Term
| most common causes of hyperfunction 4 |
|
Definition
adenoma - most common
acute inflammation
hyperplasia
cancer |
|
|
Term
| most common caues of hypofunction 6 |
|
Definition
autoimmune destruction - most common
destruction: infection, surgery, radiation
decreased stimulation
enzyme deficiency
neoplasia
congenital disorder |
|
|
Term
| how does adenoma cause hyperfunction |
|
Definition
mass of tumor causes unregulated release of hormone from anaplastic cell due to genetic changes
release of hormones from non-endocrine tissue is paraneoplastic |
|
|
Term
| how does neoplasia cause hypofunction |
|
Definition
tumor cells derived from non-functional components of endocrine gland compress functional tissue
hormones dont release due to genetic changes in the cell |
|
|
Term
| explain how the pituitary recieves blood and endocrine stimulation |
|
Definition
ap gets blood from portal venous
pp gets blood from its own artery
ap is stimulated by hypothalamus to make hormones via portal vein delivery
pp recieves hormones through stalk into gland |
|
|
Term
| 6 anterior pituitary cells and what they make |
|
Definition
lactotroph > prolactin
mammosomatotroph > GH, prolactin
somatotroph > GH
thyrotroph > thyrotropin (TSH)
gonadotroph > FSH/LH
corticotroph > corticotropin (ACTH) |
|
|
Term
| 6 anterior pituitary hormones and what regulates them |
|
Definition
prolactin +TRH +GNRH -dopamine -somatostatin
GH +GNRH - somatostatin
TSH +TRH
FSH and LH +GNRH
ACTH +CRH |
|
|
Term
| 2 posterior pitutiary hormones and their functions |
|
Definition
ADH: regulates water in CD
oxytocin: mediates uterine contraction and release of breast milk when sucking |
|
|
Term
| causes of hyperpituitarism 5 |
|
Definition
adenoma - most common
hyperplasia
cancer
non-pituitary tumor
hypothalamus |
|
|
Term
| causes of hypopotuitarism 9 |
|
Definition
destruction: ischemia, surgery, radiation, inflammation
nonfunctioning adenoma: compress and destory functional tissue
craniopharyngioma: most common in kids
hypothalamic
sheehan syndrome
rathke cleft cyst
empty sella syndrome
genetic defects
specific hypofunctions |
|
|
Term
| pituitary local mass effect signs 3 |
|
Definition
silent tumors are most likley to have mass effect
lesion near sella: compress optic chiasm and cause bitemporal hemianopia (lateral visual deficit)
elevated ICP: headache, nausea, vomiting
pituitary apoplexy |
|
|
Term
| pituitary apoplexy: cause, signs 3, prognosis |
|
Definition
acute hrmorrhage: rapid enlargement causes lesion to outgrow blood supply and hemorrhage into adenoma
headache, hypopituitary, diplopia
surgical emergency, can cause sudden death |
|
|
Term
| 5 types of pituitary adenoma, cell type, which is most common, 1nd most common |
|
Definition
prolactinoma - lacrotroph - 1st
growth hormone - somatotroph - 2nd
cortitroph - corticotroph
gonadotroph - LH/FSH
thyrotroph - TSH |
|
|
Term
| if someone has multiple cell types or lesions in their pituitary adenoma what should you suspect |
|
Definition
| multiple endocrine neoplasia 1 |
|
|
Term
| which is more common hyper or hypopituitary |
|
Definition
|
|
Term
| genetic abnormalities associated with pituitary adenoma 4 |
|
Definition
monoclonal (even plurihumoral tumors)
some come from pituitary stems cells and differentiate
MEN1 mutation in non-sporatic
RAS activation with c-MYC in agressive
GNAS1 phosphorlyation mutation in somaatototroph, corticotroph, TSH, PTH |
|
|
Term
| what are 2 diseases that have a GNAS1 mutation |
|
Definition
pituitary adenoma: somatotroph, cortioctroph, TSH, PTH
intraductal mucinous pancreatic andeoma |
|
|
Term
| if there is a plurihumoral pituitary adenoma what is the most common combination |
|
Definition
| growth hormone and prolactin |
|
|
Term
| which pituitary adenoma is often silent, what is it called |
|
Definition
| gonadotroph cell adenoma, null cell adenoma is silent form |
|
|
Term
| causes of prolactin elevation 8 |
|
Definition
intrasellar tumor producing prolactin
nonfunctioning pit tumor compresses stalk
hypothalamic neoplasm
head trauma
dopamine blocking drugs
pregnancy/breast feeding
renal failure decreases clearance
primary hypothyroidism (inc TRH) |
|
|
Term
| what is a craniopharyngioma |
|
Definition
|
|
Term
| what drugs block dopamine |
|
Definition
dopamine antagonist: metoclopramide, phenothiazine, butyrophenone
delivery blocker: MAOI, TCA
antiHTN: verapmil, reispine, methyldopa |
|
|
Term
| signs of prolactinoma in perimenopausal women 5 |
|
Definition
amenorrhea
infertility
galactorrhea
oligomenorrhea
seen after DC or OC often |
|
|
Term
| signs of prolactinoma in postmenopausal women and men 7 |
|
Definition
| mass effect: headache, neuro signs, impotence, infertility, decreased lobido, galactorrhea, gynecomastia |
|
|
Term
| diagnostic of prolactinoma: 2 tests, 3 things to rule out |
|
Definition
serum prolactin: mild elevation (pulsatile so not always high)
MRI is conformation test, usually mass is small
biopsy is not needed
rule out pregnancy, thyroid, renal dysfunction |
|
|
Term
| signs of a prolactinoma biopsy 4 |
|
Definition
dystrophic calcification
plasmomma bodies: microcalcification
pituitary stones
gross calcification of tumor |
|
|
Term
| treatment of prolactinoma |
|
Definition
dopamine agonist: bromocryptine
inhibits prolactin and shrinks tumor |
|
|
Term
|
Definition
pituitary adenoma
cancer
lymphoma
pancreatic islet cell tumors
paraneoplastic syndrome |
|
|
Term
| explain how a pituitary adenoma releases GH |
|
Definition
mutation of GTPase a-subunit of Gs.
