mucopolysaccharidosis I

MeSH: D008059ORPHA: 579

Overview

lysosomal storage 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 mucopolysaccharidosis I, sourced from HPO and Orphanet clinical annotations.

Inguinal herniaThick lower lip vermilionGingival overgrowthEverted lower lip vermilionHydrocephalusSinusitisMacrocephalyDolichocephalyAbnormality of the faceCoarse facial featuresFull cheeksLow anterior hairlineHearing impairmentChronic otitis mediaSensorineural hearing impairmentRetinopathyGlaucomaVisual impairmentOptic atrophyWidely spaced teethMicrodontiaAbnormal metaphysis morphologySplit handIntellectual disabilityJoint dislocationJoint stiffnessAbnormality of the voiceCongestive heart failureHypertrophic cardiomyopathyAbnormal aortic valve morphologyAbnormal heart valve morphologySplenomegalyMalabsorptionApneaRecurrent respiratory infectionsGeneralized hirsutismDevelopmental regressionScoliosisArthralgiaAbnormal hip bone morphologyAbnormal form of the vertebral bodiesParesthesiaSpinal canal stenosisShort statureHemiplegia/hemiparesisAbnormal nasal morphologyDepressed nasal bridgeAbnormal epiphysis morphologyCorneal opacityMucopolysacchariduriaThick nasal alaeAvascular necrosisCoughAbnormal tendon morphologyEnlarged thoraxAbnormality of the tonsilsHernia

Classification & Codes

MeSH Code

D008059

Orphanet Code

ORPHA:579
mucopolysaccharidosis I
MeSHD008059
OrphanetORPHA:579
Treatments0 drug(s)
Symptoms on record57 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO