Sinus complications in mucopolysaccharidosis I H/S (Hurler-Scheie syndrome)

Carol J. MacArthur, Richard Gliklich, Trevor J.I. McGill, Antonio Perez-Atayde

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

Hurler-Scheie syndrome is a genetic compound of two mucopolysaccharidoses, the Hurler and Scheie syndromes. The genetic error of metabolism caused by this syndrome produces intermediary systemic effects in the affected individuals. Lacking the enzyme α-l-iduronidase, glycosaminoglycans are deposited in the tissues, causing multiple systemic effects and creating many problems for the otolaryngologist. Although early bone marrow transplantation is currently being performed to prevent or reverse many of the systemic manifestations of this disorder, there remains a large population of children past the age at which bone marrow transplantation can be effective. Sinus complications have not previously been reported in the mucopolysaccharidoses and may be more frequently recognized as these patients' longevity increases. We present a patient with Hurler-Scheie syndrome who developed severe nasal polyposis requiring sinus endoscopic removal.

Original languageEnglish (US)
Pages (from-to)79-87
Number of pages9
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume26
Issue number1
DOIs
StatePublished - Feb 1993

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Keywords

  • Antral-choanal polyp
  • Endoscopic sinus surgery
  • Hurler-Scheie syndrome
  • Mucopolysaccaridoses
  • Nasal polyposis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Otorhinolaryngology

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