title: Proteus syndrome and immunodeficiency.
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.
authors.affiliation: Department of Paediatrics and Child Health, University of Leeds, Leeds, UK. D.Hodge@leeds.ac.uk
authors.forename: D
authors.initials: D
authors.lastname: Hodge
authors.forename: S A
authors.initials: SA
authors.lastname: Misbah
authors.forename: R F
authors.initials: RF
authors.lastname: Mueller
authors.forename: E J
authors.initials: EJ
authors.lastname: Glass
authors.forename: P A
authors.initials: PA
authors.lastname: Chetcuti
mesh_terms: D000361:Agammaglobulinemia / Q000097:blood / Q000150:complications* / Q000276:immunology; D002648:Child; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008231:Lymphopenia / Q000097:blood / Q000150:complications* / Q000276:immunology; D008297:Male; D010490:Pericardial Effusion / Q000209:etiology* / Q000276:immunology; D016715:Proteus Syndrome / Q000097:blood / Q000150:complications* / Q000276:immunology
chemical_list: D007074:Immunoglobulin G