[A rare cause of edema: sporadic lymphangioleiomyomatosis].
Identifieur interne : 001932 ( PubMed/Corpus ); précédent : 001931; suivant : 001933[A rare cause of edema: sporadic lymphangioleiomyomatosis].
Auteurs : H. Frank ; W. WeissSource :
- Deutsche medizinische Wochenschrift (1946) [ 1439-4413 ] ; 2013.
English descriptors
- KwdEn :
- Antibiotics, Antineoplastic (therapeutic use), Biopsy, Diuretics (therapeutic use), Drinking, Female, Humans, Hydrochlorothiazide (therapeutic use), Lung Neoplasms (complications), Lung Neoplasms (diagnosis), Lung Neoplasms (drug therapy), Lung Neoplasms (pathology), Lymphangioleiomyomatosis (complications), Lymphangioleiomyomatosis (diagnosis), Lymphangioleiomyomatosis (drug therapy), Lymphangioleiomyomatosis (pathology), Lymphedema (drug therapy), Lymphedema (etiology), Lymphedema (pathology), Magnetic Resonance Imaging, Middle Aged, Retroperitoneal Neoplasms (complications), Retroperitoneal Neoplasms (diagnosis), Retroperitoneal Neoplasms (drug therapy), Retroperitoneal Neoplasms (pathology), Sirolimus (therapeutic use), Tomography, X-Ray Computed, Vena Cava, Inferior.
- MESH :
- chemical , therapeutic use : Antibiotics, Antineoplastic, Diuretics, Hydrochlorothiazide, Sirolimus.
- complications : Lung Neoplasms, Lymphangioleiomyomatosis, Retroperitoneal Neoplasms.
- diagnosis : Lung Neoplasms, Lymphangioleiomyomatosis, Retroperitoneal Neoplasms.
- drug therapy : Lung Neoplasms, Lymphangioleiomyomatosis, Lymphedema, Retroperitoneal Neoplasms.
- etiology : Lymphedema.
- pathology : Lung Neoplasms, Lymphangioleiomyomatosis, Lymphedema, Retroperitoneal Neoplasms.
- Biopsy, Drinking, Female, Humans, Magnetic Resonance Imaging, Middle Aged, Tomography, X-Ray Computed, Vena Cava, Inferior.
Abstract
A 45-year-old woman presented with marked edema of both lower extremities over 6 weeks for a nephrological work-up; she had gained 8 kg of body weight. Voiding was asymptomatic and she had a stable diuresis. The patient took oestrogens for contraception over 10 years. Blood pressure was normotensive. Serum-creatinine was 0.8 mg/dl; a slight microalbuminuria was noted. Left and right ventricular systolic function were normal. DIAGNOSTIC FINDINGS, TREATMENT AND CLINICAL COURSE: Computed tomography of the abdomen revealed a hemodynamically relevant obstruction of the venous blood flow or the lymphatic vessels as cause for the edema of both legs. Masses of lymphangioleiomyomas located around the v. cava inferior were documented. Biopsy of the masses proved a massive proliferation of smooth muscle cells and epitheloid cells with an immunohistochemically typical staining. Furthermore, CT revealed multiple pulmonary cysts in both lungs, results which are pathognomic for lymphangioleiomyomatosis (LAM). In our patient, the structural impairment of the lungs was not substantiated clinically, i. e. she had only slight dyspnoe during exertion. By mild diuretic treatment with HCT and fluid control, a moderate regression of the edema was achieved. At present, the mTOR inhibitor rapamycin as antiproliferative treatment is considered to reduce the retroperitoneal LAM-related masses on an individual basis.
DOI: 10.1055/s-0033-1343298
PubMed: 24022453
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">[A rare cause of edema: sporadic lymphangioleiomyomatosis].</title><author><name sortKey="Frank, H" sort="Frank, H" uniqKey="Frank H" first="H" last="Frank">H. Frank</name><affiliation><nlm:affiliation>Abteilung Nephrologie, Klinikum Traunstein, Akademisches Lehrkrankenhaus der Ludwig-Maximilians-Universität München.</nlm:affiliation></affiliation></author><author><name sortKey="Weiss, W" sort="Weiss, W" uniqKey="Weiss W" first="W" last="Weiss">W. Weiss</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2013">2013</date><idno type="RBID">pubmed:24022453</idno><idno type="pmid">24022453</idno><idno type="doi">10.1055/s-0033-1343298</idno><idno type="wicri:Area/PubMed/Corpus">001932</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">001932</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">[A rare cause of edema: sporadic lymphangioleiomyomatosis].</title><author><name sortKey="Frank, H" sort="Frank, H" uniqKey="Frank H" first="H" last="Frank">H. Frank</name><affiliation><nlm:affiliation>Abteilung Nephrologie, Klinikum Traunstein, Akademisches Lehrkrankenhaus der Ludwig-Maximilians-Universität München.</nlm:affiliation></affiliation></author><author><name sortKey="Weiss, W" sort="Weiss, W" uniqKey="Weiss W" first="W" last="Weiss">W. Weiss</name></author></analytic><series><title level="j">Deutsche medizinische Wochenschrift (1946)</title><idno type="eISSN">1439-4413</idno><imprint><date when="2013" type="published">2013</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Antibiotics, Antineoplastic (therapeutic use)</term><term>Biopsy</term><term>Diuretics (therapeutic use)</term><term>Drinking</term><term>Female</term><term>Humans</term><term>Hydrochlorothiazide (therapeutic use)</term><term>Lung Neoplasms (complications)</term><term>Lung Neoplasms (diagnosis)</term><term>Lung Neoplasms (drug therapy)</term><term>Lung Neoplasms (pathology)</term><term>Lymphangioleiomyomatosis (complications)</term><term>Lymphangioleiomyomatosis (diagnosis)</term><term>Lymphangioleiomyomatosis (drug therapy)</term><term>Lymphangioleiomyomatosis (pathology)</term><term>Lymphedema (drug therapy)</term><term>Lymphedema (etiology)</term><term>Lymphedema (pathology)</term><term>Magnetic Resonance Imaging</term><term>Middle Aged</term><term>Retroperitoneal Neoplasms (complications)</term><term>Retroperitoneal Neoplasms (diagnosis)</term><term>Retroperitoneal Neoplasms (drug therapy)</term><term>Retroperitoneal Neoplasms (pathology)</term><term>Sirolimus (therapeutic use)</term><term>Tomography, X-Ray Computed</term><term>Vena Cava, Inferior</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Antibiotics, Antineoplastic</term><term>Diuretics</term><term>Hydrochlorothiazide</term><term>Sirolimus</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lung Neoplasms</term><term>Lymphangioleiomyomatosis</term><term>Retroperitoneal Neoplasms</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Lung Neoplasms</term><term>Lymphangioleiomyomatosis</term><term>Retroperitoneal Neoplasms</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Lung Neoplasms</term><term>Lymphangioleiomyomatosis</term><term>Lymphedema</term><term>Retroperitoneal Neoplasms</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Lung Neoplasms</term><term>Lymphangioleiomyomatosis</term><term>Lymphedema</term><term>Retroperitoneal Neoplasms</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Biopsy</term><term>Drinking</term><term>Female</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Middle Aged</term><term>Tomography, X-Ray Computed</term><term>Vena Cava, Inferior</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">A 45-year-old woman presented with marked edema of both lower extremities over 6 weeks for a nephrological work-up; she had gained 8 kg of body weight. Voiding was asymptomatic and she had a stable diuresis. The patient took oestrogens for contraception over 10 years. Blood pressure was normotensive. Serum-creatinine was 0.8 mg/dl; a slight microalbuminuria was noted. Left and right ventricular systolic function were normal. DIAGNOSTIC FINDINGS, TREATMENT AND CLINICAL COURSE: Computed tomography of the abdomen revealed a hemodynamically relevant obstruction of the venous blood flow or the lymphatic vessels as cause for the edema of both legs. Masses of lymphangioleiomyomas located around the v. cava inferior were documented. Biopsy of the masses proved a massive proliferation of smooth muscle cells and epitheloid cells with an immunohistochemically typical staining. Furthermore, CT revealed multiple pulmonary cysts in both lungs, results which are pathognomic for lymphangioleiomyomatosis (LAM). In our patient, the structural impairment of the lungs was not substantiated clinically, i. e. she had only slight dyspnoe during exertion. By mild diuretic treatment with HCT and fluid control, a moderate regression of the edema was achieved. At present, the mTOR inhibitor rapamycin as antiproliferative treatment is considered to reduce the retroperitoneal LAM-related masses on an individual basis.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">24022453</PMID><DateCreated><Year>2013</Year><Month>09</Month><Day>11</Day></DateCreated><DateCompleted><Year>2013</Year><Month>11</Month><Day>12</Day></DateCompleted><DateRevised><Year>2013</Year><Month>11</Month><Day>21</Day></DateRevised><Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1439-4413</ISSN><JournalIssue CitedMedium="Internet"><Volume>138</Volume><Issue>38</Issue><PubDate><Year>2013</Year><Month>Sep</Month></PubDate></JournalIssue><Title>Deutsche medizinische Wochenschrift (1946)</Title><ISOAbbreviation>Dtsch. Med. Wochenschr.</ISOAbbreviation></Journal><ArticleTitle>[A rare cause of edema: sporadic lymphangioleiomyomatosis].