History
The major symptoms of exocrine pancreatic insufficiency (EPI) include steatorrhea and weight loss. The most common symptomatic complaint is steatorrhea, which is fatty diarrhea, but sometimes the stool can be watery, reflecting the osmotic load received by the intestine.
Steatorrhea is the result of fat malabsorption and is characterized by pale, bulky, and malodorous stools. These stools often float on top of the toilet water with oily droplets and are difficult to flush.
Weight loss and fatigue are common and may be pronounced; however, patients may compensate by increasing their caloric consumption, and as a result, weight loss from malabsorption may be masked. The weight loss may be compounded by an underlying disease involving the intestine (eg, celiac disease or Crohn disease).
Flatulence and abdominal distention are caused by bacterial fermentation of unabsorbed food substances, which releases gaseous products such as hydrogen and methane. Flatulence often causes uncomfortable abdominal distention and cramps.
Edema may result from hypoalbuminemia caused by chronic protein malabsorption; loss of protein into the intestinal lumen can cause peripheral edema. With severe protein depletion, ascites may develop.
Anemia resulting from malabsorption can be either microcytic (related to iron deficiency) or macrocytic (related to vitamin B-12 deficiency). Anemia may also be associated with the underlying disease causing EPI. For instance, iron deficiency anemia is often a manifestation of celiac disease. Ileal involvement in Crohn disease or ileal resection can cause megaloblastic anemia due to vitamin B-12 deficiency.
Bleeding disorders are usually a consequence of vitamin K malabsorption and subsequent hypoprothrombinemia. Ecchymosis usually is the manifesting symptom, though melena and hematuria may occur on occasion.
Metabolic bone disease caused by vitamin D deficiency can result in osteopenia or osteomalacia. In severe cases, bone pain and pathologic fractures occur with low calcium levels leading to secondary hyperparathyroidism.
Neurologic manifestations can result from electrolyte disturbances (eg, hypocalcemia and hypomagnesemia) and can lead to tetany. Malabsorption of certain vitamins can cause generalized motor weakness (pantothenic acid and vitamin D), peripheral neuropathy (thiamine), loss of a sense of vibration and position (cobalamin), night blindness (vitamin A), or seizures (biotin).
Physical Examination
Signs of weight loss, muscle wasting, and loss of subcutaneous fat are often noted.
On abdominal examination, orthostatic hypotension may be present. The abdomen may be distended, and bowel sounds may be hyperactive. In severe hypoproteinemia, ascites may be present. Peripheral edema may be observed.
On dermatologic examination, pale skin may reflect anemia. Ecchymoses due to vitamin K deficiency may be apparent. Dermatitis herpetiformis, erythema nodosum, and pyoderma gangrenosum may be noted. Pellagra, alopecia, or seborrheic dermatitis may be present. Examination of the mouth may reveal cheilosis, glossitis, or aphthous ulcers.
On neurologic examination, motor weakness, peripheral neuropathy, or ataxia may be present. The Chvostek sign or the Trousseau sign may be noted as a consequence of hypocalcemia or hypomagnesemia.
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Complete replacement of the pancreas with cystic disease and intrahepatic biliary dilation caused by extrinsic compression of the common bile duct. Note also the renal cysts and masses. This patient had exocrine pancreatic insufficiency. Image courtesy of Wikimedia Commons.
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Residual islets in dense fibrous stroma secondary to loss of exocrine pancreatic tissue in chronic pancreatitis (hematoxylin-eosin stain, medium magnification). Image courtesy of Dr. Rose Anton.
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Pancreas anatomy.
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The duodenum and pancreas.
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The pancreas and duodenum, posterior view.
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Factors controlling release of pancreatic secretions. Image courtesy of Wikimedia Commons.
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Defective protein transmembrane conductance regulator (CFTR) in cystic fibrosis.
Tables
Pancreatic Enzyme Product |
Lipase Content, units |
Creon 1203 |
3000 |
Creon 1206 |
6000 |
Creon 1212 |
12,000 |
Creon 1224 |
24,000 |
Zenpep EURAND 3 |
3000 |
Zenpep EURAND 5 |
5000 |
Zenpep EURAND 10 |
10,000 |
Zenpep EURAND 15 |
15,000 |
Zenpep EURAND 20 |
20,000 |
Zenpep EURAND 25 |
25,000 |
Pancreaze MT 4 |
4200 |
Pancreaze MT 10 |
10,500 |
Pancreaze MT 16 |
16,800 |
Pancrease MT 24 |
21,000 |
Ultresa 13800UL |
13,800 |
Ultresa 20700UL |
20,700 |
Ultresa 23000UL |
23,000 |
Viokace 9111 |
10,440 |
Viokace 9116 |
20,880 |
Pertzye 8 |
8000 |
Pertzye 16 |
16,000 |
