Mucopolysaccharidosis type 6
Mucopolysaccharidosis type 6 (MPS 6) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate.
class Information
definition_citation
Orphanet
has_age_of_onset
Childhood
has_dbxref
- ICD-10:E76.2
- ICD-11:5C56.33
- MedDRA:10056892
- MeSH:D009087
- OMIM:253200
- UMLS:C0026709
has_inheritance
Autosomal recessive
notation
present_in
- Australia AND has_birth_prevalence_average_value : 0.37 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Brazil AND has_birth_prevalence_average_value : 0.28 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Canada AND has_birth_prevalence_average_value : 0.1 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Colombia AND has_point_prevalence_average_value : 0.059 AND has_point_prevalence_range : <1 / 1 000 000
- Czech Republic AND has_birth_prevalence_average_value : 0.05 AND has_birth_prevalence_range : <1 / 1 000 000
- Denmark AND has_point_prevalence_average_value : 3.7 AND has_point_prevalence_range : 1-9 / 100 000
- Denmark AND has_birth_prevalence_average_value : 0.05 AND has_birth_prevalence_range : <1 / 1 000 000
- Estonia AND has_birth_prevalence_average_value : 0.27 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Europe AND has_birth_prevalence_average_value : 0.16 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Europe AND has_point_prevalence_average_value : 0.16 AND has_point_prevalence_range : 1-9 / 1 000 000
- Germany AND has_birth_prevalence_average_value : 0.23 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Japan AND has_birth_prevalence_average_value : 0.03 AND has_birth_prevalence_range : <1 / 1 000 000
- Korea, Republic of AND has_birth_prevalence_average_value : 0.019 AND has_birth_prevalence_range : <1 / 1 000 000
- Netherlands AND has_birth_prevalence_average_value : 0.15 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Norway AND has_birth_prevalence_average_value : 0.07 AND has_birth_prevalence_range : <1 / 1 000 000
- Norway AND has_point_prevalence_average_value : 2.2 AND has_point_prevalence_range : 1-9 / 100 000
- Poland AND has_birth_prevalence_average_value : 0.0132 AND has_birth_prevalence_range : <1 / 1 000 000
- Portugal AND has_birth_prevalence_average_value : 0.42 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Saudi Arabia AND has_birth_prevalence_average_value : 7.85 AND has_birth_prevalence_range : 1-9 / 100 000
- Sweden AND has_point_prevalence_average_value : 3.3 AND has_point_prevalence_range : 1-9 / 100 000
- Sweden AND has_birth_prevalence_average_value : 0.23 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Switzerland AND has_birth_prevalence_average_value : 0.11 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Taiwan, Province of China AND has_birth_prevalence_average_value : 0.14 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Tunisia AND has_birth_prevalence_average_value : 0.3 AND has_birth_prevalence_range : 1-9 / 1 000 000
- Turkey AND has_birth_prevalence_average_value : 2.3 AND has_birth_prevalence_range : 1-9 / 100 000
- United States AND has_birth_prevalence_average_value : 0.04 AND has_birth_prevalence_range : <1 / 1 000 000
- United States AND has_point_prevalence_average_value : 0.011 AND has_point_prevalence_range : <1 / 1 000 000