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Step 1 (Steffanie) > Endocrine > Flashcards

Endocrine Flashcards

1
Q

What are the immunohistochemistry findings in a pheochromocytoma?

A

Positive for synaptophysin, chromogranin, and neuron-specific enolase

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2
Q

What is the abnormal bone pathology seen in CKD? What causes it?

A

Renal osteodystrophy. Caused by secondary hyperparathyroidism (caused by hyperphosphatemia and hypocalcemia)

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3
Q

What can lead to permanent diabetes insipidus (DI)

A

Damage to hypothalamic nuclei, because it results in a loss of vasopressinergic neurons

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4
Q

How is prolactin release regulated?

A

It is under inhibitory control by dopamine secretion from the hypothalamus

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5
Q

phenylethanolamine-N-methyltransferase (PNMT)

A

Enzyme in the adrenal medulla that rapidly converts norepinephrine to epinephrine

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6
Q

Mechanism of development of Graves opthalmopathy

A

Stimulation of orbital fibroblasts and adipocytes by thyrotropin receptor antibodies and activated T cells. Fibroblasts secrete excess amounts of glycosaminoglycans, leading to expansion of the ground substance of retro-orbital tissues. This, along with excess adipose deposition, displaces the globe forward.

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7
Q

When does hyperosmotic volume contraction occur?

A

In patients with diabetes insipidus or as a result of decreased fluid intake/excessive sweating

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8
Q

What causes hyperosmotic volume contraction?

A

Loss of free water with retention of electrolytes

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9
Q

Glyburide mechanism

A

Sulfonylurea - binds to their receptor on pancreatic beta cells to inhibit the ATP-dependent potassium channel. Net result is increased insulins secretion by beta cells independent of blood glucose concentrations

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10
Q

What is a major risk of sulfonylureas?

A

Hypoglycemia. Increased risk with exercise, missed meals, chronic malnourishment, or organ dysfunction

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11
Q

Name the second generation sulfonylureas. Which are long vs. short acting?

A

Glipizide, Glyburide, Glimepiride.

Glyburide and Glimepiride are long acting, higher incidence of hypoglycemia. Glipizide is short acting, significantly lower incidence of hypoglycemia

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12
Q

Intense mononuclear infiltrate consisting of lymphocytes and plasma cells, often with germinal centers. Follicles surrounded by large oxyphilic cells with granular cytoplasm (Hurthle cells)

A

Biopsy findings in chronic lymphocytic (Hashimoto) thyroiditis

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13
Q

Pancreatic islet amyloid deposition

A

Type 2 Diabetes Mellitus

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14
Q

5 alpha-reductase

A

Converts testosterone to DHT

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15
Q

Phenotypic male born with feminized external genitalia that masculinize at puberty. What enzyme is deficient?

A

5-alpha-reductase

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16
Q

Myopathy, cardiomyopathy, hypoketotic hypoglycemia in the setting of decreased muscle carnitine content suggests?

A

Primary carnitine deficiency. Caused by a defect in the protein responsible for carnitine transport across the plasma membrane

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17
Q

Elevated FSH and LH; low testosterone; long lower extremities; small, firm testes; azoospermia

A

Klinefelter syndrome (47, XXY)

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18
Q

Maldevelopment of the third and fourth pharyngeal pouches results in?

A

DiGeorge syndrome

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19
Q

Signs of hypocalcemia

A

Muscle cramps, perioral paresthesias, hypotension, possible laryngospasm, Chvostek sign, Trousseau sign

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20
Q

Male at age 2-4 years who present with early virilization, increased linear growth, and elevated levels of 17-hydroxyprogesterone and androgens

A

Classic, non-salt-wasting 21-hydroxylase deficiency

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21
Q

Treatment of congenital adrenal hyperplasia

A

Low doses of exogenous corticosteroids to suppress ACTH secretion

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22
Q

Leuprolide

A

GnRH analog. Agonist properties when administered in a pulsatile fashion and can cause transient rise in LH and testosterone production on initial administration. Long term leuprolide use suppresses pituitary LH release and subsequently leads to reduced production of testosterone by Leydig cells

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23
Q

What do decreased parathyroid hormone levels cause?

A

Decreased calcium and phosphate resoprtion from bone and decreased calcium reabsorption by the kidneys

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24
Q

Statins mechanism

A

Act through competitive inhibition of HMG-CoA reductase, preventing conversion of HMG-CoA to mevalonic acid. Decreased liver cholesterol synthesis leads to increased hepatic clearance of LDL from the circulation by LDL receptors. Increase in LDL receptor recycling.

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25
Q

Most common complication of statins

A

myopathy

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26
Q

Ezetimibe

A

Decreases intestinal absorption of cholesterol

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27
Q

Medullary thyroid cancer, pheochromocytoma, marfanoid habitus, and oral and intestinal mucosal neuromas

A

Multiple endocrine neoplasia type 2B

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28
Q

Sporadic and medullary thyroid cancers are associated with mutations in which gene?

A

RET proto-oncogene

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29
Q

Treatment for adrenal crisis

A

Immediate glucocorticoid supplementation and aggressive fluid resuscitation

30
Q

Severe hypotension, abdominal pain, vomiting, weakness, and fever

A

Adrenal crisis

31
Q

Complications of severe diabetic ketoacidosis

A

Pulmonary edema, respiratory fatigue, decreased mental status, which may lead to hypoventilation and subsequent hypercarbic respiratory failure

32
Q

Mechanism for treating hirsutism with combination OCPs

A

Suppress pituitary LH secretion and subsequently decrease ovarian androgen production

33
Q

MEN 1 Syndrome

A

Tumors of the pituitary, parathyroid gland, and pancreas

34
Q

Neurophysins

A

Carrier proteins for oxytocin and ADH. Produced within the neuronal cell bodies of hypothalamic nuclei.

