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D&T test 6 - endocrine, dermatology, MSK > Path: Adrenals > Flashcards

Path: Adrenals Flashcards

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1
Q

3 things Cortisol does to increase fuels and building blocks

A

Increase Blood sugar (^GNG, inhibiting glc uptake)
^ Blood amino acids (proteolysis)
^ Free FAs (lipolysis)

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2
Q

What is the MC cause of hypercortisolism

A

exogenous drugs

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3
Q

how to differentiate between Cushing’s disease and syndrome

A

Disease: ^ACTH from Anterior Pit.
Syndrome: ^ACTH from any other cuase

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4
Q

Size of each adrenal in cushing’s disease

A

both enlarged

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5
Q

Chc histological change in Cushing’s disease and dedscribe it’s appearnace

A

Crooke hyaline change (ACTH-producing basophils)

= Hot pink aggregation of keratin filaments

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6
Q

In Primary adrenal neoplasms what do adrenals look like

A

contralateral is atrophied

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7
Q

In Primary adrenal neoplasms: ACTH and cortisol levels

A

ACTH low

Cortisol high from neoplasm pumping it out

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8
Q

Adrenal adenomas or carcinomas: which predominates in kids?

A

Carcinomas predominate in kids

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9
Q

4 carcinomas that pump out ACTH (name the MC)

A

MC = SCC of lung
Carcinoid tumors
Medullary carcinoma of thyroid
Islet cell tumor of pancreas

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10
Q

In ectopic (neoplastic) ACTH production: adrenal size

A

both enlarged

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11
Q

Which is more likely to produce hormone: carcinoma or adenoma?

A

Carcinoma

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12
Q

What causes HTN in Cushing’s syndrome?

A

HTN (dt upregulation of α-1 receptors on arterioles)

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13
Q

WHich source of ACTH/cortisol disease process shows a response to high-dose dexamethasone

A

Cushing’s Disease - pituitary tumor

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14
Q

4 sx of Hypokalemia seen in hyperaldosteronism:

A

weakness, parasthesias, visual disturbances, tetany

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15
Q

What is MC cause of Conn syndrome?

A

MC cause = Primary Adrenocortical hyperplasia (idiopathic)

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16
Q

Glucocorticoid-remedialble hyperaldosteronism is cuased by what?

A

Chimeric gene–> steroids with GC and Aldo functions

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17
Q

Glucocorticoid-remedialble hyperaldosteronism is under the control of what

A

ACTH : therefore it responds to dexamethasone

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18
Q

Secondary hyperaldosteronism is caused by what? with what hormone level chcly high?

A

activation of RAAS (high renin) –> Aldo release

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19
Q

Hyperaldosterone-causing Adenomas show what histo change?

A

PAS+ cytoplasmic pink inclusion bodies = spironolactone bodies

20
Q

In the Classic salt-wasting type of CAH, what is the deficient enzyme?

A

21-alpha hydroxylase

21
Q

What is the status of cortisol, ACTH and adrenal size in CAH?

A

Lack of cortisol production –> ^ACTH –> adrenal hyperplasia

22
Q

In 21-alpha hydroxylase deficiency, what are you lacking, what do you have excess of?

A

lack: Mineralocorticoids (and cortisol, duh)
excess: Androgens

23
Q

CP of 21-alpha hydroxylase deficiency

A

virilization dt androgen xs

HTN, hypoNa, HyperK, acidosis, hypotension, CV collapse, death

24
Q

2 causes of Primary acute adrenocortical insufficiency (adrenal crisis)

A

a) Rapid withdrawal of exogenous steroids

Failure to increase steroids during stress

25
Q

Result of Primary acute adrenocortical insufficiency (adrenal crisis)

A

Massive adrenal hemorrhage

26
Q

Waterhouse Friderichsen syndrome is due to what

A

Neisseria Meningitidis infx–> DIC, puprpura –> hemorrhagic necrosis of adrenal glands

27
Q

Chronic Adrenal Insufficiency is called?

A

(Addison Disease)

28
Q

3 causes of Addison Disease (and which is MC)

A
  1. Autoimmune destruction of adrenals (MC)
  2. TB
  3. Metastatic neoplasms
29
Q

4 CPs of Chronic Adrenal insufficicency (Addison Disease)

A

a) Electrolyte imbalance (hyperK, hypoNa, hypotension, hypovol)
b) Hyperpigmentation (xs ACTH)
c) GC deficiency –> hypoglycemia
d) Minor stress –> acute fatal adrenal crisis

30
Q

What cuases Secondary Chronic Adrenal Insufficiency?

A

Hypothalamic-Pit axis affected–> loss of ACTH

31
Q

Hormone statuses in Secondary Chronic Adrenal Insufficiency?

A
  • Normal Aldosterone (since it is controled by RAAS- stimulated by AngII)
  • low cortisol, low Sex steroids dt lack of ACTH
32
Q

How to diff between Primary and Secondary Chronic Adrenal Insufficiency?

A

No hyperpigmentation in secondary dt normal Aldosterone levels

33
Q

10% of pheochromocytomas are what 6 things?

A

◊ Associated with one of several familial syndromes including MEN IIA and IIB, NF1, von Hippel Lindau, Sturge‐Weber
} This is 25% now!
◊ Bilateral (non‐familial cases)
◊ Malignant
◊ Extra‐adrenal (occurring in extra‐adrenal paraganglion system –called paragangliomas)
◊ Arising in childhood
◊ Not associated with hypertension

34
Q

What happens to a pheochromocytoma when dipped in dichromate?

A

Turns brown or blackdt oxidation of catecholamines

35
Q

Histo change seen in pheochromocytoma and the description of it

A

small nests and alveolar groups of cells – “zellballen”

36
Q

What is the only reliable criterion of malignancy in pheochromocytomas?

A

Metastasis are the only reliable criterion of malignancy. Atypia/micro appearnace doesn’t matter

37
Q

3 CP of Pheochromoctyomas

A
  • HTN - abrupt, sens eof doom
  • Paroxysmal catecholamine release: tachy, sweating, nausea
  • Ischemic damage 2/2 catecholamines –> cardiac complications
38
Q

Labs in Pheochromoctyomas

A

Increased urinary metanephrines and vanillymandelic acid (VMA)

39
Q

MEN I (Wermer) show tumors in what places

A

3P’s- pnacreatic, pituitary, parathyroid hyperplasia and adenomas

40
Q

IN MEN I, what tumor is highest cause of mortality

A

pancreatic tumors

41
Q

What mutation causes MEN I (gene and protein)

A

germline mutation in MEN I gene encoding menin

42
Q

What mutation causes MEN IIA

A

germline mutation in RET protooncogene (gain of function)

43
Q

What mutation causes MEN IIB

A

• Single amino acid mutation in RET

44
Q

MEN IIA manifests as what tumors

A

Pheochromocytoma, medullary thyroid ca, parathyroid hyperplasia

45
Q

MEN IIB shows tumors where

A

-100%- have medullary thyroid CA
-Pheochromocytoma
- Multiple neuromas or ganglioneuromas in skin, mouth, conjunctiva, GI tract,
respiratory tract

46
Q

Marfanoid Body Habitus is seen in what MEN syndrome

A

MEN IIB

47
Q

Familial medullary thyroid cancer is a variant of what MEN syndrome without the other features?

A

MEN IIA

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

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