Endocrine - First Aid Flashcards

1
Q

The thyroid diverticulum arises from…

A

the floor of the primitive pharynx and descends into the neck.

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2
Q

The thyroid diverticulum is connected to the…

A

tongue by the thyroglossal duct, which normally disappears but may persist as a pyramidal lobe of the thyroid.

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3
Q

The normal remnant of the thyroglossal duct is the…

A

foramen cecum.

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4
Q

The most common ectopic thyroid tissue site is the…

A

tongue.

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5
Q

A thyroglossal duct cyst presents as…

A

an anterior midline neck mass that moves with swallowing or protrusion of the tongue.

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6
Q

A persistent cervical sinus leads to a…

A

branchial cleft cyst in the lateral neck.

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7
Q

The zona glomerulosa of the adrenal is regulated by…

A

renin-angiotensin and secretes aldosterone.

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8
Q

The zona fasiculata is regulated by…

A

ACTH, CRH and secretes cortisol (and sex hormones).

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9
Q

The zona reticularis is regulated by…

A

ACTH and CRH and secretes sex hormones (androgens).

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10
Q

The chromaffin cells of the medulla are regulated by…

A

preganglionic sympathetic fibers (ACh) and secrete catecolemines (NE and epinephrine).

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11
Q

Pheochromocytoma is the…

A

most common tumor of the adrenal medulla in adults and presents with episodic HTN.

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12
Q

Neuroblastoma is the…

A

most common tumor the adrenal medulla in children. It rarely causes hypertension.

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13
Q

The left adrenal gland is drained by the…

A

left adrenal vein to the left renal vein to the IVC.

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14
Q

THe right adrneal gland is drained by the…

A

right adrenal vein to the IVC.

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15
Q

The posterior pituitary (neurohypophysis) secretes…

A

vasopressin (aka ADH) and oxytocin.

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16
Q

Vasopressin and oxytocin are made in the…

A

hypothalamus and are shipped to the posterior pituitary via neurophysins.

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17
Q

The posterior pituitary is derived from…

A

neuroectoderm.

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18
Q

The anterior pituitary (adenohypophyisis) secretes…

A

FSH, LH, ACTH, TSH prolactin, GH and melanotropin (MSH).

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19
Q

Anterior pituitary is derived from…

A

oral ectoderm (Rathke pouch).

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20
Q

Acidophils

A

GH and prolactin

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21
Q

Basophils

A

FSH, LH, ACTH, TSH

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22
Q

The Islets of Langerhans are collections of…

A

alpha, beta, and delta endocrine cells arising from pancreatic buds.

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23
Q

alpha cells secrete…

A

glucagon.

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24
Q

beta cells secrete…

A

insulin

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25
Q

delta cells secrete…

A

somatostatin

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26
Q

Preproinsulin is synthesized in the…

A

RER and cleavage of the “presignal” leads to proinsulin (stored in secretory granules).

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27
Q

Cleavage of proinsulin leads to…

A

exocytosis of insulin and C-peptide equally.

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28
Q

Insulin and C-peptide are both increased in…

A

insulinoma whereas exogenous insulin lacks C-peptide.

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29
Q

Function of Insulin

A

Binds insulin receptors (tyrosine kinase activity), inducing glucose uptake (carrier-mediated) in insulin-dependent tissues and gene transcription.

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30
Q

Anabolic effects of Insulin (7)

A
  1. increased glucose transport in skeletal muscle and adipose tissue 2. increased glycogen synthesis and storage 3. increased triglyceride synthesis 4. increased Na+ retention (kidneys) 5. increased protein synthesis 6. increased cellular uptake of K+ and amino acids 7. decreased glucagon release
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31
Q

Unlike glucose, insulin does not…

A

cross the placenta.

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32
Q

Insulin dependent glucose transporters:

A

GLUT-4 (adipose tissue and skeletal muscle) These have lower Km and increased affinity.

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33
Q

Insulin independent transproters

A

-GLUT-1 (RBCs, brain, cornea) -GLUT-5 (fructose - spermatocytes, GI tract) -GLUT-2 (bidirectional - beta islet cells, liver, kidney, SI)

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34
Q

The brain utilizes glucose for…

A

metabolism normally and ketone bodies during starvation.

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35
Q

RBCs always utilize glucose because they…

A

lack mitochondria for aerobic metabolism.

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36
Q

The major regulator of insulin release is…

A

glucose. GH and beta2 agonists also increase insulin.

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37
Q

When glucose enters beta cells, there is…

A

increased ATP generated from glucose metabolism which closes K+ channels and depolarizes the beta cell membrane. This causes opening of voltage gated Ca2+ channels leading to calcium influx and stimulation of insulin exocytosis.

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38
Q

Function of Glucagon:

A

Catabolic effects: -glycogenolysis, gluconeogenesis -lipolysis and ketone production

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39
Q

Glucagon is secreted in response to…

A

hypoglycemia.

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40
Q

Glucagon is inhibited by…

A

insulin, hyperglycemia and somatostatin.

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41
Q

CRH increases…

A

ACTH, MSH, beta-endorphin.

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42
Q

CRH decreases in…

A

chronic exogenous steroid use.

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43
Q

Dopamine decreases…

A

prolactin.

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44
Q

Dopamine antagonists can cause…

A

galactorrhea.

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45
Q

GnRH increases…

A

FSH and LH.

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46
Q

GnRH is regulated by…

A

prolactin.

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47
Q

Tonic GnRH acts to…

A

suppress the HPA axis.

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48
Q

Pulsatile GnRH leads to…

A

puberty and fertility.

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49
Q

Prolactin decreases…

A

GnRH.

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50
Q

A pituitary prolactinoma leads to…

A

amenorrhea and osteoporosis.

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51
Q

Somatostatin decreases…

A

GH and TSH.

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52
Q

Somatostatin analogs are used to treat…

A

acromegaly.

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53
Q

TRH increases…

A

TSH and prolactin.

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54
Q

Prolactin stimulates…

A

milk production in the breast; it inhibits ovulation in females and spermatogenesis in males by inhibiting GnRH syntehsis and release.

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55
Q

Excessive amounts of prolactin are associated with…

A

decreased libido.

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56
Q

Prolactin secretion from the anterior pitutiary is regulated…

A

by tonic inhibition from dopamine from the hypothalamus.

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57
Q

Prolactin in turn inhibits…

A

its own secretion by increased dopamine synthesis and secretion from the hypothalamus.

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58
Q

GH stimulates…

A

linear growth and muscle mass through IGF-1/somatomedin secretion. It causes increased insulin resistance.

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59
Q

GH is released in…

A

pulses in response to GHRH.

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60
Q

Secretion of GH is increasd during…

A

exercise and sleep.

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61
Q

Secretion of GH is inhibited by…

A

glucose and somatostatin.

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62
Q

ADH is synthesized in the…

A

supraoptic nuclei of the hypothalamus.

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63
Q

ADH regulates…

A

serum osmolarity (V2 receptors) and blood pressure (V1 receptors).

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64
Q

Primary function of ADH is…

A

serum osmolarity regulation (ADH decreases serum osmolarity and increases urine osmolarity) via regulation of aquaporin channels in the principal cells of the renal collecting duct.

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65
Q

ADH is decreased in…

A

central diabetes insipidus but normal or increased in nephrogenic DI and primary polydipsia.

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66
Q

Nephrogenic DI can be caused by…

A

a mutation in the V2 receptor.

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67
Q

Desmopressin is an…

A

ADH analog used as treatment for central DI.

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68
Q

Regulation of ADH is…

A

primaryily via osmoreceptors in the hypothalamus and secondary through hypovolemia.

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69
Q

17alpha-hydroxylase deficiency steroid/hormone levels

A

-increased mineralocorticoids -decreased cortisol -decreased sex hormones

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70
Q

17alpha-hydroxylsae deficiency labs

A

-hypertension -hypokalemia -decreased DHT

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71
Q

Presentation of 17alpha-hydroxylase

A

XY: pseudohermaphroditism (ambiguous genitalia, undescended testes) XX: lack secondary sex devleopment

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72
Q

21-hydroxylase deficiency steroid/hormone levels

A

-decreased mineralocorticoids -decreased cortisol -increased sex hormones

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73
Q

21-hydroxylase deficiency labs

A

-hypotension -hyperkalemia -increased renin -increased 17-hydroxyprogesterone

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74
Q

Presentation of 21-hydoxylase deficiency

A

in infancy with salt wasting or childhood with precocious puberty XX: virilization

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75
Q

11beta-hydroxylase deficiency steroid/hormone levels

A

-decreased aldosterone -increased 11-deoxycortisone -decreased cortisol -increased sex hormones

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76
Q

Labs of 11beta-hydroxylase deficiency

A

hypertension (low renin)

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77
Q

Presentation of 11beta-hydroxylase deficiency

A

XX: virilization

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78
Q

All congenital adrenal enzyme deficiencies are characterized by an…

A

enlargment of both adrenal glands due to increased ACTH stimulation (due to decreased cortisol).

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79
Q

Functions of cortisol (6)

A
  1. increased bp 2. increased insulin resistance 3. increased gluconeogenesis, lipolysis and proteolysis 4. decreased fibroblast activity (causes striae) 5. decreased inflammatory/immune responses 6. decreased bone formation (decreased osteoblast activity)
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80
Q

Cortisol increases bp by…

A

upregulating alpha1 receptors on arterioles leading to increased sensitivity to NE and epinephrine.

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81
Q

Cortisol causes decreased inflammatory and immune responses (5):

A
  1. inhibits production of leukotrienes and prostaglandins 2. inhibits leukocyte adhesion (leading to neutrophilia) 3. blocks histamine release from mast cells 4. reduces eosinophils 5. blocks IL-2 production
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82
Q

Cortisol is bound to…

A

corticosteroid binding globulin (CBG).

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83
Q

Exogenous cortisol can cause…

A

latent reactivation of TB and candidiasis (due to blocked IL-2).

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84
Q

PTH is secreted by…

A

the chief cells of the parathyroid.

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85
Q

Functions of PTH

A
  1. increased bone resorption of calcium and phosphate 2. increased kidney reabsorption of calcium in DCT 3. decreased reabsorption of phosphate in the PCT 4. increased 1,25-(OH)2D3 (calcitriol) production by stimulating kidney 1alpha-hydroxylase
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86
Q

Overall, PTH acts to..

A

increase serum calcium, decrease serum phosphate and increased urine phosphate.

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87
Q

PTH increases the production of…

A

macrophage colony-stimulating factor and RANK-L (receptor activator of NF-kappaB)

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88
Q

RANK-L binds…

A

RANK on osteoblasts leading to osteoblast stimulation and increasd calcium.

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89
Q

PTH-related peptide (PTHrP) functions like…

A

PTH and is commonly increased in malignancies (ex. paraneoplastic syndrome).

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90
Q

PTH secretion is increased by…

A

decreased serum calcium or magnesium.

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91
Q

PTH secretion is decreased by…

A

greatly decreased serum magnesium.

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92
Q

Common causes of decreased magnesium include..

A

diarrhea, aminoglycosides, diruetics and alcohol abuse.

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93
Q

Plasma calcium exists in 3 forms:

A
  1. ionized (45%) 2. bound to albumin (40%) 3. bound to anions (15%)
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94
Q

An increase in pH leads to an increased affinity of…

A

albumin (negative charge) for calcium leading to clinical manifestations of hypocalcemia (cramps, pain, paresthesias, carpopedal spasm).

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95
Q

Vitamin D3 comes from…

A

sun exposure.

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96
Q

Vitamin D2 is ingested from…

A

plants.

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97
Q

Both D2 and D3 are converted to…

A

25-OH in the liver and to 1,25-(OH)2 (active form) in the kidney.

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98
Q

Function of Vitamin D

A
  1. increase the absorption of dietary calcium and phosphate 2. increase bone resorption leading to increased serum calcium/phosphate
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99
Q

Production of the active form of vitamin D is stimulated by…

A

increased PTH, decreased calcium, and decreased phosphate.

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100
Q

Deficiency of Vitamin D causes…

A

rickets in kids and osteomalacia in adults.

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101
Q

Vitamin D deficiency is caused by…

A

malabsorption, decreased sunlight, poor diet and chronic kidney failure.

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102
Q

Calcitonin comes from the…

A

parafollicular (c cells) of the thyroid.

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103
Q

Function of calcitonin

A

decrease bone resorption of calcium

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104
Q

Calcitonin secretion is stimulated by…

A

increased serum calcium.

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105
Q

Calcitonin opposes the actions of…

A

PTH. It is not important in normal calcium homeostasis.

106
Q

cAMP is the signaling pathway for the following endocrine hormones:

A
  1. FSH 2. LH 3. ACTH 4. TSH 5. CRH 6. hCG 7. ADH 8. MSH 9. PTH 10. calcitonin 11. GHRH 12. glucagon
107
Q

cGMP is the signaling pathway for the following endocrine hormones:

A
  1. ANP 2. NO
108
Q

IP3 is the signaling pathway for the following endocrine hormones:

A
  1. GnRH 2. oxytocin 3. ADH 4. TRH 5. Histamine 6. AngII 7. Gastrin
109
Q

Steroid receptor is the signaling pathway for the following endocrine hormones:

A
  1. Vitamin D 2. Estrogen 3. Testosterone 4. T3/T4 5. Cortisol 6. Aldosterone 7. Progesterone
110
Q

Intrinsic tyrosine kinase is the signaling pathway for the following endocrine hormones:

A
  1. insulin 2. IGF-1 3. FGF 4. PDGF 5. EGF
111
Q

Receptor associated tyrosine kinase is the signaling pathway for the following endocrine hormones:

A
  1. Prolactin 2. immunomodulators 3. GH
112
Q

Steroid hormones are lipophilic and therefore must…

A

circulate bound to specific binding globulins which increase their solubility.

113
Q

In men, increased SHBG leads to…

A

lower free testosterone leading to gynecomastia.

114
Q

In women, decreased SHBG leads to…

A

increased free testosterone leading to hirsutism.

115
Q

SHBG is increased by…

A

OCPs and pregnancy. (free estrogen levels remain the same)

116
Q

T3 and T4 are…

A

iodine-containing hormones that control the body’s metabolic rate

117
Q

T3/T4 come from..

A

the follicles of the thyroid.

118
Q

Functions of T3/T4

A
  1. bone growth 2. CNS maturation 3. increased beta1 receptors in the heart (increased CO, HR, SV, contractility) 4. increased metabolic rate via increased Na+/K+ ATPase activity (increased O2 consumption, RR and temp) 5. increased glycogenolysis, gluconeogenesis and lipolysis
119
Q

TBG binds…

A

almost all T3/T4 in the blood. Only free hormone is active.

120
Q

The major thyroid product is…

A

T4. It is converted into T3 in the peripheral tissue by 5’-deiodinase. T3 binds receptors with greater affinity.

121
Q

Peroxidase is the enzyme responsible for…

A

oxidation and organification of iodide as well as coupliting of MIT and DIT.

122
Q

Propylthiouracil inhibtis both…

A

peroxidase and 5’-deiodinase. Methimazole inhibits peroxidase only.

123
Q

3 causes of Cushing Syndrome

A
  1. exogenous corticosteroids (#1 cause) 2. primary adrenal adenoma, hyperplasia or carcinoma 3. ACTH-secreting pituitary
124
Q

Exogenous corticosteroids result in..

A

decreased ACTh and bilateral adrenal atrophy.

125
Q

Primary adrenal adenoma, hyperplasia or carinoma results in…

A

decreased ACTH and atrophy of the uninvolved adrenal gland. This can also present as primary aldosteronism (Conn Syndrome).

126
Q

ACTH-secreting pituitary adenoma is true…

A

Cushing disease.

127
Q

An ACTH secreting pituitary adenoma or paraneoplastic ACTH secretion (SCLC or bronchial carcinoids) results in…

A

increased ACTH, bilateral adrneal hyperplasia.

128
Q

Findings of Cushing Syndrome

A

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

129
Q

Screening tests for Cushing’s include:

A
  1. increased free cortisol on 24 hr U/A 2. midnight salivary cortisol 3. overnight low-dose dexamethasone suppression test
130
Q

Primary hyperaldosteronism is caused by…

A

adrenal hyperplasia or an aldosterone-secreting adrenal adenoma (Conn syndrome) resulting in HTN, hypokalmeia, metabolic alkalosis and low plasma renin.

131
Q

In primary hyperaldosteronism, sodium is…

A

normal due to aldosterone escape.

132
Q

Treatment for primary hyperaldosteronism

A

surgery to remove tumor and/or spironolactone

133
Q

Secondary hyperaldosteronism is the…

A

renal perception of low intravascular volume resulting in an overactive RAAS. High plasma renin.

134
Q

Secondary hyperaldosteronism is due to…

A

renal artery stenosis, CHF, cirrhosis or nephrotic syndrome.

135
Q

Treatment for secondary hyperaldosteronism

A

spironolactone

136
Q

Addison Disease is…

A

primary adrenal insufficiency due to adrenal atrophy or destruction.

137
Q

In Addison disease, there is a deficiency of…

A

aldosterone and cortisol casuing hypotension, hyperkalemia, acidosis and skin/mucosal hyperpigmentation.

138
Q

Secondary adrenal insufficiency (decreased pituitary ACTH production) has no…

A

skin hyperpigmentation and no hyperkalemia.

