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Flashcards in Adrenal Deck (13):
1

Congenital adrenal hyperplasia
(CAH)

Most common cause of adrenal insufficiency in infants

Shocks/septic picture
Females: ruggated labia, clitoral hypertrophy
Males: excessive scrotal pigmentation

Due to increases androgen levels

Autosomal recessive

There is a late form CAH too. Growth delay.

2

Most common CAH enzyme deficiency

21-hydroxylase deficiency

21 hydroxylase produces aldosterone and cortisol normally

High levels of 17-hydroxyprogesterone (lab) when 21 hydroxylase is deficient (precursor)
-elevated testosterone

Family member taking testosterone gel for hypergonadism, cross contamination in patient

Checked for on PKU. If positive, repeat newborn screen. If positive, check BMP and urinary sodium/potassium excretion.

Prenatal screening: genetic test of fetal cells. Not in amniotic fluid.

3

The other type of CAH

Salt wasting

Hyponatremia

Hyperkalemia

4

CAH treatment

Glucocorticoid: hydrocortisone

In high doses, has a mineralocorticoid affect too.

Glucocorticoid: cortisol (regulates sugar and response to stress)
Mineralocorticoid: aldosterone

5

Adrenal crisis

Medical emergency
Shock
Death
Usually primary AI due to mineralocorticoid issues.
Hypoglycemia
Hyponatremia
Hyperkalemia
High ADH (appropriate)
Abdominal pain, vomiting, weakness, fever, hypertension, AMS, anorexia, lethargy

6

Adrenal crisis treatment

20 cc/kg D5NS (no K) over 1 he
Then add IV hydrocortisone
Then add glucocorticoid replacement

7

Primary adrenal deficiency

A.k.a. Addison disease

Autoimmune, infection, idiopathic

Elevated ACTH levels (appropriate brain response)
--> Hyperpigmentation (stimulates melanin production)
Cosyntropin (synthetic ACTH) stimulation test aka ACTH Stimulation test. No increase in cortisol

Weight loss, fatigue, salt wasting, hyperkalemia, hyponatremia

8

Primary adrenal deficiency treatment

Fludrocortisone: mineralocorticoid replacement

Hydrocortisone: glucocorticoid replacement

9

Secondary adrenal deficiency

Adrenal glands normal
Problem with pituitary or hypothalamus

Low ACTH levels
ACTH stimulation test: rise in cortisol levels
Low CRH = problem with hypothalamus

Midline defect: cleft lip/palate (pituitary issue)
Normal aldosterone: no salt wasting, hyponatremia, hyperkalemia.
No hyperpigmentation (low ACTH)

10

Cushing syndrome versus Cushing disease

Syndrome: glucocorticoid excess of any origin

Disease: excess corticotropin (ACTH) by pituitary gland --> excess cortisol production by adrenal gland

11

Cushing features

Acne
purple striae
hirsutism
Virilization
buffalo hump
increased BMI, GROWTH ARREST
delayed bone age

most common cause: chronic use of topical, inhaled or oral corticosteroids
-Tx: stop meds

Infants: McCune Albright syndrome

12

Gold standard test for Cushing's

Highest sensitivity test

The other test

24-hour urinary free cortisol excretion

Midnight sleeping plasma cortisol level

Dexamethasone suppression test
-normal would be low cortisol
-abnormal would be elevated cortisol

13

Lab finding for Cushing's syndrome from adrenal tumor

Undetectable morning corticotropin (ACTH) level

Next step: adrenal CT/MRI

If high corticotropin level, then it's from the pituitary. Next step is pituitary MRI