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240 Cards in this Set
- Front
- Back
what is the most common tumor affecting the hypothalamus?
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pituitary adenoma
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What hypothalamic dysfunction in boys causes precocious puberty?
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midline hamartoma
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When does true precocious puberty begin? precocious puberty that results from outside the CNS is called?
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1) before 9 years in boys and 8 years in girls from midline hamartoma
2) pseudo-precocious puberty |
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What is Kallmanns syndrome?
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decreased or absent production of GnRH
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What is above the quadrigeminal plate in the brain?
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pineal gland
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What are the majority of pineal tumors? What clinical findings are there?
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1) germ cell tumors, a few are teratomas
2) paralysis of upward gaze (parinauds syndrome), compression of 3rd ventricle and hydrocephalus |
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for hypopituitarism how much of gland must be destroyed? Is this primary or secondary hypopituitarism?
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primary must lose a least 75%
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What is secondary hypopituitarism?
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loss of hypothalamic releasing hormones
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What is the most common cause of hypopituitarism? What is it associated with?
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1) nonfuctioning pituitary adenoma
2) MEN 1 |
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What is present in MEN1?
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pituitary adenoma, hyperparathyroidism, pancreatic tumor like zollinger-ellison syndrome or insulinoma
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Craniopharyngioma causing hypopituitarism is most common in? What is it?
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1) children
2) pituitary tumor derived from Rathkes pouch |
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Does a pituitary adenoma or a craniopharyngioma typically cause by temporal hemianopia? Which one may cause central diabetes insipidus?
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craniopharygnioma
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What occurs in Sheehans postpartum necrosis?
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hypovolemic shock to pituitary causes infarct, stop lactating, sudden onset
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What cell type during pregnancy increases the pituitary size?
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lactotrope
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What is pituitary apoplexy?
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sudden loss pituitary function most often from hemorrhage or infarct of preexisting adenoma
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lymphocytic hypophositis is cuased by what and occurs in what group? When does it occur?
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female dominant autoimmune destruction of pituitary occurring after pregnancy
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What is primary empty sella turcica? Who is it seen in?
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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 |
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What causes secondary empty sella turcica?
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radiation, trauma, surgery
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How can lack of gonadotropes be distinguished from lack of hypothalamic function?
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GnRH test: increase LH/FSH if hypothalamus and no increase if pituitary
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What are characteristics of adults that do not have GH?
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hypoglycemia, loss of muscle mass, increased adipose around waist
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How can GH be tested?
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Arginine and sleep stimulation tests: no increase in GH or IGF-1, but if normal they increase around 5am
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What is secondary hypothyroidism? How is it checked?
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1) lack of TSH
2) measure TSH after TRH administration |
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What causes secondary hypocortisolsim? what are features? How is it tested?
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1) lack of ACTH
2) hypoglycemia, mild SIADH because cortisol suppresses ADH 3) short ACTH will not increase cortisol but long ACTH adminsitration will |
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What is the most common anterior pituitary tumor? what else may be secreted with it?
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1) prolactinoma a benign
adenoma 2) GH |
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What are clinical findings in women and men with prolactinoma?
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1) women have secondary amenorrhea because PRL inhibits GnRH, galactorrhea
2) Men have impotence and headache |
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Do men or woman tend to have a headache with prolactinoma?
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men becuase they are larger
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what is an example of a dopamine analogue?
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bromocriptine and cabergoline
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What commonly causes death in acromegaly?
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cardiomyopathy
macroglossia |
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How can GH adenoma be tested for?
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administer glucose and if GH and IGF1 do not fall then positive test
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Is a GH adenoma associated with hyper/hypoglycemia?
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hyperglycemia from increased gluconeogenesis
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Why do acromegalics have hypertension?
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they retain sodium by having increased GH and insulin
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How are GH adenomas treated without surgery?
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somatostatin and dopamine analogues, GH receptor antagonists
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What regulates TSH?
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negative feedback from FREE T4 AND T3 not bound T4 and T3
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A woman that is pregnant, taking birth control or hormone replacement will have what effect on thyroid? What happens to free T4?
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1) estrogen increases TBG which increases total serum T4
2) it is normal with no thyrotoxicosis or TSH elevation |
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What decreases TBG synthesis?
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anabolic steroids, nephrotic syndrome
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Why do acromegalics have hypertension?
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they retain sodium by having increased GH and insulin
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How are GH adenomas treated without surgery?
