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IJCBR|ISSN: 2521-0394
Case ReportsPeer-reviewedOpen Access · CC BY 4.0

A VERY INFREQUENT ASSOCIATION OF WILLIAM-BEURAN SYNDROME AND TETRALOGY OF FALLOT

Banashankari S Kollur1, M S Mulimani2, Timmanna Giraddi3, Bomman J V4, Shashank Gowda5, Anupama Patil6, Sushmita Managuli7

  1. 1 (corresponding author)
  2. 2
  3. 3
  4. 4
  5. 5
  6. 6
  7. 7

Published 2017-04-18 · pp. 24-26

Abstract

WB-S Autosomal Dominant Disorder is the most common genetic disorder. We report a case of 20 year old with infrequent association of WBS and TOF. Clinical examination and ECHO confirmed TOF, WB-S was suspected based on the clinical signs used in the scoring system of WB-S which were described by AAP(2001), FISH study was performed in this patient because of having more than 3 clinical signs of WB-S and FISH study showed 7q11.23 deletion and remains the gold standard laboratory investigation for WB-S.

KEYWORDS: Tetralogy of Fallot; William Beuren Syndrome; Clinical Diagnosis; Fluroscence In Situ Hybridisation.