Multiple endocrine neoplasia type 1(MEN Type 1) or Wermer syndrome is a part of a group of disorders that affect the endocrine system through development neoplastic lesions in pituitary, parathyroid gland and pancreas.
Age / Sex prevalance of Wermer Syndrome(MEN Type1)
The male female ratio is approximately equal.
The peak incidence of symptoms in MEN 1 is during the third decade of life in women and during fourth decade of life in men.
Individuals of any race can be affected by wermer syndrome.
It is basically a genetic disorder.
• Primary hyperparathyroidism caused by hyperplasia of parathyroid gland is most common manifestation of MEN1 OCCURING IN 90% of all patients.
• Hypercalcemia, nephrolithiasis, and bone abnormalities, Hypertension, loss of appetite, nausea, weakness,fatigue.
• Pancreatic islet cell tumor represents the second most common manifestation of MEN 1 occuring in 30-80% patients.
• Insulinoma causing hypoglycemia.
• Gastrinoma causing overproduction of acid in stomach and Zollinger-Elison syndrome.
• Glucagonoma causing hyperglycemia and a rash called necrolytic migratory erythema, weight loss.
• VIPomas causing watery diarrhea,hypokalemia and achlorhydria.
• GHRHomas causing acromegaly.
• Pituitary gland tumor manifested as Prolactinomas causing amenorrhea, galactorrhea in women, and loss of libido and erectile dysfunction in men
• For gastinomas:
An increased fasting gastrin level and increased basal gastrin acid secretion .
• For insulinomas:
Increased level of plasma insulin with hypoglycemia.
Elevated serum glucagon with hyperglycemia.
Elevated serum levels of vasoactive intestinal polypeptide.
• Pituitary tumors:
Excess growth hormone levels.
Calcium levels is elevated.
• Genetic testing.
Surgery is the choice of treatment.
• Proton pump inhibitor.
• Dopamine agonists.
• Anti hypoglycemic agents
The major complication of wermer syndrome is it lead to malignant tumor.
Untreated wermer syndrome patients have less life expectancy.,with probability of death at the age of 50 years.
• Adrenal adenomas
• Carcinoid lung tumor
• Duodenal ulcers
• Zollinger- Ellison syndrome
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