Gen Surg 3/13/17 Flashcards
MEN1
Pituitary (any pit tumor, functioning or non)
Parathyroid (hyper calcemia)
Pancreas (endocrine)
- Zollinger Ellison gastrinoma refractory PUD
- Insulinoma hypoglycemia… get C-peptide to check for endogenous vs exogenous, get Secratagogue screen to make sure not ingesting sulfonylureas
- The HARD P’s
- MEN Gene
MEN2
Both Ret Oncogene
MEN2A
pheochromocytoma
medullary thyroid ca (Parafollicular C-cells calcitonin)
ParAthyroid
MEN2B
pheochromocytoma
medullary thyroid ca (Parafollicular C-cells calcitonin)
Neuronal Beuronal… also Marfanoid MEN2Barfanoid
MEN syndromes
MEN1 *MEN Gene
Pituitary (any pit tumor, functioning or non)
Parathyroid (hyper calcemia)
Pancreas (endocrine)
-Zollinger Ellison gastrinoma refractory PUD
-Insulinoma hypoglycemia… get C-peptide to check for endogenous vs exogenous, get Secratagogue screen to make sure not ingesting sulfonylureas
*The HARD P’s
MEN2A *RET oncogene
pheochromocytoma
medullary thyroid ca (Parafollicular C-cells Calcitonin)
ParAthyroid
MEN2B *RET oncogene
pheochromocytoma
medullary ca (Parafollicular C-cells Calcitonin)
Neuronal Beuronal.. also Marfanoid MEN2Barfanoid
TF
Von Hippel Lindae assoc w thyoid and parathyroid issue…
F
Pheochromocytoma
not thyroid parathyroid (that would be MEN2A)
P53 mut think
retinoblastoma
colon cancer
APC mut think
colon cancer
VHL mut think
pheochromocytoma
Von-Hippel Lindau
elevated calcium low phosphorous with normal renal function think…
parathyroid neoplasm
key labs in vitamin D deficiency
HIGH PTH
stimulated by LOW CALCIUM
layers of adrenal
GFR
glomerulosa fasciculata reticulata
the deeper you go the better it gets
salt sugar sex
aldo cortisol testosterone
Cortisol excess causes
Cushing syndrome
Causes of Cushing Syndrome
Cortisol excess
ACTH secreting Small Cell Lung Cancer
ACTH secreting Pituitary Adenoma
Cortisol secreting Adrenal Neoplasm
Exogenous Corticosteroids
Cushing Syndrome vs Cushing Disease
Syndrome caused by any Cortisol Excess
- SCLC, Pit Ad secreting ACTH
- Adrenal Neoplasm secreting Cortisol, exogenous Corticosteroids
Cushing Disease is ACTH secreting Pituitary Adenoma
Cushing Syndrome Path Pres Dx Tx
ACTH dep (pit ad, sclc) or indep (cort secreting adrenal neoplasm or exogenous corticosteroids)
HTN DM Obese (not a big help given America)
Moon facies, Acne, Truncal obesity, Buffalo hump, Purple striae
Low THen High + 24hr U Cort or Late Night Saliva Cort
-Low-dose Dexameth suppression - if does not suppress – Cushing Syndrome
-acTH
if norm, adrenal tumor CT MRI Resect
-If acTH HIGH, do HIGH-dose Dexaemth suppression test
if works Cushing Dz (prim pit acth adenoma) Resect
if fails Ectopic Cortisol Tumor (eg SCLC) Pan Scan
lab workup in Cushing Disease
tx
low dose dexamethasone suppression test does not suppress cortisol or ACTH (confirms Cushing Syndrome)
ACTH high
High dose dexamethasone suppression test works, suppresses ACTH a bit
it’s a primary pituitary adenoma secreting ACTH
you need to Resect it
lab workup in cortisol secreting adrenal neoplasm
tx
low dose dexamethasone suppression test does not suppress cortisol or ACTH (confirms Cushing Syndrome)
ACTH normal
it’s a primary adrenal adenoma secreting Cortisol
Confirm with CT MRI
Resect that shit
Lab workup in ectopic ACTH producing neoplasm
tx
low dose dexamethasone suppression test does not suppress cortisol or ACTH (confirms Cushing Syndrome)
ACTH high
High dose dexamethasone suppression does not work, ACTH remains high
It’s an ectopic ACTH producing tumor (e.g. SCLC), PAN SCAN
When can you say, “Cushing syndrome”
When cushing signs and symptoms
And
2/3 disgnostic tests