139
Q

Waterhouse-Friderichsen Syndrome is…

A

acute primary adrenal insufficiency due to adrenal hemorrhage associated with Neisseria meningitidis septicemia, DIC and endotoxic shock.

140
Q

Neuroblastoma results from…

A

neural crest cells and occurs anywhere along the sympathetic chain.

141
Q

Neuroblastoma most commonly presents as…

A

abdominal distension and a firm, irregular mass that can cross the midline.

142
Q

Lab results of neuroblatoma

A

-increased HVA in urine (breakdown product of dopamine) -bombesin + -overexpression of N-myc

143
Q

Neuroblastoma histology

A

-rosettes and small, round, blue/purple nuclei

144
Q

Pheochromocytoma is derived from…

A

adrenal chromaffin cells (from neural crest).

145
Q

Pheochromocytoma rule of…

A

10s: 10% malignant 10% bilateral 10% extra-adrenal 10% calcify 10% kids

146
Q

Most pheochromocytomas secrete…

A

epinephrine, norepinephrine, and dopamine which can cause episodic HTN.

147
Q

Pheochromocytomas are associated with…

A

von Hippel-Lindau disease, MEN 2A and 2B.

148
Q

Pheochromocytoma Findings

A

-urinarry VMA (breakdown product of NE and epinephrine) -increased serum catecholamines

149
Q

Treatment of Pheochromocytoma

A
  1. irreversible alpha-antagonists (phenoxybenzamine) 2. then beta-blockers 3. then surgery (alpha then beta to aovid hypertensive crisis)
150
Q

Hypothyroidism signs/symptoms (8)

A
  1. cold intolerance 2. weight gain, decreased appetite 3. hypoactivity, lethargy, fatigue 4. constipation 5. decreased reflexes 6. myxedema (facial/periorbital) 7. dry, cool skin; coarse, brittle hair 8. bradycardia, dyspnea on exertion
151
Q

Hyperthyroidism signs/symptoms (8)

A
  1. heat intolerance 2. weight loss, increased appetite 3. hyperactivity 4. diarrhea 5. increased reflexes 6. pretibial myxedema 7. warm, moist skin; fine hair 8. chest pain, palpitations, arrhythmias
152
Q

Lab findings of hypothyroidism

A

-increased TSH -decreased T3/T4 -hypercholesterolemia (due to decreased LDL receptor)

153
Q

Lab findings of hyperthyroidism

A

-decreased TSH -increased free/total T3/T4 -hypocholesterolemia

154
Q

Hashimoto thyroiditis is…

A

an autoimmune hypothyroidism due to antithyroid peroxidase and antithyroglobulin antibodies.

155
Q

Hashimoto thyroiditis is associated with…

A

HLA-DR5 and increased risk of non-Hodgkin lymphoma.

156
Q

Hashimoto may be hyperthyroid early in the course due to…

A

thyrotoxicosis durin gfollicular rupture.

157
Q

Histologic findings of Hashimoto

A

-Hurthle cells -lymphoid aggregate with germinal centers

158
Q

In hashimoto, the thyroid will be…

A

moderately enlarged, nontender.

159
Q

Congenital hypothyroidism (cretinism)

A

severe fetal hypothyroidism due to maternal hypothyroidism, thyroid agenesis, thyroid dysgenesis, iodine deficiency or dyshormongenic goiter.

160
Q

Findings of Congential Hypothyroidism

A

6 Ps 1. Pot-bellied 2. Pale 3. Puffy-faced child 4. Protruding umbilicus 5. Protuberant tongue 6. Poor brain development

161
Q

Subacute thyroiditis de Quervain is…

A

self-limited hypothyroidism following a flu-like illness.

162
Q

Histology of Subacute thyroiditis de Quervain

A

granulomatous inflammation

163
Q

Riedel thyroiditis is…

A

thyroid replaced by fibrous tissue (hypothyroid). The fibrosis may extend to local structures miimicking anaplastic carcinoma.

164
Q

Riedel thyroiditis is considered a manifestation of…

A

IgG4 related systemic disease.

165
Q

Findings of Riedel thyroiditis

A

-fixed, hard painless goiter

166
Q

Toxic multinodular goiter (hyperthyroidism)

A

focal patches of hyperfuncitoning follicular cells working independently of TSH due to mutation in TSH receptor; increases release of T3 and T4

167
Q

Jod-Basedow phenomenon

A

thyrotoxicosis if a pt with idoine deficiency goiter is made iodine replete

168
Q

Graves disease is a form of…

A

hyperthryoidism due to autoantibodies (IgG) that stimulate TSH receptors on the thyroid, retro-orbital fibroblasts (exophthalmos, proptosis) and dermal fibroblasts (pretibilal myxedema)

169
Q

Graves disease often presents during…

A

stress (childbirth).

170
Q

A thyroid storm is a…

A

stress-induced catecholamine surge seen as a serious complication of Graves and other hyperthyroid disease.

171
Q

Thyroid storm presents with…

A

agitation, delirium, fever, diarrhea, coma and tachyarrhythmia. May be increased ALP.

172
Q

Treat thyroid storm with…

A

the 3 Ps: 1. Propranolol (beta-blockers) 2. Propylthiouracil 3. Prednisolone (corticosteroids)

173
Q

Multinodular goiter histology

A

follicles of various sizes distended with colloid and lined by flattened epithelium with areas of fibrosis and hemorrhage

174
Q

Complications of thyroidectomy include:

A
  1. hoarseness (recurrent laryngeal nerve damage) 2. hypocalcemia (removal of parathyroids) 3. transection of inferior thyroid artery
175
Q

Papillary thyroid carcinoma

A

-empty appearing nuclei (orphan annie eyes) -psammoma bodies -nuclear grooves

176
Q

There is increased risk for papillary thyroid carcinoma with…

A

RET and BRAF mutations and childhood irradiation.

177
Q

Follicular thyroid carcinoma

A

-invades thyroid capsule -uniform follicles

178
Q

Medullary thyroid carcinoma

A

-from parafollicular C cells -produces calcitonin -sheets of cells in an amyloid stroma

179
Q

Medullary thyroid carcinoma is associated with…

A

MEN 2A and 2B (RET mutations).

180
Q

Undifferentiated/anaplastic thyroid carcinoma

A

-older patients -invades local structures

181
Q

Lymphoma of the thyroid is associated with..

A

Hashimoto.

182
Q

Primary hyperparathyroidism is usually due to an…

A

adenoma and causes: -hypercalcemia -hypercalciuria (stones) -hypophosphatemia -increased PTH -increased ALP -increased cAMP in urine

183
Q

Primary hyperparathyroidism may present with…

A

weakness, constipation, flank/abdominal pain, and depression.

184
Q

Osteitis fibrosa cystica is…

A

cystic bone spaces filled with brown fibrous tissue. Associated with primary hyperparathyroidism.

185
Q

Secondary hyperparathyroidism is…

A

secondary hyperplasia due to decreaesd gut calcium absorption and increased phosphate. This most often occurs in chronic renal disease.

186
Q

Findings of secondary hyperparathyroidism include…

A

hypocalcemia, hyperhophsatemia, increased ALP and increased PTH.

187
Q

Tertiary hyperparathyroidism is…

A

refractory hyperparathryoidism from chronic renal disease. Has greatly increased PTH and increased calcium.

188
Q

Renal osteodystrophy is…

A

bone lesions due to secondary or tertiary hyperparthyroidism.

189
Q

Hypoparathyroidism is due to…

A

accidental surgical excision of parathyroid glands, autoimmune destruction or DiGeorge.

190
Q

Findings of Hypoparathyroidism

A

-hypocalcemia -tetany -Chvostek sign -Troussea sign

191
Q

The chvostek sign is…

A

tapping of the facial nerve causes contraction of facial muscles.

192
Q

The trousseau sign is…

A

occlusion fo the brachial artery with a bp cuff leads to carpal spasm.

193
Q

Pseudohypoparathyroidism (Albright hereditary osteodystrophy) is an…

A

autosomal dominant unresponsiveness of the kidney to PTH. Presents with hypocalcemia, shortened 4th/5th digit and short stature.

194
Q

The most common pituitary adenoma is a…

A

prolactinoma.

195
Q

Nonfunctional pituitary adenomas present with…

A

mass effect (bitemporal hemianopia, hypopituitarism and headache).

196
Q

Functional pituitary adenoma presentationis dependent on…

A

the hormone produced: -prolactinoma: amenorrhea, galactorrhea, low libido, infertility -somatropic adenoma: acromegaly

197
Q

Treatment of prolactinoma

A

dopamine agonists (bromocriptine or cabergoline)

198
Q

Acromegaly is…

A

excess GH in adults typically caused by pituitary adenoma

199
Q

Findings of acromegaly (5)

A

-large tongue with deep furrows -deep voice -large hands and feet -coarse facial features -imparied glucose tolerance

200
Q

Diagnose acromegaly with…

A

increased serum IGF-1; failure to suppress serum GH following oral glucose tolerance test; pituitary mass seen on brain on MRI.

