Verner-Morrison Syndrome

Synonyms

VIPoma, Water Diarrhea Hypokalemia

definition

The Verner-Morrison syndrome describes one malignant degeneration of the pancreas.

It's a very rare diseasewhich occurs in 1 in 1 million people each year. The typical age at which you get this disease is around 50 years.

This disease is one tumor of the neuroendocrine system (Nervous system and Endocrine system concerning). The gland cells are affected, so that the tumor can be found Adenocarcinoma (Carcinomas that affect glands) counts.
The degeneracy is in the D1 cells and leads to an altered release of a tissue hormone called "vasoactive polypeptide“.

Primarily produces the pancreas Hormones (Messenger substances) and Enzymesthat the body in splitting and thus in the digestion of the individual components of food like carbohydrates, Fats and Egg whites help.

In VIPoma there is a conversion of the D1 cells, which are responsible for the production of the "vasoactive polypeptide" (VIP), are responsible.
The change in the cell results in one unhindered and excessive distribution of the hormone VIP.

Symptoms

Since in Verner-Morrison syndrome there is an excessive release of the Hormone VIP comes and the "vasoactive polypeptide" is responsible for the release of water from the small intestine, the main symptom is diarrhea.
This is accompanied by a daily loss of fluids 4 - 6 liters.
In this context, the body also loses important things Electrolytes (Minerals). Due to the massive loss of fluid, there is also an excessive excretion of via the intestinal tract potassium.

A too low level of potassium in the blood is called Hypokalemia.

Hence the second name of the disease Water diarrhea hypokalemia, In German, the two main symptoms of the disease are already in the name: The Diarrhea (Diarrhea) and the decreased potassium levels (Hypokalemia) in blood.

The next symptom that occurs in Verner-Morrison syndrome is too little Stomach acid in gastric juice.
The activity of the vasoactive peptide leads to the stimulation of Glucagon continue to be one diabetic metabolic situation.

It can also be used for Loss of other minerals how magnesium (Hypomagnesaemia) and Phosphates (Hypophosphatemia) and an accumulation of calcium (Hypercalcemia) come in the blood.

A flush can occur in which the sick person has an aaccident-like redness gets in the face.

Due to the high loss of fluid and the associated low potassium levels in the blood it can also lead to an acute weakening of the kidney (acute renal insufficiency).

diagnosis

For diagnosis will blood removed which is based on the VIP is examined and the symptoms are key to the detection of this disease.

The potassium levels and levels of other electrolytes in the blood are also of enormous importance.

therapy

To treat the tumor, on the one hand there is the possibility of using the degenerate tissue in the pancreas Tumor encliation (Enucliation - gutting) operational to remove.

If it is not possible to remove the tumor by means of surgery, this is the case, for example, when the tumor Has already metastasized in the body, there is still the possibility of the symptoms of the disease medicinal to treat.

Medicines such as Ocreotide and Streptozotocin.
Ocreotide is a drug which, in its chemical structure, is the body's own hormone Somatostatin (Growth hormone) and thus similar to how this hormone works in the human body.
Somatostatin inhibits the effects of Pancreatic hormones and thus helps to inhibit the effect of the vasoactive peptide in VIPom.

Streptozotocin is one of the Glucosamines and has an inhibitory effect on cells in the pancreas and thus inhibits the excessive release of the "vasoactive peptide" and thus alleviates the symptoms.

A third variant of treatment is chemotherapy, a good way to treat the severe symptoms of already metastatic VIPoma and to prevent the growth and further spread of the tumor.

forecast

In terms of prognosis, this is a disease which, due to its serious symptoms, is definitely fatal can run.
Otherwise it is possible to come across these symptoms in the hospital and if there are no metastases (in 50% of cases), this can tumor be treated well by surgery.