[Primary lymphedema of limbs].
Identifieur interne : 000323 ( France/Analysis ); précédent : 000322; suivant : 000324[Primary lymphedema of limbs].
Auteurs : Loïc Vaillant [France] ; Valérie TauveronSource :
- Presse medicale (Paris, France : 1983) [ 2213-0276 ] ; 2010.
Descripteurs français
- KwdFr :
- Adulte, Analyse de mutations d'ADN, Diagnostic différentiel, Facteurs de transcription Forkhead (génétique), Facteurs de transcription SOX-F (génétique), Humains, Lymphangiogenèse (génétique), Lymphoedème (), Lymphoedème (diagnostic), Lymphoedème (génétique), Malformations multiples (), Malformations multiples (diagnostic), Malformations multiples (génétique), Membres, Nourrisson, Nouveau-né, Pronostic, Protéines suppresseurs de tumeurs (génétique), Protéines à homéodomaine (génétique), Récepteur-3 au facteur croissance endothéliale vasculaire (génétique), Syndrome.
- MESH :
- diagnostic : Lymphoedème, Malformations multiples.
- génétique : Facteurs de transcription Forkhead, Facteurs de transcription SOX-F, Lymphangiogenèse, Lymphoedème, Malformations multiples, Protéines suppresseurs de tumeurs, Protéines à homéodomaine, Récepteur-3 au facteur croissance endothéliale vasculaire.
- Adulte, Analyse de mutations d'ADN, Diagnostic différentiel, Humains, Lymphoedème, Malformations multiples, Membres, Nourrisson, Nouveau-né, Pronostic, Syndrome.
English descriptors
- KwdEn :
- Abnormalities, Multiple (diagnosis), Abnormalities, Multiple (genetics), Abnormalities, Multiple (therapy), Adult, DNA Mutational Analysis, Diagnosis, Differential, Extremities, Forkhead Transcription Factors (genetics), Homeodomain Proteins (genetics), Humans, Infant, Infant, Newborn, Lymphangiogenesis (genetics), Lymphedema (classification), Lymphedema (diagnosis), Lymphedema (genetics), Lymphedema (therapy), Prognosis, SOXF Transcription Factors (genetics), Syndrome, Tumor Suppressor Proteins (genetics), Vascular Endothelial Growth Factor Receptor-3 (genetics).
- MESH :
- chemical , genetics : Forkhead Transcription Factors, Homeodomain Proteins, SOXF Transcription Factors, Tumor Suppressor Proteins, Vascular Endothelial Growth Factor Receptor-3.
- classification : Lymphedema.
- diagnosis : Abnormalities, Multiple, Lymphedema.
- genetics : Abnormalities, Multiple, Lymphangiogenesis, Lymphedema.
- therapy : Abnormalities, Multiple, Lymphedema.
- Adult, DNA Mutational Analysis, Diagnosis, Differential, Extremities, Humans, Infant, Infant, Newborn, Prognosis, Syndrome.
Abstract
Limb lymphedema is frequent and not well-known. Clinical classification distinguishes primary lymphedemas due to developmental disorders of the lymphatic system (hereditary or not, sometimes associated with other malformations) and secondary lymphedemas. Primary lymphedema is a lymphedema without a cause to explain lymphatic impairment. It is due to an abnormal lymphangiogenesis in utero. It is often associated with mutation in a gene involved in lymphangiogenesis (FOX C2, VEGFR 3, SOX18, PROX 1…). To assess clinical diagnosis, non-invasive techniques are able to study structure and function of the lymphatic system (mainly isotopic lymphography). Treatment is the complex decongestive therapy which associates manual lymphatic drainage and bandage. Predisposing or precipitating factors have to be treated (particularly streptococcal infections). Surgical treatment has precise and rare indication.
