Congenital Exocrine Insufficiency
Written by Heiko Witt
Pancreatic exocrine insufficiency is defined as a reduction in pancreatic exocrine secretion below the level that allows the normal digestion of nutrients. Congenital pancreatic exocrine insufficiency, when complete, manifests from birth with soft stools, steatorrhea, failure to thrive and hypoproteinaemia-related oedema. However, due to the large reserve capacity, clinical symptoms only appear when more than 90 % of the exocrine cells have been destroyed.
Congenital pancreatic exocrine insufficiency is a rare condition. In childhood, cystic fibrosis is the most common cause. Other causes include (hereditary) chronic pancreatitis, congenital enzyme defects, organ hypoplasia, and hereditary syndromes, such as Shwachman-Diamond syndrome, Johanson-Blizzard syndrome, Pearson syndrome, and Shteyer syndrome.
Causes of congenital exocrine insufficiency
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