ANESTHESIA MANAGEMENT IN A PATIENT WITH MAROTEAUX-LAMY SYNDROME

Tutuncu A. C., Kaya G., Altintas F., Kendigelen P.

NOBEL MEDICUS, vol.8, no.1, pp.114-116, 2012 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 8 Issue: 1
  • Publication Date: 2012
  • Journal Name: NOBEL MEDICUS
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.114-116
  • Istanbul University Affiliated: Yes

Abstract

Mucopolysaccharidoses are uncommon hereditary disorders leading to organ dysfunction and anatomic abnormalities. They are accompanied by increased risk of perioperative and postoperative complications. Mucopolysaccharidoses (MPS) are a group of syndromes in which there is an inherited lack of a lysosomal enzyme. Widespread, progressive mucopolysaccharide tissue deposition may cause both organ dysfunction and anatomical abnormalities. The Maroteaux-Lamy syndrome (MPS VI) is a very rare mucopolysaccharidosis with variable severity of somatic and possible cervical spine involvement.