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somatostatin and dopamine analogues, GH receptor antagonists
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What regulates TSH?
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negative feedback from FREE T4 AND T3 not bound T4 and T3
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A woman that is pregnant, taking birth control or hormone replacement will have what effect on thyroid? What happens to free T4?
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1) estrogen increases TBG which increases total serum T4
2) it is normal with no thyrotoxicosis or TSH elevation |
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What decreases TBG synthesis?
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anabolic steroids, nephrotic syndrome
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When TBG goes down does hypothyroid occur?
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no because free T4 and TSH are normal
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what conditions have elevated free T4? decreased free T4?
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1) graves and thyroiditis
2) hypothyroidism |
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what is the best overall screening for thyroid dysfunction?
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serum TSH
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When is TSH increased? Decreased?
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1) primary hypothyroidism
2) thyrotoxicosis, hypopituitarism (seconary), hypothalamic dysfunction (tertiary) |
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decreased uptake of 131 idodine indicates what?
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1) patient taking thyroid hormone
2) acute/subacute/chronic thyroiditis |
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131 iodine can be used to evaluate nodules. What are examples of hot and cold nodules?
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1) hot: toxic nodular goiter
2) cold: cyst, adenoma, cancer |
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what is a marker for thyroid cancer?
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TBG
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What is a lingual thyroid? What are clinical features? how is the lesion treated?
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1) failed descent of thyroid anlage from base of tongue
2) dysphagia and mass lesion 3) 131 iodine Note they are usually hypofunctional |
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A cystic mass that is midline and close to the hyoid bone is likely?
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thyroglossal duct cyst
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What is the cause of acute thyroiditis?
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S areus
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someone presents with fever, tender thyroid gland with painful cervical adenopathy. Which type of thyroiditis do they have?
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acute from bacterial infection. If S. areus treat with penicillin or ampicillin
|
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What is a difference on presentation between bacterial and viral infection of the thyroid?
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in bacterial there is prominent painful lymphadenopathy where this does not occur with viral
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All causes of thyroiditis are associated with thyrotoxicosis initially except?
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reidels
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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?
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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 |
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Microscopy of a subacute granulomatous thyroiditis would reveal?
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multinucleated giant cells
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What often precedes subacute granulomatous thyroiditis?
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URI
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Hashimotos thyroiditis is an autoimmune disease. What people is it common in? What are the genetic associations?
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1) women, turners, downs, and kleinfelters syndrome
2) HLA-Dr3 and Dr5 |
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What type of hypersensitivity is hasimotos?
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Type IV initially causes thyrotoxicosis then Type II IgG autoantibodies block TSH receptor causing hypothyroid
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What autoimmune antibodies are present but have no causal role in hasimotos?
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antimicrosomal and thryroglobulin antibodies
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Gross and microscopic appearance of hashimotos shows?
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enlarged gray gland with lymphocytic infiltrate
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what is the most common cause of hypothyroidism? Is there a risk factor associated with it?
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1) hashimotos
2) malignant B cell lymphoma |
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What is reidels thyroiditis?
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fibrous replacement of gland that can extend to surrounding tissue
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A person with thyroid dysfunction and tracheal obstruction likely has?
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reidels
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What is the treatment for riedels?
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corticosteroids then tamoxifen
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What induces subacute painless lymphocytic thyroiditis? How is it distinguished from acute thyroidits microscopically? is the gland enlarged or shrunk? How is it treated`
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1) postpartum autoimmune
2) gland does not have germinal follicles 3) gland is enlarged and painless 4) treat with levothyroxine |
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What drugs can cause hypothyroidism?
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amioderone, lithium, sulfonamides, phenylbutazone
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Cretinism is associated with? What is the most common cause?
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1) severe retardation and increased weight with short stature
2) hypothyroid of mother before fetal thyroid develops |
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What are common finding in adult hypopiutiarism?
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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 |
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what type of edema is myxedema?
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nonpitting
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What type of heart damage occurs in hypothyroidism?
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DCM
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Lab tests will show what in hypothyroidism?
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1) low T4, high TSH
2) hypercholesteremia from low LDL receptors 3) antimicrosomal, antiperoxidase and antithyroglobulin antibodies if hashimotos |
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What is the myxedema coma? How is it treated?
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1) progressive stupor, hypothermia, bradycardia, hypoventilation, hypoglycemia, hypocortisolism, SIADH
2) IV levothyroxine and corticosteroids |
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What is the difference between thyroxicosis and hyperthyroidism?
