Elevated maternal serum α-fetoprotein level in a fetus with Beckwith-Wiedemann syndrome in the second trimester of pregnancy

BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is a rare disorder characterized by macrosomia, macroglossia, visceromegaly, and omphalocele and an increased risk of growing tumors. Prenatal and postnatal high levels of serum alpha-fetoprotein are associated with several diseases and neoplasms includi...

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Veröffentlicht in:Journal of prenatal medicine. - 2007. - 6(2012), 1 vom: 26. Jan., Seite 7-9
1. Verfasser: Guanciali-Franchi, Paolo (VerfasserIn)
Weitere Verfasser: Di Luzio, Luisa, Iezzi, Irene, Celentano, Claudio, Matarrelli, Barbara, Liberati, Marco, Palka, Giandomenico
Format: Online-Aufsatz
Sprache:English
Veröffentlicht: 2012
Zugriff auf das übergeordnete Werk:Journal of prenatal medicine
Schlagworte:Case Reports Journal Article Beckwith-Wiedemann syndrome maternal serum screening prenatal diagnosis
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245 1 0 |a Elevated maternal serum α-fetoprotein level in a fetus with Beckwith-Wiedemann syndrome in the second trimester of pregnancy 
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520 |a BACKGROUND: Beckwith-Wiedemann syndrome (BWS) is a rare disorder characterized by macrosomia, macroglossia, visceromegaly, and omphalocele and an increased risk of growing tumors. Prenatal and postnatal high levels of serum alpha-fetoprotein are associated with several diseases and neoplasms including hepatoblastomas and other hepatic tumors. The diagnosis of BWS is usually made in the postnatal period on the basis of physical exam features and hypermethylation of the H19 gene 
520 |a CASE: A 30-year-old woman gravida 3, para 2, underwent maternal serum screening at 15 weeks' gestation. The screening was negative for Down's syn drome (risk 1/6085), but positive for NTDs. Further ultrasound examination at 20 and 30 weeks' evidenced a fetal overgrowth and a 3-D scan at 33 weeks' gestation presented a protruding tongue, and a fixed opened mouth caused by macroglossia 
520 |a CONCLUSIONS: BWS was suspected on the basis of clinical features, and molecular analysis of critical region 11p15.5 revealing the hypermethylation of H19 gene supported the diagnosis 
650 4 |a Case Reports 
650 4 |a Journal Article 
650 4 |a Beckwith-Wiedemann syndrome 
650 4 |a maternal serum screening 
650 4 |a prenatal diagnosis 
700 1 |a Di Luzio, Luisa  |e verfasserin  |4 aut 
700 1 |a Iezzi, Irene  |e verfasserin  |4 aut 
700 1 |a Celentano, Claudio  |e verfasserin  |4 aut 
700 1 |a Matarrelli, Barbara  |e verfasserin  |4 aut 
700 1 |a Liberati, Marco  |e verfasserin  |4 aut 
700 1 |a Palka, Giandomenico  |e verfasserin  |4 aut 
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