combined oxidative phosphorylation deficiency 23

ORPHA: 444013

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 combined oxidative phosphorylation deficiency 23, sourced from HPO and Orphanet clinical annotations.

Hypertrophic cardiomyopathyLactic acidosisAbnormality of the nervous systemFloppy infantVisual impairmentCyanosisSeizureMild intellectual disabilityGlobal developmental delayFailure to thriveCongestive heart failureRight ventricular hypertrophyLeft ventricular hypertrophyWolff-Parkinson-White syndromeLeukodystrophyRespiratory failureEasy fatigabilityDecreased activity of mitochondrial complex IVFeeding difficulties in infancyStridorDecreased activity of mitochondrial complex ISeverely reduced left ventricular ejection fractionAbnormal thalamic MRI signal intensityAbnormal brainstem MRI signal intensityAbnormal basal ganglia MRI signal intensityParoxysmal dyspneaCognitive impairment

Classification & Codes

Orphanet Code

ORPHA:444013
combined oxidative phosphorylation deficiency 23
OrphanetORPHA:444013
Treatments0 drug(s)
Symptoms on record27 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO