ENDOC-ADRENAL CORTEX- PRIMARY 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________ and ____________.

Answer 2

primary, or it may be
secondary to an extra-adrenal cause

Question 3

_____________ stems from an autonomous
overproduction of aldosterone,with resultant suppressionof therenin-angiotensin system and
decreased plasma renin activity.

Answer 3

Primary hyperaldosteronism

Question 4

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

Answer 4

Blood pressure elevation

Question 5

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

Answer 5

1. • Bilateral idiopathic hyperaldosteronism (IHA)
2. • Adrenocortical neoplasm,
3. • Glucocorticoid-remediable hyperaldosteronism

Question 6

•_____________ characterized by bilateral nodular
hyperplasia of the adrenal glands, is themost commonunderlying cause of primary
hyperaldosteronism,accounting for about60% of cases.

Answer 6

Bilateral idiopathic hyperaldosteronism (IHA),

Question 7

Individuals with IHA tend to be
____________ than those presenting with adrenal
neoplasms.

The pathogenesis of IHA remains unclear.

Answer 7

older and to have less severe hypertension

Question 8

• Adrenocortical neoplasm, either an _______________-or, rarely, an ____________

Answer 8

1. aldosterone-producing adenoma (the most common cause)
2. adrenocortical carcinoma

Question 9

. In approximately 35% of cases, primary
hyperaldosteronism is caused by a_____________, a condition referred to as Conn syndrome. [70]

This syndrome occurs most frequently in adult
middle lifeand ismore common in womenthan in men(2 : 1). Multiple adenomas may be present in an occasional patient.

Answer 9

solitary aldosterone-secreting adenoma

Question 10

• _________________ is an uncommon cause of primary
familial hyperaldosteronism.

In some families, it is caused by a chimeric gene resulting
from fusion between CYP11B1 (the 11β-hydroxylase gene) and CYP11B2 (the aldosterone synthase gene).

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 10

Glucocorticoid-remediable hyperaldosteronism

Question 11

Question 12

In secondary hyperaldosteronism, in contrast, aldosterone release occurs in response to
activation of the renin-angiotensin system ( Chapter 11 ).

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

Answer 12

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

Question 13

Morphology of Aldosterone-producing adenomas are

Answer 13

• almost always solitary, small (<2 cm in diameter),
• well-circumscribed lesions,
• more often found on the left than on the right.
• 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.**_
• 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).
• In general, the cells tend to be uniform in size and shape and resemble mature cortical cells;
• occasionally, there is modest nuclear and cellular pleomorphism (see Fig. 24-50 ).

Question 14

A characteristic feature of aldesterone-producing adenomas is the _____________________.

Answer 14

presence of eosinophilic, laminated cytoplasmic
inclusions, known as spironolactone bodies, found after treatment with the
antihypertensive drug spironolactone

Question 15

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 16

_______________ is marked by diffuse and focal hyperplasia of cells resembling those of the normal zona glomerulosa.

The hyperplasia is often wedge-shaped,
extending from the periphery toward the center of the gland.

Answer 16

Bilateral idiopathic hyperplasia

Question 17

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 18

The clinical sine qua non of hyperaldosteronism is _____________-

.

Answer 18

hypertension

Question 19

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.

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 20

The long-term effects of hyperaldosteronism-induced hypertension are ____________________

Answer 20

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 21

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

Answer 21

Hypokalemia

NOTE:

Hypokalemia results from renal potassium wasting and, when
present, can cause a variety of neuromuscular manifestations, including weakness, paresthesias, visual disturbances, and occasionally frank tetany.

Question 22

The diagnosis of primary
hyperaldosteronism is confirmed by elevated ratios of plasma _________________; 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.

Answer 22

aldosterone concentration to
plasma renin activity

Question 23

In primary hyperaldosteronism, the therapy varies according to cause.

Answer 23

Adenomas are amenable
to surgical excision.

Question 24

In contrast, surgical intervention is not very beneficial in patients with** primary hyperaldosteronism** due to_______________, which often occurs in children and
young adults.

These patients are best managed medically with an aldosterone antagonist such
as spironolactone.

Answer 24

bilateral hyperplasia

Question 25

The treatment of secondary hyperaldosteronism rests on _______________

Answer 25

correcting the
underlying cause stimulating the renin-angiotensin system.