LymphedemaV1, PubMed, Corpus, bibRecord, 001451

Congenital pulmonary lymphangiectasis.

Identifieur interne : 001451 ( PubMed/Corpus ); précédent : 001450; suivant : 001452

Congenital pulmonary lymphangiectasis.

Auteurs : Friedrich Reiterer ; Karin Grossauer ; Nicholas Morris ; Sabine Uhrig ; Bernhard Resch

Source :

Paediatric respiratory reviews [ 1526-0550 ] ; 2014.

RBID : pubmed:24997116

English descriptors

KwdEn :
- Humans, Infant, Infant, Newborn, Lung Diseases (classification), Lung Diseases (congenital), Lung Diseases (diagnosis), Lung Diseases (therapy), Lymphangiectasis (classification), Lymphangiectasis (congenital), Lymphangiectasis (diagnosis), Lymphangiectasis (therapy).
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

Links to Exploration step

pubmed:24997116

Le document en format XML

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<author><name sortKey="Reiterer, Friedrich" sort="Reiterer, Friedrich" uniqKey="Reiterer F" first="Friedrich" last="Reiterer">Friedrich Reiterer</name>
<affiliation><nlm:affiliation>Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria. Electronic address: friedrich.reiterer@medunigraz.at.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Grossauer, Karin" sort="Grossauer, Karin" uniqKey="Grossauer K" first="Karin" last="Grossauer">Karin Grossauer</name>
<affiliation><nlm:affiliation>Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Morris, Nicholas" sort="Morris, Nicholas" uniqKey="Morris N" first="Nicholas" last="Morris">Nicholas Morris</name>
<affiliation><nlm:affiliation>Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria.</nlm:affiliation>
</affiliation>
</author>
<author><name sortKey="Uhrig, Sabine" sort="Uhrig, Sabine" uniqKey="Uhrig S" first="Sabine" last="Uhrig">Sabine Uhrig</name>
<affiliation><nlm:affiliation>Institute of Human Genetics, Medical University of Graz, Austria.</nlm:affiliation>
</affiliation>
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<author><name sortKey="Resch, Bernhard" sort="Resch, Bernhard" uniqKey="Resch B" first="Bernhard" last="Resch">Bernhard Resch</name>
<affiliation><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>
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<author><name sortKey="Morris, Nicholas" sort="Morris, Nicholas" uniqKey="Morris N" first="Nicholas" last="Morris">Nicholas Morris</name>
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<author><name sortKey="Uhrig, Sabine" sort="Uhrig, Sabine" uniqKey="Uhrig S" first="Sabine" last="Uhrig">Sabine Uhrig</name>
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<author><name sortKey="Resch, Bernhard" sort="Resch, Bernhard" uniqKey="Resch B" first="Bernhard" last="Resch">Bernhard Resch</name>
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<series><title level="j">Paediatric respiratory reviews</title>
<idno type="eISSN">1526-0550</idno>
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<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>
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<keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Lung Diseases</term>
<term>Lymphangiectasis</term>
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<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>
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<keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Lung Diseases</term>
<term>Lymphangiectasis</term>
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<keywords scheme="MESH" xml:lang="en"><term>Humans</term>
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<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>
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<Title>Paediatric respiratory reviews</Title>
<ISOAbbreviation>Paediatr Respir Rev</ISOAbbreviation>
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<ArticleTitle>Congenital pulmonary lymphangiectasis.</ArticleTitle>
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<Abstract><AbstractText>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.</AbstractText>
<CopyrightInformation>Copyright © 2014 Elsevier Ltd. All rights reserved.</CopyrightInformation>
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<Keyword MajorTopicYN="N">Diagnosis</Keyword>
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<Keyword MajorTopicYN="N">Review</Keyword>
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Congenital pulmonary lymphangiectasis.

Congenital pulmonary lymphangiectasis.

Source :

English descriptors

Abstract

Links to Exploration step

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