。由于这些限制,临床医生缺乏循证营养建议来支持临床决策。本系统综述总结了过去 30 年来发表的关于 Thal 中营养与发病率之间关系的观察性研究。

方法

使用全面的搜索词在 PubMed、Web of Science 和 Embase 上筛选与 Thal 营养相关的文章。纳入人类研究、英文书写且发表于 1990 年至 2020 年的研究。共确定了 2100 多篇论文,其中 97 篇被纳入。

结果

Thal 患者常缺乏维生素 A、C、D、硒和锌。营养缺乏的患病率与年龄和铁过载呈正相关。观察到维生素 D 和锌对骨骼健康有作用的证据;锌也被发现可改善葡萄糖代谢。

结论

由于存在多种营养素缺乏的风险,应每年对 Thal 患者进行营养状况评估,并在发现缺乏时及时补充营养素。建议常规补充维生素 D 和锌。

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