There has been renewed interest in examining the multiple causes of undernutrition and growth failure in cystic fibrosis. It is now recognized that undernutrition is caused by unfavourable energy...
Colipase and maximally activated pancreatic lipase in normal subjects and patients with steatorrhea.
Gaskin, K J, Durie, P R, Hill, R E, Lee, L M, Forstner, G G
Human pancreatic lipase in duodenal secretions was studied under conditions of maximal activation by porcine colipase and maximal inhibition by sodium taurodeoxycholate. In almost all samples, total...
Segregation Analysis in Shwachman-Diamond Syndrome: Evidence for Recessive Inheritance
Ginzberg, H., Shin, J., Ellis, L., Goobie, S., Morrison, J., Corey, M., ...
Shwachman-Diamond syndrome is a rare disorder of unknown cause. Reports have indicated the occurrence of affected siblings, but formal segregation analysis has not been performed. In families...
Vitamin K and the management of patients with cystic fibrosis.
OBJECTIVE: To assess the advisability of routine vitamin K supplementation in patients with cystic fibrosis (CF). DATA SOURCES: Studies identified through a MEDLINE search with the use of MeSH terms...
Colipase and maximally activated pancreatic lipase in normal subjects and patients with steatorrhea.
Gaskin, K J, Durie, P R, Hill, R E, Lee, L M, Forstner, G G
Human pancreatic lipase in duodenal secretions was studied under conditions of maximal activation by porcine colipase and maximal inhibition by sodium taurodeoxycholate. In almost all samples, total...
Segregation Analysis in Shwachman-Diamond Syndrome: Evidence for Recessive Inheritance
Ginzberg, H., Shin, J., Ellis, L., Goobie, S., Morrison, J., Corey, M., ...
Shwachman-Diamond syndrome is a rare disorder of unknown cause. Reports have indicated the occurrence of affected siblings, but formal segregation analysis has not been performed. In families...
Vitamin K and the management of patients with cystic fibrosis.
OBJECTIVE: To assess the advisability of routine vitamin K supplementation in patients with cystic fibrosis (CF). DATA SOURCES: Studies identified through a MEDLINE search with the use of MeSH terms...
Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis.
Durie, P R, Bell, L, Linton, W, Corey, M L, Forstner, G G
Fifteen patients with cystic fibrosis and pancreatic insufficiency were studied during four randomised seven day treatment periods in which they received only pancreatic supplement (Pancrelipase, 27...
Bile acid secretion in cystic fibrosis: evidence for a defect unrelated to fat malabsorption.
Weizman, Z, Durie, P R, Kopelman, H R, Vesely, S M, Forstner, G G
In order to define basic biliary defects not related to steatorrhoea in cystic fibrosis, we studied 12 control and 18 cystic fibrosis subjects, with a wide range of pancreatic function. Duodenal...
Moore, D J, Forstner, G G, Largman, C, Cleghorn, G J, Wong, S S, Durie, P R
We evaluated serum cationic trypsinogen as a marker of exocrine pancreatic function in children without cystic fibrosis. The ability of this test to determine steatorrhoea of pancreatic origin, and...
Geokas, M. C., Largman, C., Durie, P. R., Brodrick, J. W., Ray, S. B., O'Rourke, M., ...
A canine model of bile-induced pancreatitis has been employed to investigate time-dependent changes in the molecular forms of trypsin in blood and ascitic fluid in this disease. The distribution of...
Cardiac rhabdomyomata and megacystis-microcolon-intestinal hypoperistalsis syndrome.
Couper, R T, Byard, R W, Cutz, E, Stringer, D A, Durie, P R
Multiple cardiac rhabdomyomata were discovered on necropsy tissue review of a previously well child with megacystis-microcolon-intestinal hypoperistalsis syndrome, who died unexpectedly at home at 40...
Meconium ileus in the absence of cystic fibrosis.
Fakhoury, K, Durie, P R, Levison, H, Canny, G J
Although meconium ileus in the absence of cystic fibrosis is considered a rare event, it was found that eight of 37 (21.6%) newborn infants with meconium ileus had no laboratory or clinical evidence...
Appendicitis in cystic fibrosis.
Shields, M D, Levison, H, Reisman, J J, Durie, P R, Canny, G J
Appendicitis is said to be uncommon and difficult to diagnose in cystic fibrosis. The clinical and radiological features in nine patients with cystic fibrosis who had appendicitis were studied. All...