</ArticleTitle><Pagination><MedlinePgn>1892-5</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1055/s-0033-1343298</ELocationID><Abstract><AbstractText Label="HISTORY AND CLINICAL FINDINGS" NlmCategory="METHODS">A 45-year-old woman presented with marked edema of both lower extremities over 6 weeks for a nephrological work-up; she had gained 8 kg of body weight. Voiding was asymptomatic and she had a stable diuresis. The patient took oestrogens for contraception over 10 years. Blood pressure was normotensive. Serum-creatinine was 0.8 mg/dl; a slight microalbuminuria was noted. Left and right ventricular systolic function were normal. DIAGNOSTIC FINDINGS, TREATMENT AND CLINICAL COURSE: Computed tomography of the abdomen revealed a hemodynamically relevant obstruction of the venous blood flow or the lymphatic vessels as cause for the edema of both legs. Masses of lymphangioleiomyomas located around the v. cava inferior were documented. Biopsy of the masses proved a massive proliferation of smooth muscle cells and epitheloid cells with an immunohistochemically typical staining. Furthermore, CT revealed multiple pulmonary cysts in both lungs, results which are pathognomic for lymphangioleiomyomatosis (LAM). In our patient, the structural impairment of the lungs was not substantiated clinically, i. e. she had only slight dyspnoe during exertion. By mild diuretic treatment with HCT and fluid control, a moderate regression of the edema was achieved. At present, the mTOR inhibitor rapamycin as antiproliferative treatment is considered to reduce the retroperitoneal LAM-related masses on an individual basis.</AbstractText><AbstractText Label="CONCLUSION" NlmCategory="CONCLUSIONS">LAM is a rare genetically determined progressive disease occurring frequently in women in childbearing age. LAM is characterized by a proliferation of smooth muscle cells, lymphangioma, renal angiomyolipoma, pulmonary cysts and progressive destruction of lung parenchyma. Refractory edema can result from an obstruction of the venous blood and lymphatic flow by lymphangioma masses located paracaval, which was the impressive and first clinical feature of LAM in our case report.</AbstractText><CopyrightInformation>© Georg Thieme Verlag KG Stuttgart · New York.</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Frank</LastName><ForeName>H</ForeName><Initials>H</Initials><AffiliationInfo><Affiliation>Abteilung Nephrologie, Klinikum Traunstein, Akademisches Lehrkrankenhaus der Ludwig-Maximilians-Universität München.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Weiss</LastName><ForeName>W</ForeName><Initials>W</Initials></Author></AuthorList><Language>ger</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D004740">English Abstract</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList><VernacularTitle>Seltene Ursache peripherer Ödembildung: die sporadische Lymphangioleiomyomatose.</VernacularTitle><ArticleDate DateType="Electronic"><Year>2013</Year><Month>09</Month><Day>10</Day></ArticleDate></Article><MedlineJournalInfo><Country>Germany</Country><MedlineTA>Dtsch Med Wochenschr</MedlineTA><NlmUniqueID>0006723</NlmUniqueID><ISSNLinking>0012-0472</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D000903">Antibiotics, Antineoplastic</NameOfSubstance></Chemical><Chemical><RegistryNumber>0</RegistryNumber><NameOfSubstance UI="D004232">Diuretics</NameOfSubstance></Chemical><Chemical><RegistryNumber>0J48LPH2TH</RegistryNumber><NameOfSubstance UI="D006852">Hydrochlorothiazide</NameOfSubstance></Chemical><Chemical><RegistryNumber>W36ZG6FT64</RegistryNumber><NameOfSubstance UI="D020123">Sirolimus</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000903" MajorTopicYN="N">Antibiotics, Antineoplastic</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D001706" MajorTopicYN="N">Biopsy</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D004232" MajorTopicYN="N">Diuretics</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D004326" MajorTopicYN="N">Drinking</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006852" MajorTopicYN="N">Hydrochlorothiazide</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008175" MajorTopicYN="N">Lung Neoplasms</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="Y">diagnosis</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D018192" MajorTopicYN="N">Lymphangioleiomyomatosis</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="Y">diagnosis</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008209" MajorTopicYN="N">Lymphedema</DescriptorName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName><QualifierName UI="Q000209" MajorTopicYN="Y">etiology</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D008279" MajorTopicYN="N">Magnetic Resonance Imaging</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D012186" MajorTopicYN="N">Retroperitoneal Neoplasms</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="Y">complications</QualifierName><QualifierName UI="Q000175" MajorTopicYN="Y">diagnosis</QualifierName><QualifierName UI="Q000188" MajorTopicYN="N">drug therapy</QualifierName><QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D020123" MajorTopicYN="N">Sirolimus</DescriptorName><QualifierName UI="Q000627" MajorTopicYN="N">therapeutic use</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D014057" MajorTopicYN="N">Tomography, X-Ray Computed</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D014682" MajorTopicYN="N">Vena Cava, Inferior</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2013</Year><Month>9</Month><Day>12</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2013</Year><Month>9</Month><Day>12</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2013</Year><Month>11</Month><Day>13</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">24022453</ArticleId><ArticleId IdType="doi">10.1055/s-0033-1343298</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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