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uworld > endocrine, diabetes, metabolism > Flashcards

endocrine, diabetes, metabolism Flashcards

1
Q

what is the single most effective preventative measure in almost all patients, especially those with diabetes

A

smoking cesation

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2
Q

how will free and total T4 levels and TSH levels be altered in primary hypothyroidism

A

low free and total T4 and increased TSH

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3
Q

how will free and total T4 levels and TSH levels be altered in thyroid hormone resistance

A

high TSH and T4

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4
Q

how will free and total T4 levels and TSH levels be altered in central hypothyroidism

A

low TSH and T4

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5
Q

in transient hypothyroidism during pregnancy, how are free and total T4 levels and TSH levels affected

A

decreased free and total T4 and increased TSH

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6
Q

if a new born presents with elevated serum TSH and decreased T4, and a normal size and normally located thyroid, what is the likely diagnosis

A

thyroid gland resistance to TSH

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7
Q

TSH is normally produced where?

A

anterior pituitary gland

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8
Q

name the 3 layers of the cortex of the adrenal gland starting most outward to inward

A

zona glomerulosa
zona fasciculata
zona reticularis

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9
Q

what do the cells in the zona glomerulosa secrete

A

mineral corticoids, primarily aldosterone

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10
Q

what do cells in the zona fasciculata secrete

A

glucocorticoid hormones, primarily cortisol

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11
Q

what do cells in the zona reticularis secrete

A

androgens

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12
Q

the adrenal medulla is sharply demarcated from the cortex and is composed of _____ cells with a deeply basophilic cytoplasm

A

chromaffin

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13
Q

chromaffin cells are modified neuroendocrine cells derived from ____

A

neural crest

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14
Q

chromaffin cells are stimulated by ______

A

acetylcholine

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15
Q

chromaffin cells are stimulated by acetylcholine released by sympathetic preganglionic neurons and secrete _____

A

catecholamines (80% epinephrine, 20% norepinephrine)

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16
Q

the superior thyroid artery and vein and what nerve travel together in a neurovascular triad that originates superior to the thyroid gland and lateral to the thyroid cartilage

A

external branch of the superior laryngeal nerve

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17
Q

injury to the external branch of the superior laryngeal nerve during a thyroidectomy may result in what symptoms due to loss of function of what muscle

A

low, hoarse voice with limited range pitch

cricothyroid muscle

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18
Q

raloxifene MOA

A

selective estrogen receptor modulator

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19
Q

tamoxifen MOA

A

selective estrogen receptor modulator

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20
Q

what are adverse effects associated with selective estrogen receptor modulators

A

hot flashes
venous thromboembolism
endometrial hyperplasia and carcinoma (tamoxifen only)

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21
Q

where is epinephrine primarily produced

A

adrenal medulla

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22
Q

what is the rate limiting step in catecholamine synthesis

A

conversion of tyrosine to dihydroxyphenylalanine (DOPA) by tyrosine hydroxylase

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23
Q

in the adrenal medulla, norepinephrine is quickly converted to epinephrine via what enzyme

A

phenylethanolamine-N-methyltransferase (PNMT)

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24
Q

PNMT expression in the adrenal medulla is upregulated by what?

A

cortisol

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25
Q

following pituitary resection, the loss of ____ results in decreased synthesis of cortisol in the adrenal cortex which results in decreased ______ activity leading to low conversion of norepinephrine to epinephrine

A

ACTH

PNMT

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26
Q

medullary carcinoma, a primary thyroid carcinoma, is derived from what cell type

A

parafollicular calcitonin-secreting C cells

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27
Q

what is the most common type of primary thyroid carcinoma

A

papillary

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28
Q

_____ carcinoma cells are characteristically large with overlapping nuclei containing finely dispersed chromatin, giving them an empty or ground glass appearance (Orphan Annie eye nucleus). numerous intranuclear inclusions and grooves may be found. Psammoma bodies may be found within the tumor.

A

papillary

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29
Q

a majority of T4 is bound to ____

A

thyroxine binding globulin (TBG)

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30
Q

to patients with congenital thyroxine binding globulin deficiency require treatment

A

no
total T4 is low because decreased fraction of bound T4 but feedback on the thyroid still functions properly to keep free T4 levels constant

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31
Q

what presents as painful thyroid enlargement with transient hyperthyroid symptoms following the onset of a viral illness

A

subacute granulomatous thyroiditis

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32
Q

what are diagnostic tests with subacute granulomatous thyroiditis

A

elevated ESR & CRP

decreased radioiodine uptake

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33
Q

pathology of subacute granulomatous thyroiditis shows inflammatory infiltrate with ____ and ___ cells

A

macrophage

giant cells

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34
Q

what condition is an autoimmune condition that causes painless thyroid enlargement and presents with predominantly hypothyroid features

A

Hashimoto thyroiditis

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35
Q

pathology of Hashimoto thyroiditis shows lymphocytic infiltrate with well-developed _____ and ____ cells (eosinophilic epithelial cells)

