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88 Cards in this Set

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  • Back
What happens in adrenal insufficiency?
Adrenal glands are pooped: inappropriately low adrenal steroid output:

Mineralocorticoids
Glucocorticoids
Sex steroids
What happens in cushing's?
Too much cortisol
What happens in primary hyperaldosteronism?
Too much aldosterone
What is the main flavor of adrenal insufficiency?
Cortisol deficiency
What does the HPA axis look like in primary adrenal insufficiency?
What does the HPA axis look like in secondary adrenal insufficiency?
What does the HPA axis look like in tertiary adrenal insufficiency?
What's the key molecule for differentiating between primary and secondary adrenal insufficiency?
Primary: aldo will be low

Secondary: aldo won't be abnormal
Primary: aldo will be low

Secondary: aldo won't be abnormal
What is the main cause of adrenal insufficiency? Second most common?
Autoimmune adrenalitis

Pharmacologic dose of glucocorticoids
What's the most common cause of adrenal insufficiency in the elderly?
Pharmacologic doses of glucocorticoids
What are some of the causes of primary adrenal insufficiency?
Autoimmune adrenalitis (80%)
Adrenal Tb (20%)
Adrenal hemorrhage
What can autoimmune adrenalitis happen in the context of, often?
Polyglandular autoimmune syndromes:
-Polyglandular AI syndrome I
What are the diagnostic criteria for polyglandular autoimmune syndrome I?
2 of the following:
-Adrenal insufficiency (<15 YO)
-Hypoparathyroidism (<10 YO)
-Chronic mucocutaneous candidiasis (<5 YO)
What are the symptoms of primary adrenal insufficiency?
Cortisol related:
Fatigue
Weakness and malaise
Anorexia
Nausea, vomiting

Aldosterone related:
Dizziness
What are signs of primary adrenal insufficiency?
Proximal muscle weakness
Orthostatic hypotension
Hyperpigmentation (PRIMARY AI ONLY.)
HypoNa, HyperK (PRIMARY AI ONLY.)
Where do you look for hyperpigmentation in primary adrenal insufficiency?
Palmar creases
Palmar creases
Does primary aldosterone deficiency happen in isolation?
NO.
What are the electrolyte abnormalities in aldosterone deficiency?
What is the cause of hyponatremai in primary adrenal insufficiency?
1. Lack of aldosterone-->can't reabsorb Na-->volume depletion
2. ADH secretion to reabsorb water to perrfuse essential organs
3. Decreased serum osmolality, Na concentration -->hyponatremia
What are the symptoms of cortisol deficiency?
Hypotension
Hypoglycemia
Fatigue
What are the symptoms of DHEAs deficiency?
Fatigue
Depressed mood
Libidinal dysfunction
What is the source of DHEAS?
Adrenal gland.
What is the main source of androgens in males? Females?
Males: testicles

Females: adrenals
What is DHEA a precursor to?
Testosterone
If a woman doesn't have an adrenal gland and doesn't have a libido either, what can you give her?
DHEAS
What occurs in an adrenal crisis?
Acute CV collapse due to adrenal insuficiency
What are the causes of an adrenal crisis?
Hemorrhage
Infection
Withdrawal of exogenous glucoccorticoids
What is Waterhouse-Friderichsen syndrome?
Traditionally, Neiserria induced destruction of the adrenal gland
What are drugs that increase the metabolism of GCs?
Phenytoin
Phenobarbitol
Rifampin
What are the drugs that decrease the synthesis of GCs?
Ketoconazole
Mitotane
Metyrapone
What are causes of secondary and tertiary insufficiency?
Vascular
Lymphocytic hypophysitis
Infiltritative diseases: sarcoid, histocytosis
Tumor compression
Surgery
What are the symptoms of secondary adrenal insufficiency?
Mild malaise, fatigue
Proximal weakness
What are the signs of secondary adrenal insufficiency?
Milder than primary

NO hyperpigmentation
NO orthostatic hypotension
What IS NOT deficient in secondary adrenal insufficiency?
Aldosterone
When should you have a high index of suspicion for an adrenal crisis?
Catecholamine resistant hypotension
Hypotension with abdominal pain

Hyperpigmentation
Decreased pubic hair
Hyperkalemia
Hyponatremia
hypoglycemia
What's the testing for adrenal insuficciency?
AM cortisol: rule out disease
-If high, DISEASE IS RULED OUT.
-If exceptionally low, it's diagnostic
What's the gold standard for adrenal insufficiency?
ACTH stimulation test
What's the interpretation of the ACTH stimulation test?
Subnormal cortisol levels: diagnostic of AI

ACTH extremely high: consistent with diagnosis, but not diagnostic.
What's the diagnostic test for secondary adrenal insufficiency?
Insulin hypoglycemia test.
What's the underlying pathophysiology beneath secondary adrenal insufficiency?
The gland atrophies. When you then challenge it, it cannot respond.
What happens in an insulin hypoglycemia test?
You initiate hypoglycemia via insulin.

If you have subnormal cortisol--->diagnosis of AI
If you have ACTH that is subnormal--> diagnosis of secondary AI
What's the management for adrenal crisis?
IV fluids
IV cortisol
Glucose
Treat underlying cause!

DON'T WAIT FOR LABS.
What is the maintenance therapy for adrenal insufficeincy?
Hydrocortisone
Fludrocortisone
What is off in Cushing's syndrome?
Cortisol.
What is a cause of catecholamine excess?
Pheochromocytoma
What are the symptoms of a pheochromocytoma?
Headache
Sweating
Palpitation
panic
HTN
What occurs in 21 alpha hydroxylase deficiency?
Lack of aldosterone
Lack of cortisol
What happens when there's an isolated step blocked in steroid production?
Shunting down an alternate path
Build up of precursors before the block
What happens in severe forms of adrenal insensitivity syndrome? Milder forms?
Severe: virulization of females
Milder: hirsuitism + menstrual abnormalities
What is the function of testosterone in a male fetus?
Development of sexual characteristics
What's the most common gonadal steroid defect?
Androgen insensitivity syndrome
What underlies androgen insensitivity syndrome?
Loss of function at the androgen receptor
What is the pathophys of androgen insensitivity syndrome?
Block in androgen activiyt leads to increased testosterone

High levels of testosterone get converted to estrogens peripherally
What are the different types of Cushing's?
Adrenal: autonomous production of Cortisol; low ACTH

ACTH dependent; high ACTH
What are some causes of adrenal steroid excess?
Adrenal tumor (cortisol producing)   - left
Pituitary tumor (ACTH producing)  -right
Other tumors (ectopic ACTH/CRH)
Adrenal tumor (cortisol producing) - left
Pituitary tumor (ACTH producing) -right
Other tumors (ectopic ACTH/CRH)
What are the hormone changes in adrenal steroid excess?
What is Cushing's disease?
ACTH producing tumor in the PITUITARY!

It's a nomenclature thing.
What are the different kinds of cushing's syndroem?
How common are the different flavors of Cushing's?
85% pituitary
15ish% adrenal
1% ectopicc
If Cushing's is ACTH dependent, what kind is it likely to be?
Pituitary adenoma
What occurs to the adrenals in ACTH dependent Cushing's?
Bilateral hyperplasia
What kinds of tumors can cause ACTH dependent Cushing's?
Bronchial carcinoid
Oat cell carcinoma
THymic carcinoid
Pheochromocytoma
Medullary thyroid cancer
What are underlying causes of ACTH-indpendent cushing's?
Adrenal carcinoma
Adrenal adenoma
What happens to the adrenal glands in an ACTH independent Cushing's?
One is big (the one with the tumor, that is.)

The contralateral gland is SMALL!
If you take a primary adrenal tumor out that has been causing ACTH independent Cushing's out, what do you need to give the patient?
Steroid replacement: you don't want to just shove them into adrenal insufficiency
What are the responses of the body to cortisol overproduction?
Glucose production
Muscle breakdown
Fat production
What are the manifestations of cortisol excess?
HTN
DM
Obesity
Muscle wasting
What are the clinical manifestations of cortisol excess?
Increased protein catabolism
DM
Truncal obesity
Osteoperosis
HTN
Opportunistic infections
Amenorrhea, impotence
What finding will you see only with an ACTH dependent cushing's?
Pigmentation due to MSH
How do we go about diagnosing Cushin's?
1. Diagnose cortisol excess
2. Diagrnose the pathologic cortisol excess
3. See if it's ACTH dependent or not.
How do you check for a pathologic cortisol excess?
See if the axis is suppressed with low-dose dexamethasone
IN what patients will low dose dexamethasone have no effect?
ACTH dependent cushing's due to a pitituitary adenoma or ectopic ACTH

ACTH independent cushings due to adrenal tumors
In what patients will low-dose dexamethasone have an effect?
Normal patients
Physiologic hypercortisolism
How do you differentiate between a pituitary or exctopic cause of cushing'?
High dose DEX

Ectopic tumors NEVER suppress to high dose DEX
Pituitary MAY suppress

Inferior petrosal sinus sampling:
-Measure ACTH levels in different placeces (near pituitary, in the periphery)
What are the most common types of ectopic tumors?
Lung (SCC, bronchial)
What's the treatment for Cushing's?
Adrenal adenoma: resect, replace cortisol

Pituitary adenoma: transphenoidal resection, cortisol replacement

Ectopic: FIND THE TUMOR.
How does a female present with adrenal steroid excess?
Hirsuitism
Cliteromegaly
Amenorrhea
Acne
How does a male present with adrenal sex steroid excess?
Gynecomastia
Erectinal dysfunction
Libidinal dysfunction
What are the symptoms of aldosterone excess?
HTN
Low potassium
What are the symptoms of low potassium?
NM problems: paresthesias, weakness, tetany
What are the the other symptoms of hypoaldosteronism?
NM problems due to K
Renal: polyuria
Carbs: abnormal glucose tolerance test
HTN
What is the quality of the HTN during hypoaldosteronism?
It's not malignant
What are causes of hyperaldosteronism?
Renal tumor making aldosterone (MOST COMMON)
Hyperplasia of the glomerulosa
JGA tumor making renin
Receptor problems.

RENAL ARTERY STENOSIS!
What do you need to find out about hyperaldosteronism?
Is the aldo indpendent or not.
How do you check to see if a tumor is renin caused or not?
Give the person volume.
-If both go down, then they're just volume depleted
-If just renin goes down, it's hyperaldosteronism
-If neither goes down, it's either renal artery stenosis or a renin-producing tumor
What are the most common causes of hyperaldosteronism?
ZG aldo tumor - 70%
ZG aldo hyperplasia - 30%
What's the workup for hyperaldosteronism?
1. Work up other causes of low K

24 hr. urine aldosterone
-If high, then they're hyperaldosterone

Then, check renin
-If high, JGA renin tumor or RAS
-If low, primary hyperaldosteronism
What do you do after you confirm hyperaldosteronism?
CT scan: look for tumor.
Look to see if there's high aldo on 1 side via a catheter.