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Endocrine 2 > Adrenals Glands path > Flashcards

Adrenals Glands path Flashcards

1
Q

Adrenal cortex is divided into what layers?

What does each layer produce?

A
  • Zona Glomerulosa: mineralocorticoids (aldosterone)
  • Zona Fasciculata: Glucocorticoids (Cortisol)
  • Zona Reticularis: Adrenal androgens and estrogens
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2
Q

What cells are in the adrenal medulla and what do they make and secrete

A

Chromaffin cells: catecholamines, mainly epinephrine

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3
Q

Excess of cortisol is called what

A

Cushing syndrome

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4
Q

What are the syndromes called that are caused from excessive androgens

A

-Adrenogenital or virilizing syndromes

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5
Q

What is the most common cause of Cushing syndrome

A

-Exogenous: Glucocorticoids (“steroids”)

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6
Q

What are the Endogenous causes of Cushing syndrome that are ACTH dependent?

A
  • -Cushing disease: pituitary adenoma

- Ectopic coricotropin syndrome (ACTH)

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7
Q

What are the Endogenous causes of Cushing syndrome that are ACTH independent?

A
  • Adrenal adenoma or carcinoma
  • Macronodular hyperplasia
  • Primary pigmented nodular adrenal disease
  • McCune-Albright syndrome
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8
Q

What makes up 70% of endogenous hypercortisolism?

A

ACTH-secreting pituitary adenoma

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9
Q

What is the epidemiology of an ACTH secreting pituitary adenoma

A
  • W>M (4x)
  • most frequent in young adults
  • Majority are Microadenomas
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10
Q

Ectopic ACTH secretion by a nonpituitary tumor is most often due to what

A

small cell carcinoma of the lung

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11
Q

Sometimes a neuroendocrine neoplasm produces ectopic corticotrophin releasing hormone (CRH) . . leading to what

A

-ACTH secretion and hypercortisolism

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12
Q

What is the most common causes of ACTH independent Cushing syndrome

A

Primary Adrenal neoplasms

  • Adenoma (10%)
  • Carcinoma (5%): produce the most profound hypercortisolism
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13
Q

in a primary adrenal neoplasm producing hypercortisolism, what happens to ACTH levels

A

go down

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14
Q

Explain the cortical atrophy associated with administration of exogenous glucocorticoids

A

-suppression of ACTH –> lack of stimulationg of Zona Fasciculata and reticularis

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15
Q

Describe Diffuse hyperplasia of adrenals in Cushing syndrome

A
  • both glands enlarged
  • Endogenous hypercortisolism
  • ACTH-dependent
  • Cortex can be variably nodular
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16
Q

What are the two types of adrenal gland hyperplasia?

A
  • Macronodular

- micronodular

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17
Q

Describe macronodular hyperplasia of adrenal glands

A
  • Endogenous hypercortisolsim
  • Adrenals almost entirely replaced by prominent nodules of varying sizes (less than or equal to 3 cm)
  • Areas b/t macroscopic nodules also demonstrate evidence of microscopic nodularity
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18
Q

Describe micronodular hyperplasia of adrenal glands

A
  • endogenous hyperplasia
  • Composed of 1 to 3 mm darkly pigmented (brown to black) micronodules, with atrophic intervening areas
  • pigment is thought to be lipfuscin
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19
Q

Generalities for Primary adrenocortical neoplasms

A
  • Can be functional (Cushing syndrome) or non-Functional
  • Morphologically indistinct
  • in functional tumors: adjacent adrenal cortex and the contralateral adrenal gland are atrophic
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20
Q

Age and gender for Primary adrenocorical neoplasms

A

women aged 30-50

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21
Q

Describe a primary adrenocortical adenoma

A
  • benign
  • Yellow tumors surrounded by thin or well developed capsules
  • most weigh < 30gms
  • microscopically see cells that look like normal fasciculata
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22
Q

Describe a primary adrenocortical carcinoma

A
  • malignant
  • Larger than the adenomas (usually > 200-300 gms)
  • UNencapsulated
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23
Q

