Primary disorders of the lymphatic vessels--a unified concept.
Identifieur interne : 008190 ( Ncbi/Checkpoint ); précédent : 008189; suivant : 008191Primary disorders of the lymphatic vessels--a unified concept.
Auteurs : C. LevineSource :
- Journal of pediatric surgery [ 0022-3468 ] ; 1989.
Descripteurs français
- KwdFr :
- Adolescent, Adulte, Ascite chyleuse (), Chylothorax (), Enfant d'âge préscolaire, Femelle, Humains, Lymphangiectasie intestinale (), Lymphangiome (), Lymphoedème (étiologie), Lymphographie, Maladies lymphatiques (), Maladies lymphatiques (diagnostic), Mâle, Nouveau-né, Système lymphatique (malformations).
- MESH :
- diagnostic : Maladies lymphatiques.
- malformations : Système lymphatique.
- étiologie : Lymphoedème.
- Adolescent, Adulte, Ascite chyleuse, Chylothorax, Enfant d'âge préscolaire, Femelle, Humains, Lymphangiectasie intestinale, Lymphangiome, Lymphographie, Maladies lymphatiques, Mâle, Nouveau-né.
English descriptors
- KwdEn :
- Adolescent, Adult, Child, Preschool, Chylothorax (surgery), Chylous Ascites (surgery), Female, Humans, Infant, Newborn, Lymphangiectasis, Intestinal (surgery), Lymphangioma (surgery), Lymphatic Diseases (complications), Lymphatic Diseases (diagnosis), Lymphatic Diseases (surgery), Lymphatic System (abnormalities), Lymphedema (etiology), Lymphography, Male.
- MESH :
- abnormalities : Lymphatic System.
- complications : Lymphatic Diseases.
- diagnosis : Lymphatic Diseases.
- etiology : Lymphedema.
- surgery : Chylothorax, Chylous Ascites, Lymphangiectasis, Intestinal, Lymphangioma, Lymphatic Diseases.
- Adolescent, Adult, Child, Preschool, Female, Humans, Infant, Newborn, Lymphography, Male.
Abstract
Congenital defects of lymphatics constitute a spectrum of disorders that may manifest with a variety of clinical presentations including lymphedema, chylous effusions, lymphangiomatous malformations with cystic masses and localized gigantism, and intestinal lymphangiectasia with malabsorption. These entities constitute a relatively rare group of disorders, the origin of which remains somewhat controversial, but in some it appears to be due to early lymphatic obstruction. Five cases are described, which demonstrate the anatomical pathology of these entities. A classification and description of the defects is also presented. An attempt is made to present a unified theory of origin for this seemingly diverse group of diseases. While these entities may be challenging from a diagnostic and therapeutic standpoint, a wide variety of imaging modalities, which includes lymphography, computed tomography scanning, and ultrasound, may be used to diagnose the extent and internal structural characteristics of the abnormalities.
PubMed: 2709285
Affiliations:
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pubmed:2709285Le document en format XML
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Child, Preschool</term>
<term>Chylothorax (surgery)</term>
<term>Chylous Ascites (surgery)</term>
<term>Female</term>
<term>Humans</term>
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<term>Adulte</term>
<term>Ascite chyleuse ()</term>
<term>Chylothorax ()</term>
<term>Enfant d'âge préscolaire</term>
<term>Femelle</term>
<term>Humains</term>
<term>Lymphangiectasie intestinale ()</term>
<term>Lymphangiome ()</term>
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<term>Maladies lymphatiques ()</term>
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<term>Mâle</term>
<term>Nouveau-né</term>
<term>Système lymphatique (malformations)</term>
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</keywords>
<keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Maladies lymphatiques</term>
</keywords>
<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Lymphedema</term>
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<keywords scheme="MESH" qualifier="malformations" xml:lang="fr"><term>Système lymphatique</term>
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<keywords scheme="MESH" qualifier="surgery" xml:lang="en"><term>Chylothorax</term>
<term>Chylous Ascites</term>
<term>Lymphangiectasis, Intestinal</term>
<term>Lymphangioma</term>
<term>Lymphatic Diseases</term>
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<keywords scheme="MESH" qualifier="étiologie" xml:lang="fr"><term>Lymphoedème</term>
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<term>Adult</term>
<term>Child, Preschool</term>
<term>Female</term>
<term>Humans</term>
<term>Infant, Newborn</term>
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<term>Adulte</term>
<term>Ascite chyleuse</term>
<term>Chylothorax</term>
<term>Enfant d'âge préscolaire</term>
<term>Femelle</term>
<term>Humains</term>
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<term>Lymphangiome</term>
<term>Lymphographie</term>
<term>Maladies lymphatiques</term>
<term>Mâle</term>
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<front><div type="abstract" xml:lang="en">Congenital defects of lymphatics constitute a spectrum of disorders that may manifest with a variety of clinical presentations including lymphedema, chylous effusions, lymphangiomatous malformations with cystic masses and localized gigantism, and intestinal lymphangiectasia with malabsorption. These entities constitute a relatively rare group of disorders, the origin of which remains somewhat controversial, but in some it appears to be due to early lymphatic obstruction. Five cases are described, which demonstrate the anatomical pathology of these entities. A classification and description of the defects is also presented. An attempt is made to present a unified theory of origin for this seemingly diverse group of diseases. While these entities may be challenging from a diagnostic and therapeutic standpoint, a wide variety of imaging modalities, which includes lymphography, computed tomography scanning, and ultrasound, may be used to diagnose the extent and internal structural characteristics of the abnormalities.</div>
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