- Low dose dexamethasone suppression test (Cortisol not suppressed)
- 24hr urine cortisol
- Late night salivary cortisol
Addison's disease Define/Path Pres Dx Tx
Primary hypOCortisolism (Adrenal deficiency, not pituitary)
Acute (eg adrenal hemorrhage)
-hypotension because no cortisol for tone no aldosterone for fluid retention
-n/v, coma
Chronic (eg infiltrative.. autoimmune, mets)
-hypotension more Orthostatic (no Cortisol)
-hyperpigmentation (high ACTH)
-low sodium high potassium (no Aldo)
Early AM cortisol (if normal, not Addison’s)
If normal get
Cosyntropin (ACTH analogue) stim test
-if works, stims cortisol, pituitary issue,
get MRI, give Cortisol
-if does not work, cortisol still low, it’s primary adrenal addison’s
get CT and MRI, give Cortisol and Fludricortisone
What secretes renin
Juxtaglomerular apparatus
in the Thick Ascending Limb
What does Aldosterone do
Activates sodium transport resorption at the expense of potassium secretion so that more water can be resorbed across aquaporins
When is renin secreted by juxtaglomerular cells in the thick ascending limb
When it sees low flow through the tubules
TF
Aldosterone secreting tumor from adrenal affects hpa axis like cortisol secreting tumor
F
Aldo affects completely different axis, the RAAS
Conn’s syndrome
Define
Primary aldosterone secreting adrenal tumor causing refractory hypertension (3+ meds) and hypokalemia (on test but not always in life)
HyperAldosteronism Path Pres Dx Tx
Conn Syndrome -primary aldosterone secreting adrenal tumor Renovascular Hypertension -fibromyscular dysplasia young female -atherosclerosis old man
Refractory Secondary Hypertension
-refractory to 3+ antihypertensive meds
HypoKalemia (on test, maybe not in life)
Aldo/Renin ratio
-if both normal, licorice induced pseudohyperaldosteronism (by inhibiting an enzyme and making mineralcorticoid receptors super sensitive to cortisol… or a few genetic syndromes
-if both elevated and ratio v10, Renovascular (still driven by renin),
Angiogram to confirm stenosis
FMD gets stented Atherosclerosis tx medically
-if Aldo elevated ratio ^30, Conn Syndrome
Salt Suppression test fails to vAldo
MRI mass maybe but not always the cause
Adrenal Vein Sampling to confirm Conn
RESECT Conn
Pheochromocytoma Path Pres Dx Tx
Primary catecholamine secreting tumor of adrenal medulla
Paroxismal Pain (headache) Pressure (htn) Palpitation (tachyc) Persperation
Plasma free catecholamines if pretty sure and needs urgent tx
24Hr Urine Metanephrines or VMA more sensitive if less urgent have more time
CT MRI abd
Adrenal Vein Sampling
Alpha block (don't want to respond to masdive cc release with adrenal manipulation) Beta block (don't want beta unopposed by alpha) Resect
Adrenal Incidentaloma Path Pres Dx Tx
Probably nothing, no pathogenesis
Mass found incidentally on imaging for something else
Rule out Cushing Pheo Conn
with 24Hr Urine study (Cortisol, Metanephrines/VMA)
and with Aldo:Renin ratio
Resect if ^4cm or hyperfunctioning
Follow if v4cm not hyperfunctioning
Toddler with claudication limiting ambulation
Suspect this
Get this
suspect Coarctation of the aorta
get Chest CT Angiogram
Adult with Resistant HTN
and Rib Notching on CXR
Suspect this
Get this
Coarctation of the Aorta
Chest CT Angiogram
Which has reduced pulses and pressures below the waist
Aortic Coarctation
or
PAD
both
If suspect coarctation,
Chest CT Angiogram
Barterr syndrome looks like….
Gitelman syndrome looks like….
Barterr syndrome looks like….
Furosemide…
Gitelman syndrome looks like….
HCTZ…
When does HTN cause CKD
vs CKD cause HTN
early closer to normal CKD caused By HTN
later closer to dialysis CKD Causes HTN