5C56.30 Mucopolysaccharidosis type 1

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01


Mucopolysaccharidosis type 1 (MPS 1) is a rare lysosomal storage disease belonging to the group of mucopolysaccharidoses. There are three variants, differing widely in their severity, with Hurler syndrome (57% of cases) being the most severe, Scheie syndrome (20% of cases) the mildest and Hurler-Scheie syndrome (23% of cases) giving an intermediate phenotype.

synonyms

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