1. Pathology I Flashcards

(45 cards)

1
Q

Endocrine organs
• Secrete hormones to target cells and organs
• Peptides
• Steroids
• Amino acids and arachidonic acid analogs
– Catecholamines
• Regulation usually through ____ feedback

• Any organ that produces a hormone > endocrine
	○ Kidney
		§ EPO
		§ Renin
	○ Stomach
		§ Digest and control appetite
	○ Pituitary, hypothalamus, sex organs > ones that are initially thought about
	○ Panc and parathyroids
• Peptides = small \_\_\_\_
• Steroids
	○ \_\_\_\_
	○ Estrogen
• Adrenaline/noradrenaline (epi/noriepi) = \_\_\_\_
• Mechanism of regulation for most is negative feedback regulation
	○ ACTH and cortisol from adrenal
		§ Once ACTH > adrenal to produce corticos > ciruclation and threshold > if high enough > shuts down ACTH secretion from the pituitary
			□ Ngeative feedbcak regulation
A

negative
proteins
testosterone
catecholamines

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2
Q

Pancreas
• ____ and endocrine gland
• Excretes digestive juices

• Secretes 
– Insulin
– \_\_\_\_
– Somatostatin
– \_\_\_\_
• Can't live w/o a pancreas
• Exocrine
	○ Producing substance that exit into the environment
		§ Salivary gland is also an exocrine
		§ Here producing enzymes and lubricant that goes into the \_\_\_\_ to neutralize stomach acid contents
• Endocrine
	○ Produces insulin, glucagon, somatostatin and panc polypeptide
A

exocrine
glucagon
pancreatic polypeptide

duodenum

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3
Q
Endocrine pancreas
Islets of Langerhans
1 – 3 million islets in human pancreas
– Most found in \_\_\_\_
Up to 3000 cells per islet
\_\_\_\_-staining cells
• Small component of the actual pancreas
• Purple = serous acini (the \_\_\_\_ portion)
	○ Produces digestive enzymes and juices
• Arrows highlight the \_\_\_\_ portion > characterized by pale-staining islets of langs
• Most of the hormone is produced in the tail region of the pancreas
• The islets are smalled copared to what's next to it
A

tail
pale
exocrine
endocrine

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4
Q
Endocrine pancreas
• α (A) cells (15-20%) 
– Glucagon
• β (B) cells (\_\_\_\_%) 
– Insulin
• δ (D) cells (5-10%) 
– Somatostatin
• F cells (< 1%)
– Pancreatic polypeptide
• B cells far outnumber the alpha cells
	○ Don't see delta or F cells here
A

60-70

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5
Q

Insulin – activated by blood glucose > ____ mg / 100 mL

  • Decreases blood glucose levels
  • Promotes uptake of glucose
  • Glucose storage via ____
  • Glucose utilization via ____
  • ____ breakdown
  • ____ synthesis in skeletal muscle cells and hepatocytes
	• Glycogenesis occurs in the liver
	• Lipoproteins = chylomicron
		○ Contain \_\_\_\_ cholesterol and protein
		○ Insulin breaks these down for energy
	• Start as \_\_\_\_/pre-hormones before getting metabolized into the functional product
A
70
glycogenesis
glycolysis
chylomicron
protein synthesis
TG
pre-peptide
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6
Q

Glucagon
• Blood glucose < ____ mg / 100 mL stimulates release
• Increases blood glucose levels
• Reciprocal action to insulin

Somatostatin
• Inhibits ____ and glucagon secretion
• Suppresses ____ function

Pancreatic polypeptide
• Suppresses ____ function
• Inhibits gastrointestinal ____
• Controls ____ and weight

• Somatostatin
	○ Regulates dig enzymes from the pancreas
	○ Regulates appetide by reducing the digestive enzymes > not eating > don't need more digestion into th duodenum
• Panc polypep
	○ \_\_\_\_ suppressor
		§ Regualtes in similar way to somato > suppresses exocrine function of panc
		§ Limits ability of GI tract to move
• Panc polypep expression is NOT related to \_\_\_\_
	○ The other three ARE
		§ Once normalized > no reason to further produce insulin, glucagon or somatostatin
A

