TBX1

(redirected from DORV)
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TBX1

A gene on chromosome 22q11.21 that encodes a DNA-binding member of the phylogenetically conserved family of genes that have a common DNA-binding domain, the T-box. T-box genes encode transcription factors involved in regulating development.
 
Molecular pathology
TBX1 deletions are associated with DiGeorge/velocardiofacial syndrome, which is characterised by neural-crest developmental defects.
References in periodicals archive ?
11 Findings vary greatly in the cases of DORV and the management and treatment of different cases are tailored according to the specific requirements.
ConclusionDespite the rarity DORV should be considered in the differential diagnoses of patients presenting with generalised cyanosis since birth.
Correction of VSD type DORV was not difficult when an intracardiac channel procedure was performed, and the results were generally satisfactory.
There were 19 patients with TGA-type DORV whom underwent arterial switch operations in this group.
DORV is associated with varying degrees of AO; however, operative mortality did not appear to be related to this.
Except for TOF type, which usually has lower PAP, DORV is a group of complex congenital heart abnormalities that is initially associated with excessive pulmonary blood flow and frequent recurrent pulmonary infections.
sup][12],[13],[14],[15] Therefore, according to our observation, it was safe for patients with DORV and PH to receive surgical treatment between 1 and 3 years old.
Correction of VSD type DORV is not difficult when an intracardiac channel procedure is performed, with generally good results.
VSD type of DORV is usually associated with PH; the operative results are acceptable in this group.
However, the operative treatment of DORV with remote VSD is more difficult.
All our patients under 3 years of age (including infants) with TGA type DORV underwent an arterial switch procedure, with satisfactory results.
Pathological changes in patients with DORV are varied, necessitating an individualized surgical strategy to optimize success.