LymphedemaV1, PubMed, Curation, bibRecord, 001451

Congenital pulmonary lymphangiectasis.

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

Congenital 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.

RBID : pubmed:24997116

Descripteurs français

KwdFr :
- Humains, Lymphangiectasie (), Lymphangiectasie (diagnostic), Maladies pulmonaires (), Maladies pulmonaires (diagnostic), Nourrisson, Nouveau-né.
MESH :
- diagnostic : Lymphangiectasie, Maladies pulmonaires.
- Humains, Lymphangiectasie, Maladies pulmonaires, Nourrisson, Nouveau-né.

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

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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>
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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>
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Congenital pulmonary lymphangiectasis.

Congenital pulmonary lymphangiectasis.

Source :

Descripteurs français

English descriptors

Abstract

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

Links to Exploration step

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