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Title: Pathology of adrenal cortex
Description: The organized description of diseases of adrenal cortex
Description: The organized description of diseases of adrenal cortex
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DISEASES OF THE ADRENAL GLAND
HYPERALDOSTERONISM
In this excess aldosterone is secreted
...
It causes are:
1,BILATERAL IDIOPATHIC HYPERALDOSTERONISM(60% OF THE
CASES)
It is characterized by bilateral nodular hyperplasia of aldosterone-secreting zona glomerulosa
cells of the adrenal glands
...
They are almost always solitary, small (<2 cm in diameter),
well-circumscribed lesions, more often found on the left than on the right
...
A characteristic
feature of aldosterone-producing adenomas is the presence of eosinophilic, laminated
cytoplasmic inclusions, known as spironolactone bodies, found after treatment with the
antihypertensive drug spironolactone
...
Adrenal carcinoma which rarely causes the disease
...
In this there is aberrant expression of aldosterone
synthase in the fasciculata due to genetic mutation
...
Its
causes are renovascular hypertension, juxtaglomerular cell tumors (renin-producing), and
edema (eg, cirrhosis, heart failure, nephrotic syndrome)
...
TREATMENT
●
●
Surgical adrenalectomy
Spironolactone
DISEASES MIMICKING HYPERALDOSTERONISM
1, LIDDLE SYNDROME
A
...
Presents as child with HTN, hypokalemia, and metabolic alkalosis, but with low
aldosterone and low renin
C
...
Treatment is potassium-sparing diuretics (e
...
, amiloride or triamterene), which block
tubular sodium channels; spironolactone is not effective
...
11β- hydroxysteroid dehydrogenase 2 (11β-HSD2) deficiency allows cortisol to
activate renal aldosterone receptors; autosomal recessive
B
...
Diagnosed by low urinary free cortisone and genetic testing
D
...
CAUSES
ACTH INDEPENDENT CAUSES
●
●
Iatrogenic cause(most common cause) which includes exogenous glucocorticoids
administration as a result of this bilateral adrenal atrophy occurs
...
ACTH DEPENDENT CAUSES
●
ACTH-secreting pituitary adenoma (Cushing disease)
...
● paraneoplastic ACTH secretion (eg, small cell lung cancer, bronchial carcinoids)
Both of these causes result in bilateral adrenal hyperplasia
...
SCREENING/ DIAGNOSIS
●
Serum cortisol & CBG is not recommended due to fluctuations
...
Cataract
...
Ecchymosis
...
TREATMENT
●
●
Management of underlying cause(alternative of steroid medications and resection of
tumors)
Ketoconazole and metyrapone for inhibiting cortisol synthesis
...
Its symptoms depend upon different hormone levels
...
EXCESS OF ACTH
It is seen in primary adrenal insufficiency because of high ACTH
...
Addison's generalized hyperpigmentation
which occur in sun exposed areas of skin(neck, face & back of hands) and areas of
friction(elbows, knees & knuckles)
PATTERNS OF THE DISEASE
Three major patterns of adrenocortical insufficiency are recognized:
(1) primary acute adrenocortical insufficiency (adrenal crisis)
...
(3) secondary adrenocortical insufficiency
...
ADRENAL(ADDISONIAN) CRISIS
This is acute primary adrenal insufficiency
...
It main manifestation is shock
...
Another cause is Waterhouse-Friderichsen syndrome(rare cause) in which there is bilateral
adrenal hemorrhage in the setting of sepsis (eg, meningococcemia)
...
Its common cause include:
● Autoimmune Adrenalitis in this antibodies against 21 alpha hydroxylase are seen
...
It is the cause of
addison in high income countries
...
Its symptoms include those which are seen in loss of cortisol and excess of ACTH
...
It is due to pituitary disorders
...
It is most
commonly due to abrupt cessation of chronic glucocorticoid therapy
...
DIAGNOSIS OF ADRENAL INSUFFICIENCY
1,8 AM SERUM CORTISOL
Low levels indicate the disease(levels should be highest for negative results)
...
3,SERUM ACTH
Low cortisol with high ACTH=1st
Low cortisol with low ACTH=2nd
TREATMENT
Glucocorticoids and mineralocorticoids
CONGENITAL ADRENAL HYPERPLASIA
Loss of the one of the 4 enzymes involved in the cortisol biosynthesis:
● 21 𝛂 hydroxylase
● 17 𝛂 hydroxylase
● 11 𝛃 hydroxylase
● 3 𝛃 hydroxysteroid dehydrogenase
The deficiency of any of these causes low cortisol which stimulates ACTH secretion resulting
in skin hyperpigmentation and bilateral adrenal hyperplasia
...
SYMPTOMS
It depends on which hormone is excessively produced or whose levels are low
LOW CORTISOL
●
●
Hypoglycemia
Nausea and vomiting
LOW MINERALOCORTICOID
●
●
●
Shock
Hyperkalemia
Metabolic acidosis
EXCESS MINERALOCORTICOIDS
●
●
●
Hypertension
Hypokalemia
Metabolic alkalosis
EXCESS ANDROGENS
●
●
Female= ambiguous genitalia(virilisation)
Male = precocious puberty
LOW ANDROGEN
●
●
Female=2nd sexual characteristic fails to develop
...
Increased in 21- and 11-hydroxylase deficiency
2
...
Classic form presents in neonates as hyponatremia and hyperkalemia with life
threatening hypotension (salt-wasting type); females have clitoral enlargement
(genital ambiguity)
...
In non classic
form enzymes are working 20-50%
Title: Pathology of adrenal cortex
Description: The organized description of diseases of adrenal cortex
Description: The organized description of diseases of adrenal cortex