n SA Journal of Radiology - Roentgenographic diagnosis of mucopolysaccharidosis with particular reference to Morquio syndrome : review
|Article Title||Roentgenographic diagnosis of mucopolysaccharidosis with particular reference to Morquio syndrome : review|
|Journal||SA Journal of Radiology|
|Affiliations||1 Era's Lucknow Medical College, India, 2 Era's Lucknow Medical College, India, 3 Era's Lucknow Medical College, India and 4 Era's Lucknow Medical College, India|
|Publication Date||Feb 2012|
|Pages||32 - 34|
Mucopolysaccharidosis (MPS) comprises a group of conditions associated with an abnormality in glycoprotein or mucopolysaccharides metabolism. Types of MPS identified are MPS I-H (Hurler's syndrome, gargoylism), MPS II (Hunter's syndrome), MPS III (Sanfilippo's syndrome), MPS IV (Morquio-Brailsford syndrome), MPS I-S (Scheie's syndrome) and MPS VI (Maroteaux-Lamy syndrome). The Hunter type is inherited as an X-linked recessive; the others are autosomal recessive. Patients with MPS IV can usually be clinically distinguished from patients with other forms of MPS; their intelligence is unimpaired, in contrast with other forms of MPS. Husler coined the term dysostosis multiplex to describe the skeletal findings.
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