Proteus syndrome and immunodeficiency. Issue 3 (1st March 2000)

• Record Type: Journal Article
• Authors: Hodge, D; Misbah, S A; Mueller, R F; Glass, E J; Chetcuti, P A J
• Is Part Of: Archives of disease in childhood. Volume 82:Issue 3(2000)
• Page Start: 234
• Page End: 235
• Publication Date: 2000-03-01
• Subjects: Proteus syndrome -- hypogammaglobulinaemia -- lymphopenia; Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/adc.82.3.234
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : A 10 year old boy with Proteus syndrome presented with a pericardial effusion of unknown aetiology. Immunological investigation revealed low serum IgG and IgA, accompanied by low levels of specific antibodies to pneumococcal and haemophilus type B polysaccharides. Circulating lymphocyte surface marker profile revealed T and B cell lymphopenia. This is the first report of hypogammaglobulinaemia occurring in the Proteus syndrome.