A

germinal centers

Hurthle

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36
Q

what is the Whipple triad

A

symptoms consistent with hypoglycemia (tremor, diaphoresis, confusion)
low blood glucose level
relief of symptoms when glucose is administered

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37
Q

nocturnal back pain, spinal tenderness, and indurated prostate suggests what diagnosis

A

prostate adenocarcinoma with bony metastases

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38
Q

leuprolide MOA

A

GnRH analog ( treats prostate cancer)

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39
Q

what are androgen levels in a patient starting leuprolide

A 
transiently elevated (GnRH analog)
then decrease as it leads to downregulation of GnRH receptors
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40
Q

in a patient with an insulin deficiency, how can glucose be formed in the liver

A

triglycerides in adipose tissue is degraded and produces glycerol which can be converted to glucose in gluconeogenesis via glycerol kinase

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41
Q

How does testosterone use alter serum LH

A

Decreases serum LH

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42
Q

How does testosterone use alter serum estrogen

A

Increases

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43
Q

How does testosterone use aler spermatogenesis

A

Decreases spermatogenesis

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44
Q

Spermatogenesis is driven by what hormone

A

FSH

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45
Q

Testosterone is produced by ____ cells in the testes

A

Leydig cells

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46
Q

Excess testosterone is converted by aromatase to ____

A

Estradiol

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47
Q

The thyroid gland is formed from evagination of the ____

A

Pharyngeal epithelium

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48
Q

The thryoid gland descends to the lower neck. Failure of migration can cause the thyroid to reside anywhere along the _________’s usual path

A

Thyroglossal duct

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49
Q

The thyroglossal duct extends from the ____ on the dorsal surface of the tongue to the superior border of the thyroid isthmus

A

Foramen cecum

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50
Q

Primary adrenal insufficieny is also called what

A

Addison disease

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51
Q

In Addison disease, how is serum sodium and urine sodium altered

A

Decreases
Increased
(Decreased aldosterone –> renal salt wastin)

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52
Q

In Addison disease, how is serum and urine potassium effected?

A

Increased
Decreased
(In renal collecting duct principal cells, decreased aldosterone –> increased potassium absorption)

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53
Q

Low cortisol levels in Addison disease stimulates increased _____ which leads to water retention and hyponatremia

A

ADH

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54
Q

Paraneoplastic hypercortisolim, most commnly caused by small cell lung cancer, is due to ectopic ____ secretion

A

ACTH

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55
Q

What common feature of Cushing syndrome is uncommon in paraneoplastic hypercortisolism

A

Central obesity

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56
Q

Hyperthyroidism causes upregulation of ________ leadng to increased catecholamine effect

A

Beta adrenergic receptor expression

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57
Q

Beta blockers are used to blunt adrenergic manifesttions of hyperthyroidism as wll as reduce _______ activity in peripheral tissues

A

5’-monodeiodinase

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58
Q

Familial chylomicronemia syndrome(type I hyperlipoproteinemia) is due to a defect in what protein(s)

A

Lipoprotein lipase

ApoC-2

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59
Q

What lipoproteins are elevated in familial chylomicrnoemia syndrome (type I hyperlipoproteinemia)

A

Chylomicrons

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60
Q

What are the major manifestations offamilial chylomicronemia syndrome (type I hyperlipoproteinemia)

A

Acute pancreatitis
Lipemia retinalis
Eruptive xanthomas

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61
Q

What proteins are defected ini familial hypercholesterolemia (type II A hyperlipoproteinemia)

A

LDL receptor

ApoB-100

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62
Q

What lipoproteins are elevated in familial hypercholesterolemia (type II A hyperlipoproteinemia)

A

LDL

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63
Q

What are the major manifestations of familial hypercholesterolemia (type II A hyperlipoproteinemia)

A

Premature atherosclerosis
Tendon xanthomas
Xanthelasmas

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64
Q

What proteins are defected in familial dysbetalipoproteinemia (type III hyperlipoproteinemia)

A

ApoE

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65
Q

What lipoproteins are elevated in familial dysbetalipoproteinemia (type III hyperlipoproteinemia)

A

Chylomicron

VLDL remanants

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66
Q

What are the major manifestations of familial dysbetalipoproteinemia (type III hyperlipoproteinemia)

A

Premature aterosclerosis

Tuboeruptive and palmar xanthomas

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67
Q

What protein is defective in familial hypertriglyceridemia (type IV hyperlipoproteinemia)

A

Polygenic

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68
Q

What lipoproteins are elevated in familial hypertriglyceridemia (type IV hyperlipoproteinemia)

A

VLDL

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69
Q

What are the clinical manifestations of familial hypertriglyceridemia (type IV hyperlipoproteinemia)

A

Associated with coronary disease, pancreatitis, and diabetes

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70
Q

Where are ApoE3 and ApoE4 found

A

On chylomicrons and VLDLs

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71
Q

ApoE3 and ApoE4 function

A

Bind hepativ apolipoprotein receptors (allow liver to remove chylomicrons and VLDLs from circulation)

