LymphedemaV1, Main, Exploration, bibRecord, 002F65

[A rare cause of edema: sporadic lymphangioleiomyomatosis].

Identifieur interne : 002F65 ( Main/Exploration ); précédent : 002F64; suivant : 002F66

[A rare cause of edema: sporadic lymphangioleiomyomatosis].

Auteurs : H. Frank [Allemagne] ; W. Weiss

Source :

Deutsche medizinische Wochenschrift (1946) [ 1439-4413 ] ; 2013.

RBID : pubmed:24022453

Descripteurs français

KwdFr :
- Adulte d'âge moyen, Antibiotiques antinéoplasiques (usage thérapeutique), Biopsie, Consommation de boisson, Diurétiques (usage thérapeutique), Femelle, Humains, Hydrochlorothiazide (usage thérapeutique), Imagerie par résonance magnétique, Lymphangioléiomyomatose (), Lymphangioléiomyomatose (anatomopathologie), Lymphangioléiomyomatose (diagnostic), Lymphangioléiomyomatose (traitement médicamenteux), Lymphoedème (anatomopathologie), Lymphoedème (traitement médicamenteux), Lymphoedème (étiologie), Sirolimus (usage thérapeutique), Tomodensitométrie, Tumeurs du poumon (), Tumeurs du poumon (anatomopathologie), Tumeurs du poumon (diagnostic), Tumeurs du poumon (traitement médicamenteux), Tumeurs du rétropéritoine (), Tumeurs du rétropéritoine (anatomopathologie), Tumeurs du rétropéritoine (diagnostic), Tumeurs du rétropéritoine (traitement médicamenteux), Veine cave inférieure.
MESH :
- anatomopathologie : Lymphangioléiomyomatose, Lymphoedème, Tumeurs du poumon, Tumeurs du rétropéritoine.
- diagnostic : Lymphangioléiomyomatose, Tumeurs du poumon, Tumeurs du rétropéritoine.
- traitement médicamenteux : Lymphangioléiomyomatose, Lymphoedème, Tumeurs du poumon, Tumeurs du rétropéritoine.
- usage thérapeutique : Antibiotiques antinéoplasiques, Diurétiques, Hydrochlorothiazide, Sirolimus.
- étiologie : Lymphoedème.
- Adulte d'âge moyen, Biopsie, Consommation de boisson, Femelle, Humains, Imagerie par résonance magnétique, Lymphangioléiomyomatose, Tomodensitométrie, Tumeurs du poumon, Tumeurs du rétropéritoine, Veine cave inférieure.

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

Affiliations:

Le document en format XML

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<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>
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<term>Lymphedema (pathology)</term>
<term>Magnetic Resonance Imaging</term>
<term>Middle Aged</term>
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<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>
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<keywords scheme="KwdFr" xml:lang="fr"><term>Adulte d'âge moyen</term>
<term>Antibiotiques antinéoplasiques (usage thérapeutique)</term>
<term>Biopsie</term>
<term>Consommation de boisson</term>
<term>Diurétiques (usage thérapeutique)</term>
<term>Femelle</term>
<term>Humains</term>
<term>Hydrochlorothiazide (usage thérapeutique)</term>
<term>Imagerie par résonance magnétique</term>
<term>Lymphangioléiomyomatose ()</term>
<term>Lymphangioléiomyomatose (anatomopathologie)</term>
<term>Lymphangioléiomyomatose (diagnostic)</term>
<term>Lymphangioléiomyomatose (traitement médicamenteux)</term>
<term>Lymphoedème (anatomopathologie)</term>
<term>Lymphoedème (traitement médicamenteux)</term>
<term>Lymphoedème (étiologie)</term>
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<term>Tumeurs du rétropéritoine (diagnostic)</term>
<term>Tumeurs du rétropéritoine (traitement médicamenteux)</term>
<term>Veine cave inférieure</term>
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<term>Diuretics</term>
<term>Hydrochlorothiazide</term>
<term>Sirolimus</term>
</keywords>
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<term>Lymphoedème</term>
<term>Tumeurs du poumon</term>
<term>Tumeurs du rétropéritoine</term>
</keywords>
<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lung Neoplasms</term>
<term>Lymphangioleiomyomatosis</term>
<term>Retroperitoneal Neoplasms</term>
</keywords>
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<term>Lymphangioleiomyomatosis</term>
<term>Retroperitoneal Neoplasms</term>
</keywords>
<keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Lymphangioléiomyomatose</term>
<term>Tumeurs du poumon</term>
<term>Tumeurs du rétropéritoine</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>
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<term>Lymphangioleiomyomatosis</term>
<term>Lymphedema</term>
<term>Retroperitoneal Neoplasms</term>
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<term>Lymphoedème</term>
<term>Tumeurs du poumon</term>
<term>Tumeurs du rétropéritoine</term>
</keywords>
<keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Antibiotiques antinéoplasiques</term>
<term>Diurétiques</term>
<term>Hydrochlorothiazide</term>
<term>Sirolimus</term>
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</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>
<keywords scheme="MESH" xml:lang="fr"><term>Adulte d'âge moyen</term>
<term>Biopsie</term>
<term>Consommation de boisson</term>
<term>Femelle</term>
<term>Humains</term>
<term>Imagerie par résonance magnétique</term>
<term>Lymphangioléiomyomatose</term>
<term>Tomodensitométrie</term>
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<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>
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<tree><noCountry><name sortKey="Weiss, W" sort="Weiss, W" uniqKey="Weiss W" first="W" last="Weiss">W. Weiss</name>
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<country name="Allemagne"><region name="Bavière"><name sortKey="Frank, H" sort="Frank, H" uniqKey="Frank H" first="H" last="Frank">H. Frank</name>
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Serveur d'exploration sur le lymphœdème

[A rare cause of edema: sporadic lymphangioleiomyomatosis].

[A rare cause of edema: sporadic lymphangioleiomyomatosis].

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri

Pour générer des pages wiki

	Serveur d'exploration sur le lymphœdème
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