[A case of Hennekam syndrome presenting with massive pericardial effusion].
Identifieur interne : 003097 ( PubMed/Corpus ); précédent : 003096; suivant : 003098[A case of Hennekam syndrome presenting with massive pericardial effusion].
Auteurs : Kemal Ni Li ; Naci Oner ; Hülya Kayserili ; Türkan Ertu RulSource :
- Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir [ 1016-5169 ] ; 2008.
English descriptors
- KwdEn :
- Abnormalities, Multiple, Child, Preschool, Humans, Lymphangiectasis, Intestinal (complications), Lymphangiectasis, Intestinal (congenital), Lymphatic System (abnormalities), Lymphedema (complications), Lymphedema (congenital), Male, Pericardial Effusion (diagnosis), Pericardial Effusion (etiology), Syndrome.
- MESH :
- abnormalities : Lymphatic System.
- complications : Lymphangiectasis, Intestinal, Lymphedema.
- congenital : Lymphangiectasis, Intestinal, Lymphedema.
- diagnosis : Pericardial Effusion.
- etiology : Pericardial Effusion.
- Abnormalities, Multiple, Child, Preschool, Humans, Male, Syndrome.
Abstract
Hennekam syndrome is an autosomal recessive disease characterized by intestinal lymphangiectasia accompanied by severe lymphedema of the limbs, genitalia, and face, and learning difficulties. A 38-month-old boy was admitted with breathing difficulty. He had facial abnormalities and preputial hyperplasia consistent with Hennekam syndrome. Lymphangiography showed lymphedema in the left eye and right foot. Teleradiography showed cardiomegaly and echocardiography showed massive pericardial effusion. He first underwent pericardiocentesis for the removal of pericardial effusion, but pericardial tube drainage was required upon recurrence of effusion. On the fifth day, the drain was removed because of significant decrease in the drainage.
PubMed: 18984984
Links to Exploration step
pubmed:18984984Le document en format XML
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<author><name sortKey="Ni Li, Kemal" sort="Ni Li, Kemal" uniqKey="Ni Li K" first="Kemal" last="Ni Li">Kemal Ni Li</name>
<affiliation><nlm:affiliation>Istanbul Universitesi Istanbul Tip Fakültesi, Cocuk Sağliği ve Hastaliklari Anabilim Dali, Istanbul, Turkey. kemalnisli@yahoo.com</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Oner, Naci" sort="Oner, Naci" uniqKey="Oner N" first="Naci" last="Oner">Naci Oner</name>
</author>
<author><name sortKey="Kayserili, Hulya" sort="Kayserili, Hulya" uniqKey="Kayserili H" first="Hülya" last="Kayserili">Hülya Kayserili</name>
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<author><name sortKey="Ertu Rul, Turkan" sort="Ertu Rul, Turkan" uniqKey="Ertu Rul T" first="Türkan" last="Ertu Rul">Türkan Ertu Rul</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en">[A case of Hennekam syndrome presenting with massive pericardial effusion].</title>
<author><name sortKey="Ni Li, Kemal" sort="Ni Li, Kemal" uniqKey="Ni Li K" first="Kemal" last="Ni Li">Kemal Ni Li</name>
<affiliation><nlm:affiliation>Istanbul Universitesi Istanbul Tip Fakültesi, Cocuk Sağliği ve Hastaliklari Anabilim Dali, Istanbul, Turkey. kemalnisli@yahoo.com</nlm:affiliation>
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<author><name sortKey="Oner, Naci" sort="Oner, Naci" uniqKey="Oner N" first="Naci" last="Oner">Naci Oner</name>
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<author><name sortKey="Kayserili, Hulya" sort="Kayserili, Hulya" uniqKey="Kayserili H" first="Hülya" last="Kayserili">Hülya Kayserili</name>
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<author><name sortKey="Ertu Rul, Turkan" sort="Ertu Rul, Turkan" uniqKey="Ertu Rul T" first="Türkan" last="Ertu Rul">Türkan Ertu Rul</name>
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<series><title level="j">Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir</title>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Abnormalities, Multiple</term>
<term>Child, Preschool</term>
<term>Humans</term>
<term>Lymphangiectasis, Intestinal (complications)</term>
<term>Lymphangiectasis, Intestinal (congenital)</term>
<term>Lymphatic System (abnormalities)</term>
<term>Lymphedema (complications)</term>
<term>Lymphedema (congenital)</term>
<term>Male</term>
<term>Pericardial Effusion (diagnosis)</term>
<term>Pericardial Effusion (etiology)</term>
<term>Syndrome</term>
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<keywords scheme="MESH" qualifier="abnormalities" xml:lang="en"><term>Lymphatic System</term>
