we report the case of a boy with the johanson-blizzard syndrome who died at the age of 8 years with complications of pancreatic exocrine insufficiency , and at autopsy was found to have a small thyroid filled with colloid , virtually complete replacement of the pancreas with adipose tissue , and a brain of normal size but with evidence of a cortical developmental defect consisting of abnormalities of gyral formation and of cortical neuronal organization .
C0267963
pancreatic exocrine insufficiency
C0030274
pancreas