Gorham's syndrome: a case report and review of the literature.
Am J Med
; 83(6): 1151-6, 1987 Dec.
Article
em En
| MEDLINE
| ID: mdl-3332570
ABSTRACT
Gorham's syndrome is a rare disorder involving a proliferation of vascular channels associated with extensive loss of bony matrix. A case report is presented with a review of the 97 previously reported cases. The age of patients at presentation has ranged from less than one to 75 years (mean 27 years). Sixty-four percent have been men. Fifty-seven percent have had a history of prior trauma. Laboratory values for systemic measures have usually been normal. The disease usually arrests spontaneously, but this is unpredictable. Sixteen patients (16 percent) have died of the disorder, with 10 deaths due to chest wall involvement, three to spinal cord transection, two to sepsis, and one to asphyxia and aspiration. Although the mechanism of bone loss is unknown, osteoclasts were focally increased in the case described herein. Further information and investigation are needed to better understand this unusual disorder.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Osteólise
/
Ombro
/
Osteólise Essencial
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Am J Med
Ano de publicação:
1987
Tipo de documento:
Article