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72
Q

How is hyperprolactinemia treated (ex. Prolactinoma)

A

Dopamine agonist (cabergoline, bromocriptine)

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73
Q

Diabetes insipidus is due to impaired activity of what hormone

A

ADH

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74
Q

Injection of exogenous ADH can help distinguish between central and nephrogenic diabetes insipidus. Which will have an increase in urine osmolality

A

Central diabetes insipidus

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75
Q

_____ injury results in dath of magnocellular neurons, causing permanent central diabetes insipidus

A

Hypothalamic

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76
Q

Damage to ___ causes transient diabetes insipidus because the cell bodies of the magnocellular neurona remain intact

A

More distal portions of the hypothalamic hypophyseal tract (below the infundibulum)

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77
Q

what may present months after birth with an enlarged fontanelle, lethargy, poor feeding, protruding tongue, puffy face, umbilical hernia, and constipation

A

congenital hypothyroidism

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78
Q

ADH administration substantially increases urine osmolality in patients with ____ diabetes insipidus (central or nephrogenic)

A

Central

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79
Q

Permanant central DI is due to injury where? while transient central diabetes insipidus is due to damage where?

A

Permanent: hypothalamic injury –> death of magnocellular neurons
Transient: distal portions of hypothalamic-hypophyseal tract (below infundibulum) –> madocellular neurons remain intact

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80
Q

How does hyperthyroidism caus osteoporosis

A

T3 stimulates osteoclast differentiation –> increased bone resorption –> release of calcium

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81
Q

Insulin causes activation of PFK-1 or PFK-2

A

PFK-2

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82
Q

Insulin causes activation of PFK-2 leading to increased levels of ______ which ihibits gluconeogenesis

A

Fructose 2,6-bisphosphate

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83
Q

High levels of fructose 2,6-bisphosphate inhibit gluconeogenesis leading to decreased conversion of alanine and other substrates to ____

A

Glucose

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84
Q

What is the likely diagnosis of a patient with severe hypertension, headaches, and an adrenal mass

A

Pheochromocytoma

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85
Q

Pheochromocytoma is a catecholamine-secreting tumor of ___ cells in the adrenal ____

A

Chromaffin

Medulla

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86
Q

Multiple endocrine neoplasia (MEN) type 2 is associated with germ-line mutations in the ____ gene

A

RET

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87
Q

What are the characteristics of MEN type 2

A

Pheochromocytomas
Medulary thyroid cancer
Either parathyroid hyperplasia (MEN 2A) or mucosal neuromas and marfanoid habitus (MEN 2B)

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88
Q

The chromaffin cells of the adrenal medulla re derived from ____ tissue

A

Neural crest

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89
Q

The adrenal cortex is derived from _____

A

Mesoderm

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90
Q

Medullary thryoid cancer is a malignancy of ____ cells

A

Parafollicular C

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91
Q

What are the structures arising from neural crest cells

A 
"SOME SALTS"
Schwann cells
Odontoblasts
Melanocytes
Enterochromaffin cells
Spinal membranes (pia and arachnoid)
Adrenal medulla/ganglia
Laryngeal cartilage
Tracheal cartilage
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92
Q

Progressive symptoms of hypothyroidism and diffuse goiter are consistent with what diagnosis

A

Lymphocytic (hoshimoto)thyroiditis)

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93
Q

Characteristic findings of hashimoto thyroiditis biopsy

A

Intense mononuclear infiltrate: lymphocytes and plasma cells
Germinal centers
Hurthle cells surounding residual follicles

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94
Q

What are the 3 reactions carried out by thyroid peroxidase

A

(Thyroid peroxidase = TPO)
Oxidation of iodide
Iodination of thyroglobulin
Coupling reaction between 2 iodized tyrosine residues

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95
Q

Antibodies against ____ are present in >90% of patients with chronic lymphocytic (hoshimoto) thyroiditis

A

TPO (thyroid peroxidase)

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96
Q

alkaptonuria is an autosomal recessive disorder of ____ metabolism

A

Tyrosine

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97
Q

Alkapotonuriais an auto recessive disorder of tyrosine metabolism that leads to the accumulation of ______

A

Homogentisic acid

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98
Q

The build up of homogentisic acid in alkaptonuria leads to what change in urine

A

Black color (homogentisic acid undergoes oxidation if sits around long enough and turns black)

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99
Q

What physical exam findings are seen in alkaptonuria

A

Ochronosis (blue-black pigmentation most evident in ears, nose, cheeks)
Ochronotic artropathy

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100
Q

Wha hormone influences development of internal male genitalia, spermatoenesis, male sexual differentiation at puberty

A

Testosterone

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101
Q

What hormone influences the development of external male genitalia, growth of prostate, male pattern hair growth

A

Dihydrotestosterone (DHT)

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102
Q

What hormone influnces endometrial proiferation, development of ovarian granuosa cells, and breast development

A

Estrogen

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103
Q

Deficiency in what enzyme resultls in deminished conversion of testosterone to DHT in the male urogenital tract

A

5 alpha reductase type 2

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104
Q

Male pseudohermaphroditism will have what clinical features

A

Genitalia at birth can range from small phallus with hypospadias to ambiguous or female-type genitalia (many are raised female util reaching puberty)

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105
Q

Where is 5 alpha reductase type 1 found

A

Postpubescent skin

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106
Q

Where is 5 alpa reductase type 2 found

A

Genitals

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107
Q

For a patient with male pseudohermaphroditism who have a deficiency in 5alpha reductase type 2, what changes happen at peuberty?

