Adrenal Pathologies

Question 1

Within many endocrine pathologies, the location of dysfunction is denoted by what?

Answer 1

• tertiary
• secondary
• primary

Question 2

tertiary refers to dysfunction at the ?

Answer 2

hypothalamus

Question 3

secondary refers to dysfunction at the ?

Answer 3

the level of the pituitary gland

Question 4

primary refers to dysfunction within the what?

Answer 4

“final” endocrine organ

Question 5

pathologies of the adrenal glands can involve ? or ?

Answer 5

hyperfunction or hypofunction

Question 6

what level of causes are quite common for hyperfunction

Answer 6

secondary causes

Question 7

What are the 3 hyperfunction pathologies of the adrenal glands we discussed in this lecture?

Answer 7

• Cushing’s syndrome (hypercortisolism)
• hyperaldosteronism
• pheochromocytoma

Question 8

What are the 2 hypofunction pathologies of the adrenal glands we discussed in this lecture?

Answer 8

• adrenocortical insufficiency
• congenital adrenal hyperplasia

Question 9

what is Cushing syndrome?

Answer 9

disorder caused by any condition that produces an elevation in glucocorticoid levels

Question 10

what are the 4 causes of excess cortisol for Cushing syndrome?

Answer 10

• latrogenic
• hypothalamic-pituitary diseases associated with hypersecretion of ACTH
• Hypersecretion of cortisol by an adrenal adenoma, carcinoma or nodular hyperplasia
• Secretion of ectopic ACTH by a nonendocrine neoplasm

Question 11

what is Cushing Disease?

Answer 11

pituitary gland contains an ACTH-producing microadenoma causing hypercortisolism

Question 12

what are the main tumours for paraneoplastic syndrome?

Answer 12

small cell lung cancer or renal adenocarcinoma

Question 13

what happens in paraneoplastic syndrome

Answer 13

usually involve rapid increases in the levels of ACTH and evolution of symptoms/signs

Question 14

describe adrenocortical carcinoma

Answer 14

uncommon cause of primary Cushing’s syndrome, typically large mass with excess production glucocorticoids and androgens

Question 15

what is the most common cause of Cushing syndrome

Answer 15

Iatrogenic Cushing Syndrome

Question 16

what is Iatrogenic Cushing Syndrome

Answer 16

Exogenous administration of glucocorticoids prescribed by a health care practitioner to treat other diseases

Question 17

how does the glucocorticoids have to be administered in Iatrogenic Cushing Syndrome?

Answer 17

systemically administered

Question 18

what is the mnemonic for clinical course of Cushing Syndrome?

Answer 18

C - Central obesity, Cervical fat pads, Collagen fibre weakness, Comedones (acne)

U - Urinary free cortisol and glucose increase

S - Striae, Suppressed immunity

H - Hypercortisolism, Hypertension, Hyperglycemia, Hirsutism

I - Iatrogenic (Increased administration of corticosteroids)

N - Noniatrogenic (Neoplasms)

G - Glucose intolerance, impaired Growth

Question 19

how is cortisol often measured?

Answer 19

24-hour free urine cortisol and/or serum cortisol within a low dexamethasome suppression test

Question 20

explain the low dose dexamethasone suppression test

Answer 20

A small dose of dexamethasone is given the night before & endogenous glucocorticoid levels are assessed the next morning
- In a healthy patient we would expect dexamethasome to inhibit the anterior pituitary gland and suppress endogenous glucocorticoids.
- No suppression is indicative of Cushing syndrome

Question 21

what is high dose dexamethasone suppression test used for?

Answer 21

differentiate Cushing’s disease from other causes of Cushing’s syndrome

Question 22

explain the high dose dexamethasone suppression test

Answer 22

Larger dose of dexamethasone is given the night before & endogenous glucocorticoid levels are assessed the next morning
- Suppression of endogenous glucocorticoids => Cushing’s disease
- No suppression => ectopic ACTH-dependent

Question 23

Patterns of adrenal insufficiency can be considered under the following headings:

Answer 23

○ Primary acute adrenocortical insufficiency (Adrenal crisis)
○ Primary chronic adrenal insufficiency (Addison Disease)
○ Secondary adrenocortical insufficiency

Question 24

What is primary acute adrenocortical insufficiency?

