Endocrine Flashcards

Question

Calcium homeostasis

Answer 1

Plasma Ca exists in 3 forms 1) Ionized (45%) 2) Bound to albumin (40%) 3) Bound to anions (15%) Increase in pH leads to increased affinity of albumin (increased negative charge) to bind to Ca This leads to hypocalcemia (cramps, pain, paresthesias, carpopedal spasm)

Answer 2

1) Source: D3 from sun exposure in skin. D2 ingested from plants. Both converted to 25-OH in liver and to 1,25-(OH)2 (active form) in kidney 2) Function: - Increased absorption of dietary Ca and PO4 - Increased bone resorption leads to increased Ca and PO4 3) Regulation: Increased PTH, low Ca, low PO4 all lead to increased 1,25-(OH)2 production 1,25-(OH)2 feedback inhibits its own production Deficiency leads to rickets in kids, osteomalacia in adults. Caused by malabsorption, lower sunlight, poor diet, chronic kidney failure 24,25-(OH)2D3 is an inactive form of vitamin D. PTH leads to increased Ca reabsorption and lower PO4 reabsorption in the kidney, whereas 1,25-(OH)2D3 leads to increased absorption of both Ca and PO4 in the gut

Answer 3

Chief Cells of parathyroid 1) Increases bone resorption of Ca and PO4 2) Increased kidney reabsorption of Ca in DCT 3) Decreased reabsorption of PO4 in PCT 4) Increased 1,25-(OH)2D3 (calcitriol) production by stimulating kidney 1a-hydroxylase in PCT PTH increases serum Ca and lowers serum PO4. PTH increases urine PO4 Increased production of macrophage colony-stimulating factor and RANK-L (receptor activator of NF-kB ligand). RANK-L (ligand) secreted by osteoblasts and osteocytes binds RANK (receptor) on osteoclasts and their precursors to stimulate osteoclasts and increase Ca Intermittent PTH release can stimulate bone formation PTH = Phosphate Trashing Hormone PTH-related peptide (PTHrP) functions like PTH and is commonly increased in malignancies

Answer 4

Lower serum Ca leads to higher PTH secretion High serum PO4 leads to higher PTH secretion Low serum Mg leads to high PTH secretion Very low serum Mg leads to lower PTH secretion Common causes of lower Mg include diarrhea, aminoglycosides, diuretics, alcohol abuse

Answer 5

1) Source: Parafollicular cells (C cells) of thyroid 2) Function: Lowers bone resorption of Ca 3) Regulation: Increased serum Ca leads to calcitonin secretion Calcitonin opposes action of PTH. Not important in normal Ca homeostasis. CalciTONin TONes done Ca levels

Answer 6

``` FSH LH ACTH TSH CRH hCG ADH (V2 receptor) MSH PTH calcitonin GHRH glucagon ``` FLAT ChAMP + cgg

Answer 7

ANP BNP NO (EDRF) Think vasodilation

Answer 8

``` GnRH Oxytocin ADH (V1 receptor) TRH Histamine (H1 receptor) Angiotensin II Gastrin ``` GOAT HAG

Answer 9

``` Vitamin D Estrogen Testosterone T3/T4 Cortisol Aldosterone Progesterone ``` VETTT CAP

Answer 10

``` Insulin IGF-1 FGF PDGF EGF ``` MAP kinase pathway - think growth factors

Answer 11

``` Prolactin Immunomodulators (cytokines IL2, IL6, IFN) GH G-CSF Erythropoietin Thrombopoietin ``` JAK/STAT pathway - think acidophils and cytokines PIGG(L)ET

Answer 12

Steroid hormones are lipophilic and therefore must circulate bound to specific binding globulins, which increase their solubility In men, increased sex hormone-binding globulin (SHBG) lowers free testosterone leading to gynecomastia In women, lower SHBG raises free testosterone and leads to hirsutism OCPs, pregnancy raise SHBG (free estrogen levels remain unchanged)

Answer 13

Iodine-containing hormones that control the body's metabolic rate Source: Follicles of thyroid. Most T3 formed in target tissues Functions: 1) Bone growth (synergism with GH) 2) CNS maturation 3) Increases B1 receptors in heart = Higher CO, HR, SV, contractility 4) Increases basal metabolic rate via N/K/ATPase activity leads to higher O2 consumption, RR, body temperature 5) Increases glycogenolysis, gluconeogenesis, lipolysis ``` T3 functions - 4 B's Brain maturation Bone growth B-adrenergic effects Basal metabolic rate goes up ``` Thyroxine-binding globulin (TBG) binds most T3/T4 in blood; only free hormone is active. Lower TBG in hepatic failure, steroids; Increased TBG in pregnancy or OCP use (estrogen increases TBG) T4 is major thyroid product; converted to T3 in peripheral tissue by 5'-deiodinase T3 binds nuclear receptor with greater affinity than T4 Peroxidase is the enzyme responsible for oxidation and organification of iodide as well as coupling of monoiodotyrosine (MIT) and di-iodotyrosine (DIT) Propylthiouracil inhibits both peroxidase and 5'deiodinase. Methimazole inhibits peroxidase only

Answer 14

TRH (hypothal) stimulates TSH (pit), which stimulates follicular cells. Negative feedback by free T3, T4 to anterior pit lowers sensitivity to TRH Thyroid-stimulating immunoglobulins (TSH) stimulate follicular cells (Graves Disease) Wolff-Chaikoff Effect - excess iodine temporarily inhibits thyroid peroxidase leading to lower iodine organification leading to low T3/T4 production

Answer 15

High cortisol due to a variety of causes 1) Exogenous corticosteroids - results in lower ACTH, bilateral adrenal atrophy. Most common cause. 2) Primary adrenal adenoma, hyperplasia, or carcinoma - result in lower ACTH, atrophy of uninvolved adrenal gland. Can also present with pseudohyperaldosteronism 3) ACTH-secreting pituitary adenoma (Cushing Disease*); paraneoplastic ACTH secretion (small cell lung cancer, bronchial carcinoids) - result in high ACTH, bilateral adrenal hyperplasia. * Cushing disease is responsible for the majority of endogenous cases of Cushing Syndrome

Answer 16

HTN, weight gain, moon facies, truncal obesity, buffalo hump, skin changes (thinning, striae), osteoporosis, hyperglycemia (insulin resistance), amenorrhea, immunosuppression

Answer 17

Screening tests include: 1) Increased free cortisol on 24-hr urinalysis 2) Increased midnight salivary cortisol, and no suppression with overnight low-dose dexamethasone test 3) Measure serum ACTH. If low, suspect adrenal tumor. If high, distinguish between Cushing disease and ectopic ACTH secretion with a high dose (8mg) dexamethasone suppression test and CRH stimulation test. Ectopic secretion will not decrease with dexamethasone bc the source is resistant to negative feedback; ectopic secretion will not increase with CRH bc pit ACTH is suppressed

Answer 18

Inability of adrenal glands to generate enough glucocorticoids +/- mineralocorticoids for the body's needs. Symptoms include: weakness, fatigue, orthostatic hypotension, muscle aches, weight loss, GI disturbances, sugar and/or salt cravings Diagnosis involves measurement of serum electrolytes, morning/random serum cortisol and ACTH, and response to ACTH stimulation test Alternatively, can use metyrapone stimulation test: metyrapone blocks last step of cortisol synthesis (11-deoxycortisol becomes cortisol). Normal response is decreased cortisol and compensatory increased ACTH. In adrenal insufficiency, ACTH remains low after test.

Answer 19

