Endocrine pancreas cancer-Vipomul (WDHA syndrome)

Endocrine pancreas cancer-Vipomul (WDHA syndrome)

    * Introduction
    * Signs and symptoms
    * Diagnosis
    * Treatment
Vipomul is a rare pancreatic tumor that produces excess vasoactive intestinal peptide called substance (VIP) that causes severe watery diarrhea.
Vipomul is the leading cause of WDHA syndrome: watery diarrhea, hypokaliemia and achlorhydria (watery diarrhea, hypokalemia and aclorhidrie). Because symptoms are similar to cholera terminology suggested a third condition, the pancreatic cholera.
A small percentage of patients experience and hipercalciemie, hyperglycemia, hypochlorhydria and hot flushes. Multiendocrina neoplasia type I syndrome (MEN I) is associated in some patients. 50-75% of these tumors are malignant. 6% of vipoame appear as multiple endocrine neoplasia syndrome belonging. They are virtually intrapancreatice but can be ectopic in liver and jejunum.
Vipomului syndrome in children is caused by one or ganglioneurom ganglioneuroblastom.
The major symptom is prolonged watery diarrhea. Quantity of seats removed per day is between 1000 and 3000 ml. In 50% of patients symptom constant diarrhea, ranging in severity over the disease. Since the state quantity diarrhea patient physiologic remove salt from the body, is in clinical hypokalemia and acidosis. These changes can lead to lethargy, muscle weakness, grata, vomiting and abdominal cramps.
The doctor based his diagnosis on the patient's symptoms and the discovery of high levels of VIP in the blood. It is also necessary to locate an imaging exam vipomul.
Treatment initially consists of liquids and electrolytes lost reinlocuirea. Administration of bicarbonate to reduce acidosis. Surgery to remove tumor cure in 50% of the time if it has not spread. Surgery can be a temporary solution in improving symptoms in persons who had already been disseminating the tumor. Chemotherapy does not cure the disease.
Morbidity untreated WDHA syndrome is associated with dehydration and finally determining diselectrolitemia renal failure. Death occurs after chronic renal failure and cardiac arrest by volume depletion, acidosis and severe hypokalemia.
Pathogenesis and causes
Pathogenesis of WDHA syndrome is best explained by vasoactive intestinal peptide-knowledge about the properties of VIP. VIP sunstanta is widespread in brain tissue and gastrointestinal tract. It is secreted by non-beta pancreatic island cells in response to ingestion of food containing fat, protein and alcohol. Enter the portal circulation and is metabolized by the liver. VIP relaxes smooth muscles causes a decrease of lower esophageal sphincter pressure, gastric antrum and body relaxation and inhibition of gastric and gallbladder muscle contraction in the gut circular muscle.
Exogenous administration of VIP has many pharmacological actions including inotropic action on the heart, vasodilation, increased watery secretion and electrolyte intestinal gastrin and gastric acid inhibition and stimulation of pancreatic secretion, lipolysis and glycolysis. Occasionally patients with WDHA syndrome have high levels of peptide histidine methionine (PHM). It works on different receptors but VIP has similar actions.
Vipoamele are located in 90% of cases in the pancreas but can be found in the lymph nodes, lung, colon, liver and adrenal glands, especially in children. These tumors are usually solitary, in less than 5% of cases are multicentric. VIPomul is less than 3 cm in diameter and the revelation of the diagnosis is made in the pancreas. Approximately 60-80% of VIPoame are malignant and present metastases at diagnosis. Metastases are found most often pulmonary and renal lymph nodes in li8mfatici. Approximately 10% of neuroendocrine tumors of the gastrointestinal tract are VIPoame.
Causes:
MEN1 gene-changes -Loss nature of the heterozygous MEN1 gene locus in 93% of tumors Tumor-suppressor gene alterations p16/MTS1 Involvement of BRAF gene-frequency and k-RAS-2.