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532 Vacterl Association: a new Case with Biotinidase Deficiency and Annular Pancreas
  1. RG Sezer1,
  2. G Aydemir2,
  3. A Bozaykut1,
  4. C Paketci1,
  5. S Aydinoz2
  1. 1Department of Pediatrics, Zeynep Kamil Maternity and Childrens’ Diseases Training and Research State Hospital
  2. 2Pediatrics, GATA Haydarpasa Teaching Hospital, Istanbul, Turkey

Abstract

VACTERL (V - Vertebral anomalies, A - Anal atresia, C - Cardiovascular anomalies, T - Tracheoesophageal fistula, E - Esophageal atresia, R - Renal (Kidney) and defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal dysplasia, and limb anomalies. Less frequent defects seen with VACTERL association are prenatal and postnatal growth deficiency, laryngeal stenosis, ear anomaly, large fontanels, defect of lower limb, rib anomaly, tethered cord, and defects of external genitalia. We report a case of VACTERL association who had concomitant biotinidase deficiency and annular pancreas, which has not been previously reported.

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