very long chain acyl-CoA dehydrogenase deficiency

ORPHA: 26793

Overview

human disease

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with very long chain acyl-CoA dehydrogenase deficiency, sourced from HPO and Orphanet clinical annotations.

Increased circulating free fatty acid levelJaundiceSmall for gestational ageVentricular septal defectAtrial septal defectPatent foramen ovaleHypoketotic hypoglycemiaHypothermiaRespiratory distressHepatomegalyEpisodic tachypneaElevated circulating hepatic transaminase concentrationElevated circulating creatine kinase concentrationExercise-induced rhabdomyolysisFeeding difficultiesOverweightMacrocephalyLethargyObesityAnteriorly placed anusDilated cardiomyopathyTachycardiaProlonged QT intervalVentricular fibrillationAtrioventricular blockPericardial effusionMetabolic acidosisHyperammonemiaVomitingPneumoniaEnlarged cisterna magnaTachypneaHypocalcemiaHypoproteinemiaMuscle spasmVentricular tachycardiaFloppy infantInflammatory abnormality of the skinArrhythmiaPain

Classification & Codes

Orphanet Code

ORPHA:26793
very long chain acyl-CoA dehydrogenase deficiency
OrphanetORPHA:26793
Treatments0 drug(s)
Symptoms on record40 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO