<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0873-2159</journal-id>
<journal-title><![CDATA[Revista Portuguesa de Pneumologia]]></journal-title>
<abbrev-journal-title><![CDATA[Rev Port Pneumol]]></abbrev-journal-title>
<issn>0873-2159</issn>
<publisher>
<publisher-name><![CDATA[Sociedade Portuguesa de Pneumologia]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0873-21592006000300008</article-id>
<title-group>
<article-title xml:lang="pt"><![CDATA[A propósito de um caso de imunodeficiência comum variável: Revisão das hipogamaglobulinemias]]></article-title>
<article-title xml:lang="en"><![CDATA[About a case of common variable immunodeficiency: Revision of hypogammaglobulinemias]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Lopes]]></surname>
<given-names><![CDATA[Alexandra]]></given-names>
</name>
<xref ref-type="aff" rid="A01"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Barata]]></surname>
<given-names><![CDATA[Fernando]]></given-names>
</name>
<xref ref-type="aff" rid="A02"/>
</contrib>
</contrib-group>
<aff id="A01">
<institution><![CDATA[,Centro Hospitalar de Coimbra Serviço de Medicina ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<aff id="A02">
<institution><![CDATA[,Centro Hospitalar de Coimbra Serviço de Pneumologia ]]></institution>
<addr-line><![CDATA[ ]]></addr-line>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>05</month>
<year>2006</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>05</month>
<year>2006</year>
</pub-date>
<volume>12</volume>
<numero>3</numero>
<fpage>293</fpage>
<lpage>301</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_arttext&amp;pid=S0873-21592006000300008&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_abstract&amp;pid=S0873-21592006000300008&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.pt/scielo.php?script=sci_pdf&amp;pid=S0873-21592006000300008&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="pt"><p><![CDATA[A imunodeficiência comum variável é uma imunodeficiência primária caracterizada por distúrbios imunológicos heterogéneos, de etiologia desconhecida. As suas manifestações clínicas incluem infecções recorrentes, doenças auto-imunes, hiperplasia linfóide, doenças granulomatosas e neoplasias; pode aparecer em doentes com deficiência de IgA. Os autores apresentam o caso clínico de doente com imunodeficiência comum variável e história de infecções respiratórias de repetição desde os 9 meses, associadas ao aparecimento de bronquiectasias.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Common variable immunodeficiency is a primary immune deficiency characterized by heterogeneous immunologic disorders of unknown etiology. Its clinical manifestations include recurrent infections, autoimmune diseases, lymphoid hyperplasia, granulomatous diseases and malignancy. It can appear in patients with immunoglobulin A deficiency. The authors report the clinical case of a patient with common variable immunodeficiency and history of respiratory infections from the age of 9 months old, associated with the appearing of bronchiectasis.]]></p></abstract>
<kwd-group>
<kwd lng="pt"><![CDATA[Imunodeficiência comum variável]]></kwd>
<kwd lng="en"><![CDATA[Common variable immunodeficiency]]></kwd>
</kwd-group>
</article-meta>
