Adrenal Path- Krafts Flashcards

1
Q

What is Cushing Syndrome?

A

Excess cortisol

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2
Q

Truncal obesity
Buffalo hump
Moon facies

What do you think…?

A

Cushing syndrome

Gluconeogenesis
High blood sugar
Excess insulin produced
Fat storage in:
Face = moon facies
Back = buffalo hump
Trunk = truncal obesity
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3
Q

Why is hypertension seen in Cushing syndrome?

A

Cortisol upregulates alpha 1 receptor on arterioles, amplifying effects of NE

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4
Q

Why is abdominal striae seen in Cushing syndrome?

A

Cortisol impairs synthesis of collagen

Weaken of blood vessels/rupture = abdominal striae

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5
Q

What 3 ways does cortisol cause immune suppression?

A
  1. Inhibits phospholipase A2 - can’t make AA metabolites for inflammation
  2. Inhibits IL-2 (an important T-cell growth factor)
  3. Inhibits histamine release from mast cells (essential for vasodilation and increased permeability)
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6
Q

What are the 4 main causes of Cushing syndrome?

A
  1. Exogenous corticosteroids
  2. Primary adrenal adenoma, hyperplasia, or carcinoma
  3. ACTH-secreting pituitary adenoma
  4. Paraneoplastic ACTH secretion (small cell lung cancer)
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7
Q

What cancer commonly produces paraneoplastic ACTH causing Cushing Syndrome?

A

Small Cell Lung Cancer

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8
Q

What is Cushing “DISEASE”

A

Pituitary Adenoma

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9
Q

What is the most common cause of cushing syndrome?

A

Exogenous corticosteroids

**bilateral adrenal atrophy

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10
Q

What is the only cause of Cushing syndrome that will give you bilateral adrenal atrophy?

A

Exogenous corticosteroids

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11
Q

What is the point of doing the high-dose dexamethasone test?

A

To distinguish a pituitary adenoma from ectopic ACTH production….

Dex suppresses ACTH production in pituitary adenoma

Dex does NOT suppress ACTH is ectopic production

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12
Q

Will dexamethasone shut down ACTH production from a small cell carcinoma of the lung?

A

NOPE! That is a cancer and it ain’t going to listen to nobody…..

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13
Q

What part of the adrenal gland secretes aldosterone?

A

Adrenal cortex- glomerulosa

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14
Q

Describe the renal/aldosterone axis

A

Low BP/flow
JGA secretes renin
Renin converts angiotensinogen to angiotensin I
ACE converts ATN 1 to ANT2
Angiotensin II stimulates aldosterone secretion from adrenal

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15
Q

What are the functions of aldosterone?

A

Increased Na+ resorption in kidney
Increased K+ excretion
H+ is excreted from alpha-intercalated cells

RAISES BLOOD PRESSURE

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16
Q

What is the usual cause of primary hyperaldosteronism?

A

Adrenal Adenoma:

High aldosterone
LOW RENIN!!!!

17
Q

What are some causes of secondary hyperaldosteronism?

A

Activation of RAS system leading to increased Renin/Aldosterone

CHF
Decreased renal blood flow (renal artery stenosis, or fibromuscular dysplasia)
Renin-producing tumors

18
Q

What is the pathology for congenital adrenal hyperplasia?

A

Congenital adrenal enzyme deficiency

Characterized by an enlargement of both adrenal glands due to increased ACTH stimulation (due to low cortisol)

19
Q

What is the most common deficient enzyme in congenital adrenal hyperplasia?

A

21 hydroxylase

20
Q

Which congenital adrenal hyperplasia….

Hyponatremia
Hyperkalemia
Hypovolemia & hypotension
Females have clitoral enlargement (genital ambiguity)

A

21-hydroxylase

21
Q

Which congenital adrenal hyperplasia….

HTN w/ mild hypokalemia
Renin and aldosterone are low

A

11-hydroxylase

*get HTN because some 11-deoxycorticosterone is a weak mineralcorticoid thus raising blood pressure

(But still can’t make aldosterone and cortisol)

22
Q

Which congenital adrenal hyperplasia….

Females = amenorrhea, lack of pubic heair
Males = pseudohermaphroditism 

HTN

A

17-hydroxylase

23
Q

What infection is commonly associated with Waterhouse-friderichsen syndrome?

A

N. Meningititidis

DIC causes hemorrhagic necrosis of the adrenal glands

Abrupt withdrawal of glucocorticoids

Lack of cortisol exacerbates hypotension , often leading to death

24
Q

What are the 3 things that can cause chronic adrenal insufficeincy?

A

Autoimmune destruction
TB
Metastatic Carcinoma

25
Q

What type of cancer LOVES to metastasize to the adrenal glands?

A

LUNG CANCER!

26
Q
Hypotension
Hyponatremia
Hypovolemia
Hyperkalemia
Hypoglycemia
Hyperpigmentaiton
Vomiting and diarrhea

What do you think?

A

Addison’s Disease!

Chronic Adrenal Insufficiency

27
Q

What is the adrenal cortex composed of? Where is it derived from?

What is it’s main function?

A

Chromaffin cells
Neural crest-derived

Main physiologic source of catecholamines (Epi and NE)

28
Q
Episodic HTN
Headaches
Palpitaitons
Tachycardia
Sweathing

Increased serum metanephrines and increased VMA

What do you think…?

A

Pheochromocytoma!

29
Q

Why do you need to give phenoxybenzamine before surgical removal of a pheochromocytoma?

A

It is an irreversible alpha antagonist

Need to give before surgery because whey surgeons grab the tumor, may squeeze out NE and epi causing a surge…. can lead to massive HTN and death

30
Q

What lesions are present in MEN-1?

A

Three P’s!

Pituitary Adenoma
Parathyroid Tumors
Pancreatic Tumors

31
Q

What is the genetic abnormality in MEN-1?

A

MEN-1 gene

Tumor suppressor gene (like the breaks on the cell cycle)

32
Q

What lesions are present in MEN2a?

A

Two P’s!

Medullary thyroid Carcinoma
Pheochromocytoma
Parathyroid Hyperplasia

33
Q

What is the genetic abnormality in MEN2a?

A

RET gene

Protooncogene

Encodes tyrosine kinase receptor that binds to neurotrophic ligands

34
Q

What lesions are present in MEN2b?

A

1 P!

Pheochromocytoma
Medullary thryoid carcinoma

35
Q

What is the genetic abnormality in MEN2b?

A

RET gene

Protooncogene

Encodes tyrosine kinase receptor that binds to neurotrophic ligands

36
Q

What type of inheritance for MEN syndromes?

A

Autosomal Dominant