The pancreas--development, adaptation and malfunction in infancy and childhood.

Pancreatic development begins during the fourth week of gestation. In annular pancreas, 
the ventral pancreas fails to migrate to the right of the 
duodenum. Duplication of the pancreatic ducts is a rare entity, probably 
arising from accidental misplacement of mucosal cells from the duodenum or 
stomach during the development of the pancreatic pouches. Congenital pancreatic cysts 
may be single, multiple, unilocular or multilocular and can be associated 
with polycystic disease of the kidney, liver or spleen. Ectopic pancreas 
occurs before or during the rotation of the ventral pancreas and 
its fusion with the dorsal pancreas. Few tumours of the pancreas 
have been reported in children less than 15 years of age. 
The evaluation of patients via different pancreatic function tests is significant 
only when the results are interpreted in the light of clinical 
manifestations. Tests of exocrine pancreatic function in childhood include stool examination, 
the triolein test, blood examination, analysis of duodenal contents, the secretin-pancreozymin 
test, pancreatic scanning with selenomethionine, transduodenal pancreatogram, abdominal ultrasound and arteriography, 
and upper gastrointestinal x-ray. The most prevalent disorder of the pancreas 
in infancy and childhood is exocrine pancreatic insufficiency. Well over 96 
per cent of infants and children with pancreatic insufficiency have cystic 
fibrosis. Shwachman syndrome is the second most common cause of pancreatic 
insufficiency and is associated with neutropenia, metaphyseal dystosis, sever stunting of 
growth, eczema, and susceptibility to infections. Patients with cystic fibrosis who 
have partial pancreatic insufficiency sometimes show dissociation of different enzyme activities. 
Although pancreatitis is comparatively rare in infancy and childhood, we suspect 
that it is often overlooked. 