Answer 24

A life-threatening emergency caused by sudden inability of adrenal glands to produce adequate glucocorticoids

Question 25

What are the main etiologies of acute adrenocortical insufficiency?

Answer 25

1. Chronic adrenocortical insufficiency + stress (glands can’t increase steroid output) 2. Exogenous corticosteroid use → rapid withdrawal or failure to increase dose during stress (atrophic adrenals can’t respond) 3. Adrenal hemorrhage

Question 26

Why can withdrawal of corticosteroids trigger adrenal crisis?

Answer 26

Exogenous steroids suppress the HPA axis → adrenal glands become atrophic → sudden withdrawal leaves them unable to produce glucocorticoids.

Question 27

Why are adrenal glands prone to hemorrhage?

Answer 27

They are among the best-perfused tissues in the body (very high blood flow per gram of tissue).

Question 28

Causes of adrenal hemorrhage?

Answer 28

- Newborns after prolonged/difficult delivery - Anticoagulant therapy - Post-surgical DIC (disseminated intravascular coagulation) - Waterhouse-Friderichsen syndrome (meningococcal sepsis)

Question 29

What is Addison Disease?

Answer 29

Uncommon disorder resulting from progressive destruction of adrenal cortex

Question 30

90% of all the addison disease cases are attributable to one of four disorders

Answer 30

- Autoimmune destruction of the adrenal cortex - TB - AIDS - Sarcoidosis or malignancy

Question 31

What are the autoimmune causes of addison disease?

Answer 31

- Autoimmune polyendocrine syndrome (APS) type 1 - APS type 2 - idiopathic

Question 32

Describe the APS type 1 for addison disease

Answer 32

§ Rare, autosomal recessive condition § Begins in older children and adolescents § Several endocrine organs can be attacked

Question 33

Describe the APS type 2 for addison disease

Answer 33

§ More common than type I § Begins in ealry adulthood (20-40s) § Does not have skin or dental findings § Often autoimmune attack of multiple endocrine organs

Question 34

what are the initial manifestations of addison disease?

Answer 34

progressive weakness and easy fatigability

Question 35

in addison disease do you have loss or more of sodium and potassium

Answer 35

Loss of sodium and increase in potassium in blood

Question 36

what is the secondary adrenocortical insufficiency?

Answer 36

Any disorder of hypothalamus and pituitary that reduces the output of ACTH → syndrome of hypoadrenalism

Question 37

secondary adrenocortical insufficiency is deficient in ? and ? but normal ?

Answer 37

- cortisol and androgen output - aldosterone levels

Question 38

what is primary hyperaldosteronism?

Answer 38

Characterized by chronic excess aldosterone secretion

Question 39

What is Conn syndrome?

Answer 39

Primary hyperaldosteronism caused by an aldosterone-secreting adrenal adenoma.

Question 40

What is secondary hyperaldosteronism

Answer 40

- Characterized by increased levels of plasma renin

Question 41

What is congenital adrenal hyperplasia (CAH)

Answer 41

Autosomal recessive defect in enzyme in cortisol synthesis pathways

Question 42

what is the most common enzyme deficiency for congenital adrenal hyperplasia?

Answer 42

21-Hydroxylase

Question 43

the classic type of CAH affects who?

Answer 43

newborns

Question 44

What are the two types of classic CAH?

Answer 44

- salt-wasting - simple virilizing

Question 45

what is salt-wasting in terms of classic CAH?

Answer 45

□ Complete inactivation of 21-hydroxylase

Question 46

what is simple virilizing in terms of classic CAH?

Answer 46

Significantly reduced function of 21-hydroxylase

Question 47

salt-wasting results in no synthesis of what?

Answer 47

aldosterone

Question 48

what do female infants have when they have simple virilizing CAH?

Answer 48

ambiguous genitalia at birth

Question 49

what is the most common form of CAH

Answer 49

non-classic (late-onset)

Question 50

In women, late-onset CAH can often mimics ?

Answer 50

poly cystic ovary syndrome (PCOS)

Question 51

what are the lab test for non-classic CAH?

Answer 51

17 hydroxyprogesterone (17-OHP) levels as assessed

Question 52

what are pheochromocytoma?

Answer 52

- Rare tumour of chromaffin cells

Question 53

what is the dominant picture of pheochromocytoma?

Answer 53

hypertension, ○ 2/3 will experience paroxysmal hypertension