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[Clinical case of the month. A recurrent chylothorax in Bourneville tuberous sclerosis].

Authors :
Bustin F
Gustin M
Robin M
Cambier P
Warling X
Chantraine JM
Source :
Revue medicale de Liege [Rev Med Liege] 2002 Aug; Vol. 57 (8), pp. 493-6.
Publication Year :
2002

Abstract

Bourneville's disease, first described in 1862, is a phacomatosis that is either autosomal dominant or sporadic. Its typical clinical signs include mental retardation, epilepsy and cutaneous adenomas. The pulmonary form is rare, less than 1%, and is secondary to occlusion of the bronchus, vascular and lymphatics by immature smooth muscle cells. Chylothorax may appear in more than 50% of all cases. No guidelines currently exist for treatment of recurrent chylothorax. However, several possibilities are described in the literature.

Details

Language :
French
ISSN :
0370-629X
Volume :
57
Issue :
8
Database :
MEDLINE
Journal :
Revue medicale de Liege
Publication Type :
Academic Journal
Accession number :
12405020