| Objective: 1, To investigate the relation between the location of papilla of Vater and the length of common channel in patients of pancreaticobiliary diseases with pancreaticobiliary maljunction (PBM). 2, To observe different characteristics of clinical manifestation and hepatic fibrosis in congenital biliary dilation (CBD) between infants and older child, and to discuss the role of PBM in formation of hepatic fibrosis.3, To find out the effects of PBM on the bile using capillary reversed-phase liquid chromatography (cRPLC). 4, To determine the isoelectric points (pI) and molecular masses (MS) of amylase isoforms in the bile of patients with CBD companied by PBM.Methods: 1, Seventy-four PBM cases including 64 children with congenital biliary dilation (CBD) and 10 adults with pancreaticobiliary diseases other than CBD were investigated. Other 30 adults suffered from comparable diseases but without PBM were used as controls. The cases whose papilla of Vater opened at the middle third of the second part of duodenum were defined as normal group, and those with the papilla distal to middle third were defined as ectopic group,to find out the difference between them. For standardization of measurements from the films of ERCP, the length index was introduced as the length divided by the height of lumbar vertebra. 2, Forty-eight children with choledochal cysts were divided into infantile (n=12) and older children (n=36) groups, clinical data including cholangiopancreatography were recorded. Semiquantitive scoring systems were used to liver fibrosis by the evaluation in HE sections, proliferation of periportal ductule by the staining with antibodies to the Cytokeratin (AE1/AE3), and infiltration of inflammatery cells by staining with antibodies to the HLA-DR Immunohistochemistry studies were also done in 18 cases of biliary atresia as positive controls and 14 live autopsy as negitive controls. 3, We analysed the amylase and trypsin components in the juices of pancreases, hepatic duct and common bile duct in patients with PBM using c-RPLC. 4, We seperated and depurated amylase in serum of normal and bile of PBM patients using cellulose acetate membrane electrophoresis, then analysed the pI and MS of amylase isoformsby two-dimension electrophoresis.Results: 1, In the children with CBD, the incidences of papilla of Vater located at the middle third, distal third of the second part of duodenum, the joint of second and third parts, and the third part of duodenum were 42 %, 22 %, 27 % and 9 %, respectively. The length index of common channel in ectopic group was significantly higher than that in normal group (0.89 ±0.42 v 0.66 ±0.31, p=0.03). The incidence of ectopic papilla of Vater in adult patients with PBM was higher than in those without PBM (8/10 v 2/30, p=0.03). 2, The rate of jaundice appearance in infants (12/12) was significantly higher than that of older children (8/36), P<0.01. The rate of PBM was lower in infant (2/5) than that in child group (12/14) .The grade of liver fibrosis, AE1/AE3 positive periportal ductule proliferation in infants were higher than those in negirive congtrols and in older children, P<0.05. The the grade of hepatic fiberosis. had positive correlations with jaundice and scores of AE1/AE3 express in periportal ductile. 3, The elution phas of trypsinogen and amylase were small and single in pancreatic juice at nonsecretion phrase,almost none in bile juice in hepatic duct, large and multiple in bile juice of CBD. 4, There were about six amylase isoforms in the bile juice of CBD with PBM, which had almost the same MS of 55KD but different pI ranging from 6.0 to 7.2.Conclusions: 1, There is a significantly higher incidence of ectopic of Vater in patients with PBM than in those without PBM. The more distal the location of the papilla of Vater, the longer the common channel of PBM. 2, Infantile choledohal cyst develop hepatic fiberosis more frequently than those of older children. Bile duct obstruction and proliferation of periportal ductule may play an important rol...
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