congenital sialidosis type 2

ORPHA: 934001 Treatment Available

Overview

human disease

Available Treatments (1)

DrugFormStatusCountriesLead Time
laronidase
Orphan Cold Chain
IV infusion, 0.58 mg/mLFDA Approved, EMA Approved814d

Clinical Presentation

Signs and symptoms associated with congenital sialidosis type 2, sourced from HPO and Orphanet clinical annotations.

Coarse facial featuresHearing impairmentLow-set earsCataractDysostosis multiplexEdemaHypotoniaGlobal developmental delayHepatosplenomegalyAscitesCherry red spot of the maculaInguinal herniaAbnormality of the kidneyHydrocephalusStrabismusDevelopmental cataractVisual lossNystagmusOptic atrophyPetechiaeTelangiectasiaSeizureAtaxiaSpasticityDysmetriaMyoclonusUmbilical herniaAbnormal heart morphologyDevelopmental regressionHypoplasia of the foveaCorneal opacityUnderdeveloped supraorbital ridgesRespiratory tract infectionYellow/white retinal lesionGingival overgrowthMacrocephalyHigh foreheadHepatomegalyAbnormal EKGGeneralized hypertrichosisPolydactylyProtruding tongue

Classification & Codes

Orphanet Code

ORPHA:93400
congenital sialidosis type 2
OrphanetORPHA:93400
Treatments1 drug(s)
Symptoms on record42 signs
Statuspublished

Treatment Summary

Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
congenital sialidosis type 2 | OrphanDrug