endo path Flashcards
cushings etiology
increased cortisol d/t
- endogenous corticosteroids
- primary adrenal adenoma, hyperplasia, or carcinoma
- ACTH-secreting pit adenoma (Cushings disease) or pareneoplasitc ACTH secretion
endogenous corticosteroids
decreased ACTH
b/l adrenal atrophy
MCC
primary adrenal adenoma, hyperplasia, or carcinoma
decreased ACTH
atrophy of uninvolved adrenal gland
can also present w/pseudohyperaldosteronism
ACTH secreting adenoma or paraneoplastic ACTH secretion
results in increased ACTH
b/l adrenal hyperplasia
cushings findings
HTN weight gain moon facies truncal obestiy buffalo hump skin changes osteoporosis hyperglycemia amenorrhea immunosupression
Dexamethasone suppression test
cushings disease will suppress
ectopic ACTH secretion will not supress
CRH stimulation test
cushings dis-> increase in ACTH and cortisol
ectopic ACTH secretion -> no increase
symptoms of adrenal insufficiency
weakness fatigue orthostatic hypotension mm aches weight loss GI sugar and/or salt cravings
Dx of adrenal insufficiency
measurement of serum electrocluces am cortisol ACTH ACTH stimulation test metyrapone stimulation test
metyrapone stimulation test
blocks last step of cortisol synthesis
normal response in decreased cortisol and increased ACTH
in adrenal insufficiency ACTH does not increase
primary adrenal insufficiency
hypotension hyperkalemia metaboic acidosis skin and mucosal hyperpigmentation chronic -> addisons disease
causes of addisons
autoimmune (MC)
TB
mets
Waterhouse-friderichsen syndomre
N. menigitidis
secondary adrenal insufficiency
decreased ACTH
no hyperpigmentation
no hyperkalemia
tertiary adrenal insufficiency
pt w/chronic exogenous steroid use precipitated by abrupt withdrawal
aldosterone unaffected
neuroblastoma
MC tumor of adrenal medulla in kids
usually
neuroblastoma presentation
distended abdomen firm irregular mass that can cross midline (wilms-tumor) opsoclonus-myoclonus sundroem HVA and VMA increased in urine bombesin and neuron-specific enolase _ rarely causes HTN N-myc
pheo etiology
MC tumor of adrenal medulla in adults
derived from chromaffin cells from neural crest
pheo rule of 10’s
10% malignant 10% b/l 10% extra-adrenal 10% calcify 10% kids
pheo symptoms
5 Ps pressure (increased BP) pain (HA) perspiration palpitations pallor
pheo associations
NF1
VHL
MEN2A and 2B
pheo findings
increased catecholamines and metanephrines in urine and plasma
Tx of pheo
irreversilbe alpha antagonists (phenoxybenzamine)
followed by BB prior to tumor resection
if BB first -> HTN crisis
hypothyroidism symptoms
cold intolerance weight gain, decreased appetite hypoactivity, lethargy, fatigue, weakness constipation decreased reflexes myxedema (facial/periorbital) dry, cool skin, coarse brittle hair bradycardia, dyspnea on exertion
hypothyroidism labs
increased TSH (best test)
decreased free T3/4
hypercholesterolemia (d/t decreased LDL R)
hyperthyroidism symptoms
heat intolerance weight loss, increased appetite hyperactivity diarrhea increased reflexes pretibial myxedema periorbital edema warm moist skin, fine hair chest pain, palpitations, arrhythmias (increased number and sensitivity of betaRs)
hyperthyroidism labs
decreased TSH if primary
increased free or total T3/4
hypocholesterolemia (d/t increased LDL R)
hashimotos
MCC of hypothyroidism in iodine sufficient regions anti-thyroid peroxidase anti-microsomal anti-thyroglobulin HLA-DR5 increased risk of Hodgkin lymphoma
hashimotos presentation
may have transient hyperthyroid stage d/t thyrotoxicosis during follicular rupture
moderately enlarged, non-tender thyroid
hashitmotos histo
Hurthle cells
lymphoid aggregate w/germinal centers
congenital hypothyroidism/cretinism
severe fetal hypothyroidism
thyroid dsygenesis MCC in US
findings of congenital hypothyroidism
6Ps pot-bellied pale puffy face w/protruding umbilicus protuberant tongue poor brain development
subacute thyroiditis
aka de Quervain
self-limited, often follows flu-like illness
may be hyperthyroid early then hypo
subacute thyroiditis histo
granulomatous inflammation
subacute thyroiditis findings
increased ESR
jaw pain
early inflammation
very tender thyroid
riedel thyroiditis
thyroid replaced by fibrous tissue
mimics anaplastic carcinoma
may be manifestation of IgG4 related systemic disease
fixed, hard, painless goiter
IgG4 systemic diseases
autoimmune pancreatitis
retroperitoneal fibrosis
noninfectious aortitis
graves
MCC of hyperthyroidism autoAbs stim TSH R hyperthyroid, diffuse goiter retro-orbital fibroblasts dermal fibroblasts (pre-tibial myxedema)
toxic multinodular goiter
focal patches of hyperfnxing follicular cells, working independently of TSH d/t mutation in TSH R
increased release of T3/4
hot nodules are rarely malignant
thyroid storm
stress-induced catecholamine surge
serious complication of thyrotoxicosis
agitation, delirium, fever, diarrhea, coma, tacharrythmia (COD)
increased ALP d/t bone turnover
Tx of thyroid storm
3 Ps
Propanolol (or other BB)
PTU
Prednisolone (or other corticosteroids)
Jod-Basedow phenomenon
thyrotoxicosis after iodine deficient goiter given iodine
papillary carcinoma
MC thyroid CA excellent prognosis orphan annie eyes psammoma bodies nuclear grooves lymphatic invasion common
orphan annie eyes
empty appearing nuclei w/central celaring
diagnostic of papillary carcinoma
papillary carcinoma risks
childhood radiation
RET
BRAF
follicular carcinoma
good prognosis
invases thyroid capsule (unlike follicular adenoma)
uniform follicles
medullary carcinoma
parafollicular C-cells
produces calcitonin
sheets of cells in amyloid stroma
hematogeneous spread
medullary carcinoma assocaitions
MEN 2A, 2B -> RET mutations
undifferentiated anaplastic carcinoma
older pts
invades local structures
poor prognosis
lymphoma
associated w/hashimotos
hodgkin type
hypoparathyroidism signs
chvostek sign- tapping of cheek (facial nn) -> contractions of facial mm
trousseau sign- occulusion of brachial a w/BP cuff -> carpal spasm
psudohyperparathyroidism
albright hereditary osteodystophy unresponsiveness of kidney to PTH hypocalcemia shortened 4th and 5th digits short stature AD
familial hypocalciuric hypercalcemia
defective Ca sensingR on parathyroid
PTH cannot be suppressed by increased Ca -> mild hypercalcemia w/normal to increased PTH
primary hyperparathyroidism
stones, bones, groans, and psychiatric overtones
usually d/t parathyroid adenoma or hyperplasia
hypercalcemia, hypercalciuria (stones)
hypophosphatemia
increased PTH
increased ALP, cAMP, in urine
asymptomatic
may present w/constipation and/or derpession
osteitis fibrosa cystica
cystic bone spaces filled w/brown fibrous tissue and hemosiderin (brown tumor)
painful
complication of primary hyperparathyroidism
secondary hyperparathyroidism
d/t decreased Ca and/or increased phos
MCC chronic renal disease
hypocalcemia, hyperphosphatemia
increased ALP, PTH
renal osteodystrophy
bone lesions d/t a secondary or tertiary hyperparathyroidsim that is d/t renal disease
tertiary hyperparathyroidism
refractory/autonomous hyperparathyroidism from chronic renal disease
very increased PTH
increased Ca
pituitary adenoma
MC is prolactinoma (bengin)
Tx bromocriptine or cabergoline
acromegaly
excessive GH in adults, usually d/t pit adenoma
large tongue w/deep furrows
imparied glucose tolerance
increased risk of colorectal polyps and CA
acromegaly Dx
increased serum IGF-1
failure to suppress serum GH with oral glucose tolerance test
pit mass on MRI
acromegaly Tx
surgery
octreotide (somatostatin analog)
pegvisomant (GHR antagonist)
SIADH
euvolemic hyponatremia (d/t decreased aldosterone)
urine osm>blood
very low Na -> cerebral edema and seizures
correct slowly
what happenes if you correct hyponatremia too quickly
osmotic demyelination syndrome/central pontine meylinolysis
causes of SIADH
ectopic ADH (small cell lung CA)
CNS disorders/head trauma
pulmonary disease
drugs (cyclophosphamide)
Tx of SIADH
fluid restriction IV hypertonic saline conivaptan tolvaptan demeclocycline
hypopituitarism causes
nonsecreting pit adenoma craniopharyngioma sheehan syndrome empty sella syndroem pit apoplexy brain injury radiation
glucagonoma
alpha cell tumor dermatitis (necrolytic migratory erythema) DM DVT depression
insulinoma
beta cell tumor
hypoglycemia
whipple triad
whipple triad
low blood glucose
hypoglycemia
resolution of symptoms w/glucose
carcinoid syndroem
rare, but MCC malignancy of small intestine
usually small bowel tumors secreting 5-HT
non seen if tumor limited to GI
recurrent diarrhea, cutaneous flushing, asthmatic wheezing, right valvular disease
increased 5-HIAA in urine
niacin deficiency
carcinoid Tx
surgery
somatostatin analog- octreotide
carcinoid rule of thirds
1/3 mets
1/3 present w/secondary malignancy
1/3 are multiple
zollinger-ellison syndrome
Gastrin-secreting tumor of pancreas or duodenum acid hypersecretion -> recurrent ulcers pain, diarrhea \+ secretin stimulation test MEN1
secretin stimulation test
gastrin levels remain elevated after administration of secretin
MEN1
Parathyroid Pituitary tumors (prolactin or GH) Pancreatic endocrine (ZE, insulinomas, VIPomas, glucagonomas) MEN1 gene (tumor supressor)
MEN 2A
Parathyroid Pheo Medullary thyroid carcinoma (secretes calcitonin) Marfanoid RET gene (TKR)
MEN 2B
Pheo medullary thyroid carcinoma (secretes calcitonin) oral/intestinal ganglioneuromatosis marfanoid mutation in RET