35
Q

Clinical presentation of impaired beta oxidation of fatty acids

A

Hypoglycemia after prolonged fasting and inappropriately low levels of ketone bodies

36
Q

Most commonly deficient enzyme in impaired beta oxidation of fatty acids

A

Acyl-CoA dehydrogenase

37
Q

How do chronically elevated free fatty acid levels contribute to insulin resistance

A

Impairing insulin-dependent glucose uptake and increasing hepatic gluconeogenesis

38
Q

Erythrocytosis, testicular atrophy, acne, and virilization in women are sign os what?

A

Androgenic steroid abuse

39
Q

What drugs decrease peripheral conversion of T4 to T3?

A

Lipid-soluble beta blockers, glucocorticoids, PTU

40
Q

Cause of familial hypocalciuric hypercalcemia

A

Autosomal dominant disorder caused by defective calcium-sensing receptors in the parathyroid gland and kidneys

41
Q

What is a feared complication of antithyroid drugs (Methimazole, PTU)

A

Agranulocytosis - manifesting as fever, sore throat oral ulcerations

42
Q

Low plasma sodium and osmolality, inappropriately concentrated urine, clinically normal volume status (euvolemic hyponatremia)

A

Features of SIADH

43
Q

Glucocorticoids effect on the liver

A

Increase hepatic gluconeogenesis and glycogenesis

44
Q

What is responsible for the intracellular effects of the G protein-mediated adenylate cyclase second messenger system

A

Protein kinase A

45
Q

Pioglitazone

A

TZD. Exert glucose lowering effects by improving insulin sensitivity. Bind to PPAR-gamma, an intracellular nuclear receptor that acts as a transcription regulator of many genes in glucose and lipid metabolism

46
Q

Main side effects of TZDs

A

Fluid retention, with resultant weight gain and edema

47
Q

What are symptoms of vitamin D intoxication?

A

Mental status changes, muscle weakness, constipation, polyuria/polydipsia

48
Q

1-alpha-hydroxylase

A

The enzyme that converts 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D

49
Q

What do activated macrophages in sarcoidosis and other granulomatous diseases express?

A

1-alpha-hydroxylase

50
Q

Why is there a delay in action of TZDs?

A

The medication is dependent on alteration of gene expression and subsequent protein synthesis

51
Q

Pretibial myxedema and Graves ophthalmopathy

A

Features of Graves disease

52
Q

What causes the pretibial myxedema and opthalmopathy in Graves?

A

An autoimmune response directed against the TSH receptor that results in the accumulation of glycosaminoglycans within the affected tissue

53
Q

What are the acute effects of corticosteroids on the white blood cell count?

A

Increased neutrophil count and decreased lymphocyte, monocyte, basophil, and eosinophil counts

54
Q

17-alpha hydroxylase deficiency

A

Boys appear phenotypically female at birth, but girls develop normal genitalia. Patients typically present with hypogonadism, hypertension, and hypokalemia

55
Q

Functional hypothalamic amenorrhea

A

Loss of pulsatile gonadotropin-releasing hormone release from the hypothalamus. Caused by weight loss, strenuous exercise, systemic illness, or abnormal eating habits

56
Q

Cushing disease dexamethasone test results

A

ACTH is high and cortisol production can be suppressed by high-dose, but not low-dose dexamethasone

57
Q

Ectopic ACTH production dexamethasone test results

A

Cortisol and ACTH production are not suppressed by low or high dose dexamethasone

58
Q

What does long term glucocorticoid use do to the adrenal glands

A

Leads to bilateral adrenocortical atrophy involving the zona fasciculata and reticularis

59
Q

Hypoglycemia, ketoacidosis, hepatomegaly, muscle weakness, and hypotonia that present in infancy or childhood. There is cytosolic accumulation of limit dextrans

A

Debranching enzyme deficiency (Cori disease)

60
Q

Repaglinide mechanism

A

Act by binding to and closing the ATP-dependent K+ channel in pancreatic beta cell membrane, inducing depolarization and L-type calcium channel opening. Increased Ca2+ influx stimulates beta cell insulin release

61
Q

What is the predominant initial means of rapidly increasing blood glucose levels during hypoglycemia?

A

Glucagon-induced glycogenolysis by the liver

62
Q

What is the most common cause of adrenal insufficiency?

A

Suppression of the hypothalamus-pituitary-adrenal axis by glucocorticoid therapy

63
Q

What is the 2nd most common cause of congenital adrenal hyperplasia?

A

11 beta-hydroxylase deficiency

64
Q

Girl with ambiguous genitalia, hypertension, and hypokalemia

A

11 beta-hydroxylase deficiency

65
Q

Episodic HTN, tachycardia, headaches, diaphoresis, and tremors

A

Pheochromocytoma

66
Q

How is the diagnosis of pheochromocytoma confirmed?

A

Elevated levels of catecholamines and metanephrines in the urine or plasma

67
Q

Cause of primary hyperalosteronism

A

Excessive aldosterone secretion, typically as a result of bilateral nodular hyperplasia of the zona glomerulosa or an aldosterone producing adrenal adenoma

68
Q

What happens to thyroid hormone with increase in estrogen activity

A

There is an increase in thyroxine-binding globulin. This leads to increase in total thyroid hormone levels, but feedback control maintains normal free thyroid hormone

69
Q

Primary hyperparathyroidism skeletal manifestation

A

Osteitis fibrosa cystica - bone pain, subperiosteal erosions affecting the phalanges of the hand, a “salt and pepper” skull, and brown tumor bone cysts

70
Q

Pathway used by growth hormone

A

Binds cell surface receptors, leading to intracellular activation of the JAK-STAT pathway

Step 1 (Steffanie) (21 decks)

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