Mucopolysaccharidosis type 2

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Mucopolysaccharidosis type 2, attenuated form

ORPHA:217093Clinical subtype

Also called Hunter syndrome type B · Iduronate 2-sulfatase deficiency type B · MPS2B · MPSIIB · Mucopolysaccharidosis type 2, non-neuropathic form · Mucopolysaccharidosis type 2, slowly progressive form · Mucopolysaccharidosis type 2B · Mucopolysaccharidosis type II, attenuated form · Mucopolysaccharidosis type IIB

What it is

Mucopolysaccharidosis type 2, attenuated form (MPS2att), the less severe form of MPS2, leads to a massive accumulation of glycosaminoglycans and a wide variety of symptoms including distinctive facies, short stature, cardiorespiratory and skeletal findings. It is differentiated from mucopolysaccharidosis type 2, severe form by the absence of cognitive decline.

Key facts

Age of onset
Childhood
Inheritance
X-linked recessive
Classified as
Clinical subtype

Recorded for the broader condition

Prevalence
1-9 / 1 000 000 (Europe)Mucopolysaccharidosis type 2

Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.

Gene

IDSDisease-causing germline mutation(s)

ICD-10 codes

E76.1filed under a broader ICD-10 category — shared with 2 other rare diseases

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

MONDO 0016316OMIM 309900UMLS C5679815

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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