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Polyhydramnios as a prenatal symptom of the DiGeorge/velo-cardio-facial syndrome

✍ Scribed by Koenraad Devriendt; Dominique Van Schoubroeck; Benedicte Eyskens; Greet Vantrappen; Ann Swillen; Marc Gewillig; Monique Dumoulin; Philippe Moerman; Kamiel Vandenberghe; Jean-Pierre Fryns


Publisher
John Wiley and Sons
Year
1998
Tongue
English
Weight
37 KB
Volume
18
Category
Article
ISSN
0197-3851

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✦ Synopsis


Prenatal diagnosis of the DiGeorge/velo-cardio-facial syndrome has become possible since it was recognized that this syndrome is caused by a submicroscopic deletion in chromosome 22q11. In a sporadic patient presenting a conotruncal heart defect and polyhydramnios, the del 22q11 was made prenatally by fluorescence in situ hybridization (FISH) after amniocentesis. Seven additional patients with a del 22q11 were identified, who presented during pregnancy with polyhydramnios. In one of them, unilateral hydronephrosis was present. These findings further add to a growing list of clinical presentations of a del 22q11 and suggest that in patients with polyhydramnios and a conotruncal heart defect or uropathy, fetal karyotyping should be complemented by FISH for a del 22q11.