201
Q

Treatment for acromegaly

A

-pituitary adenoma resection -treat with octreotide or pegvisomant

202
Q

Increased GH in children leads to…

A

gigantism (increased linear bone growth); cardiac failure is the most common cause of death

203
Q

Diabetes insipidus is characterized by…

A

intense thirst and polyuria with an inability to concentrate urine due to lack of ADH.

204
Q

Central DI is due to…

A

pituitary tumor, autoimmune, trauma, surgery, encephalopathy

205
Q

Findings of Central DI

A

-decreased ADH -urine specific gravity < 1.006 -serum osmolarity > 290 -hyperosomotic volume contraction

206
Q

Diagnosis of Central DI

A

water restriction test: >50% increase in urine osmolarity

207
Q

Treatment of Central DI

A

-intranasal DDAVP -hydration

208
Q

Nephrogenic DI is due to…

A

hereditary ADH receptor mutation, secondary to hypercalcemia, lithium or demeclocycline.

209
Q

Findings of Nephrogenic DI

A

-normal ADH levels -urine specific gravity < 1.006 -serum osmolarity > 290 -hyperosomtic volume contraction

210
Q

Diagnosis of Nephrogenic DI

A

water restriction test: no change in urine osmolarity

211
Q

Treatment of Nephrogenic DI

A

-HCTZ -indomethacin -amiloride -hydration

212
Q

The water restriction test is…

A

no water intake for 2-3 hrs followed by hourly measurements of urine volume, osmolarity and plasma sodium and osmolarity.

213
Q

SIADH features (3):

A
  1. excessive water retention 2. hyponatremia with continued urinary Na+ excretion 3. urine osmolarity > serum osmolarity
214
Q

In SIADH, the body responds to water retention with…

A

decreased aldosterone (which causes hyponatremia) to maintain near normal volume status.

215
Q

In SIADH, very low serum soidum levels can lead to…

A

cerebral edema and seizures.

216
Q

SIADH should be corrected…

A

slowly to prevent cetnral pontine myelinosis.

217
Q

Causes of SIADH (4):

A
  1. ectopic ADH (small cell lung carcinoma) 2. CNS disorders/head trauma 3. pulmonary disease 4. drugs
218
Q

Treatment of SIADH:

A
  1. fluid restriction 2. IV hypertonic saline 3. Conivaptan 4. Tolvaptan 5. demeclocycline
219
Q

Hypopituitarism is uncersecretion of the pituitary hormones due to:

A

-nonsecreting pituitary adenoma, craniopharyngioma -Sheehan syndrome -empty sella syndrome -brain injury, hemorrhage -radiation

220
Q

Empty sella syndrome is…

A

atrophy or compression of the pituitary common in obese women.

221
Q

Treatment for hypopituitarism

A

HRT (corticosteroids, thyroxine, sex steroids, GH)

222
Q

Acute manifestations of diabetes mellitus

A

-polydipsia -polyuria -polyphagia -weight loss -DKA (type 1) -hyperosmolar coma (type 2)

223
Q

Nonenzymatic glycosylation due to chronic DM leads to:

A
  1. small vessel disease (diffuse thickening of the basement membrane) 2. large vessel atherosclerosis, CAD, peripheral vascular occlusive disease and gangrene
224
Q

Small vessel disease in DM leads to…

A
  1. retinopathy (hemorrhage, exudates, microaneurysms, vessel proliferation) 2. glaucoma 3. nephropahty (nodular sclerosis, progressive proteinuria, chronic renal failure, arteriolosclerosis, HTN, Kimmelstiel-Wilson nodules)
225
Q

In DM, large vessel atherosclerosis, CAD, peripheral vascular occlusive disease and gangrene lead to…

A

limb loss, cerebrovascular disease.

