Chyloperitoneum, chylothorax and lower extremity lymphedema in woman with sporadic lymphangioleiomyomatosis successfully treated with sirolimus: a case report.
Identifieur interne : 004450 ( Main/Exploration ); précédent : 004449; suivant : 004451Chyloperitoneum, chylothorax and lower extremity lymphedema in woman with sporadic lymphangioleiomyomatosis successfully treated with sirolimus: a case report.
Auteurs : A. Chachaj [Pologne] ; K. Drozdz ; M. Chabowski ; P. Dziegiel ; I. Grzegorek ; A. Wojnar ; P. Jazwiec ; A. SzubaSource :
- Lymphology [ 0024-7766 ] ; 2012.
Descripteurs français
- KwdFr :
- Adulte d'âge moyen, Ascite chyleuse (diagnostic), Ascite chyleuse (traitement médicamenteux), Chylothorax (diagnostic), Chylothorax (traitement médicamenteux), Femelle, Humains, Immunosuppresseurs (usage thérapeutique), Jambe, Lymphangioléiomyomatose (traitement médicamenteux), Lymphoedème (diagnostic), Lymphoedème (traitement médicamenteux), Pronostic, Sirolimus (usage thérapeutique), Tomodensitométrie.
- MESH :
- diagnostic : Ascite chyleuse, Chylothorax, Lymphoedème.
- traitement médicamenteux : Ascite chyleuse, Chylothorax, Lymphangioléiomyomatose, Lymphoedème.
- usage thérapeutique : Immunosuppresseurs, Sirolimus.
- Adulte d'âge moyen, Femelle, Humains, Jambe, Pronostic, Tomodensitométrie.
English descriptors
- KwdEn :
- Chylothorax (diagnosis), Chylothorax (drug therapy), Chylous Ascites (diagnosis), Chylous Ascites (drug therapy), Female, Humans, Immunosuppressive Agents (therapeutic use), Leg, Lymphangioleiomyomatosis (drug therapy), Lymphedema (diagnosis), Lymphedema (drug therapy), Middle Aged, Prognosis, Sirolimus (therapeutic use), Tomography, X-Ray Computed.
- MESH :
- chemical , therapeutic use : Immunosuppressive Agents, Sirolimus.
- diagnosis : Chylothorax, Chylous Ascites, Lymphedema.
- drug therapy : Chylothorax, Chylous Ascites, Lymphangioleiomyomatosis, Lymphedema.
- Female, Humans, Leg, Middle Aged, Prognosis, Tomography, X-Ray Computed.
Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease characterized by diffuse thin-walled cysts throughout the lungs on computed tomography and diffuse proliferation of abnormal smooth muscle-like cells (LAM cells) on lung biopsy. LAM affects women almost exclusively, predominantly in their reproductive age. The most typical presenting symptoms include dyspnea, spontaneous pneumothorax, cough and chylothorax. Abdominal findings represent less common initial manifestations of the disease and may pose diagnostic difficulties. The treatment of LAM has not been fully established. Recent studies report effectiveness of sirolimus in LAM patients. We report the case of a 45-year-old woman with sporadic LAM, successfully treated with sirolimus, in whom the first manifestation of the disease was chyloperitoneum and after three and nine years, respectively, lymphedema of the left lower extremity and right sided chylothorax occurred.
