Document Detail


Jugular lymphatic maldevelopment in Turner syndrome and trisomy 21: different anomalies leading to nuchal edema.
MedLine Citation:
PMID:  18421024     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Increased nuchal translucency (NT), morphologically known as nuchal edema, is an ultrasound marker for aneuploidy. Turner syndrome presents with massive NT, called cystic hygroma. Conflicting data exist as to whether cystic hygroma and increased NT are different entities. Both are associated with jugular lymphatic distension. The authors investigated jugular lymphatics of trisomy 21, Turner syndrome, and normal karyotype fetuses. Fetuses were investigated using immunohistochemistry for blood vascular, lymphatic, and smooth muscle cell markers. Trisomy 21 fetuses showed nuchal cavities within the mesenchymal edema negative for endothelial markers. These were extremely large in Turner fetuses, showing similar characteristics. The skin showed numerous dilated lymphatics in the case of trisomy 21 and scanty small lymphatics in Turner fetuses. A jugular lymphatic sac was present in control and trisomy 21 fetuses and was enlarged in trisomy 21 cases. In Turner fetuses, no jugular lymphatic sac was observed. Nuchal edema in trisomy 21 and Turner syndrome appears to be a similar entity caused by different lymphatic abnormalities.
Authors:
Mireile N Bekker; Nynke M S van den Akker; Yolanda M de Mooij; Margot M Bartelings; John M G van Vugt; Adriana C Gittenberger-de Groot
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Reproductive sciences (Thousand Oaks, Calif.)     Volume:  15     ISSN:  1933-7205     ISO Abbreviation:  Reprod Sci     Publication Date:  2008 Apr 
Date Detail:
Created Date:  2008-04-18     Completed Date:  2008-05-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101291249     Medline TA:  Reprod Sci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  295-304     Citation Subset:  IM    
Affiliation:
Department of Obstetrics and Gynecology, VU University Medical Center, Amsterdam, the Netherlands.
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MeSH Terms
Descriptor/Qualifier:
Down Syndrome / complications*,  embryology,  genetics,  pathology
Edema / embryology*,  genetics,  pathology
Endothelium, Lymphatic / abnormalities
Female
Gestational Age
Humans
Immunohistochemistry
Karyotyping
Lymphangioma, Cystic / embryology*,  genetics,  pathology
Lymphatic Vessels / abnormalities*,  pathology
Myocytes, Smooth Muscle / pathology
Neck / embryology*,  pathology
Nuchal Translucency Measurement
Phenotype
Pregnancy
Turner Syndrome / complications*,  embryology,  genetics,  pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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