Winchester syndrome. A case report and literature review

The mucopolysaccharidoses are a group of inherited lysosomal storage diseases that are caused by a deficiency of specific enzymes. The acid mucopolysaccharides are stored in tissue and excreted in large quantities in the urine. The storage of this material leads to effects on a wide variety of tissu...

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Veröffentlicht in:Oral surgery, oral medicine, oral pathology oral medicine, oral pathology, 1992-11, Vol.74 (5), p.671-677
Hauptverfasser: Prapanpoch, S, Jorgenson, R J, Langlais, R P, Nummikoski, P V
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Sprache:eng
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Zusammenfassung:The mucopolysaccharidoses are a group of inherited lysosomal storage diseases that are caused by a deficiency of specific enzymes. The acid mucopolysaccharides are stored in tissue and excreted in large quantities in the urine. The storage of this material leads to effects on a wide variety of tissues and to remarkable changes in morphologic features. Winchester syndrome is a rare disorder in the group of mucopolysaccharidoses. This article is a report of a case with classic clinical, radiologic, and biochemical characteristics of the Winchester syndrome.
ISSN:0030-4220
DOI:10.1016/0030-4220(92)90363-U