A

5 alpha reductase type 1 is still functional –> male pattern muscle ass, voice deepening, penile and scrotal growth, testicular descent

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108
Q

Stress hyperglycemia is due to secretion of cortisol, catecholamines, glucagon, and proinflammatory cytokines increasing what metabolic processes

A

Glycogenolysis and gluconeogenesis in the liver

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109
Q

The release of proinflammatory cytokines is associated ith the increased expression of what GLUT transporter and therefore the decreased expression of what GLUT transporter

A

Increased GLUT1

Decreased GLUT4

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110
Q

Proinflammatory cytokines promote increased expression of GLUT1 and decreased expression of GLUT4 resulting in increeased glucose uptake where?

A

Brain

Immune cells

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111
Q

Ezetimibe MOA

A

Decrease intestinal absorption of cholesterol by inhibiting the Niemann-Pik C1-like 1 (NPC1L1) transporter

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112
Q

What physical exam findings can be seen in glucagonoma

A

Necrolytic migratory erythema
Erythematous papules/plaques on face, perineum, and extremities
Lesions enlarge and coalesce, leaving a central indurated area with peripheral blitering and scaling
Diabetes mellitus
GI symptoms

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113
Q

Glucagonoma arises from ____ cells

A

Alpha

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114
Q

Insulin increases or decreases pancreatic glucagon secretion?

A

Decreases

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115
Q

Insulin increases or decreases glycogen syntheis in the liver?

A

Increases

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116
Q

Insulin increases or decreases glycogenolysis in the liver?

A

Decreases

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117
Q

In diabetic keto acidosis describe the intracellular and extracellular potassium levels as either increased or decreased

A

Intracellular: decreased
Extracellular: normal or increased

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118
Q

A nonselective beta blocker will decrease neurogenic or neuroglycopenic symptoms of hypoglycemia?

A

Neurogenic

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119
Q

Neurogenic symptoms of hypoglycemia are mediated via _____ and ___ release

A

Norepinephrine/epinephrine

Acetylcholine

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120
Q

What are some neurogenic symptoms of hypoglycemia

A 
Trmulousness
Tachycardia
Anxiety/arousal
Sweating
Hunger
Paresthesias
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121
Q

Neuroglycopenic symptoms of hypoglycemia include what?

A

Behavioral changes, confusion, visual disturbances, stupor, seizure

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122
Q

Thiazolidinedione binds PPAR gamma to decrease insulin resistance by upregulating what 2 genes

A

GLUT4

Adiponectin

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123
Q

Fucntion of adiponectin

A

Increases number of insulin-responsive adipocytes
Stiulates fatty acid oxidation
(Cytokine secreted by fat tissue)

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124
Q

DiGeorge syndrome is due to a ______ microdeletion

A

22q11.2

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125
Q

In DiGeorge sydnrome, the neural crest fails to migrate into the derivatives of what 2 pharyngeal pouches

A

3 and 4

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126
Q

Why will patients with DiGeorge syndrome have hypocalcmia

A

Insuficienct parathyroid growth (arises from 3rd (inferior) and 4th (superior) pouches)

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127
Q

Describe Chvostek sign seen in patients with DiGeorge syndrome

A

Tapping on the facial nerve –> twitching of nose and lips

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128
Q

Describe Trousseau sign seen in patients with DiGeorge syndrome

A

Inflation of bloo pressure cuff –> carpal spasm

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129
Q

Sheehan syndrome presents with panhypopituitarism due to ______ of the pituitary gland

A

Ischeic necrosis

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130
Q

Why are women susceptible to ischemic necrosis of the pituitary gland peripartum

A

Estrogen levels during pregnancy –> enlargement of pituitary gland without proportional increase in blood supply (even higher risk if paeripartum hypotension occurs due to peripartym hemorrhage (low hemoglobin can indicate this))

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131
Q

Estrogen effects what part of the bone which results in ceasation of bone growth

A

Epiphyseal plate

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132
Q

Why might a patient with thyroid hormone resistance present with tachycardia and ADHD

A

Most cases are due to an inherited mutation in thyroid hormone receptor beta. Thyroid receptor alpha is found in higher level in the CNS and heart

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133
Q

Congenital adrenal cortical hyperplasia and elevated 17-hydroxyprogesterone and testosterone is most commonly due to a deficiency in what enzyme

A

21-hydroxylase

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134
Q

Why do girls present earlier (at birth) than boys (around age 2-4) with 21-hydroxylase deficiency

A

21-hydrozylase deficiency –> increased testosterone –> ambiguous genitalia for females at birth
Boys will have normal genitalia but begin early virilization (body odor, pubic hair)

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135
Q

Deficiency of 21-hydroxylase causes adrenal cortical hyperplasia due to excess stimulation of the adrenal cortex by _____

A

ACTH

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136
Q

Treatment of congenital adrenal hyperplasia due to 21-hydroxylase defiency can be treated by targeting the supression of what hormone in order to limit androgen over production?

