congenital disorder of glycosylation Ih

ORPHA: 79325

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 Ih, sourced from HPO and Orphanet clinical annotations.

Abnormality of the nervous systemHypotoniaAbnormal facial shapeAbnormality of the eyeCataractEdemaSeizureGlobal developmental delayAscitesPremature birthHydrops fetalisThrombocytopeniaHyponatremiaElevated circulating hepatic transaminase concentrationAbnormality of the coagulation cascadeType I transferrin isoform profileAbnormality of the gastrointestinal tractAbnormal skin morphologyMacroglossiaHypertelorismLow-set earsRetinopathyNystagmusOptic atrophyCutis laxaAbnormality of subcutaneous fat tissueBrachydactylyAtaxiaFailure to thriveIntrauterine growth retardationSmall for gestational ageOligohydramniosTalipes equinovarusAnemiaReduced factor XI activityReduced antithrombin III activityVomitingDiarrheaHypoplasia of the corpus callosumVentriculomegalyCerebral cortical atrophyLeukoencephalopathyLeukodystrophyInverted nipplesReduced protein C activityFeeding difficultiesCamptodactylyPremature skin wrinkling

Classification & Codes

Orphanet Code

ORPHA:79325
congenital disorder of glycosylation Ih
OrphanetORPHA:79325
Treatments0 drug(s)
Symptoms on record48 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO