ENDOC-ADRENAL CORTEX- PRIMARY HYPERALDOSTERONISM Flashcards Preview

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Flashcards in ENDOC-ADRENAL CORTEX- PRIMARY HYPERALDOSTERONISM Deck (26):
1

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

Hyperaldosteronism

2

 Hyperaldosteronism may be________ and ____________. 

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

3

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

 

Primary hyperaldosteronism

4

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

Blood pressure elevation

5

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

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

6

•_____________ characterized by bilateral nodular
hyperplasia of the adrenal glands
, is the most common underlying cause of primary
hyperaldosteronism,
accounting for about 60% of cases.

 Bilateral idiopathic hyperaldosteronism (IHA),

7

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

The pathogenesis of IHA remains unclear.
 

older and to have less severe hypertension

8

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

 

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

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 life
and is more common in women than in men (2 : 1). Multiple adenomas may be present in an occasional patient.
 

 solitary aldosterone-secreting adenoma

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.

Glucocorticoid-remediable hyperaldosteronism

11

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:
 

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)

13

Morphology of  Aldosterone-producing adenomas are

  •  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 ).

14

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

 

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

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.

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.

Bilateral idiopathic hyperplasia

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.

18

The clinical sine qua non of hyperaldosteronism is _____________-

hypertension 

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]

 

20

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. 

21

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

 

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.

 

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.

aldosterone concentration to
plasma renin activity

23

In primary hyperaldosteronism, the therapy varies according to cause.

 

Adenomas are amenable
to surgical excision.

 

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.

 

 bilateral hyperplasia

25

The treatment of secondary hyperaldosteronism rests on _______________

correcting the
underlying cause stimulating the renin-angiotensin system.

26

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