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Werner-Morrison Syndrome
Last reviewed: 23.04.2024
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Werner-Morrison syndrome is a disease that manifests itself as severe water-resistant diarrhea, hypokalemia, and gastric chlorhydria or hypochlorhydria and is also called WDHA or WDHH syndrome (Hypokalemia Achlorhydria, Hypochlorhydria). Because of its significant similarity with cholera, one more synonym is used - "pancreatic cholera".
Causes of Werner-Morrison Syndrome
The picture of the disease was first described by Morrison in 1958. In most cases (90%) the syndrome is caused by a hormone-producing pancreatic tumor, in 5-10% the tumor has extra-pancreatic localization. With extrapancreatic arrangement, the tumor is predominantly a hormone-producing ganglionic or ganglionic fibroblast. Somewhat more often (60%) benign tumors are found.
Approximately 80% of patients in tumor tissue and in plasma find high concentrations of VIP. In these cases, the tumor is also called the vipoma. In 20% of patients, Werner-Morrison syndrome is caused by the production of a non-VIP aphoma, and PP or also prostaglandin E, whose spectrum of actions is very similar to the effects of VIP.
Symptoms of Werner-Morrison Syndrome
The leading symptom of the disease is massive watery diarrhea. The loss of water per day reaches 4-6 and even 8-10 liters. Only in 20% of cases the volume of stool is less than 3 liters per day. Due to dehydration of the body, patients quickly lose body weight. Together with water, potassium and sodium are excreted from the body. As a result, hypokalemia, metabolic acidosis and hypohydration develop, which in turn can lead to cardiovascular, renal insufficiency. Diarrhea is often accompanied by pain in the abdomen. It is caused by the influence of VIP on the water-sodium flow in the small intestine - instead of absorbing water and electrolytes, it causes their secretion. The effect of a polypeptide, like that of cholera vibrio toxins , is accomplished by stimulating adenylate cyclase of cell membranes. A 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 in histologically unchanged gastric mucosa.
As a concomitant symptom, there may be a violation of glucose tolerance (VIP increases glycogenolysis and glucagon secretion) and hypomagnesemia, which, despite simultaneous hypercalcemia, can lead to tetany.
Often, patients with vipome detect 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 seizures of tides (the peptide produced by the tumor is a vasodilator substance, for which he received his name). The arising erythema is in part the character of the urticaria.
In connection with pronounced exciticosis 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 with a daily stool volume of at least 0.7 liters (or 0.7 kg mass). The test with fasting for 3 days (all this time the loss of water and electrolytes is compensated for by parenteral administration) does not lead to a decrease in the daily volume of stool below 0.5 l. Hypo-or achlorhydria is proven by examining gastric secretion. The final diagnosis is established when a high content of VIP in the plasma is detected. The normal concentration of VIPs requires the exclusion of elevated plasma levels of PP and prostaglandin E.
Differential diagnosis is primarily carried out between Werner-Morrison syndrome and Zollinger-Ellison syndrome. Investigation of gastric secretion (hypo- or achlorhydria at the first and hypersecretion with hyperchlorhydria - in the second) and determination in the plasma of VIP and gastrin allows it to be done.
Diarrhea is common in patients who abuse laxatives and diuretics. Serum levels of VIPs are normal.
The clinical picture, characteristic for Werner-Morrison syndrome, can be observed not only with a pancreatic tumor, but also with dysfunctional hyperplasia of insulocytes.
Increased VIP content in plasma, in addition to Werner-Morrison syndrome, is possible in patients with mesenteric infarction and with 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 recovery occurs only after a radical operation, if it is possible, which is observed in 30% of cases. With an inoperable tumor, cytostatic therapy with streptozotocin is performed. Chemotherapy can induce a remission phase within a few years. In cases of vipoma resistance to treatment with streptozotocin, primary or developing against previously successful therapy, it is often possible to keep diarrhea under control, at least temporarily, with corticosteroids (prednisolone 20 to 60 mg).
In patients with Werner-Morrison syndrome caused by a tumor producing prostaglandin E, good results are reported when treated with an inhibitor of the synthesis of prostaglandins - indomethacin (50 to 200 mg / day inwards).
In all cases, symptomatic therapy is carried out, primarily aimed at eliminating or alleviating diarrhea and its consequences - hypohydration, electrolyte disorders.