Diacono Daniela, Fagbemi Andrew, Puleston Joanne, Banerjee Indraneel
Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, UK.
BMJ Case Rep. 2012 Nov 14;2012:bcr2012006413. doi: 10.1136/bcr-2012-006413.
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.