European Journal of Pediatrics

, Volume 129, Issue 1, pp 11–16 | Cite as

Mucopolysaccharidosis II (Hunter disease) with corneal opacities

Report on two patients at the extremes of a wide clinical spectrum
  • J. Spranger
  • M. Cantz
  • J. Gehler
  • I. Liebaers
  • W. Theiss


Clinically visible corneal opacities were observed in a patient with an extremely severe form of mucopolysaccharidosis II. In a second patient with an unusually mild form of mucopolysaccharidosis II, discrete corneal opacities were detected by slit-lamp examination. Thus clear corneae can no longer be regarded as a hallmark of mucopolysaccharidosis II.

Key words

Mucopolysaccharidoses Hunter disease Hurler disease Corneal opacities Sulfoiduronate sulfatase deficiency 


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Copyright information

© Springer-Verlag 1978

Authors and Affiliations

  • J. Spranger
    • 1
  • M. Cantz
    • 1
  • J. Gehler
    • 1
  • I. Liebaers
    • 2
  • W. Theiss
    • 3
  1. 1.Children's HospitalUniversity of MainzMainzFederal Republic of Germany
  2. 2.Section on Human Biochemical GeneticsNational Institute of Arthritis, Metabolism and Digestive DiseasesBethesdaUSA
  3. 3.Städtische Krankenanstalten BenrathDüsseldorfFederal Republic of Germany

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