MEN/Hypoglycemia Flashcards
Multiple Endocrine Neoplasia
general
Also known as Familial Endocrine Adenomatosis
Rare group of autosomal dominant inherited syndromes that cause a predisposition to the development of hormone-producing tumors in two or more different endocrine glands
Tumors can be benign or malignant
Multiple Endocrine Neoplasia (MEN)
types
MEN 1
MEN 2A
MEN 2B
MEN 4
Major forms are type 1, 2, and 4
MEN 1
general
Develop tumors, or excessive growth and activity, of two or more of the following glands:
Parathyroid glands
Pancreas
Pituitary gland
Thyroid gland (less often affected)
Adrenal glands(less often affected)
PPP
MEN 1
almost all have
Almost all patients have tumors of the parathyroid glands
Most of the tumors are noncancerous
Hyperparathyroidism is usually the first clinical manifestation (kidney stones)
MEN 1
Other common tumors
Gastrinomas
Secrete excessive amounts of gastrin → stimulates overproduction of stomach acid (peptic ulcers with bleeding/perforation)
Insulinomas
Secrete insulin (hypoglycemia)
Tumor with the highest frequency for malignancy
Pituitary adenomas (secretory or non-secretory)
Lipomas and facial angiofibromas (non-endocrine)
MEN 2A
general
Develop tumors or excessive growth and activity in two or three of the following glands:
Adrenal glands
Thyroid gland
Parathyroid glands
Pheochromocytoma
Overactive parathyroid glands → hypercalcemia (kidney stones)
ATP
MEN 2A
Almost everyone develops
medullary thyroid cancer
Patients with MEN and diabetes should NOT receive therapy with a glucagon-like peptide 1 (GLP-1) agonist because it may increase the risk for development of medullary thyroid cancer
MEN 2A
Other common tumors
Pheochromocytomas
Neuroendocrine tumor of the adrenal glands that secretes catecholamine hormones → increased adrenaline (high blood pressure, tachycardia, headache, sweating)
Often bilateral and noncancerous
MEN 2B
General
Associated with Marfan-like habitus
Thin-build, long extremities, loose joints
Development of:
Medullary thyroid cancer
Develop at an early age, grow faster, and spread more rapidly than those in MEN 2A
Pheochromocytomas
Neuromas (mucous membranes)
Glistening bumps around the lips, tongue, and lining of the mouth
Eyelids and lips may thicken, and the lips may turn inside out (become everted)
MEN 4
general
Most recently discovered type
Exhibits signs and symptoms similar to type 1, but is caused by a different gene mutation
Development of:
Parathyroid adenoma → hyperparathyroidism
Pituitary adenoma
Pancreatic neuroendocrine tumor
Gonadal, renal, and/or adrenal tumors
MENs
Dx
Genetic testing: patient and family members
Hormone levels (blood and urine)
Imaging studies to access tumors
MENs
Tx
No cure for any type
Tumor removal if possible
Total thyroidectomy for all those at risk for medullary thyroid cancer
Hypoglycemia
general
Medical emergency in which blood glucose levels are lower than normal (< 70 mg/dL)
Common in patients with diabetes (type I > type 2)
Often the result of inadequate oral intake with insulin administration
After 5 years with diabetes, patients lose their glucagon response (glucagon: ↑ glucose in the blood)