RESOLUTION OF A PROXIMAL HUMERAL DEFECT IN TYPE-1 GAUCHER DISEASE BY ENZYME REPLACEMENT THERAPY

Authors
PASTORES GM HERMANN G NORTON K DESNICK RJ
Citation
Gm. Pastores et al., RESOLUTION OF A PROXIMAL HUMERAL DEFECT IN TYPE-1 GAUCHER DISEASE BY ENZYME REPLACEMENT THERAPY, Pediatric radiology, 25(6), 1995, pp. 486-487
Citations number
15
Categorie Soggetti
Radiology,Nuclear Medicine & Medical Imaging",Pediatrics
Journal title
Pediatric radiology → ACNP
ISSN journal
03010449
Volume
25
Issue
6
Year of publication
1995
Pages
486 - 487
Database
ISI
SICI code
0301-0449(1995)25:6<486:ROAPHD>2.0.ZU;2-I
Abstract
Although rarely they are a normal variant in children, significant def ects in the medial aspect of the proximal humeral metaphysis occur in patients with Gaucher disease due to cortical infiltration and erosion of the periosteum by Gaucher cells. Such changes may lead to patholog ical fractures in Gaucher patients. These crescentic erosions resolved in a 19-year-old patient with type-1 Gaucher disease following enzyme replacement therapy.