2. Pathology II Flashcards
1
Q
Hyperadrenocorticism (Cushing syndrome) • Chronic exposure to high levels \_\_\_\_ • ACTH-dependent or ACTH-independent • Most cases \_\_\_\_ • Other etiologies – \_\_\_\_ tumor (Cushing disease) – \_\_\_\_-secreting tumors – \_\_\_\_ disease
• Cushing disease causes syndrome, and causes pigmentation; cushing syndrome doesn't always cause \_\_\_\_ • Can get cushing's without altering adrenal gland > giving patient chronic prednisone ○ Iatrogenic is the most common cause • Can get ACTH-dependent or independent ○ Dependent § \_\_\_\_ gland produces ACTH > adrenals at high levels > high steroid ○ Independent § Not pit causing the problem, but something within the \_\_\_\_ gland > a tumor (functional) in the cortex § Iatrogenic • Face becomes big and round ○ Become more \_\_\_\_ • Gaining weight • \_\_\_\_
A
glucocorticoids iatrogenic pituitary steroid genetic
pigmentation
pit
adrenal
edematous
osteoporosis
2
Q
Clinical manifestations
- ____ gain
- Easy bruising
- Moon ____
- Abdominal striae
- ____ weakness
- Fatigue
- ____
- Acne and other infections
- ____ disorders
- Hypertension
- ____
- Irregular menstruation
- ____
- Back pain
- ____ hump
- Erectile dysfunction
- Mucocutaneous ____
A
weight
facies
muscle
osteoporosis
mood diabetes hirsutism buffalo pigmentation
3
Q
• Pigmentation a conseq of cushing ____ not ____
○ Syndrome may be ACTH-indepdnent
§ Pigmentation only occurs in presence of ____ overexpression (and ACTH therefore)
A
DISEASE
SYNDROME
MSH
4
Q
- Treat underlying cause
- Reduce corticosteroid intake
- Block cortisol synthesis
- ____ (Nizoral®)
- Mitotane (Lysodren®)
- ____ (Metapirone®)
- Control cortisol tissue effects
- ____ (Korlym®)
- Bilateral adrenalectomy
- Inoperable pituitary tumor
- Refractory cases• Cushing disease
○ Treat the brain
○ Remove the pathology of the pit
• Cushing syndrome
○ Treat the cause
§ Too much steroids - stop the medication, carefully!
□ If giving patient prednisone for a few weeks > the patients adrenals will shut down entirely > body senses steroids in the system and systemically, the adrenals shut down, when taken off > take them off ____ > need adrenals to readapt decreased steroids in the circulation
® These patients still retain FFF response - and in a stressful situation in a dental office > if taking prednisone and you stop it > may experience an adrenal crisis > body is trying to respond to FFF, but it can’t do it > body crashes
® Pills from 40, 20, 10, 5mg > tapers you down to make sure you don’t experience an adrenal crisis
○ Reduce steroid production from adrenals, or reduce effect of steroids on the downstream tissues > ____ > prevents cortisol (steroid) synthesis from the adrenal gland
§ Used for ____ infections
□ Including patients w ____ disease
® High risk of developing adrenal insufficiency bc of the drugs they’re taking (in addition to protease inhibitors)
○ Drugs that control cortisol on downstream tissues
§ Mifepristone
○ Surgical excision if medication fails > supplement w steroids to maintain normal levels
A
ketoconazole
metyrapone
mifeprestone
slowly
ketoconazole
fungal
HIV
5
Q
Adrenocortical insufficiency (Addison disease)
• Primary
– Destruction or dysfunction of ____
– Only form that induces ____
• Secondary
– Reduced pituitary ____ secretion
• Tertiary
– Reduced ____ secretion
• Important cause of mucocutaneous pigmentation, but not all causes of addisons trigger pigmentation ○ Only addisons caused by \_\_\_\_ pathology to the adrenals cause pigmentation § That's what triggers ACTH to be released from the brain • Can get secondary addisons, or tertiary addisons ○ Secondary addisons > reduced pituitary ACTH secretion § Hypopit > decreased ACTH > not enough steroids produced, and the adrenals are hypo-active § Won't cause \_\_\_\_, bc ACTH is reduced ○ Tertiary § If hypo reduces CRH secretion > less ACTH > less activity from the glands § No \_\_\_\_
A
adrenal cortex
pigmentation
ACTH
hypothalamus CRH
primary
pigmentation
pigmentation
6
Q
Primary adrenocortical insufficiency
• Most often due to autoimmune destruction
– ____
– Anti-21-hydroxylase enzyme
• Infection
– ____
– Fungal
– ____
A
anti-adrenal antibodies
mycobacterial
AIDS
7
Q
Primary adrenocortical insufficiency
• Cancer
• Bilateral adrenal hemorrhage
– Adults – ____
– Children – ____
• Taking warfarin or cumidine > excessive bleeding from the glands ○ In adults • In children > WFS
A
anticoagulant therapy
waterhouse-friderichsen syndrome
8
Q
Adrenocortical insufficiency
ACTH accumulates within blood (____ disease only) Systemic complications
Diffuse mucocutaneous ____
Treat by ____ therapy
A
primary
pigmentation
replacement
9
Q
Cushing disease vs Addison disease
Dexamethasone suppression test
Measures suppression of ACTH secretion from pituitary
1. Measure ____ level in morning
2. 1 mg of ____ at 11 p.m.
3. Measure cortisol following morning
If ACTH not suppressed, then cortisol levels remain ____
ACTH stimulation test 1. Measure cortisol level in morning 2. \_\_\_\_ injection 3. Blood drawn after 60 minutes 4. Measure cortisol Increase in cortisol after stimulation by ACTH then adrenals are \_\_\_\_
• Addison's can be dx via ACTH stim test ○ Makes use of synthetic analog of ACTH ○ First in morning > blood drawn > measure baseline cortisol ○ After blood draw > injected IV w ACTH analog (cosyntropin) > one hour later > blood drawn again > cortisol measured a second time § If adrenals are proper fxn > analog stim should inc cortisol § If doesn't stim cortisol after an hour > glands not fucntioning properly > dx addison's • Cushing disease is tested w the dexamethasone suppression ○ Patient in morning gets blood drawn and cortisol measured ○ Befroe bed > take oral dosage of steroid (dexamethasone) ○ Next morning in lab > blood draw > measure steroid levels § Normal circumstances > big dose of steroid > suppress ACTH and suppress further steroid release from the adrenals § If doesn’t > ACTH const expressed from the pit > indicative of cushing's disease • Usually dex first, ACTH stim second
A
cortisol
dexamethasone
high
cosyntropin
normal
10
Q
Hyperaldosteronism
• Primary (Conn syndrome)
– Usually due to functional ____
• Secondary
– Due to excessive ____ secretion
• Conn ○ Tumor in adrenal that's producing aldosterone § Goes to kidney > complications that arise from aldosterone activity □ Patient will have \_\_\_\_, \_\_\_\_ and \_\_\_\_ (alkalosis) • Secondary ○ Adrenals function properly, except the \_\_\_\_ is not fucntioning properly in the kidney ○ Kidmey insenstive to aldosterone > renin secretion from the kidney > goes to adrenal gland to produce ald > does what it does on the kidney ○ Same result
A
adenoma
renin
HTN
hypokalemia
alkalotic
aldosterone
11
Q
Thyroid gland • \_\_\_\_ neck • Originates from base of \_\_\_\_ • Composed of thyroid follicles – Contain \_\_\_\_ • Hormones and Iodine
• On the backside of the thyroid are the parathyroids • Physically attached at the level of foramen cecum, and then the stalk of tissue is dissolved throughout development ○ IF not > \_\_\_\_ duct cyst • Follicles are lined by \_\_\_\_ epi cells ○ The pink stuff is \_\_\_\_ material § Fluid § Composed of hormones and iodine □ Hormone = \_\_\_\_ (makes up the colloid) • Bt the follicles > parafollicular cells ○ \_\_\_\_ § Directly and completely counteracts action of PTH □ Work on bone and kidney (same structures) • T3 and T4 ○ Produced from thyroglobulin ○ \_\_\_\_ cells (cuboidal) upon stim > convert thyroglobulin to T3 and T4 § Once produced > enter circulation
A
anterior
tongue
colloid
thyroglossal
cuboidal
colloid
thyroglobulin
calcitonin
follicular
12
Q
Thyroid gland • Follicular cells produce – \_\_\_\_ – \_\_\_\_ – \_\_\_\_
• Parafollicular cells produce calcitonin
– Regulates ____ metabolism
– Antagonist to ____
A
thyroglobulin
triiodothyronine (T3)
thyroxine (T4)
calcium
parathyroid hormone
13
Q
Hypothalamus-pituitary-thyroid axis
Hypothalamus > ____
Pituitary > \_\_\_\_ Thyroid > TSH binds to TSH-receptor (TSH-R) > Converts thyroglobulin to \_\_\_\_ - T3 / T4 bound to \_\_\_\_, transthyretin, thyroxine-binding globulin
• Three organs work synergistically, part of the same axis ○ Begins with hypo > produces TRH > acts on the pit to stim TSH > acts on thyroid follicles to trigger conversion thyroglobulin to T3 and T4 ○ TSH binds to TSH receptor on follicular cells > once binds > cascade of events § Most hormones act through G protein receptor > cascade that's mediated via \_\_\_\_ (and \_\_\_\_) (propagates signal into cell)
A
TRH
TSH
T3 and T4
albumin
cAMP
adenylate cyclase
14
Q
• Organs affected by T3/T4 ○ \_\_\_\_ development ○ Brain ○ \_\_\_\_ ○ Skin ○ \_\_\_\_ rate
A
eye
kidney
heart
15
Q
Thyroid diseases • Hyperthyroidism • Hypothyroidism • Developmental • Goiter • Neoplasia
• When hormone levels are out of whack > things go awry • Goiter ○ Enlarged \_\_\_\_ gland ○ \_\_\_\_ of the thyroid manifesting as a diffuse mass of the thyroid • Hypothyroidism ○ Slowing of \_\_\_\_ • Hyperthyroidism ○ Accel of \_\_\_\_ ○ Nervous, agitated
A
thyroid
hyperplasia
metabolism
metabolism
16
Q
Hyperthyroidism • Graves disease due to \_\_\_\_ autoantibodies • Pituitary tumor secreting \_\_\_\_ • Excessive TRH from \_\_\_\_ • Serum thyroid hormones elevated • Plasma TSH elevated or suppressed
• Hyperactivity = hyperthyroidism ○ Most common development > graves disease § \_\_\_\_ disease □ \_\_\_\_ mechanism □ Autoab to TSH receptor on follicular cells > once ab binds to the receptor > overactivates > const activated > whole cascade happens ove r and over again ® Thyroglobulin cont converted to T3 and T4 ○ Tumor § Producing TSH > cont stimulating receptor and producing T3 and T4 ○ Pathology of hypothalamus § Excessive secretion of TRH > pit > high levels of TSH • Treatment pit patholgy diff from graves disease, etc. ○ For a pit tumor > treat w surgery ○ For graves dx > require \_\_\_\_ therapy, or surgery of the thyroid • T3 and T4 are elevated, TSH may be elevated or not ○ Bc of negative feedback mechanism ○ Specifically in context of Grave's dx
A
TSH-R
TSH
hypothalamus
autoimmune
type II
medicinal
17
Q
Graves disease
• Most common cause of hyperthyroidism – \_\_\_\_ with genetic component • \_\_\_\_>>M • \_\_\_\_ thyroid enlargement • Autoantibodies to TSH-R – Thyroid stimulating immunoglobulin – TSH levels \_\_\_\_ • untreated hyperthyroidism may lead to \_\_\_\_
A
multifactorial F symmetric reduced thyroid storm
18
Q
Graves disease
• Commonly manifesting in older females • Have a \_\_\_\_ (thyroid enlargement) • Mechanistically ○ Developing autoab to the TSH receptor > binding receptor > triggering activation of receptor constantly > conversion of thyroglobulin to T3 and T4 ○ TSH reduced § Activated > brain thinks TSH isn't needed anymore > hypo is sensing, and asking why producing more? □ Result is that the brain shuts down TSH secretion and production > bc the \_\_\_\_ mistakenly thinks it has enough already • [???] • Patients w hyperthyroidism > \_\_\_\_ metabolism, eat a lot (not bc overly hungry but bc they're using all the energy theyr'e getting), inc appetite, palpating very quickly, jittery • Thyroid storm ○ Life threatning complication > in response to underlying \_\_\_\_ state that releases TSH under normal circumstances > may result in more activation of the thyroid beyond what they're currently experiencing § More exacerbated phenotypes § May die of a \_\_\_\_ • Follicles are mishapen, the colloid is less pink (not uniform) ○ Spcaes bt the colloid and the actual cells ○ \_\_\_\_ has been converted to T3 and T4 (less of it in the follicle)
A
goiter brain rapid stressful heart attack colloid
19
Q
Clinical manifestations
• Array of complications for Graves ○ Excessive \_\_\_\_ ○ Hair loss and thinner hair ○ \_\_\_\_ start flaking off ○ Moist skin ○ \_\_\_\_ damage (cannot keep up w metabolic demand) ○ Weakness of bone (osteoclasts are helping to resorb bone - bone's not keeping up w the demand) ○ \_\_\_\_ (irregular beat, rapid pulse) ○ Protrusion of eyeballs (not patho) ○ \_\_\_\_ loss (even though eating a lot, losing a lot of weight)
A
hunger nails muscle heart weight
20
Q
Hypothyroidism
- ____
- Hashimoto thyroiditis
- Congenital (____)
- Severe iodine deficiency• Exact opposite in terms of manifestations
○ Not as hungry
○ Hair is not healthy looking
○ Skin is dry
○ ____ are unaffected
○ Weight gain
○ HR slows down, bc the metabolism has decreased
• Most common cause - ____
○ Autoimmune
• Severe hypothyroidism > ____ (also used to describe congential hypothyroidism)
• Table salt is supplemented w iodine > prveents hypothyroidism
• Thyroid can be dysfunctional bc of:
○ At the level of thyroid
○ Hypopit can cause hypothyroidism
○ Hypothalamus shuts down > CRH drops > thyroid hormone levels drop
○ Like w diabetes, or adrenals > downstream organs insensitive of T3/T4 > hypothyroidism
§ Born/develop mutation in T3 T4 receptor > defect found in cell that require hormone > tissues are less sensitive to the hormone
A
surgery myxedema eyeballs hashimoto thyroiditis myxedema
21
Q
Hypothyroidism
• Skin tone is \_\_\_\_ • Mental state and self esteem goes down ○ Strong \_\_\_\_ component
A
duller
psychological
22
Q
Hashimoto thyroiditis
• Most common cause of \_\_\_\_ • \_\_\_\_ destruction of thyroid follicles with lymphocytic infiltrates – May occur after \_\_\_\_ • Gland \_\_\_\_ • Treat with \_\_\_\_
• Autoimmune, unlike graves > the follicles look normal, what's not normal > lymphoid aggregates within the glandular tissue ○ Blue things on the slide § \_\_\_\_ § Helps destroy the glandular tissue • Formation of autoab's that aggravate the state • Over time > the gland shrinks • Not producing TRH, or TSH from the pit > if your thyroid is not responsive to TSH or CRH > then the gland will also shrink • Treatment is not easy, may require surgery or medications that will reduce the formation of antibodies in GRAVE's ○ In hypothyroidism > prescribe levothyroxine (synthyroid) > replaces \_\_\_\_
A
hypothyroidism autoimmune hyperthyroidism shrinks levothyroxine
t cells and b cells
T3 and T4
23
Q
Hashimoto thyroiditis • Autoantibodies – \_\_\_\_ – \_\_\_\_ blocking – \_\_\_\_
- TSH elevated
- ____ increases susceptibility• Abs to thyroglobulin
○ Destory and prveneting conversion to T3 and T4
• Can block activation of receptor > TSH-R blocking
• May have ab to thyroidal peroxidase > conevrsion of thyroglobuin to T3 and T4
• TSH is elevated > bc not producing T3 and T4 > result is that the pit senses that more TSH should be secreted > pit producing more TSH in repsonse
• Not a ____ disease, but a link > runs in families (not 1:1) > inc risk of developing hashimoto
A
thyroglobulin
TSH-R
thyroidal peroxidase
HLA-DR5
genetic
24
Q
Clinical manifestations
• Hypothyroidism ○ Skin, hair and eyes changed before and after • Patients have \_\_\_\_ metabolism > to the point that if you offer them certain drugs > drugs may not \_\_\_\_ properly ○ One particular instance > prescribing opioids to these patients > opioids reduce metabolism on their own to their background condition > double-whammy > could lead to \_\_\_\_ or death § Not ever prescribed an opioid!
A
slower
metabolized
coma
25
Q
Congenital hypothyroidism (Myxedema, Cretinism) • 1 in 4000 births • \_\_\_\_:M, 2:1 • \_\_\_\_ / hypoplasia of thyroid • Defective \_\_\_\_ biosynthesis • Mental \_\_\_\_
• Severe hypothyroidism > myxedema or congenital hypothyroidism > born being hypothryoid > developmental of the thyroid gland where patients don't have enough thyroid being formed • More commonly seen in girls (not boys) • If thyroid developent is perturbed > hypoplasia or aplasia > most important reasons for being hypothyroid at birth • Gland may be \_\_\_\_, but mutation in gene or protein > inhibits proper hormone biosynthesis • Earlier the onset > more likely the kids will be born severly \_\_\_\_ delated • Severe adult case > myxedema > severe hypothyroidism ○ After treatment > feet and face returned to \_\_\_\_ • Coma is a possibility ○ Add opioid on top of hypothyroid > develop death
A
F
aplasia
hormone
retardation
normal
developmentally
normal
26
Q
Lingual thyroid • Developmental failure of thyroid to descend • \_\_\_\_ >> M • Most patients are \_\_\_\_ • Treat with \_\_\_\_
• Thyroid develpos from \_\_\_\_ > migration never happens in some patients > develop w a thyroid in the post region of mouth in base of tongue area > they never descended • Bc of where they are anatomically > invariably these patients will become \_\_\_\_ > treated w levothyroxine • Rare cases > compeltely \_\_\_\_ > euthyroid > normal > never will be hypothyroid unless develops another dx that exarcesbates • Don’t treat > functional thyroid tissue, but not enough functional thyroid ○ Does not warrant a \_\_\_\_ ○ The mass is very vascular, and bc of anatomical location > indicative of what it is clinically
A
F
hypothyroid
levothyroxine
foramen cecum
hypothyroid
normal
biopsy
27
Q
Goiter
• Diffuse thyroid enlargement due to prolonged stimulation by ____
• Multiple causes – \_\_\_\_ deficiency or excess – Hashimoto thyroiditis – \_\_\_\_ disease – Pituitary adenoma – Dietary \_\_\_\_ – Goitrogenic medications
• Can be in hyperthyroidism or hypothyroidism • Hyperplasia of the thyroid gland via stim via TSH ○ Hyperactivity and enlarged gland compensating for the hyperactivity • This is why table salt is iodinated • Grapefruit can result in goiter
A
TSH
iodine
graves
goitrogens
28
Q
Thyroid neoplasia
• Follicular adenoma
– Most ____ tumor
• Papillary carcinoma
– Most common ____
– Usually history of ____ exposure
• Medullary carcinoma
– Cancer of ____ cells
– Produces ____
A
common malignancy radiation parafollicular C amyloid
29
Q
Thyroid neoplasia
• Follicular adenoma ○ Follicle cells are neoplastic > mass in thyroid ○ Look at the neck of patient and the enlargement is \_\_\_\_ > NOT A TUMOR, mor likely to be a goiter § If asymmetric > most likely to be a \_\_\_\_ in the gland and not goiter ○ Most common cause of unilateral enlargement > \_\_\_\_ > may be FUNCTIONAL (can develop hyperthyroidism) • Most common cancer of thyroid > papillary carcinoma ○ Radiation exposure is a common factor ○ After meltdown in \_\_\_\_ > epidemiologic study > following kids over time to assess risk of papillary carcninoma as a result of the H bombs ○ Radiologists have a higher chance of developing as well • Medullary carcinoma ○ Produce calcitionin > bones bc denser § Preserves \_\_\_\_ in the bone ○ Produce amyloid § Commonly found in multiple myeloma § Looks \_\_\_\_ under the microscope > quite aggressive > affiliated w a genetic disease • \_\_\_\_ is the primary mode of treatment ○ If benign > excise the portion of the gland w the tumor ○ \_\_\_\_ next, and chemo if necessary
A
symmetric
tumor
follicular adenoma
Japan
calcium
pink
surgery
radiation
30
Q
Parathyroid gland
____ on each side
Located on ____
____ cells synthesize parathyroid hormone
– Regulates calcium and phosphate in blood
* There's four total * Regulates calcium by acting on the bone (any bone), or acts on kidney to stim vit D synthesis > goes to gut and reabsorbed CaPO43- from food > inc Ca2+ in circ
A
two
posterior thyroid
chief
31
Q
• Type 1 and Type 2 PTH receptors
– ____predominant
• PTH and calcitonin oppose each other
• Two types of PT receptors ○ Type 1 § Found in all \_\_\_\_ § Kidney § More common ○ Type 2 § Some \_\_\_\_ and kidney • PTH \_\_\_\_ ca2+ release, calcitoini \_\_\_\_ Ca2+ release ○ Counteract each other
A
bones
bones
stim
inhib
32
Q
- Released when the body senses a low Ca2+ state > hypocalcemia > ____ is released > acts on kidney and bones to increase Ca2+ mobilization from the bone, and acts on vitamin D > activated > helps reabsorb Ca2+ from food products > all to help promote Ca2+ localization into the BS to restore Ca2+ levels
- Ca2+ levels higher > ____ is secreted
A
PTH
calcitonin
33
Q
Primary hyperparathyroidism
• Excessive PTH from parathyroids
• Caused by
– Parathyroid ____
– Parathyroid ____
– Parathyroid hyperplasia of ≥ ____ glands
• Secondarily > patients have kidney failure > secondary hyperPTH • Primary hyperPTH > primiarly presents from PT gland > xs productino of PTH bc of tumor, cancer or hyperplasia of >2 glands ○ More PTH being released > more Ca2+ being immobilized from the bones and intestine • Normal ca > \_\_\_\_ mg/dL > higher is hyperPTH ○ 12-13+ > hypercalcemic, and having hyperPTH
A
adenoma
carcinoma
2
10
34
Q
Secondary hyperparathyroidism
Chronic ____ failure
Severe ____
____ deficiency
• When dx is manifesting from a kidney dx > chronic renal failure ○ Kidney dysfunctions > asborption of fluids and Ca PO43- metbaolism/reabsorption ○ Vitamin D is not being produced properly > not acting on intestines to reabsorb Ca from food > hypocalcemic state > PT produces more PTH
A
renal
hypocalcemia
vitamin D
35
Q
Clinical manifestations
• Primary mechanism of getting Ca is through bone ○ \_\_\_\_ (weak bones) or \_\_\_\_ (weak and structurally deficient bone - highly prone to fracture) • PTH has other effects elsewhere in the body ○ \_\_\_\_ problems ○ Dizziness/nervous ○ \_\_\_\_ ○ Bone problems/joint problems
A
osteopenia
osteoporosis
digestive
irritability
36
Q
Clinical manifestations
• Don't memorize the criteria for diagnoising osteopenia/porosis • Diagnosed via a bone scan > DEXA boen scan > inject radioactive dye > scan patient and wherever the dye lights up = bone is not strong in those areas ○ The higher the score > better \_\_\_\_ ○ Lower score > lower \_\_\_\_ > more likely to be \_\_\_\_
A
density
densisty
osteoporosis
37
Q
• Brown tumors
○ Not tumors > ____ found in any bone in a multifocal pattern > highly characteristic of ____ (primary or secondary)
§ Especially in an ____ patient
• Microscopically > central giant cell granulomas
A
CGCG
hyperPTH
older
38
Q
Subperiosteal resorption
• Prone to other bone defects esp in the periphery > \_\_\_\_ bones may become narrow > subperiosteal resorption ○ Treat primary path first > give patients \_\_\_\_, encourage taking calcium pills • If chronic renal failure > \_\_\_\_; and will restore some of the calcium
A
finger
bisphosphonates
dialysis
39
Q
Hypercalcemia of malignancy
• Solid tumors may produce PTH-related protein – Acts on \_\_\_\_ receptor • Solid tumor metastases • \_\_\_\_ • PTH levels remain unchanged
• Upon metastasis > extension mobil of ca from the bones ○ Cancers may produce PTH-related protein > molecular mimic to PTH > can act on PTH 1 receptors (prim on the bones) ○ When cancers metastasize > produce \_\_\_\_ protein > stimulating the receptor on bones to help stimulate calcium mobilization > patients who have hypercalcemia malginancy > patients who have broadly \_\_\_\_ dx, or dx that affects bones (\_\_\_\_) > sig calcium mobil from the bones > hypercalcemic § The \_\_\_\_ levels are unchanged § Not a consequence of PTH > they may \_\_\_\_, bc the body senses it doesn't need PTH • Treat w \_\_\_\_ to prevent bone resorption ○ IV bisphophonates § Risk > MRONJ
A
type I PTH
multiple myeloma
mimicry
metastatic
MM
PTH
40
Q
Multiple endocrine neoplasia
• Autosomal ____
• MEN Type 1
– Mutation in tumor suppressor ____
• MEN Type 2 (aka 2A) and 3 (aka 2B)
– Mutation in ____
• Type 1 ○ Mutation in MEN1 • Type 2 and 3 ○ Mutation in RET ○ Similar except 3 has relevance to dentists > characterized by development of \_\_\_\_ of oral soft tissues and other soft tissue sites ○ Pheochromocytomas ○ Thyroid carcinomas ○ Parathyroid hyperplasia ○ Tall, gangly (look like Marfan) • Know the chart
A
MEN1
proto-oncogene RET
41
Q
• MEN 1 ○ Diamond § \_\_\_\_ § \_\_\_\_ § \_\_\_\_ lesions • MEN 2A/2B ○ \_\_\_\_ ○ \_\_\_\_
A
pit
parathyroid
panc
parathyroid
pheo
42
Q
Mucosal neuromas
• Lips, tongue, ____
• Neuromas of ____, GI tract, bronchi
• ____ histology
A
commissures
eyes
pathognomonic
43
Q
McCune – Albright syndrome
• \_\_\_\_ somatic mutation • \_\_\_\_ spots • Precocious \_\_\_\_ • Multiple endocrinopathies – \_\_\_\_ – Adenomas of endocrine organs – \_\_\_\_ syndrome – Acromegaly – Benign \_\_\_\_ cysts
• Not a genetic disease > sporadically occurring disease ○ Occurs after fertilization ○ \_\_\_\_ disease • Café-au-lait spots • Polyostotic fibrous dysplasia ○ Common disease in the jaw bones • Prone to several endocrinopathies ○ Gene regulates endocrine function § Prone to pit adenomas, ovarian pathology, precocious puberty, agromegaly □ Depending on when exposed > predisposes to various
A
GNAS1 cafe-au-lait puberty hyperthyroidism cushing ovarian
somatic
44
Q
Hyperparathyroidism – jaw tumor syndrome
- Germline mutation in ____ tumor suppressor
- ____ or carcinomas
- ____
- Polycystic ____ disease• When HRPT2 mutated in germline > hyperPTH jaw tumor syndrome
○ Patients develop parathyroid (adenomas or carcinomas; primary) and jaw tumors (ossifying fibromas) and kidney disease
A
HRPT2
parathyroid adenomas
ossifying fibromas
kidney