PubMed: 23057149
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream PubMed, to step Corpus: 001F14
- to stream PubMed, to step Curation: 001F14
- to stream PubMed, to step Checkpoint: 001F14
- to stream Ncbi, to step Merge: 005207
- to stream Ncbi, to step Curation: 005207
- to stream Ncbi, to step Checkpoint: 005207
- to stream Main, to step Merge: 004471
- to stream Main, to step Curation: 004450
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Chyloperitoneum, chylothorax and lower extremity lymphedema in woman with sporadic lymphangioleiomyomatosis successfully treated with sirolimus: a case report.</title><author><name sortKey="Chachaj, A" sort="Chachaj, A" uniqKey="Chachaj A" first="A" last="Chachaj">A. Chachaj</name><affiliation wicri:level="1"><nlm:affiliation>Department of Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.</nlm:affiliation><country xml:lang="fr">Pologne</country><wicri:regionArea>Department of Internal Medicine, Wroclaw Medical University, Wroclaw</wicri:regionArea><wicri:noRegion>Wroclaw</wicri:noRegion></affiliation></author><author><name sortKey="Drozdz, K" sort="Drozdz, K" uniqKey="Drozdz K" first="K" last="Drozdz">K. Drozdz</name></author><author><name sortKey="Chabowski, M" sort="Chabowski, M" uniqKey="Chabowski M" first="M" last="Chabowski">M. Chabowski</name></author><author><name sortKey="Dziegiel, P" sort="Dziegiel, P" uniqKey="Dziegiel P" first="P" last="Dziegiel">P. Dziegiel</name></author><author><name sortKey="Grzegorek, I" sort="Grzegorek, I" uniqKey="Grzegorek I" first="I" last="Grzegorek">I. Grzegorek</name></author><author><name sortKey="Wojnar, A" sort="Wojnar, A" uniqKey="Wojnar A" first="A" last="Wojnar">A. Wojnar</name></author><author><name sortKey="Jazwiec, P" sort="Jazwiec, P" uniqKey="Jazwiec P" first="P" last="Jazwiec">P. Jazwiec</name></author><author><name sortKey="Szuba, A" sort="Szuba, A" uniqKey="Szuba A" first="A" last="Szuba">A. Szuba</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2012">2012</date><idno type="RBID">pubmed:23057149</idno><idno type="pmid">23057149</idno><idno type="wicri:Area/PubMed/Corpus">001F14</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001F14</idno><idno type="wicri:Area/PubMed/Curation">001F14</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">001F14</idno><idno type="wicri:Area/PubMed/Checkpoint">001F14</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">001F14</idno><idno type="wicri:Area/Ncbi/Merge">005207</idno><idno type="wicri:Area/Ncbi/Curation">005207</idno><idno type="wicri:Area/Ncbi/Checkpoint">005207</idno><idno type="wicri:doubleKey">0024-7766:2012:Chachaj A:chyloperitoneum:chylothorax:and</idno><idno type="wicri:Area/Main/Merge">004471</idno><idno type="wicri:Area/Main/Curation">004450</idno><idno type="wicri:Area/Main/Exploration">004450</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Chyloperitoneum, chylothorax and lower extremity lymphedema in woman with sporadic lymphangioleiomyomatosis successfully treated with sirolimus: a case report.</title><author><name sortKey="Chachaj, A" sort="Chachaj, A" uniqKey="Chachaj A" first="A" last="Chachaj">A. Chachaj</name><affiliation wicri:level="1"><nlm:affiliation>Department of Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.</nlm:affiliation><country xml:lang="fr">Pologne</country><wicri:regionArea>Department of Internal Medicine, Wroclaw Medical University, Wroclaw</wicri:regionArea><wicri:noRegion>Wroclaw</wicri:noRegion></affiliation></author><author><name sortKey="Drozdz, K" sort="Drozdz, K" uniqKey="Drozdz K" first="K" last="Drozdz">K. Drozdz</name></author><author><name sortKey="Chabowski, M" sort="Chabowski, M" uniqKey="Chabowski M" first="M" last="Chabowski">M. Chabowski</name></author><author><name sortKey="Dziegiel, P" sort="Dziegiel, P" uniqKey="Dziegiel P" first="P" last="Dziegiel">P. Dziegiel</name></author><author><name sortKey="Grzegorek, I" sort="Grzegorek, I" uniqKey="Grzegorek I" first="I" last="Grzegorek">I. Grzegorek</name></author><author><name sortKey="Wojnar, A" sort="Wojnar, A" uniqKey="Wojnar A" first="A" last="Wojnar">A. Wojnar</name></author><author><name sortKey="Jazwiec, P" sort="Jazwiec, P" uniqKey="Jazwiec P" first="P" last="Jazwiec">P. Jazwiec</name></author><author><name sortKey="Szuba, A" sort="Szuba, A" uniqKey="Szuba A" first="A" last="Szuba">A. Szuba</name></author></analytic><series><title level="j">Lymphology</title><idno type="ISSN">0024-7766</idno><imprint><date when="2012" type="published">2012</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Chylothorax (diagnosis)</term><term>Chylothorax (drug therapy)</term><term>Chylous Ascites (diagnosis)</term><term>Chylous Ascites (drug therapy)</term><term>Female</term><term>Humans</term><term>Immunosuppressive Agents (therapeutic use)</term><term>Leg</term><term>Lymphangioleiomyomatosis (drug therapy)</term><term>Lymphedema (diagnosis)</term><term>Lymphedema (drug therapy)</term><term>Middle Aged</term><term>Prognosis</term><term>Sirolimus (therapeutic use)</term><term>Tomography, X-Ray Computed</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte d'âge moyen</term><term>Ascite chyleuse (diagnostic)</term><term>Ascite chyleuse (traitement médicamenteux)</term><term>Chylothorax (diagnostic)</term><term>Chylothorax (traitement médicamenteux)</term><term>Femelle</term><term>Humains</term><term>Immunosuppresseurs (usage thérapeutique)</term><term>Jambe</term><term>Lymphangioléiomyomatose (traitement médicamenteux)</term><term>Lymphoedème (diagnostic)</term><term>Lymphoedème (traitement médicamenteux)</term><term>Pronostic</term><term>Sirolimus (usage thérapeutique)</term><term>Tomodensitométrie</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Immunosuppressive Agents</term><term>Sirolimus</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Chylothorax</term><term>Chylous Ascites</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Ascite chyleuse</term><term>Chylothorax</term><term>Lymphoedème</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Chylothorax</term><term>Chylous Ascites</term><term>Lymphangioleiomyomatosis</term><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="traitement médicamenteux" xml:lang="fr"><term>Ascite chyleuse</term><term>Chylothorax</term><term>Lymphangioléiomyomatose</term><term>Lymphoedème</term></keywords><keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Immunosuppresseurs</term><term>Sirolimus</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Female</term><term>Humans</term><term>Leg</term><term>Middle Aged</term><term>Prognosis</term><term>Tomography, X-Ray Computed</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte d'âge moyen</term><term>Femelle</term><term>Humains</term><term>Jambe</term><term>Pronostic</term><term>Tomodensitométrie</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Lymphangioleiomyomatosis (LAM) is a rare disease characterized by diffuse thin-walled cysts throughout the lungs on computed tomography and diffuse proliferation of abnormal smooth muscle-like cells (LAM cells) on lung biopsy. LAM affects women almost exclusively, predominantly in their reproductive age. The most typical presenting symptoms include dyspnea, spontaneous pneumothorax, cough and chylothorax. Abdominal findings represent less common initial manifestations of the disease and may pose diagnostic difficulties. The treatment of LAM has not been fully established. Recent studies report effectiveness of sirolimus in LAM patients. We report the case of a 45-year-old woman with sporadic LAM, successfully treated with sirolimus, in whom the first manifestation of the disease was chyloperitoneum and after three and nine years, respectively, lymphedema of the left lower extremity and right sided chylothorax occurred.</div></front></TEI><affiliations><list><country><li>Pologne</li></country></list><tree><noCountry><name sortKey="Chabowski, M" sort="Chabowski, M" uniqKey="Chabowski M" first="M" last="Chabowski">M. Chabowski</name><name sortKey="Drozdz, K" sort="Drozdz, K" uniqKey="Drozdz K" first="K" last="Drozdz">K. Drozdz</name><name sortKey="Dziegiel, P" sort="Dziegiel, P" uniqKey="Dziegiel P" first="P" last="Dziegiel">P. Dziegiel</name><name sortKey="Grzegorek, I" sort="Grzegorek, I" uniqKey="Grzegorek I" first="I" last="Grzegorek">I. Grzegorek</name><name sortKey="Jazwiec, P" sort="Jazwiec, P" uniqKey="Jazwiec P" first="P" last="Jazwiec">P. Jazwiec</name><name sortKey="Szuba, A" sort="Szuba, A" uniqKey="Szuba A" first="A" last="Szuba">A. Szuba</name><name sortKey="Wojnar, A" sort="Wojnar, A" uniqKey="Wojnar A" first="A" last="Wojnar">A. Wojnar</name></noCountry><country name="Pologne"><noRegion><name sortKey="Chachaj, A" sort="Chachaj, A" uniqKey="Chachaj A" first="A" last="Chachaj">A. Chachaj</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/LymphedemaV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 004450 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 004450 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Sante
|area= LymphedemaV1
|flux= Main
|étape= Exploration
|type= RBID
|clé= pubmed:23057149
|texte= Chyloperitoneum, chylothorax and lower extremity lymphedema in woman with sporadic lymphangioleiomyomatosis successfully treated with sirolimus: a case report.
}}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/Main/Exploration/RBID.i -Sk "pubmed:23057149" \
| HfdSelect -Kh $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd \
| NlmPubMed2Wicri -a LymphedemaV1
| This area was generated with Dilib version V0.6.31. |  |