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Anaesthetic management of patients with mucopolysaccharidosis IV presenting for major orthopaedic surgery
Author(s) -
Bartz H.J.,
Wiesner L.,
Wappler F.
Publication year - 1999
Publication title -
acta anaesthesiologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.738
H-Index - 107
eISSN - 1399-6576
pISSN - 0001-5172
DOI - 10.1034/j.1399-6576.1999.430614.x
Subject(s) - medicine , mucopolysaccharidosis , orthopedic surgery , connective tissue , hunter syndrome , surgery , keratan sulfate , glycosaminoglycan , pathology , anatomy , chondroitin sulfate
Mucopolysaccharidosis IV, also known as Morquio‐Brailsford syndrome, is an inherited autosomal recessive disorder of mucopolysaccharide metabolism leading to accumulation of keratan sulphate in the connective tissue of multiple organ systems. Based on a case report, the anaesthetic implications for the treatment of patients with MPS IV presenting for major orthopaedic surgery are discussed.