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51 Cards in this Set

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  • Back
What is the Adrenal Cortex derived from?

What is the Adrenal Medulla derived from?
Cortex = Mesoderm

Medulla = Neural Crest
Label the layers of the Adrenal Glands
List the hormone secreted from each layer of the Adrenal Gland
Glomerulosa = Aldosterone = increases Na+ reabsorption; increase K+ & H+ secretion

Fasciculata = Cortisol

Reticularis = Sex Hormones = Testosterone, DHEA, Androstenedione

Medulla = Catecholamines
What are the stimulators & inhibitors of release of each layer of the Adrenal Glands?
-Stimulators = Angiotensin II; hyperkalemia; ACTH

-Stimulator = ACTH
-Inhibitor = Cortisol

-Stimulator = ACTH
-Inhibitor = Cortisol

-Stimulator = ACh from SNS
Another name for "Acute Bilateral Hemorrhagic Adrenal Infarction"
Waterhouse-Friderichsen Syndrome
What is Waterhouse-Friderichsen Syndrome usually associated with?
Septicemia from Neisseria Meningitidis
What bacteria can cause Waterhouse-Friderichsen Syndrome?
1. N. menigitidis
2. Pneumococcus
3. Staph
4. Strep
5. Hemophilus
What virus can cause Waterhouse-Friderichsen Syndrome?
Other than infections, what are other causes of Acute Adrenocortical Insufficiency?
1. abrupt withdrawal of Corticosteroids
2. Stress
3. Surgery
What is the mechanism of Waterhouse-Friderichsen Syndrome?
release of tissue Thromboplastin due to Endotoxin causes DIC
Waterhouse-Friderichsen Syndrome due to N. menigitidis
What is this called?
Waterhouse-Friderichsen Syndrome = Acute bilateral adrenal hemorrhage
What is the name of this condition?
Waterhouse-Friderichson Syndrome = DIC
This is a Glomerulus. What adrenal condition is associated with this?
Chronic Adrenocortical insufficiency = ?
Addison Disease
What are the clinical features of Addison's Disease?
Insidious onset

Signs of decreased Glucocorticoids
-weakness & fatigue
-GI disturbances
-SKIN HYPERPIGMENTATION = due to MSH, a by-product of increased ACTH production

Decreased Alodsterone
Addison's disease = primary adrenocortical insufficiency

Low Cortisol does not produce feedback inhibition on ACTH production = high ACTH yields MSH as a by-product of ACTH production from POMC
What adrenal condition would cause this? What is the mechanism?
What are the causes of Addison's disease?
1. 60% = ?
2. 25% = ?
3. 15% = ?
1. Autoimmune destruction of Adrenal Gland = Autoimmune Adrenalitis

2. Granulomatous Adrenalitis due to TB or Histoplasmosis

3. Other = trauma, metastatic tumor, amyloidosis
Tuberculous granulomas
What would be the cause of this Addison's diseased adrenal gland?
Autoimmune Adrenalitis
What would be the cause of this Addison's diseased adrenal gland?
Addison's Disease due to Autoimmune Adrenalitis
-atrophied cortex with lymphocytic infiltration
What are these pictures showing?
What are the clinical features of Cushing Syndrome
Weight gain = due to hyperinsulinism fom hyperglycemia (due to Cortisol)
-truncal obesity, moon facies, buffalo hump

Hypertension = due to increase in weak mineralcorticoids & glucocorticoids = Na+ retention

Osteoporosis = due to high cortisol

Lymphopenia & Eosinopenia
What are the lab findings in Cushing Syndrome?
1. increased Cortisol levels

2. increased 17-hydroxycorticoids & Cortisol in urine
Cushing Syndrome
What syndrome are these clinical findings associated with?
List the causes of Cushing Syndrome
Iatrogenic = Corticosteroid therapy

Cushing Disease = ACTH-secreting Pituitary adenoma

Adrenal Cortical Adenoma/Carcinoma = decreased ACTH; increased Cortisol

Ectopic ACTH = Small Cell Carcinoma of Lung = markedly increased ACTH & Cortisol
A 45 yoa woman presents complaining of increased urination over the past several months. She has also gained 35 lbs. over the past year, bruises easily, & has grown hair on her chin. While speaking with her, you note that she has moon facies & increased fat pads on the back of her neck. Blood tests reveal hyperglycemia, increased Cortisol levels, & increased ACTH levels. Dexamethasone suppression test causes decreased Cortisol levels
Cushing Syndrome caused by Pituitary Adenoma secreting ACTH
How do you differentiate b/w the different causes of Cushing Syndrome?
Pituitary Adenoma
-Cortisol suppression with Dexamethasone suppression test
-high ACTH & Cortisol

Paraneoplastic Syndrome
-markedly high ACTH; high cortisol
-no cortisol suppression by Dexamethasone

Adrenocortical Adenoma
-high Cortisol; low ACTH
-No cortisol suppression by Dexamethasone
What would 17-alpha-hydroxylase deficiency cause?
low Sex hormones & Cortisol
high Mineralcorticosteroids

Hypertension, Hypokalemia
Phenotypically female but no maturation
What would 21-beta-hydroxylase deficiency cause?
low Cortisol & Mineralcorticoids
high Sex Hormones

Salt wasting can lead to Hypovolemia
Ambiguous genitalia in females
Precocious puberty in males
What would 11-beta-hydroxylase deficiency cause?
low Cortisol & Aldosterone
high Sex hormones & 11-deoxycorticosterone

Hypertension = due to increased 11-deoxycorticosterone
Congenital Adrenal Hyperplasia due to 21-hydroxylase deficiency
-Clitoral enlargement
What is the cause of this?
Congenital Adrenal Hyperplasia (21-hydroxylase deficiency)
-Adrenal gland is under constant stimulation by ACTH but only the Reticularis can responds b/c it contains the only cells that do not need 21-hydroxylase to make their product = Sex hormones
What is the cause of this?
Primary Hyperaldosteronism due to a benign adenoma in the Zona Glomerulosa
Conn's Syndrome
What are the clinical features of Conn's Syndrome?
-Hypertension = due to Aldosterone causing Na+ retention
-Metabolic Alkalosis & Hypokalemia = due to increase K+ & H+ secretion
-Decreased Renin levels
-decreased Serum Potassium
List the causes of Conn's Syndrome
1. 90%
2. 10&
3. very rare
1. Cortical Adenoma = Aldosteronoma

2. Nodular Cortical Hyperplasia

3. Adrenocortical Carcinoma
Adrenal Cortical Adenoma as seen in Conn's Syndrome = Primary Hyperaldosteronism
What is seen here?
Lipid-filled Adrenal Cortical cells in a Benign Adrenal Adenoma = Conn's Syndrome
What is seen here?
Describe a Neuroblastoma
Small cell malignant neoplasm arising in the Adrenal Medulla
1. Most common presenting age?
2. How does it present?
3. What are the frequent metastasis sites?
4. Who has a better prognosis?
5. What do the tumor cells secrete?
6. What is increased in the urine?
1. 1-10 years of age
2. large abdominal mass
3. lungs & marrow
4. pts under 1 year of age
5. Epi or NE
6. VMA
Neuroblastoma = malignant tumor of Adrenal Medulla
What is seen here?
-small, hyperchromatic malignant cells

Neural Ectoderm
What is this? What is it derived from?
Ganglioneuroma = Neuroblastoma that has reverted to a benign state
What is seen here?
What gene is Neuroblastoma associated with?
N-myc oncogene
Define Pheochromocytoma
Neoplasm of Chromaffin cells of the Adrenal Medulla that produces Catecholamines
1. peak incidence?
2. what do tumor cells secrete?
3. unilateral or bilateral
4. benign or malignant?
1. 30-50
2. Epi &/or NE = cause surgically-correctable HTN
3. Unilateral
4. Benign
What conditions are Pheochromocytomas associated with?
1. Neurofibromatosis
2. MEN II = Medullary Thyroid CA + Pheochromocytoma + Parathyroid Hyperplasia or Adenoma
3. von Hippel-Lindau disease
What are the clinical manifestations of Pheochromocytoma?
Intermittent attacks of HTN, headache, palpitations


Drenching sweats
What are the laboratory findings in Pheochromocytomas?
increased 24-hour urinary VMA & Metanephrine levels

increased urinary & plasma Catecholamines
-expanded Medullary space in the Adrenal gland
What is seen here?
-nests of polygonal chromaffin cells containing catecholamine-rich granules
What is this from?
-secretory granules staining brownish with the chromate stain
What is seen here?
List the "Rule of 10's" associated with Pheochromocytomas
1. 10% occur in children
2. 10% are bilateral
3. 10% occur outside the Adrenal Gland = Paraganglioma
4. 10% are malignant
5. 10% are familial (MEN II & III)