What are the early manifestations of a Cushing syndrome

A
  • HTN

- weigh gain

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24
Q

Later more characteristic features of a primary Cushing syndrome

A
  • Central pattern of fat deposition (truncal obesity)
  • Moon facies
  • Fat in the posterior neck and back (buffalo hump)
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25
Q

Clinical course of cushing syndrome

A
  • develp slowly over time
  • Atrophy of fast-twitch myofibers–> decreased muscle mass and proximal limb weakness
  • Glucocorticoids induce gluconeogenesis and inhibit the uptake of glucose by cells . . .Hyperglycemia, glucosuria, and polydispia (SECONDARY DIABETES)
  • increased risk of infections (IMMUNE SUPPRESSION)
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26
Q

The catabolic effect of Cushing syndrome causes loss of collagen and resorption of bone. . .this leads to what symptoms

A
  • Skin is thin, fragile, and easily bruised
  • poor wound healing
  • cutaneous striae are particularly common in the abdominal area
  • Osteoporosis
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27
Q

How do you diagnose Cushing syndrome?

A
  • increased 24 hour urine free cortisol

- Loss of normal diurnal pattern of cortisol secretion

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28
Q

How do you determine the cause of Cushing syndrome?

A
  • Serum ACTH

- Dexamethasone suppression test: Urinary excretion of 17-hydroxycorticosteroids after administration of dexamethasone

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29
Q

What cause of Cushing syndrome is the one that will suppress ACTH with HIGH dose dexamethoasone and therefore reduce urinary excretion of 17-hydroxycorticosteroids

A

Pituitary Cushing

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30
Q

Autonomous overproduction of aldosterone

A

Primary hyperaldosteronism

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31
Q

overproduction of aldosterone leads to suppression of the Renin-angiotensin system and decreased plasma renin activity leading to what?

A

Elevated blood pressure . . most common manifestation of Primary hyperaldosteronism

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32
Q

What are the 3 mechanisms that cause primary hyperaldosteronism?

A
  • Adrenocortical neoplasm
  • Bilateral idiopathic hyperaldosteronism
  • Glucocorticoid-remediable hyperaldosteronism
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33
Q

What neoplasm most commonly causes primary hyperaldosteronism? . . what syndrome is this

A
  • adenoma . . . Conn syndrome

- rarely carcinomas do this

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34
Q

age and gender for aldosterone secreting adenoma

A

middle ages most common

-W>M (2:1)

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35
Q

What side is preferentially affected by aldosterone secreting adenoma

A

Left

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36
Q

Morphology of aldosterone secreting adenoma

A
  • usually solitary
  • Small (<2 cm)
  • well circumscribed
  • brightly yellow grossly
  • Characteristic microscopic feature: SPIRONOLACTONE BODIES
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37
Q

Explain the the spironolactone bodies in an aldosterone secreting adenoma are

A
  • Eosinophilic, laminated cytoplasmic inclusions

- Found after treatment with spironolactone (antihypertensive drug)

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38
Q

Do aldosterone secreting adenomas usually suppress ACTH secretion?

A

NO, so adjacent adrenal cortex and contralateral gland are not atrophic

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39
Q

What is the most common cause of primary hyperaldosteronism

A

Bilateral idiopathic hyperaldosteronism

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40
Q

What are the features of bilateral idiopathic hyperaldosteronism

A
  • older pts
  • less sever HTN
  • Pathogenesis unclear
  • Bilateral nodular hyperplasia of the adrenal glands
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41
Q

Rearrangement of chromosome 8 that places the gene for aldosterone synthase under the control of the ACTH responsive gene promoter

  • so ACTH stimulates the production of aldosterone
  • suppressible by dexamethasone
A
  • Glucocorticoid-remediable Hyperaldosteronism
  • uncommon
  • familial hyperaldosteronism
42
Q

What is secondary hyperaldosteronism

A

Aldosterone is released in response to activation of the renin-angiotensin system by increase plasma renin

43
Q

When do you see secondary hyperaldosteronism?

A
  • 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
44
Q

What are the long term effects of HTN on the CV system

A
  • left ventricular hypertrophy and reduced diastolic volumes

- Stroke and myocardial infarction

45
Q

Aldosterone also promotes sodium reabsorption which in turn increases reabsorption of water . . this does what?

A
  • expands the extracellular fluid volume

- Elevated cardiac output

46
Q

hyperaldosteronism lead to hypokalemia . .causing what?

A
  • weakness
  • paresthesias
  • visual disturbances
  • occasionally tetany
47
Q

What is the screening test for hyperaldosteronism?

confirmation test?

A
  • Elevated ratio of plasma aldosterone concentration to plasma renin
  • Aldosterone suppression test: Administer oral saline load, IV saline load, or fludrocortisone. If aldosterone is not suppressed then it confirms primary hyperaldosteronism
48
Q

Treatment of hyperaldosteronism

A

varies depending on cause

  • Adenomas: surgical resection
  • Bilateral hyperplasia: aldosterone antagonist (spironolactone)
  • Secondary hyperaldosteronism . .treat underlying cause
49
Q

What are the categories of adrenogenital syndromes?

A
  • Disorders of sexual differentiation: virilization or feminization
  • Primary gonadal disorders
  • Primary adrenal disorders
50
Q

ACTH regulates adrenal androgen formation. What does the adrenal cortex secrete that gets converted to testosterone in peripheral tissues?

A
  • dehyroepiandrosterone

- and androstenedione

51
Q

What adrenocortical neoplasms are more likely to produce adrenogenital syndromes

A

more likely carcinomas

52
Q

What congenital disorder is a “pure” adrenogenital syndrome or as component of Cushing disease

A

Congenital adrenal Hyperplasia (CAH)

53
Q

inheritance of CAH

A

autosomal recessive, inherited metabolic error

54
Q

What enzyme is lacking or deficient in CAH?

A

there are several enzymes that can be affected involving the biosynthesis of cortical steroids, eps. cortisol

  • increase in androgens –>virilization
  • decrease in cortisol –> increase in ACTH and adrenal hyperplasia
55
Q

If an enzyme in CAH impairs aldosterone secretion what happens

A

salt wasting along with virilizing syndrome

56
Q

> 90% of CAH is deficiency in what enzyme

A

21-hydroxylase

57
Q

what mutation involved in 21-hyroxylase deficiency

A

CYP21A2

58
Q

What are the 3 distinctive syndrome found in 21-Hydroxylase deficiency?

A
  • Salt-wasting syndrome
  • Simple virilizing adrenogenital syndrome without salt wasting
  • Nonclassic or late onset adrenal virilism
59
Q

Salt wasting syndrome is the inability to covert _______ into ______

A

progesterone into deoxycoricosterone

60
Q

how does salt wasting syndrome present

A
  • Typically present soon after birth
  • Virilization recognized in the female at birth or in utero
  • Males come to clinical attention 5-15 days later because of some salt losing crisis
61
Q

Describe what happens in salt wasting

A
  • hyponatremia and hyperkalemia
  • Acidosis
  • hypotension
  • cardiovascular collapse
  • possibly death
62
Q

Describe the features of simple virilizing adrenogenital syndrome without salt wasting

A
  • presents as genital ambiguity
  • about 1/3 of 21-hydroxylase deficiency
  • progressive virilization
63
Q

Describe the features of Nonclassic or late onset adrenal virilism

A
  • most common form of 21-hydroxylase deficiency
  • only a partial deficiency
  • Asymptomatic
  • Hirsutism, acne, menstrual irregularities
64
Q

Morphology of CAH

A
  • Adrenals are Bilaterally hyperplastic
  • Cortex is thickened and nodular
  • Cortex looks BROWN: due to total depletion of all lipid
65
Q

