congenital disorder of glycosylation Ie

ORPHA: 79322

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 congenital disorder of glycosylation Ie, sourced from HPO and Orphanet clinical annotations.

SeizureElevated circulating creatine kinase concentrationSecondary microcephalyFloppy infantNeurodevelopmental delayHypertelorismFailure to thriveReduced antithrombin III activityHigh, narrow palateElevated circulating hepatic transaminase concentrationReduced protein S activityReduced protein C activityDelayed myelinationAbnormal dentate nucleus morphologyTrigonocephalyFull cheeksSmooth philtrumMicrognathiaStrabismusRetinopathyDownslanted palpebral fissuresEsotropiaNystagmusOptic atrophyAtaxiaSpasticityCerebellar atrophyEncephalopathyHepatic fibrosisHepatic steatosisHepatosplenomegalyLong halluxSandal gapDiarrheaProminent glabellaCerebral atrophyVentriculomegalyGeneralized myoclonic seizureNail dysplasiaHepatomegalyInverted nipplesExternal genital hypoplasiaMuscular dystrophyDepressed nasal bridgeFlat occiputKnee flexion contracturePontocerebellar atrophyHypoplasia of the frontal lobesLimb undergrowthPeripheral neuropathyTented upper lip vermilionU-Shaped upper lip vermilionGeneralized tonic seizureAtonic seizureEarly onset absence seizuresGastrostomy tube feeding in infancyCamptodactylyAbnormal visual fixationCerebral visual impairment

Classification & Codes

Orphanet Code

ORPHA:79322
congenital disorder of glycosylation Ie
OrphanetORPHA:79322
Treatments0 drug(s)
Symptoms on record59 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
congenital disorder of glycosylation Ie | OrphanDrug