Dysplasia of the lymphatics with lymphoedema, generalized lymphangiectasis, chylothorax and "pseudo-storage-disease".
Identifieur interne : 006E81 ( PubMed/Curation ); précédent : 006E80; suivant : 006E82Dysplasia of the lymphatics with lymphoedema, generalized lymphangiectasis, chylothorax and "pseudo-storage-disease".
Auteurs : H. Heimpel ; J R Bierich ; J M Herrmann ; H. Meister ; J. VollmarSource :
- Lymphology [ 0024-7766 ] ; 1979.
Descripteurs français
- KwdFr :
- Adolescent, Adulte, Chylothorax (anatomopathologie), Chylothorax (étiologie), Enfant, Enfant d'âge préscolaire, Erreurs innées du métabolisme lipidique (), Erreurs innées du métabolisme lipidique (anatomopathologie), Humains, Lymphangiectasie (), Lymphangiectasie (anatomopathologie), Lymphoedème (), Lymphoedème (anatomopathologie), Maladies lymphatiques (anatomopathologie), Mâle, Système lymphatique (anatomopathologie).
- MESH :
- anatomopathologie : Chylothorax, Erreurs innées du métabolisme lipidique, Lymphangiectasie, Lymphoedème, Maladies lymphatiques, Système lymphatique.
- étiologie : Chylothorax.
- Adolescent, Adulte, Enfant, Enfant d'âge préscolaire, Erreurs innées du métabolisme lipidique, Humains, Lymphangiectasie, Lymphoedème, Mâle.
English descriptors
- KwdEn :
- Adolescent, Adult, Child, Child, Preschool, Chylothorax (etiology), Chylothorax (pathology), Humans, Lipid Metabolism, Inborn Errors (complications), Lipid Metabolism, Inborn Errors (pathology), Lymphangiectasis (complications), Lymphangiectasis (pathology), Lymphatic Diseases (pathology), Lymphatic System (pathology), Lymphedema (complications), Lymphedema (pathology), Male.
- MESH :
- complications : Lipid Metabolism, Inborn Errors, Lymphangiectasis, Lymphedema.
- etiology : Chylothorax.
- pathology : Chylothorax, Lipid Metabolism, Inborn Errors, Lymphangiectasis, Lymphatic Diseases, Lymphatic System, Lymphedema.
- Adolescent, Adult, Child, Child, Preschool, Humans, Male.
Abstract
A patient showing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous angle. Chylous effusions and subcutaneous chyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The "pseudo"-storage disease is regarded as secondary to the backflow of chylus into the tissues.
PubMed: 529825
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Heimpel</name></author><author><name sortKey="Bierich, J R" sort="Bierich, J R" uniqKey="Bierich J" first="J R" last="Bierich">J R Bierich</name></author><author><name sortKey="Herrmann, J M" sort="Herrmann, J M" uniqKey="Herrmann J" first="J M" last="Herrmann">J M Herrmann</name></author><author><name sortKey="Meister, H" sort="Meister, H" uniqKey="Meister H" first="H" last="Meister">H. Meister</name></author><author><name sortKey="Vollmar, J" sort="Vollmar, J" uniqKey="Vollmar J" first="J" last="Vollmar">J. Vollmar</name></author></analytic><series><title level="j">Lymphology</title><idno type="ISSN">0024-7766</idno><imprint><date when="1979" type="published">1979</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Child</term><term>Child, Preschool</term><term>Chylothorax (etiology)</term><term>Chylothorax (pathology)</term><term>Humans</term><term>Lipid Metabolism, Inborn Errors (complications)</term><term>Lipid Metabolism, Inborn Errors (pathology)</term><term>Lymphangiectasis (complications)</term><term>Lymphangiectasis (pathology)</term><term>Lymphatic Diseases (pathology)</term><term>Lymphatic System (pathology)</term><term>Lymphedema (complications)</term><term>Lymphedema (pathology)</term><term>Male</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adolescent</term><term>Adulte</term><term>Chylothorax (anatomopathologie)</term><term>Chylothorax (étiologie)</term><term>Enfant</term><term>Enfant d'âge préscolaire</term><term>Erreurs innées du métabolisme lipidique ()</term><term>Erreurs innées du métabolisme lipidique (anatomopathologie)</term><term>Humains</term><term>Lymphangiectasie ()</term><term>Lymphangiectasie (anatomopathologie)</term><term>Lymphoedème ()</term><term>Lymphoedème (anatomopathologie)</term><term>Maladies lymphatiques (anatomopathologie)</term><term>Mâle</term><term>Système lymphatique (anatomopathologie)</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Chylothorax</term><term>Erreurs innées du métabolisme lipidique</term><term>Lymphangiectasie</term><term>Lymphoedème</term><term>Maladies lymphatiques</term><term>Système lymphatique</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lipid Metabolism, Inborn Errors</term><term>Lymphangiectasis</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Chylothorax</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Chylothorax</term><term>Lipid