Sialidosis type 2

Orpha code: 87876OMIM code: 256550

Definition

Sialidosis type 2 (ST-2) is a rare lysosomal storage disease, and the severe, early onset form of sialidosis (see this term) characterized by a progressively severe mucopolysaccharidosis-like phenotype (coarse facies, dysostosis multiplex, hepatosplenomegaly), macular cherry-red spots as well as psychomotor and developmental delay. ST-2 displays a broad spectrum of clinical severity with antenatal/congenital, infantile and juvenile presentations.

Disease data
Classification

Disease

Synonyms
Infantile dysmorphic sialidosis
Dziecięca sialidoza dysmorficzna
ORPHA code
87876
OMIM code
256550
ICD10 code
E77.1
ICD11 code
-

No additional description.

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