Endocrine Path Flashcards
1 cause of cushing’s syndrome
Iatrogenic–exogenous steroids
increased cortisol decreased ACTH (negative feedback)
Cushing’s syndrome Sx
HTN hyperglycemia osteoporosis Moon Facies Weight Gain tuncal obesity abd. striae, thinning skin Buffalo hump immune suppression amenorrhea
Cushing’s Disease
ACTH secreting pituitary adenoma
increased ACTH and Cortisol
Cort stim test with Cortisol levels remaining high after low and high doses of DEX
Ectopic ACTH producing tumor
Cortisol producing tumor
Cort stim test with Cortisol levelssuppressed after low and high doses of DEX
Normal response
Cort stim test:
Cortisol remains high after low dose and suppressed after high dose DEX
ACTh pituitary adenoma
HTN, Hypokalemia, metabolic alkalosis, LOW plasma renin
1º Conn’s Syndrome
adrenal hyperplasia or aldosterone secreting adrenal adenoma
HTN, Hypokalemia, metabolic alkalosis, HIGH plasma renin
2º Conn’s Syndrome
Renal artery stenosis
CHF
Cirrhosis
Nephrotic Syndrome
Conn’s Tx
1º–surgery, spironolactone
2º–spironolactone
Hypotension
hyperkalemia
acidosis
skin hyperpigmentation
1º Addison’s Disease
adrenal atrophy and absence of hormone production
all 3 cortical layers but spares medulla
destruction by disease (TB, autoimmune, mets)
DDx 2º from 1º Addison’s Disease
Low aldosterone low cortisol no skin hyperpigmentation no hyperkalemia decreased ACTH production in pituitary
Post Neisseria meningitidis sepsis
1º adrenal insufficiency due to adrenal hemorrhage
DIC
Endotoxic Shock
Waterhouse-Friederichsen Syndrome
Most common tumor in adults of adrenal medulla
Pheochromocytoma
Secretes Epi/NE/DA
Pheochromocytoma is composed of what type of cells?
Chromaffin cells
derrived from neural crest
Pheochromocytoma Sx
Episodic Hyperadrenergic Sx–5 P’s
Pressure (high BP) Pain (HA) Perspiration Palpitation Pallor
Pheochromocytoma associated with what syndromes?
MEN 2A and 2B
Neurofibromatosis Type 1
Pheochromocytoma Tx
Phenoxybenzamine (alpha blocker) prior to surgical excision
prevents hypertensive crisis
Pheochromocytoma Lab test
Urine VMA
Why is there hyperpigmentation of skin with 1º addison’s?
Increased MSH
byproduct of ACTH production from POMC
Most common adrenal medullary tumor in kids
Neuroblastoma
less likely to develop HTN
can occur anywhere on sympathetic chain
Lab test for neuroblastoma
Elevated HVA in urine (homovanillic Acid)
breakdown product of DA
Neuroblastoma oncogene
N-myc overexpression –> rapid tumor progression
Cold intolerance weight gain despite decreased appetite constipation lower activity and energy Myxedema (face, periorbital) bradycardia
Hypothyroidism
Hypothyroidism Labs
decreased free T4
Increased TSH (1º) Decreased in 2º
Heat intolerance weight loss, increased appetite hyperactivity increased reflexes diarrhea pretibial myxedema warm moist skin/hair palpitations/arrhythmia
Hyperthyroidism
Hyperthyroidism Labs
Decreased TSH (1º) Increased Free or total T3 and T4
Most common cause of hypothyroidism
Hashimoto’s
Hashimoto’s Thyroiditis Pathophys
autoimmune disease against thyroid
moderately enlarged, NONTENDER thyroid
Hashimoto’s Thyroiditis histo
Hurthle Cells
lymphocytic infiltrate with germinal centers
HLA marker associated with Hashimoto’s
HLA-DR5
Severe Fetal hypothyroidism Pot-Bellied Pale Puffy Faced kid protruding umbilicus Protuberant tongue
Cretinism
Types of Cretinsim
Endemic goiter–dietary Iodine deficiency
Sporadic Cretinsim–defect in T4 formation or dev. failure of thyroid
Self-limited hypothyroidism following flu infection
VERY TENDER thyroid
increased ESR, jaw pain
granulomatous inflammation
De Quervain’s Subacute thyroiditis
Thyroid replaced by fibrous tissue
hypothyroidism
PAINLESS, rock-hard goiter
Riedel’s Thyroiditis
manifestation of IgG related systemic disease
Wolf Cheikoff Effect
Hypothyroidism due to ingestion of excessive Iodine
Focal patches of hyperfunctioning follicular cells working independently of TSH
Hot nodule
Increased T3 and T4
Toxic Multinodular Goiter
Mutation of TSH-R
Jod-Basedow Phenomenon
Thyrotoxicosis when iodine defficient patient is made iodine replete
Graves Disease pathophys
Autoimmune hyperthyroidism
Ab that stimulate TSH receptors
Pretibial Myxedema and exophthalmos seen with Graves–mechanism
increase in CT deposition
Stress induced thyroid storm
Death by Arrhythmia
increased ALP
Thyroid Storm
Thyroid mass Empty appearing nuclei (orphan annie eyes) psammoma bodies nuclear grooves Excellent prognosis
Papillary CA of thyroid
Risk of Papillary CA
Childhood irradiation
Calcitonin Producing mass on thyroid
sheets of cells in amyloid stroma
Medullary CA of Thyroid
Cancer associated with Hashimoto’s
Lymphoma
Medullary CA of thyroid cell type
C Cells
secrete calcitonin
Undifferentiated mass on thyroid
poor prognosis
old pt.
