Endo - FA Patho p 333 - 347 Flashcards
High-dose dexamethasone suppression test causes what in Cushings dx?
Free cortisol suppression
How many mg is administred during High-dose dexamethasone test?
8mg - high dose
3 causes for inc cortisol?
- Exogenous corticosteroids - Primary adrenal adenoma, hyperplasia, or carcinoma - ACTH-secreting pituitary adenoma, paraneoplastic ACTH
Difference between Cushing syndrome and disease?
ACTH-secreting pituitary adenoma - C. disease incr Cortisol from variety of conditions - C. syndrome
2 paths causing paraneoplastic secretion of ACTH?
small cell lung cancer, bronchial carcinoids
Is adrenal tumor an ACTH-independent or dependent Cushing syndrome?
ACTH independent Cushing syndrome
Is ectopic ACTH secretion an ACTH-independent or dependent Cushing syndrome?
ACTH dependent
CRH stimulation test helps diagnose which pathology?
Cushing disease (inc ACTH) vs ectopic ACTH secretion (no inc of ACTH)
What an alternative test used to dx adrenal insufficiency?
Metyrapone stimulation test
Metyrapone blocks which step of cortisol synthesis?, What is normal response to it
The last one - 11DOC (deoxycortisol) –> cortisol. Normal response is dec cortisol and compensatory inc in ACTH, 11DOC. If they dont INC, then its a primary/secondary ACTH insufficiency, not a cortisol issue
Pt presents w/ sudden onset of massive hemorrhage and shock. What is the adrenal path?
Primary Acute adrenal insufficiency or Waterhouse-Friderichsen syndrome
Competitive athlete on school break shows signs of adrenal insufficiency, which type is his pathology?
Tertiary - chronic exogenous steroid use precipitated by abrupt withdrawal
skin and mucosal hyperpigmentation is seen in which type of adrenal insuff.?
primary
Why can Cushing lead to coronary a disease?
Inc cortisol –> inc stimulation of a1R on arterioles –> hypertension –> CAD
Cause of truncal obesity and buffalo hump in Cushing?
Inc cortisol –> glucose in blood –> deposit in fat, leading to inc fat deposition on face, trunk, back
Most accurate test for Cushings?
Los dose dexamethasone suppression test
Different pathological features of adrenal cortex with ACTH independent and dep Cushing?
In ACTH independent - atrophy in ZF, ZR In ACTH dep (Bilat adrenal hyperplasia) - ZF and ZR are diffuse, nodular
TB can cause what kind of adrenal insuff?
Chronic—aka Addison disease. Due to adrenal atrophy or destruction by disease
— is a byproduct of ACTH production from —-.
MSH pro-opiomelanocortin
In which type of Adrenal insuff. is Aldosterone synthesis unaffected?
2ndary and tertiary
Primary adrenal insuff. is associated with which autoimmune syndromes?
Polyglandular syndromes
Septicemia in Waterhouse Friderichsen is usually caused by what pathogen?
Neisseria
Which adrenal insuff. spares the skin and mucosa? and why?
Tertiary and Secondary, because there is a decr in ACTH production –> less MSH by product
What is the MC tumor of the adrenal medulla in children?
Neuroblastoma
Classic histology presentation of Neuroblastoma:
Homer-Wright rosettes and small, round, blue/purple nuclei
2 path from neural crest?
Neuroblastoma Pheochromocytoma
Homovanillic acid (HVA) a breakdown product of?
Dopamine
Signs of primary adrenal insuff?
Hypotension (hyponatremic volume contraction), Hyper Kalemia, metabolic acidosis, skin and mucosal hyperpigmentation
—- is a breakdown product of norepinephrine.
vanillylmandelic acid (VMA)
“dancing eyes-dancing feet” are seen in?
Neuroblastoma, Subacute sclerosing panencephalitis. - opsoclonus & myoclonus
Name episodic hyperadrenergic symptomsof pheochromocytoma (5 P’s):
Pressure (incr BP) Pain (headache) Perspiration Palpitations (tachycardia) Pallor
T or F? Wilms tumor can cross the midline vs. Neuroblastoma which is smooth and unilateral.
F - opposite
Why is α-blockade administred in Pheochromocytoma before giving β-blockers prior to tumor resection?
to avoid a hypertensive crisis.
What % of Pheo’s calcify and why?
10% bc of inc in pTrp
Neuroblastoma has an overexpression of which oncogene?
N-myc
What malignancy is associated with c-myc (as opposed to n-myc)
Burkitt’s lymphoma
What is a typical histology of Pheochomocytoma?
enlarged pleomorphic nuclei, typical of malignancy.
2 tumor markers for Neuroblastoma?
Bombesin and neuron specific enolase
Name one path that use Bombesin and neuron-specific enolase as tumor markers?
Lung cancer (bronchial carcinoid)
Homer Wright rosettes also seen in what other path?
Medulloblastoma
Urine and plasma findings is Pheochromocytoma?
catecholamines and metanephrines in urine and plasma.
Which thyroid disorder is associated with Inc CK?
Hypothyroidism - myopathy (Normal Ck in Hyper)
Which thyroid disorder is associated with thinning, fine hair?
hyperthyroidism
Thyroid disorder with inc #/sensitivity of B adr receptors?
Hyperthyroid
Causes of smooth/diffuse goiter?
Graves, Hashimoto, Iodine def, TSH secreting pit adenoma
Causes of nodular goiter?
Toxic multinodular goiter, Thyroid adenoma, Thyroid cancer, Thyroid cyst
HLA # associated with Hashimoto’s?
HLA - DR3; HLA-DR5
Which thyroid disorder is assoc w/ non Hodgkin Lymphoma?
Hashimoto
Most common cause of thyroid disgenesis?
Congential hypothyroid (Cretinism)
Neck and jaw pain is assoc with which thyroid disorder?
Subacute granulomatous thyroiditis - jaw pain, very tender thyroid
Which disease is assoc with a rock like painless goiter? Why rock-like? (patho)
Riedel thyroiditis - thyroid replaced with fibrous tissue w/ inflammatory infiltrate.
What is the Wolff Chaikoff effect?
Thyroid gland will downregulate in response to inc iodide
Graves disease has what type of Ig, HS?
HS-II, IgG
The release of cytokines in the retroorbital space leads to secretion of _____ by _____ cells? (Eventually resulting in exophthalmus in Graves)
Inc secretion of hydrophilic GAGs by fibroblasts
focal patches of hyperfunctioning follicular cells = which thyroid disease?
Toxic multinodular goiter
Tall follicular epithelial cells with scalloped colloid is seen in which thyroid disease?
Graves
Tx for Thyoid Storm?
Propranolol (Nadolol), Propylthiouracil, Corticosteroids, Potassium Iodide
Jod-Basedow phenomenon
If a patient that is previously iodine deficient and has partially autonomous thryroid tissue, is then made iodine replete —> thyrotoxicosis
Most common histology of thyroid adenoma?
follicular - with no capsular or vascular invasion
Why is thyroid cancer treatment associated with hypocalcemia?
Due to surgery leading to possible removal of parathyroid glands
Which two Thyroid cancers are associated with Ret mutation?
Papillary CA and MEN 2A & 2B (medullary CA)
Empty appearing nuclei with a central clearing and nuclear grooves - which Thyroid disease?
Papillary (Orphan Annie eyes)
Which thyroid cancer stains with Congo Red?
Medullary CA - sheets of cells in amyloid stroma