Deficiency of aldosterone and cortisol production due to loss of gland function leading to hypotension (hyponatremic volume contraction), hyperkalemia, metabolic acidosis, skin and mucosal hyperpigmentation (due to MSH, a byproduct of increased ACTH production from pro-opiomelanocortin) Acute - sudden onset (due to massive hemorrhage) - may present with shock in acute adrenal crisis Chronic - aka Addison Disease. Due to adrenal atrophy or destruction by disease (e.g. autoimmune, TB, metastasis) Primary Pigments the skin/mucosa. Autoimmunity most common cause of primary chronic adrenal insufficiency in Western world. Associated with autoimmune polyglandular syndromes Waterhouse-Friderichsen Syndrome - acute primary adrenal insufficiency due to adrenal hemorrhage associated with septicemia (usually N. Meningitides), DIC, endotoxic shock

Answer 20

Seen with lower pituitary ACTH production No skin/mucosal hyperpigmentation, no hyperkalemia (aldosterone synthesis preserved) Secondary Spares the Skin/mucosa

Answer 21

Seen in patients with chronic exogenous steroid use, precipitated by abrupt withdrawal. Aldosterone synthesis unaffected Tertiary from Treatment

Answer 22

Most common tumor of the adrenal medulla in children, usually less than 4 years old. Originates from neural crest cells; Homer-Wright rosettes characteristic Occurs anywhere along the sympathetic chain. Most common presentation is abdominal distension and a firm, irregular mass that can cross the midline (vs Wilms Tumor, which is smooth and unilateral) Can also present with opsoclonus-myoclonus syndrome ("dancing eyes-dancing feet"). Homovanillic acid (HVA; a breakdown product of dopamine) and vanillylmandelic acid (VMA; a breakdown product of NE) are increased in urine Bombesin and neuron-specific enolase (+). Less likely to develop HTN. Associated with overexpression of N-myc oncogene

Answer 23

Most common tumor of the adrenal medulla in adults Derived from chromaffin cells (arise from neural crest) Rule of 10's ``` 10% malignant 10% bilateral 10% extra-adrenal 10% calcify 10% kids ``` Symptoms: Most tumors secrete Epinephrine, NE, and dopamine, which can cause episodic HTN Associated with neurofibromatosis type 1, von Hippel-Lindau disease, MEN 2A and MEN 2B Symptoms occur in "spells" - relapse and remit Episodic hyperadrenergic symptoms (5 Ps) ``` Pressure (higher BP) Pain (HA) Perspiration Palpitations (tachycardia) Pallor ```

Answer 24

Increased catecholamines and metanephrines in urine and plasma Tx = Irreversible alpha-antagonists (phenoxybenzamine) followed by B-blockers prior to tumor resection. Alpha blockade must be achieved before giving B-blockers to avoid a hypertension crisis

Answer 25

Cold intolerance (lower heat production) Weight gain, lower appetite Hypoactivity, lethargy, fatigue, weakness Constipation Lower reflexes Myxedema (facial/ periorbital) Dry, cool skin; coarse, brittle hair Bradycardia, dyspnea on exertion ``` Labs: High TSH (sensitive test for primary hypothyroidism) ``` Low free T3 and T4 Hypercholesterolemia (due to lower LDL receptor expression)

Answer 26

Heat intolerance (higher heat production) Weight loss, increased appetite Hyperactivity Diarrhea Higher reflexes Pretibial myxedema (Graves), periorbital edema Warm, moist skin; fine hair Chest pain, palpitations, arrhythmias, increased number and sensitivity of B-adrenergic receptors ``` Labs: Low TSH (if primary) ``` High free or total T3 and T4 Hypocholesterolemia (due to increased LDL receptor expression)

Answer 27

Most common cause of hypothyroidism in iodine-sufficient regions; an autoimmune disorder (anti-thyroid peroxidase, antimicrosomal and antithyroglobulin antibodies). Associated with HLA-DR5 Increased risk of NH Lymphoma May be hyperthyroid early in course due to thyrotoxicosis during follicular rupture Histologic findings: Hurthe cells, lymphoid aggregate with germinal centers Findings: moderately enlarged, nontender thyroid

Answer 28

Severe fetal hypothyroidism due to maternal hypothyroidism, thyroid agensis, thyroid dysgenesis (most common cause in US), iodine deficiency, dyshormonogenetic goiter Findings = 6Ps ``` Pot-bellied, Pale, Puffy-faced child with Protruding umbilicus, Protuberant tongue, and Poor brain development ```

Answer 29

A hypothyroidism Self-limited disease often following a flu-like illness May be hyperthyroid early in course, followed by hypothyroidism Histology: granulomatous inflammation Findings: Increased ESR, jaw pain, early inflammation, very tender thyroid dequerVAIN = PAIN

Answer 30

A hypothyroidism Thyroid replaced by fibrous tissue (hypothyroid) Fibrosis may extend to local structures (airway), mimicking anaplastic carcinoma Considered a manifestation of IgG4 related systemic disease (autoimmune pancreatitis, retroperitoneal fibrosis, noninfectious aortitis) Findings: fixed, hard (rock-like) painless goiter

Answer 31