A 
Adrenocorticotropic hormone (ACTH)
(Impaired cortisol synthesis --> increased ACTH release --> stimulate adrenal cortex and adrenal adrogen overproduction)
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137
Q

Thiomides (methimazole, propylthiouracil) MOA

A

Inhibit thyroid peroxidase

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138
Q

How can chronic adrenal insufficiency be fatal during a medical procedure?

A

Severe stress normally –> increased glucocorticoid

Adrenal insufficiency cannot produce more –> adrenal crisis (hypotension, shock)

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139
Q

In a patient with adrenal insuficiency why would skin hyperpigmentation be highly suggestive of primary adrenal insufficiency rather than secondary?

A

Primary adrenal insufficiency –> increased ACTH secretion
ACTH is derived from POMC (proopiomelanocortin) which is prohormone of melanocyte stimulating hormone as well, therefore increased ACTH secretion –> increased MSH secretion –> hyperpigmentation

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140
Q

What is the most common cause of primary adrenal insuficiency? Is it more often bilateral or unilateral?

A

Autoimmune adrenalitis

Bilateral

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141
Q

What is the main function of brown adipose tissue in humans

A

Heat production

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142
Q

Brown adipose cells contain more miochondria than white adipose cells and function to produce heat by uncoupling oxidative phosphorylation with the protein ______

A

Thermogenin

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143
Q

In a patient with hypertension, elevated triglyceride, and a an elevated fasting glucose, what is another physical characteristic that is most suggestive of increased insulin resistance?

A

Increased weight circumfrance (indicates high visceral fat)

This question was getting at metabolic syndrome

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144
Q

Glucocorticoids have what effect on hepatic gluconeogenesis? Glycogenosis?

A

Increase, increase

Liver enyzme expression with be increased as a result

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145
Q

Metformin inhibits glycerophosphate dehydrogenase and complex I in the mitochondria to reduce substrates for what metabolic process

A

Gluconeogenesis

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146
Q

Metformin upregulates AMPK in hepatocytes which inhibits what metabolic process

A

Lipogenesis

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147
Q

Gynecomastia is due to increased _____ to androgen ratio in males

A

Estrogen

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148
Q

Why can tamoxifen be useful in treating males with gynecomastia

A

Selective estrofen receptor modulator that acts as an estrgen antagonist in the breast

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149
Q

Leuprolide MOA

A

GnRH analog (used to suppress testosterone levels in tx of prostate cancer)

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150
Q

In what tissues are the glucose transporter proteins found that are upregulated in response to insulin?

A

Muscle cells

Adipocytes

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151
Q

A patient with type 1 diabetes is having overflow incontinence. What is the likely source of these symptoms?

A

Diabetic autonomic neuropathy affecting detrusor muscle innervation
(Initially infrequent urination bc unable to sense full bladder then incomplete emptying due to loss of inervation to detruser)

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152
Q 
Which of the following is most likely to cause hypoglycemia in insulin-treated diabetes
Infection
Pain
Sleep deprivation
Intense exercise
Mental stress
A

Intense exercise
(The rest cause hyperglycemia due to production of counterregulatory hormones such as catecholamines and cortisol which increase glyconolysis and gluconeogenensis)

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153
Q

What hormones are produced in cells with highly developed, smooth endoplasmic reticulum?

A

Steroid hormones

Smooth ER involved in synthesis and processing of hydrophobic compounds

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154
Q

The initial step in the synthesis of steroid hormones is the conversion of ______ to pregnenolone in the mitochondria

A

Cholesterol

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155
Q

In a patient with cushing syndrome with normal-elevated ACTH levels, high-does dexamethasone supression test resulted in supression of ACTH and cortisol levels. What is causing the Cushing syndrome?

A

Pituitary adenoma

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156
Q

In a patient with Cushing syndrome and normal-elevated ACTH levels, a high-dose dexamethasone supression test does not change ACTH or cortisol levels. What is the cause of the patient’s cushing syndrome?

A

Ectopic ACTH production

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157
Q

In a patient with Cushing syndrome and supressed levels of ACTH, what are 3 possible causes of the cushing syndrome?