proliferation of somatotroph and mamosomatotroph cells causing release of GH that induces IGF-1 which induces cell proliferation and stops apoptosis |
|
|
Term
|
Definition
dense granulated: slow growing, more in adults >50yo
sparse granulated: fast growing, more in young |
|
|
Term
| signs of GH adenoma in adults 10 |
|
Definition
7-10y until diagnosis
acromeagly: big soft tissue, distal bones (feet, hands, jaw, ears, nose, visceral organs, heart),
thick skin
DEGENERATIVE ARTHRITIS
HEART FAILURE - #1 COD
HTN
headache
visual disturbance
sleep apnea
SECONDARY DIABETES: GH INCREASES GLUCOSE UPTAKE INTO CELLS |
|
|
Term
| signs of GH adenoma in kids |
|
Definition
| gigantism: linear bone growth increases body size, long arm snad legs |
|
|
Term
| how is GH adenoma diagnosed 3 |
|
Definition
elevated GH and IGF-1: GH goes to liver and causes increased IFG-1 which mediates tissue growth
AUTONOMOUS SECRETION OF GH: MOST SENSITIVE - FAILURE TO SUPRESS GH WITH ORAL GLUCOSE LOAD
MRI with contrast: most sensitive for determining source of GH |
|
|
Term
| treatment of GH adenoma 2 |
|
Definition
octerotide: blocks response in anterior pituitary to GNRH
GH receptor antagonist |
|
|
Term
| causes of increased ACTH 4 |
|
Definition
cortiticotroph adenoma
cushing syndrome
cushing disease: ACTH made due to tumor
post-adrenalectomy: nelson syndrome |
|
|
Term
|
Definition
pre-existing corticotroph tumor grows rapidly after surgical removal of adrenal gland due to loss of inhibitor effect of adrenal corticosteroids
corticotrophs pump out ACTH to help body demand for cortisol and adenoma grows large because adrenal glands are gone
NO HYPERCORTISOLISM |
|
|
Term
|
Definition
mass effect
hyperpigmentation: POMC > ACTH + MSH + B-endorphin |
|
|
Term
| signs of gonadotroph adenoma 3 |
|
Definition
neurologic symptoms due to mass effect
reduced testosterone in men decreases energy and libido
amenorrhea in premenopausal women |
|
|
Term
| cause of gonadotroph tumor symptoms |
|
Definition
| HYPOFUNCTION (NOT HYPERFUNCTION): non-functioning clonal cells compress functioning tissue |
|
|
Term
| how can you differentiate pituitary adenoma and pituitary carcinoma |
|
Definition
| histologically similar, diagnosis needs demonstration of metastasis |
|
|
Term
| what is the definition of hypopituitary |
|
Definition
| insufficient hormones when >75% of the pituitary parenchyma is lost |
|
|
Term
| non-functioning pituitary adenoma: signs 3 |
|
Definition
compresses pituitary causing loss of function
apoplexy: bleeding causing it to rapidly grow and compress
bitemporal hemianopsia |
|
|
Term
| what is the most common ischemic necorosis of the pituitary |
|
Definition
|
|
Term
| cause of sheehan syndrome |
|
Definition
| in pregnancy there is high demand for pituitary hormones so it enlarges 2x but blood supply does not increase, anterior pituitary uses portal venous system so if there is blood loss (obstretic shock or hemorrhage) it causes infarct |
|
|
Term
| signs of sheehan syndrome 7 |
|
Definition
poor lactation
loss of pubic hair (LH)
ovarian failure
amenorrhea
adrenal insurfficiency
hypothyroid (later)
pallor (MSH deficiency) |
|
|
Term
|
Definition
anterior pituitary is embryologically derived from rathke pouch
cysts accumulate proteinaceous fluid and expand, compormising normal gland |
|
|
Term
| empty sella syndrome 2 types |
|
Definition
condition that destorys part of all of the pituitary
primary: diaphragm sella defect allows arachnoid and CSF to hermiate compressing pituitary in obese and women with multiple pregnancies
secondary: surgical removal of pituitary due to mass or pituitary necrosis from loss of function |
|
|
Term
| genetic defects that cause deficiencies of pituitary hormones 2 |
|
Definition
pit01 mutation
a pituitary transcription factor |
|
|
Term
| specific pituitary hormone deficiencies and their signs 4 |
|
Definition
GH: pituitary dwarfism (proportional)
TSH: hypothyroid
ACTH: hypoadrenalism, pallor
prolactin: no postpartum lactation |
|
|
Term
|
Definition
| kidney cannot resorb water from urine causing large volume (polyuria) dilute urine (LOW SPECIFIC GRAVITY), polydypsia, thirst, hyponatremia |
|
|
Term
| 2 types of DI and how to diagnostially distinguish |
|
Definition
central: water deprivation test, cannot concentrate urine
nephrogenic: no response to desmopressin |
|
|
Term
|
Definition
head trauma
tumor
infection
inflammation of hypothalamus/pituitary |
|
|
Term
|
Definition
|
|
Term
| nephrogenic DI: cause and pathogenesis |
|
Definition
| inherited mutation or drug (lithium) makes tubules not respond to ADH |
|
|
Term
|
Definition
malignant neoplasm: small CC
non-neoplastic diease of lung COPD, pulmonary infection
hypothalamus or post. pituitary injury
drugs |
|
|
Term
|
Definition
| ADH causes reabsorption of water and hyponatremia, cerebral edema, neurological dysfunction (mental status change, seizure) |
|
|
Term
|
Definition
water restriction
dimethylcycline (ADH blocker) |
|
|
Term
| explain how the thyroid makes hormones |
|
Definition
stimulated by TSH from anterior pituitary
takes up tyrosine which turns into thyroglobin and goes into follicle lumen
takes up iodine which is OXIDIZED with peroxidase into I2
I2 and thyroglobin ORGANIFIED with peroxidase to monodiotyrosine or diididothyrosine
mono and di COUPLED via peroxidase to T3 (most powerful)
di and di COUPLED via peroxidase to T4 (most prevelant)
T4 binds to TBG |
|
|
Term
| what are some things that effect the TBG levels 3, how does this change other thyroid marker levels |
|
Definition
estrogen increases it
steroids and nephrotic syndrome decrease it
affects total T4 not free T4 |
|
|
Term
| causes of primary hypothyroid 4 |
|
Definition
thyroprivic: radiation or thyroidectomy
goiterous: increased TSH enlarges thyroid
cretinism
myexedema |
|
|
Term
| causes of secondary hypothyroid |
|
Definition
| pituitary abnormality decreases TSH |
|
|
Term
| causes of tertiary hypothyroid |
|
Definition
| hypothalamus abnormality decreases TRH |
|
|
Term
| causes of goiterous hypothyroid 4 |
|
Definition
iodine deficiency
AUTOIMMUNE: HASHIMOTOS
drugs
inborn errors of thyroid metabolism |
|
|
Term
| what is the most common cause of hypothyroid in iodine deficient and iodine prevalent areas |
|
Definition
deficient: iodine deficiency
prevalent: HASHIMOTOS |
|
|
Term
| 4 drugs causing hypothyroid |
|
Definition
methimazole
PTU
lithium
aminosalicylic acid |
|
|
Term
|
Definition
hashimotos
painless postpartum
painless sporatic
painful subacute
suppurative
ridels |
|
|
Term
|
Definition
painless postpartum
painless sporatic |
|
|
Term
| 3 chronic lymphocytic thyroiditis |
|
Definition
painless postpartum
painless sporatic
hashimotos |
|
|
Term
| causes of primary hyperthyroidism 3 |
|
Definition
hyperplasia GRAVES DISEASE
hyperfunction multinodular goiter
hyperfunction adenoma of thyroid |
|
|
Term
| causes of secondary hyperthyroidism |
|
Definition
| pituitary abnormality increases TSH |
|
|
Term
| causes of tertiary hyperthyroidism |
|
Definition
| hypothalamic abnormality increases TRH |
|
|
Term
| 2 thyroid goiter diseases |
|
Definition
diffuse nontoxic simple gouter
multinodular goiter |
|
|
Term
| 2 benign thyroid neoplasms |
|
Definition
thyroid nodules
follicular adenomas |
|
|
Term
| 4 thyroid carcinomas, which is most common |
|
Definition
papillary - most common
follicular
medullary
anaplastic |
|
|
Term
|
Definition
inborn error of thyroid metabolism
maternal hypothyroid: before fetus develops
thyroid agenesis in infant
dyshormonogenic goiter: unable to make TH due to peroxidase deficiency
endemic iodine deficiency area |
|
|
Term
|
Definition
severe mental retardation: christ like
short stature
skeletal abnormalities
coarse facial features
enlarged protruding tongue
umbilical hernia |
|
|
Term
|
Definition
| INFANT TO EARLY CHILDHOOD |
|
|
Term
|
Definition
| MANIFESTATIONS MORE SEVERE IN YOUNG BUT MORE COMMON IN OLDER CHILDREN AND ADULTS |
|
|
Term
|
Definition
iodine deficiency
hashimotos
lithium
thyroid ablation or removal |
|
|
Term
|
Definition
decreased BMR and SNS
fatigue, apathy, mental slug
depression
muscle weakness
EDEMA
broad face
enlarged tongue
voice deepens
cold interolance
cool pale skin
weight gain (normal appetite)
reduced CO
breathlessness
decreased exertion capacity
bradycardia
SOA
constipation
decreased sweating
oligomenorrhea
hypercholesterolemia |
|
|
Term
| how can some thyroiditis syndomes make thyrotoxicosis and hypothyroidism |
|
Definition
TH is stored in gland
in thyroiditis inflammatory cell damages follicles which release TH into circulation causing thyrotoxicosis
as stores deplete and thyroid heals hypothyroidism results untils cells replaced and enough TH is made
(rare occasions hypothyroid persists) |
|
|
Term
|
Definition
hashimotos: all, peak 30-50
painless postpartum: childbearing
painless sporatic: all, peak 30-40
painful subacute: 20-60
suppurative: kids, 20-40
riedels: 30-60 |
|
|
Term
| 6 thyroditis gender predominances |
|
Definition
hashimotos: male
painless postpartum: female
painless sporatic: female
painful subacute: female
suppurative: even
riedels: male |
|
|
Term
|
Definition
hashimotos: autoimmuune
painless postpartum: autoimmune
painless sporatic: autoimmune
painful subacute: viral resp infection
suppurative: infection
riedels: unk |
|
|
Term
| 6 thyroditis pathological findings |
|
Definition
hashimotos: lymphocyic infiltrate, germinal centers, fibrosis
painless postpartum: lymphocytic infiltrate
painless sporatic: lymphocytic infiltrate
painful subacute: giant cells, granulomas
suppurative: abscess
riedels: dense fibrosis |
|
|
Term
| 6 thyroditis thyroid function |
|
Definition
hashimotos: hypo
painless postpartum: tox > hypo
painless sporatic: tox > hypo
painful subacute: tox > hypo
suppurative: eithroid
riedels: euthroid |
|
|
Term
| 6 thyroditis TPO antibodies |
|
Definition
hashimotos: high
painless postpartum: high
painless sporatic: high
painful subacute: low or none
suppurative: no
riedels: usually |
|
|
Term
|
Definition
hashimotos: normal
painless postpartum: normal
painless sporatic: normal
painful subacute: high
suppurative: high
riedels: normal |
|
|
Term
| 6 thyroditis iodine uptake |
|
Definition
hashimotos: variable
painless postpartum: low
painless sporatic: low
painful subacute: low
suppurative: normal
riedels: low or normal |
|
|
Term
|
Definition
45-65 yo F>M
major cause of non-endemic goiter in children |
|
|
Term
| what are the three areas of hashimotos pathogenesis |
|
Definition
autoimmune thyroid antigens
immunologic mechanism
genetic susceptability |
|
|
Term
| 3 autoimmune thyroid antigens in hashimotos |
|
Definition
anti-TSH receptor
anti-thyroglobulin
anti-thyroid peroxidase |
|
|
Term
| explain the immunologic mechanism of hashimotos |
|
Definition
thyroid epithelium sensitizes T cells to thyroid antigen
T helper differentiates into CD4/8
Th activates plasma cells which make auto Ab
CD4 activates macrophages
CD8 causes T cell mediated toxicity |
|
|
Term
| what is some proof for the genetic connection in hashimotos 4 |
|
Definition
60% concordance in monozygotics
50% asymptomatic relatives have anti-thyroid Ab
associated with turner syndrome and down syndrome |
|
|
Term
| explain the pathological progression of hashimotos |
|
Definition
initiall hashitoxicosis: current follicles destoryed, TH leaks into blood
then hypothyroid: cells gone, T4 decreases while TSH increases |
|
|
Term
| what markers can we use for hashimots and their significance 2 |
|
Definition
anti-thyroGLOBIN and anti-microsomal Ab
signs of thyroid damage
do not mediate disease
only used as markers |
|
|
Term
| 1 complication of hashimotos and why |
|
Definition
| B cell lymphoma: increased germinal centers make post germinal center B cells that generate maginal zone which leads to lymphma and thyroid enlargement |
|
|
Term
| morphological characteristcs of hashimotos 5 |
|
Definition
thyroid gradually replaced by mononuclear cell infiltrates and fibrosis
chronic inflammation and formation of germinal centers
diffuse thyroid enlargement
HURTHLE CELLS: metaplastic response of follicular eithelium to injury with eosinophilic, granular cytoplasm
lymphocytes |
|
|
Term
|
Definition
PAINLESS ENLARGEMENT OF THYROID
middle aged woman
hashitoxicosis
hypothyroidism |
|
|
Term
| autoimmune diseases associated with hashimotos 3 |
|
Definition
SLE
myasthenia gravis
sjogerns |
|
|
Term
| signs of hyperthyroidism 30, which 3 are specific to graves only |
|
Definition
increased BMR and SNS
weight loss (increased appetite)
warm skin
flushing
peripheral vasodilation
heat intolerance
sweating
TACHYCARDIA
increased cardiac contractility
increased peripheral O2 requirements
arrhythmia
tremor
hyperactivity
emotional liability
anxiety
insomnia
thyroid myopathy: decreased muscle mass b/c its used to make proteins
ocular staring gaze - graves
lid lag - graves
exothalamus - graves
hypermotility
malabsorption
diarrhea
OSTEOPEROSIS: bone reabsorption stimulated
fracture
hypercalcemia
oligomenorrhea
hyperchlesterolemia
hyperglycemia: TH causes gluconeogenesis |
|
|
Term
