5C56.31 Mucopolysaccharidosis type 2
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01
Mucopolysaccharidosis type 2 (MPS 2) is a lysosomal storage disease belonging to the group of mucopolysaccharidoses. The clinical picture ranges from severe (the most frequent form) with early psychomotor regression, facial dysmorphism (macroglossia, constantly opened mouth, coarse features), hepatosplenomegaly, limited joint motion, carpal tunnel syndrome, dysostosis multiplex, small size, behavioural disorders and psychomotor regression leading to intellectual deficit, deafness, cardiac and respiratory disorders, and cutaneous signs, to mild (normal intelligence, milder dysmorphism and dysostoses).
inclusions
- Hunter syndrome
synonyms
- Mucopolysaccharidosis type 2
- Hunter syndrome
- Iduronate 2-sulfatase deficiency
- MPS2 - [mucopolysaccharidosis 2]
- iduronate 2-sulphatase deficiency
- iduronate sulfatase deficiency
- iduronate sulphatase deficiency
- sulfo-iduronate sulfatase deficiency
- sulfoiduronidate sulfatase deficiency
- sulpho-iduronate sulphatase deficiency
- sulphoiduronidate sulphatase deficiency
- Mucopolysaccharidosis type 2A
- Hunter syndrome type A
- Iduronate 2-sulfatase deficiency type A
- Mucopolysaccharidosis type 2, severe form
- Mucopolysaccharidosis type 2B
- Hunter syndrome type B
- Iduronate 2-sulfatase deficiency type B
- Mucopolysaccharidosis type 2, attenuated form
- Mucopolysaccharidosis type 2, mild form
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