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Endocrine II AH > Adrenal Gland - Guerin > Flashcards

Adrenal Gland - Guerin Flashcards

1
Q

CFs of Addison’s?

Labs?

A

Begins insidiously
Progressive weakness and fatigue
GI disturbance
Hyperpigmentation

Hyponatremia, hyperkalemia

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3
Q

Major morphology of pheochromocytomas?

A

Avg weigh 100gm, range from 1-4000

Small to large hemorrhagic masses

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4
Q

What are the sex steroids found in the adrenal gland cortex?

Made where?

A

Estrogen and androgen

Zona reticularis

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5
Q

CFs of pheochromocytomas?

Dx?

Tx?

A

HTN, palpitations, sweating

Elevated urine VMA and metanephrines

Surgery after admin adrenergic-blocking agent

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9
Q

What is the most common site of gastrinomas in MEN1?

What other tumors?

A

Duodenum

Carcinoid, thyroid, adrenocortical, lipomas

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10
Q

A virilizing neoplasm is more likely a what?

Hyperaldosteronism and Cushing?

A

Carcinoma

Functional adenoma

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11
Q

Endogenous hypercortisolism w/ACTH-depended Cushing syndrome causes what?

A

Diffuse hyperplasia

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12
Q

Stress can precipitate what in Addison’s?

A

Acute adrenal crisis

Hypoglycemia can also occur

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13
Q

Familial medullary thyroid cancer is a variant of what?

Pts are what?

Clinical course?

A

MEN2A

Older

Indolent

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14
Q

What structures are found in the adrenal medulla?

A

Chromaffin cells
Medullary veins
Splanchnic nerves

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16
Q

MEN2B has what tumors?

What mutation?

A

Medullary carcinoma of the thyroid (MORE aggressive than 2A), pheochromocytomas, neuromas, Marfanoid habitus

RET
Sporadic medullary carcinoma is aggressive and has adverse prognosis

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17
Q

What are 2 familial cancer syndromes w/inc risk of adrenocortical carcinomas?

A

Li-Fraumeni syndrome (TP53 mutation)

Beckwith Wiedemann

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19
Q

Phechromocytomas are composed of what?

Rule of 10s?

A

Chromaffin cells

10% have:
paragangliomas
Sporadic are B/l
Malignant
No HTN
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20
Q

Glucocorticoids induce ______ and inhibit _____?

A

Gluconeogenesis

Uptake of glucose by cells

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22
Q

90% of CAH occurs due to what?

Mutation where?

A

21-Hydroxy deficiency

CYP21A2

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27
Q

Adenomas causing hyperaldosteronism are usually solitary or multiple? What location?

Size?

Color?

Characteristic feature?

A

Solitary, Left > right

Small (> 2cm)

Bright yellow grossly

Spironolactone bodies (eosinophilic cytoplasmic inclusions)

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30
Q

Any disorder of the hypothalamus and pituitary that reduces the output of ACTH can cause what?

What else?

A

Secondary Adrenocortical Insufficiency

Exogenous glucocorticoids

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31
Q

What causes secondary Adrenocortical insufficiency?

A

Def of ACTH

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32
Q

What is the most common cause of an adrenocortical neoplasm?

Called what?

What demographic?

A

Adenoma

Conn syndrome

Middle aged women (30-40 y/o)

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33
Q

Primary adrenocortical neoplasms occur most often in whom?

Functioning tumors cause what?

A

Women aged 30-50

Atrophic adrenal cortex and contralateral adrenal gland

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34
Q

What system is composed of adrenal medulla?

A

Paraganglion

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35
Q

The pineal gland secretes what?

Although rare, what tumors are there?
Resemble what?

A

Melatonin

Germ cell tumors
Testicular seminomas

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36
Q

tumors of MEN syndromes are typically what compared to sporadic tumors?

What stage precedes tumor?

A

More aggressive

Asymptomatic Hyperplasia

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37
Q

Aldosterone released in response to the activation of RAAS by Inc renin is what?

A

Secondary hyperaldosteronism

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39
Q

Inability to convert progesterone into deoxycorticosterone is what?

In females presents when? Males?

CFs?

A

Salt-wasting syndrome

At birth/in utero
Males 5-15 days after birth

Hyponatremia and hyperkalemia -> acidosis, HTN, CV collapse

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41
Q

Primary acute adrenocortical insufficiency occurs in what settings?

A

Crisis due to stress
Exogenous corticosteroids w/rapid withdrawal or failure to INC dose w/stress
Massive adrenal hemorrhage due to damage of adrenal cortex

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42
Q

What happens when the pituitary shows Crooks hyaline change?

