Zellweger syndrome

MeSH: D015211ORPHA: 912

Overview

congenital disorder of nervous system

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 Zellweger syndrome, sourced from HPO and Orphanet clinical annotations.

Abnormal pinna morphologyRespiratory insufficiencyHepatomegalyEEG abnormalitySkeletal dysplasiaShort statureDepressed nasal bridgeSevere muscular hypotoniaCorneal opacityVery long chain fatty acid accumulationFeeding difficulties in infancyEpiphyseal stipplingFlat faceProfound global developmental delayCognitive impairmentMulticystic kidney dysplasiaCryptorchidismHypospadiasHydronephrosisHigh palateMicrocephalyMacrocephalyMicrognathiaSensorineural hearing impairmentVisual impairmentCataractAbnormal chorioretinal morphologyPosterior embryotoxonNystagmusOptic atrophySeizurePremature birthPyloric stenosisMalabsorptionPolymicrogyriaFlat occiputClitoral hypertrophyUnderdeveloped supraorbital ridgesAbnormality of the tongueThickened nuchal skin foldGlaucomaBrushfield spotsVentricular septal defectAbnormality of coagulationPrimary adrenal insufficiencyWide anterior fontanelEpicanthusHigh foreheadWide nasal bridgeUpslanted palpebral fissureJaundiceReduced tendon reflexesHepatic failureFailure to thrive

Classification & Codes

MeSH Code

D015211

Orphanet Code

ORPHA:912
Zellweger syndrome
MeSHD015211
OrphanetORPHA:912
Treatments0 drug(s)
Symptoms on record54 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO