Fibroblast growth factor 21 as a biomarker for long-term complications in organic acidemias.

ABSTRACT:

Background

There is increasing evidence that long-term complications in organic acidemias are caused by impaired mitochondrial metabolism. Currently, there is no specific biomarker to monitor mitochondrial dysfunction in organic acidemias. Serum fibroblast growth factor 21 (FGF-21) is a biomarker for mitochondrial disease and could be a candidate to monitor mitochondrial function in the deleterious course of disease.

Methods

Data of 17 patients with classical organic acidemias (11 propionic acidemia (PA), four methylmalonic acidemia (MMA) and two isovaleric acidemia (IVA) patients) were included. The clinical course was evaluated; metabolic decompensations and long-term complications were correlated with plasma FGF-21 levels. Cardiomyopathy, prolonged QT interval, renal fail

SUBMITTER: Molema F 

PROVIDER: S-EPMC6327009 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature