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J Pediatr. 1994 Sep;125(3):406-8.

Pathophysiology of the pancreatic defect in Johanson-Blizzard syndrome: a disorder of acinar development.

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Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.


We compared pancreatic acinar and ductal secretion in two patients with Johanson-Blizzard syndrome, age-matched control subjects, and patients with other primary pancreatic diseases. Patients with Johanson-Blizzard syndrome had preservation of ductular output of fluid and electrolytes, as in patients with Shwachman syndrome but differing from those with cystic fibrosis, who have a primary ductular defect. They also had decreased acinar secretion of trypsin, colipase and total lipase, and low serum immunoreactive trypsinogen levels, consistent with a primary acinar cell defect.

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