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Aseptic necrosis of the capitate secondary to gaucher's disease: a case report

✍ Scribed by WOUNLUND, J; LOHMANN, M

Book ID
122244494
Publisher
Elsevier Science
Year
1989
Tongue
English
Weight
294 KB
Volume
14
Category
Article
ISSN
0363-5023

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Adult-onset, or type 1, Gaucher's disease is an autosomal disorder, characterized by deficiency of a lysosomal enzyme, p-glucocerebrosidase. This results in the accumulation of a glycolipid, glucosylceramide, in distinctive "foam" cells which are seen in the bone marrow, reticuloendothelial tissue,