ENDOC_ ADRENAL CORTEX- HYPERALDOSTERONISM

Question 1

______________ is the generic term for a group of closely related conditions characterized
by chronic excess aldosterone secretion.

Answer 1

Hyperaldosteronism

Question 2

Hyperaldosteronism may be primary, or it may be
secondary to anextra-adrenal cause.

Primary hyperaldosteronism stems from an autonomous
overproduction of aldosterone, with_________________ and _____________.

Answer 2

1. resultant suppression of the renin-angiotensin system and
2. **decreased plasma renin activity **

Question 3

________________ is the most common manifestation of
primary hyperaldosteronism, which is caused by one of three mechanisms

Answer 3

Blood pressure elevation

Question 4

. Blood pressure elevation is the most common manifestation of
primary hyperaldosteronism, which is caused by one of three mechanisms ( Fig. 24-44 ):
•

Answer 4

1. Bilateral idiopathic hyperaldosteronism (IHA),
2. Adrenocortical neoplasm, either an aldosterone-producing adenoma or, rarely, an adrenocortical carcinoma.
3. Glucocorticoid-remediable hyperaldosteronism

•

Question 5

Bilateral idiopathic hyperaldosteronism (IHA), characterized by ___________________, is the most common underlying cause of primary
hyperaldosteronism, accounting for about60% of cases.Individuals with IHAtend to beolder and to have less severe hypertensionthanthose presenting with adrenal
neoplasms. The pathogenesis of IHA remains unclear.

Answer 5

bilateral nodular
hyperplasia of the adrenal glands

Question 6

• *Adrenocortical neoplasm**, either an aldosterone-producing adenom*_a (the most common
• cause)**_ or, rarely, an adrenocortical carcinoma.

In approximately 35% of cases, primary
hyperaldosteronism is caused by a solitary aldosterone-secreting adenoma, a condition
referred to as ________________. [70]

This syndrome occurs most frequently in adult middle life and is more common in women than in men (2 : 1).

Multiple adenomas may
be present in an occasional patient.

Answer 6

Conn syndrome

” ADULT”

“MIDDLE lif”

WOMEN

Question 7

• *Glucocorticoid-remediable hyperaldosteronism** is an uncommon cause of primary
• *________________**.

Answer 7

familial hyperaldosteronism

Question 8

Glucocorticoid-remediable hyperaldosteronism In some families, it is caused by a chimeric gene resulting
from fusion between _________________

This leads to a sustained production of hybrid steroids in
addition to both cortisol and aldosterone. The activation of aldosterone secretion is
under the influence of ACTH and hence is suppressible by exogenous administration of
dexamethasone.

Answer 8

CYP11B1 (the 11β-hydroxylase gene) and CYP11B2 (the
aldosterone synthase gene).

Question 9

In secondary hyperaldosteronism, in contrast, aldosterone release occurs in response to_________________( Chapter 11 )

Answer 9

activation of the renin-angiotensin system

Question 10

Secondary Hyperaldosteronism is characterized by increased levels of plasma renin and is encountered in conditions such as the following:

Answer 10

1. • Decreased renal perfusion (arteriolar nephrosclerosis, renal artery stenosis)
2. • Arterial hypovolemia and edema (congestive heart failure, cirrhosis, nephrotic syndrome)
3. • Pregnancy (due to estrogen-induced increases in plasma renin substrate)

Question 11

Morphology. Aldosterone-producing adenomas are almost always _____________________ They
tend to occur in the 30s and 40s, and in women more often than in men.

These lesions are** often buried within the gland** and do not produce visible enlargement, a point to be
remembered in interpreting sonographic or scanning images.

Answer 11

• solitary, small (<2 cm in diameter),
• well-circumscribed lesions
• more often found on the left than on the right.
• They are bright yellow on cut
  section and, surprisingly,
• are composed of lipid-laden cortical cells that more closely resemble fasciculata cells than glomerulosa cells (the normal source of aldosterone).

Question 12

In general the appearance of Aldosterone producing adenomas are, (see Fig. 24-50 )

Answer 12

the cells tend to be uniform in size and** shape and resemble mature cortical cells;** occasionally,
there is modest nuclear and cellular pleomorphism

Question 13

. A characteristic feature
of aldesterone-producing adenomas is the presence of eosinophilic, laminated cytoplasmic
inclusions,known as________________ found after treatment with the
antihypertensive drug spironolactone.

Answer 13

spironolactone bodies,

Question 14

In contrast to cortical adenomas associated with

• *Cushing syndrome**, those associated with hyperaldosteronism do not usually suppress ACTH
• *secretion.**

Therefore, the adjacent adrenal cortex and that of the contralateral gland are not
atrophic.

