combined oxidative phosphorylation deficiency 7

ORPHA: 254930

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 7, sourced from HPO and Orphanet clinical annotations.

Visual impairmentOptic atrophySkeletal muscle atrophyOphthalmoplegiaVisual field defectGlobal developmental delayGait disturbanceFailure to thrivePes cavusSpastic paraparesisLower limb hyperreflexiaDistal sensory impairmentPeripheral axonal neuropathyDistal amyotrophyAbnormal pyramidal signLower limb muscle weaknessImpaired tandem gaitCognitive impairmentPtosisNystagmusAtaxiaDysarthriaBulbar palsyAreflexiaFacial diplegiaHypoplasia of the corpus callosumDevelopmental regressionAbnormal cerebral white matter morphologyInability to walkParalytic ileusThoracic scoliosisDecreased number of peripheral myelinated nerve fibersUpper limb muscle weaknessImpaired masticationDyschromatopsiaFloppy infantGastrostomy tube feeding in infancyAbnormal thalamic MRI signal intensityElevated brain lactate level by MRSAbnormal brainstem MRI signal intensityExodeviationOral-pharyngeal dysphagia

Classification & Codes

Orphanet Code

ORPHA:254930
combined oxidative phosphorylation deficiency 7
OrphanetORPHA:254930
Treatments0 drug(s)
Symptoms on record42 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO