hrp0089p2-p190 | Fetal, Neonatal Endocrinology and Metabolism P2 | ESPE2018

Atypical Hepatoblastoma and Wilm’s Tumour in an Infant with Beckwith-Wiedemann Syndrome and Diazoxide Resistant Congenital Hyperinsulinism

Uppal Saurabh , Senniappan Senthil , Didi Mohammad , Hayden James

Introduction: Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder that could be associated with embryonal tumours. Genotype based categorisation of patients enables better screening strategies. We present a patient with BWS who developed atypical congenital hepatoblastoma and atypical Wilms tumour in infancy.Case Report: A 2 day old infant was referred with recurrent hypoglycaemia and high intravenous glucose requirement [>12 mg/kg/min]. He ...