</front><body><![CDATA[ <p><b>A propósito de um caso de imunodeficiência comum variável – Revisão das hipogamaglobulinemias</b></p>      <p>&nbsp;</p>      <p><i>About a case of common variable immunodeficiency – Revision of hypogammaglobulinemias</i></b></p>      <p>&nbsp;</p>      <p><b>Alexandra Lopes<a href="#1">*</a><a name="top1"></a></b></p>      <p><b>Fernando Barata<a href="#2">**</a><a name="top2"></a></b></p>      <p>&nbsp;</p>      <p align="center"><b>Resumo</b></p>      <p>A imunodeficiência comum variável é uma imunodeficiência primária caracterizada por  distúrbios imunológicos heterogéneos, de etiologia desconhecida. As suas manifestações clínicas incluem infecções recorrentes, doenças auto-imunes, hiperplasia linfóide, doenças granulomatosas e neoplasias; pode aparecer em doentes com deficiência de IgA.  Os autores apresentam o caso clínico de doente com imunodeficiência comum variável e história de infecções respiratórias de repetição desde os 9 meses, associadas ao aparecimento de bronquiectasias. </p>      <p><b>Palavras-chave: </b>Imunodeficiência comum variável</p>      ]]></body>
<body><![CDATA[<p>&nbsp;</p>      <p align="center"><b>Abstract</b></p>      <p align="justify">Common variable immunodeficiency is a primary immune deficiency    characterized by heterogeneous immunologic disorders of unknown etiology. Its    clinical manifestations include recurrent infections, autoimmune diseases, lymphoid    hyperplasia, granulomatous diseases and malignancy. It can appear in patients    with immunoglobulin A deficiency. The authors report the clinical case of a    patient with common variable immunodeficiency and history of respiratory infections    from the age of 9 months old, associated with the appearing of bronchiectasis.</p>      <p align="justify"><b>Key-words:</b> Common variable immunodeficiency.</p>      <p>&nbsp;</p>     <p>&nbsp;</p>      <p>Texto completo disponível apenas em PDF.</p>     <p>Full text only available in PDF format.</p>      <p>&nbsp;</p>     <p>&nbsp;</p>      ]]></body>
<body><![CDATA[<p align="center"><b>Bibliografia</b></p>      <!-- ref --><p>1. Sneller MC. Common variable immunodeficiency. Am J Med Sci 2001; 321(1): 42-8&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[&#160;<a href="javascript:void(0);" onclick="javascript: window.open('/scielo.php?script=sci_nlinks&ref=000024&pid=S0873-2159200600030000800001&lng=','','width=640,height=500,resizable=yes,scrollbars=1,menubar=yes,');">Links</a>&#160;]<!-- end-ref --><p>2. Vorechovsky I, Zetterquist H, Paganelli R, <i>et al</i>. Family and linkage study  of selective IgA deficiency and common variable immunodeficiency. Clin Immunol Immunopathol 1995; 77(2):185-92</p>      <p>3. Nijenhuis T, Klasen I, Weemaes CM, <i>et al</i>. Common variable immunodeficiency  (CVID) in a family: an autosomal dominant mode of inheritance. Neth J Med 2001; 59(3): 134-9</p>      <p>4. Primary immunodeficiency diseases. Report of a WHO scientific group. Clin Exp Immunol 1997; 109 Suppl: 1:1.</p>      <p>5. Hammarstrom L, Vorechovsky I, Webster D. Selective IgA deficiency  (SigAD) and common variable immunodeficiency (CVID). Clin Exp Immunol 2000; 120:225.</p>      <p>6. Hermaszewski RA, Webster ADB. Primary hypogammaglobulinemia:  A survey of clinical manifestations and complications. Q J Med 1993;  86:31.</p>      <p>7. Cunningham-Rundles C, Bodian C: Common Variable immunodeficiency:  clinical and immunological features of 248 patients. Clin Immunol  1999; 92(1): 34-48.</p>      <p>8. Spickett GP, Webster ADB, Farrant J. Cellular abnormalities in common variable    immunodeficiency. In: Rosen FS, Seligmann M, eds. Imunodeficiencies. Philadelphia:    Harwood Academie 1993: 111-26.</p>      <p>9. Conley ME, Park CL, Douglas SD. Childhood common variable immunodeficiency    with autoimmune disease. J Pediatr 1986; 108(6): 915-22.</p>      ]]></body>
