Congenital pulmonary lymphangiectasis with chylothorax: A heterogeneous lymphatic vessel abnormality
Identifieur interne : 00CF49 ( Main/Exploration ); précédent : 00CF48; suivant : 00CF50Congenital pulmonary lymphangiectasis with chylothorax: A heterogeneous lymphatic vessel abnormality
Auteurs : Philippe Moerman [Belgique] ; Kamiel Vandenberghe [Belgique] ; Hugo Devlieger [Belgique] ; Chris Van Hole [Belgique] ; Jean-Pierre Fryns [Belgique] ; Joseph M. Lauweryns [Belgique]Source :
- American Journal of Medical Genetics [ 0148-7299 ] ; 1993-08-01.
Abstract
We report on 7 perinatal autopsy cases of primary congenital pulmonary lymphangiectasis (CPL) with bilateral chylothorax. This study demonstrates that primary CPL is often complicated by chylous pleural effusions with ensuing pulmonary hypoplasia. Conversely, CPL appears to be a constant pathological finding in spontaneous congenital chylothorax. These observations indicate a common pathogenesis for both disorders. The basic defects is not an intrinsic lung abnormality, but a developmental error of the lymphatic system resulting in a pulmonary lymphatic obstruction sequence. The cause of CPL is heterogeneous. Apparently, most cases are sporadic occurrences. We report the second instance of CPL in sibs. This indicates that some cases are genetically determined with autosomal recessive inheritance. CPL may also be part of a multiple congenital anomalies (MCA) syndrome such as Noonan, Ullrich‐Turner, and Down syndrome. © 1993 Wiley‐Liss, Inc.
Url:
DOI: 10.1002/ajmg.1320470112
Affiliations:
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This study demonstrates that primary CPL is often complicated by chylous pleural effusions with ensuing pulmonary hypoplasia. Conversely, CPL appears to be a constant pathological finding in spontaneous congenital chylothorax. These observations indicate a common pathogenesis for both disorders. The basic defects is not an intrinsic lung abnormality, but a developmental error of the lymphatic system resulting in a pulmonary lymphatic obstruction sequence. The cause of CPL is heterogeneous. Apparently, most cases are sporadic occurrences. We report the second instance of CPL in sibs. This indicates that some cases are genetically determined with autosomal recessive inheritance. CPL may also be part of a multiple congenital anomalies (MCA) syndrome such as Noonan, Ullrich‐Turner, and Down syndrome. © 1993 Wiley‐Liss, Inc.</div></front></TEI><affiliations><list><country><li>Belgique</li></country><region><li>Province du Brabant flamand</li><li>Région flamande</li></region><settlement><li>Louvain</li></settlement><orgName><li>Katholieke Universiteit Leuven</li></orgName></list><tree><country name="Belgique"><region name="Région flamande"><name sortKey="Moerman, Philippe" sort="Moerman, Philippe" uniqKey="Moerman P" first="Philippe" last="Moerman">Philippe Moerman</name></region><name sortKey="Devlieger, Hugo" sort="Devlieger, Hugo" uniqKey="Devlieger H" first="Hugo" last="Devlieger">Hugo Devlieger</name><name sortKey="Fryns, Jean Ierre" sort="Fryns, Jean Ierre" uniqKey="Fryns J" first="Jean-Pierre" last="Fryns">Jean-Pierre Fryns</name><name sortKey="Lauweryns, Joseph M" sort="Lauweryns, Joseph M" uniqKey="Lauweryns J" first="Joseph M." last="Lauweryns">Joseph M. 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