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Successful management of late-onset primary lymphatic hypoplasia.

Identifieur interne : 00FB40 ( Main/Merge ); précédent : 00FB39; suivant : 00FB41

Successful management of late-onset primary lymphatic hypoplasia.

Auteurs : A K Rustgi ; F S Rotolo ; W P Peete ; R T Vollmer ; W C Meyers

Source :

RBID : pubmed:4002118

Descripteurs français

English descriptors

Abstract

Primary lymphedema of the extremities, abdomen, or chest is an unusual and difficult clinical problem with few guidelines for management. A case is reported of lymphedema acquired at the age of 61 years, with associated massive chylous ascites and chylothorax. No underlying condition was discovered and the patient was found to have hypoplastic lymphatics by lymphangiography. Initial management consisted of extremity elevation, diuresis, and repeated paracenteses and thoracenteses. A peritoneojugular shunt provided temporary relief. Surgical pleurodesis combined with intensive diuresis has given prolonged relief of his symptoms allowing him to return to a functional life. Diagnostic and therapeutic guidelines for the management of this unusual condition are suggested.

PubMed: 4002118

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pubmed:4002118

Le document en format XML

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<term>Chylothorax (etiology)</term>
<term>Chylothorax (therapy)</term>
<term>Chylous Ascites (etiology)</term>
<term>Chylous Ascites (therapy)</term>
<term>Humans</term>
<term>Lymphatic System (abnormalities)</term>
<term>Lymphedema (diagnosis)</term>
<term>Lymphedema (etiology)</term>
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<term>Male</term>
<term>Middle Aged</term>
<term>Time Factors</term>
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<term>Adulte d'âge moyen</term>
<term>Ascite chyleuse ()</term>
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<term>Chylothorax ()</term>
<term>Chylothorax (étiologie)</term>
<term>Facteurs temps</term>
<term>Humains</term>
<term>Lymphoedème ()</term>
<term>Lymphoedème (anatomopathologie)</term>
<term>Lymphoedème (diagnostic)</term>
<term>Lymphoedème (étiologie)</term>
<term>Mâle</term>
<term>Système lymphatique (malformations)</term>
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<term>Lymphatic System</term>
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<term>Lymphoedème</term>
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<keywords scheme="MESH" qualifier="diagnosis" xml:lang="en">
<term>Lymphedema</term>
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<term>Lymphoedème</term>
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<term>Chylothorax</term>
<term>Chylous Ascites</term>
<term>Lymphedema</term>
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<term>Système lymphatique</term>
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<term>Lymphedema</term>
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<term>Ascite chyleuse</term>
<term>Chylothorax</term>
<term>Facteurs temps</term>
<term>Humains</term>
<term>Lymphoedème</term>
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<front>
<div type="abstract" xml:lang="en">Primary lymphedema of the extremities, abdomen, or chest is an unusual and difficult clinical problem with few guidelines for management. A case is reported of lymphedema acquired at the age of 61 years, with associated massive chylous ascites and chylothorax. No underlying condition was discovered and the patient was found to have hypoplastic lymphatics by lymphangiography. Initial management consisted of extremity elevation, diuresis, and repeated paracenteses and thoracenteses. A peritoneojugular shunt provided temporary relief. Surgical pleurodesis combined with intensive diuresis has given prolonged relief of his symptoms allowing him to return to a functional life. Diagnostic and therapeutic guidelines for the management of this unusual condition are suggested.</div>
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