A

Adrenal adenoma
Adrenal malignancy
Exogenous glucocorticoid intake

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158
Q

Corticotropin-releasing hormone is released from the ___ to stimulate release of _____

A

Hypothalamus

ACTH

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159
Q

How will long term exogenous glucocorticoid use effect endogenous CRH, ATCH, and cortisol synthesis/release

A

Decrease all of them

160
Q

How is insulin cleared

A

Hepatic and renal

161
Q

Why might a patient with CKD who is being treated for type 1 diabetes suddenly begin having hypoglycemic episoides despite no change in treatment

A

CKD –> less renal clearance

162
Q

Insulin causes an increase in glycolysis by activating _____ , the enzyme that produces fructos 2,6-bisphosphate

A

Phosphofructokines-2 (PFK2)

163
Q

The most potent stimulator of phosphofructokinase-1 (PFK1) is _____

A

Fructose 2,6-bisphospate

164
Q

Hypercortisolism causes hyper- or hypo-glycemia?

A

Hyperglycemia

165
Q

What pathologic finding is preseent in the adrenal glads with ACTH dependent Cushing Syndrome

A

Bileteral hyperplasia involving the zona fasciculata and reticularis

166
Q

ACTH is the major trophic hormone for which area(s) of the adrenal gland while angiotensin II is the major trophic hormone for which area(s)?

A

ACTH: zon fasciculata and reticularis

Angiotensin II: zona glomerulosa

167
Q

Exopthalmos in Grave’s disease is treatd by what medication

A

Glucocorticoids

168
Q

Binding of PCSK9 to _____ increases degradation –> decreased uptake of circulating LDL into hepatocytes

A

LDL receptor

169
Q

Evolocumab MOA

A

PCSK9 inhibitor (decrease LDL-R degredation)

170
Q

What is the peak for subcutaneously administered regular insulin

A

2-4 hours

171
Q

What is the most sensitive test for primary hypothyroidism

A

TSH

172
Q

how does TNF alpha affect the insulin receptor

A

TNF alpha activate serine kinases –> phosphorylate beta subunits on insulin receptor and insulin receptor substrate 1 –> inhibits tyrosine phosphorylation of insulin receptor substrate by the insulin receptr

173
Q

what are 4 things that inhibit the insulin receptor by phosphorylation by serine kinase

A

TNF alpha
catecholamines
glucocorticoids
glucagon

174
Q

what tyrosine panel levels will be abnormal in euthryoid sickness sydrome

A

low T3

aka low T3 syndrome

175
Q

what causes low T3 levels in euthyroid sick syndrome

A

high cortisol, inflammatory cytokines and free fatty acids suppress 5’ deiodinase

176
Q

multiple endocrine neoplasia type 1 classification (3)

A

primary hyperparathyroidism
pituitary tumors
pancreatic tumors

177
Q

multiple endocrine neoplasia type 2A classifications (3)

A

medullary thyroid cancer
pheochromatoma
primary hyperparathyroidism

178
Q

multiple endocrine neoplasia type 2B classifications (3)

A

medullary thryoid cancer
pheochromocytoma
mucosal neuromas/marfanoid habitus

179
Q

estrogen is synthesized from androgens by _____

A

aromatase

180
Q

expression of aromatase in ovarian tissue is dependent on _____

A

gonadotropin

181
Q

anastrozole MOA

A

aromatase inhibitor

182
Q

maple syrup urine disease, an autosomal recessive disorder characterized by the defective breakdown of _______

A

branched chain amino acids

183
Q

what are the branched chain amino acids

A

leucine
isoleucine
valine

184
Q

branched chain alpha ketoacid dehydrogenase complex requires what 5 cofactos

A 
thiamine
lipoate
coenzyme A
FAD
NAD (tender loving care for nancy)
185
Q

diet restriction and ___ supplementation can be used in managing patients with maple syrup urine disease

A

thiamine

186
Q

methimazole is preferred for most patients due to what side effect of PTU

A

hepatotoxicity

187
Q

what are the two potential diagnoses for a thyroid nodule that reveals neoplastic follicular cells and microfollicles

A 
follicular adenoma (benign)
follicular carcinoma (malignant)
188
Q

how are folicullar adenomas and follicular thyroid carcinomas differentiated

A

follicular thyroid carcinomas invade the tumor capsule and/or surrounding blood vessels

189
Q

what thyroid neoplasm tends to spread hematogenously

A

follicular thyroid carcinoma

190
Q

11 beta hydroxylase leads to a build up of aldosterone and cortisol precursors which are then shunted toward adrenal ____ synthesis

A

androgen

191
Q

what deficiency can cause ambiguous genitalia (virilization) in XX females

A

11 beta hydroxylase

192
Q

11 beta hydroxylase deficiency leads to hypertension and hypokalemia how?

A

build up of 11-deoxycorticosterone which acts a mineralcorticoid

193
Q

Hormone sensitive lipase is activated by what?

A

Stress hormones (catecholamines, glucagon, ACTH)

194
Q

Hormone sensitive lipase is inhibitted by ____

A

Insulin

195
Q

In Graves disease antibodies against ___ are present

A

Thyrotropin (TSH) receptor

196
Q

In chornic lymphocytic (Hashimoto) thyroidits, ____ antibody is present

A

Thyroid peroxidase

197
Q

What effect do the antibodies in Grave’s disease have?

