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A novel mutated sequence in the T-box transcription factor-5 (TBX-5) gene (c.241A>T) in Holt-Oram syndrome.
Ersoy, Ali Özgür; Topçu, Vehap; Kale, Ibrahim; Ersoy, Ebru; Özler, Sibel; Danisman, Nuri.
Afiliación
  • Ersoy AÖ; Clinic of Perinatology, Zekai Tahir Burak Women's Health Care Training and Research Hospital, Ankara, Turkey.
  • Topçu V; Clinic of Medical Genetics, Zekai Tahir Burak Women's Health Care Training and Research Hospital, Ankara, Turkey.
  • Kale I; Clinic of Obstetrics and Gynecology, Sar Hospital, Rize, Turkey.
  • Ersoy E; Clinic of Perinatology, Zekai Tahir Burak Women's Health Care Training and Research Hospital, Ankara, Turkey.
  • Özler S; Clinic of Perinatology, Zekai Tahir Burak Women's Health Care Training and Research Hospital, Ankara, Turkey.
  • Danisman N; Clinic of Perinatology, Zekai Tahir Burak Women's Health Care Training and Research Hospital, Ankara, Turkey.
J Turk Ger Gynecol Assoc ; 17(1): 55-7, 2016.
Article en En | MEDLINE | ID: mdl-27026770
We report a case of a 31-year-old pregnant woman who was admitted to our perinatology outpatient clinic because of a fetal ventricular septal defect and limb reduction in the upper extremities of fetus revealed by ultrasonographic investigation diagnosed in the 16(th) week of gestation. First child of the family was diagnosed with Holt-Oram syndrome who had atrial septal defect and upper limb anomalies, whereas the father was documented to have arrhythmia and shortening of upper limbs. The pregnancy was terminated in the 16(th) week of gestation with the consent of the family. We performed mutation analysis in T-box transcription factor-5 (TBX5) gene coding exons, including exon/intron boundaries from peripheral blood or skin fibroblasts. The sequence analysis revealed c.241 adenine (A)>thymine (T) [p. arginine (Arg) 81 Tryptophan (Trp)] alteration in exon-3 of the TBX5 gene in affected family members and fetus. This is a novel mutation causing Holt-Oram syndrome.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Turk Ger Gynecol Assoc Año: 2016 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Turquía

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: J Turk Ger Gynecol Assoc Año: 2016 Tipo del documento: Article País de afiliación: Turquía Pais de publicación: Turquía