Causes of Exocrine Pancreatic Insufficiency Other Than Chronic Pancreatitis

Abstract

Exocrine pancreatic insufficiency (EPI), an important cause of maldigestion and malnutrition, results from primary pancreatic disease or is secondary to impaired exocrine pancreatic function. Although chronic pancreatitis is the most common cause of EPI, several additional causes exist. These include pancreatic tumors, pancreatic resection procedures, and cystic fibrosis. Other diseases and conditions, such as diabetes mellitus, celiac disease, inflammatory bowel disease, and advanced patient age, have also been shown to be associated with EPI, but the exact etiology of EPI has not been clearly elucidated in these cases. The causes of EPI can be divided into loss of pancreatic parenchyma, inhibition or inactivation of pancreatic secretion, and postcibal pancreatic asynchrony. Pancreatic enzyme replacement therapy (PERT) is indicated for the conditions described above presenting with clinically clear steatorrhea, weight loss, or symptoms related to maldigestion and malabsorption. This review summarizes the current literature concerning those etiologies of EPI less common than chronic pancreatitis, the pathophysiology of the mechanisms of EPI associated with each diagnosis, and treatment recommendations.

Clinical manifestation of Johanson-Blizzard syndrome in patient with nucleotide variants in UBR1 gene

“…Exocrine pancreatic insufficiency could be manifestation of many childhood diseases, so in the differential diagnosis, apart from JBS, the following conditions should be considered: cystic fibrosis, Shwachman-Diamond syndrome, Pearson syndrome, Jeune syndrome, pancreatic aplasia and hypoplasia, isolated enzyme deficiencies, chronic pancreatitis (hereditary and autoimmune) 9,10 . In patients with cystic fibrosis, the pancreatic juice is abnormally thick, causing its retention in the pancreatic canalicular system.…”

Effect of malnutrition on postoperative results after pancreatic resection: An entropy balancing analysis

“…Other factors responsible for the imbalance could have a controversial role. The pancreatic neoplasms, particularly the PDAC, produced structural changes in pancreatic tissue such as pancreatic fibrosis and Wirsung duct dilatation, favoring malabsorption [19]. This phenomenon could be attributable not only to mechanical obstruction of the pancreatic duct but also to the fibrosis generated by pancreatic stellate cells themselves activated by the tumor [20,21].…”

Current trends in the diagnosis of pancreatic cancer

“…In contrast, tumors of the pancreatic body tend to invade adjacent vascular structures and are more likely to cause back pain on presentation; tail tumors can often grow unimpeded due to fewer anatomical neighbors (19). Malignant obstruction of the main pancreatic duct (MPD) can result in symptoms of pancreatic enzyme insufficiency (diarrhea, flatulence, steatorrhea, and postprandial abdominal pain) and occasionally in acute pancreatitis (19,34). (37,38).…”