A

TSH receptor antagonist (Gs)

198
Q

What causes hypercalcemia in sarcoidosis

A

Excessive calitriol formation by activated macrophages

199
Q

A hypothalamic mass such as neurosarcoidosis (sarcoidosis involvement of hypothalamus) could cause increase in which of the pituitary hormones

A

Prolactin

200
Q

Multiple endocrine neoplasia type 2 is characterized by what 3 things?

A

Medullary thyroid cancer
Pheochromocytoma
Parathyroid hyperplasia (type 2A) or marganoid habitus and mucosal neuromas (type 2B)

201
Q

Medullary thyroid cancer is a neuroendocrine tumor that arises from what cells

A

Parafollicular calcitonin-secreting C cells

202
Q

What is seen microscopically in medullay thyroid cancer

A

Nests or sheets of polygonal or spindle shaped cells

Extracellular amyloid deposits

203
Q

Amyloid deposits in medullary thyroid cancer is derived from ____ secreted by neoplastic C cells and stains with ____

A

Calcitonin

Congo red

204
Q

Pulsatile secretion of GnRH from hypothalamus stimulates the release of ____ and ____

A

FSH

LH

205
Q

FSH and LH are prouced by ____ cells in the anterior pituitary

A

Gonadotroph

206
Q

LH stimulates _____ release from ___ cells

A

Testosterone

Leydig (in testes)

207
Q

FSH stimulates the release of ____ from ____ cells

A

Inhibin B

Sertoli (testes)

208
Q

Inhibin B suppresses _____ secretion

A

FSH

209
Q

Why will patients with only one testicle have elevated FSH

A

Less sertoli cells –> less inhibin B to supress FSH release

210
Q

What causes hyperpigmentation in a patient with Addison disease

A

Increased release of melanocyte stimulating hormone (decreased cortisol feedback on pituitary gland)

211
Q

The most common cause of primary adrenal insufficiency is autoimmune adrenalitis which results from autoantibodies against _____ zones in the adrenal cortex

A

All 3

212
Q

How does Addisons disease affect Na, K, Cl, and bicarb levels

A

Decreased aldosterone –> decreased Na and increased K and H
Low H excretion –> nonanion gap metabolic acidosis with low plasma bicarb –> Cl retention to maintain electrical neutrality of extracellular fluid
(Decreased Na and bicarb, increased K and Cl)

213
Q

What is the most common cause of acromegaly

A

Pituitary somatotroph adenoma –> increased growth hormone release

214
Q

How does excessive GH in acromegaly cause joint pain

A

Hyperplasia of articular chondrocytes and synovial hypertrophy –> degeneration of articular cartilage and periarticualr bone resembling osteoarthritis

215
Q

If a patient presents with antidiabetic drug induced hypoglycemia and an increased C peptide level, what are the possible drugs that could cause this

A

Sulfonylureas

Meglitinides

216
Q

Gastrin secreating tumors can occur in association with what?

A

Multiple endocrine neoplasia

217
Q

What is Zollinger-Ellison syndrome?

A

A gastric secreting pancreatic tumor

218
Q

Why would you ask a patient with Zollinger-Ellison syndrome if they have a family history of recurrent kidney stones

A

Zollinger-Ellison could be due to MEN1 which is also associated with hyperparathyroidism (can cause kidney stones)

219
Q

Diabetic ketoacidosis most commonly affects patients with which type of diabetes?

A

Type 1

220
Q

The destruction of beta cells in type I diabetes is primarily through _______

A

Cell mediated immunity (islet leukocytic infiltration)

221
Q

Congenital goiter causing hypothyroidism with increased TSH and low free T4 can be caused by maternal medication with what drug?

A

Propulthiouracil or antithyroid medication

222
Q

Congenital goiter that causes hyperthyroidism with decreased TSH and elevated free T4 can be due to transplacental _______ antibodies

A

TSH receptor-stimulating

Neonatal Graves disease

223
Q

Patients with sheehan syndrome will have ______ (central or peripheral) _____(hyper or hypo) thyroidism

A

Central

Hypo

224
Q

What is the purpose of treating patients with thyroid cancer with levothyroxine

A

Supress pituitary secretion of TSH which can promote growth f residual malgnant cells following thyroidectomy

225
Q

Agranulocytosis due to propylthioruacil use most commonly present as _____

A 
Oropharyngeal infection (fever, sore throat, oral ulcerations) 
(Sepsis can develop quickly after)0
226
Q

Why can erythrocytes not use ketone bodies for energy

A

They lack mitochondria

227
Q

Why can the liver not use ketone bodies for energy

A

Liver lacks succinyl CoA-acetoacetate CoA transferase

Required to convert acetoacetate to acetoactyl CoA

228
Q

How does cortisol cause increased blood pressure

A

Stimualtion of mineralcorticoid receptors (sodium retention)
Increased angiotensinogen production (increased vasoconstriction)
Increased adrenergic sensitivity (vasoconstriction)

