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Proteus syndrome and immunodeficiency

Identifieur interne : 004076 ( Pmc/Checkpoint ); précédent : 004075; suivant : 004077

Proteus syndrome and immunodeficiency

Auteurs : D. Hodge ; S. Misbah ; R. Mueller ; E. Glass ; P. Chetcuti

Source :

RBID : PMC:1718237

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.




Url:
DOI: 10.1136/adc.82.3.234
PubMed: 10685928
PubMed Central: 1718237


Affiliations:


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PMC:1718237

Le document en format XML

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<name sortKey="Misbah, S" sort="Misbah, S" uniqKey="Misbah S" first="S" last="Misbah">S. Misbah</name>
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<name sortKey="Mueller, R" sort="Mueller, R" uniqKey="Mueller R" first="R" last="Mueller">R. Mueller</name>
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<name sortKey="Glass, E" sort="Glass, E" uniqKey="Glass E" first="E" last="Glass">E. Glass</name>
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<p>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.

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<aff>Department of Paediatrics and Child Health, University of Leeds, Leeds, UK.
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<p>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.

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