| what is the most common feature of hyperthyroidism, who is most likley to get them |
|
Definition
cardiac manifestations
arrhythmia in the elderly |
|
|
Term
|
Definition
|
|
Term
| 2 parts to the pathognesis process in graves |
|
Definition
genetic susceptability
auto IgG thyroid effect thyroid growth and hormone release |
|
|
Term
| 3 factors of genetic susceptability in graves |
|
Definition
HLA-B8
HLA-DR4
polymorphism in CD8 T cell CTLA-4 gene |
|
|
Term
| 3 auto-antibodies possible graves and what each does |
|
Definition
TSI: IgG mimics TSH, binds TSH receptor, stimulates adenylyl cyclase and causes hormone release
TGI: activates TSG receptors causing proliferation of follicles
TBII: mimic TSH to cause hyperfunction OR inhibit TSH binding causing HYPOfunction |
|
|
Term
| what are the possible combinations of autoantibodies in graves |
|
Definition
HAS TO HAVE TSI which is SPECIFIC TO GRAVES ONLY
could also have TGI or TBII
both forms of TBII (hyper/hypo function) can be in the same person |
|
|
Term
| morpholigcal characteristics of the graves thyroid 2 |
|
Definition
hypertrophy and hyperplasia of follicular epithelium
scalloping of colloid: white area of colloid around thyroid follicles |
|
|
Term
| graves disease triad of symptoms |
|
Definition
hyperplasia of thyroid causes hyperthyroidism
infiltrative opthalmopathy
localized infiltrative dermopathy |
|
|
Term
|
Definition
| permant fibrous deposition may cause persistance of signs over life |
|
|
Term
| hi i'm out of order. why is there edema like broad facial features, enlarged tongue, hoarsness etc in myexedema |
|
Definition
| accumulation of matrix (GAGs, hyaluronic acid) in sin, SC tissue, visceral sites (esp larynx) |
|
|
Term
| graves triad: signs of hyperplasia 2 |
|
Definition
increased blood flow causes bruit
diffuse goiter |
|
|
Term
| graves triad: signs of infiltrative opthalmopathy and why |
|
Definition
EXOPTHALMOS - GRAVES SPECIFIC
orbit perdipocyte fibroblasts have TSH receptors so IgG and T cell cytokines stimulate them to make more GAG causing myexedema
volume of extraocular muscles is increased displacing eyeball forward and weakening muscles |
|
|
Term
| graves triad: signs of infiltrative dermopathy and why |
|
Definition
usually on shins (pretibial myexedema)
scaky thickening orange peel pigmented papule/nodules
fibroblasts of shin have TSH receptors so in response to IgG they secrete GAG causing tibial myexedma and dough like feel |
|
|
Term
| what is the most common cause of thyroid storm |
|
Definition
|
|
Term
| cause and signs 4 of thyroid storm |
|
Definition
abrupt hyperthyroidism releases excess catecholamines during sress
arrhythmia, hyperthermia, vomiting, hypovolemic shock |
|
|
Term
| tx and prognosis of thyroid storm |
|
Definition
medical emergency, arrhythmia popssible
PTU stops oxidation, organification, and coupling in thyroid production and stops conversion of T4>T3 |
|
|
Term
| lab signs of graves disease 4 |
|
Definition
increased T4
decreased TSH: T4 down regulates receptors on anterior pituitary
hypercholesterolemia
hyperglycemia |
|
|
Term
|
Definition
increase B adrenergic tone
decrease thyroid hormone synthesis: PTU blocks peroxidase, radioiodine ablation, thyroidectomy |
|
|
Term
|
Definition
impaired TH synthesis increases TSH causing hypertrophy and hyperplasia of follicular cells
many overcome TH deficiency making patient euthyroid but with goiter
some dont causing goiterous hypothyroid
proportional to level of TH deficiency |
|
|
Term
| cause of diffuse nontoxic goiter |
|
Definition
goiterous diet: interferes with TH synthesis
brassica, cruciferae (cabbage, caluliflower, turnips, cassava) inhibit iodine transport |
|
|
Term
| morphological signs of diffuse nontoxic goiter 3 |
|
Definition
diffuse involvement of entire gland
no nodularity
follicles filled with colloid |
|
|
Term
| 2 types of diffuse nontoxic goiter and their causes |
|
Definition
endemic: soil, water, and food has low iodine
sporatic: goitrogen diet, hereditary enzyme defects (dyshormonogenic goiter) |
|
|
Term
| 2 conditions caused by dyshormonogenic goiter |
|
Definition
cretinism
diffuse nontoxic goiter - sporatic |
|
|
Term
| who is most likley to get a dyshormongenic goiter |
|
Definition
|
|
Term
| cause of multinodular goiter |
|
Definition
| activating mutation in TSH signaling causes better response to trophic hormones in some nodes causing enlarged thyroid with nodules due to relative iodine reficiency |
|
|
Term
| morphological signs of multinodular goiter 5 |
|
Definition
uneven accumulation of colloid: causes tension, hemorrhage, scars, calcifications, increased nodularity
if unredolved recurrent hyperplasia causes involution |
|
|
Term
| 2 types of multinodular goiter, which is most common |
|
Definition
nontoxic euthyroid - most common
plummer syndrome thyrotoxicosis |
|
|
Term
| plummer syndrome: pathogenesis, morphology, signs |
|
Definition
regions become TSH independent and secrete TH without TSH control
hyperactive nodules concentrate radioactive iodine (hot)
causes hyperthyroid symptoms |
|
|
Term
| other than being odd, what is the clinical significance of a multinodular goiter 3 |
|
Definition
| mass effect: airway obstruction, dysphagia, compresses vessels in neck and thorax |
|
|
Term
|
Definition
4x more in women
higher in endemic goiter regions |
|
|
Term
| prognosis of thyroid nodules |
|
Definition
majority not neoplastic
usually indolent (self limited) with 90% survival at 20y |
|
|
Term
| morphology of thyroid nodules |
|
Definition
solitary nodule
palpable discrete swelling
normal in thyroid gland |
|
|
Term
| clues that a thyroid nodule has a high chance of being neoplastic 8 |
|
Definition
solitary
<20 >65yo
rapid growth
male
signs of invasion: dysphagia, neck pain, hoarsness
hx radiation to head or neck
family hx or cancer or polyposis (gardners syndrome)
hot nodule more likley benign and cold 10% malignant |
|
|
Term
| follicular adenma pathognesis |
|
Definition
activation mutation in TSH receptor G protein a-subunit on follicular epithelium
increases cAMP and follicular cell proliferation and hormone release
non-functional, some make TH causing thyrotoxicosis (toxic adenoma) |
|
|
Term
| morphology of follicular adenoma 7 |
|
Definition
3cm
neoplastic: WELL DEFINED CAPSULE, local atypia, clear cells, mitotic figures
intact capsule distinguishes these as benign
HURTHLE CELLS: possible |
|
|
Term
| what are 2 diseaes that have hurthle cells |
|
Definition
follicular adenoma
hashimots thyroiditis |
|
|
Term
| clinical signs of follicular adenoma 3 |
|
Definition
SOLITARY PAINLESS MASS
COLD NODULE NON-FUNCTIONAL
hemosiderin pigment |
|
|
Term
| who gets thyroid carcinoma |
|
Definition
female adults
if in kid or late adult life male rate equals female |
|
|
Term
| who gets papillary thyroid carcinoma |
|
Definition
| 20-40yo female exposed to ionizing radiation as kid (acne tx) |
|
|
Term
| genetic changes in papillary thyroid carcinoma 5 |
|
Definition
RET or NTRK1 rerrangement
BRAF oncogene activating mutation
RAS mutations
general: MAP KINASE PATHWAY ACTIVATED VIA TYROSINE KINASE RECEPTORS INCREASING GROWTH AND DIFFERENTIATION |
|
|
Term
| morphology of papillary thyroid carcinoma 6 |
|
Definition
solitary or multifocal
encapsulated or ill defined
papillae: fibrovascular stalk covered with cuboidal epithelium
nuclei: dispersed chromatin, clear, GROUND GLASS / ORPHAN ANNIE NUCLEI
PLASMOMMA (SIMMONA) BODIES: layers of calcification |
|
|
Term
| signs of papillary thyroid carcinoma 9 |
|
Definition
asymptomatic
freely moving nodule
cold mass
CERVICAL NODE MASS 1ST SIGN
hoarsness
dysphagia
cough
dyspnena
hematogenous metastasis to lung |
|
|
Term
| prognosis of papillary thyroid carcinoma |
|
Definition
excellent
10y survival 95% even if spread to local nodes
worse if <40yo or distant metastasis |
|
|
Term
| pneumonic to remember papillary thyroid carcinoma |
|
Definition
papillary
popular
papillae
palpable nodes
pupil nuclei
psammoma bodies
pediatric pt
positive prognosis |
|
|
Term
| hi, im out of order. pneumonic for thyroid storm |
|
Definition
storm HITS girls cAMP
hyperthyroidism
infection/illness
trauma
surgery
girls more common
cAMP tx involves BB which stop cAMP |
|
|
Term
| follicular thyroid carcinoma: who gets it |
|
Definition
40-60yo women
increased in areas of iodine deficiency |
|
|
Term
| follicular thyroid carcinoma: morphology 3 |
|
Definition
minimally invasive or very invasive
plenty of colloid (looks like papillary adenoma somtimes)
NO CAPSULE OR INVASION THROUGH CAPSULE |
|
|
Term
| follicular thyroid carcinoma: mutations 2 |
|
Definition
RAS oncogene mutation
PAX8-PPARyI fusions |
|
|
Term
| follicular thyroid carcinoma: signs 4 |
|
Definition
slowly enlarging painless nodule
cold usually on scan
rare invades lymphatics
hematogenous dissemination to bone, lung, liver |
|
|
Term
| why do you use fine needle aspiration on thyroid and not normal biopsy |
|
Definition
|
|
Term
| prognosis of follicular thyroid carcinoma |
|
Definition
poor if widley invasive form
10y survival 50% |
|
|
Term
| 4 carcinomas that disseminate hematogenously instead of the normal nodes first |
|
Definition
follicular of thyroid
renal cell
hepatocellular
choriocarcinoma |
|
|
Term
| who gets medullar thyroid carcinoma |
|
Definition
|
|
Term
| medullar thyroid carcinoma: 5 causes |
|
Definition
MEN2A
MEN2B
activating RET mutation seen in MEN2A/B too
familial medullary thyroid carcinoma
80% sporatic |
|
|
Term
| what mutation permits prophylactic thyroidectomy, what diseases is it associated with |
|
Definition
MEN2A/B and medullary thyroid carcinoma
activating RET mutations |
|
|
Term
| medullar thyroid carcinoma: pathogenesis 5 |
|
Definition
PARAFOLLICULAR C CELL HYPERPLASIA - SUSPICIOUS FOR FAMILIAL SYNDROMES
calcitonin secretion: does not caucse hypocalcemia our calcitonin sucks
secrete somatostatin, serotonin, VIP causing VIP diarrhea |
|
|
Term
| medullar thyroid carcinoma: morphology 4, and how it tells us the cause of the cancer |
|
Definition
sporatic: solitary nodule
familial: multiple lesions, C-cell hyperplasia
stroma: amyloid deposits from altered calcitonin |
|
|
Term
| medullar thyroid carcinoma: signs |
|
Definition
neck mass
dysphagia
hpoarsness
PARANEOPLASTIC VIP DIARRHEA
SINGLE COLD NODULE |
|
|
Term
| medullar thyroid carcinoma: prognosis |
|
Definition
MEN2-RET mutation has poor prognosis
prophylactic thyroidectomy needed |
|
|
Term
| anaplastic thyroid carcinoma: who gets it |
|
Definition
|
|
Term
| anaplastic thyroid carcinoma: signs 3 |
|
Definition
HX MULTINODULAR GOITER
hx of thyroid cancer
rapidly enlarging bulky neck mass (compression and invasion symptoms) |
|
|
Term
| anaplastic thyroid carcinoma: morphology |
|
Definition
| malignant cells that look nothing like thyroid tissue |
|
|
Term
| anaplastic thyroid carcinoma: prognosis |
|
Definition
agressive
mortality 100%
no effective therapy
often invades local structures causing dysphagia and respiratory compormise |
|
|
Term
| explain the normal embryology and function of the parathyroid and the hormone functions |
|
Definition
derived from pharyngeal pouches, descends from thymus so parathyroid tissue can be anywhere from thyroid to thymus
chief cells: regulate free Ca via PTH secetion
PTH increases osteoblasts which increase osteoclast which reasorb bone and release Ca and P, increase SI absorption of Ca and P, increase renal Ca reabsorption and decrease P reabsorption in kidney |
|
|
Term
| digeorge syndrome sign and cause |
|
Definition
agenesis of parathyroid due to improper descent of pharyngeal arches
hypocalcemia |
|
|
Term
| causes of primary hyperparathyroid 3, which is most common |
|
Definition
parathyroid adenoma - most common
primary hyperplasia diffuse or nodular
parathyroid carcinoma |
|
|
Term
| causes of secondary hyperparathyroid 4, which is most common |
|
Definition
renal failure - most common
dietary Ca deficiency
stratorrhea
vitamin D deficiency |
|
|
Term
| causes of hypercalcemia, which is most common |
|
Definition
immobilization: promotes bone loss
thiazide diuretics
vitamin D toxicity
sarcoidosis and OTHER GRANULOMATOUS DISEASES which activate vitamin D and Ca
thyrotoxicosis: T3 STIMULATES OSTEOCLAST BONE REABSORPTION
MALIGNANCY - MOST COMMON |
|
|
Term
| 2 malignancies that cause hypercalcemia |
|
Definition
osteolytic metastasis: cancer erodes bone
paraneoplastic: SQUAMOUS cell carcinoma of the lung makes PTH |
|
|
Term
| causes of hypoparathyroid 5 |
|
Definition
surgery
congenital parathyroid absence
familial hypoparathyroid: autoimmune polyendocrine syndrome 1
idiopathic hypoparathyroid: autoimmune Ab against CASR
digeorge syndrome |
|
|
Term
| causes of pseudohypoparathyroid |
|
Definition
mutation in G-protein a-subunit GNAS1 stops PTH signal on organs
GNAS1 is selectivly imprinted on different tissues
pituitary and kidney paternal allele silenced so mutation in mom expressed
other tissues: no imprinting so 50% decrease in function when there is a mutation |
|
|
Term
| possible mutations causing familial primary hyperparathyroidism 3 |
|
Definition
MEN1 OR RET ACTVATING MUTATIONS
Ca sensing receptor (CASR) mutation
familial hypocaluric hyperclacemia: Ca receptors in PT cannot sense Ca so it release PTH, Ca receptors in kidne are deficient and percieve low Ca, PTH stimulates reabsorption of Ca in kidney (less Ca in urine) |
|
|
Term
| possible mutations causing sporatic primary hyperparathyroidism |
|
Definition
MEN1 OR RET ACTIVATING MUTATIONS
sporatic parathyroid adenoma (PRAD1): mutation in cyclin D1 |
|
|
Term
| what is more common hyper/hypo parathyroid benign/malignant |
|
Definition
hyperparathyroid
benign: cancer of thyroid is rare more likley adenoma |
|
|
Term
| causes of asymptomatic primary hyperparathroidism 2 |
|
Definition
sporatic parathryoid adenoma 1
familial hypocalcuric hypercalcemia |
|
|
Term
| how does morphology of primary hyperparathyroid distinguish between causes |
|
Definition
adenoma involves 1 gland
hyperplasia involves 4 glands (familial)
PT carcinoma cannot be distinguished from adenoma |
|
|
Term
| how do you diagnose parathyroid carcinoma |
|
Definition
| PT carcinoma cannot be distinguished from adenoma and metastasis or local invasion is only proof |
|
|
Term
| signs of primary hyperparathyroid 15 |
|
Definition
asymptomatic hypercalcemia
nephrolithiasis: Ca oxylate kidney stones
nephrocalcinosis: metastasic calficiation, polyuria
constipation
nausea
peptic ulcers
gall stones
acute pancreatitis: Ca activates enzymes
depression
lethargy
seizures
weakness of muscles
fatigue
heart valve calcifications
osteofibrosis cystica: fibrosis, cyst, hemorrhage in bone due to increased PTH |
|
|
Term
| pneumonic for primary hyperparathyroid |
|
Definition
painful bones
renal stones
abdominal groans
psychic moans |
|
|
Term
| lab signs of primary hyperparathyroid |
|
Definition
increased PTH, Ca
decreased P
increased urinary cAMP
increased alkaline phosphatase |
|
|
Term
| why is there increased urinary cAMP in primary hyperparathyroid |
|
Definition
| PTH binds tubular cells of kidney activating Gs then adenylylate cyclase which converts ATP to cAMP which mediates PTH |
|
|
Term
| why is there increased alkaline phosphatase in primary hyperparathyroid |
|
Definition
PTH does not directly activate osteoclast
osteoblast activated first and lays down bone making alkaline phosphatas ebefore osteoclast breaks down bone |
|
|
Term
| how does renal failure cause secondary hyperparathyroid |
|
Definition
decreased P excretion causes hyperphosphatemia
Ca and P can percipitate and are taken up into bone causing hypocalcemia
PT sees less Ca so it increaes PTH from ALL 5 GLANDS
decreased a1-hydroxylases for Vitamin D activation |
|
|
Term
| lab signs of secondary hyperparathyroid 4 |
|
Definition
increased PTH
Ca low
increased P
increased alkaline phosphatase |
|
|
Term
| signs of secondary hyperparathyroid |
|
Definition
renal osteodystrophy
calciphylaxis: ventricular calcifications cause ischemic damage to skin and organs |
|
|
Term
| cause of tertiary hyperparathyroid |
|
Definition
| secondary hyperparathyroid leads to autonomous excessive parathyroid production |
|
|
Term
| signs of hypoparathyroid 24, which is the hallmark |
|
Definition
tetany - hallmark
muscle apasm
numbness
tingling
chovsteck sign: tap cheek causes facial muscle spasm
trosseau sign: BP cuff induces carpal spasm
laryngospasm
seizure
emotional instability
anxiety
depression
confusion
psychosis
hallucinations
calcification of basal galgnia causes parkinson like movement
increased ICP
papilledema
cataracts
conduction defecit prolonged QT
dental abnormalities if occurs early in development
dental hypoplasia
failed eruption of teeth
defective enamel and root
abraded carious teeth |
|
|
Term
| labs of hypoparathyroid 2 |
|
Definition
|
|
Term
| cause of pseudohypoparathyroidism 1A |
|
Definition
mutation in maternal allele which is only one in pituitary and kidney decreases stimulation by anything (not just PTH) that uses cAMP
so TSH, GNRH, PTH |
|
|
Term
| cause of pseudohypoparathyroidism 1B |
|
Definition
| mutation is on paternal allele but maternal is fine, so no multihormone resistance |
|
|
Term
| signs of pseudohypoparathyroidism 1A (5) |
|
Definition
multihormone resistance: short stature, short 4th-5th digits, obesity, mental deficits
albright hereditary osteodystrophy: skeletal and developmental defects |
|
|
Term
| signs of pseudohypoparathyroidism 1B (1) |
|
Definition
| albright hereditary osteodystrophy: skeletal and developmental defects |
|
|
Term
| adrenal medulla: embryology, hormone release and breakdown |
|
Definition
neural crest cells > chromaffin cells > catecholamines
epinepherine > meanephrine + MAO > VMA > trash
NE > normetanepherine + MAO > VMA > trash |
|
|
Term
| how does the adrenal cortex make hormones and in what locations |
|
Definition
cholesterol > pregneolone...
... + 21-hydroxylase + 11-hydroxylase > ALD in glomerulus
... +17-hydroxylase + 21-hydroxylase > weak mineral corticoid > 11-hydroxylase > glucacorticoids (cortisol in fasiculata)
... + 17-hydroxylase > sex streoids in reticularis |
|
|
Term
|
Definition
principal cells od CD: retain Na, excrete K
a-intercalated cells of CD: excrete H |
|
|
Term
| 3 adrenocortico hyperfunction syndromes |
|
Definition
hypercortisolism
hyperaldosteronism
androgenital syndromes |
|
|
Term
| causes of hypercortisolism, which is most common |
|
Definition
cushing disease - most common endogenous
ACTH independent cushing syndrome (adrenal cushing syndrome)
iratogenic cushing syndrome - most common
paraneoplastic cushing syndrome |
|
|
Term
| causes of primary hyperaldoseronism 4 |
|
Definition
adrenocortical neoplasm (conn syndrome, adrenocortical carcinoma)
primary adrenocorticol hyperplasia (idopathic hyperaldosteronism)
glucocorticoid-remediable hyperaldoseronism |
|
|
Term
| causes of secondary hyperaldosteronism |
|
Definition
decreased renal perfusion
arterial hypobolemia and edema: CHF, cirrhosis, nephrotic syndrome
pregnancy: estrogen induced increase in plasma renin |
|
|
Term
| 4 causes and how does decreased renal perfusion cause secondary hyperaldoseronism |
|
Definition
renal artery stenosis, arteriolar nephrosclerosis, fibromuscular dysplasia of renal artery, athlerosclerosis of renal artery (eso old men)
decreases flow and increases renin |
|
|
Term
| 2 adrenogenital syndromes |
|
Definition
adrenocortical neoplasm associated with virilization
congenital adrenal hyperplasia |
|
|
Term
| 4 adrenocorticol hypofynction diseases |
|
Definition
adrenal crisis: primary acute adrenocortical insufficiency
addisons: primary chronic adrenocortical insufficiency
secondary adrenal insufficiency |
|
|
Term
| causes of adrenal crisis 3 |
|
Definition
rapid withdrawl of steroids
stress in pt with chrnic adrenocortical insufficiency
massive adrenal hemorrhage |
|
|
Term
| causes of addisons disease 10 |
|
Definition
90% autoimmune adrenalitis, TB, AIDS, metastic lung cancer
10% lymphoma, amyloidosis, sarcoidosis, hemochromatosis, fungal, adrenal hemorrhage |
|
|
Term
| cause of secondary adrenal insufficiency 2, signs 3 |
|
Definition
any disorder that reduces ACTH (hypopituitary, prolonged steroids)
simillar to addisons
NO hyperpigmentation
normal ALD synthesis |
|
|
Term
|
Definition
adrenocortical adenoma
adrenocortical carcinoma |
|
|
Term
| 3 diseases of the adrenal medulla |
|
Definition
pheochromocytoma
paraganglioma
neuroblastoma |
|
|
Term
| 2 causes of cushings disease, which is most common |
|
Definition
ACTH producing pituitary microadenoma - most common
corticotroph cell hyperplasia due to hypothalamic tumor releasing CRH |
|
|
Term
|
Definition
|
|
Term
| morphology of cushing disease 3 |
|
Definition
adrenal glands hyperplastic due to INCREASED ACTH STIMULATION
ONE GLAND LARGE, ONE ATROPHIC |
|
|
Term
| cause of ACTH independent cushing syndrome 3 |
|
Definition
adrenal adenoma and carcinoma in adults
adrenal carcinoma in kids |
|
|
Term
| pathogenesis of ACTH independent cushing syndrome |
|
Definition
| autonomous production of cortisol by adrenal glands increases cortisol despite DECREASED ACTH |
|
|
Term
| morphology of ACTH independent cushing syndrome 2 |
|
Definition
adrenal cortex OPPOSITE GLAND ATROPHY DUE TO DECREASED ACH
if adenoma gland atrophies due to decreased stimulation |
|
|
Term
| cause of iratogenic cushing syndrome |
|
Definition
| administration of exogenous steroids causes BOTH GLANDS TO ATROPHY due to supression of ACTH by therapy |
|
|
Term
| causes of paraneoplastic cushing syndrome 4, which is most common |
|
Definition
small CC of lung - most common
carcinoid tumor
medullary thyroid carcinoma
pancreatic islet cell tumor |
|
|
Term
| 6 ways cushings syndrome changes physiology and the symptoms each of these ways causes, 6 other symptoms |
|
Definition
HTN: cortisol upregulates a1 on arterioles increasing effects of NE
high glucose releases insulin which results in fat storage (truncal obesity, moon facies, buffalo hump, weight gain (central adipose)
cortisol protein catabolism stops synthesis of collagen making vessels weak and rupture (striae on abdomen, osteoperosis, easy bruising)
cortisol raises blood sugar by breaking down muscles for gluconeogenesis (muscle weakness, thin extremities, hyperlgycemia, glucosuria, polydypsia, secondary DM, atrophy of fast twitch myofibrils, decreased muscle mass)
cortisol inhibits phospholipase A2 so cannot make arachadonic acid for inflammation, inhibits IL-2 so cannot make T cells, inhibits histamine from mast cells so no vasodilation or permeability (immunodeficiency)
mental disturbances, mood swings, depression, psychosis
histurism, menstural abnormalities |
|
|
Term
| labs of cushings syndrome 2 |
|
Definition
increased urine 24h cortisol showing loss of diumal cortisol secretion (cortisol increases in morning and decreases at night so blood cortisol is not as consistant as urine cortisol)
dexamethasone supression test helps determine cause |
|
|
Term
| explain how dexamethasone supression test works |
|
Definition
pituitary adenoma increases ACTH
adrenal adenoma decreases ACTH
ectopic PNP increases ACTH
if ACTH is decreased it is adrenal adenoma
if cortisol decreases with DEX it is pituitary adenoma
if cortisol does not decrease with DEX it is small cell lung cancer
low DEX is designed to decrease normal cortisol so if they have disease it shouldnt return them to normal levels
high DEX will decrease cortisol in pituitary adenoma |
|
|
Term
| pneumonic for cushing syndrome |
|
Definition
cushing
4C 2U 2S 4H 1I 1N 2G
central obesity
cervical fat pad
collagen fiber weakness
comedones - acne
urinary free cortisol and glucose
striae
supressed immunity
hypercorticolism
hypertension
hyperglycemia
hirsturism
iratogenic - increased steroid pills
noniratogenic - neoplasms
glucose intolerance
growth retardation |
|
|
Term
| labs in primary hyperALD 3 |
|
Definition
increased Na, blood volume
decreased renin |
|
|
Term
| labs in secondary hyperALD 3 |
|
Definition
increased renin
increased Na and blood volume |
|
|
Term
| cause of glucocorticoid remediable hyperALD and dx |
|
Definition
fusion of 11-B-hydroxylase and ALD synthase causes chimeric gene that continues producing chimeric proteins that increase cortisol and ALD
supressed by exogenous dexamethasone because ACTH can supress ALD
chimeric gene is under infence of 11B-hydroxylase so steroids work |
|
|
Term
| signs of primary adernocortical hyperplasia 2 |
|
Definition
idiopathic bilateral nodular hyperplasia
most common in kids and young adults |
|
|
Term
| how can you diagnost adrenocortical neoplasms |
|
Definition
conn syndrome: adrenocortical adenome that makes ALD in middle age women
does not supress ACTH secretion like cushings, no atrophy of normal glands |
|
|
Term
|
Definition
hypernatremia
hypokalemia
metabolic acidosis
HTN
EKG changes
cardiac decomposition
weakness
parasthesias
visual disturbances
tatany |
|
|
Term
| define adrenogenital syndrome |
|
Definition
| sex steroid release by adrenal cortex caused by autonomous production by cells in zona reticularis (neoplasms, congenital adrenal hyperplasia0 or as component of cushings disease (stimulation by increased ACTH) |
|
|
Term
| who gets congenital adrenal hyperplasia 2 |
|
Definition
hispanics
aschkenazi jews |
|
|
Term
| cause of congenital adrenal hyperplasia 3 |
|
Definition
90% 21-hydroxylase (CYP21B) recombination with neighboring non-functional pseudogene causing chymeric gene (deg non functioning pseudogene determines severity)
11-hydroxylase deficiency
17-hydroxylase deficiency |
|
|
Term
| morphology of congenital adrenal hyperplasia 2 |
|
Definition
adrenals hyperplastic bilateral
anerior pituitary corticotroph cell hyperplasia |
|
|
Term
| explain the pathogenesis and 3 forms of 21-hydroxylase deficiency, which form is most common |
|
Definition
unable to make ALD and cortisol so increases production of sex steroids
severity is based on amount CYP21B replaced with pseudogene
salt wasting (Classic) androgenitalism: most severe
simple virilizing: less salt wasting
non-classic: most common, few symptoms |
|
|
Term
| signs of 21-hydroxylase deficiency 7 |
|
Definition
decreased cortisol and ALD...
soon after birth: salt wasting, hyponatremia, hyperkalemia
later: hypotension, CV collapse, possible death
virilization: clitoral enlargement in females (boys cant tell until salt wasting)
precocious puberty in males
decreased catecholamines causes hypotenion and circilatory collapse |
|
|
Term
| explain pathogenesis of 11-hydroxylase deficiency |
|
Definition
cant make ALD and strong glucocorticoids so increased production of sex streoids
enough ALD for salt reabsorption but lowered cortisol fails to inhibit ACTH so precursors are shunted to androgen pathway |
|
|
Term
| signs of 11-hydroxylase deficiency 2 |
|
Definition
virilization
precocious puberty |
|
|
Term
| explain pathogenesis of 17-hydroxylase deficiency |
|
Definition
unable to make glucocorticoids and sex steroids
increased production of ALD |
|
|
Term
| signs of 17-hydroxylase deficiency 4 |
|
Definition
asymptomatic
histurism
oligomenorrhea
delayed menarche |
|
|
Term
| causes of massive adrenal hemorrhage that leads to hypofunction 4 |
|
Definition
newborn delivery trauma and hypoxia
anticoagulant therapy
DIC with adrenal infarct
waterhouse friderichsen syndrome: bacterial infection and adrenal hemorrhage |
|
|
Term
| cause 4 and pathogenesis of waterhouse fridrichen syndrome |
|
Definition
child with N. meningitis, pseudomonas, H. influenza, staph
infection causes DIC, hypotension, sock, purpura which causes bilateral necrosis of adrenal glands
acute decrease in cortisol exacerbates hypotension due to DIC/shock
adrenal glands look like sac of blood |
|
|
Term
| signs 5 and prognosis of adrenal crisis |
|
Definition
intractable vomiting
abdominal pain
hypotension
coma
vascular collapse
die in days wo tx |
|
|
Term
| cause of autoimmune adrenalitis 3 |
|
Definition
auto-ab destruction of 21-hydroxylase
autoimmune polyendocrine syndrome 1: mutation in autoimmune regulator AIRE gene (causes death of self reactive T cells in thymus)
autoimmune polyendocrine syndrome 2: not single gene disorder, isolated immune addisons disease |
|
|
Term
| signs of autoimmune polyendocrine syndrome 1 (6) |
|
Definition
chronic mucocutaneous candidiasis
ectodermal dystrophy: abnormalities of skin, dental enamel, nails
adrenalitis
hypoPTH
idiopathic hypogonadism
pernicious anemia |
|
|
Term
| signs of autoimmune polyendocrine syndrome 2 (4) |
|
Definition
adrenal insufficiency
autoimmune thyroiditis
DM1
early adult presentation |
|
|
Term
| TB adrenalitis: epidemiology, cause |
|
Definition
once 90% of addisons disease, less common now
infection at other sits (lung, GU) |
|
|
Term
| metastic neoplasm to adrenal gland primary locations 5, which is most common |
|
Definition
lung - most
breast - most
GI
melanoma
hematopoietic |
|
|
Term
| genetic disorder of adrenal insufficiency |
|
Definition
|
|
Term
|
Definition
insidous 90% of gland must be destoried
progressive weakness
easy fragility
anorexia
N/V
weight loss
diarrhea
hyperpigmentation (esp oral mucosa)
hyperkalemia
hyponatremia
volume depletion
hypotension
hypoglycemia |
|
|
Term
| why is there hyperpigmentation in addisons |
|
Definition
| no cortisol means increased ACTH which comes from POMC which breaks into MSH |
|
|
Term
|
Definition
autoimmune
DIC - meningococcus
Destruction by cancer, infection, vascular insfficiency
iratogenic
sarcoidosis, granulomatoous like TB, histomycosis
hypOtension/hypOnatremia
nelson syndrome: post adrenalectomy, increased ACTH |
|
|
Term
| signs of adrenocortical adnoma 2 |
|
Definition
USUALLY SILENT found on autopsy
if functional may atrophy neighboring cortex and opposite gland due to supression of ACTH by tumor products |
|
|
Term
| adrenocortical carcinoma: signs, 2 assciated diseases, prognosis |
|
Definition
if virilizing (causes masculinization) more likley carcinoma
li-fraumeni, beckwith-weidmann
strong risk to invade adrenal vein, vena cava, lymphatics |
|
|
Term
| pheochromocytoma pathogenesis |
|
Definition
| neoplasm of chromaffin cells that makes catecholamines, hormones, or seroids (PNP cushings or endocrinopathies) |
|
|
Term
| pheochromocytoma rule of 10s (5) |
|
Definition
10% familial
10% extra-adrenal
10% nonfamilial are bilateral
10% in kids (familial, male)
10% malignant |
|
|
Term
| familial mutations of pheochromocytomas 5 |
|
Definition
MEN2A
MEN2B
von recklinghausen (NF1)
von hippel-lindau: AD loss VHL tumor supressor
sturge-weber syndrome |
|
|
Term
| 3 tumors of von-hippel-lindau |
|
Definition
pheochromoytoma
hemangioblastoma of cerebellum
renal cell carcinoma |
|
|
Term
| most common age of pheochromocytome |
|
Definition
|
|
Term
| common place of pheochromocytoma malignancy and sign |
|
Definition
bladder wall (episodic HTN on urination)
more chance of metastis when extra-adrenal |
|
|
Term
| morphology pheochromocytoma 3 |
|
Definition
small 1g to large 4kg
brown with yellow edge (cortex cholesterol)
lesion and L dichromatic dark brown (oxidation of catecholamines) |
|
|
Term
| how do we determine if pheochromocytoma is malignant |
|
Definition
| we dont we see if it metastasis |
|
|
Term
| signs of pheochromoctyoma |
|
Definition
acute HTN <50%
chronic HTN 2/3
tachycardia
plapitations
headache
sweating
tremor
apprehension
abdominal pain
chest pain
nausea
vomiting |
|
|
Term
| what are pheochromocytoma symptoms percipitated by 4 and why, what symptoms does this cause 6 |
|
Definition
emotions, exercise, postural change, palpation of tumor
causes release of catecholamines causing catecholamine cardiomyopathy, myocardial instability, ventricle arrhythrmia, CHF, MI, CVA |
|
|
Term
|
Definition
| increased urinary catecholamines and metabolites (VMA, metanepherines) |
|
|
Term
| treatment pheochromocytoma |
|
Definition
| surgical excision AFTER phenobenzomine (irreversible a blocker so when they touch it catecholemines dont releease and kill pt due to massive HTN) |
|
|
Term
|
Definition
| extra renal pheochromocytoma |
|
|
Term
| what is the most common extracranial solid tumor of childhood (first 5y) |
|
Definition
|
|
Term
| pneumonic pheochromocytoma |
|
Definition
PHE
palpitations
headache
episodic sweating (diaphoresis) |
|
|
Term
|
Definition
occur at young age
arise in multiple endocrine organs (sometimes synchronized)
often multifocal tumors in single organ
tumors usually preceded by hyperplasia stage
tumors more agressive and recur |
|
|
Term
| men 1 mutation aka, pathogenesis |
|
Definition
wener syndrome
germline mutation of menin tumor supressor that regulates cell cycle and transcription |
|
|
Term
| abnormalities in men 1 mutation |
|
Definition
primary hyperparathyroid: 40-50yo, precered by hyperplasia
pancreas: agressive, metastasis, leading mortality, seen with hypersecretion
prolactinoma
enodenomal gastrinoma - frequent |
|
|
Term
| hypersecretion syndromes associated with men 1 (2) |
|
Definition
zollinger ellison: gastrinoma, peptic ulcers
insulinoma: hypoglycemia, nueurologic signs |
|
|
Term
|
Definition
| activating mutation iin RET protoncogene receptor TK transmits growth and differentiation signals |
|
|
Term
| men 2A mutation aka, 4 parts |
|
Definition
sipple syndrome
medullary thyroid carcinoma 100%!: multifocal agressive PROPHYLACTIC THYROIDECTOMY
familial medillary thyroid cancer: varient with medulla carcinma risk
pehochromocytoma: bilateral, common extranodal
PARATHYROID HYPERPLASIA: hypercalcemia, nephrolithiasis |
|
|
Term
|
Definition
medullary thyroid carcinoma: more agressive than 2a
pheochromocytoma
neuroma: skin, oral mucosa, eye, respiratory tract, GI tract
marfanoid habitus: long axial skeleton, hyperextensible joints |
|
|
Term
|
Definition
men 1: pituitary parathyroid pancreas
men 2: carcinoma of thyroid, catecholamines (pheochromocytoma) |
|
|
