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Bezafibrate to prevent relapsing pancreatitis in WAGR syndrome
Daniela Diacono1, Andrew Fagbemi, Joanne Puleston
1Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, UK.
Abstract:
Recurrent episodes of acute, severe pancreatitis are a feature of the rare WAGR congenital anomaly syndrome. Although pancreatitis is well recognised as a life-threatening association of WAGR syndrome, treatment to prevent pancreatitis is not recommended. We report the successful prevention of hypertriglyceridaemia-associated relapsing pancreatitis in a child with WAGR syndrome with Bezafibrate. In addition to dietary fat restriction, Bezafibrate may be a useful therapeutic adjunct in WAGR related relapsing pancreatitis.
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