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EndocrineSystemPath2

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lustypan's version from 2016-11-21 21:17

Section 1

Question Answer
PTH levels controlled byfree (ionized) Ca in blood
PTH effectsincreases renal tubular Ca reabsorption, inceases renal 1,25 dihydroxy vitD production, increased intestinal Ca uptake, promotes renal phosphate loss, mobilizes bone Ca by osteoclast activation
hypercalcemia seen inCHIMPS->cancer, hyperthyroid, iatorgenic (thiazides, Ca/anatacids/milk, increase vitD), multiple myeloma, primary hyperparathyroidism (most common cause of asymtpomatic hyperCa), sarcoidosis
cancer and hypercalcemiametastatic cancers by osetolytic mechanim, squamous cell lung CA produces PTH-like protein)
most common cause of colinical hyperCamalignancy
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Section 2

Question Answer
primary hyperparathyroidismexcess PTH->hyperCa and seen more in adult women then men
most primary hyperparathyroidism adenoma->(1 gland, cyclin D1 inversion on chromosome 11=adjacent PTH/cyclinD1 genes=overexpression of cyclinD1=cell proliferation
other causes of primary hyperparathyroidismprimary hyperplasia (all 4 glands enlarged), carcinoma ( Dx based on invasion or mets)
clinical of primary hyperparathyroidismbones-stones-abdominalg roans-psychic moans (osteoporosis and osteitis fibrosa cystica, kidney stones, peptic ulcer/pancreatitis, depression and seizurss)
lab of primary hyperparathyroidismincrease PTH leads to icnrease serum Ca and decrease phosphate
most common cause of secondary hyperparathyroidismchronic renal falure->phoisphate retention/Ca loss, parathryoid hyperplasia, renal osteodystrophy
renal parenchyma los alsodecrease vitD conversiona nd thus decreased Ca absorption
in secondary hyperparathyroidismall 4 parathyroid glands are hyperplastic because increased PTH
lab of secondary hyperparathyroidismincrease serum phosphate, decrease Ca, increase PTH
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Section 3

Question Answer
primary hyperparathyroid Ca, PTH, causeincrease, increase, adenoma or hyperplasia
non-parathyroid Ca, PTH, causeincrease, decrease, CHIMPS (no P)
secondary hyperparathyroid Ca, PTH, causedecrease, increase, CRF/decrease vitD
hypoparathyroid Ca, PTH, causedecrease, decrease, surgery or autoimmune
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Section 4

Question Answer
hypoparathyroid isuncommon, autoimmune (often seen with APS1 autoimmune polyendocrine syndrome type 1), congenital ( digeorge), surgical, idiopathic
lab of hypoparathyroidno/decrease PTH->decrease Ca->increase neuromuscualr axcitabiluty (tetany), prolonged QT, depression/confusion/halucination
pseudohypoparathyroidsmmaternal inheritance->albright hereditary osteodystorphy (AHO skeletal defects) and end organ resistance to PTH and possibly to TSH/LH (nrmal or increase PTH, decrease Ca, increase PO4)
pseudopseudohypoparathyroidsmpaternal inheritance0>AHO without hormone resistance->phneotype of AHO but normal Ca and PTH
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Section 5

Question Answer
adrenal cortex hormone pathwayCRH of hypothalamus->ACTH from pituitary->cortisol from adrenal
glomerulosaaldosterone (mineralcortiocid regulated by RAS and K_)
fasciculaacortisl (glucocorticoid)
increase ACTH->adrenal hyperplasia, bilateral
decrease ACTH->adrenal atrophy, bilateral
IV cortisol->rapid decrease ACTH so chronic exogenous steroids->chronic ACTH decrease->adrenal atrophy, bilateral
reticularisweak androgen and estrogen
hyperadrenalism=increase cortisol or increader aldosterone or increase androgens
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Section 6

Question Answer
cushing syndromehypercortisl most commonly due to exogenous glucocorticoids leading to bilateral adrenal atrophy
endogenous cushing syndroe=mecushing disease->pituitary ACTH tumor->icnrease ACTH->bilateral adrenal hyperplasai / cushing syndrome->ectopic ACTH tumor (small cell CA lung)->increase ACTH / cushing syndrome->adrenal adenoma/carcinoma or nodular hyperplasia->decrease ACTH0>contralateral gland atorphy
clinical of cushingHTN and wt gain early, then truncal obestiy, moon faces, buffalo hump, weakness, diabetes/glucose intolerance, osteoporosis, pink skin striae, increased infections, mental disturbances
excellent to screen cushing24hr urine free cortisol
dexamethasone suppression testpotent synthetic GC that suppresses ACTH and cortisol->bedtime Dex measure AM cortisol->low DEX->normal cortisol level suppressed excludes cushing) / high dose DEX=cause of cushing syndrome (increasew ACTH, suppression is pituitary cushing, increase ACTH and no suppression is ectopic ACTH tumor, decrease ACTh and no suppression is adrenal cushing
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Section 7

Question Answer
aldosterone effectrenal Na retentiona nd K loss->increase BP and hypokalema
aldosterone regulated byrenin-angiotensin
primary hyperaldosteronismincreased aldosterone0>HTN and weakness
etiology of primary hyperaldosteronismidiopathic bilaeral adrenal hyperplasia / adrenal adenoma=conn yndrome
lab of primary hyperaldosteronismHTN, hypokalemia, decrease renin, metabolic alkalosis due to hypokalemia
screenign test for primary hyperaldosteronismincreased plasma aldosteorne/renin ratio
secondary hyperalosteronismdue to increased renin due to renal ischemia, pregnancy, or renin-producing lesins ( JG hyperplasia=bartter sundrome, wilms tumor)
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Section 8

Question Answer
adrenogenital syndromescomponent of cushign disease, adrenocortical tumor, congenital adrenal hyperplasia
congenital adrenal yperplasiaautosomal recessive adrenal enzyme defects
suspect congenital adrenal yperplasia inany newborn with refracotry dehydration and sexual differentiation abnormality with amiguous genitalia
congenital adrenal yperplasia due todefective cortisol synthesis->increased ACTH0>bilaterla hyperplsasia
increase androgen production in congenital adrenal yperplasiavirtilization i femals
most common deficiency in congenital adrenal yperplasia21-hydroxylase def->salt wasting syndrome due to lack of mineralcorticoids (decrwase Na, decrease BP, increase K, acidosis postnatal, CV collapse, death), simple virilizing without salt watsing (amiguuous genitlaia, progressive cirilization), late onset adrenal virislim (milder def reuslt in delayed menarcha, oligomenorrhea, hirsutium in females or precocious puberty and oligospermia in males)
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Section 9

Question Answer
hypoaldrenalismdecrease cortisol and decrease aldosterone
most common causes of hypoadrenalism70% autoimmune (autoimmune polyendocrine syndromes APS1 and 2) / infnats-fulminant meningococcemia with DIC, skin purpura, and massive bilateral adrenal hemorrhage (waterhouse friderichsen syndome) / adults-DIC, infections, metastatic carcinoma, lymphoma, amylodisis, sarcodisos, hemochromatosis / AIDS / rapid withdrawal of steroids / adrenal crisis in chronically def pateitn that need higher levels of steroids
autoimmune polyendorcine syndromeslymphoid infiltrate in glands with gland destructuon
APS type 1mucocutanoeus candidiasis->hypoparathryoidism-addison disease / mutation in AIRE
APS type 2schmidt syndrome->most common, addison disease-hypothyroidism-diabetes type 1
XPIDx-linked polyendocrinopathy immunodefficiency, diarrhea->rare, diarrhea and diabetes in neonates and death, primary acute adrenocrotical insufficiency (adrenal crsis)
clinical of XPIDhypotension-shock-coma, fever, dehydration, nausea-vomtijng, weakness, hyperK, hypoglycemia
Rx for APSIV cortisol
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Section 10

Question Answer
primary chronic adrenocortical insufficiency isaddison disease
addison disease clinicalweakness, nausea-vomting, wt loss, hypotension, skin pigmentation (increased ACTH and MSH)
labs of addison diseaselow GC-low gluucose and icnrease ACTh / low MC-hypotenion, low Na, low BP, high K, acidosis due to hyperK
seocndary adrenalcortical insufficencypituitart or hypothalamus lesion, prolonged exogenous GC->lab shows fall in ACTH and no skin pigemntation
adrenal cortical tumorsmost adenoas are clinically silent, others produce Cushings or Conns syndrome; virilizing tumors likely to be carcinoma
adrenal myelolipomamature fat and hematopoietic cells, usually an incidental finding
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Section 11

Question Answer
adrenal medulla consists ofneural crest cells (chromaffin cells)
pheochromocytomaspositive chrmaffin reacton wtih potassium dichromate, increased catecholamines
increased catecholamines=paroxysmal HTN, tachycardia, diaphoresis, headaches, anxiety, catecholamine cardiomyopathy
lab for pheochromocytomabest screenign test is increased plasma metanephrne
histlogy of pheochromocytomazellballen neurosecrtory granules+stain chromogranin and synaptophysin
pheochromocytomas have association with MEN2, von hipple-landau, von recklinghausen, sturge-weber
extra-adrenal tumors are calleparagangliomas, and are usually chromaffin negative->carotid body tumor, chemodectoma
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Section 12

Question Answer
neuroblastoma/ganglionneurblastoma/ganglioneuromasmall round blue cell tumor in infants 1-2yrs that present with abdominal mass, fever, wt loss
histology of neuroblastoma/ganglionneurblastoma/ganglioneuromahomer-wright rosettes (tumor cells around central space filled with fibrillary cytoplasm); scattered ganglionc ells
behavior of neuroblastoma/ganglionneurblastoma/ganglioneuromamay spontaneously mature or regress
poor prognosis is1p deletion or c-myc gene amplification
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Section 13

Question Answer
multiple endocrine neoplasiatumors in younger age group, multiple endocrine sites, more aggressive
MEN 1P lesions (wermer syndrome)->parathryoid (hyperpara, hyperCa), pituitary (prolactinoma, acromegaly), pancrratic islets (gastirnoma or insulinoma), peptid ulcers (from gastrinoma), MEN1 gene mutation ( a TSG)
MEN2asipple syndrome->emdullary carcinoma thyroid and pheochromocytoma, parathyroid hyperplasia, mutation of RET protooncogene (screen family members and get prophylatic total thyroidectomy if there is RET mutation present)
MEN2bwithout parathyroid hyperplasai
only sig pathology of pineal glandtumor-rare
must pineal gland tumors aregerminomas, resembling testicular seminoma
pineoblastomasprimtive, cells, kids, aggressive
pineocytomasmore mature cells, adults, benign
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