Iodine deficiency Goitrogens Wolff-Chaikoff effect (thyroid gland downregulation in response to increased iodine)

Answer 32

Most common cause of hyperthyroidism. Autoantibodies (IgG) stimulates TSH receptors on thyroid (hyperthyroidism, diffuse goiter), retro-orbital fibroblasts (exophthalmos: proptosis, extraocular muscle swelling), and dermal fibroblasts (pretibial myxedema) often presents during stress (childbirth)

Answer 33

A hyperthyroidism Focal patches of hyperfunctioning follicular cells working independently of TSH due to mutation in TSH receptor. Increased release of T3 and T4. Hot nodules are rarely malignant

Answer 34

Hyperthyroidism cause Stress-induced catecholamine surge seen as a serious complication of thyrotoxicosis due to disease and other hyperthyroid disorders Presents with agitation, delirium, fever, diarrhea, coma, and tachyarrhythmia (cause of death) May see increased ALP due to increased bone turnover. Treat with 3Ps = B blockers (Proranolol), Propylthiouracil, corticosteroids (prednisolone)

Answer 35

Hyperthyroidism cause Thyrotoxicosis if a patient with iodine deficiency goiter is made iodine replete

Answer 36

Thyroidectomy is an option for thyroid cancers and hyperthyroidism. Complications of surgery include hoarseness (due to recurrent laryngeal nerve damage), hypocalemia (due to removal of parathyroid glands), and transection of recurrent and superior laryngeal nerves (during ligation of inferior and superior laryngeal arteries respectively)

Answer 37

Most common thyroid cancer, excellent prognosis Empty-appearing nuclei with central clearing ("Orphan Annie" eyes), psammoma bodies, nuclear grooves. Lymphatic invasion common Increased risk with RET and BRAF mutations, childhood irradiation

Answer 38

Thyroid cancer Good prognosis, invades thyroid capsule (unlike follicular adenoma), uniform follicles

Answer 39

Thyroid cancer From parafollicular "C Cells" Produces calcitonin, sheets of cells in an amyloid stroma, hematogenous spread common Associated with MEN 2A and 2B (RET mutations)

Answer 40

Thyroid cancer Older patients Invades local structures (airway) Very poor prognosis

Answer 41

Associated with Hashimoto thyroiditis

Answer 42

Due to accidental surgical excision of parathyroid glands, autoimmune destruction, or DiGeorge Findings = hypocalcemia, tetany Chvostek sign - tapping of facial nerve (tap the Cheek) leads to contraction of facial muscles Trousseu sign - occlusion of brachial artery with BP cuff (cuff the Triceps) leads to carpal spasm

Answer 43

"Albright hereditary osteodystrophy" Unresponsiveness of kidney to PTH. Hypocalcemia, shortened 4th/5th digits, short stature Autosomal dominant

Answer 44

Defective Ca sensing receptor on parathyroid cells PTH cannot be suppressed by an increase in Ca level This causes mild hypercalcemia with normal to increased PTH levels

Answer 45

usually due to parathyroid adenoma or hyperplasia Hypercalcemia, hypercalciuria (renal STONES), hypophosphatemia, increased PTH, ALP, cAMP in urine. Most often asymptomatic. May present with weakness and constipation ("groans"), abdominal/flank pain (kidney stones, acute pancreatitis), depression ("psychiatric overtones" Stones, groans, and psychiatric overtones Osteitis fibrosa cystica - cystic BONE spaces filled with brown fibrous tissue ("brown tumor" consisting of deposited hemosiderin from hemorrhages; causes bone pain) Stones, bones, groans and psych overtones

Answer 46

Secondary hyperplasia due to low Ca absorption and/or high PO4, most often in chronic renal disease (causes hypovitaminosis D leading to low Ca absorption) Hypocalcemia, hyperphosphatemia in chronic renal failure (vs hypophosphatemia with most other causes), high ALP, high PTH Renal Osteodystrophy - bone lesions due to secondary or tertiary hyperparathyroidism due in turn to renal disease.

Answer 47

Refractory (autonomous) hyperparathyroidism resulting from chronic renal disease. HUGE increase in PTH, high Ca Renal Osteodystrophy - bone lesions due to secondary or tertiary hyperparathyroidism due in turn to renal disease.

Answer 48

Most commonly prolactinoma (benign). Adenoma may be functional (hormone producing) or nonfunctional (silent. Nonfunctional tumors present with mass effect (bitemporal hemianopia, hypopituitarism, HA) Functional tumor presentation is based on the hormone produced (prolactinoma: amenorrhea, galactorrhea, low libido, infertility; somatotropic adenoma: acromegaly) Tx for prolactinoma = dopamine agonists (bromocriptine or cabergoline), transsphenoidal resection

Answer 49

Excess GH in adults. Typically caused by pituitary adenoma Findings: Large tongue with deep furrows, deep voice, large hands and feet, coarse facial features, impaired glucose tolerance (insulin resistance) Increased risk of colorectal polyps and cancer Dx: Increased serum IGF-1; failure to suppress serum GH following oral glucose tolerance test; pituitary mass seen on brain MRI Tx: Pit adenoma resection: If not cured, treat with octreotide (somatostatin analog) or pegvisomant (GH receptor antagonist) Increased GH in children leads to gigantism (increased linear bone growth). HF most common cause of death

Answer 50

Characterized by intense thirst and polyuria with inability to concentrate urine due to lack of ADH (central) or failure of response to circulating ADH (nephrogenic) 1) Central DI Cause: Pit tumor, autoimmune, trauma, surgery, ischemic encephalopathy, idiopathic Findings: Low ADH Urine specific gravity 290 mOsm/kg Hyperosmotic volume contraction Water deprivation test (No water intake for 2-3 hrs followed by hourly measurements of urine volume and osmolarity and plasma Na concentration and osmolarity. ADH analog - desmopressin acetate - is administered if normal values are not clearly reached) > 50% increase in urine osmolality only after administration of ADH analog Tx: Intranasal desmopressin acetate. Hydration. 2) Nephrogenic DI Cause: hereditary (ADH receptor mutation), secondary to hypercalcemia, lithium, demeclocycline (ADH antagonist) Findings: Normal ADH levels Urine specific gravity 290 mOsm/kg Hyperosmotic volume contraction Water deprivation test: Minimal change in urine osmolality, even after administration of ADH analog Tx: HCTZ, indomethacin, amiloride. Hydration.

Answer 51

Syndrome of Inappropriate ADH secretion Excessive free water retention Euvolemic hyponatremia with continued urinary Na excretion Urine osmolality > Serum osmolality Body responds to water retention with reduced aldosterone (hyponatremia) to maintain near-normal volume status. Very low serum Na levels can lead to cerebral edema, seizures. Correct slowly to prevent osmotic demyelination syndrome (formerly known as central pontine myelinolysis) ``` Causes include: Ectopic ADH (small cell lung cancer) CNS disorder/head trauma Pulmonary disease Drugs (cyclophosphamide) ``` Tx: Fluid restriction, IV hypertonic saline, conivaptan, tolvaptan, demeclocycline

Answer 52

Undersecretion of pituitary hormones due to: 1) Nonsecreting pituitary adenoma, craniopharyngioma 2) Sheehan Syndrome - ischemic infarct of pituitary following postpartum bleeding; usually presents with failure to lactate, absent menstruation, cold intolerance 3) Empty Sella Syndrome - atrophy or compression of pituitary, often idiopathic, common in obese women 4) Pituitary apoplexy - sudden hemorrhage of pituitary gland, often in the presence of an existing pituitary adenoma 5) brain injury 6) Radiation Tx: hormone replacement therapy (corticosteroids, thyroxine, sex steroids, human growth hormone)

Answer 53

Insulin deficiency or insensitivity (and glucagon excess) leads to: 1) Decreased serum glucose uptake. This leads to hyperglycemia, glycosuria, osmotic diuresis, electrolyte depletion. All this leads to dehydration +/- acidosis. Finally this can cause coma, death. 2) Increased protein catabolism leading to increased plasma amino acids, nitrogen loss in urine. This leads to hyperglycemia, glycosuria, osmotic diuresis, electrolyte depletion. All this leads to dehydration +/- acidosis. Finally this can cause coma, death. 3) Increased lipolysis (insulin deficiency only). This leads to increased plasma FFAs, ketogenesis, ketonuria, ketonemia. This all leads to dehydration +/- acidosis. Finally this can cause coma, death. Polydipsia, polyuria, polyphagia, weight loss, DKA (type 1), hyperosmolar coma (type 2) Rarely, can be caused by unopposed secretion of GH and Epinephrine. Also seen in patients on glucocorticoid therapy (steroid diabetes)

Answer 54

1) Nonenzymatic glycation: - small vessel disease (diffuse thickening of basement membrane) leads to retinopathy (hemorrhage, exudates, microaneurysms, vessel proliferation), glaucoma, neuropathy, nephropathy (nodular glomerulosclerosis, aka Kimmelstiel-Wilson nodules leads to progressive proteinuria and arteriolosclerosis leading to HTN; both lead to chronic renal failure) - Large vessel atherosclerosis, CAD, peripheral vascular occlusive disease, gangrene leading to limb loss, cerebrovascular disease. MI most common cause of death 2) Osmotic damage (sorbitol accumulation in organs with aldose reductase and low or absent sorbitol dehydrogenase): Neuropathy (motor, sensory, and autonomic degeneration) Cataracts

Answer 55

Fasting serum glucose, oral glucose tolerance test, HbA1C (reflects avg blood glucose over prior 3 months)

Answer 56

1) Type 1 Primary defect = Autoimmune destruction of B cells Insulin necessary in Tx? Always Age: Less than 30 Association with obesity? No Genetic predisposition? Relatively weak (50% concordance in identical twins), polygenic Association with HLA system? Yes (HLA-DR3 and DR4) Glucose intolerance: Severe Insulin sensitivity: High Ketoacidosis: Common B-cell numbers in the islets: Low Serum insulin: Low Classic symptoms of polyuria, polydipsia, polyphagia, weight loss: Common Histology: Islet leukocytic infiltrate 2) Type 2 Primary defect = Increased resistance to insulin, progressive pancreatic B cell failure Insulin necessary in Tx? Sometimes Age: Over 40 Association with obesity? Yes Genetic predisposition? Relatively strong (90% concordance in identical twins), polygenic Association with HLA system? No Glucose intolerance: Mild to moderate Insulin sensitivity: Low Ketoacidosis: Rare B-cell numbers in the islets: Variable (with amyloid deposits) Serum insulin: Variable Classic symptoms of polyuria, polydipsia, polyphagia, weight loss: Sometimes Histology: Islet amyloid polypeptide (IAPP) deposits

Answer 57

One of the most feared complications of diabetes. Usually due to increased insulin requirements from increased stress (infection). Excess fat breakdown and increased ketogenesis from increased free fatty acids, which are then made into ketone bodies (B-hydroxubutyrate > acetoacetate). Usually occurs in type 1 diabetes, as endogenous insulin in type 2 diabetes usually prevents lipolysis. Signs/Symptoms: Kussmaul respirations (rapid/deep breathing), nausea/vomiting, abdominal pain, psychosis/delirium, dehydration. Fruity breath odor (due to exhaled acetone) Labs: Hyperglycemia, increased H, Low HCO3 (higher anion gap metabolic acidosis), increased blood ketone levels, leukocytosis. Hyperkalemia, but depleted intracellular K due to transcellular shift from low insulin insulin (therefore total body K is depleted) Complications: Life-threatening mucormycosis (usually Rhizopus infection), cerebral edema, cardiac arrhythmias, heart failure Tx: IV fluids, IV insulin, and K (to replete intracellular stores); glucose if necessary to prevent hypoglycemia

Answer 58

Tumor of pancreatic alpha cells leading to overproduction of glucagon Presents with dermatitis (necrolytic migratory erythema), diabetes (hyperglycemia), DVT, and depression

Answer 59

Tumor of pancreatic B cells leading to overproduction of insulin leading to hypoglycemia. May see Whipple triad: Low blood glucose, symptoms of hypoglycemia (lethargy, syncope, diplopia), and resolution of symptoms after normalization of glucose levels. Symptomatic patients have low blood glucose and increased C peptide levels (vs exogenous insulin use). Tx = surgical resection

Answer 60

Rare syndrome caused by carcinoid tumors (neuroendocrine cells), especially metastatic small bowel tumors, which secrete high levels of 5-HT (serotonin) not seen if tumor is limited to GI tract (5HT undergoes first-pass metabolism in liver) Results in recurrent diarrhea, cutaneous flushing, asthmatic wheezing, right-sided valvular disease Increased 5-hydroxyindoleacetic acid (5-HIAA) in urine Niacin deficiency (pellagra) Tx = surgical resection, somatostatin analog (octreotide)

Answer 61

Gastrin-secreting tumor (gastrinoma) of pancreas or duodenum Acid hypersecretion causes recurrent ulcers in duodenum and jejunum Presents with abdominal pain (peptic ulcer disease, distal ulcers), diarrhea (malabsorption). Positive secretin stimulation test: Gastrin levels remain elevated after administration of secretin, which normally inhibits gastrin release. May be associated with MEN 1

Answer 62

Auto Dom 3 P's Parathyroid tumors Pituitary tumors (prolactin or GH) Pancreatic endocrine tumors - Zollinger-Ellison Syndrome, insulinomas, VIPomas, glucagonomas (rare) Associated with mutation of MEN1 gene (menin, a tumor suppressor)

Answer 63

Auto Dom 2 P's Parathyroid hyperplasia Pheochromocytoma Medullary thyroid carcinoma (secretes calcitonin) Associated with marfanoid habitus; mutations in RET gene (codes for receptor tyrosine kinase)

Answer 64

Auto Dom Only 1 P now Pheochromocytoma Medullary thyroid carcinoma (secretes calcitonin) Oral/intestinal ganglioneuromatosis (mucosal neuromas) Associated with marfanoid habitus; mutations in RET gene