Metabolism, Inborn Errors</term><term>Lymphangiectasis</term><term>Lymphatic Diseases</term><term>Lymphatic System</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="étiologie" xml:lang="fr"><term>Chylothorax</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Child</term><term>Child, Preschool</term><term>Humans</term><term>Male</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adolescent</term><term>Adulte</term><term>Enfant</term><term>Enfant d'âge préscolaire</term><term>Erreurs innées du métabolisme lipidique</term><term>Humains</term><term>Lymphangiectasie</term><term>Lymphoedème</term><term>Mâle</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">A patient showing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous angle. Chylous effusions and subcutaneous chyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The "pseudo"-storage disease is regarded as secondary to the backflow of chylus into the tissues.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">529825</PMID><DateCreated><Year>1980</Year><Month>04</Month><Day>23</Day></DateCreated><DateCompleted><Year>1980</Year><Month>04</Month><Day>23</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>03</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0024-7766</ISSN><JournalIssue CitedMedium="Print"><Volume>12</Volume><Issue>4</Issue><PubDate><Year>1979</Year><Month>Dec</Month></PubDate></JournalIssue><Title>Lymphology</Title><ISOAbbreviation>Lymphology</ISOAbbreviation></Journal><ArticleTitle>Dysplasia of the lymphatics with lymphoedema, generalized lymphangiectasis, chylothorax and "pseudo-storage-disease".</ArticleTitle><Pagination><MedlinePgn>228-40</MedlinePgn></Pagination><Abstract><AbstractText>A patient showing an unusual association of various abnormalities of the lymphatic vascular system is reported. These abnormalities became first evident in early childhood and consisted of lymphoedema of the left leg, lymphangiectasis in various organs and occlusion of the thoracic duct at its entrance into the venous angle. Chylous effusions and subcutaneous chyloedema appeared in adolescence. Diagnostic biopsies of spleen, liver and bone-marrow revealed the presence of multiple foamcells, suggesting the diagnoses of lipid storage disease. The patient died from severe honeycomb-lungs at the age of 20. This case cannot be attributed to one of the wellknown disease entities of the lymphatic system. The "pseudo"-storage disease is regarded as secondary to the backflow of chylus into the tissues.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Heimpel</LastName><ForeName>H</ForeName><Initials>H</Initials></Author><Author ValidYN="Y"><LastName>Bierich</LastName><ForeName>J R</ForeName><Initials>JR</Initials></Author><Author ValidYN="Y"><LastName>Herrmann</LastName><ForeName>J M</ForeName><Initials>JM</Initials></Author><Author ValidYN="Y"><LastName>Meister</LastName><ForeName>H</ForeName><Initials>H</Initials></Author><Author ValidYN="Y"><LastName>Vollmar</LastName><ForeName>J</ForeName><Initials>J</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Lymphology</MedlineTA><NlmUniqueID>0155112</NlmUniqueID><ISSNLinking>0024-7766</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000293" MajorTopicYN="N">Adolescent</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002648" MajorTopicYN="N">Child</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002675" MajorTopicYN="N">Child, Preschool</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002916" MajorTopicYN="N">Chylothorax</DescriptorName><QualifierName UI="Q000209" MajorTopicYN="N">etiology</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008052" MajorTopicYN="N">Lipid Metabolism, Inborn Errors</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008200" MajorTopicYN="N">Lymphangiectasis</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008206" MajorTopicYN="N">Lymphatic Diseases</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008208" MajorTopicYN="N">Lymphatic System</DescriptorName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008209" MajorTopicYN="N">Lymphedema</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1979</Year><Month>12</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1979</Year><Month>12</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1979</Year><Month>12</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">529825</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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