70
insulin
exocrine

exocrine
motility
satiety

appetite
sugar

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7
Q

Diabetes mellitus

• Prevalence across the world is increasing > more obese > more likely they'll develop type 2 diabetes
	○ Type 1 is \_\_\_\_
		§ Young people
		§ Diff mech from type 2
		§ Inability fi panc to produce \_\_\_\_
			□ Autoimmune destructino of b-cells > dec insulin production from the panc
			□ No insulin > no glucose being taken up by the cells > become hyperglyc
	○ Type 2 Is not \_\_\_\_
		§ Multifactorial in development
		§ Result of 1 and/or 2 things:
			□ Cells are \_\_\_\_ to glucose
				® Receptors are defective (glucose trasnproters)
			□ \_\_\_\_ produces less insulin
	○ Irrespective of type; DM > glucose accum in BS and urine > hyperglycemic > serious complications
• Most expensive disease in this country
	○ 1 or 3 $ is involved in treating DM and complics:
		§ \_\_\_\_
		§ Athersclerosis
		§ \_\_\_\_ (cerebrovascular accidents)kidney dysfunction
		§ \_\_\_\_/infections
		§ Dementia
A
autoimmune
insulin
autoimmune
insensitive
panc
HTN
CVA
amputations
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8
Q

Diabetes mellitus – Diagnostic criteria
• Fasting plasma glucose > ____ mg/dL
• Typical signs and symptoms of hyperglycemia and
random plasma glucose ≥ ____ mg/dL
• Plasma glucose ≥ ____ mg/dL two hours after oral glucose tolerance test
• Glycated hemoglobin (HbA1C) ≥ ____%

• When he says Diabetes > means DM
• >126 mg/dL> patient is DIABETIC; irrespective of type 1 or type 2
	○ No eating the night prior
• Random blood glucose during the day > >200 mg/dL > diabetic
	○ Doesn't matter if you eat, dirnk, etc.
• GTT
	○ Test where you fast over night > like you're going into GA
	○ Blood drawn next morning > base line glucose > then give a \_\_\_\_g dose of sugar (orally)
	○ Two hours > another blood draw > test blood glucose at that time
		§ If at that point is >200 mg/dL > indicative of diabetes
• A1C - isoform of HbA > glycoprotein that years ago they determined the level correlated w blood sugar
	○ Hb is a reflection of blood cells
	○ HbA1C reflects a number (average amount) over a \_\_\_\_ month period of time > blood cells have a 3 month period of life
		§ Reflects average of blood sugar over 3 month period of time
• All are measurements of diabetes
	○ Can also be pre-diabetic
		§ If the numbers are in the table > pre-diabetes
			□ Risk fo developing if not contorlling diet
			□ Fasting BG > \_\_\_\_
			□ GTT > \_\_\_\_
			□ A1C > \_\_\_\_
A

126
200
200
6.5

75
3
100-125
140-199
5.7-6.4
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9
Q

Type I Diabetes mellitus

• ____ destruction of β cells

• Viral infection trigger
– ____ mimicry

• ____-DR, -DQ, -DP

• Islet cell antibodies
– \_\_\_\_ (IAA)
– Glutamic acid decarboxylase (GAD)
– \_\_\_\_ (ZnT8)
– Tyrosine phosphatase-IA2 protein (IA2)
• Also called \_\_\_\_ diabetes
• Autoimmune dx that if left unchecked > life threatening
	○ Constantly measuring their blood
	○ Taking care of their diet
		§ Carry sugar packets in case they need it, bc at risk of hypoglycemia
• Mutlficatorail - there is a \_\_\_\_ link, but not 1:1 (not AD, or AR) > storng genetic component > regulated nby the HLA molecules
	○ DR, DQ, and DP inc the risk fo diabetes type I
	○ \_\_\_\_ infection can trigger the dx
		§ Molecular mimicry is the mechanism
			□ Strep infection causing systemic complications > RF and PSGM
			□ Same mechanisms play the role
• Once dx triggered > autoab produced across array of antigens in the pancrease
• Don't manifest w diabetes from time of birth, but rarely during their single digit years > more likely in adolescence and late-teens/early-adults
	○ Takes \_\_\_\_ to manifest > takes a while for ab to damaged the panc to the extent of causing insulin deficiency
A
autoimmune
molecular
HLA
insulin autoantibody
b cell-zinc transporter

juvenile
genetic
viral
time

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10
Q

Type II Diabetes mellitus
• ____ pathogenesis
• ____ tissue resistance to insulin
• Insufficient ____ of insulin by β cells

• Not the result of AI-type dx
• Mechanism results in:
	○ Cells resistant to insulin
		§ \_\_\_\_ receptor being mutated in those cells
	○ Insufficient secretion of insulin
		§ Iatrogenic
			□ Panc surgeyr > lose chunk of \_\_\_\_ > less insulin being secreted
				® Presence of a tumor
• Don't memorize the picture
	○ Rare cases > b cell failure > insuffieicnt secretion
A

multifactorial
target
secretion

insulin
tail

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11
Q

• KNOW THE CHART
• Type 1
○ Juvenile diabetes
○ Age of onset = ____ (late childhood, adolescence, into teen)
§ Older people bc of a ____ trigger (RARE!)
○ Rather ____ onset
§ Kids start losing weight, fail to thrive properly, meet their growth thresholds, feeling run down and thirsty
○ No ____ link; the link is more so in risk
○ ____ destrucitno of beta cells is the mechanism
○ Test insulin levels > ____ to speak off

• Type 2
	○ Non-\_\_\_\_ dependent diabetes
	○ \_\_\_\_ dx (more younger people are getting it now)
	○ \_\_\_\_ onset (years!)
		§ Stage of pre-diabetes (prolonged) before full blown diabetes
	○ Far more \_\_\_\_ than type 1
	○ \_\_\_\_ genetic link; but not a direct gene-to-gene link
		§ Parents are obese, if predisposed to weight gain > important risk factor
	○ Insulin \_\_\_\_ and insuff \_\_\_\_ of insulin
	○ Test insulin levels > initially \_\_\_\_ bc the target cells are not responding to insulin
		§ Panc produces more and more insulin > early on in these patients > high insulin levels, eventually they drop > the target tissuesa ren't responding > circ at high levels > panc adapts > producing less insulin > what's in the circ will \_\_\_\_ off (due to the half-life)

• Nutritional status - ____ in younger, and in the olde rpop - overweight and obese
○ Houston has the highest rate in the country

A
young
viral
quick
genetic
AI
none
insulin
adult
slower
common
stronger
resistance
secretion
high
die

undernourished

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12
Q

2018 Recommendations for type 2 diabetes testing in asymptomatic adults:

Overweight/obese with risk factors
– \_\_\_\_-degree relative
– High-risk ethnicity
– History of \_\_\_\_ disease / hypertension
– HDL < 35 mg/dL and/or triglycerides > 250 mg/dL
– \_\_\_\_ inactivity
Patients with \_\_\_\_
Women diagnosed with \_\_\_\_ diabetes 
Age > \_\_\_\_ years

2018 Recommendations for type 2 diabetes testing in under 18 years:

Overweight / obese with risk factors
– ____ history of diabetes during pregnancy
– ____ history
– High-risk ____

• Memorize all of these
• Advocating screening for at risk children (for the first time)
• High risk ethinicty > Hispanic and \_\_\_\_ and NA
• Obsese and atherosclerosis
• Higher the HDL level > the \_\_\_\_ it is
	○ High TG or low HDL > risk factor that warrants screening
• Screened every \_\_\_\_ years for diabetes once you're prediabetic
• Even if you're \_\_\_\_, once over 45 you're screened
A
first
cardiovascular
physical
prediabetes
gestational
45

maternal
family
ethnicity

AA
better
3
healthy

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13
Q

• Part of screening includes a ____ history
○ Includes a visit to the dentist
• Advocate a dental examination as part of that process

A

dental

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14
Q

Acute clinical complications

• Hyperglycemia
– Glycosuria causes osmotic ____
– Induces ____ and ____
– ____

• Diabetic ketoacidosis
– ____ breathing

• Hyperglycemia can be life threatening
	○ First sign is the 3 P's: polyuria, polydipsia and polyphagia
		§ Pee a lot, very thirsty and very hungry
			□ Glucose in the urine > creates an osmotic gradient that forces the urine to become even more diluted
			□ Peeing more > losing more H2o > more thirsty
			□ Cells aren't eating enough > body senses you have to eat more > become more hungry
• Seen commonly w \_\_\_\_ > weight loss
	○ Kids and young adults > first signs that something is wrong in that patient > start losing weight rather rapidly
• Diabetic ketoacidosis
	○ \_\_\_\_ balance is out of wack > significant complication that one sees in DM > extrmeely high \_\_\_\_ levels (>200 mg/threshold; have 500-700 mg/dL) > more likely that they're hyperglycemic, and using it in weird ways > formatino of \_\_\_\_ bodies that end up in the circulation (urine) > gives breath that smells like \_\_\_\_
	○ Most commonly seen in \_\_\_\_
		§ Body produces more HCO3 > converted to CO2 > expelled through the lungs
	○ Have a unique breathing pattern > kussmaul breathing pattern
A

diuresis
polyuria
polydipsia
polyphagia

kussmaul

type 1
acid base
sugar
ketone
acetone

type 1

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15
Q

Acute clinical complications

Hyperosmolar coma in ____ diabetes
– No ____

Extremely high levels of plasma glucose – > ____ mg/dL

____ onset than ketoacidosis

High risk of mortality

• Another rcomplication seen in type iI > hyperosmolar coma > comatose from high sugar levels > don't get KETOSIS
	○ Ketones found in type I and not \_\_\_\_ diabetes
	○ For the coma to occur > levels of glucose must be sky high > blood congealed bc of the glucose sitting in the circ
		§ Complication occurs slower than ketoacidosis; with coma > may get death
• Know the text in this photo
A

type 2
ketosis

600
slower

type 2

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16
Q

Acute clinical complications

____ resulting from treatment

Develops during ____, fasting or stress

Treat with ____ or intramuscular glucagon

• Some patients w diabetes may experience hypoglycemia
	○ Can occur thorugh treatment > do not \_\_\_\_ properly > hypoglycemic state > insulin levels being regulated in dramatic fashion > and then glucagon secreted instread of insulin
	○ Patients tend to be ones w high preference for \_\_\_\_, fast or get stressed out > metabolic imbalance > hypoglycemia in some patients
		§ Treatment > w glucose to counterbalance that fast and dramatic reduction; and glucagon to release glucose from the system
A

hypoglycemia
exercise
glucose

titrate
exercise

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17
Q

• Standard of care in a dental office > patient may become hypoglycemic
• Patients put in ____, and given a ____ packet to restore sugar levels
○ Know the diagram

A

trendelenburg

sugar

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18
Q

Chronic clinical complications

____ disease

\_\_\_\_ disease 
– Retinopathy
– Neuropathy
– Nephropathy
 • End stage renal disease 

Diabetic ____ ulcers

• Infections

• From type II standpoint > patient at risk for developing severe and morbid, chronic complications > most impt: macrovascualr disease
	○ Implies large artery disease > \_\_\_\_
		§ Risk for developing MI, severe HTN, kidney failure and for a CVA (stroke)
		§ Blood flow is much \_\_\_\_ > more cxn w sugar > blood flows less rapidly > will settle in and carry the plaque/chol in the BS > deposit on BV walls bc flow is so slow
			□ Patients at risk for microvascualr dx > smaller vessels in the peripehry > eyes, fingertips, lower extemeites, brain, kdiney
				® Patients may become \_\_\_\_
				® May dvelop \_\_\_\_
				® May develop peripheral \_\_\_\_
				® Or \_\_\_\_ > may result in end stage renal dx > complete kidney failure
			□ Explains why skin breaks down in the perpihery > feet
				® Foot ulcers are common (furthest from heart), and patients are at risk for infection > slower BF > bacteria have higher propensity to aggregate ina. Specific site
				® Single most common cause of below knee amputation is \_\_\_\_
A

macrovascular
microvascular
foot

atherosclerosis
slower
blind
dementia
neuropathy
nephropathy

diabetes

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19
Q

Chronic clinical complications

• Prone to ifnection elsewhere:
	○ \_\_\_\_
		§ Deep fungal infection
	○ \_\_\_\_ one of the most common appearances for diabetes
		§ Treatment: make sure the diabetes is under \_\_\_\_ > the periodontal dx will recur if it's not under control
A

mucormycosis
periodontitis
control

20
Q

Hypothalamus
• Found in floor and lateral walls of ____ ventricle
• Connected to ____ gland via stalk of axons
• Produces
– ____
– ____ (Anti-diuretic hormone)
– ____
– ____
• The axis start w the hypothalamus
○ Endocrine organ
§ ADH produced in hypo and acts on the kidney via the pit
○ Physically connected to the pit via a string of axons
• These four are produced in hypo and moved to the pit (can act or store there)
○ Two components
§ Adenohypohysis
□ ____ pouch
® The mouth is also derived from rathke’s pouch
§ Neurohyphysis
□ Posterior portion - a different embryonic origin
□ Stores ____
® Produced by the hypothalamus and stored here

A

third
pituitary

CRH
vasopressin
oxytocin
TRH

rathke’s
ADH and oxytocin

21
Q

Pituitary gland
• Adenohypophysis
– Derived from ____ pouch
– ____ tissue

• Neurohypophysis
– Derived from ____ (diencephalon)
– No ____ function
– Stores ____

A
rathke's
glandular
neural ectoderm
endocrine
neurosecretions
22
Q

Pituitary gland

	• GH
		○ \_\_\_\_ development
	• ADH
		○ Kidney
		○ Reabsorbs \_\_\_\_
	• Oxytocin
		○ Helps to stim \_\_\_\_, contractions during pregnancy, and the orgasmic response
	• TSH
		○ Acts on thyroid to produce hormones
	• PRL
		○ During \_\_\_\_
		○ Also used in > goes to sexual organs > maintains fertility
	• FSH, LH
		○ \_\_\_\_ hormones
		○ Helps during sperm formation and ova formation
A
bone
water
lactation
lactation
sexual
23
Q

Pro-opiomelanocortin

POMC post-translationally modified
– \_\_\_\_
– α-, β- and γ- melanotropins (MSH)
– \_\_\_\_
– β-endorphin
– \_\_\_\_
* ACTH regulates steroid production in the adrenals (corticosteroids)
* \_\_\_\_ acts on pit to produce POMC > post translationally modified to produce a whole bunch of smaller hormones and peptides from the common precursor
A

ACTH
b and y lipotropins
metenkephalin
CRH

24
Q

Pituitary adenoma
Incidentally found in ____ autopsies
Functional vs non-functional
Signs / symptoms related to secreted hormone(s)

Hyperprolactinemia
Excessive prolactin – most common ____, infertility, hypogonadism, reduced ____ density

• Benign tumors can become functional and can produce hormones
	○ One of the most common tumors that exist
		§ Only handful will manifest systemically during life w this tumor
• Functionality is related to whatever hormone is being produced by this tumor
• Hyperprolactinemia
	○ Frequent bleeding > amenorrhea
	○ Breasts become engorged
	○ Males - hypogonadism
	○ Reduced bone density
A

amenorrhea

bone

25
Pituitary adenoma Gigantism Excessive growth hormone – ____ closure of epiphyses • Born w a pit adenoma before their bones mature and develop > and tumor is producing GH > patients become giants • Rock hit him in the head > burst pit gland and lead to his death ○ David v Goliath story
before
26
Pituitary adenoma Acromegaly Excessive growth hormone – ____ closure of epiphyses • Occurs after maturation - an adult patient develops a pit adenoma > acromegaly ○ This is a patient w ____ acromegaly (just one foot)
after | partial
27
Pituitary adenoma Cushing disease Excessive ____ • Caused by excessive ACTH production from the pituitary
ACTH
28
Hypopituitarism • Loss of hormone secretion • Trauma to ____ • Ischemic destruction after systemic hypotension – ____ syndrome due to excessive childbirth- related blood loss • Vascular disease often related to ____ • If gland is not functioning properly > atrophied > less hormone produced and you have a systemic array of complications • May happen during surgery (iatrogenic) bc of physical trauma to the pit stalk • Some women during childbirth may bleed excessively > ____ > sheehan syndrome (may lead to death) ○ Diabetes can also cause ____ to a BV that innervates the pit gland resulting in the same complication • Cannot treat this syndrome, unless you give all these extra ____ (not very effective)
pituitary stalk sheehan diabetes mellitus ischemia ischemia hormones
29
Diabetes insipidus • Inability to conserve ____ and concentrate ____ • Central diabetes insipidus – Reduced ____ secretion • Nephrogenic diabetes insipidus – Reduced ability of kidney tubules to respond to ____ – Defect in ____ water channels in collecting ducts or vasopressin receptors • Unique form of diabetes that's cahracterized by the inability to conserve water and concentrate urine > pee a lot, excessive thirst and diluted urine, w/ ____ > results from reduced vasopression (ADH) secretion > central DI or; if the kideny doesn't respond properly to ADH > nephrogenic DI (ADH secreted properly) > end result same same
water urine vasopressin ADH aquaporin-2 hypernatremia
30
Syndrome of inappropriate ADH secretion (SIADH) due to excessive vasopressin secretion * ____ and polydipsia are common to both * ____ is only DM, not DI * ____ only DI, not DM * ____ is DM, not DI • Not enough ADH secretion, on the other end you may have too much secretion of ADH ○ SIADH § Exact opposite of DI § Not enough urine ____ § Too ____ a urine § ____ • Both will present w patients who have excessive thirst > hyponatremia > electrolyte balance is low > tend to be more thirsty > both result in excessive thirst [???] ○ When you lose electrolytes you feel like you need to take in more water ○ ____ > taking in more water > ____ water (you're bloated, but your body senses you need more water to drink)
polyuria polyphagia hypernatremia glucosuria output cxn hyponatremia overhydrated retaining
31
``` Obesity • Amount and type of food • Central control of satiety • Hormonal control • Metabolic rate ``` • Hormones play a critical role in regulating appetite ○ Some mutations that can increase the risk for obesity ○ Hormones at the hypo and pancreas and stomach • If metabolic rate is ____ > increases risk for obesity • BMI ○ Puts patients into a category of underweight, overweight or potentially obese ○ Normal § ____ kg/m2 § Much more difficult to return from obese state § Risk factors are much harder
low 25 25-29 30
32
• How appetiite suppression and regulation is regualted by: ○ ____ cells ○ ____ glands § Corticosteroids □ Cushing's patients gain ____ □ Release more roids > augment appetite
fat adrenal weight
33
Obesity • Leptin or leptin receptor mutation – Normally suppresses ____ – Activates ____ expression • POMC ____ • People who harbor mutations in POMC > inactivating mutation that ____ POMC activity that in turn increases appetite • From POMC > ____ > can regulate appetite as well • Leptin is a critical regulator of appetite ○ This protein is expressed in the ____ to regulate POMC from the pituitary
appetite POMC mutation reduces alpha-MSH hypothalamus
34
Adrenal glands – adrenal medulla • Chromaffin cells produce and secrete: – Epinephrine – cells contain ____ granules (90%) – Norepinephrine – cells contain ____ granules (10%) – Dopamine • Most catecholamine output is ____ • Metabolism into metanephrines and ____ – Excreted in urine • Glands contain diff cell types > most important: chromaffin cells > secrete and produce epi, norepi and dopamine ○ Cells found within the adrenal medulla ○ Most of the cells produce epi § Epi and norepi metabolized into metbaolites into the BS and into the urine > metanephrine and VMA (vanilia-mandilic acid)
large small epinephrine VMA
35
Adrenal glands - adrenal medulla • VMA is very important metabolite used to test functionality of the adrenal gland ○ High levels of VMA > indicated pathology of the ____ (doesn’t tell you what type of pathology)
adrenal gland
36
Fight, Flight or Freeze • Adrenergic receptors with array of target tissues • Norepinephrine > vasoconstriction via ____ receptors > increase heart rate and contractility via ____ receptors • Epinephrine > vasodilation in skeletal muscle / liver via ____ receptors (major) > vasoconstriction everywhere else via ____ receptors (minor) > net reduction in ____
alpha1 beta1 beta2 alpha1 peripheral resistance
37
Fight, Flight or Freeze • Norepinephrine > ↑ systolic and diastolic ____ – Induces reflex ____ and decreased ____ • Epinephrine > ↑ ____ • Epinephrine regulates ____, lipolysis, insulin secretion – ↑ Circulating ____ and free fatty acids
blood pressure bradycardia cardiac output pulse glycogenolysis glucose
38
Pheochromocytoma • Functional neoplasm – Secretes excessive catecholamines • Usually ____ • Induces ____ and increases blood pressure • Frequent association with genetic disease – ____ – ____ • Tumor that's aggressive and malignant of the adrenal gland • Found in the adrenal medulla, often times functional to the point its secreting epi and norepi > patient has a ____ response over and over again ○ Pulse is always racing ○ HR is always racing ○ Will have HTN • Tumor is uncommon ○ Manifesting in a genetic dx > NF type 1 and MEN type 2 and 3
norepinephrine vasoconstriction multiple endocrine neoplasia type 2 and 3 neurofibromatosis type 1 FFF
39
Pheochromocytoma ``` • Clinical signs and symptoms – Sustained or episodic ____ – Tachycardia – ____ – Anxiety – ____ – Hyperglycemia ``` • Diagnosed by measuring ____ and/or metabolites • ____ used to differentiate essential hypertension from pheochromocytoma hypertension – Should suppress plasma ____ levels • FFF response is constant • Easily diagnosed - 10 y/o patient comes in w a racing heart ○ Checks BP - high ○ Pulse is 115 bpm (abnormal) ○ Test for VMA within the urine > VMA Is a breakdown of catecholamines > good metric to predict a tumor from the adrenals ○ Another test - clonidine test § Clonidine is a drug that's used in hypertensive patients □ If BP is suppressed > suggests it's completed through the adrenal gland; if not suppressed > suggests it's central (not PC!) ® Suppresses ACTH secretion of hypothalamus, suppressing catechol release from the gland itself
hypertension angina pectoris diaphoresis catecholamines clonidine norepinephrine
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Neuroblastoma * Most common solid cancer in toddlers * Associated with ____ amplification * Produce urinary ____ and/or metabolites • If patient is very young (2-5 y/o) ○ This tumor is more likely • Can be easily diagnosed bc this tumor produces ____ that could be tested in the urine ○ This tumor can be aggressive ○ High levels of n-myc > high levels of metastasis and ____ prognosis § No n-myc > much better prognosis • Adrenal medullar tumor
MYCN catecholamines VMA poor
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``` Adrenal glands – adrenal cortex • Glucocorticoids – Stimulate catabolism of ____, protein – Promote ____ in liver – Increase ____ – Suppress ____ – Anti-____ ``` • Cortex - ACTH ○ Produces glucocorticoids § Regulates BP § Used as an anti-inflam agent
``` peripheral fat gluconeogenesis blood glucose immunity inflammatory ```
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Adrenal cortex • Mineralocorticoids – Regulate ____ excretion – Maintain ____ homeostasis • Aldosterone regulation via – ____ system – Pituitary ACTH – ____+and Na+ • Mineralocorticoids ○ Aldosterone § ____ pathway □ Regulates Na and H2O uptake in the kidney > regulates BP, and K+ and Na+ regulation
``` sodium intravascular renin-angiotensin K RAT ```
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Juxtaglomerular apparatus • Regulates ____ • Renin-angiotensin-aldosterone system – ____ homeostasis – ____ hemodynamics • Once kidney senses a low BP and fluid > activates ____ formation from macula densa cells within the JG app > goes to adrenals > converts AT to ATI > adrenal gland to trigger gland to release ____ > goes back to kidney to regulate fluid levels
``` blood pressure sodium renal renin aldosterone ```
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Juxtaglomerular apparatus • Juxtaglomerular cells express ____ • Renin converts angiotensinogen to ____ • Angiotensin I converted to angiotensin II – Angiotensin converting enzyme • Angiotensin II acts on adrenal gland to produce ____ • Aldosterone acts on connecting tubules and ____ – Increases absorption of ____+ and water – Increases ____+ secretion – Increases ____ and volume
``` renin angiotensin I aldosterone collecting ducts Na K blood pressure ```
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Diseases of adrenal cortex * Hyperadrenocorticism * Hypoadrenocorticism * Hyperaldosteronism ``` • Cushings (hyperadrenocorticism) • Hypoadrenocorticism ○ ____ ○ ____ • Hyperaldosteronism ○ Patient has a ____ face ○ Can also get excessive hair in cushings ```
addison's pigmentation hairy