ANESTHESIA MANAGEMENT IN A PATIENT WITH MAROTEAUX-LAMY SYNDROME
NOBEL MEDICUS, cilt.8, sa.1, ss.114-116, 2012 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 8 Sayı: 1
- Basım Tarihi: 2012
- Dergi Adı: NOBEL MEDICUS
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.114-116
- İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet
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.