Individual dietary intervention in adult patients with mitochondrial disease due to the m.3243 A&gt;G mutation

Zweers, H.; Smit, Debbie; Leij, S.; Wanten, Geert; Janssen, Mirian C. H.

doi:10.1016/j.nut.2019.06.025

Cited by 9 publications

(14 citation statements)

References 30 publications

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“…The low physical functioning in MD patients may also be related to alterations in nutrition intake and nutrition status. Recent observational studies suggested that MD patients have inadequate protein intake and are at risk of malnutrition 9,24‐26 . This could affect both body composition and physical functioning.…”

Section: Introductionmentioning

confidence: 99%

“…Standard screening tools aimed at screening acute malnutrition are not applicable in MD patients, as these patients suffer from chronic malnutrition 24 . Recent literature advises to perform nutrition assessment to determine nutrition state in MD patients, 9,24,26 but it is not known which measurements are valid or which cutoff values should be applied.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Association of Body Composition, Physical Functioning, and Protein Intake in Adult Patients With Mitochondrial Diseases

Zweers

Bordier

Hulst

et al. 2020

J Parenter Enteral Nutr

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Background Whether decreased physical functioning of patients with mitochondrial disease (MD) is related to altered body composition or low protein intake needs clarification at the background of the nutrition state. Methods In this 2‐site cross‐sectional study, MD patients were age‐, body mass index (BMI)–, and gender‐matched to controls. Body composition was assessed by dual‐energy x‐ray absorptiometry. Physical functioning was measured by handgrip strength, 6‐minute walking test, 30‐second sit‐to‐stand test (30SCT), and 6‐minute mastication test. Total daily protein intake was calculated by 3‐day food records. Malnutrition was assessed by Patient‐Generated Subjective Global Assessment and the Global Leadership Initiative on Malnutrition (GLIM) criteria and sarcopenia by the 2018 consensus. Data were analyzed using independent samples t‐tests, Fisher exact test, and Spearman and Pearson correlation coefficients. Results Thirty‐seven MD patients (42 ± 12 years, BMI: 23 ± 4 kg/m2, 59% females) and 37 matched controls were included. Handgrip strength was moderate, inversely related to fat mass index in both MD patients and controls, whereas it correlated with fat‐free mass index in controls solely. Protein intake was associated with muscle strength (handgrip strength and 30SCT) in MD patients but not in controls. Twenty‐seven MD patients (73%) were malnourished, and 5 (14%) were classified as sarcopenic. Conclusions Muscle strength is related to body composition and protein intake in MD patients. This, in combination with the high incidence of both malnutrition and sarcopenia, warrants individual nutrition assessment in MD patients.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Association of Body Composition, Physical Functioning, and Protein Intake in Adult Patients With Mitochondrial Diseases

Zweers

Bordier

Hulst

et al. 2020

J Parenter Enteral Nutr

View full text Add to dashboard Cite

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“…Frequently occurring symptoms are hearing loss, gastrointestinal symptoms, exercise intolerance, diabetes, and myopathy 2 . Because specific treatment options remain limited, treatment is based on symptoms' management, like exercise 3 and nutrition care 4 …”

Section: Introductionmentioning

confidence: 99%

“…Accurate prediction of total energy expenditure (TEE) is crucial to guide individual dietary treatment 4 because mitochondrial disease (MD) patients are at risk for both malnutrition 5‐7 and overnutrition, with a higher risk for comorbidities and metabolic syndrome 4,8,9 …”

Section: Introductionmentioning

confidence: 99%

“…Defects in the OXPHOS system could theoretically lead to a lowered oxygen consumption rate at the cellular level, 10 with lower resting energy expenditure (REE). A lower fat‐free mass (FFM), as is frequently seen in MD, 4‐7 might also lower the REE 11 . Previously, Fiuza‐Luces et al (2016) did not find a lower oxygen consumption rate or lower REE when assessing indirect calorimetry (IC) in 20 children with MD 12 .…”

Section: Introductionmentioning

confidence: 99%

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Optimal Estimate for Energy Requirements in Adult Patients With the m.3243A>G Mutation in Mitochondrial DNA

Zweers

Janssen

Wanten

2020

J Parenter Enteral Nutr

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Aim We aimed to identify the optimal method to estimate total energy expenditure (TEE) in mitochondrial disease (MD) patients. Methods Resting energy expenditure (REE) was measured in MD patients carrying the m3243A>G mutation using indirect calorimetry (IC) and compared with results of 21 predictive equations (PEs) for REE and with REE‐IC measurements in healthy controls. Physical activity level (PAL) was measured using accelerometery (SenseWear) and compared with a fixed average PAL (1.4) as well as patients’ self‐estimated activity levels. TEE was calculated as REE‐IC × PAL SenseWear and compared with usual care and energy recommendations for healthy adults. Results Thirty‐eight MD patients (age: 48 ± 13 years; body mass index 24 ± 4 kg/m2; male 20%) and 25 matched controls were included. The accuracy of most PEs was between 63% and 76%. The difference in REE‐IC in healthy controls (1532 ± 182 kcal) and MD patients (1430 ± 221) was borderline not significant (P = .052). Patients’ estimations PAL were 18%–34% accurate at the individual level. The fixed activity factor was 53% accurate. Patients overestimated their PAL. Usual care predicted TEE accurately in only 32% of patients. Conclusion TEE is lower in these MD patients than the recommendations for healthy adults because of their lower physical activity. In MD patients, 6 PEs for REE provide a reliable alternative for IC, with an accuracy of 71%–76%. As PAL is highly variable and not reliably estimated by patients, measurement of PAL using accelerometery is recommended in this population.

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Dietary advice with or without oral nutritional supplements for disease-related malnutrition in adults

Baldwin

Schueren

Kruizenga

et al. 2021

Cochrane Database of Systematic Reviews

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Individual dietary intervention in adult patients with mitochondrial disease due to the m.3243 A>G mutation

Cited by 9 publications

References 30 publications

Association of Body Composition, Physical Functioning, and Protein Intake in Adult Patients With Mitochondrial Diseases

Association of Body Composition, Physical Functioning, and Protein Intake in Adult Patients With Mitochondrial Diseases

Optimal Estimate for Energy Requirements in Adult Patients With the m.3243A>G Mutation in Mitochondrial DNA

Dietary advice with or without oral nutritional supplements for disease-related malnutrition in adults

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