Forskning
Udskriv Udskriv
Switch language
Rigshospitalet - en del af Københavns Universitetshospital
Udgivet

Impaired lipolysis in propionic acidemia: A new metabolic myopathy?

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

  1. Genotype and phenotype classification of 29 patients affected by Krabbe disease

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. Impaired fat oxidation during exercise in multiple acyl-CoA dehydrogenase deficiency

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Diagnostic pitfalls in vitamin B6-dependent epilepsy caused by mutations in the PLPBP gene

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  4. Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease: A Nationwide Study and Review of the Literature

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  1. The effect of acute dual SGLT1/SGLT2 inhibition on incretin release and glucose metabolism after gastric bypass surgery

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  2. Glucagon Resistance at the Level of Amino Acid Turnover in Obese Subjects with Hepatic Steatosis

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  3. Increased risk of sudden death in untreated Primary Carnitine Deficiency

    Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

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