ACUTE-PANCREATITIS IN PROPIONIC ACIDEMIA

It is well known that patients with organic acidurias, aminoacidopathi es and congenital hyperammonaemias often show gastrointestinal dysfunc tion including feeding refusal, nausea, vomiting and gastro-oesophagea l and abdominal pain during episodes of acute metabolic decompensation . In some cases, signs of gastrointestinal dysfunction may also be pre sent when they are metabolically stable.More recently, acute pancreati tis, a rather uncommon condition in childhood, has been reported in pa tients with disorders of branched-chain organic acid metabolism (Kahle r et al 1994). We describe two unrelated children with propionic acida emia complicated by acute pancreatitis. To our knowledge, this is the first report of acute pancreatitis in propionic acidaemia.