What is it?
Pancreatic insufficiency is the inability of the
exocrine pancreas to produce and/or transport enough digestive enzymes to break down food in the intestine and to allow its absorption. It typically occurs as a result of progressive pancreatic damage that may be caused by recurrent
acute pancreatitis or by
chronic pancreatitis due to a variety of conditions. In children, for example, it is most frequently associated with
cystic fibrosis (CF).
Shwachman-Diamond Syndrome (SDS) is the second most common cause of inherited pancreatic insufficiency after CF. All patients with SDS have some degree of pancreatic insufficiency beginning in infancy. Pancreatic insufficiency can also be associated with
type 1 or autoimmune diabetes. It is less frequently but sometimes associated with
pancreatic cancer.
Pancreatic insufficiency usually presents with symptoms of malabsorption, malnutrition, vitamin deficiencies, and weight loss (or inability to gain weight in children) and is often associated with steatorrhea (loose, fatty, foul-smelling stools). Diabetes may also be present in adults with pancreatic insufficiency.
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