Anaplastic
Mass on Thyroid with uniform follicles
good prognosis
Follicular CA
1 tumor on thyroid
Papillary CA of Thyroid
1 cause of primary hyperparathyroidism
adenoma
Osteitis Fibrosa Cystica
cystic bone spaces filled with brown fibrous tissue –> bone pain
Sx of Hyperparathyroidism
Hypercalcemia –> Stones, bones, and groans
Renal stones
constipation (GI)
bone pain
1º hyperparathyroidism labs
Increased PTH, Alk Phos, and urine cAMP
2º hyperparathyroidism causes
Chronic Renal disease –> decreased Vit. D
decreased Ca reabsorption from gut and increased Pi
2º hyperparathyroidism labs
Hypocalcemia
hyper Pi
increased Alk. phos
Increased PTH
3º hyperparathyroidism
Refractory (autonomous) hyperparathyroidism due to chronic kidney disease
very very high PTH
elevated Ca
Hypoparathyroidism causes
Accidental surgical excision (thyroid surgery)
Autoimmune destruction
DiGeorge Syndrome
hypoparathyroidsim Sx
Hypo Ca
tetany
Chvostek’s sign
Trousseau’s Sign
Chvostek’s sign
Tap on facial nerve and get spasm of face
hypo Ca sign
Trousseau’s Sign
Occlusion of brachial a. with BP cuff –> carpal spasm
hypo Ca sign
Pseudohypoparathyroidsim
Albright’s hereditary ostrodystrophy
AD kidney unresponsive to PTH
Short stature, short 4/5 digits, hypocalcemia
Most common pituitary adenoma
Prolactinoma
Sx of prolactinoma
amenorrhea
galctorrhea
low libido
infertility (low GnRH)
Bitemporal hemianopsia (optic chiasm)
Prolactinoma Tx
DA agonists–bromocriptine & cabergoline
Excessive GH in adults and kids
acomegaly–adults
gigantism–kids
Achromegaly Dx
High IGF-1, GH
No GH suppression with glucose suppression test
pituitary mass on MRI
Dx of DI
Water deprivation test does not increase urine osmo
Desmopressin response–central
not response–nephrogenic
DI Tx
Fluid intake
Central–nasal ADH (desmopressin)
Nephrogenic–hydrochlorothiazide, indomethacin, or amiloride
Sheehan Syndrome
Hypopituitarism due to post partum bleeding (could occur at end of preg)
Hypertrophy of pituitary with no ∆ in vascular supply
failure to lactate
Empty Sella Syndrome
atrophy or compression of pituitary
idiopathic
obese women
Hypopituitary causes
Non-secreting pituitary adenoma craniopharyngioma Sheehan's Syndrome Empty Sella Brain injury Hemorrhage Radiation
Hypopituitarism Tx
Substituinon Tx
Corticosteroids
Thyroxine
Sex steroids
human growth factor
Concentrated urine
dilute blood–relative hyponatremia
SIADH
SIADH Tx
Fluid restriction
IV saline
Tolvaptan/conivaptan–Vasopressin Rec. antagonist
demeclocycline
Kimmelsteil Wilson Nodules
DM buzz word for non-enxymatic glycosylation in kidney vasculature
DM Type 1 HLA associated
HLA-DR3 and DR4
Histology of DM Type 1 vs. Type 2
- Islet leukocytic infiltrate
2. Islet amyloid deposition (AIAPP)
DKA pathophys
Stress –> excessive fat breakdown –> ketogenesis from free FA –> Ketone bodies
Most common Ketone bodies in DKA
ß-hydroxybutyrate > acetoacetate
Hyperkalemia with DKA
Serum levels are high but overall hypokalemia due to intracellular depletion from insulin
DKA complications
mucor Rhizopus infection cerebral edema cardiac arrhythmias heart failure
Tx DKA
Fluids
Insulin
K+–replete intracellular
glucose–if necessary to prevent hypoglycemia
Recurrent Diarrhea, Cutaneous flushing,asthmatic wheezing, right sided valvular disease
Elevated 5HT
Carcinoid Syndrome
Carcinoid Syndrome Cause
Carcinoid tumors OUTSIDE of GI
If in GI–Liver will filter out 5HT due to first pass
Carcinoid Tumor cells
Neuroendocrine cells
Carcinoid Syndrome Tx
Somatostatin anologue–octreotide
Gastrin secreting tumor of pancreas or duodenum rugal thickening of stomach stomach acid hypersecretion Recurrent ulcers (down to ileum) MEN Type 1
Zollinger Ellison Syndrome
MEN Type 1 (Wermer’s Syndrome)
Parathyroid Tumors
Pituitary Tumors
Pancreati Endocrine Tumors (ZE Syndrome)
Kidney stones, stomach ulcers
“3 P’s”
MEN 2A (Sipple Syndrome)
Pheochromocytoma
Parathyroid Tumor
Medullary Thyroid Tumor–Calcitonin
“2 P’s”
MEN 2B
Pheochromocytoma
Medullary Thyroid CA
Oral/intestinal ganglioneuromatosis–associated marfanoid habits
“1 P”
Gene mutation associated with MEN 2A and 2B
ret gene mutation
Inheritance patter of MEN syndromes
Autosomal Dominant
