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[A case of Hennekam syndrome presenting with massive pericardial effusion].

Identifieur interne : 002D89 ( PubMed/Checkpoint ); précédent : 002D88; suivant : 002D90

[A case of Hennekam syndrome presenting with massive pericardial effusion].

Auteurs : Kemal Ni Li [Turquie] ; Naci Oner ; Hülya Kayserili ; Türkan Ertu Rul

Source :

RBID : pubmed:18984984

Descripteurs français

English descriptors

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


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pubmed:18984984

Le document en format XML

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<title xml:lang="en">[A case of Hennekam syndrome presenting with massive pericardial effusion].</title>
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<nlm:affiliation>Istanbul Universitesi Istanbul Tip Fakültesi, Cocuk Sağliği ve Hastaliklari Anabilim Dali, Istanbul, Turkey. kemalnisli@yahoo.com</nlm:affiliation>
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<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>
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