congenital disorder of glycosylation type II

ORPHA: 79329

Overview

congenital disorder of glycosylation that involves malfunctioning trimming/processing of the protein-bound oligosaccharide chain

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 type II, sourced from HPO and Orphanet clinical annotations.

Posteriorly rotated earsGlobal developmental delayAbnormal facial shapeReduced level of N-acetylglucosaminyltransferase IIProgressive microcephalySeizureHypotoniaFailure to thriveOpen mouthDolichocephalyHypertelorismAbnormal earlobe morphologyProminent antihelixConvex nasal ridgeDownslanted palpebral fissuresLong eyelashesDental crowdingPectus excavatumAbnormality of the endocrine systemOsteopeniaHirsutismBrachydactylyGeneralized hypotoniaAbnormal heart morphologyVentricular septal defectPatent ductus arteriosusHydrops fetalisAbnormal bleedingReduced factor XI activityAbnormal scalp morphologyGastroesophageal refluxRespiratory distressHypsarrhythmiaHypoplastic nipplesGastroparesisScoliosisKyphosisInverted nipplesImpaired lymphocyte transformation with phytohemagglutininImpaired platelet aggregationDecreased circulating immunoglobulin concentrationDecreased circulating IgG concentrationCombined immunodeficiencyLow hanging columellaAbnormality of the common coagulation pathwayGastrostomy tube feeding in infancyArrhythmiaFeeding difficultiesStereotypical hand wringingBrain atrophyInfantile spasmsDecreased mitogen-induced T-cell proliferationRecurrent upper and lower respiratory tract infectionsReflex asystolic syncope

Classification & Codes

Orphanet Code

ORPHA:79329
congenital disorder of glycosylation type II
OrphanetORPHA:79329
Treatments0 drug(s)
Symptoms on record54 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO