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D&T test 6 - endocrine, dermatology, MSK > Adrenal Gland: 1/3 > Flashcards

Adrenal Gland: 1/3 Flashcards

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Dermatology Board Prep flashcards
1
Q

derivation and type of cells in medulla of adrenal

A

i. Embryonic Nueral Crest cells

Chromaffin Cells

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2
Q

Controlled by: medulla and cortex

A

medulla- direct innv

cortex- HP axis and RAAS

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3
Q

What controls Aldosterone production

A

RAAS. NOT ACTH

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4
Q

cortisol has negatoive fb on what

A

Hypo and pit

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5
Q

Primary Hyperaldosteronism aka?

A

Conn syndrome

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6
Q

MC cuase of Primary Hyperaldosteronism

A

Adrenocortical neoplasm: solitary aldosterone-secreting adenoma

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7
Q

Primary Adrenocortical hyperplasia: cause and describe

A

Bilateral nodular hyperplasia of the adrenals

caused by overacrtivity of aldosterone synthase gene (18HSD)

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8
Q

Under what condition is Aldosterone production no longer controlled by RAAS. Now controlled by ACTH?

A

*****Glucocorticoid-remedial hyperaldo

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9
Q

How does Glucocorticoid-remedial hyperaldo couple ACTH to Aldo production?

A

Fusion between 11B-hydroxylase gene and aldosterone synthase gene (18HSD)

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10
Q

Renin, K, Na levels in Primary Hyperaldosteronism

A

Renin: low
K+ = low
Na = high

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11
Q

4 sx of hypokalemia

A

Weakness, parathesias, visual disturbances, tetany

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12
Q

how to dx Primary Hyperaldosteronism

A

high Aldo with low renin

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13
Q

Cushings syndrome defintion

A

high cortisol

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14
Q

Cushing’s disease will alwayd be due to?

A

Apituitary tumor–> ACTH –> cortisol

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15
Q

3 casuses of Cushing syndrome

A

• Ectopic ACTH production (SCC of lung)
• Autonomous Cortisol Production (Aderenal)
Iatrogenic

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16
Q

Consistent sign of Cushings in Children

A

growth failure with weight gain

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17
Q

What two cortisol tests help dx cushings

A

24 hour urinary free cortisol

Late-night salivary cortisol (level is normally lowest at night)

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18
Q

hat effect does Low-dose dexamethasone (corticosteroid) suppression test have on cushing syndrome? why?

A

None- problem isn’t in HPA axis

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19
Q

What diagnostic effect will high-0dose dexamethasone have

A

supression of cushings disease: pit adenoma (no effect on SCC of lung)

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20
Q

Who to Screen for Cushing Syndrome (3)

A
  1. pts with unusual features: HTN, T2DM, thin skin
  2. progessive features
  3. kids with reduced height but ^ weight
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21
Q

Addison disease aka?

A

Primary Adrenal insufficiency

22
Q

Primary Adrenal insufficiency basic derangement/mechanism

A

low cortisol (and Aldo) –> loss of negative fb –> increased ACTH production

23
Q

Causes of Primary Adrenal insufficiency (7) and basic cause

A

Basic: destruction of adrenal cortex

a. Congenital Adrenal Hyperplasia
b. Adrenoleukodystrophy
c. Autoimmune adrenal insufficiency
d. Infection (AIDS, TB, Waterhouse-Friedrichsen Syndrome)
e. Amyloidosis, sarcoidosis, hemochromatosis
f. Metastatic cancer
g. Metabolic failure (congenital adrenal hyperplasia, drug indiced ACTH inhibition)

24
Q

Classic SX of Primary Adrenal insufficiency (2)

A

hyperpigmentation (buccal mucosa, scars)

fatigue

25
Q

cLASSIC LABS IN Primary Adrenal insufficiency (3)

A

Low Na
. High K
Low Blood Sugar

26
Q

tx for primary adrenal insufficiency

A

cortsiol, aldosterone

27
Q

Waterhouse-Friderichsen Syndrome what is it and cause

A

hemorrhagic necrosis of adrenals dt DIC in young children with Neisseria meningitidis infx. lack of cortisol –> hypotension–> death

28
Q

CP of Waterhouse-Friderichsen syndrome

A

DIC with purpura (non-blnaching), hypotension–> shock

29
Q

Inheritance pattern of congenital adrenal hyperplasia

A

AR

30
Q

general cause ofcongenital adrenal hyperplasia

A

deficiency of enzyme that makes cortisol

31
Q

morphology of adrenals in congenital adrenal hyperplasia

A

hyperplastic up to 15x normal weight

32
Q

MC deiciency in congenital adrenal hyperplasia

A

21-hydroxylase

33
Q

congenital adrenal hyperplasia sign in females

A

ambiguous genitalia

34
Q

If 3B-HSD is absent or deficient –> ??

A

undervirilized males dt no sex steroid production:

35
Q

Tx of congenital adrenal hyperplasia (3)

A

i. Cortisol and mineralcorticoid replacement
ii. Normalize growth and androgens
Salt supplementation (lack of aldo–> salt wasting)

36
Q

What generally causes Secondary Adrenal Insufficiency

A

Any disorder of the Hypothalamus or Pituitary gland that reduces ACTH

37
Q

When is adrenarche abnormal in boys and girls

A

boys < 9

girls < 8

38
Q

Which is typically functional: adrenal carcinomas or adenomas?

A

Carcinomas are functional

39
Q

how does a carcinoma typically metastasize

A

via LN

40
Q

Classic triad of Pheochromocytoma

A

Headache, diaphoresis (sweating), palpitations

41
Q

MC sx of Pheochromocytoma

A

HTN

42
Q

Pheochromocytoma: 5 rules of 10

A

10% are:

  1. Malignant
  2. bilateral
  3. extra-adrenal
  4. familial syndromes
  5. childhood
43
Q

Tx of pheochromocytoma (2)

A

i. Normalize BP

Excision

44
Q

What familial mutations is pheochromocytoma asssociated with? (4)

A

MEN2A and MEN2B, VHL, Neurofibromatosis type 1

45
Q

MEN1 is associated with what 3 tumors

A

Pituitary
Pancreatic
Parathyroid adenomas

46
Q

What is gene mutation and product in MEN1? oncogene or suppressor?

A

MEN1 gene encodes menin = tumor suppressor

47
Q

MC manifestation of MEN1

A

primary hyperparathyroidism

48
Q

mutation and function in MEN2A and MEN2B

A

both - gain of function in RET

49
Q

3 associations with MEN2A

A

Pheochromocytoma
Medullary CA
Parathyroid hyperplasia

50
Q

RET mutations occur in cells of what nature

A

those that developed from NCCs

51
Q

MEN2B is associated with what tumors (3)

A

Pheochromocytoma
Medullary CA of thryoid
Neuromas, gangliomas

52
Q

Marfanoid habitus is associated with what MEN?

A

MEN2B

D&T test 6 - endocrine, dermatology, MSK (22 decks)

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