Lecture 92, 94 - Adrenal Disorders + Adrenal Steroid Pharm Flashcards

(36 cards)

1
Q

labs of primary adrenal insufficeincy

A

Low cortisol;

High ACTH, CRH

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2
Q

etiologies of acute adrenal insufficiency

A

massive hemorrhage
Tumors
Drugs (ketoconazole, Etomidate)
abrupt withdrawal of glucocorticoids

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3
Q

etiolgoes of chronic adrenal insuffieicny

A

TB Adrenalitis

Auto immune (APS 1 – APECED, APS 2)

XLALD

CAH

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4
Q

what is APECED (APS1)

defect to what gene?

A

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy

AIRE gene

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5
Q

what is APS2?

A

Genes: AD with incomplete penetrance
More Common than APS1

Hallmark: Adrenal Insufficiency + Thyroid Disease (Hypo or Hyper)

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6
Q

what is XLADL

manifestation

A

XL genetic mutation to ATP binding casettes which code for proteins necessary to break down long chain fatty acids

CNS, AI

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7
Q

what is the most common defect of Congential Adrenal Hyperplasia

how may it presetnt early in life?

A

21 Alpha Hydroxylase

most common cause of salt wasting adrenal crisis in the first two weeks of life

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8
Q

in 21 alpha hydroxylase deficincey – what features may present in females

A

virulizing effects

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9
Q

labs of secondary adrenal failure

A

Labs: Low Cortisol; Low ACTH (CRH)

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10
Q

what is the most common etiology for secondary adrenal failure?

A

Pituitary/Hypothalamic Tumor

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11
Q

hyperpigmentation is present in ______ adrenal fuction.. why?

A

If Primary AI – Hyperpigmentation (bc ACTH –> MSH)

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12
Q

serum electrolyte values of AI

what changes to the CBC?

A

Low adolsterone –

Hyponatremia
Hyperkalemia
Hypoglycemia
Hypercalcemia – cortisol nomrally decreases intestinal calcium
Eosinophilia (cortisol is toxic to eosinophils)

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13
Q

gold standard for diagnosis of AI

other good test ?

A

ACTH Stimulation Test – GOLD STANDARD

Primary – no rise in cortisol

Secondary – some response to cortisol

Morning cortisol check:
Cortisol should be high in the morning. If Low, check ACTH to determine if primary or secondary

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14
Q

Treatment for AI

A

Hydrocortisone – but this is short acting and requires multiple injections per day

Fludrocortisone – Mineralocorticoid and Glucocortisol effects

DHEA – replacement of Androgens for Women

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15
Q

what is cushing’s syndrome

what is cushing’s disease

A

syndrome – excess cortisol production

Disease – ACTH producing adenoma

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16
Q

Causes of Cushing’s Syndrome

A

Unilateral Adrenal Adenoma;

Bilateral Adrenal Hyperplasia (usually bilateral)

17
Q

three tests to help dx cushing’s

A

Dexamethasone Suppression Test (ACTH doesn’t decrease)

24 hour urine – increased

Late night salivary cortisol – higher than it should be

18
Q

clinical features of cushing’s

A

muscle weakness and thin extremitiis

Moon facies, buffalo hump, truncal obesity

high insulin

abdominal stira

HTN

Osteoporsis

Immune suppression; poor wound healing

19
Q

Hyperaldosteronism

what is conn syndrome?
what is the most common cause?

A

Most common cause: bilateral adrenal hyperplasia

Conn syndrome – adrenal adenoma over producing aldosterone

20
Q

Clinical presesntation of hyperaldosterone

A

hypernatremia; HTN
Hypokalemia
weakness, cramps, periodic paralysis

21
Q

best way to diagnose and locate hyperaldosterone

A

CT – 1/3 of time its unilateral; if B

Adrenal Vein Sampling – -the gold standard

22
Q

treamtnet for hyperaldosterone

unilateral vs bilateral

A

Surgery – if unilateral

If Bilateral -- Medical: Spironolactone, Eplerenone
23
Q

what is a pheochromocytoma

A

Catecholamine producing tumor of the adrenal medulla
“attacks” of release of catecholamines;

Symptoms can be paroxysmal

24
Q

classic triad of symptoms of pheo

other sx:

A

HA, Diaphoresis, Palpitations

HTN, Diaphoresis, Palpitations

25
diagnosis of pheo
measure Metanephrines Clonidine suppression test
26
4 familial forms of pheo
VHL NF1 MEN2A MEN2B
27
MEN 2a vs MEN2B -- what is the associated mutation
RET gene
28
Treatment of Pheo --
alpha and beta blockers A before B) CCBs Metyrosine Surgery
29
which glucocorticoid agonist has the highest anti-inflammatory potency ? whaich has the highest NA retntion potency?
anti-inflammatory Dexamethasone 9 alpha Fluorocortisol -- highest Na Retention potency;
30
indications for glucocorticoid use
Hormone Replacement Therapy Suppress Inflammation and Immune Responses
31
what are the primary targets of Cortisol in the periphery
Liver and Kidneys --
32
what is the difference between 11B HSD 1 and 11B HSD 2
Glucocorticoid Receptor Rich Tissues: Express High level of 11 Beta HSD Type 1: converts cortisol to active form (cortisone --> Cortisol) Mineralocorticoid Receptor Rich Tissues: Express High level of 11 Beta HSD Type 2: converts cortisol to inactive form (cortisone)
33
indications for glucocorticoid antagonists
Severe Hypercoretisolism Persistent Hypercortisolism following surgery Cushings disease
34
Synthesis inhibitors of Glucocorticoids
Ketoconazole -- Mitotane -- Metyrapone
35
Mineralocorticoid Receptor Agonists
Fludrocortisone
36
MR Antagonists
Spironolactone (+ Eplerenone) --