Conditions / Genetic

mucopolysaccharidosis II

info · Genetic · ICD-10: E76.1

A mucopolysaccharidosis characterized by a deficiency of the lysosomal enzyme iduronate sulfatase.

Signs and symptoms

  • Hepatomegaly
  • Thick lower lip vermilion
  • Splenomegaly
  • Urinary glycosaminoglycan excretion
  • Decreased iduronate sulfatase level
  • Coarse facial features
  • Macrocephaly
  • Hepatosplenomegaly
  • Dysostosis multiplex
  • Flexion contracture

Medications that may treat it

idursulfase

Also known as: Hunter syndrome; Hunter's syndrome; MPS II - Hunter syndrome; Mucopolysaccharidosis, MPS-II; deficiency of iduronate-2-sulphatase