Proteus syndrome and immunodeficiency
Identifieur interne : 004076 ( Pmc/Checkpoint ); précédent : 004075; suivant : 004077Proteus syndrome and immunodeficiency
Auteurs : D. Hodge ; S. Misbah ; R. Mueller ; E. Glass ; P. ChetcutiSource :
- Archives of Disease in Childhood [ 0003-9888 ] ; 2000.
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:1718237Le document en format XML
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<author><name sortKey="Hodge, D" sort="Hodge, D" uniqKey="Hodge D" first="D" last="Hodge">D. Hodge</name>
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<author><name sortKey="Misbah, S" sort="Misbah, S" uniqKey="Misbah S" first="S" last="Misbah">S. Misbah</name>
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<author><name sortKey="Mueller, R" sort="Mueller, R" uniqKey="Mueller R" first="R" last="Mueller">R. Mueller</name>
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<author><name sortKey="Glass, E" sort="Glass, E" uniqKey="Glass E" first="E" last="Glass">E. Glass</name>
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<author><name sortKey="Chetcuti, P" sort="Chetcuti, P" uniqKey="Chetcuti P" first="P" last="Chetcuti">P. Chetcuti</name>
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<front><div type="abstract" xml:lang="en"><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.
</p>
</div>
</front>
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<pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Arch Dis Child</journal-id>
<journal-title>Archives of Disease in Childhood</journal-title>
<issn pub-type="ppub">0003-9888</issn>
<issn pub-type="epub">1468-2044</issn>
<publisher><publisher-name>BMJ Group</publisher-name>
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<article-meta><article-id pub-id-type="pmid">10685928</article-id>
<article-id pub-id-type="pmc">1718237</article-id>
<article-id pub-id-type="doi">10.1136/adc.82.3.234</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>General and Specialist Paediatrics</subject>
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<title-group><article-title>Proteus syndrome and immunodeficiency</article-title>
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<contrib-group><contrib contrib-type="author"><name><surname>Hodge</surname>
<given-names>D</given-names>
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<contrib contrib-type="author"><name><surname>Misbah</surname>
<given-names>S</given-names>
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<contrib contrib-type="author"><name><surname>Mueller</surname>
<given-names>R</given-names>
</name>
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<contrib contrib-type="author"><name><surname>Glass</surname>
<given-names>E</given-names>
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<contrib contrib-type="author"><name><surname>Chetcuti</surname>
<given-names>P</given-names>
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<aff>Department of Paediatrics and Child Health, University of Leeds, Leeds, UK.<email>D.Hodge@leeds.ac.uk</email>
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<pub-date pub-type="ppub"><month>3</month>
<year>2000</year>
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<volume>82</volume>
<issue>3</issue>
<fpage>234</fpage>
<lpage>235</lpage>
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<abstract><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.
</p>
</abstract>
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</front>
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<name sortKey="Hodge, D" sort="Hodge, D" uniqKey="Hodge D" first="D" last="Hodge">D. Hodge</name>
<name sortKey="Misbah, S" sort="Misbah, S" uniqKey="Misbah S" first="S" last="Misbah">S. Misbah</name>
<name sortKey="Mueller, R" sort="Mueller, R" uniqKey="Mueller R" first="R" last="Mueller">R. Mueller</name>
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