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线粒体疾病患者的营养摄入不足。

Patients With Mitochondrial Disease Have an Inadequate Nutritional Intake.

机构信息

Radboud Center for Mitochondrial Medicine, Radboudumc, Nijmegen, The Netherlands.

Department of Gastroenterology and Hepatology, Radboudumc, Nijmegen, The Netherlands.

出版信息

JPEN J Parenter Enteral Nutr. 2018 Mar;42(3):581-586. doi: 10.1177/0148607117699792. Epub 2017 Dec 18.

Abstract

BACKGROUND

Mitochondrial disease (MD) is a group of disorders caused by dysfunctional mitochondria, the organelles that generate energy for the cell. Malnutrition in patients with MD may lead to increased mitochondrial dysfunction, which may enhance already existing symptoms. The aim of this study was to investigate whether patients with MD have an insufficient or unbalanced food intake and to establish which nutrients and product groups are particularly compromised in this patient group.

METHODS

In this observational, cross-sectional, retrospective study, sixty 3-day nutrition diaries of adult patients with MD were analyzed and compared with the Dutch recommended daily allowance and the Dutch National Food Consumption Survey (DNFCS).

RESULTS

The intake of all macronutrients and micronutrients of patients with MD was significantly different from Dutch recommended daily allowance values with the exception of fat and iron. In particular, protein and calcium intake in patients with MD was significantly lower when compared with the DNFCS. Interindividual differences were high. Also, intake of fiber, sugars, saturated fat, and vitamin D differed from recommendations for the overall population. In comparison with DNFCS, the intake of dairy products and drinks was significant lower in patients.

CONCLUSIONS

Our study demonstrates that many patients with MD have an inadequate diet. Specifically, intake of protein, calcium, dairy products, and fluids were low. Overall, eating a healthy diet seems as difficult for patients with MD as for the general population. Since interindividual differences are high, individual diet counseling is recommended for all adult patients with MD.

摘要

背景

线粒体疾病(MD)是一组由功能失调的线粒体引起的疾病,线粒体是为细胞产生能量的细胞器。MD 患者的营养不良可能导致线粒体功能进一步受损,从而加重已有的症状。本研究旨在调查 MD 患者的饮食摄入是否不足或不平衡,并确定该患者群体中哪些营养素和产品组受到了特别影响。

方法

在这项观察性、横断面、回顾性研究中,分析了 60 名成年 MD 患者的 3 天营养日记,并将其与荷兰推荐的每日摄入量和荷兰国家食品消费调查(DNFCS)进行了比较。

结果

MD 患者的所有宏量营养素和微量营养素的摄入量与荷兰推荐的每日摄入量值显著不同,除了脂肪和铁。特别是与 DNFCS 相比,MD 患者的蛋白质和钙摄入量明显较低。个体间差异很大。此外,纤维、糖、饱和脂肪和维生素 D 的摄入量也与一般人群的建议值不同。与 DNFCS 相比,患者的乳制品和饮料摄入量显著较低。

结论

我们的研究表明,许多 MD 患者的饮食摄入不足。具体来说,蛋白质、钙、乳制品和液体的摄入量较低。总的来说,对于 MD 患者和一般人群来说,健康饮食似乎都很困难。由于个体间差异很大,建议对所有成年 MD 患者进行个体化饮食咨询。

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