Clinical course of CAH

A
  • Androgen excess, with or without aldosterone and glucocorticoid deficiency
  • onset of symptoms can be perinatal period, later childhood, or adulthood (least common)
  • CAH should be suspected in any neonate with ambiguous genitalia
  • severe enzyme deficiency in infancy can be life threatening with vomiting, dehydration, and salt wasting
66
Q

describe in detail 21-hydroxylase deficiency in females

A
  • Clitoral hypertrophy and pseudohermaphroditism in infants

- Oligomenorrhea, hirsutism, and acne in postpubertal

67
Q

Describe in detail 21-hydroxylase deficiency in males

A
  • enlargement of external genitalia and other evidence of precocious puberty in perpubertal pts
  • Oligospermia in older males
68
Q

CAH effects on the Adrenal medulla

A
  • High levels of intra adrenal glucocorticoids are required to make catecholamines
  • pts with severe salt wasting 21 hydroxylase deficiency have low cortisol –> adrenomedullary dysplasia (decreased catecholamine secretion) –> hypotension and circulatory collapse
69
Q

What is the treatment of CAH?

A
  • Exogenous glucocordicoids: replaces the glucocorticoids AND suppresses the ACTH levels which decreases synthesis of the steroid hormones
  • Mineralocorticoid supplementation is required in the salt wasting variants of CAH
70
Q

What is primary acute adrenocortical insufficiency called?

A

Adrenal crisis

71
Q

What is primary chronic adrenocortical insufficiency called

A

Addison disease

72
Q

Secondary adrenocortical insufficiency is from what?

A

decreased stimulation of the adrenal due to deficiency of ACTH

73
Q

Primary acute adrenocortical insufficiency occurs in what settings?

A
  • Crisis
  • Exogenous corticosteroids in whom rapid withdrawal of steroids or failure to increase steroid doses in response to an acute stress
  • Massive adrenal hemorrhage –> damage to adrenal cortex
74
Q

What situations can cause massive adrenal hemorrhage leading to primary acute adrenocortical insufficiency?

A
  • newborns following prolonged and difficult delivery with considerable trauma and hypoxia
  • pts on anticoagulant therapy
  • post surgical patients who develop DIC
  • Disseminated bacterial infections (Waterhouse-Friderichsen syndrome)
75
Q

Waterhouse Friderichsen syndrome is caused by an overwhelming bacterial infections, classically what?

A

-Neisseria meningitides septicemia

Also see Pseudomonas, Pneumococci, H. Flu or even staph

76
Q

Describe the features of Waterhouse Friderichsen syndrome

A
  • uncommon: any age but more common in kids
  • rapidly progressive hypotension leading to shock
  • DIC associated with widespread purpura, particularly of the skin
  • Rapidly developing adrenocortical insufficiency associated with massive bilateral adrenal hemorrhage
  • Fatal unless promptly recognized and rteated
77
Q

Uncommon

  • progressive destruction of adrenal cortex
  • take about 90% of cortex loss to get symtoms
A

Addison Disease

78
Q

What accounts for 60-70% of Addison Disease

A

-Autoimmune Adrenalitis

79
Q

Describe what autoimmune adrenalitis is? . . . . causes Addison disease

A
  • autoimmune destruction of steroidogenic cells

- Autoantibodies to 21-Hydroxylase and 17-hydroxylase have been detected

80
Q

Autoimmune adrenalitis occurs in what two clinical settings

A

-Autoimmune polyendocrine syndrome types 1 and 2 (APS1 and APS2)

81
Q

What is the other name for APS1

A

-Autoimmune polyendocrinopathy, candidiasis, and ectoderma dystrophy (APECED)

82
Q

Describe APS1 (APECED)

A
  • Chronic mucocutaneous candidiasis
  • Abnormalities of skin, dental enamel, and nails (ectodermal dystrophy)
  • Autoimmune disorders: autoimmune adrenalitis, hypoparathyroidism, idiopathic hypogonadism, pernicious anemia
83
Q

What is the mutation in APS1

A
  • AIRE gene on chromosome 21q22
  • central T cell tolerance to peripheral tissues antigens (in the thymus) is compromised –>autoimmunity
  • Also develop autoantibodies against IL-17 and IL-22 (crucial for defense against fungal infections
84
Q

Describe APS2

A
  • usually starts in early childhood

- Presents as a combination of adrenal insufficiency and autoimmune thyroiditis or type 1 diabetes

85
Q

other miscellaneous causes of Addison Disease

A

-Infections: particarly Tb and fungals. AIDS at increased risk from several infections (CMV, MAI) and noninfectious (Kaposi sarcoma)
-Metastatic neoplasms: Lung and breast carcinomas most common. Others: GI carcinomas, melanoma, hematopoietic neoplasms
Genetic: congenital adrenal hypoplasia (rare X linked disease), and Adrenoleukodystrophy

86
Q

Morphology of Primary autoimmune adrenalitis

A
  • irregularly shrunken glands which may be difficult to identify grossly
  • Histologically: cortex contains scattered residual cortical cells in a collapsed network of connective tissue. Variable lymphoid infiltrate
87
Q

Morphology of Tb and fungal disease causing Addison

A

Adrenal architecture is effaced by a granulomatous inflammatory reaction

88
Q

Morphology of a metastatic carcinoma causing Addison

A

normal architecture obscured by the infiltrating neoplasm

89
Q

Early manifestations of Addison Disease

A
  • Progressive weakness and easy fatigability

- often dismissed as nonspecific complaints

90
Q

GI disturbances in Addison

A
  • Anorexia
  • Nausea
  • vomiting
  • Weight loss
  • diarrhea
91
Q

Skin manifestations in Primary Adrenal disease in Addison

A
  • hyperpigmentation of the skin
  • Especially on sun exposed areas and at pressure points (neck, elbows, knees, knuckles
  • Results from elevated levels of POMC .. . precursor of both ACTH and MSH
92
Q

Glucose and Addison Disease

A
  • Hypoglycemia can occur

- from impaired gluconeogenesis

93
Q

Stresses can precipitate an acute adrenal crisis in Addison Disease such as what?

A
  • stresses: infections, trauma, or surgical procedures
  • intractable vomiting, abdominal pain, hypotension, coma, and vascular collapse
  • Death occur rapidly unless corticosteroid therapy begins immediately
94
Q

Secondary adrenocortical insufficiency is caused by what general process

A

any disorder of the hypothalamus and pituitary that reduces the output of ACTH
-Prolonged exogenous glucocorticoids –>suppressed ACTH and adrenal function

95
Q

Contrast Primary from Secondary adrenocortical insufficiency

A

Secodary: don’t see hyperpigmentation
-also low cortisol and androgen output but notmal or near normal aldosterone so you don’t see hyponatremia and hyperkalemia

96
Q

treatment of secondary adrenocortical insufficiency

A

-exogenous ACTH –> prompt rise in plasma cortisol levels

97
Q

how do adrenocortical neoplasma arise

A
  • Majority are sporadic
  • Two familial cancer syndrome with increased risk of adrenocortical carcinomas: Li-Fraumeni sydndrome (germline TP53 mutations) and Beckwith-Wiedemann syndrome
98
Q

Functional and non functional Adrenocortical neoplasm are indistinguishable morphologically so how do you differentiate them

A

clinically and by measuring hormone and hormone metabolites

99
Q

Hyperaldosteronism and Cushing syndrome are more likely from what neoplasm?
Virilizing neoplasm?

A
  • functional Adenoma

- carcinoma

100
Q

Describe Adrenocortical Adenomas

A
  • Most are clinically silent and discovered incidentally
  • Well-circumscribed nodular lesion
  • Up to 2.5 cm in diameter
  • usually yellow to yellow brown from lipids
  • Cortex adjacent to nonfunctional adenoma is normal vs. Functional adenoma where it’s usually atrophic

Endocrine 2 (10 decks)

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