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161 Cards in this Set

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A woman presents with diffuse goiter and hyperthyroidism. What are the expected TSH and thyroid hormone values?
Low TSH and high thyroid hormones
A 48-year-old female presents with progressive lethargy and extreme sensitivity to cold temperatures. What is the diagnosis?
Hypothyroidism
A pt with elevated serum cortisol levels undergoes a dexamethasone suppression test. 1 mg of dexamethasone does not decrease cortisol levels, but 8 mg does. What is the diagnosis?
Pituitary Tumor
A 50-year-old man complains of diarrhea. On physical exam, his face is plethoric and a heart murmur is detected. What is the diagnosis?
Carcinoid syndrome
A woman of short stature presents with shortened 4th and 5th metacarpals. What endocrine disorder comes to mind?
Albright's herditary osteodystrophy, or pseudohypothyroidism
A nondiabetic pt presents with hypoglycemia but low levels of C peptide. What is the diagnosis?
Surreptitious insulin injection
A pt's MRI shows filling of sella tursica with cerebrospinal fluid. What is the most likely clinical presentation?
Normal. Residula pituitary tissue is functional and can compensate
The adrenal cortex comes from what embryological cell layer?
Mesoderm
The adrenal medulla comes from what embryological cell layer?
neural crest
What are the three levels of the adrenal cortex?
Zona Glomerulosa, zona fasciculata, zona reticularis
Which hormones are released from the three layers of the adrenal cortex?
G= aldosterone, F= cortisol,sex hormones, R= sex hormones
The adrenal medulla secretes what hormones?
Catecholamines = EPI, NE
Trace the drainage of the left adrenal gland?
Left adrenal -> left adrenal vein -> left renal vein -> IVC
Trace the drainage of the right adrenal gland?
Right adrenal -> right adrenal vein -> IVC
What hormones are secreted by the posterior pituitary (neurohypophysis)?
vasopressin, oxytocin
The posterior pituitary is derived from what embryolic germ layer?
neuroectoderm
The anterior pituitary (adenohypophysis) secretes what hormones?
FSH, LH, ACTH, TSH, Prolactin, GH
The anterior pituitary is derived from what embryolic germ layer?
oral ectoderm
The a-subunit is common to which hormones?
TSH, LH, FSH, hCG
Which subunit determines hormone specificity?
b-subunit
What are the endocrine pancreas cell types and for what hormones do they specify?
a=glucagon, b=insulin, d=somatostatin
What hormone is required for the anterior pituitary to release prolactin? Where does it come from?
Throtropin Releasing hormone (TRH) from the hypothalamus
What hormones inhibit the secretion of prolactin and where do they come from?
Dopamine from the hypothalamus and prolactin itself by stimulating dopamine
TRH, released from the hypothalamus stimulates the release of which hormones from the pituitary?
TSH, prolactin
Dopamine, released from the hypothalamus inhibits the release of which hormones from the pituitary?
prolactin
CRH, released from the hypothalamus stimulates the release of which hormones from the pituitary?
ACTH
GHRH, released from the hypothalamus stimulates the release of which hormones from the pituitary?
GH
Somatostatin, released from the hypothalamus inhibits the release of which hormones from the pituitary?
GH, TSH
GnRH, released from the hypothalamus stimulates the release of which hormones from the pituitary?
FSH, LH
What is the rate limiting step in the synthesis of adrenal steroids?
the conversion of cholesterol to pregnenalone via 20,22 desmolase
What are the 3 congenital bilateral adrenal hyperplasias?
17a-hydroxylase deficiency, 21b-hydroxylase deficiency, 11b-hydroxylase deficiency
What is the most common congenital bilateral adrenal hyperplasia?
21b-hydroxylase deficiency
What function does 17a-hydroxlase have in the synthesis of adrenal steroids?
it converts pregnenalone to 17-OH-pregnenalone and progesterone to 17-OH-progesterone producing cortisol and sex hormones
What are the signs and symptoms of 17a-hydroxylase deficiency?
decreased sex hormones, decreased cortisol high aldosterone (mineralcort); sx: HTN, hypokalemia; phenotypically female but no maturation
What function does 20b-hydroxlase have in the synthesis of adrenal steroids?
converts progesterone to 11-deOH-corticosterone and 17a-OH-progesterone to 11-deOH-cortisol
What are the signs and symptoms of 20b-hydroxylase deficiency?
decreased cortisol, increased ACTH, decreased mineralcorticoids, increased sex hormones; Sx: masculinization, femal pseudohermaphrodism, hypotension, hyponatremia, hyperkalemia, increased PRA, volume depletion
What function does 11b-hydroxlase have in the synthesis of adrenal steroids?
converts 11-deOH-corticosterone to corticosterone and 11-deoxycortisol to cortisol
What are the signs and symptoms of 11b-hydroxylase deficiency?
decreased cortisol, decreased aldo and corticosterone, increased sex hormones; Sx: masculinization, HTN
What structures are insulin-dependent?
skeletal muscle and adipose tissue
What structures are insulin-independent?
RBC and Brain
What glucose uptake receptors are in skeletal and adipose tissue?
GLUT-4 (insulin-dependent)
What glucose uptake receptors are in the brain and RBCs?
GLUT-1 (insulin independent)
What does the brain use for metabolism in episodes of starvation?
ketone bodies
From where in the adrenal gland does cortisol come?
adrenal fasciculata
What are the 5 main functions of cortisol?
(1) anti-inflammatory, (2) increase gluconeogenesis, lipolysis, proteolysis, (3) decrease immune function, (4) maintain BP, (5) decrease bone formaiton
What stimulates the production of cortisol in the adrenal fasciculata?
CRH (hypothalamus) -> ACTH (pituitary) -> cortisol
What are the 4 main causes of Cushing's Syndrome? What would ACTH levels look like in each case?
(1) Cushing's Dz (1' pit adenoma, increased ACTH), (2) 1' adrenal (decreased ACTH), (3) Ectopic ACTH production (increased ACTH), (4) iatrogenic (chronic steroids, decreased ACTH)
What is the clinical picture of Cushing's Syndrome?
wt gain, moon fascies, buffalo hump, truncal obesity, supraclavicular fat, purple striae, hyperglycemia, osteoporosis
Describe the cortisol levels following a dexamethasone supression test in the following conditions: healthy, ACTH-producing tumor, Cortisone-producing tumor
healthy: decreased cortisol after low dose; ACTH tumor: high cortisol after low dose/low after large dose; Cortisone tumor: high after low and high dose
Primary hyperaldosteronism (Conn's syndrome) is the result of what?
aldosterone secreting tumor
What are the signs and sx of Conn's syndrome?
HTN, hypokalemia, metabolic alkalosis, low plasma renin
What is the treatment for Conn's syndrome?
Spironolactone, a K-sparing diuretic and aldo antagonist
What are some of the main causes of secondary hyperaldosteronism?
renal artery stenosis, chronic renal failure, CHF, cirrhosis, nephrotic syndrome
What is the MOA behind secondary hyperaldosteronism?
kidney perceives low intravascular volume -> overactive renin-angiotensin system (high plasma renin)
What is Addison's Dz?
primary deficiency of aldo and cortisol due to adrenal atrophy
What are the signs and SX of Addison's Dz?
hypotension, skin hyperpigmentation (due to MSH) Atrophy of all 3 adreal cortical divisions
What is the distinguishing feature between 1' and 2' adrenal insufficiency?
2' insufficiency does not have hyperpigmentation of skin (decreased pit ACTH production)
What is the most common tumor of the adrenal medulla in adults?
Pheochromocytoma
What other dzs may be associated with pheochromocytomas?
Neurofibromatosis, MEN types II and III
Chromaffin cells in the adrenal medulla are derived from what embryological precursor?
neural crest cells
What is the most common tumor of the adrenal medulla in children?
neuroblastoma
What is Sheehan's syndrome?
postpartum hypopituitarism caused by infarction of the pituitary gland following severe bleeding and hypoperfusion during delivery
What are the signs of Sheehan's syndrome?
fatigue, anorexia, poor lactation and loss of pubic and axillary hair
What is the tx for pheochromocytomas?
irreversible, nonselective, a-blockers (phenoxybenzamine)
What are the five most common symptoms of pheochromocytoma?
Pressure, Pain, Perspiration, Palpitations, Pallor
What is the 10% rule in regards to pheochromocytomas?
10% malignant, 10% bilateral, 10% extra-adrenal, 10% calcify, 10% kids, 10% familial
What organs are affected in MEN Type I (Multiple endocrine Neoplasias)?
Pancreas, Parathyroid, Pituitary tumors
What is the other name for MEN type I?
Werner's Syndrome
MEN type II are what types of tumors?
medullary carcinoma of the thyroid, pheochromocytomas, parathyroid tumors
What is the other name for MEN type II?
Sipple's Syndrome
MEN type III involve what types of tumors?
medullary carcinoma of the thyroid, pheochromocytomas, oral and intestinal ganglioneuromatosis (mucosal neuromas)
Which MEN types are associated with the ret gene?
types II and III
All MEN syndromes have what pattern of inheritance?
autosomal-dominant
What are the relative lab values associated with hypothyroidism?
increased TSH, decreased toal T4 and free T4, decreased T3 uptake
What are the sx of hypothyroidism?
cold intolerance, hypoactivity, wt gain, fatigue, lethargy, decreased appetite, decreased reflexes, myxedema (facial/periorbital), dry skin, brittle hair
What is Riedel's thyroiditis and with what dz?
thyroid is replaced with fibrous tissue; found in hypothyroidism
From what cells in the parathyroid is PTH produced?
Chief cells
What are the 4 main functions of PTH?
(1) increase bone reabsorption, (2) increase kidney reabsorption of Ca, (3) decrease kidney reabsorption of phosphate, (4) increased 1,25 Vitamin D prdxn by stimulating kidney 1a-hydroxylase
How is PTH regulated?
increases in serum Ca decreases PTH release and vice versa
How many parathyroid glands are there?
4
1,25-(OH)2vitamin D has what effects on bones?
releases phosphate from the matrix of bone
1,25-(OH)2vitamin D has what effects on intestines?
increases calcium and phosphate reabsorption
From what two sources do human receive vitamin D?
(1) D3 from sun exposure, (2) D2 from plants
Describe how Vitamins D3 and D2 get converted to active form of Vitamin D?
Both D3 and D2 are converted to 25-OH vit D in liver and to active form 1,25-OH2 vit D in the kidney
What are the three main functions of active Vitamin D?
(1) increase absorption of dietary calcium, (2) increased absorption of dietary phosphate, (3) increased bone reabsorption of Ca and phosphate
What are the four main regulators of active vitamin D?
all of the above increase active vit D: (1) increased PTH, (2) decreased [Ca], (3) decreased phosphate; Active vit D inhibits its own production
What are the clinical results in childhood and adulthood from a deficiency of Vitamin D?
child: rickets adult:osteomalacia
State the Ca, phosphate, and alk phos levels for the following disease state: hyperparathyroidism
Ca: increased, Phosphate: decreased, Alk Phos: increased
State the Ca, phosphate, and alk phos levels for the following disease state: Paget's Disease of Bone
Ca: nl/high Phos: nl, Alk phos: very high
State the Ca, phosphate, and alk phos levels for the following disease state: Vitamin D intoxication
Ca: high Phos: high Alk Phos: nl/high
State the Ca, phosphate, and alk phos levels for the following disease state: Osteoporosis
Ca: nl, Phos: nl, Alk Phos: nl
State the Ca, phosphate, and alk phos levels for the following disease state: Renal Insufficiency
Ca: low, Phos: high, Alk Phos: nl
What hormone opposes the actions of PTH?
Calcitonin
Where is the source of calcitonin?
Parafollicular cells (C cells) of the thyroid
What is the function of calcitonin?
decreased bone reabsorption of calcium
How is calcitonin regulated?
increased serum [Ca] increases calcitonin secretion
What is the role of binding globulins in steroid hormone maintenance?
increases solubility and allows for increase delivery of hormone to the target organ
Increased levels of sex hormmone binding globulin leads to what condition?
gynecomastia (lowered free testosterone)
Decreased levels of sex hormmone binding globulin leads to what condition?
hirsutism (increased free testosterone)
Where are the target organ steroid hormone receptors found?
nuclear membrane
Where is most T3 formed?
in the blood/periphery
What are the 5 main functions of TH?
(1) aid in bone growth, (2) CNS maturation, (3) b-adrenergic effects, (4) increased BMR, (5)_increased glycogenolysis, gluconeogenesis, lipolysis
What protein binds to T3/T4 in the blood?
Thyroxine Binding Globulin (TBG)
In what conditions do you see high/low TBG levels?
high: pregnancy low: hepatic failure
What factors increase the release of TH?
TRH stimulates TSH from pit which stimulates follicular cells
What factor inhibits TH secretion?
T3 decreases anterior pit sensitivity to TRH
What are the signs and sx of subacute thyroiditis?
hypothyroidism that often follows a flu-like illness; elevated ESR; painful thyroid gland; may present hyper- early in the course
What is the etiology of toxic multinodular goiter?
Iodine restoration following a period of deprivation; causes a release of T3 and T4
Papillary carcinoma of the thyroid is characterized by what features?
ground glass nuclei (Orphan Annie eyes), psammoma bodies;good prognosis
An increased risk of papillary carcinoma of the thyroid is associated with what treatment?
irradiation in childhood
Medullary carcinoma of the thyroid is associated with what other comorbidities?
MEN Types II and IIb (III)
What cells are affected by medullary carcinoma of the thyroid?
parafollicular "C cells"
What is the demographic and prognosis for anaplastic/undifferentiated cancer of the thyroid?
Older pts; poor prognosis
Thyroid lymphoma is associated with what comorbidity?
Hashimoto's
What is the etiology of endemic cretinism?
lack of dietary iodine (endemic goiter)
What is the etiology of sporadic cretinism?
defect in T4 formation or developmental failre in thyroid formation
How will a patient with cretinism appear?
pot-bellied, pale, puffy-faced child with protruding tongue and umbilicus
What is excess GH in adults called? Children? how do you treat it?
adults: acromegally
children: gigantism
Treat with Octreotide
How do you test for excessive GH?
oral glucose tolerance test
What is the etiology of primary hyperparathyroidism?
adenoma
What are the signs and sx of primary hyperparathyroidism?
hyper-Ca, hypercalciuria (renal stones), hypo-P, increase PTH and cAMP in urine, constipation

"Stones, bones and groans"
Secondary hyperparathyroidism is seen most often in with other illness?
chronic renal dz; decreased serum Ca (Hypo-Ca, Hyper-P, increased PTH)
What is a common sequelae of secondary hyperparathyroidism?
renal osteodystrophy - bone lesions
What are the two most common causes of hypoparathyroidism?
DiGeorge and accidental surgical excision in thyroid surgery
What are the two signs one can test for hypoparathyroidism?
Chvostek's sign - contraction of facial muscles with tap

Trousseau's sign - occlusion of brachial artery with BP cuff -> carpal spasm
What causes pseudohypoparathyroidism?
AutoDom kidney unresponsiveness to PTH
What is the most common pituitary adenoma?
Prolactinoma
What are the acute manifestations of diabetes mellitus?
polydypsia, polyuria, polyphagia, weight loss, DKA (type 1), coma (type 2)
What tests are used to diagnose diabetes mellitus?
fasting serum glucose, glucose tolerance test, HbA1c
What is the primary defect in Type 1 DM?
viral or autoimmune destruction of B-cells
What is the primary defect in Type II DM?
increased resistance to insulin
Which Type of DM has a strong genetic predisposition?
Type II
Which HLA's are associated with Type I DM?
DR3 and DR4
In which Type of DM is DKA common?
Type I
What are the signs and sx of DKA?
Kussmaul respirations (rapid deep breathing), hyperthermia, n/v, dementia, dehydration, fruity breath odor
What fungal infection is a potential complication of DKA?
Rhizopus causing mucormycosis (fungal infection of the sinuses)
What is the treatment for DKA?
Fluids, insulin, potassium
What characterized diabetes insipidus?
intensive thirst and polyuria with an inability to concentrate urine (lack of ADH, or lack of kidney response to ADH)
Central DI has what etiologies?
pituitary tumor, trauma, surgery, histiocytosis X)
What test is used to diagnose DI?
water deprivation test - urine osmolality doesn't change
What is the treatment for DI?
Fluids; intranasal desmopressin for central DI; for nephrogenic DI - hydrochlorothiazide, indomethacin, amiloride
What are the three features of SAIDH?
1. excessive water retention
2. hyponatremia
3. urine osmols > serum osmols
What are the 4 main causes of SIADH?
1. ectopic ADH (small cell lung cancer)
2. CNS disorders/head trauma
3. Pulmonary dz
4. drugs (cyclophosphamide)
What is the diagnostic lab for carcinoid syndrome?
increased 5-HIAA in the urine
What is the rule of 1/3s for carcinoid syndrome?
1/3 metastasize
1/3 present with 2nd malignancy
1/3 multiple
Carcinoid syndrome is derived from what type of cells?
Neuroendocrine cells of the GI tract
What is the treatment for carcinoid syndrome?
Octreotide
carcinoid syndrome is the most common tumor of which organ?
appendix
What is the sx of Zollinger-Ellison syndrome?
recurrent ulcers
Zollinger-Ellison is associaed with what other comorbidity?
MEN Type I
What is the mechanism of sulfonylureas in the treatment of DM?
close K channels in B-cell membrane -> depolarization -> Ca influx into cell triggers insulin release
Sulfonylueas have what suffix?
-ide (tolbutamide, glyburide, Glipizide)
Orlistat inhibits fat metabolism via what mechanism?
inhibits pancreatic lipases
What are the side fx of orlistat?
steatorrhea, GI discomfort, loss of fat soluble vitamins, headache
What is the MOA of sibutramine (obesity management)?
sympathomimetic serotonin and NE reuptake inhibitor
Whic drugs, used in the tx of hyperthyroidism inhibit the organification and coupling of TH synthesis
Propylthiouracil, methimazole
What are the side fx of Propylthiouracil and methimazole?
skin rash, agranulocytosis, aplastic anemia
Which drug is used to stimulate labor, uterine contractions?
Oxytocin
Desmopressing (ADH) is used for the treatment of what disease?
Central DI
What is the MOA of glucocorticoids?
decreases the production of leukotrienes and PGs by inhibiting phospholipase A2 and expression of COX2
Glucocorticoids are used to treat what conditions?
Addison's, inflammation, immune suppression, asthma