Endocrine 5

Question 1

Conn’s Syndrome:

Primary Hyperaldosteronism is a rare disease that is typically caused by an adenoma of the _________ (70% of cases), whereas the remaining 30% have cortical hyperplasia.

Answer 1

Zona Glomerulosa

Question 2

Clinical presentation of which condition?

retention of sodium and loss of potassium, accompanied by retention of water, resulting in hypertension (hypernatremic hypokalemic hypertension)

Answer 2

Conn’s Syndrome

Question 3

How is Conn’s Syndrome treated?

Answer 3

Tx is surgery with complete cure

Question 4

_________ is much more common than primary (Conn’s)

where the secretion of Aldosterone is physiologically stimulated by angiotensin (formed from angiotensinogen under the influence of renin)

Answer 4

Secondary Hyperaldosteronism

Question 5

Secondary Hyperaldosteronism-

Renin is secreted from the _____ cells of the kidneys, and elevated levels of renin are typically found in various renal diseases.

Answer 5

J-G cells

Question 6

Secondary Hyperaldosteronism is associated with ________, in contrast to primary (AKA Conn’s syndrome), which is renin-independent and associated with normal levels of renin in the circulation.

Answer 6

hyperreninemia

Question 7

Is Conn’s syndrome renin independent or dependent?

Answer 7

Renin independent

Question 8

How do you determine whether hyperaldosteronism is caused by renal or adrenal disease?

Answer 8

Blood test

(b/c renin and aldosterone measurements are in the blood)

Question 9

Adrenogenital syndrome (Adrenal Virilism) is a rare disease that can affect _______ or ______

Answer 9

neonates or adults

Question 10

The disease is typically found in______ (males or females) who experience what?

Answer 10

Females

who experience virilization due to an excess of adrenogenic hormones.

Question 11

Which syndrome?

May be congenital or may develop in adulthood

In neonates, the disease is related to one of several inborn errors of steroid metabolism (i.e. 21-hydroxylase deficiency) that causes an overproduction of testosterone

Answer 11

Adrenogenital syndrome (Adrenal Virilism)

Question 12

Which syndrome?

An excess in Androgens results in partial virilization of the external female genitalia causing:

1. enlargement of the clitoris
2. partial fusion of the labioscrotal folds.

Answer 12

Adrenogenital syndrome

(although they are genetically female, they are sometimes reared as males)

Question 14

In adult women, Adrenogenital syndrome is related to _________

Answer 14

androgen producing tumors

Question 15

Adrenogenital syndrome causes what 4 sxs in adult women?

Answer 15

1. virilization
2. hirsuitism
3. deepening of the voice
4. loss of menstruation

Question 16

Adrenogenital syndrome-

what is treatment of adrenocortical hyperfunction

Answer 16

depends on the cause of the disease

usually involves surgical resection of hyperfunctioning tumors.

Question 17

Adrenocortical hypofunction is usually a consequence of what 2 things

Answer 17

consequence of adrenal destruction:

1. either acutely (Waterhouse-Freidreichsen’s Syndrome) OR
2. slowly, owing to destruction of the adrenocortex by an autoimmune disease (Addison’s Disease)

Question 18

Infections (TB or Histoplasmosis), primary or metastatic malignancies (Lung or Breast) may also destroy the adrenals, causing

Answer 18

insufficiency

Question 19

Which dz accounts for the vast majority (70-80%) of adrenal insufficiency today

Answer 19

Addison’s Disease

Question 20

Addison’s Disease accounts for the vast majority (70-80%) of adrenal insufficiency today, the remaining are still due to________, that affect the immunosuppressed (AIDS).

Answer 20

infections, such as TB or fungal diseases

Question 21

T/F: malignant tumors are a common cause for Addison’s

Answer 21

FALSE
they are a rare cause for Addisons

Question 23

Pathologically, the adrenal in Addison’s, early on, is characterized in infiltration of ______ and_______ in the cortex.

Answer 23

lymphocytes and plasma cells

Question 24

Addison’s disease- In later stages, the entire cortex is destroyed and may be replaced by _______ or fat cells.

Answer 24

fibrous tissue

Question 25

Clinically, Addison’s Disease presents with what 3 things? Affected patients are hypotensive.

Answer 25

1. Fatigue
2. Weight loss
3. Nausea

(note that this is opposite from Cushing’s)

Question 26

What is the name of the neoplasm of the adrenal medulla that synthesize and release Catecholamines (Epinephrine and Norepinephrine).

Answer 26

Pheochromocytoma

Question 27

Pheochromocytoma: Although uncommon, they give rise to a surgically correctable form of _____.

Answer 27

HTN

Question 28

Pheochromocytoma-

•____% are bilateral and _____% may arise from other sites (i.e. carotid body) where they are called Paragangliomas.

Answer 28

10% and 10%

Question 29

Pheochromocytomas range from small, circumscribed lesions confined to the adrenal to ________

Answer 29

large hemorrhagic masses.

Question 30

• They are tan-yellow, well-defined lesions that compress the adjacent adrenal, with varying amounts of hemorrhage.
• The larger lesions tend to have necrosis and large areas of hemorrhage.

Answer 30

Pheochromocytoma

Question 31

Pheochromocytoma-

•The diagnosis of _______, which occurs in about 10% of pheochromocytomas, is based on the presence of_______.

Answer 31

malignancy, metastasis

Question 32

Pheochromocytoma metastases may involve _________ as well as more distant sites, such as liver, lungs and bone.

Answer 32

regional lymph nodes

Question 33

pheochromocytoma metastases involve which 3 distant sites?

Answer 33

1. liver
2. lungs
3. bone

Question 34

Dominant clinical manifestation is HTN, which is:

1. abrupt,
2. with tachycardia,
3. palpitations,
4. H/A,
5. sweating
6. tremor,
7. often associated with N/V, abdominal, and chest pain.

Answer 34

Pheochromocytoma

Question 35

What would you be concerned about if a patient is complaining of paroxysmal HTN only w/ exercise?

Answer 35

Pheochromocytoma

(just bending over can irritate pheo)

Question 36

in how many patients w/ a pheochromocytoma does the hypertension occur in the form of chronic, sustained elevations in blood pressure.

Answer 36

2/3

Question 37

in 1/3 of patients w/ pheochromocytoma, the HTN paroxysms may be precipitated by what 3 things?

Answer 37

1. stress
2. exercise
3. change in posture

Question 38

the elevations in blood pressure of a person w/ a pheochromocytoma are induced by what?

Answer 38

the sudden release of catecholamines.

Question 39

Pheochromocytoma- the sudden release of catecholamines may acutely precipitate what 5 thngs

Answer 39

1. pulmonary edema
2. MI
3. V-fib
4. CVA
5. CHF

Question 40

In some cases, pheochromocytomas secrete other hormones, such as ______ and _______

may therefore be associated with clinical features related to these hormone secretions.

Answer 40

ACTH and Somatostatin

Question 41

The lab diagnosis of pheochromocytoma is based on the demonstration of what?

Answer 41

increased urinary excretion of free catecholamines and their metabolites, such as Vanillylmandelic Acid (VMA’s) and Metanephrines.

Question 42

Pheochromocytoma- isolated benign tumors are treated with what?

Answer 42

surgical excision after pre-operative and intraoperative medication with adrenergic blocking agents.