Congenital pulmonary lymphangiectasis.
Identifieur interne : 002C18 ( Main/Exploration ); précédent : 002C17; suivant : 002C19Congenital pulmonary lymphangiectasis.
Auteurs : Friedrich Reiterer [Autriche] ; Karin Grossauer [Autriche] ; Nicholas Morris [Autriche] ; Sabine Uhrig [Autriche] ; Bernhard Resch [Autriche]Source :
- Paediatric respiratory reviews [ 1526-0550 ] ; 2014.
Descripteurs français
- KwdFr :
- MESH :
- diagnostic : Lymphangiectasie, Maladies pulmonaires.
- Humains, Lymphangiectasie, Maladies pulmonaires, Nourrisson, Nouveau-né.
English descriptors
- KwdEn :
- MESH :
- classification : Lung Diseases, Lymphangiectasis.
- congenital : Lung Diseases, Lymphangiectasis.
- diagnosis : Lung Diseases, Lymphangiectasis.
- therapy : Lung Diseases, Lymphangiectasis.
- Humans, Infant, Infant, Newborn.
Abstract
Congenital pulmonary lymphangiectasis (CPL) is a rare vascular malformation causing dilated lymph vessels and disturbed drainage of lymph fluid. Based on the pathogenesis and clinical phenotype it can be classified as primary or secondary CPL. Associated genetic syndromes with or without lymphedema, familial occurrence and gene mutations have been described. In utero, it may present as non-immune hydrops with pleural effusions. At birth neonates may have respiratory failure due to chylothorax and pulmonary hypoplasia, causing very high short term mortality rates. Other cases may become symptomatic any time later in childhood or even during adult life. CPL is usually diagnosed based on the combination of clinical signs, imaging and histological findings. Open-lung biopsy is considered the gold standard for the diagnosis of CPL. Treatment is primarily supportive featuring aggressive mechanical ventilation and the management of problems associated with congenital chylothorax including chest-drainage, medium-chain triglycerides (MCT) diet, and octreotide.
DOI: 10.1016/j.prrv.2014.05.002
PubMed: 24997116
Affiliations:
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Electronic address: friedrich.reiterer@medunigraz.at.</nlm:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Division of Neonatology, Department of Paediatrics, Medical University of Graz</wicri:regionArea><wicri:noRegion>Medical University of Graz</wicri:noRegion></affiliation></author><author><name sortKey="Grossauer, Karin" sort="Grossauer, Karin" uniqKey="Grossauer K" first="Karin" last="Grossauer">Karin Grossauer</name><affiliation wicri:level="1"><nlm:affiliation>Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria.</nlm:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Division of Neonatology, Department of Paediatrics, Medical University of Graz</wicri:regionArea><wicri:noRegion>Medical University of Graz</wicri:noRegion></affiliation></author><author><name sortKey="Morris, Nicholas" sort="Morris, Nicholas" uniqKey="Morris N" first="Nicholas" last="Morris">Nicholas Morris</name><affiliation wicri:level="1"><nlm:affiliation>Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria.</nlm:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Division of Neonatology, Department of Paediatrics, Medical University of Graz</wicri:regionArea><wicri:noRegion>Medical University of Graz</wicri:noRegion></affiliation></author><author><name sortKey="Uhrig, Sabine" sort="Uhrig, Sabine" uniqKey="Uhrig S" first="Sabine" last="Uhrig">Sabine Uhrig</name><affiliation wicri:level="1"><nlm:affiliation>Institute of Human Genetics, Medical University of Graz, Austria.</nlm:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Institute of Human Genetics, Medical University of Graz</wicri:regionArea><wicri:noRegion>Medical University of Graz</wicri:noRegion></affiliation></author><author><name sortKey="Resch, Bernhard" sort="Resch, Bernhard" uniqKey="Resch B" first="Bernhard" last="Resch">Bernhard Resch</name><affiliation wicri:level="1"><nlm:affiliation>Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria; Research Unit for Neonatal Infectious Diseases and Epidemiology, Medical University of Graz, Austria.</nlm:affiliation><country xml:lang="fr">Autriche</country><wicri:regionArea>Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria; Research Unit for Neonatal Infectious Diseases and Epidemiology, Medical University of Graz</wicri:regionArea><wicri:noRegion>Medical University of Graz</wicri:noRegion></affiliation></author></analytic><series><title level="j">Paediatric respiratory reviews</title><idno type="eISSN">1526-0550</idno><imprint><date when="2014" type="published">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Humans</term><term>Infant</term><term>Infant, Newborn</term><term>Lung Diseases (classification)</term><term>Lung Diseases (congenital)</term><term>Lung Diseases (diagnosis)</term><term>Lung Diseases (therapy)</term><term>Lymphangiectasis (classification)</term><term>Lymphangiectasis (congenital)</term><term>Lymphangiectasis (diagnosis)</term><term>Lymphangiectasis (therapy)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Humains</term><term>Lymphangiectasie ()</term><term>Lymphangiectasie (diagnostic)</term><term>Maladies pulmonaires ()</term><term>Maladies pulmonaires (diagnostic)</term><term>Nourrisson</term><term>Nouveau-né</term></keywords><keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Lung Diseases</term><term>Lymphangiectasis</term></keywords><keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Lung Diseases</term><term>Lymphangiectasis</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Lung Diseases</term><term>Lymphangiectasis</term></keywords><keywords scheme="MESH" qualifier="diagnostic" xml:lang="fr"><term>Lymphangiectasie</term><term>Maladies pulmonaires</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Lung Diseases</term><term>Lymphangiectasis</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Humans</term><term>Infant</term><term>Infant, Newborn</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Humains</term><term>Lymphangiectasie</term><term>Maladies pulmonaires</term><term>Nourrisson</term><term>Nouveau-né</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Congenital pulmonary lymphangiectasis (CPL) is a rare vascular malformation causing dilated lymph vessels and disturbed drainage of lymph fluid. Based on the pathogenesis and clinical phenotype it can be classified as primary or secondary CPL. Associated genetic syndromes with or without lymphedema, familial occurrence and gene mutations have been described. In utero, it may present as non-immune hydrops with pleural effusions. At birth neonates may have respiratory failure due to chylothorax and pulmonary hypoplasia, causing very high short term mortality rates. Other cases may become symptomatic any time later in childhood or even during adult life. CPL is usually diagnosed based on the combination of clinical signs, imaging and histological findings. Open-lung biopsy is considered the gold standard for the diagnosis of CPL. Treatment is primarily supportive featuring aggressive mechanical ventilation and the management of problems associated with congenital chylothorax including chest-drainage, medium-chain triglycerides (MCT) diet, and octreotide.</div></front></TEI><affiliations><list><country><li>Autriche</li></country></list><tree><country name="Autriche"><noRegion><name sortKey="Reiterer, Friedrich" sort="Reiterer, Friedrich" uniqKey="Reiterer F" first="Friedrich" last="Reiterer">Friedrich Reiterer</name></noRegion><name sortKey="Grossauer, Karin" sort="Grossauer, Karin" uniqKey="Grossauer K" first="Karin" last="Grossauer">Karin Grossauer</name><name sortKey="Morris, Nicholas" sort="Morris, Nicholas" uniqKey="Morris N" first="Nicholas" last="Morris">Nicholas Morris</name><name sortKey="Resch, Bernhard" sort="Resch, Bernhard" uniqKey="Resch B" first="Bernhard" last="Resch">Bernhard Resch</name><name sortKey="Uhrig, Sabine" sort="Uhrig, Sabine" uniqKey="Uhrig S" first="Sabine" last="Uhrig">Sabine Uhrig</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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