Mucopolysaccharidosis - in the ICD-10-CM Index

Annotation Back-References in the 2026 ICD-10-CM Index to Diseases and Injuries

Browse the ICD-10-CM codes with references applicable to the clinical term "mucopolysaccharidosis"

  • Mucopolysaccharidosis - E76.3 Mucopolysaccharidosis, unspecified

    • beta-gluduronidase deficiency - E76.29 Other mucopolysaccharidoses

    • cardiopathy - E76.3 Mucopolysaccharidosis, unspecified

    • Hunter's syndrome - E76.1 Mucopolysaccharidosis, type II

    • Hurler's syndrome - E76.01 Hurler's syndrome

    • Hurler-Scheie syndrome - E76.02 Hurler-Scheie syndrome

    • Maroteaux-Lamy syndrome - E76.29 Other mucopolysaccharidoses

    • Morquio syndrome - E76.219 Morquio mucopolysaccharidoses, unspecified

      • A - E76.210 Morquio A mucopolysaccharidoses

      • B - E76.211 Morquio B mucopolysaccharidoses

      • classic - E76.210 Morquio A mucopolysaccharidoses

    • Sanfilippo syndrome - E76.22 Sanfilippo mucopolysaccharidoses

    • Scheie's syndrome - E76.03 Scheie's syndrome

    • specified NEC - E76.29 Other mucopolysaccharidoses

    • type

      • I

        • Hurler's syndrome - E76.01 Hurler's syndrome

        • Hurler-Scheie syndrome - E76.02 Hurler-Scheie syndrome

        • Scheie's syndrome - E76.03 Scheie's syndrome

      • II - E76.1 Mucopolysaccharidosis, type II

      • III - E76.22 Sanfilippo mucopolysaccharidoses

      • IV - E76.219 Morquio mucopolysaccharidoses, unspecified

      • IVA - E76.210 Morquio A mucopolysaccharidoses

      • IVB - E76.211 Morquio B mucopolysaccharidoses

      • VI - E76.29 Other mucopolysaccharidoses

      • VII - E76.29 Other mucopolysaccharidoses

Applicable Clinical Terms Definitions

Mucopolysaccharidosis I: A group of autosomal recessive lysosomal storage disorders caused by mutations in the gene encoding the enzyme, alpha-L-iduronidase (IDUA), required for the degradation of heparan and dermatan sulfates. This leads to abnormal accumulation of these glycosaminoglycans in various tissues causing a wide range of clinical presentations including cognitive and musculoskeletal disorders.