Exocrine pancreatic insufficiency
- Tyler Stevens, MD
Tyler Stevens, MD
- Assistant Professor of Medicine
- Department of Gastroenterology and Hepatology
- Cleveland Clinic
- Darwin L Conwell, MD, MS
Darwin L Conwell, MD, MS
- Professor of Medicine
- Division of Gastroenterology, Hepatology, and Nutrition
- The Ohio State University
Exocrine pancreatic insufficiency is an under recognized complication of pancreatic disease [1-3]. While patients with advanced exocrine pancreatic insufficiency usually present with abdominal pain and steatorrhea, those with less severe insufficiency may only have mild symptoms. This topic will review the etiology, clinical manifestations, diagnosis, and management of exocrine pancreatic insufficiency. An overview of nutrient absorption, malabsorption, and the evaluation and management of exocrine pancreatic insufficiency in cystic fibrosis are discussed in detail separately. (See "Clinical features and diagnosis of malabsorption" and "Mechanisms of nutrient absorption and malabsorption" and "Overview of the treatment of malabsorption" and "Cystic fibrosis: Assessment and management of pancreatic insufficiency" and "Cystic fibrosis: Overview of gastrointestinal disease".)
NORMAL PANCREATIC PHYSIOLOGY
The pancreas secretes approximately 1.5 liters of enzyme-rich fluid every day for the digestion of fats, starch, and protein. Normal pancreatic juice is clear, colorless, isotonic, and alkaline. The composition of pancreatic juice differs in basal and stimulated phases. In the fasting state, pancreatic fluid is protein-rich and has a bicarbonate concentration of 80 mEq/L. Stimulation of the pancreas after a meal increases the flow of water and the bicarbonate concentration of pancreatic juice. A large volume of alkaline, enzyme-rich fluid enters the duodenum to neutralize gastric chyme for optimal digestion.
Pancreatic secretion is controlled by hormonal and neuronal mechanisms. The principal regulatory hormones are secretin and cholecystokinin (CCK). Both are tightly regulated by negative feedback mechanisms. Secretin is released from the duodenal mucosa in response to the presence of acid in the duodenum (figure 1). Secretin primarily stimulates the release of bicarbonate and water from the interlobular duct cells and causes a gradual rise in the flow of pancreatic fluid through the ducts and a typical pattern of electrolyte secretion (figure 2). As bicarbonate concentration rises to a peak concentration of 120 mEq/L, chloride concentration decreases reciprocally to maintain isotonicity. CCK is released from gut endocrine cells in response to the entry of fat and protein into the proximal intestine (figure 3). CCK acts directly and through vagal afferents to stimulate pancreatic acinar cells to release digestive proenzymes. (See "Mechanisms of nutrient absorption and malabsorption", section on 'Fat absorption' and "Physiology of cholecystokinin" and "Secretin".)
ETIOLOGY AND PATHOGENESIS
Several conditions are associated with exocrine pancreatic insufficiency.
Chronic pancreatitis — Chronic pancreatitis is the most common cause of exocrine pancreatic insufficiency in adults. Progressive inflammatory changes in the pancreas in chronic pancreatitis results in permanent structural damage, which can lead to impairment in exocrine function of pancreatic duct and acinar cells. (See "Etiology and pathogenesis of chronic pancreatitis in adults", section on 'Pathogenesis'.)
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- NORMAL PANCREATIC PHYSIOLOGY
- ETIOLOGY AND PATHOGENESIS
- Chronic pancreatitis
- Cystic fibrosis
- Gastric, pancreatic, or small bowel resection
- Pancreatic duct obstruction
- Shwachman-Diamond syndrome
- CLINICAL MANIFESTATIONS
- Asymptomatic/mild symptoms
- Moderate to severe symptoms
- LABORATORY FINDINGS
- IMAGING FINDINGS
- DIFFERENTIAL DIAGNOSIS
- Diagnostic approach
- Indirect pancreatic function tests
- - Fecal elastase-1
- - Fecal chymotrypsin
- - Serum trypsinogen
- Direct pancreatic function tests
- - Secretin test
- - CCK test
- - Secretin-CCK test
- - Other
- ESTABLISHING THE ETIOLOGY
- SUMMARY AND RECOMMENDATIONS