peroxisomal acyl-CoA oxidase deficiency

MeSH: C536662ORPHA: 2971

Overview

peroxisomal disease that is characterized by hypotonia and seizures in the neonatal period and neurological regression in early infancy

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 peroxisomal acyl-CoA oxidase deficiency, sourced from HPO and Orphanet clinical annotations.

EpicanthusHypertelorismLow-set earsSensorineural hearing impairmentStrabismusAbnormal electroretinogramMyopiaNystagmusOptic atrophyAbnormality of visual evoked potentialsHypodontiaHand polydactylySeizureHypotoniaGlobal developmental delayHypertoniaGait disturbanceHyperreflexiaFailure to thriveAbnormality of metabolism/homeostasisRespiratory insufficiencyAbnormal speech patternHepatomegalyEEG abnormalityDevelopmental regressionDepressed nasal bridgeSevere intellectual disabilityAbnormal nervous system morphology

Classification & Codes

MeSH Code

C536662

Orphanet Code

ORPHA:2971
peroxisomal acyl-CoA oxidase deficiency
MeSHC536662
OrphanetORPHA:2971
Treatments0 drug(s)
Symptoms on record28 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO