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Endocrine > Adrenal Disorders in Infants > Flashcards

Adrenal Disorders in Infants Flashcards

1
Q

What are the cells of the adrenal medulla that produce hormones?

A

Chromaffin cells

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2
Q

What are the cells of the parathyroid gland that secreted PTH?

A

Chief cells

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3
Q

What is the MOA of ketoconazole?

A

Blocks 17-alpha-hydroxylase

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4
Q

What is the most common cause of congenital adrenal hyperplasia?

A

Deficiency/defect in 21-hydroxylase

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5
Q

What are the deficiencies associated with 21-hydroxylase deficiency?

A

Loss of mineralocorticoids and glucocorticoids, overproduction of androgens

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6
Q

What are the classic lab findings of 21-hydroxylase deficiency?

A

Hyponatremia
Hyperkalemia
Low BG

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7
Q

What are the two major forms of 21-hydroxylase deficiency?

A

Salt losing (“Classic”) vs non-salt losing (“non-classic”)

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8
Q

What are the clinical symptoms of CAH in females?

A

Ambiguous genitalia (clitoral enlargement, fusion and formation of urogenital sinus

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9
Q

What are the clinical symptoms of CAH in males?

A

Subtle hyperpigmentation of scrotum or enlarged phallus

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10
Q

What are the clinical symptoms with 21-hydroxylase deficiency?

A

FTT
Emesis
Dehydration

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11
Q

What is the non-classic form of CAH? What are the ssx of females vs males?

A

Non-salt losing form–
females = symptoms appear later in life as precocious puberty

Males = symptoms of early virilization at 2-4 yo

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12
Q

What is the enzymatic difference between classic and non-classic 21-hydroxylase deficiency?

A

Classic is full defect

non-classic is mild defect

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13
Q

What are the defects seen with 11-beta-hydroxylase deficiency? Why?

A

11-deoxycorticosterone still has some aldosterone-life effects, thus no salt wasting seen in 21-hydroxylase deficiency

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14
Q

What are the ssx of 11-beta-hydroxylase deficiency?

A

HTN
Virilization
No salt loss

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15
Q

What are the lab findings with 11-beta hydroxylase deficiency?

A

Hypokalemia

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16
Q

What is 17-alpha-hydroxylase deficiency?

A

Lack of androgen and cortisol production, causing excess aldosterone, HTN, ad hypokalemia

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17
Q

What is the best way to diagnose 21-hydroxylase deficiency?

A

Draw 17-hydroxyprogesterone levels (which will be supe high)

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18
Q

What are the ACTH and renin levels with 21-hydroxylase deficiency?

A

Both high

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19
Q

What is the gold standard for diagnosing 21-hydroxylase deficiency?

A

Cosyntropin (synthetic ACTH) stimulation test

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20
Q

What molecule is elevated with 11-beta-hydroxylase defect?

A

11-deoxycorticosterone and 11-deoxycortisol

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21
Q

What are the chemicals that are elevated with 17-hydroxylase deficiency?

A

Elevated aldosterone levels

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22
Q

How do you manage 21-hydroxylase deficiency?

A

Exogenous hydrocortisone and fludrocortisone

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23
Q

What is the treatment for the ambiguous genitalia that can occur with 21- or 11B hydroxylase deficiency?

A

Surgical correction

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24
Q

What is the synthetic aldosterone given to patients with 21 or 11B hydroxylase deficiency?

A

Fludrocortisone

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25
Q

Lack of pubic hair is classic for what type of pituitary pathology? Why?

A

Prolactinoma, since prolactin inhibits LH/FSH secretion, causing a loss of estrogen

26
Q

What are the two hormones that act to induce and suppress prolactin secretion?

A

Dopamine inhibits

TRH stimulates

27
Q

What is the natural course of addison’s disease?

A

Slow progression, with ssx appearing around age 10-14

28
Q

What are the causes of Addison’s disease?

A

Autoimmune or TB

29
Q

What is Waterhouse-Friderichsen syndrome?

A

Hemorrhage into the adrenal glands causing Primary adrenal insufficiency

30
Q

What is the classical cause of Waterhouse-Friderichsen syndrome?

A

Neisseria meningitidis infection

31
Q

What is autoimmune polyglandular syndrome type 1?

A

Hypoparathyroidism
Chronic mucocutaneous candidiasis
Adrenal insufficiency

32
Q

What is autoimmune polyglandular syndrome type 2?

A

Adrenal insufficiency
Thyroiditis
DM I

33
Q

Which is more common, APS type 1 or 2?

A

2

34
Q

What are the ssx of APS type 1/ 2?

A

Muscular weakness
Dehydration
Weight loss
Hypotension

35
Q

What are the electrolyte abnormalities that can occur with Addison’s disease?

A

Hyponatremia
Hyperkalemia
Hypoglycemia

36
Q

How do you confirm adrenal insufficiency?

A

Low Morning cortisol levels

37
Q

What is the confirmatory test for primary adrenal insufficiency?

A

Cosyntropin test (synthetic ACTH)–given and if there is no increase in cortisol, then primary

38
Q

What is the most common cause of Cushing’s in Infants? Children?

A 
Infants = Adrenal tumor
Children = pituitary adenoma
39
Q

What are the two most common malignancies that produce ACTH?

A

Small cell lung carcinoma

Renal cell cancer

40
Q

What lung cancer typically leads to the development of gynecomastia?

A

Large cell carcinoma

41
Q

What is Eaton-Lambert syndrome, and what type of lung cancer can cause this?

A

Autoimmune attack against Ca channels–gets better with more muscle movement

SCLC

42
Q

What lung cancer leads to PTHrP secretion?

A

Squamous cell CA

43
Q

What sign/symptom of cushing’s is specific to children?

A

Growth retardation

44
Q

What will a growth pattern plot show with Cushing’s?

A

Slowed height, gain in weight

45
Q

How do you diagnose Cushing’s?

A

24 hours urine cortisol

46
Q

What is the low dose dexamethasone test used to differentiate? High dose?

A

Low dose = if primary or secondary

High dose = Pituitary or ectopic ACTH secretion

47
Q

What is the treatment for Cushing’s?

A

Treat the underlying cause–stop exogenous steroids, excise a tumor etc

48
Q

What are the cells that a pheochromocytoma arises from?

A

Chromaffin cells

49
Q

What are the two major areas outside of the adrenal glands where pheochromocytomas usually arise from?

A

Superior and inferior para-aortic regions

50
Q

What are the three hereditary disorders that pheochromocytoma are usually found in? What is the genetic defect in these?

A

MEN2A and MEN2B
RET protooncogene

VHL
VHL tumor suppressor gene on Chr 3

51
Q

What are the diseases that are associated with MEN2A? Does this have marfanoid ssx?

A

-Medullary thyroid cancer
-Pheochromocytoma
-Parathyroid adenoma
No marfanoid

52
Q

What are the diseases that are associated with MEN2B? Does this have marfanoid ssx?

A

Medullary thyroid cancer
Pheochromocytoma
Mucosal neuroma

Has marfanoid

53
Q

What is the primary hormone secreted by pheochromocytomas?

A

NE

54
Q

How do you differentiate between adrenal and extra-adrenal sites of pheochromocytomas?

A

Extra adrenal sites only produce NE, since adrenal gland has enzyme that converts NE to epi

55
Q

What are the three classical signs of a pheochromocytoma?

A

Episodic HA
Sweating
Tachycardia

56
Q

What are the eye findings of a pheochromocytoma?

A

Papilledema d/t elevated ICP

57
Q

What is the heart condition caused by a pheochromocytoma?

A

Dilated cardiomyopathy 2/2 HTN

58
Q

How do you diagnose a pheochromocytoma?

A

24 hour urinary fractionated metanephrines and catecholamines

59
Q

What is the f/u test done if a 24 hour urine tests shows elevated levels of metanephrines and catecholamines?

A

CT or MRI of abdomen and pelvis

60
Q

What is a MIBG scan?

A

Iodinated form of NE that pools to areas of NE secretion

61
Q

What is the drug that is used to treat a pheochromocytoma?

A

Alpha blocker–Phenoxybenzamine

62
Q

Why alpha blockers before beta blockers with a pheochromocytoma?

A

If beta blocks first, then alpha will cause super HTN

Endocrine (32 decks)

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