Pancreatic Insufficiency

 

 

Cystic Fibrosis

 

Cystic Fibrosis - Patients have an inability to secrete thick pancreatic secretions which leads to pancreatic autodigestion and progressive damage.  Impaired secretion of pancreatic lipase leads to fat malabsorption, steatorrhea and failure to thrive.  

 

 

Shwachman-Diamond Syndrome

 

Shwachman-Diamond Syndrome - Patients have exocrine pancreatic insufficiency, bone marrow failure, and metaphyseal chondroplasia.  Infants typically present with failure to thrive, foul smelling stools from steatorrhea, and invasive bacterial infections from neutropenia.