Question 15

Bilateral idiopathic hyperplasia is marked by diffuse and focal hyperplasia of cells
resembling those of the normal ______________.

Answer 15

zona glomerulosa

BIH resembles Zona Glomerulosa

Aldosterone producing adenoma is more of FASICULATA

( weird tlga ang life kasi originating ang tumor sa Glomerulosa

Question 16

What is the appearance in BIH?

Answer 16

The hyperplasia is often wedge-shaped,
extending from the peripherytoward the center of the gland. Bilateral enlargement can be
subtle in idiopathic hyperplasia, and as a rule, an adrenocortical adenoma should be carefully
excluded as the cause for hyperaldosteronism.

Question 17

The clinical sine qua non of hyperaldosteronism is _________________. With an estimated prevalence
rate of 5% to 10% among nonselected hypertensive patients, primary hyperaldosteronism may
be the most common cause of secondary hypertension (i.e., hypertension secondary to an
identifiable cause).

The prevalence of hyperaldosteronism increases with the severity of
hypertension, reaching nearly 20% in patients who are classified as having treatment-resistant
hypertension.

Answer 17

hypertension

Question 18

Through its effects on the renal mineralocorticoid receptor, aldosterone promotes
sodium reabsorption, which secondarily increases the reabsorption of water, expanding the
extracellular fluid volume and elevating cardiac output.

In addition, aldosterone contributes to
endothelial dysfunction by decreasing glucose-6-phospate dehydrogenase levels, which, in
turn, reduces endothelial nitric oxide synthesis and causes oxidative stress. [71]

Question 19

The long-term
effects of hyperaldosteronism-induced hypertension are cardiovascular compromise (e.g., left
ventricular hypertrophy and reduced diastolic volumes) and an increase in the prevalence of
adverse events such as stroke and myocardial infarction.

Question 20

________________ was considered a
mandatory feature of primary hyperaldosteronism, but increasing numbers of normokalemic
patients are now diagnosed.

Answer 20

Hypokalemia

Question 21

_________________results from renal potassium wasting and, when

• *present, can cause a variety of neuromuscular manifestations, including weakness,**
• *paresthesias,** visual disturbances, and occasionally frank tetany.

Answer 21

Hypokalemia

Question 22

The diagnosis of primary
hyperaldosteronismis confirmed byelevated ratios of plasma aldosterone concentration to
plasma renin activity; if this screening test is positive, a confirmatory aldosterone suppression
test must be performed, since many unrelated causes can alter the plasma aldosterone and
renin ratios.

Question 23

In primary hyperaldosteronism, the therapy varies according to cause.

Adenomas are amenable
to surgical excision

. In contrast, surgical intervention is not very beneficial in patients withprimary hyperaldosteronism due to bilateral hyperplasia, which often occurs in children and
young adults.

These patients are best managed __________________

Answer 23

medically with an aldosterone antagonist such
as spironolactone.

Question 24

The treatment of secondary hyperaldosteronism rests on correcting the
underlying cause stimulating the renin-angiotensin system.

Question 25

Adrenogenital Syndromes
Disorders of sexual differentiation, such as virilization or feminization, can be caused by ___________________ and ____________

Answer 25

primary
gonadal disorders ( Chapter 22 ) and several primary adrenal disorders.

Question 26

The adrenal cortex
secretes two compounds—______________—that can be
converted to testosterone in peripheral tissues.

Answer 26

dehydroepiandrosterone and androstenedione

Unlike gonadal androgens, ACTH regulates
adrenal androgen formation ( Fig. 24-45 ); thus, excess secretion can occur either as a “pure”
syndrome or as a component of Cushing disease. The adrenal causes of androgen excess
include adrenocortical neoplasms and a group of disorders that have been designated
congenital adrenal hyperplasia (CAH) .

Question 27

Adrenocortical neoplasms associated with virilization are more likely to be androgen-secreting
adrenal carcinomas than adenomas.

Such tumors are often also associated with
hypercortisolism (“mixed syndrome”).

They are morphologically identical to other cortical
neoplasms and will be discussed later.

Question 28

CAH represents a group of autosomal-recessive, inherited metabolic errors, each characterized
by a deficiency or total lack of a particular enzyme involved in the biosynthesis of cortical
steroids, particularly cortisol.

Steroidogenesis is then channeled into other pathways, leading to
increased production of androgens, which accounts for virilization.

Question 29

Simultaneously, the
deficiency of cortisol results in increased secretion of ACTH, resulting in adrenal hyperplasia.
Certain enzyme defects may also impair aldosterone secretion, adding salt wasting to the
virilizing syndrome. Other enzyme deficiencies may be incompatible with life or, in rare