Patients With Mitochondrial Disease Have an Inadequate Nutritional Intake

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


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.

Keywords: malnutrition; mitochondrial disease; nutrition diaries; nutritional intake.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Calcium, Dietary / administration & dosage
  • Cross-Sectional Studies
  • Dairy Products
  • Diet Records
  • Diet Surveys
  • Diet*
  • Diet, Healthy
  • Dietary Proteins / administration & dosage
  • Dietary Sugars / administration & dosage
  • Eating / physiology*
  • Female
  • Humans
  • Male
  • Malnutrition / complications
  • Malnutrition / physiopathology
  • Micronutrients
  • Middle Aged
  • Mitochondria / physiology
  • Mitochondrial Diseases / complications
  • Mitochondrial Diseases / physiopathology*
  • Netherlands
  • Nutritional Status
  • Recommended Dietary Allowances
  • Retrospective Studies


  • Calcium, Dietary
  • Dietary Proteins
  • Dietary Sugars
  • Micronutrients