Klippel-Trénaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction.
Identifieur interne : 004D02 ( PubMed/Checkpoint ); précédent : 004D01; suivant : 004D03Klippel-Trénaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction.
Auteurs : S C Muluk [États-Unis] ; L C Ginns ; M J Semigran ; J A Kaufman ; J P GertlerSource :
- Journal of vascular surgery [ 0741-5214 ] ; 1995.
Descripteurs français
- KwdFr :
- MESH :
- diagnostic : Syndrome de Klippel-Trénaunay.
- étiologie : Dyspnée, Embolie pulmonaire, Thrombophlébite.
- Adulte, Humains, Lymphoedème, Maladie chronique, Mâle, Syndrome de Klippel-Trénaunay, Études de suivi.
English descriptors
- KwdEn :
- MESH :
- complications : Klippel-Trenaunay-Weber Syndrome.
- congenital : Lymphedema.
- diagnosis : Klippel-Trenaunay-Weber Syndrome.
- etiology : Dyspnea, Pulmonary Embolism, Thrombophlebitis.
- Adult, Chronic Disease, Follow-Up Studies, Humans, Male.
Abstract
A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented.
PubMed: 7707572
Affiliations:
Links toward previous steps (curation, corpus...)
Links to Exploration step
pubmed:7707572Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Klippel-Trénaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction.</title><author><name sortKey="Muluk, S C" sort="Muluk, S C" uniqKey="Muluk S" first="S C" last="Muluk">S C Muluk</name><affiliation wicri:level="1"><nlm:affiliation>Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston 02114, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston 02114</wicri:regionArea><placeName><settlement type="city">Boston</settlement><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Ginns, L C" sort="Ginns, L C" uniqKey="Ginns L" first="L C" last="Ginns">L C Ginns</name></author><author><name sortKey="Semigran, M J" sort="Semigran, M J" uniqKey="Semigran M" first="M J" last="Semigran">M J Semigran</name></author><author><name sortKey="Kaufman, J A" sort="Kaufman, J A" uniqKey="Kaufman J" first="J A" last="Kaufman">J A Kaufman</name></author><author><name sortKey="Gertler, J P" sort="Gertler, J P" uniqKey="Gertler J" first="J P" last="Gertler">J P Gertler</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1995">1995</date><idno type="RBID">pubmed:7707572</idno><idno type="pmid">7707572</idno><idno type="wicri:Area/PubMed/Corpus">005406</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">005406</idno><idno type="wicri:Area/PubMed/Curation">005406</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">005406</idno><idno type="wicri:Area/PubMed/Checkpoint">005406</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">005406</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Klippel-Trénaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction.</title><author><name sortKey="Muluk, S C" sort="Muluk, S C" uniqKey="Muluk S" first="S C" last="Muluk">S C Muluk</name><affiliation wicri:level="1"><nlm:affiliation>Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston 02114, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston 02114</wicri:regionArea><placeName><settlement type="city">Boston</settlement><region type="state">Massachusetts</region></placeName></affiliation></author><author><name sortKey="Ginns, L C" sort="Ginns, L C" uniqKey="Ginns L" first="L C" last="Ginns">L C Ginns</name></author><author><name sortKey="Semigran, M J" sort="Semigran, M J" uniqKey="Semigran M" first="M J" last="Semigran">M J Semigran</name></author><author><name sortKey="Kaufman, J A" sort="Kaufman, J A" uniqKey="Kaufman J" first="J A" last="Kaufman">J A Kaufman</name></author><author><name sortKey="Gertler, J P" sort="Gertler, J P" uniqKey="Gertler J" first="J P" last="Gertler">J P Gertler</name></author></analytic><series><title level="j">Journal of vascular surgery</title><idno type="ISSN">0741-5214</idno><imprint><date when="1995" type="published">1995</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Chronic Disease</term><term>Dyspnea (etiology)</term><term>Follow-Up Studies</term><term>Humans</term><term>Klippel-Trenaunay-Weber Syndrome (complications)</term><term>Klippel-Trenaunay-Weber Syndrome (diagnosis)</term><term>Lymphedema (congenital)</term><term>Male</term><term>Pulmonary Embolism (etiology)</term><term>Thrombophlebitis (etiology)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Dyspnée (étiologie)</term><term>Embolie pulmonaire (étiologie)</term><term>Humains</term><term>Lymphoedème ()</term><term>Maladie chronique</term><term>Mâle</term><term>Syndrome de Klippel-Trénaunay ()</term><term>Syndrome de Klippel-Trénaunay (diagnostic)</term><term>Thrombophlébite (étiologie)</term><term>Études de suivi</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Klippel-Trenaunay-Weber Syndrome</term></keywords><keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Klippel-Trenaunay-Weber Syndrome</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Syndrome de Klippel-Trénaunay</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Dyspnea</term><term>Pulmonary Embolism</term><term>Thrombophlebitis</term></keywords><keywords scheme="MESH" qualifier="étiologie" xml:lang="fr"><term>Dyspnée</term><term>Embolie pulmonaire</term><term>Thrombophlébite</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Chronic Disease</term><term>Follow-Up Studies</term><term>Humans</term><term>Male</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Humains</term><term>Lymphoedème</term><term>Maladie chronique</term><term>Mâle</term><term>Syndrome de Klippel-Trénaunay</term><term>Études de suivi</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">7707572</PMID><DateCreated><Year>1995</Year><Month>05</Month><Day>11</Day></DateCreated><DateCompleted><Year>1995</Year><Month>05</Month><Day>11</Day></DateCompleted><DateRevised><Year>2012</Year><Month>10</Month><Day>03</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0741-5214</ISSN><JournalIssue CitedMedium="Print"><Volume>21</Volume><Issue>4</Issue><PubDate><Year>1995</Year><Month>Apr</Month></PubDate></JournalIssue><Title>Journal of vascular surgery</Title><ISOAbbreviation>J. Vasc. Surg.