title: Bezafibrate to prevent relapsing pancreatitis in WAGR syndrome.
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.
authors.affiliation: Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Daniela
authors.initials: D
authors.lastname: Diacono
authors.forename: Andrew
authors.initials: A
authors.lastname: Fagbemi
authors.forename: Joanne
authors.initials: J
authors.lastname: Puleston
authors.forename: Indraneel
authors.initials: I
authors.lastname: Banerjee
mesh_terms: D001629:Bezafibrate / Q000627:therapeutic use*; D002648:Child; D003131:Combined Modality Therapy; D018752:Diet, Fat-Restricted; D005260:Female; D006801:Humans; D015228:Hypertriglyceridemia / Q000150:complications / Q000188:drug therapy; D000960:Hypolipidemic Agents / Q000627:therapeutic use*; D019283:Pancreatitis, Acute Necrotizing / Q000517:prevention & control*; D011379:Prognosis; D055502:Secondary Prevention; D017624:WAGR Syndrome / Q000150:complications*
chemical_list: D000960:Hypolipidemic Agents; D001629:Bezafibrate