Wermer syndrome

Multiple Endocrine Neoplasia type 1 (MEN-1) syndrome is an inherited congenital endocrine disorder characterized primarily by primary hyperparathyroidism, tumors of the endocrine pancreas, and pituitary gland tumors. The underlying cause is mutations in a gene located on chromosome 11. The estimated incidence of MEN-1 is approximately 2–20 cases per 100,000 people per year.

Treatment:
Wermer syndrome (MEN-1) is a serious condition with increased mortality. Prognosis depends on early diagnosis and treatment of the individual components. Treatment may include:

•  Surgical removal of the affected parathyroid gland(s) 
•  Administration of estrogens and bisphosphonates if indicated 
•  Use of proton pump inhibitors (e.g., omeprazole) for gastric acid management

• Primary hyperparathyroidism in 95–100% of individuals
• Osteoporosis
• Kidney stones
• Peptic ulcers of the stomach and duodenum
• Nausea, vomiting, and constipation
• High blood pressure
• Cardiac rhythm disorders
• Weakness, fatigue, and depression
• Endocrine pancreatic tumors in 30–80% of patients
• Most commonly gastrinomas and insulinomas
• Increased gastric acid secretion
• Severe, recurrent stomach ulcers with complications (e.g., bleeding, strictures)
• Abdominal pain
• Heartburn, diarrhea
• Typically hypoglycemia (low blood sugar)
• Tachycardia (increased heart rate)
• Palpitations (heart pounding)
• Tremors, nervousness, anxiety, feelings of fear, sweating, hunger
• Paresthesias – most commonly tingling around the mouth, tongue, and extremities
• Impaired concentration and thinking
• Behavioral disorders, including apathy and aggression
• Mood disorders, such as euphoria or depression
• Seizures and altered consciousness
• May progressively lead to hypoglycemic coma and death
• Pituitary adenomas in up to 25% of patients
• Most commonly prolactinomas
• In women:
• Menstrual cycle disorders
• Anovulation – absence of ovulation
• Amenorrhea – absence of menstruation
• Infertility – inability to carry a pregnancy
• Galactorrhea – production and secretion of milk outside the postpartum period
• In men:
• Reduced libido and erectile dysfunction
• Occasionally gynecomastia – enlargement of male breast tissue
• Rarely galactorrhea
• Vision problems
• Headaches
• Rarely, altered consciousness