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Werner-Morrison syndrome
Last reviewed: 05.07.2025
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Werner-Morrison syndrome is a disease that manifests itself as severe, treatment-resistant watery diarrhea, hypokalemia, and gastric achlorhydria or hypochlorhydria and is also called WDHA or WDHH syndrome (Hypokalemia Achlorhydria, Hypochlorhydria). Due to its significant similarity to cholera, another synonym is used - "pancreatic cholera".
Causes of Werner-Morrison Syndrome
The disease picture was first described by Morrison in 1958. In most cases (90%) the syndrome is caused by a hormone-producing tumor of the pancreas, in 5-10% the tumor is extrapancreatic. In extrapancreatic location the tumor is predominantly a hormone-producing ganglioneuroma or ganglioneuroblastoma. Benign tumors are somewhat more common (60%).
Approximately 80% of patients have high concentrations of VIP in the tumor tissue and plasma. In these cases, the tumor is also called VIPoma. In 20% of patients, Werner-Morrison syndrome is caused by the production of VIPoma by the apudoma, not VIP, but PP or prostaglandin E, the spectrum of action of which is very similar to the effects of VIP.
Symptoms of Werner-Morrison Syndrome
The leading symptom of the disease is massive watery diarrhea. Water loss per day reaches 4-6 and even 8-10 liters. Only in 20% of cases is the stool volume less than 3 liters per day. Due to dehydration of the body, patients quickly lose weight. Potassium and sodium are excreted from the body along with water. As a result, hypokalemia, metabolic acidosis and hypohydration develop, which in turn can lead to the development of cardiovascular and renal failure. Diarrhea is often accompanied by abdominal pain. It is caused by the effect of VIP on the water-sodium flow in the small intestine - instead of absorbing water and electrolytes, it causes their secretion. The effect of the polypeptide, like the toxins of the cholera vibrio, is achieved by stimulating the adenylate cyclase of cell membranes. The similar mechanism of action of both factors explains the similarity of the clinical manifestations of the two diseases.
VIP, along with intestinal and pancreatic hypersecretion of water and electrolytes, causes inhibition of gastric secretion, which causes another symptom of Werner-Morrison syndrome - hypo- or achlorhydria with a histologically unchanged gastric mucosa.
As an accompanying symptom, impaired glucose tolerance (VIP increases glycogenolysis and glucagon secretion) and hypomagnesemia may occur, which, despite the simultaneously observed hypercalcemia, can lead to tetany.
Often, patients with VIPoma are found to have cholelithiasis with a large atonic gallbladder - a consequence of the relaxing effect of VIP on the smooth muscle of this organ (but not the small intestine).
Every fifth patient develops recurrent attacks of hot flashes (the peptide produced by the tumor is a vasodilator substance, for which it received its name). The resulting erythema is partly urticarial in nature.
Due to severe exsicosis and electrolyte shifts, changes equivalent to psychosis may occur.
Diagnosis of Werner-Morrison syndrome
Werner-Morrison syndrome should be suspected in the presence of diarrhea for at least 3 weeks and a daily stool volume of at least 0.7 L (or a weight of 0.7 kg). A 3-day fasting test (during which water and electrolyte losses are replaced by parenteral administration) does not result in a decrease in daily stool volume below 0.5 L. Hypo- or achlorhydria is demonstrated by studying gastric secretion. The final diagnosis is established by detecting elevated plasma VIP levels. Normal VIP concentrations require exclusion of elevated plasma PP and prostaglandin E levels.
Differential diagnostics is primarily carried out between Werner-Morrison syndrome and Zollinger-Ellison syndrome. A study of gastric secretion (hypo- or achlorhydria in the first and hypersecretion with hyperchlorhydria in the second) and determination of VIP and gastrin in plasma allows this to be done.
Diarrhea is common in patients who abuse laxatives and diuretics. Serum VIP levels are normal in these patients.
The clinical picture characteristic of Werner-Morrison syndrome can be observed not only with a pancreatic tumor, but also with diffuse hyperplasia of islet cells.
Increased VIP content in plasma, in addition to Werner-Morrison syndrome, is possible in patients with mesenteric infarction and shock. This pathology is characterized by acute development of symptoms.
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Treatment and prognosis of Werner-Morrison syndrome
Untreated patients with Werner-Morrison syndrome die within a few months. Complete cure occurs only after radical surgery, if possible, which is observed in 30% of cases. Inoperable tumors are treated with cytostatic therapy with streptozotocin. Chemotherapy can induce a remission phase for several years. In cases of resistance of the vipoma to streptozotocin treatment, either primary or developing against the background of previously successful therapy, diarrhea can often be kept under control, at least temporarily, with corticosteroids (prednisolone from 20 to 60 mg).
In patients with Werner-Morrison syndrome caused by a tumor producing prostaglandin E, good results have been described with treatment with the prostaglandin synthesis inhibitor indomethacin (50 to 200 mg/day orally).
In all cases, symptomatic therapy is also carried out, aimed primarily at eliminating or alleviating diarrhea and its consequences - hypohydration, electrolyte disturbances.