<body><![CDATA[<p>10. Alvarez-Cuesta C, Molinos L, Cascante JÁ, <i>et al</i>. Cutaneous granulomas in a patient with common variable immunodeficiency.  Acta Derm Venereol 1999; 79(4): 334.</p>      <p>11. Krupnick Al, Shim H, Phelps RG, <i>et al</i>. Ctaneous granulomas  masquerading as tuberculoid leprosy in a patient with congenital combined  immunodeficiency. Mt Sinai J Med 2001; 68(4-5): 326-30.</p>      <p>12. Pierson JC, Camisa C, Lawlor KB, Elston DM. Cutaneous and visceral granulomas    in common variable immunodeficiency. Cutis 1993; 52(4): 221-2</p>      <p>13. Ziegler EM, Seung LM, Soltani K, Medenica MM. Cutaneous granulomas  with two clinical presentations in a patient with common variable  immunodeficiency. J Am Acad Dermatol 1997; 37(3 Pt 1): 499-500.</p>      <p>14. Ariatti C, Rossi D, Vivenza D, <i>et al</i>. Molecular characterization  of common variable immunodeficiency-related lymphomas. Ann Ital Med Int  2001; 16(3): 163-9.</p>      <p>15. Elenitoba_jonhnson KS, Jaffe ES. Lymphopro­liferative disorders  associated with congenital immunodeficiencies. Semin Diagn Pathol 1997;  14(1): 35-47</p>      <p>16. Johnson ML, Keeton LG, Zhu ZB, <i>et al</i>. Age-related changes  in serum imunoglobulins in patients with familial IgA deficiency and  common variable immunodeficiency (CVID). Clin Exp Immunol 1997; 108(3):  477-83</p>      <p>17. Olerup O, Smith Cl, Bjorkander J, Hammarstrom L. Shared HLA class  II-associated genetic susceptibility and resistance, related to the  HLA-DQB1 gene, in IgA deficiency and common variable immunodeficiency.  Proc Natl Acad Sci USA 199; 89(22): 10653-7</p>      <p>18. Gutierrez MG, Kirkpatrick CH. Progressive immunodeficiency in a  patient with IgA deficiency. Ann Allergy Asthma Immunol 1997; 79:297</p>      <p>19. Litzman J, Burianova M, Thon V, Lokaj J. Progression of selective  IgA deficiency to common va­riable immunodeficiency in a 16 year old boy.  Allergol Immunopathol 1996; 24:174.</p>      ]]></body>
<body><![CDATA[<p>20. Espanol T, Catala M, Hernandez M, <i>et al</i>. Development of  common variable immunodeficiency in IgA-deficient patients. Clin  Immunol Immunopathol 1996; 80:333.</p>      <p>&nbsp;</p>      <p><a href="#top1">*</a><a name="1"></a> Internato Complementar de Medicina Interna</p>      <p><a href="#top2">**</a><a name="2"></a> Assistente Hospitalar Graduado de Pneumologia</p>      <p><a href="#top1">*</a><a name="1"></a> Serviço de Medicina do Centro Hospitalar    de Coimbra. Director: Dr. Ricardo Conceição</p>      <p><a href="#top2">**</a><a name="2"></a> Serviço de Pneumologia do Centro Hospitalar    de Coimbra (Director: Dr. Rui Pato)</p>      <p>Serviço onde foi realizado o trabalho: Serviço de Pneumologia do Centro Hospitalar de  Coimbra</p>      <p><b>Correspondência:</b></p>     <p>Drª. Alexandra Lopes, Serviço de Medicina do Centro Hospitalar de Coimbra</p>     <p>Quinta dos Vales,</p>     ]]></body>
<body><![CDATA[<p>3040 Coimbra</p>      <p>&nbsp;</p>      <p>Recebido para publicação/<i>received for publication</i>: 05.03.02</p>     <p>Aceite para publicação/<i>accepted for publication</i>: 06.03.24</p>       ]]></body><back>
<ref-list>
<ref id="B1">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sneller]]></surname>
<given-names><![CDATA[MC.]]></given-names>
</name>
</person-group>
<article-title xml:lang="en"><![CDATA[Common variable immunodeficiency.]]></article-title>
<source><![CDATA[Am J Med Sci]]></source>
<year>2001</year>
<volume>321</volume>
<numero>1</numero>
<issue>1</issue>
<page-range>42-8</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
