Mucopolysaccharidosis type 1ICD-11 Sub-sub category
ICD-11 code : 5C56.30;
Preferred Label : Mucopolysaccharidosis type 1;
ICD-11 definition : 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.;
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.