</ISOAbbreviation></Journal><ArticleTitle>Klippel-Trénaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction.</ArticleTitle><Pagination><MedlinePgn>686-90</MedlinePgn></Pagination><Abstract><AbstractText>A 32-year-old man with progressive dyspnea and congenital lymphedema was transferred to our hospital for evaluation as a lung transplant candidate with the suspected diagnosis of primary pulmonary hypertension. Evaluation revealed the additional history of previous limb-shortening procedures for the left leg, the presence of syndactyly, long-standing bilateral (left to right) lower extremity varices, as well as soft tissue asymmetry with the left leg and arm larger than the right-sided counterparts. A diagnosis of Klippel-Trénaunay syndrome was made on the basis of these findings. Because of the deep venous malformations known to occur in this syndrome, we sought evidence of recurrent pulmonary emboli as an explanation for the patient's progressive dyspnea, despite negative pulmonary arteriography and ventilation-perfusion scanning results at another institution. Repeat pulmonary arteriography demonstrated evidence of chronic and subacute pulmonary emboli. The patient is presently being treated with warfarin anticoagulation, with plans for placement of a caval filter if anticoagulation alone is insufficient to prevent further embolism. Klippel-Trénaunay syndrome is a rare cause of chronic pulmonary emboli, and this entity should be considered when the characteristic historical and physical findings are present. A case report and review of the syndrome, with particular focus on the aspects relevant to the vascular surgeon, are presented.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Muluk</LastName><ForeName>S C</ForeName><Initials>SC</Initials><AffiliationInfo><Affiliation>Department of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston 02114, USA.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Ginns</LastName><ForeName>L C</ForeName><Initials>LC</Initials></Author><Author ValidYN="Y"><LastName>Semigran</LastName><ForeName>M J</ForeName><Initials>MJ</Initials></Author><Author ValidYN="Y"><LastName>Kaufman</LastName><ForeName>J A</ForeName><Initials>JA</Initials></Author><Author ValidYN="Y"><LastName>Gertler</LastName><ForeName>J P</ForeName><Initials>JP</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>J Vasc Surg</MedlineTA><NlmUniqueID>8407742</NlmUniqueID><ISSNLinking>0741-5214</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D002908" MajorTopicYN="N">Chronic Disease</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D004417" MajorTopicYN="N">Dyspnea</DescriptorName><QualifierName UI="Q000209" MajorTopicYN="N">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D005500" MajorTopicYN="N">Follow-Up Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007715" MajorTopicYN="N">Klippel-Trenaunay-Weber Syndrome</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008209" MajorTopicYN="N">Lymphedema</DescriptorName><QualifierName UI="Q000151" MajorTopicYN="N">congenital</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D011655" MajorTopicYN="N">Pulmonary Embolism</DescriptorName><QualifierName UI="Q000209" MajorTopicYN="Y">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D013924" MajorTopicYN="N">Thrombophlebitis</DescriptorName><QualifierName UI="Q000209" MajorTopicYN="N">etiology</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>1995</Year><Month>4</Month><Day>1</Day></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>1995</Year><Month>4</Month><Day>1</Day><Hour>0</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>1995</Year><Month>4</Month><Day>1</Day><Hour>0</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">7707572</ArticleId><ArticleId IdType="pii">S0741-5214(95)70199-0</ArticleId></ArticleIdList></PubmedData></pubmed><affiliations><list><country><li>États-Unis</li></country><region><li>Massachusetts</li></region><settlement><li>Boston</li></settlement></list><tree><noCountry><name sortKey="Gertler, J P" sort="Gertler, J P" uniqKey="Gertler J" first="J P" last="Gertler">J P Gertler</name><name sortKey="Ginns, L C" sort="Ginns, L C" uniqKey="Ginns L" first="L C" last="Ginns">L C Ginns</name><name sortKey="Kaufman, J A" sort="Kaufman, J A" uniqKey="Kaufman J" first="J A" last="Kaufman">J A Kaufman</name><name sortKey="Semigran, M J" sort="Semigran, M J" uniqKey="Semigran M" first="M J" last="Semigran">M J Semigran</name></noCountry><country name="États-Unis"><region name="Massachusetts"><name sortKey="Muluk, S C" sort="Muluk, S C" uniqKey="Muluk S" first="S C" last="Muluk">S C Muluk</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/LymphedemaV1/Data/PubMed/Checkpoint
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 004D02 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/PubMed/Checkpoint/biblio.hfd -nk 004D02 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Sante
|area= LymphedemaV1
|flux= PubMed
|étape= Checkpoint
|type= RBID
|clé= pubmed:7707572
|texte= Klippel-Trénaunay syndrome with multiple pulmonary emboli--an unusual cause of progressive pulmonary dysfunction.
}}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/PubMed/Checkpoint/RBID.i -Sk "pubmed:7707572" \
| HfdSelect -Kh $EXPLOR_AREA/Data/PubMed/Checkpoint/biblio.hfd \
| NlmPubMed2Wicri -a LymphedemaV1
| This area was generated with Dilib version V0.6.31. |  |