beta-mannosidosis

ICD-10: Q77.1MeSH: D044905ORPHA: 118

Overview

lysosomal storage disease that has material basis in deficiency of the beta-A-manosidase enzyme resulting in the disruption of N-linked glycoprotein oligosaccharide catabolism

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 beta-mannosidosis, sourced from HPO and Orphanet clinical annotations.

Hearing impairmentIntellectual disabilitySeizureAbnormal facial shapeRecurrent respiratory infectionsHypoplasia of the abdominal wall musculature

Classification & Codes

ICD-10 Code

Q77.1

MeSH Code

D044905

Orphanet Code

ORPHA:118
beta-mannosidosis
ICD-10Q77.1
MeSHD044905
OrphanetORPHA:118
Treatments0 drug(s)
Symptoms on record6 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO