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240 Cards in this Set

  • Front
  • Back
what is the most common tumor affecting the hypothalamus?
pituitary adenoma
What hypothalamic dysfunction in boys causes precocious puberty?
midline hamartoma
When does true precocious puberty begin? precocious puberty that results from outside the CNS is called?
1) before 9 years in boys and 8 years in girls from midline hamartoma
2) pseudo-precocious puberty
What is Kallmanns syndrome?
decreased or absent production of GnRH
What is above the quadrigeminal plate in the brain?
pineal gland
What are the majority of pineal tumors? What clinical findings are there?
1) germ cell tumors, a few are teratomas
2) paralysis of upward gaze (parinauds syndrome), compression of 3rd ventricle and hydrocephalus
for hypopituitarism how much of gland must be destroyed? Is this primary or secondary hypopituitarism?
primary must lose a least 75%
What is secondary hypopituitarism?
loss of hypothalamic releasing hormones
What is the most common cause of hypopituitarism? What is it associated with?
1) nonfuctioning pituitary adenoma
2) MEN 1
What is present in MEN1?
pituitary adenoma, hyperparathyroidism, pancreatic tumor like zollinger-ellison syndrome or insulinoma
Craniopharyngioma causing hypopituitarism is most common in? What is it?
1) children
2) pituitary tumor derived from Rathkes pouch
Does a pituitary adenoma or a craniopharyngioma typically cause by temporal hemianopia? Which one may cause central diabetes insipidus?
craniopharygnioma
What occurs in Sheehans postpartum necrosis?
hypovolemic shock to pituitary causes infarct, stop lactating, sudden onset
What cell type during pregnancy increases the pituitary size?
lactotrope
What is pituitary apoplexy?
sudden loss pituitary function most often from hemorrhage or infarct of preexisting adenoma
lymphocytic hypophositis is cuased by what and occurs in what group? When does it occur?
female dominant autoimmune destruction of pituitary occurring after pregnancy
What is primary empty sella turcica? Who is it seen in?
1) defect above pituitary allows subarachnoid space to extend into sella and fill with CSF. pressure forces pituitary up
2) often seen in obese people with high BP
What causes secondary empty sella turcica?
radiation, trauma, surgery
How can lack of gonadotropes be distinguished from lack of hypothalamic function?
GnRH test: increase LH/FSH if hypothalamus and no increase if pituitary
What are characteristics of adults that do not have GH?
hypoglycemia, loss of muscle mass, increased adipose around waist
How can GH be tested?
Arginine and sleep stimulation tests: no increase in GH or IGF-1, but if normal they increase around 5am
What is secondary hypothyroidism? How is it checked?
1) lack of TSH
2) measure TSH after TRH administration
What causes secondary hypocortisolsim? what are features? How is it tested?
1) lack of ACTH
2) hypoglycemia, mild SIADH because cortisol suppresses ADH
3) short ACTH will not increase cortisol but long ACTH adminsitration will
What is the most common anterior pituitary tumor? what else may be secreted with it?
1) prolactinoma a benign
adenoma
2) GH
What are clinical findings in women and men with prolactinoma?
1) women have secondary amenorrhea because PRL inhibits GnRH, galactorrhea
2) Men have impotence and headache
Do men or woman tend to have a headache with prolactinoma?
men becuase they are larger
what is an example of a dopamine analogue?
bromocriptine and cabergoline
What commonly causes death in acromegaly?
cardiomyopathy

macroglossia
How can GH adenoma be tested for?
administer glucose and if GH and IGF1 do not fall then positive test
Is a GH adenoma associated with hyper/hypoglycemia?
hyperglycemia from increased gluconeogenesis
Why do acromegalics have hypertension?
they retain sodium by having increased GH and insulin
How are GH adenomas treated without surgery?
somatostatin and dopamine analogues, GH receptor antagonists
What regulates TSH?
negative feedback from FREE T4 AND T3 not bound T4 and T3
A woman that is pregnant, taking birth control or hormone replacement will have what effect on thyroid? What happens to free T4?
1) estrogen increases TBG which increases total serum T4
2) it is normal with no thyrotoxicosis or TSH elevation
What decreases TBG synthesis?
anabolic steroids, nephrotic syndrome
Why do acromegalics have hypertension?
they retain sodium by having increased GH and insulin
How are GH adenomas treated without surgery?
somatostatin and dopamine analogues, GH receptor antagonists
What regulates TSH?
negative feedback from FREE T4 AND T3 not bound T4 and T3
A woman that is pregnant, taking birth control or hormone replacement will have what effect on thyroid? What happens to free T4?
1) estrogen increases TBG which increases total serum T4
2) it is normal with no thyrotoxicosis or TSH elevation
What decreases TBG synthesis?
anabolic steroids, nephrotic syndrome
When TBG goes down does hypothyroid occur?
no because free T4 and TSH are normal
what conditions have elevated free T4? decreased free T4?
1) graves and thyroiditis
2) hypothyroidism
what is the best overall screening for thyroid dysfunction?
serum TSH
When is TSH increased? Decreased?
1) primary hypothyroidism
2) thyrotoxicosis, hypopituitarism (seconary), hypothalamic dysfunction (tertiary)
decreased uptake of 131 idodine indicates what?
1) patient taking thyroid hormone
2) acute/subacute/chronic thyroiditis
131 iodine can be used to evaluate nodules. What are examples of hot and cold nodules?
1) hot: toxic nodular goiter
2) cold: cyst, adenoma, cancer
what is a marker for thyroid cancer?
TBG
What is a lingual thyroid? What are clinical features? how is the lesion treated?
1) failed descent of thyroid anlage from base of tongue
2) dysphagia and mass lesion
3) 131 iodine
Note they are usually hypofunctional
A cystic mass that is midline and close to the hyoid bone is likely?
thyroglossal duct cyst
What is the cause of acute thyroiditis?
S areus
someone presents with fever, tender thyroid gland with painful cervical adenopathy. Which type of thyroiditis do they have?
acute from bacterial infection. If S. areus treat with penicillin or ampicillin
What is a difference on presentation between bacterial and viral infection of the thyroid?
in bacterial there is prominent painful lymphadenopathy where this does not occur with viral
All causes of thyroiditis are associated with thyrotoxicosis initially except?
reidels
subacute granulomatous thyroiditis is caused by? who does it occur in? how is it distinguished from acute thyroiditis on physical exam? How is it treated?
1) virus (coxsackievirus, mumps)
2) women 40-50
3) no cervical lymphadenopathy but very painful thyroid gland whereas bacteria cause tender thyroid
4) self-limited no treatment
Microscopy of a subacute granulomatous thyroiditis would reveal?
multinucleated giant cells
What often precedes subacute granulomatous thyroiditis?
URI
Hashimotos thyroiditis is an autoimmune disease. What people is it common in? What are the genetic associations?
1) women, turners, downs, and kleinfelters syndrome
2) HLA-Dr3 and Dr5
What type of hypersensitivity is hasimotos?
Type IV initially causes thyrotoxicosis then Type II IgG autoantibodies block TSH receptor causing hypothyroid
What autoimmune antibodies are present but have no causal role in hasimotos?
antimicrosomal and thryroglobulin antibodies
Gross and microscopic appearance of hashimotos shows?
enlarged gray gland with lymphocytic infiltrate
what is the most common cause of hypothyroidism? Is there a risk factor associated with it?
1) hashimotos
2) malignant B cell lymphoma
What is reidels thyroiditis?
fibrous replacement of gland that can extend to surrounding tissue
A person with thyroid dysfunction and tracheal obstruction likely has?
reidels
What is the treatment for riedels?
corticosteroids then tamoxifen
What induces subacute painless lymphocytic thyroiditis? How is it distinguished from acute thyroidits microscopically? is the gland enlarged or shrunk? How is it treated`
1) postpartum autoimmune
2) gland does not have germinal follicles
3) gland is enlarged and painless
4) treat with levothyroxine
What drugs can cause hypothyroidism?
amioderone, lithium, sulfonamides, phenylbutazone
Cretinism is associated with? What is the most common cause?
1) severe retardation and increased weight with short stature
2) hypothyroid of mother before fetal thyroid develops
What are common finding in adult hypopiutiarism?
1) proximal muscle myopathy
2) increased CK
3) weight gain
4) dry brittle hair with loss of out third of eye brow
5) yellow skin from formation of retinoic acid
6) periorbital puffiness and hoarseness from hyluronic acid and chondroitin sulfate deposition
7) fatigue, cold intolerance, constipation
8) menorrhagia
9) diastolic hypertension from retention of Na and H2O
10) dialated cardiomyopathy
what type of edema is myxedema?
nonpitting
What type of heart damage occurs in hypothyroidism?
DCM
Lab tests will show what in hypothyroidism?
1) low T4, high TSH
2) hypercholesteremia from low LDL receptors
3) antimicrosomal, antiperoxidase and antithyroglobulin antibodies if hashimotos
What is the myxedema coma? How is it treated?
1) progressive stupor, hypothermia, bradycardia, hypoventilation, hypoglycemia, hypocortisolism, SIADH
2) IV levothyroxine and corticosteroids
What is the difference between thyroxicosis and hyperthyroidism?
thyroxicosis is excess T4 regardless of cause and includes initial stages of thyroiditis and hyperthyroidism, whereas hyperthyroidism is only increased T4 synthesis
What is the most common cause of hyperthyroidism? Thyroxicosis?
Graves
Who is Graves mostly seen in? what is it associated with?
1) woman
2) associated with HLA-B8 and HLA-Dr3
Graves disease is autoimmune what causes it?
IgG antibodies directed against TSH receptor that activate it. Type II hypersensitivity
On gross exam how does the thyroid present? histologically?
1) symmetrically enlarged and nontender
2) scant colloid with papillary infoldings of glands
proptosis and exopthalmos are due to what?
deposition of GAGs and adipose tissue in orbital area
What characteristics of the extermities are seen in Graves?
1) pretibial myxedema
2) thryroid acropachy which is digital swelling and clubbing, nail separation from bed
apathetic hyperthyroidism is seen in what?
Graves in elderly, associated with atrial fib, and CHF, muscle weakness, apathy
How is plummers disease distinguished from Graves?
lacxk of exophthalmos and pretibial myxedema
plummers is also known as?
multinodular goiter diagnosed as hot on a 131 iodine scan
thyrotoxicosis has what common constitutional findings?
weight loss, fine hand tremor, heat intolerance, diarrhea, oligomenorrhea, lid stare
thyrotoxicosis has what common cardiac findings?
1) sinus tach
2) risk of atrial fib
3) systolic hypertension
4) high output failure from T3 increasing beta receptor
Note T3 has both inotropic and chronotropic effects
What effect does excess T3 have on reflexes and bone?
1) brisk reflexes
2) osteoporosis
What does a person have if they have thyrotoxicosis and decreased 131 iodine uptake?
thyroiditis
Blood work of someone with hyperthyroid shows?
hyperglycemia, hypocholesterolemia, hypercalcemia, absolute LYMPHCYTOSIS
How is Graves treated?
beta blockers, thionamides to decrease hormone synthesis
people predisposed to thyroid storm are?
1) inadequately treated graves
2) infection, trauma
3) iodine, pregnancy
What are the findings of someone with thyroid storm? How is it treated?
1) tachycardia
2) hyperplexia
3) shock from volume depletion from vomiting
4) coma
Treat with propylthiouracil and sympathetic blockers
What happens in euthyroid sick syndrome?
peripheral tissue outer ring deiodinase converts T4 to T3, but in ESS this enzyme in inactive and the inner ring deiodionase converts T4 to reverse T3 which is inactive
What are common blood work findings in euthyroid sick syndrome?
normal gland function but decreased T3, normal to decreased T4 and TSH and increased reverse T3
What are causes of nontoxic goiter?
1) iodine deficiency, cabbage, puberty, pregnancy
What is subacute thyroiditis also known as?
DeQuervians
in mulitnodular goiter is colloid scant or abundant?
abundant
nontoxic goiter is followed by?
multinodular goiter
What are complications of nontoxic goiter?
1) hemorrhage into cyst - sudden and painful gland enlargement
2) Compression of jugular vein - pembertons sign
3) primary hypothyroid
4) toxic nodular goiter - become hot
5) hoarseness
6) dyspnea
What is pembertons sign associated with?
nontoxic goiter compress jugular vein producing neck congestion
What does a hot nodule mean?
it is TSH independent
Are solitary thyroid nodules generally cold or hot?
cold
What causes solitary thyroid nodules? what is the difference between men, woman and children with thyroid nodule
1) majority are cysts, second are follicular adenoma, a few are malignant
2) men and children have a greater chance of malignancy
Are most solitary nodules euthyroid?
yes
What is the most common benign thyroid tumor? how is it characterized?
1) follicular adenoma
2) has a complete capsule surrounded by normal thyroid tissue, painless. it is a cold nodule
On microscopic exam how is a follicular adenoma characterized? What do 10% of adenomas progress to?
1) small packed follicles with compressed flat normal adjacent follicles
2) follicular carcinoma
What is the most common endocrine cancer? Is it more common in men or women?
