Paediatric and hereditary aspects of chronic pancreatitis.
Cystic fibrosis is by far the commonest cause of chronic pancreatitis in children, but pancreatitis itself is only rarely its presenting feature. In this paper an hypothesis for the development of the pancreatic lesions is presented. Impaired activation of pancreatic proteases in the small intestine is perceived as the pivotal problem that leads to continual feedback release of cholecystokinin, thus, in effect, causing a chronic hyperstimulation pancreatitis with intra-acinar activation of zymogens and, when bicarbonate secretion falls, precipitation of 'Reg' and other proteins in the duct system. This position contrasts with that in hereditary pancreatitis in which a mutation in the cationic trypsinogen gene leads to a form of trypsin that resists degradation by mesotrypsin and enzyme Y. A survey of the literature suggests that oxidant stress is a plausible contributor to pancreatic injury in both these diseases and in several other conditions linked with childhood pancreatitis.
Dodge
J A
JA
Department of Child Health, Singleton Hospital, Swansea, UK. J.A.Dodge@btinternet.com
D000208:Acute Disease; D002648:Child; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000150:complications*; D006801:Humans; D006949:Hyperlipidemias / Q000150:complications*; D010195:Pancreatitis / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology