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Start free with EleplanMucopolysaccharidosis type 2, severe form
ORPHA:217085Clinical subtype
Also called Hunter syndrome type A · Iduronate 2-sulfatase deficiency type A · MPS2A · MPSIIA · Mucopolysaccharidosis type 2, early progressive form · Mucopolysaccharidosis type 2, neuropathic form · Mucopolysaccharidosis type 2A · Mucopolysaccharidosis type II, severe form · Mucopolysaccharidosis type IIA
What it is
Mucopolysaccharidosis type 2 (MPS2), severe form (MPS2S), is associated with a massive accumulation of glycosaminoglycans and a wide variety of symptoms including a rapidly progressive cognitive decline; it is most often fatal in the second or third decade.
Key facts
- Prevalence
- 1-9 / 1 000 000 (at birth, Europe)
- Age of onset
- Childhood
- Inheritance
- X-linked recessive
- Classified as
- Clinical subtype
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Gene
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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