1) papillary carcinoma
2) women in 2nd and 3rd decades
the most common thyroid cancer is?
papillary carcinoma
A biopsy of a thryroid gland shows multifocal involvement of papillary fronds intermixed with follicles and orphan annie nuclie. What disease is present? what other features are seen?
1) papillary carcinoma
2) psammoma bodies and lymphatic invasion with metastisis to cervical nodes and lungs
What thyroid cancer is the most common solitary cold nodule? Is it more common in men than women?
follicular carcinoma more common in women
What are gross and microscopic findings of follicular carcinoma?
encapsulated and/or invasive, has new blood vessels, spreads hematogenously
the familial type of medullary cancer occurs in 20% of cases. What condition is it associated with?
autosomal dominant MEN IIa/IIb
What is MEN IIa?
autosomal dominant condition with thyroid medullary carcinoma, hyperparathyroidism, pheochromocytoma
What is MEN IIb?
autosomal dominant condition with medullary carcinoma, mucosal neuromas (lips/tongue) pheochromocytomas
In medullary carcinoma where do tumors derive from? What is seen on microscopic exam? What tumor marker is used?
1) parafollicular C cells
2) C cells converted to amyloid that stain with Congo Red
3) calcitonin levels are elevated and Ca levels are down
In medullary carcinoma does the sporadic or familial type have a better prognosis? genetic screening in someone with familial type will have what marker?
1) familial
2) RET proto-oncogene
Anaplastic tumor of the thyroid is commonly seen in who? What are the risk factors? What is the prognonsis?
1) elderly women
2) multinodular goiter or follicular carcinoma
3) five year survival is only 5%
What might symptoms might a patient present with that would lead you to suspect anaplastic tumor of the thyroid?
dyspnea and dysphagia from invasive action tumor into esophagous and trachea
Where do the parathyroid glands derive from?
superior from 4th pharyngeal pouch and inferior from 3rd
What are the effects of PTH in kidney?
1) increase Ca reabsorption in DCT
2) Decrease bicarb reabsorption in PCT
3) decrease phosphorous reabsorption in PCT
4) inhibit 24-hydroxylase so the 1-alpha-hydroxylase is active to make calcitriol
What effect does hyperphosphatemia have on PTH?
increases PTH production so that phosphate is lost in urine
What suppresses PTH?
high Ca and low phosphates
What vitamin D reaction occurs in the skin?
1) 7-dehydrocholesterol is photoconverted to cholecalciferol
2) cholecalicerol converted to calcidiol in liver via 25-hydroxylase
3) calcidiol converted to calcitriol in PCT of kidney via 1-alpha-hydroxylase
What is the function of calcitriol? Include where Ca and phosphorous are reabsorbed, etc
attaches to nuclear receptors and increases Ca reabsorption in duodenum, increase phosphorous reabsorption in jejunem and ileum and activates osteoclasts
What is another name for calcitriol? calcidiol?
1) calcitriol = 1,25-(OH)2-D
2) calcidiol = 24,25-(OH)2-D
40% of serum Ca is bound where? What happens if the pH lowers? 47% of Ca is free in plasma. what is its function?
1) to albumin
2) albumin is negatively charged and at acidic pH they may protonate and decrease binding to Ca
3( metabolically active form that has negative feedback on PTH
How does serum Ca change when albumin decreases?
Ca decreases but the ionized level is normal
metabolic alkalosis has what effect on Ca? What happens to PTH? What is overall result?
increases negative binding sites on albumin so that bound Ca blood level increases but free Ca decreases. Total serum is normal initially then PTH increases to raise free Ca but cannot completely compensate so tetany occurs and
Why does tetany result in metabolic alkalosis? What are the clinical findings?
1) decreased free Ca lowers threshold for nerve and muscle firing so that smaller stimulus can initiate AP.
2) find carpopedal spasm (thumb flexed to palm) and chvosteks sign (facial twitching after tapping facial nerve)
What is the most common cause of hypoparathyroidism? What syndrome occurs when the 3rd and/or 4th pharyngeal pouches fail to descend? What is the most common cause of hypocalcemia in the hospital?
1) autoimmune
2) DiGeorge (also have absent thymus)
3) hypomagnesemia, Mg is cofactor for adenylate cyclase which is needed by PTH,
What can cause hypomagnesemia? What are clinical findings with hypomagnesemia?
1) diarrhea, aminoglycosides, diuretics and alcoholism
2) tetany, basal ganglia calcification from increased phorphorous driving Ca into brain tissue, and cataracts
If the Mg is low in the blood, what is the level of Ca, PTH and phosphate?
low Mg = low Ca, low PTH and high phosphate
List several causes of hypocalcemia that are not do to the parathyroid glands?
1) actue pancreatitis from fat calcification
2) hypovitaminosis D from lack of sunlight, celiacs, cirrhosis, chronic renal failure
3) pseudohypoparathyroidism where there is end organ resistance to PTH so that 1-alpha hydroxylase is low
4) rickets type I and type II
what is rickets type I? type II?
1) autosomal recessive absent 1-alpha hydroxylase
2) autosomal recessive absent receptors for calcitriol
What disease is characterized by mental retardation, basal ganglia calcification, short fourth and fifth metacarpals?
pseudohypoparathyroidism (knuckle-knuckle-dimple-dimple sign)
Hyperparathyroidism is associated with what endocrine disorders?
MEN I and MEN IIa
What is the most common cause of hyperparathyroidism
benign adenoma... remaining glands atrophy... right inferior parathyroid mostly involved
What is seen in primary hyperplasia of the parathyroid?
all four glands enlarge, usually a chief cell hyperplasia, though clear cell hyperplasia (wasserhelli cell hyperplasia)
What are common clinical findings with hyperparathyroidism with the kidney?
1) calcium stones (most common presenting symptom)
2) nephrocalcinosis - polyuria and renal failure
What are common clinical findings with hyperparathyroidism with the GI?
1) PUD - Ca stimulates gastrin release
2) acute pancreatitis - Ca activates phospholipase
3) constipation
What are common clinical findings with hyperparathyroidism with bones and joints?
1) osteitis fibrosa cystica - cystic hemorrhagic bone lesion from increased osteoclasts (commonly in jaw)
2) salt and pepper appearance of skull
3) chondrocalcinosis (pseudogout)
4) bone resorption in phalanges and tooth sockets
What are common clinical findings with hyperparathyroidism in the eyes and with blood pressure?
1) causes diastolic hypertension
2) band keratopathy in limbus of eye
3) pyschosis, confusion, anxiety and coma
Does hyperparathyroidism results metablic alkalosis or acidosis? What occurs in the kidney?
1) normal anion gap metabolic acidosis - due to decreased proximal reabsorption of bicarb
2) type II renal tubular acidosis
If someone has a ratio of Cl:phosphorous of 26 do they have hyperparathyroidism?
no has to be greater than 33 and if less than 29 it is not hyperparathyroidism
What does an EKG show in someone with hyperparathyroidism? How can the hypercalcemia be treated?
1) shortened QT interval
2) IV furosemide
How can hypercalcemia of malignancy be differentiated from primary hyperparathyroidism
PTH will be high in hyperparathyroidism but low in the malignancy
What is the most common form of malignancy induced hypercalcemia?
bone metastisis with increased osteoclasts producing lytic lesions in bone
What types of tumors can secrete PTH related protein?
1) squamous cell carcinoma of lung and renal cell carcinoma
2) osteoclast activity WITHOUT lytic lesions in bone
How could an ectopic tumor producing PTH related peptide be distinguished from other malignancy induced hypercalcemias?
because it does not produce lytic lesions in bone
multple myeloma causes increase in what blood electrolyte? why?
Ca is increased because of a tumor malignant plasma cells releasing osteoclast activating factor. Note that there are lytic bone lesions
What is familial hypocalciuric hypercalcemia?
1) autosomal dominant
2) altered set point for calcium sensing in renal tubule and parathyroid so that more calcium is reabsorbed and PTH is high
How does sarcoidosis contribute to hypercalcemia?
macrophages in granulomas produce 1-alpha hydroxylase
Are thiazide associated with elevated or depleted blood calcium?
elevated becuase the cause volume depletion and reabsorption of Ca
what is secondary hyperparathyroidism? what can develop?
1) hyperplasia of glands from low Ca in blood. Could be from renal failure
2) can lead to tertiary hyperparathyroidism where glands are autonomous
what are the PTH and Ca levels in hypoalbuminemia? In secondary hyperparathyroidism? In respiratory alkalosis? `
1) PTH norm and Ca low
2) PTH high and Ca low
3) PTH high and Ca norm
What effect does extrarenal lack of vit D have on phosphorous? Insulin does what to phophate?
1) it decreases phosphorous
2) decreases phosphorous because glucose uptake requires phorphylation of glucose
Blood alkalosis has what effect on phosphorous?
decreases it by alkalosis activates phosphofructokinase increasing glucose phosphorylation
What causes vitamin D resistant rickets?
x linked dominant defect in GI and renal reabsorption of phosphorus
What are common findings with hypophosphatemia?
1) muscle weakness/paralysis, rahdomyalsis from decreased ATP synthesis
2) RBC lysis
3) osteomalacia
hyperphosphatemia is seen in?
1) renal failure
2) children NORMAL
3) primary hypoparathyroidism
4) pseudohypoparathyroidism
Angiotensin II activates what enzyme in glomerulosa?
1) 18-hydroxylase
What are the metabolic products of epinephrine and norepinephrine? What is the metabolic product of dopamine?
1) metanephrine and vanillylmandelic acid
2) homovanillic acid
What causes Waterhouse-Friderichsen syndrome?
1) septicemia from N meningitidis
2) develop DIC and bilaterally adrenal hemorrhage
chronic adrenal insufficiency is called? What causes it?
1) addisons disease
2) caused by autoimmune (80%), miliary TB, androgenital syndrome, metasis from lung, AIDS
A person presents with weakness, hypotension, buccucal mucosa hyperpigmentation and darkened skin in general. What might they have?
addisions disease, elevated ACTH darkens skin
When given the metyrapone test what results in someone with addisons?
1) increased ACTH but no increase in 11-deoxycortisol
A blood chem of someone with Addissons shows what? A WBC would show?
1) hyponatremia, hyperkalemia, and metabolic acidosis (normal anion gap)
2) eosinophilia, lymphocytosis, NEUTROPENIA from low cortisol
adrenogenital syndrome is also known as? What happens?
1) CAH
2) autosomal recessive deficiency in cortisol synthesis leads to excess 17-KS, testosterone and DHT, but elevated ACTH
A child that grows rapidly but as an adult has a small stature may have what genetic condition?
CAH increased sex hormones causes rapid growth in children but then epiphyses fuse early
When do males develop psuedohermaphroditism?
lack normal male genital because development requires DHT
What is the most common CAH enzyme deficiency? What is elevated?
1) 21-hydroxylase
2) increase in 17-KS, testosterone, DHT
3) decrease mineralcorticoids
4) decrease in cortisol
What is nonclassic 21-hydroxylase deficiency? What occurs in females and males?
1) impaired cortisol production but not mineralcorticoid production (Na sparing)
2) virilization in females and precocious puberty in males
What is elevated in 11-hydroxylase deficiency?
1) increased 17-ketosteroids, testosterone, DHT
2) increased 17-hydroxyprogesterone
3) 11-deoxycortisol
4) increased mineralcorticoids
What does a loss of 17-hydroxylase lead to?
1) decreased keto-steroids, testosterone and DHT
2) decreased 17-hydroxyprogesterone
3) decreased 11-deoxycortisol and cortisol
4) increased mineralcorticoids
How do you treat someone with 21 hydroxylase deficiency?
glucocorticoids and mineralcorticoids
What is the most common cause of Cushings disease?
corticosteroid therapy
What is the cause of cushings disease?
cushings disease is pituitary tumor whereas syndrome incorporates any cause of excess cortisol
Ectopic cushing syndrome commonly occurs where?
ACTH production via small cell carcinoma of the lung, less commonly thymus and thyroid
What type of hypertension develops in cushings syndrome?
diastolic hypertension from increase in weak mineralcorticoids and glucocorticoids but not aldosterone
Why do purple stria develop in cushings syndrome? What commonly occurs to bone?
1) cortisol weakens collagen and causes rupture of blood vessels into stretch marks
2) osteoporosis
Low dose dexamethasone is good at suppressing a tumor causing cushings syndrome in which part of the body?
no part... it cant suppress it anywhere
High dose dexamethasone can suppress what condition in cushings syndrome?
pituitary tumor but not other tumors
What is seen on blood chem in someone with Cushings?
hyperglycemia, hypokalemia, metabolic alkalosis
Why does hypokalemia occur in cushings syndrome?
hypokalemia occurs because of the increased weak mineralcorticoids. metabolic alkalosis also occurs
Bilateral adrenalectomy with preexisting pituitary adenoma causes what? what are the features?
1) Nelsons syndrome
2) headache and diffuse hyperpigmentation from ACTH production in tumor
The ACTH in a pituitary tumor vs that of an ectopic tumor is what?
high to normal in the pituitary tumor and very high in the ectopic tumor
Primary aldosteronism is called?
1) Conns syndrome
2) caused by benign adenoma of glomerulosa
3) have diastolic hypertension, muscle weakness and tetany from metabolic alkalosis
What are lab findings in Conns syndrome? Is renin high or low?
1) hypernatremia, hypokalemia, metabolic alkalosis
2) renin is low
Secondary aldosteronism is associated with what?
decreased CO, so kidneys try to retain water, renin increased
Pheochromocytomas generally arise unilaterally in adrenal medulla but can also arise in other areas of the body. What are they?
bladder, organ of Zuckerkandl near bifurcation of aorta and posterior mediastinum
What enzyme converts norepi to epi? Where in the body is this enzyme found? Can a pheochromocytoma occur outside of these sites?
1) N-methyltransferase
2) found in adrenal medula and organ of Zuckerkandl
3) yes but they can only produce norepi
What is pheochromocytoma associated with?
1) neurofibromatosis
2) MEN IIa and IIb (mutation of RET proto-oncogene), Von Hippel-lindau disease (mutation of VHL gene)
Characteristics on exam of a pheochromocytoma are?
adrenal medulla is brown, hemorrhagic and often necrotic
What are unique findings with a pheochromocytoma?
1) palpitations
2) paroxysmal hypertension
3) anxiety
4) drenching sweats
5) headache
6) orthostatic hypotension
7) chest pain
8) ILEUS
Lab tests for someone with pheochromocytoma are?
1) increased plasma metanephrines (best test)
2) increased plasma normetanephrine
3) increased 24-hr urine metanephrine (100% sensivity)
4) increased 24-hr urine VMA
What effect does clonidine have on a pheochromocytoma? What effect does it have on neutrophil levels?
1)it is unable to suppress norepi and is therefore a good evaluator
2) nuetrophil leukocytosis via inhibition of adhesion molecules
Someone with pheochromocytoma and be stabilized preoperatively with what drugs?
1) phenoxybenzamine
2) beta-blocker
3) metyrosine
What is neuroblastoma and in what group does it occur in?
malignant neoplasm of postganglionic sympathetic neurons occurring in children under 5
What oncogene is associated with neuroblastoma?
N-MYC
Opsoclonus-myoclonus syndrome is seen it what? What is it?
1) neuroblastoma
2) paraneoplastic syndrome, myclonic jerks of extremities, chaotic eye movements
Where does neuroblastoma commonly metastize to?
skin and bone... normal this occurs before diagnosis
What is the prognosis of neuroblastoma dependent on?
age if less than 1 year there is a better prognosis
How is the neuroblastoma characterized?
"small cell" tumor composed of malignant neuroblasts, homer-wright rosettes (neuroblasts located around central space)
What are clinical findings of pheochromocytoma?
palpable abdominal mass, diastolic hypertension, increased urine VMA and HVA
A person presents with hyperglycemia and necrolytic migratory erythema. The hyperglycemia responds to octreotide. What does the person have?
malignant glucogonoma
What is the most common islet cell tumor? What is it associated with? How is it treated?
1) Insulinoma associated with MEN I
2) streptozotocin
How can an insuloma be distinguished from over use of exogenous insulin?
by measuring C peptide
A patient has achlorhydria, cholelithiasis and steatorrhea, and diabetes mellitus. What is the underlying problem? How is it treated?
malignant somatostatinoma treated with streptozotocin
What is found in someone with a vipoma?
secretory diarrhea and achlorhydria. hypokalemia with normal anion gap metabolic acidosis (loss of bicarb in stool)
What is zollinger ellison associated with?
MEN I
What group is diabetes increasing in?
native americans
What conditions is diabetes the leading cause of in the US?
1) legal blindness
2) peripheral neuropathy
3) chronic renal failure
4) below the knee amputation
What are drugs that can cause diabetes? What genetic diseases are associated with diabetes?
1) glucocorticoids, pentamide, thiazides and alpha interferon
2) hemochromatosis
Acanthosis nigricans is a phenotypic marker of what?
insulin receptor deficiency. Note that Acanthosis nigricans is a brown to black, poorly defined, velvety hyperpigmentation of the skin. It is usually found in body folds, such as the posterior and lateral folds of the neck, the axilla, groin, umbilicus, forehead
Which viruses are associated with diabetes?
mumps and CMV
What is maturity onset diabetes caused by? when does it present? In general what is seen?
1) autosomal dominant disorder of genes like glucokinase
2) usually less than 25
3) NOT obese, do have hyperglycemia from impaired release of insulin, may develop type II diabetes, treat with hypoglycemic agents
In metabolic syndrome what causes insulin resistance?
genetic deficiency that is exacerbated by obesity
metabolic syndrome is commonly associated with what? What are other less common associations?
1) polycystic ovary syndrome
2) acanthosis nigricans
3) Alzheimers disease
Clinical and lab findings in someone with metabolic syndrome are?
hyperinsulinemia, increased VLDL from increased triglyceride syn, hypertension because insulin retains Na in kidney, CAD
adipose tissue has what effect on insulin receptors? What is the definition of metabolic syndrome?
1) decreases
2) waistline >40 in men, >35 in women
HDL <40 in men, <50 in women
fasting glucose >110
In type I diabetes what type of hypersensitivity occurs? What condition is type II diabetes associated with?
1) Type IV and type II against insulin and beta cells
2) alzheimers
On microscopic exam of the islets what do beta cells have in a person with type II diabetes? What type of coma do type II diabetics succumb to?
1) amyloid stains with congo red
2) hyperosmotic nonketotic coma
What are a few signs that someone has Type II diabetes?
1) recurrent blurry vision from dysregulated sorbitol production
2) recurrent bacterial and candidal infections
What is an early test finding in someone with type II diabetes?
reactive hypoglycemia where too much insulin is released for the amount of glucose given
What type of arteriosclerosis occurs in diabetes?
hyaline arteriosclerosis
What occurs in both proliferative and nonproliferative diabetes retinopathy?
1) nonproliferative: flame hemorrhages and microaneurysms, exudate
2) proliferative: neovascularization, retinal detachment
A patient comes in complaining of burning feet and pressure ulcers on feet. What does he have and how do you treat these symptoms?
1) diabetes
2) duloxetine (selective serotonin and NE reuptake inhibitor), topical capsaicin and amitptyline
Which cranial nerves are commonly involved in diabetes?
CN III, IV and VI
What common infections are seen in diabetes?
candida, malignant external otitis media (P aeurginosa), rhinocerebral mucormycosis, cutaneous S aureus infections
Skin disorders present in diabetes?
1) necrobiosis lipoidica diabeticorum (yellow plaques on anterior surface of legs and dorsum of ankles
2) lipoatrophy: atrophy at insulin injection sites from impure insulin
3) lipohypertrophy: increased fat synthesis at insulin injection sites
What enzyme is responsible for the vision problems when glucose is elevated? What other problems does this same pathway lead to?
1) aldose reductase converts glucose to sorbitol
2) schwann cells swell and are damaged causing peripheral neuropathy
What is occurring in diabetic microangiopathy?
increased synthesis of type IV collagen in basement membrane and mesangium
What is the sympathetic response to hypoglycemia? parasympathetic?
1) sweating, tachy, palpitations, tremulousness
2) nausea and hunger
Why is beta oxidation increased in diabetes as far as what is occurring in mitochondria?
decreased malonyl-CoA to inhibit carnitine acyltransferase
What type of hyperlipoproteinemia occurs in diabetes?
type V
hyperchylomicronemia syndrome is seen in what condition and is the result of what?
increased chylomicrons from lack of insulin activity in diabetes. leads to eruptive xanthomas in skin
Will hyper/hypokalemia result in diabetes? Is the anion gap normal or elevated? what type of azotemia occurs?
1) hyperkalemia because H+ moves into cells in exchange for K+
2) elevated because of ketoacidosis and lactic acidosis
3) prerenal from volume depletion
What hormones are involved in producing gestational diabetes? When should screening begin?
1) human placental lactogen, cortisol, progesterone
2) 24 to 28 weeks gestation
With gestational diabetes what are the risks to the newborn?
1) macrosomia- increased release of fetal insulin causes increased fat and muscle
2) RDS - insulin inhibits surfactant
3) possible open neural tube defects
4) neonatal HYPOGLYCEMIA
What causes in type I polyglandular syndrome? When is onset? What are the findings?
1) autosomal recessive
2) mean age is 12
3) addisons, primary hypoparathyroidism, mucocutaneous candidiasis
What causes in type II polyglandular syndrome? When is onset? What are the findings?
1) autosomal dominant HLA-DR3 and DR4 relation
2) 24 yo
3) addisons, hashimotos thyroiditis, type I diabetes
hypoglycemia occurs at what level?
1) <50mg/dL