MEN/Hypoglycemia

Question 1

Multiple Endocrine Neoplasia

general

Answer 1

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

Question 2

Multiple Endocrine Neoplasia (MEN)

types

Answer 2

MEN 1
MEN 2A
MEN 2B
MEN 4
Major forms are type 1, 2, and 4

Question 3

MEN 1

general

Answer 3

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

Question 4

MEN 1

almost all have

Answer 4

Almost all patients have tumors of the parathyroid glands
Most of the tumors are noncancerous
Hyperparathyroidism is usually the first clinical manifestation (kidney stones)

Question 5

MEN 1

Other common tumors

Answer 5

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)

Question 6

MEN 2A

general

Answer 6

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

Question 7

MEN 2A

Almost everyone develops

Answer 7

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

Question 8

MEN 2A

Other common tumors

Answer 8

Pheochromocytomas
Neuroendocrine tumor of the adrenal glands that secretes catecholamine hormones → increased adrenaline (high blood pressure, tachycardia, headache, sweating)
Often bilateral and noncancerous

Question 9

MEN 2B

General

Answer 9

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)

Question 10

MEN 4

general

Answer 10

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

Question 11

MENs

Dx

Answer 11

Genetic testing: patient and family members
Hormone levels (blood and urine)
Imaging studies to access tumors

Question 12

MENs

Tx

Answer 12

No cure for any type
Tumor removal if possible
Total thyroidectomy for all those at risk for medullary thyroid cancer

Question 13

Question 14

Question 15

Hypoglycemia

general

Answer 15

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)

Question 16

hypoglycemia

can occur in patients without diabetes

Answer 16

Defined by clinical features and not by a defined limit for the glucose level

Occurs with eating less and exercising more, alcohol consumption, medications, hypermetabolic states like sepsis and burns, and tumors (insulinomas)

Question 17

Glucose Homeostasis
Normal range

Answer 17

Serumglucose levels - normal range of 71–99 mg/dL
Maintained by the coordinated balance between insulin, glucagon, and the sympatheticnervous system

Question 18

Glucose Homeostasis

With ↓ glucose levels
At ~80 and ~68

Answer 18

At ~80 mg/dL:insulin secretion decreases
At ~68 mg/dL:glucagon,norepinephrine, and epinephrine aresecreted

Question 19

Glucagon hormone

Answer 19

mobilizes glycogen from the liver - raising blood glucose

Question 20

Norepinephrine

Answer 20

acts systemically to cause the adrenergic symptoms of hypoglycemia, including tachycardia and diaphoresis

Question 21

Epinephrine acts on multiple organs

Answer 21

Stimulates glycogenolysis and gluconeogenesis in the liver
Decreases insulin secretion and increases glucagon secretion in pancreatic islet cells
Increaseslipolysis in adipose tissue, which provides glycerol for gluconeogenesis
Decreasesglucose uptake by muscle cells

Question 22

At ~66-58 mg/dL:

Answer 22

Growth hormone and cortisol are secreted

Increaseslipolysis in adipose tissue
Increasesketogenesis andgluconeogenesis in the liver

Question 23

Medications causing Hypoglycemia

Answer 23

Insulin
Meglitinides (repaglinide, nateglinide)
Sulfonylureas (glipizide, glimepiride, glyburide)
Beta-blockers
Ethanol
Quinolones
Salicylates
MAO inhibitors

Question 24

Answer 24

Signs and symptoms may not occur until plasma glucose concentrations drop below 55 mg/dL

Question 25

# hypoglycemia Sx

Answer 25

Initial symptoms occur with a blood glucose of ~60 mg/dL Cognitive/behavioral impairment will occur at ~50 mg/dL Low blood glucose triggers the release of epinephrine (adrenaline) → “fight-or-flight” symptoms If blood glucose levels continue to fall and the brain goes without glucose → neuroglycopenic symptoms Includes: (mild → severe) Feeling shaky/anxious Sweaty and pale (pallor) Tachycardic Light-headed Impaired vision (blurry) Numbness/tingling in the lips, tongue, or cheeks Confused Clumsiness Seizures Coma Death

Question 26

# Whipple Triad ## Footnote asscoiated with NON-diabetics

Answer 26

Triad associated with hypoglycemia in nondiabetic patients: Confirmed glucose concentration ≤ 70 mg/dL Signs and symptoms associated with hypoglycemia Resolution of symptoms after the management of hypoglycemia Nondiabetic patients who exhibit the Whipple triad require further workup to determine the etiology of a possible hypoglycemic disorder

Question 27

# hypoglycemia Dx Levels of hypoglycemia

Answer 27

Requires a reliable means of measuring blood glucose Hospital point-of-care testing Laboratory measurement Level of Hypoglycemia Level 1: 54-70 mg/dL Level 2: < 54 mg/dL Level 3: severe event characterized by altered mental status and/or physical status

Question 28

# hypoglycemia Tx

Answer 28

Mild-moderate hypoglycemia **“15-15 rule”** 1. Check blood glucose 2. Eat 15 g of a simple carbohydrate Simple carbohydrates Glucose tablets 4 oz of juice or regular soda 1 tablespoon of sugar or honey Hard candies 3. Recheck blood glucose in 15 minutes Repeat until blood sugar levels are ≥ 70 mg/dL Identify the underlying cause and make corrections as needed

Question 29

# **Severe** hypoglycemia Tx (3)

Answer 29

Amp of D50 IV (25 g of dextrose monohydrate in 50 mL of water) 1 mg of glucagon IM 3 mg of glucagon intranasally Repeat point-of-care blood glucose measurement in 15 minutes Dose may be repeated if glucose level is still low Once glucose is corrected and mental status is regained, normal oral intake should be restarted Add dextrose to maintenance fluids if the patient is unable to tolerate oral intake

Question 30

Hypoglycemia

Question 31