Found in what?

A

Basophilic cytoplasm becomes homogeneous and pale

Cushing’s

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44
Q

What is the most common CF in hyperaldosteronism?

Effects on CV system?

What else?

A

HTN

LVH -> Dec diastolic volume, stroke and MI

Weakness, parasthesias, visual disturbances

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46
Q

Tx of CAH?

A

Exogenous glucocorticoids and mineralcorticoids for salt-wasting variants

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47
Q

Describe adrenocortical adenomas

A

Well-circumscribed nodular
Yellow to yellow-brown
Cortex adjacent to nonfx adenoma is normal

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49
Q

Describe morphology of CAH

A

Adrenals are b/l hyperplastic

Cortex thickened and nodular, and brown

51
Q

Adrenocortical carcinomas invade what?

Pt survival?

Size?

A

Adrenal vein, vena cava, lymphatics

2 yrs

Large and invasive, > 20 cm

53
Q

What has the morphology of irregular shrunken glands w/a cortex containing scattered residual cortical cells in a collapsed network of ct?

A

Primary autoimmune adrenalitis

54
Q

What is the most common reason for ectopic ACTH-dependent Cushing Syndrome?

A

Small-cell carcinoma of the lung

56
Q

What 3 things can cause primary hyperaldosteronism?

A

Adrenocortical neoplasm
Bilateral idiopathic hyperaldosteronism
Glucocorticoid-remediable hyperaldosteronism

57
Q

pineal gland composed of what?

What functions?

A

Pineocytes

Photosensory and neuroendocrine

58
Q

Tx of bilateral hyperplasia?

Secondary hyperaldosteronism?

Adenoma?

A

Spironolactone

Tx underlying cause

Surgery

60
Q

What are the causes of ACTH independent Cushing syndrome?

A

Adrenal adenoma or carcinoma
Macro nodular hyperplasia
Primary pigmented nodular adrenal disease
McCune-Albright syndrome

62
Q

MEN2A has what tumors?

Called what?

Mutation in what gene?

A

Pheochromocytomas, medullary carcinoma of thyroid (usually AGGRESSIVE), parathyroid hyperplasia

Sipple syndrome

RET

64
Q

What is the Diagnose of the following?

ACTH Inc
Dexamethasone suppression low dose = no suppression
Dexamethasone suppression high dose = no suppression

A

Ectopic ACTH

65
Q

21-H deficiency in males causes what CFs?

A

Enlargement of external genitalia, precocious puberty

Oligospermia in older males

66
Q

What is the screening test for primary hyperaldosteronism?

Confirmation test?

A

Elevated ratio of plasma aldosterone to renin

Aldosterone suppression test
Admin saline and if aldosterone is not suppressed then confirmed primary

67
Q

Familial pheochromocytomas occur more often with what?

Pts?

A

50% b/l
25% paragangliomas

Younger

69
Q

What is autoimmune destruction of steroidogenic cells?

Antibodies to what?

Occurs in what setting?

A

Autoimmune adrenalitis

AutoAbs to 21-H and 17-H

APS1 and APS2

71
Q

What presents as genital ambiguity w/progressive virilization w/1/3 of 21-H def?

A

Simple virilizing andreogenital syndrome w/out salt wasting

73
Q

How to Dx Cushing?

How to determine the cause?

A

Increased 24-H urine free cortisol

Serum ACTH
Dexamethasone suppression test: urinary excretion of 17-H after admin

74
Q

What causes glucocorticoid-Remediated hyperaldosteronism?

ACTH does what?

Tx?

A

Mutated CYP11B2 on ch. 8

ACTH stimulates production of aldosterone

Dexamethasone

76
Q

What is the most common CAH w/only a partial def in 21-H?

Sx?

A

Nonclassic or Late-onset adrenal virilism

Asymptomatic or hirsuitism, acne, menstrual irregularity

77
Q

Primary adrenocortical insufficiency is called what?

Caused by what?

A

Addision’s

Progressive destruction of adrenal cortex (need 90% destruction)

78
Q

What syndromes have pheochromocytomas?

A

MEN2A, MEN2B
NF1
VHL
SDHD-B

82
Q

APS2 starts when?

Presents how?

A

Early adulthood

Combo of AI, autoimmune thyroiditis or T1DM

83
Q

MEN1 has what tumors?

Called what else?

Mutations in what?

A

Primary hyperparathyroidism, endocrine tumors of pancreas, pituitary (PRL)

Wermer Syndrome

Menin protein (MLL, JunD)

85
Q

Chromaffin cells are from what?

A

Neural crest

86
Q

What are some random other causes of Addison’s?

A

Infections (Tb and fungal, AIDS)
Lung and breast carcinoma
Congenital adrenal hypoplasia
Adrenoleukodystrophy

88
Q

Adrenocortical adenomas are what color?

Weight what?

Composed of what cells?

A

Yellow w/well-developed capsule

< 30 gm

Look like normal ZF

91
Q

What causes Waterhouse-Friderichsen Syndrome?

CFs?

A

N meningitidis, pseudomonas, pneumococci, H influ, staph

Rapidly progressive hypotension leading to shock
DIC w/purpura
Massive b/l adrenal hemorrhage

92
Q

What is congenital adrenal hyperplasia (CAH)?

Inheritance?

A

Deficiency/lack of an enzyme in biosynthesis of cortical steroids (esp. cortisol)

AR

93
Q

What is the Diagnose of the following?

ACTH Inc
Dexamethasone suppression low dose = no suppression
Dexamethasone suppression high dose = suppression

A

Pituitary Cushing

97
Q

What is the most common cause of primary hyperaldosteronism?

Morphology?

Pt demographic?

A

Bilateral Idiopathic Hyperaldosteronism

Bilateral nodular wedge-shaped hyperplasia of the adrenal glands

Older

98
Q

Autoimmune adrenalitis, hypoparathyroidism, idiopathic hypogonadism and pernicious anemia associated with what?

Mutation in what?

Develop autoAbs to what?

A

APS1

AIRE on ch. 21q22

IL-17 and IL-22 (need to fight fungus)

100
Q

21-H deficiency in females causes what CFs?

A

Clitoral hypertrophy, pseudo hermaphroditism in infants

Oligomenorrhea, hirsutism, acne

101
Q

What does primary hyperaldosteronism do to the RAAS system?

Plasma renin?

Most common manifestation?

A

Suppresses it

Dec

Elevated BP

102
Q

Early CF of Cushing’s?

Later?

A

HTN, weight gain

turn all obesity, moon facies, buffalo hump

103
Q

What are the most common causes of ACTH-independent Cushing syndrome?

Cortisol level?
ACTH?

A

Adrenal adenoma and carcinoma

Inc
Dec

104
Q

What secondary diabetic effects may occur w/glucocorticoid excess?

A

Hyperglycemia
Glucosuria
Polydipsia

107
Q

What the CAH effects on the adrenal medulla?

A

Hypotension and circulatory collapse due to DEC catecholamines

111
Q

Pts w/ familial idiopathic hyperaldosteronism have mutations in what?

A

KCNJ5, encodes K+ channel

112
Q

What is secondary hyperaldosteronism seen in?

A

Dec renal perfusion
Arterial hypovolemia and edema
Pregnancy

114
Q

What hormones are found in the adrenal medulla?

A

Catecholamines - Epinephrine and Norepinephrine

116
Q

What is a mutation activating GNAS which causes Inc Gs, Inc cAMP and primary macronodular hyperplasia?

A

McCune-Albright syndrome

122
Q

What is the Diagnose of the following?

ACTH Dec
Dexamethasone suppression low dose = no suppression
Dexamethasone suppression high dose = no suppression

A

Adrenal tumor

123
Q

Adrenocortical carcinomas are what size?

What important feature?

A

> 200-300 gm

Unencapsulated

124
Q

What is composed of 1-3 mm darkly pigmented (brown/black) micronodules w/atrophic intervening areas w/Endogenous hypercortisolism?

Pigment is what?

A

Micronodular hyperplasia

Lipofuscin

131
Q

What is described by adrenals almost entirely replaced by prominent nodules of varying size (< 3cm), and areas between the macroscopic nodules also demonstrate evidence of microscopic nodularity?

Due to what?

A

Macronodular hyperplasia

Endogenous hypercortisolism

141
Q

The majority of ACTH-secreting pituitary adenomas are what?

Account for what % of endogenous hypercortisolism?

Most frequently in whom?

A

Microadenoma

70%

Young adults

143
Q

Exogenous glucocorticoids cause what morphology of adrenal glands?

A

Cortical atrophy, lack of stimulation of ZF and ZR

147
Q

What are the causes of ACTH dependent Cushing syndrome?

A

Pituitary ademona

Ectopic corticotropin syndrome (ACTH)

Endocrine II AH (6 decks)

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