long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency

ORPHA: 5

Overview

medical condition

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 long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, sourced from HPO and Orphanet clinical annotations.

PhotophobiaHypoglycemiaHypoketotic hypoglycemiaAbnormal electroretinogramVisual lossExotropiaHypotoniaGlobal developmental delayHypertrophic cardiomyopathyAbnormality of metabolism/homeostasisHepatomegalyPeripheral neuropathyRetinopathyAbnormal chorioretinal morphologyChorioretinal atrophyMyopiaNyctalopiaIntellectual disabilitySeizureGeneralized hypotoniaFailure to thriveCholestatic liver diseaseAbnormal retinal pigmentationFeeding difficultiesPosterior staphyloma

Classification & Codes

Orphanet Code

ORPHA:5
long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency
OrphanetORPHA:5
Treatments0 drug(s)
Symptoms on record25 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO