[Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)].
Identifieur interne : 000566 ( France/Analysis ); précédent : 000565; suivant : 000567[Limb lymphedema as a first manifestation of primary intestinal lymphangiectasia (Waldmann's disease)].
Auteurs : V. Boursier [France] ; S. VignesSource :
- Journal des maladies vasculaires [ 0398-0499 ] ; 2004.
Descripteurs français
- KwdFr :
- MESH :
- anatomopathologie : Duodénum, Lymphangiectasie intestinale.
- diagnostic : Lymphangiectasie intestinale.
- imagerie diagnostique : Lymphoedème.
- Adulte, Biopsie, Humains, Lymphangiectasie intestinale, Lymphoedème, Membres, Mâle, Scintigraphie.
English descriptors
- KwdEn :
- MESH :
- complications : Lymphangiectasis, Intestinal, Lymphedema.
- diagnosis : Lymphangiectasis, Intestinal.
- diagnostic imaging : Lymphedema.
- pathology : Duodenum, Lymphangiectasis, Intestinal.
- therapy : Lymphedema.
- Adult, Biopsy, Extremities, Humans, Male, Radionuclide Imaging.
Abstract
Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.
PubMed: 15229406
Affiliations:
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pubmed:15229406Le document en format XML
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Boursier</name><affiliation wicri:level="1"><nlm:affiliation>Unité de Lymphologie, Hôpital Cognacq-Jay, Site Broussais, 102, rue Didot, 75674 Paris 14. virginie.boursier@hopital-cognacq-jay.fr</nlm:affiliation><country wicri:rule="url">France</country></affiliation></author><author><name sortKey="Vignes, S" sort="Vignes, S" uniqKey="Vignes S" first="S" last="Vignes">S. Vignes</name></author></analytic><series><title level="j">Journal des maladies vasculaires</title><idno type="ISSN">0398-0499</idno><imprint><date when="2004" type="published">2004</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Biopsy</term><term>Duodenum (pathology)</term><term>Extremities</term><term>Humans</term><term>Lymphangiectasis, Intestinal (complications)</term><term>Lymphangiectasis, Intestinal (diagnosis)</term><term>Lymphangiectasis, Intestinal (pathology)</term><term>Lymphedema (complications)</term><term>Lymphedema (diagnostic imaging)</term><term>Lymphedema (therapy)</term><term>Male</term><term>Radionuclide Imaging</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Biopsie</term><term>Duodénum (anatomopathologie)</term><term>Humains</term><term>Lymphangiectasie intestinale ()</term><term>Lymphangiectasie intestinale (anatomopathologie)</term><term>Lymphangiectasie intestinale (diagnostic)</term><term>Lymphoedème ()</term><term>Lymphoedème (imagerie diagnostique)</term><term>Membres</term><term>Mâle</term><term>Scintigraphie</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Duodénum</term><term>Lymphangiectasie intestinale</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lymphangiectasis, Intestinal</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Lymphangiectasis, Intestinal</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Lymphangiectasie intestinale</term></keywords><keywords scheme="MESH" qualifier="diagnostic imaging" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="imagerie diagnostique" xml:lang="fr"><term>Lymphoedème</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Duodenum</term><term>Lymphangiectasis, Intestinal</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Biopsy</term><term>Extremities</term><term>Humans</term><term>Male</term><term>Radionuclide Imaging</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Biopsie</term><term>Humains</term><term>Lymphangiectasie intestinale</term><term>Lymphoedème</term><term>Membres</term><term>Mâle</term><term>Scintigraphie</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Primary intestinal lymphangiectasia (Waldmann's disease) is characterized by protein-losing enteropathy occurring more frequently in childhood. Chronic diarrhea and diffuse edema are the main clinical manifestations. Peripheral lymphedema may also be associated. Lymphedema is usually present at the time of diagnosis or appears later in the course of the disease. We report the observation of a 31-year-old man suffering from an upper, lower limb and genital lymphedema many years before diagnosis of primary intestinal lymphangiectasia was established. Lower limb lymphoscintigraphy confirmed lymphedema and duodenal biopsies lymphangiectasia. Hypoproteinemia, lymphopenia and hypogammaglobulinemia were also noted. Treatment of lymphedema included low stretch bandaging and elastic stocking. No dietary management with a low-fat diet was added. Search for primary intestinal lymphangiectasia with biological parameters would be useful when primary lymphedema is present. Especially since primary intestinal lymphangiectasia may be complicated by occurrence of B cell lymphoma.</div></front></TEI><affiliations><list><country><li>France</li></country></list><tree><noCountry><name sortKey="Vignes, S" sort="Vignes, S" uniqKey="Vignes S" first="S" last="Vignes">S. Vignes</name></noCountry><country name="France"><noRegion><name sortKey="Boursier, V" sort="Boursier, V" uniqKey="Boursier V" first="V" last="Boursier">V. Boursier</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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