Paediatric and hereditary aspects of chronic pancreatitis

被引:14
作者
Dodge, JA [1 ]
机构
[1] Singleton Hosp, Dept Child Hlth, Swansea SA2 8QA, W Glam, Wales
关键词
cystic fibrosis; hereditary pancreatitis; hyperlipidaemia; chronic pancreatitis;
D O I
10.1159/000051442
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
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.
引用
收藏
页码:49 / 59
页数:11
相关论文
共 88 条
[1]   PANCREATIC-SECRETION BY NONPARALLEL EXOCYTOSIS - POTENTIAL RESOLUTION OF A LONG CONTROVERSY [J].
ADELSON, JW ;
MILLER, PE .
SCIENCE, 1985, 228 (4702) :993-996
[2]   PANCREATITIS IN YOUNG-CHILDREN WITH CYSTIC-FIBROSIS [J].
ATLAS, AB ;
ORENSTEIN, SR ;
ORENSTEIN, DM .
JOURNAL OF PEDIATRICS, 1992, 120 (05) :756-759
[3]   DEFECTIVE ENZYME PROTEIN IN LIPOPROTEIN-LIPASE DEFICIENCY [J].
AUWERX, JH ;
BABIRAK, SP ;
FUJIMOTO, WY ;
IVERIUS, PH ;
BRUNZELL, JD .
EUROPEAN JOURNAL OF CLINICAL INVESTIGATION, 1989, 19 (05) :433-437
[4]   Premature atherosclerosis in patients with familial chylomicronemia caused by mutations in the lipoprotein lipase gene [J].
Benlian, P ;
DeGennes, JL ;
Foubert, L ;
Zhang, HF ;
Gagne, SE ;
Hayden, M .
NEW ENGLAND JOURNAL OF MEDICINE, 1996, 335 (12) :848-854
[5]  
BESS MA, 1980, JAMA-J AM MED ASSOC, V243, P246
[6]   PANCREATIC FUNCTION AND GENE DELETION-F508 IN CYSTIC-FIBROSIS [J].
BORGO, G ;
MASTELLA, G ;
GASPARINI, P ;
ZORZANELLO, A ;
DORO, R ;
PIGNATTI, PF .
JOURNAL OF MEDICAL GENETICS, 1990, 27 (11) :665-669
[7]  
BOUE A, 1986, HUM GENET, V74, P288
[8]   REGULATION OF PLASMA-MEMBRANE RECYCLING BY CFTR [J].
BRADBURY, NA ;
JILLING, T ;
BERTA, G ;
SORSCHER, EJ ;
BRIDGES, RJ ;
KIRK, KL .
SCIENCE, 1992, 256 (5056) :530-532
[9]  
Braganza JM, 1996, QJM-INT J MED, V89, P243
[10]   HYPERTRIGLYCERIDEMIA ASSOCIATED WITH DEFICIENCY OF APOLIPOPROTEIN-C-II [J].
BRECKENRIDGE, WC ;
LITTLE, JA ;
STEINER, G ;
CHOW, A ;
POAPST, M .
NEW ENGLAND JOURNAL OF MEDICINE, 1978, 298 (23) :1265-1273