ANESTHESIA MANAGEMENT IN A PATIENT WITH MAROTEAUX-LAMY SYNDROME


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

NOBEL MEDICUS, cilt.8, ss.114-116, 2012 (SCI İndekslerine Giren Dergi) identifier identifier

  • Cilt numarası: 8 Konu: 1
  • Basım Tarihi: 2012
  • Dergi Adı: NOBEL MEDICUS
  • Sayfa Sayıları: ss.114-116

Özet

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.