DOI: 10.1016/j.lpm.2010.09.010
PubMed: 21035299
Affiliations:
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pubmed:21035299Le document en format XML
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<term>Abnormalities, Multiple (therapy)</term>
<term>Adult</term>
<term>DNA Mutational Analysis</term>
<term>Diagnosis, Differential</term>
<term>Extremities</term>
<term>Forkhead Transcription Factors (genetics)</term>
<term>Homeodomain Proteins (genetics)</term>
<term>Humans</term>
<term>Infant</term>
<term>Infant, Newborn</term>
<term>Lymphangiogenesis (genetics)</term>
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<term>Lymphedema (diagnosis)</term>
<term>Lymphedema (genetics)</term>
<term>Lymphedema (therapy)</term>
<term>Prognosis</term>
<term>SOXF Transcription Factors (genetics)</term>
<term>Syndrome</term>
<term>Tumor Suppressor Proteins (genetics)</term>
<term>Vascular Endothelial Growth Factor Receptor-3 (genetics)</term>
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<term>Analyse de mutations d'ADN</term>
<term>Diagnostic différentiel</term>
<term>Facteurs de transcription Forkhead (génétique)</term>
<term>Facteurs de transcription SOX-F (génétique)</term>
<term>Humains</term>
<term>Lymphangiogenèse (génétique)</term>
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<term>Lymphoedème (diagnostic)</term>
<term>Lymphoedème (génétique)</term>
<term>Malformations multiples ()</term>
<term>Malformations multiples (diagnostic)</term>
<term>Malformations multiples (génétique)</term>
<term>Membres</term>
<term>Nourrisson</term>
<term>Nouveau-né</term>
<term>Pronostic</term>
<term>Protéines suppresseurs de tumeurs (génétique)</term>
<term>Protéines à homéodomaine (génétique)</term>
<term>Récepteur-3 au facteur croissance endothéliale vasculaire (génétique)</term>
<term>Syndrome</term>
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<term>Homeodomain Proteins</term>
<term>SOXF Transcription Factors</term>
<term>Tumor Suppressor Proteins</term>
<term>Vascular Endothelial Growth Factor Receptor-3</term>
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<term>Lymphedema</term>
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<term>Malformations multiples</term>
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<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Abnormalities, Multiple</term>
<term>Lymphangiogenesis</term>
<term>Lymphedema</term>
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<term>Facteurs de transcription SOX-F</term>
<term>Lymphangiogenèse</term>
<term>Lymphoedème</term>
<term>Malformations multiples</term>
<term>Protéines suppresseurs de tumeurs</term>
<term>Protéines à homéodomaine</term>
<term>Récepteur-3 au facteur croissance endothéliale vasculaire</term>
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<keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Abnormalities, Multiple</term>
<term>Lymphedema</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>DNA Mutational Analysis</term>
<term>Diagnosis, Differential</term>
<term>Extremities</term>
<term>Humans</term>
<term>Infant</term>
<term>Infant, Newborn</term>
<term>Prognosis</term>
<term>Syndrome</term>
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<term>Analyse de mutations d'ADN</term>
<term>Diagnostic différentiel</term>
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<term>Lymphoedème</term>
<term>Malformations multiples</term>
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<front><div type="abstract" xml:lang="en">Limb lymphedema is frequent and not well-known. Clinical classification distinguishes primary lymphedemas due to developmental disorders of the lymphatic system (hereditary or not, sometimes associated with other malformations) and secondary lymphedemas. Primary lymphedema is a lymphedema without a cause to explain lymphatic impairment. It is due to an abnormal lymphangiogenesis in utero. It is often associated with mutation in a gene involved in lymphangiogenesis (FOX C2, VEGFR 3, SOX18, PROX 1…). To assess clinical diagnosis, non-invasive techniques are able to study structure and function of the lymphatic system (mainly isotopic lymphography). Treatment is the complex decongestive therapy which associates manual lymphatic drainage and bandage. Predisposing or precipitating factors have to be treated (particularly streptococcal infections). Surgical treatment has precise and rare indication.</div>
</front>
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