Clinical Trial: Nutritional Assessment in Mitochondrial Cytopathy

Study Status: Completed
Recruit Status: Completed
Study Type: Observational

Official Title: Nutritional Assessment in Patients Affected by Mitochondrial Cytopathy

Brief Summary: The aim of this study is to assess nutritional intake (quantitatively and qualitatively), nutritional state and body composition of patients suffering from mitochondrial cytopathy, compared to healthy controls. The energy intake will be calculated through dietary protocols, the energy expenditure by indirect calorimetry and body composition will be performed with bio-impedance analysis. Further on, the investigators expect to be able to provide nutritional counselling to this population in order to increase energy and protein intake, which may improve health and well-being.

Detailed Summary:

Background

Mitochondrial diseases are a group of genetic disorders. The prognosis for patients with mitochondrial myopathies varies greatly, depending largely on the type of disease and the degree of involvement of various organs. This situation can lead into malnutrition and worse the outcome. In the literature there are no data, which deal with malnutrition or nutritional intake in patients with mitochondrial myopathy. Sorensen et al. assessed the malnutrition risk in European hospitals and showed that 45% of patients with neurological illness (neurological vascular disease excluded) are at high risk of malnutrition. Furthermore, the patient at risk exhibited higher rates of complications and mortality. These findings suggest that malnutrition play an important role in the clinical outcome of this patient's population, reflecting the investigators' many years of experience. In several case reports, it was shown that malnutrition itself could aggravate myopathy. Sometimes there are challenging symptoms facing patients with mitochondrial myopathy that interfere with obtaining balanced and healthy nutrition, such as fatigue, muscle weakness, dysmotility, dysphagia, nausea and vomiting, ataxia, and reflux. In addition, in the investigators' clinical experience, they see many patients who describe that some specific food intake would even modify their symptoms.

This is an observational cohort study, and the investigators expect a total sample size of 30 patients and 20 healthy age- and sex-matched controls. The entire study will be conducted during the period from first of October 2014 till End of March 2015.

The investigator is a pharmacy student (master level) interested in clinical nutrition. She will collect the data und do all measurements along with the support of the cl
Sponsor: University Hospital Inselspital, Berne

Current Primary Outcome: Energy and nutrients intake (Measured in kcal) [ Time Frame: At baseline ]

Measured in kcal (7 days food recall protocol to check for nutrients which aggravate symptoms & to provide nutritional counselling)


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Differences in nutritional risk (Measured in NRS2002) [ Time Frame: At baseline ]
    Measured in NRS2002 score points
  • Differences in nutritional status Measured in laboratory parameters (blood and urine) [ Time Frame: At baseline ]
    Measured in laboratory parameters (blood and urine)
  • Difference in body composition Measured in bioimpedance analysis BIA and anthropometrics (TSF [mm], MAMC (cm^2), Handgrip test (kg)) [ Time Frame: At baseline ]
  • Difference in resting energy expenditure Measured in kcal (indirect calorimetry) [ Time Frame: At baseline ]
    Measured in kcal (indirect calorimetry)
  • Difference in quality of life Measured in SF36v2 score [ Time Frame: At baseline ]
    Measured in SF36v2 score


Original Secondary Outcome: Same as current

Information By: University Hospital Inselspital, Berne

Dates:
Date Received: February 24, 2015
Date Started: December 2014
Date Completion:
Last Updated: April 5, 2016
Last Verified: April 2016