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<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lymphangiectasis, Intestinal</term>
<term>Lymphedema</term>
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<keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Lymphangiectasis, Intestinal</term>
<term>Lymphedema</term>
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<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Pericardial Effusion</term>
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<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Pericardial Effusion</term>
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<keywords scheme="MESH" xml:lang="en"><term>Abnormalities, Multiple</term>
<term>Child, Preschool</term>
<term>Humans</term>
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<front><div type="abstract" xml:lang="en">Hennekam syndrome is an autosomal recessive disease characterized by intestinal lymphangiectasia accompanied by severe lymphedema of the limbs, genitalia, and face, and learning difficulties. A 38-month-old boy was admitted with breathing difficulty. He had facial abnormalities and preputial hyperplasia consistent with Hennekam syndrome. Lymphangiography showed lymphedema in the left eye and right foot. Teleradiography showed cardiomegaly and echocardiography showed massive pericardial effusion. He first underwent pericardiocentesis for the removal of pericardial effusion, but pericardial tube drainage was required upon recurrence of effusion. On the fifth day, the drain was removed because of significant decrease in the drainage.</div>
</front>
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<Month>11</Month>
<Day>05</Day>
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<DateCompleted><Year>2009</Year>
<Month>02</Month>
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<DateRevised><Year>2008</Year>
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<Title>Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir</Title>
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<ArticleTitle>[A case of Hennekam syndrome presenting with massive pericardial effusion].</ArticleTitle>
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<Abstract><AbstractText>Hennekam syndrome is an autosomal recessive disease characterized by intestinal lymphangiectasia accompanied by severe lymphedema of the limbs, genitalia, and face, and learning difficulties. A 38-month-old boy was admitted with breathing difficulty. He had facial abnormalities and preputial hyperplasia consistent with Hennekam syndrome. Lymphangiography showed lymphedema in the left eye and right foot. Teleradiography showed cardiomegaly and echocardiography showed massive pericardial effusion. He first underwent pericardiocentesis for the removal of pericardial effusion, but pericardial tube drainage was required upon recurrence of effusion. On the fifth day, the drain was removed because of significant decrease in the drainage.</AbstractText>
</Abstract>
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<ForeName>Kemal</ForeName>
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<AffiliationInfo><Affiliation>Istanbul Universitesi Istanbul Tip Fakültesi, Cocuk Sağliği ve Hastaliklari Anabilim Dali, Istanbul, Turkey. kemalnisli@yahoo.com</Affiliation>
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<VernacularTitle>Hennekam sendromlu bir olguda başvuru nedeni olarak perikard efüzyonu.</VernacularTitle>
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<MeshHeadingList><MeshHeading><DescriptorName UI="D000015" MajorTopicYN="N">Abnormalities, Multiple</DescriptorName>
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<MeshHeading><DescriptorName UI="D008201" MajorTopicYN="N">Lymphangiectasis, Intestinal</DescriptorName>
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<MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName>
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<MeshHeading><DescriptorName UI="D010490" MajorTopicYN="N">Pericardial Effusion</DescriptorName>
<QualifierName UI="Q000175" MajorTopicYN="Y">diagnosis</QualifierName>
<QualifierName UI="Q000209" MajorTopicYN="Y">etiology</QualifierName>
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<MeshHeading><DescriptorName UI="D013577" MajorTopicYN="N">Syndrome</DescriptorName>
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