1All adrenocortical hormones are steroid compounds derived from ______
Cholesterol
2Three main hormones of the adrenal cortex are _______, _____, & _____
- Mineralocorticoids = Aldosterone
- Glucocorticoids = Cortisol
- Adrenal Androgen = Dehydroepiandrosterone
(DHEA)
3Aldosterone Promotes ______ and _______. Without aldosterone, the kidney loses excessive amounts of _____ an _____
- Sodium Reabsorption
- Potassium Excretion
- Sodium and water
4Aldosterone also affects _______ absorption in the ______, especially the ______
- sodium
- intestine
- colon
5Signs and Symptoms of Mineralocorticoid (aldosterone) Deficiency
Weight Loss
Fatigue
Nausea, Vomiting, Anorexia
Salt-Craving
Hypotension
Hyperkalemia, Hyponatremia
Metabolic Acidosis with Normal Anion Gap
6Signs and Symptoms of Mineralocorticoid (Aldosterone) Excess
Hypertension
Elevated Sodium
Low Potassium
High Calcium
Fatigue
Headache
Muscle Weakness
7Cortisol release is controlled by the secretion of _____ by the _____ gland, which is controlled by _______ hormone secreted by the ______
- ACTH
- anterior pituitary
- corticotropin-releasing hormone (CRH)
- hypothalamus
8CRH, ACTH, and cortisol secretory rates peak in in the ______; and trough in the ______
- early morning
- evening
9Cortisol stimulates ______ in the _____ and decreases ____ use in the body; leading to ____; Inhibits _____ & stimulates catabolism of _____; has _____ effects; and decreases _____ & _____
- gluconeogenesis in the liver
- glucose
- Increased blood glucose
- Protein synthesis
- muscle protein
- Anti-inflammatory
- eosinophils and lymphocytes
10Signs and Symptoms of Glucocorticoid (Cortisol) Deficiency
Hypoglycemia
Nausea, Vomiting
Fatigue
Muscle Weakness
11Signs and Symptoms of Glucocorticoid (Cortisol) Excess
Hypertension
Weight Gain
Poor Growth
Stria
Sleep Disturbance
Anxiety
Menstrual Irregularity
12The adrenal cortex continually secretes several male sex steroid hormones (androgens) including ______, ______, _______, ______ that are converted to _____; Androgen release is stimulated by _____
- DHEA, DHEA-Sulfate (DHEAS), Androstenedione, 11-Hydroxyandrostenedione
- Testosterone
- ACTH
13Congenital Adrenal Hyperplasia (CAH) is a ______ genetic condition that leads to decreased or absent ______ function in the _______ leading to abnormal ______
- recessive
- enzyme function
- adrenal steroidogenesis pathway
- steroid production
14Addison's Disease is an _____ Disease Characterized by decreased or absent production of one or all three groups of adrenal steroid hormones.
- Autoimmune
15Signs and symptoms of Addison's Disease typically develop slowly and include
Fatigue
Muscle Pain
Weight Loss
Orthostatic Hypotension
GI symptoms (Nausea, Vomiting, Mild
Diarrhea)
Growth Failure
HYPERPIGMENTATION – Over 90% of patients
16Long Term Effects of Steroids
Poor Growth
Increased Weight Gain
Decreased Bone Mineral Density
Hypertension
Adrenal Suppression
17Primary adrenal insufficiency is when there’s a problem with the adrenal glands themselves. As a result, both _____ & ____ production is deficient
cortisol and aldosterone
18Secondary adrenal insufficiency is when theres a problem with the _______ gland, which secretes insufficient ______
- Pituitary
- ACTH
19ACTH regulates _____ production
Cortisol
20Tertiary adrenal insufficiency is when there's a problem that originates with the _____ and there’s insufficient _____ secretion
- hypothalamus
- CRH
21CRH stimulates the ____ to release ____
- Pituitary
- ACTH
22Cortrosyn Stimulation Testing for ______ diagnosis; Cosyntropin is a synthetic _____; Allows assessment of ______ production under physiological stress
- Congenital Adrenal Hyperplasia (CAH)
- ACTH
- cortisol
23Cortrosyn Stimulation Testing procedure includes baseline labs- _____ & _____; Cortrosyn injection- _____ & ______; Repeat labs- _____ & _____ minute
- Cortisol & 17-Hydroxyprogesterone (CAH)
- Primary AI: 125mcg IM x 1 & Central AI: 10mcg x 1
- 30 & 60 min.
24Cortrosyn Stimulation Testing is also used to diagnose ______ in various adrenal conditions
adrenal suppression
25CAH treatment includes replacement of _____, ______, and _______ as needed
Salt, Fludrocortisone, and Hydrocortisone
26Treatment Goals of Congenital Adrenal Hyperplasia (CAH) include
Normal growth
Normal Weight gain
Normal Blood Pressure
27Adrenoleukodystrophy is an _________ genetic disorder caused by a _______, resulting in Elevated plasma and tissue concentrations of _______; leading to the progressive loss of ________, and the degradation of the ________
- X-linked recessive
- mutation in the ABCD1 gene
- very long chain fatty acids (VLCFA)
- white matter in the nervous system
- adrenal glands
28Because Adrenoleukodystrophy is an X-linked disorder, it primarily affects _____
- Males
29Adrenoleukodystrophy is diagnosed with a blood test that detects _______ and a genetic test
- VLCFAs levels
30Adrenoleukodystrophy treatment includes _______ & _________
- Hematopoietic stem cell transplantation (HSCT)
- Adrenal cortisol replacement (Glucocorticoid replacement & Hydrocortisone)
31Morning (8AM) Fasting Cortisol level: at least ____
8 -10 mcg/dL
32Stress dose steroids = Hydrocortisone _____ mg/m2/day divided _____
- 50
- TID
33Solu-Cortef Injection for management of acute adrenal crisis= _______ mg Hydrocortisone given IM
25-100
34Cushing’s syndrome is a metabolic disease resulting from ______ production; Characterized by the body's inability to _____
- Excessive cortisol
- Regulate Cortisol or ACTH production
35Cushing’s syndrome is UNUSUAL IN _____ & ______
INFANCY AND CHILDHOOD
36Cushing’s syndrome is caused by ______ or ______
- Tumor of Adrenal Gland
- Tumor of Pituitary Gland
*If overproduction is caused by a pituitary tumor, it is called CUSHING’S DISEASE
3724-Hour Urine Free Cortisol is the ________ for cushing syndrome; It is a direct reflection of ______ in the blood
- Gold-Standard of Testing
- Unbound Cortisol
38Dexamethasone is a high-potency oral steroid, given to ________
suppress the pituitary secretion of ACTH (Dexamethasone Suppression Testing)
39_____ are the study of choice for the Adrenal Glands
CT scans
40Pituitary Radiation is _____ line therapy for pituitary adenoma ; Side effect seen in > 80% of patients is ______
- Second
- Growth Hormone Deficiency
41In young children, 80% of Cushing syndrome is caused by a unilateral ________ tumor; They're often ______
- Adrenal tumor
- Malignant
42Pheochromocytoma is a Rare childhood tumor that originates in the ______ , that causes the overproduction of _______
- adrenal medulla
- catecholamines
43Peak Incidence for a Pheochromocytoma is ____ to _____ years of age
9-12
44Clinical Manifestations of a Pheochromocytoma= "Five Hs":
- Hypertension
- Headache
- Hyperhidrosis
- Hypermetabolism
- Hyperglycemia
45Which is the most appropriate way to monitor whether a patient with Addison disease is receiving the correct amount of mineralocorticoid?
Daily Weight
46Most common form of CAH causes
Low cortisol
+/- Low aldosterone
High androgens