Proteus syndrome and immunodeficiency

Authors
Hedge, D Misbah, SA Mueller, RF Glass, EJ Chetcuti, PAJ
Citation
D. Hedge et al., Proteus syndrome and immunodeficiency, ARCH DIS CH, 82(3), 2000, pp. 234-235
Citations number
5
Categorie Soggetti
Pediatrics,"Medical Research General Topics
Journal title
ARCHIVES OF DISEASE IN CHILDHOOD
ISSN journal
00039888 → ACNP
Volume
82
Issue
3
Year of publication
2000
Pages
234 - 235
Database
ISI
SICI code
0003-9888(200003)82:3<234:PSAI>2.0.ZU;2-K
Abstract
A 10 year old boy with Proteus syndrome presented with a pericardial effusi on of unknown aetiology. Immunological investigation revealed low serum Ige and IgA, accompanied by low levels of specific antibodies to pneumococcal and haemophilus type B polysaccharides. Circulating lymphocyte surface mark er profile revealed T and B cell lymphopenia. This is the first report of h ypogammaglobulinaemia occurring in the Proteus syndrome.