Path: Adrenals Flashcards by Thomas Ems

3 things Cortisol does to increase fuels and building blocks

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

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What is the MC cause of hypercortisolism

exogenous drugs

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how to differentiate between Cushing’s disease and syndrome

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

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Size of each adrenal in cushing’s disease

both enlarged

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

Crooke hyaline change (ACTH-producing basophils)

= Hot pink aggregation of keratin filaments

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In Primary adrenal neoplasms what do adrenals look like

contralateral is atrophied

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In Primary adrenal neoplasms: ACTH and cortisol levels

ACTH low

Cortisol high from neoplasm pumping it out

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Adrenal adenomas or carcinomas: which predominates in kids?

Carcinomas predominate in kids

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4 carcinomas that pump out ACTH (name the MC)

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

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In ectopic (neoplastic) ACTH production: adrenal size

both enlarged

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

Carcinoma

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What causes HTN in Cushing’s syndrome?

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

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

Cushing’s Disease - pituitary tumor

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4 sx of Hypokalemia seen in hyperaldosteronism:

weakness, parasthesias, visual disturbances, tetany

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What is MC cause of Conn syndrome?

MC cause = Primary Adrenocortical hyperplasia (idiopathic)

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Glucocorticoid-remedialble hyperaldosteronism is cuased by what?

Chimeric gene–> steroids with GC and Aldo functions

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Glucocorticoid-remedialble hyperaldosteronism is under the control of what

ACTH : therefore it responds to dexamethasone

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

activation of RAAS (high renin) –> Aldo release

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Hyperaldosterone-causing Adenomas show what histo change?

PAS+ cytoplasmic pink inclusion bodies = spironolactone bodies

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

21-alpha hydroxylase

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

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

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

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

CP of 21-alpha hydroxylase deficiency

virilization dt androgen xs

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

2 causes of Primary acute adrenocortical insufficiency (adrenal crisis)

a) Rapid withdrawal of exogenous steroids

Failure to increase steroids during stress

Result of Primary acute adrenocortical insufficiency (adrenal crisis)

Massive adrenal hemorrhage

Waterhouse Friderichsen syndrome is due to what

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

Chronic Adrenal Insufficiency is called?

(Addison Disease)

3 causes of Addison Disease (and which is MC)

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

4 CPs of Chronic Adrenal insufficicency (Addison Disease)

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

What cuases Secondary Chronic Adrenal Insufficiency?

Hypothalamic-Pit axis affected--> loss of ACTH

Hormone statuses in Secondary Chronic Adrenal Insufficiency?

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

How to diff between Primary and Secondary Chronic Adrenal Insufficiency?

No hyperpigmentation in secondary dt normal Aldosterone levels

10% of pheochromocytomas are what 6 things?

◊ 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

What happens to a pheochromocytoma when dipped in dichromate?

Turns brown or blackdt oxidation of catecholamines

Histo change seen in pheochromocytoma and the description of it

small nests and alveolar groups of cells – “zellballen”

What is the only reliable criterion of malignancy in pheochromocytomas?

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

3 CP of Pheochromoctyomas

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

Labs in Pheochromoctyomas

Increased urinary metanephrines and vanillymandelic acid (VMA)

MEN I (Wermer) show tumors in what places

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

IN MEN I, what tumor is highest cause of mortality

pancreatic tumors

What mutation causes MEN I (gene and protein)

germline mutation in MEN I gene encoding menin

What mutation causes MEN IIA

germline mutation in RET protooncogene (gain of function)

What mutation causes MEN IIB

• Single amino acid mutation in RET

MEN IIA manifests as what tumors

Pheochromocytoma, medullary thyroid ca, parathyroid hyperplasia

MEN IIB shows tumors where

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

Marfanoid Body Habitus is seen in what MEN syndrome

MEN IIB

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

MEN IIA