Fucosidosis

Orpha code: 349OMIM code: 230000

Definition

Fucosidosis is an extremely rare lysosomal storage disorder characterized by a highly variable phenotype with common manifestations including neurologic deterioration, coarse facial features, growth retardation, and recurrent sinopulmonary infections, as well as seizures, visceromegaly, angiokeratoma and dysostosis.

Disease data
Classification

Disease

Synonyms
Niedobór alfa-L-fukozydazy
Alpha-L-fucosidase deficiency
ORPHA code
349
OMIM code
230000
ICD10 code
E77.1
ICD11 code
5C56.21

No additional description.

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