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Are patients affected by mitochondrial disorders at nutritional risk?
Emilie Aubry1, Carla Aeberhard1, Lia Bally1
1Division of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Bern University Hospital and University of Bern, Switzerland.
Nutrition (Burbank, Los Angeles County, Calif.)
|February 13, 2018
Summary
Patients with mitochondrial disorders (MD) often have malnutrition, despite standard screening tools missing this. This study highlights the need for better nutritional assessment and increased protein intake in MD patients to improve their health and quality of life.
Area of Science:
- Clinical Nutrition
- Mitochondrial Disorders
- Gastrointestinal Health
Background:
- Mitochondrial disorders (MD) frequently cause gastrointestinal issues, impacting food intake and nutritional status.
- Deterioration in nutritional state can exacerbate the progression of mitochondrial disorders.
- Effective identification of nutritional deficiencies is crucial for managing MD patients.
Purpose of the Study:
- To evaluate a simple screening tool for nutritional risk in MD patients.
- To conduct an extended nutritional assessment to identify deficiencies in MD patients compared to controls.
- To explore the relationship between nutritional status, symptoms, and quality of life in MD.
Main Methods:
- Prospective cohort study comparing MD outpatients with matched healthy controls.
- Nutritional screening (NRS-2002) and comprehensive nutritional assessments were performed.
- Included dietary habits, body composition, energy expenditure, quality of life (QoL), and biochemical analyses.
Main Results:
- No MD patients were flagged as malnourished by the NRS-2002 screening tool.
- MD patients exhibited significantly lower muscle mass, reduced handgrip strength, and poorer QoL.
- MD patients showed a significantly lower protein intake and abnormal creatinine height index, indicating malnutrition.
Conclusions:
- Standard nutritional screening tools may be insensitive for chronically ill MD outpatients.
- All MD patients met malnutrition criteria based on ESPEN/ASPEN definitions.
- Increased protein intake and adapted energy stores are recommended to improve MD patient outcomes and QoL.
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