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Udgivet

Impaired lipolysis in propionic acidemia: A new metabolic myopathy?

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Vis graf over relationer

The objective of this study was to investigate the fat and carbohydrate metabolism in a patient with propionic acidemia (PA) during exercise by means of indirect calorimetry and stable isotope technique. A 34-year-old patient with PA performed a 30-minute submaximal cycle ergometer test. Data were compared to results from six gender- and age-matched healthy controls. Main findings are that the patient with PA had impaired lipolysis, blunted fatty acid oxidation, compensatory increase in carbohydrate utilization, and low work capacity. Our findings indicate that PA should be added to the list of metabolic myopathies.

OriginalsprogEngelsk
TidsskriftJIMD Reports
Vol/bind53
Udgave nummer1
Sider (fra-til)16-21
Antal sider6
ISSN2192-8304
DOI
StatusUdgivet - maj 2020

Bibliografisk note

© 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.

ID: 59938310