226
Q

Osmotic damage in chronic DM is due to…

A

sorbitol accumulation in organs with aldose reductase and decreased sorbitol dehyrogenase. It causes: -neuropathy -cataracts

227
Q

Tests for Diabetes Mellitus

A
  1. fasting serum glucose (> 126) 2. oral glucose tolerance test 3. HbA1c (reflects average blood glucose over prior 3 months)
228
Q

Primary defects of T1 and T2 DM

A

T1: autoimmune destruction of beta cells T2: increased resistance to insulin, progressive pancreatic beta-cell failure

229
Q

Treatment with insulin in T1 and T2 DM

A

T1: always T2: sometimes

230
Q

Age of T1 and T2 DM

A

T1: less than 30 T2: more than 40

231
Q

Association of T1 and T2 DM with obesity

A

T1: no T2: yes

232
Q

Genetic predisposition of T1 and T2 DM

A

T1: relatively weak (50% concordance in identical twins), polygenic T2: relatively strong (90% concordance in identical twins), polygenic

233
Q

Association of T1 and T2 DM with HLA system

A

T1: HLA DR3 and 4 T2: none

234
Q

Glucose intolerance of T1 and T2 DM

A

T1: severe T2: mild to moderate

235
Q

Insulin sensitivity of T1 and T2 DM

A

T1: high T2: low

236
Q

Ketoacidosis T1 and T2 DM

A

T1: common T2: rare

237
Q

beta-cell numbers in the islets of T1 and T2 DM

A

T1: decreased T2: variable (with amyloid deposits)

238
Q

Serum insulin level of T1 and T2 DM

A

T1: decreased T2: variable

239
Q

Classic symptoms of polyuria, polydipsia, polyphagia and weight loss in T1 vs. T2 DM

A

T1: common T2: sometimes

240
Q

Histology of T1 and T2 DM

A

T1: islet leukocyte infiltrate T2: islet amyloid polypeptide (IAPP) deposits

241
Q

Diabetic ketoacidosis is usually due to…

A

increased insulin requirements from increased stress (infxn). It leads to excess fat breakdown and increased ketogenesis from increased free fatty acids, which are then made into ketone bodies.

242
Q

Signs/symptoms of diabetic ketoacidosis (6)

A

-Kussmaul respirations (rapid/deep breathing) -nausea/vomiting -abdominal pain -psychosis/delirium -dehydration -fruity breath odor (due to exhaled acetone)

243
Q

Diabetic Ketoacidosis Labs

A

-hyperglycemia -increased H+ -decreased HCO3- (anion gap metabolic acidosis) -increased ketone levels -leukocytosis -hyperkalemia (with depleted intracellular K+)

244
Q

Complications of diabetic ketoacidosis

A

-life threatening mucormycosis (usually caused by Rhizopus infxn) -cerebral edema -cardiac arrhythmias -heart failure

245
Q

Treatment for diabetic ketoacidosis

A

-IV fluids -IV insulin -K+ -glucose if necessary

246
Q

Insulinoma is a…

A

tumor of the beta cells of the panreas taht leads to hypoglycemia.

247
Q

Insulinoma presents with the…

A

whipple triad of episodic CNS symptoms (lethargy, syncope, and diplopia)

248
Q

Symptomatic insulinoma pts have…

A

decreased blood glucose and increaesed C-peptide levels.

249
Q

Carcinoid syndrome is caused by…

A

carcinoid tumors (neuroendocrine cells) esp. metastatic small bowel tumors which secrete high levels of serotonin.

250
Q

In carcinoid syndrome, high serotonin is not seen if…

A

the tumor is limited to the GI tract bc 5-HT undergoes first-pass metabolism.

251
Q

Carcinoid syndrome results in…

A

-diarrhea -flushing -asthmatic wheezing -right-sided valvular disease

252
Q

Labs for Carcinoid syndrome

A

-increased 5-HIAA in urine -niacin deficiency

253
Q

Treatment for Carcinoid syndrome

A

-resection -somatostatin analog

254
Q

Zollinger-Ellison syndrome is…

A

a gastrin-secreting tumor of the pancreas or duodenum. May be associated with MEN 1.

255
Q

In ZE syndrome, acid hypersecretion causes…

A

recurrent ulcers in the distal duodenum and jejunum.

256
Q

ZE syndrome presents with…

A

abdominal pain and diarrhea.

257
Q

MEN 1 Characteristics

A
  1. parathyroid tumors 2. pituitary tumors 3. pancreatic endocrine tumors (ZE, insulinomas, VIPomas, glucagonomas)
258
Q

MEN1 commonly presents with…

A

kidney stones and stomach ulcers.

259
Q

MEN 2A characteristics

A
  1. medullary thyroid carcinoma 2. pheochromocytoma 3. parathyroid hyperplasia
260
Q

MEN 2B charcteristics

A
  1. medullary thryoid carcinoma 2. pheochromocytoma 3. oral/intestinal ganglioneuromatosis (associated with Marfanoid habitus)
261
Q

MEN 2A and 2B are associated with…

A

ret gene mutations.