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thyroxicosis is excess T4 regardless of cause and includes initial stages of thyroiditis and hyperthyroidism, whereas hyperthyroidism is only increased T4 synthesis
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What is the most common cause of hyperthyroidism? Thyroxicosis?
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Graves
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Who is Graves mostly seen in? what is it associated with?
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1) woman
2) associated with HLA-B8 and HLA-Dr3 |
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Graves disease is autoimmune what causes it?
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IgG antibodies directed against TSH receptor that activate it. Type II hypersensitivity
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On gross exam how does the thyroid present? histologically?
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1) symmetrically enlarged and nontender
2) scant colloid with papillary infoldings of glands |
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proptosis and exopthalmos are due to what?
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deposition of GAGs and adipose tissue in orbital area
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What characteristics of the extermities are seen in Graves?
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1) pretibial myxedema
2) thryroid acropachy which is digital swelling and clubbing, nail separation from bed |
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apathetic hyperthyroidism is seen in what?
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Graves in elderly, associated with atrial fib, and CHF, muscle weakness, apathy
|
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How is plummers disease distinguished from Graves?
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lacxk of exophthalmos and pretibial myxedema
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plummers is also known as?
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multinodular goiter diagnosed as hot on a 131 iodine scan
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thyrotoxicosis has what common constitutional findings?
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weight loss, fine hand tremor, heat intolerance, diarrhea, oligomenorrhea, lid stare
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thyrotoxicosis has what common cardiac findings?
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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 |
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What effect does excess T3 have on reflexes and bone?
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1) brisk reflexes
2) osteoporosis |
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What does a person have if they have thyrotoxicosis and decreased 131 iodine uptake?
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thyroiditis
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Blood work of someone with hyperthyroid shows?
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hyperglycemia, hypocholesterolemia, hypercalcemia, absolute LYMPHCYTOSIS
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How is Graves treated?
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beta blockers, thionamides to decrease hormone synthesis
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people predisposed to thyroid storm are?
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1) inadequately treated graves
2) infection, trauma 3) iodine, pregnancy |
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What are the findings of someone with thyroid storm? How is it treated?
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1) tachycardia
2) hyperplexia 3) shock from volume depletion from vomiting 4) coma Treat with propylthiouracil and sympathetic blockers |
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What happens in euthyroid sick syndrome?
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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
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What are common blood work findings in euthyroid sick syndrome?
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normal gland function but decreased T3, normal to decreased T4 and TSH and increased reverse T3
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What are causes of nontoxic goiter?
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1) iodine deficiency, cabbage, puberty, pregnancy
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What is subacute thyroiditis also known as?
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DeQuervians
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in mulitnodular goiter is colloid scant or abundant?
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abundant
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nontoxic goiter is followed by?
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multinodular goiter
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What are complications of nontoxic goiter?
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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 |
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What is pembertons sign associated with?
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nontoxic goiter compress jugular vein producing neck congestion
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What does a hot nodule mean?
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it is TSH independent
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Are solitary thyroid nodules generally cold or hot?
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cold
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What causes solitary thyroid nodules? what is the difference between men, woman and children with thyroid nodule
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1) majority are cysts, second are follicular adenoma, a few are malignant
2) men and children have a greater chance of malignancy |
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Are most solitary nodules euthyroid?
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yes
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What is the most common benign thyroid tumor? how is it characterized?
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1) follicular adenoma
2) has a complete capsule surrounded by normal thyroid tissue, painless. it is a cold nodule |
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On microscopic exam how is a follicular adenoma characterized? What do 10% of adenomas progress to?
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1) small packed follicles with compressed flat normal adjacent follicles
2) follicular carcinoma |
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What is the most common endocrine cancer? Is it more common in men or women?
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1) papillary carcinoma
2) women in 2nd and 3rd decades |
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the most common thyroid cancer is?
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papillary carcinoma
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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?
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1) papillary carcinoma
2) psammoma bodies and lymphatic invasion with metastisis to cervical nodes and lungs |
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What thyroid cancer is the most common solitary cold nodule? Is it more common in men than women?
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follicular carcinoma more common in women
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What are gross and microscopic findings of follicular carcinoma?
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encapsulated and/or invasive, has new blood vessels, spreads hematogenously
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the familial type of medullary cancer occurs in 20% of cases. What condition is it associated with?
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autosomal dominant MEN IIa/IIb
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What is MEN IIa?
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autosomal dominant condition with thyroid medullary carcinoma, hyperparathyroidism, pheochromocytoma
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What is MEN IIb?
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autosomal dominant condition with medullary carcinoma, mucosal neuromas (lips/tongue) pheochromocytomas
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In medullary carcinoma where do tumors derive from? What is seen on microscopic exam? What tumor marker is used?
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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 |
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In medullary carcinoma does the sporadic or familial type have a better prognosis? genetic screening in someone with familial type will have what marker?
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1) familial
2) RET proto-oncogene |
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Anaplastic tumor of the thyroid is commonly seen in who? What are the risk factors? What is the prognonsis?
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1) elderly women
2) multinodular goiter or follicular carcinoma 3) five year survival is only 5% |
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What might symptoms might a patient present with that would lead you to suspect anaplastic tumor of the thyroid?
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dyspnea and dysphagia from invasive action tumor into esophagous and trachea
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Where do the parathyroid glands derive from?
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superior from 4th pharyngeal pouch and inferior from 3rd
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What are the effects of PTH in kidney?
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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 |
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What effect does hyperphosphatemia have on PTH?
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increases PTH production so that phosphate is lost in urine
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What suppresses PTH?
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high Ca and low phosphates
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What vitamin D reaction occurs in the skin?
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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 |
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What is the function of calcitriol? Include where Ca and phosphorous are reabsorbed, etc
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attaches to nuclear receptors and increases Ca reabsorption in duodenum, increase phosphorous reabsorption in jejunem and ileum and activates osteoclasts
|
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What is another name for calcitriol? calcidiol?
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1) calcitriol = 1,25-(OH)2-D
2) calcidiol = 24,25-(OH)2-D |
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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 |
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How does serum Ca change when albumin decreases?
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Ca decreases but the ionized level is normal
|
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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
|
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Why does tetany result in metabolic alkalosis? What are the clinical findings?
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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) |
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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?
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1) autoimmune
2) DiGeorge (also have absent thymus) 3) hypomagnesemia, Mg is cofactor for adenylate cyclase which is needed by PTH, |
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What can cause hypomagnesemia? What are clinical findings with hypomagnesemia?
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1) diarrhea, aminoglycosides, diuretics and alcoholism
2) tetany, basal ganglia calcification from increased phorphorous driving Ca into brain tissue, and cataracts |
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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
|
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List several causes of hypocalcemia that are not do to the parathyroid glands?
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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?
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1) autosomal recessive absent 1-alpha hydroxylase
2) autosomal recessive absent receptors for calcitriol |
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What disease is characterized by mental retardation, basal ganglia calcification, short fourth and fifth metacarpals?
|
pseudohypoparathyroidism (knuckle-knuckle-dimple-dimple sign)
|
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Hyperparathyroidism is associated with what endocrine disorders?
|
MEN I and MEN IIa
|
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What is the most common cause of hyperparathyroidism
|
benign adenoma... remaining glands atrophy... right inferior parathyroid mostly involved
|
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What is seen in primary hyperplasia of the parathyroid?
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all four glands enlarge, usually a chief cell hyperplasia, though clear cell hyperplasia (wasserhelli cell hyperplasia)
|
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What are common clinical findings with hyperparathyroidism with the kidney?
|
1) calcium stones (most common presenting symptom)
2) nephrocalcinosis - polyuria and renal failure |
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What are common clinical findings with hyperparathyroidism with the GI?
|
1) PUD - Ca stimulates gastrin release
2) acute pancreatitis - Ca activates phospholipase 3) constipation |
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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 |
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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
|
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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
|
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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?
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ACTH production via small cell carcinoma of the lung, less commonly thymus and thyroid
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What type of hypertension develops in cushings syndrome?
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diastolic hypertension from increase in weak mineralcorticoids and glucocorticoids but not aldosterone
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Why do purple stria develop in cushings syndrome? What commonly occurs to bone?
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1) cortisol weakens collagen and causes rupture of blood vessels into stretch marks
2) osteoporosis |
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Low dose dexamethasone is good at suppressing a tumor causing cushings syndrome in which part of the body?
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no part... it cant suppress it anywhere
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High dose dexamethasone can suppress what condition in cushings syndrome?
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pituitary tumor but not other tumors
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What is seen on blood chem in someone with Cushings?
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hyperglycemia, hypokalemia, metabolic alkalosis
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Why does hypokalemia occur in cushings syndrome?
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hypokalemia occurs because of the increased weak mineralcorticoids. metabolic alkalosis also occurs
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Bilateral adrenalectomy with preexisting pituitary adenoma causes what? what are the features?
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1) Nelsons syndrome
2) headache and diffuse hyperpigmentation from ACTH production in tumor |
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The ACTH in a pituitary tumor vs that of an ectopic tumor is what?
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high to normal in the pituitary tumor and very high in the ectopic tumor
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Primary aldosteronism is called?
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1) Conns syndrome
2) caused by benign adenoma of glomerulosa 3) have diastolic hypertension, muscle weakness and tetany from metabolic alkalosis |
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What are lab findings in Conns syndrome? Is renin high or low?
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1) hypernatremia, hypokalemia, metabolic alkalosis
2) renin is low |
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Secondary aldosteronism is associated with what?
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decreased CO, so kidneys try to retain water, renin increased
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Pheochromocytomas generally arise unilaterally in adrenal medulla but can also arise in other areas of the body. What are they?
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bladder, organ of Zuckerkandl near bifurcation of aorta and posterior mediastinum
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What enzyme converts norepi to epi? Where in the body is this enzyme found? Can a pheochromocytoma occur outside of these sites?
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1) N-methyltransferase
2) found in adrenal medula and organ of Zuckerkandl 3) yes but they can only produce norepi |
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What is pheochromocytoma associated with?
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1) neurofibromatosis
2) MEN IIa and IIb (mutation of RET proto-oncogene), Von Hippel-lindau disease (mutation of VHL gene) |
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Characteristics on exam of a pheochromocytoma are?
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adrenal medulla is brown, hemorrhagic and often necrotic
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What are unique findings with a pheochromocytoma?
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1) palpitations
2) paroxysmal hypertension 3) anxiety 4) drenching sweats 5) headache 6) orthostatic hypotension 7) chest pain 8) ILEUS |
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Lab tests for someone with pheochromocytoma are?
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1) increased plasma metanephrines (best test)
2) increased plasma normetanephrine 3) increased 24-hr urine metanephrine (100% sensivity) 4) increased 24-hr urine VMA |
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What effect does clonidine have on a pheochromocytoma? What effect does it have on neutrophil levels?
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1)it is unable to suppress norepi and is therefore a good evaluator
2) nuetrophil leukocytosis via inhibition of adhesion molecules |
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Someone with pheochromocytoma and be stabilized preoperatively with what drugs?
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1) phenoxybenzamine
2) beta-blocker 3) metyrosine |
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What is neuroblastoma and in what group does it occur in?
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malignant neoplasm of postganglionic sympathetic neurons occurring in children under 5
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What oncogene is associated with neuroblastoma?
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N-MYC
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Opsoclonus-myoclonus syndrome is seen it what? What is it?
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1) neuroblastoma
2) paraneoplastic syndrome, myclonic jerks of extremities, chaotic eye movements |
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Where does neuroblastoma commonly metastize to?
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skin and bone... normal this occurs before diagnosis
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What is the prognosis of neuroblastoma dependent on?
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age if less than 1 year there is a better prognosis
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How is the neuroblastoma characterized?
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"small cell" tumor composed of malignant neuroblasts, homer-wright rosettes (neuroblasts located around central space)
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What are clinical findings of pheochromocytoma?
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palpable abdominal mass, diastolic hypertension, increased urine VMA and HVA
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A person presents with hyperglycemia and necrolytic migratory erythema. The hyperglycemia responds to octreotide. What does the person have?
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malignant glucogonoma
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What is the most common islet cell tumor? What is it associated with? How is it treated?
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1) Insulinoma associated with MEN I
2) streptozotocin |
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How can an insuloma be distinguished from over use of exogenous insulin?
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by measuring C peptide
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A patient has achlorhydria, cholelithiasis and steatorrhea, and diabetes mellitus. What is the underlying problem? How is it treated?
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malignant somatostatinoma treated with streptozotocin
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What is found in someone with a vipoma?
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secretory diarrhea and achlorhydria. hypokalemia with normal anion gap metabolic acidosis (loss of bicarb in stool)
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What is zollinger ellison associated with?
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MEN I
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What group is diabetes increasing in?
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native americans
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What conditions is diabetes the leading cause of in the US?
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1) legal blindness
2) peripheral neuropathy 3) chronic renal failure 4) below the knee amputation |
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What are drugs that can cause diabetes? What genetic diseases are associated with diabetes?
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1) glucocorticoids, pentamide, thiazides and alpha interferon
2) hemochromatosis |
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Acanthosis nigricans is a phenotypic marker of what?
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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
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Which viruses are associated with diabetes?
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mumps and CMV
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What is maturity onset diabetes caused by? when does it present? In general what is seen?
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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 |
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In metabolic syndrome what causes insulin resistance?
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genetic deficiency that is exacerbated by obesity
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metabolic syndrome is commonly associated with what? What are other less common associations?
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1) polycystic ovary syndrome
2) acanthosis nigricans 3) Alzheimers disease |
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Clinical and lab findings in someone with metabolic syndrome are?
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hyperinsulinemia, increased VLDL from increased triglyceride syn, hypertension because insulin retains Na in kidney, CAD
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adipose tissue has what effect on insulin receptors? What is the definition of metabolic syndrome?
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1) decreases
2) waistline >40 in men, >35 in women HDL <40 in men, <50 in women fasting glucose >110 |
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In type I diabetes what type of hypersensitivity occurs? What condition is type II diabetes associated with?
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1) Type IV and type II against insulin and beta cells
2) alzheimers |
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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?
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1) amyloid stains with congo red
2) hyperosmotic nonketotic coma |
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What are a few signs that someone has Type II diabetes?
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1) recurrent blurry vision from dysregulated sorbitol production
2) recurrent bacterial and candidal infections |
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What is an early test finding in someone with type II diabetes?
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reactive hypoglycemia where too much insulin is released for the amount of glucose given
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What type of arteriosclerosis occurs in diabetes?
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hyaline arteriosclerosis
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What occurs in both proliferative and nonproliferative diabetes retinopathy?
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1) nonproliferative: flame hemorrhages and microaneurysms, exudate
2) proliferative: neovascularization, retinal detachment |
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A patient comes in complaining of burning feet and pressure ulcers on feet. What does he have and how do you treat these symptoms?
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1) diabetes
2) duloxetine (selective serotonin and NE reuptake inhibitor), topical capsaicin and amitptyline |
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Which cranial nerves are commonly involved in diabetes?
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CN III, IV and VI
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What common infections are seen in diabetes?
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candida, malignant external otitis media (P aeurginosa), rhinocerebral mucormycosis, cutaneous S aureus infections
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Skin disorders present in diabetes?
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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 |
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What enzyme is responsible for the vision problems when glucose is elevated? What other problems does this same pathway lead to?
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1) aldose reductase converts glucose to sorbitol
2) schwann cells swell and are damaged causing peripheral neuropathy |
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What is occurring in diabetic microangiopathy?
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increased synthesis of type IV collagen in basement membrane and mesangium
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What is the sympathetic response to hypoglycemia? parasympathetic?
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1) sweating, tachy, palpitations, tremulousness
2) nausea and hunger |
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Why is beta oxidation increased in diabetes as far as what is occurring in mitochondria?
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decreased malonyl-CoA to inhibit carnitine acyltransferase
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What type of hyperlipoproteinemia occurs in diabetes?
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type V
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hyperchylomicronemia syndrome is seen in what condition and is the result of what?
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increased chylomicrons from lack of insulin activity in diabetes. leads to eruptive xanthomas in skin
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Will hyper/hypokalemia result in diabetes? Is the anion gap normal or elevated? what type of azotemia occurs?
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1) hyperkalemia because H+ moves into cells in exchange for K+
2) elevated because of ketoacidosis and lactic acidosis 3) prerenal from volume depletion |
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What hormones are involved in producing gestational diabetes? When should screening begin?
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1) human placental lactogen, cortisol, progesterone
2) 24 to 28 weeks gestation |
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With gestational diabetes what are the risks to the newborn?
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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 |
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What causes in type I polyglandular syndrome? When is onset? What are the findings?
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1) autosomal recessive
2) mean age is 12 3) addisons, primary hypoparathyroidism, mucocutaneous candidiasis |
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What causes in type II polyglandular syndrome? When is onset? What are the findings?
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1) autosomal dominant HLA-DR3 and DR4 relation
2) 24 yo 3) addisons, hashimotos thyroiditis, type I diabetes |
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hypoglycemia occurs at what level?
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1) <50mg/dL
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