229
Q

Primary hyperaldosteronism causes what changes in Na, K, and bicarb

A

Na: normal
K: low
Bicarb: high

230
Q

How does hyperaldosteronism cause metabolic alkalosis

A

Increased H+ excretion by alpha intercalated cells –> bicarb production

231
Q

Why are normal serum Na levels seen in hyperaldosteronism depsite increased Na reabsorption

A

Aldosterone escape
Incerased intravascular volume –> increased renal blood flow –> pressure natriuresis –> limits net sodium retention and prevents development of overt volume overloadand significant hypernatremia

232
Q

After glucose enters the beta cell, it is metabilized to glucose-6-phosphate by what enzyme

A

Glucokinase

233
Q

What is the role of glucokinase in beta cells

A

Functions as a glucose sensor
Its lower glucose affinity than other hexokinases allows it to limit the rate of glucose entry into the glyolytic pathway based on glucose levels

234
Q

How does maturity onset diabetes of the young differ from type 2 diabetes mellitus

A

MODY: mild hyperglycemia that often woresns with pregnancy induced insulin resistance. Patients are typically younger and nonobese. Non progressive condition, even without treatment

235
Q

Mutations in glucokinase gene lead to what effect on insulin secretion

A

Higher glucose levels are required to stimulate insulin secretion

236
Q

What are incretins

A

Gastrointestinal hormones produced by gut mucosa

Stimulate insulin secretion in response to sugar containing meals

237
Q

Incretins are secreted in response to what? Do blood glucose levels affect incretin release?

A

Oral consumption of glucose

No

238
Q

Pituitary apoplexy usually occurs in a preexisting _____

A

Pituitary adenoma

239
Q

Patients with pituitary apoplexy can develop cardiovascular colapse, how?

A

ACTH deficiency and subsequent adrenocortical insufficiency

240
Q

Pituitary apoplexy is a medical emergency that requires urgen neurosurgical consultation and what pharmalogic treatment?

A

Glucocorticoids (to treat the adrenal isufficiency which can cause cardiovascular colapse)

241
Q

Define macrosomia, a common finding in infants of diabetic mothers

A

Used to describe newborns who are much larger than average (more than 8 lbs 14 oz)

242
Q

Poorly controld maternal glucose leads to excessive transplacental ____ transfer to the fetus

A

Glucose

243
Q

What pancreatic changes occur in a fetus who is recieving excessive transplaental glucose from a mother who is poorly controling their glucose levels

A

Beta cell hyperplasia –> hyperinsulinism

244
Q

_____ is a catecholamine secreting neoplasm arising from the chromaffin cells of the adrenal medulla

A

Pheochromocytoma

245
Q

Why must patients with medullary thyroid cancer and RET mutation be screened for pheochromocytoma before surgery?

A

Induction of anesthesia –> catecholamine surge –> hypertensive crisis, flash pulmonary edema, and atrial fibrillation

246
Q

How do SGLT2 inhibitors reduce hyperfiltration

A

They increase sodium delivery to macula densa –> decreased renin secretion (–> delayed progression of nephropathy)

247
Q

How does diabetes stimulate RAAS in the kidneys?

A

Excessive filtered glucose –> sodium reabsorbed with glucose by SGLT2 –> less sodium delivered to macula densa –> renin secretion

248
Q

Functional hypothalamic amenorrhea, seen in patients with anorexia nervosa, is caused by decreased serum ____ levels

A

Leptin

249
Q

In funcitonal hypothalamic amenorrhea, decreased serum leptin has what effect on GnRH?

A

Decreased leptin levels –> inhibits pulsatile GnRH

250
Q

How does functional hypothalamic amenorrhea affect LH and FSH secretion

A

Both are decreased

251
Q

How are circulating estrogen levels effected in functional hypothalamic amnorrhea

A

Decreased

252
Q

Prostate enlargement in BPH is driven primarily by what hormone

A

Dihydrotestosterone (DHT)

253
Q

DHT is derivedfrom testosterone by ____ in peripheral tissues

A

5 alpha reductase

254
Q

Finasteride MOA

A

5 alpha reductase inhibitor

255
Q

What are the side effects associated with 5 alpha reductase inhibitors

A

Decrease libido, erectile dysfunction, decreased ejaculation volume (due to low DHT)
Gynecomastia (residual testosterone availible for conversion to estradiol)

256
Q

In menopause, how is FSH effected?

A

Increased (lack of feeback from inhibin from follicles)

257
Q

An elevated serum ____ level confirms diagnosis of menopause

A

FSH

258
Q

What is the function of FSH in the sertoli cells

A

Induce androgen binding protein production which concentrates. Testosterone in teh seminiferous tubuls and facilitates spermatogenesis

259
Q

What provides feedback regulation to FSH

A

Inhibin

260
Q

How does a unilateral orchiectomy affect erectile function and sperm count

A

Sperm count: decreased

Erectile function: normal

261
Q

A patient with 45, XO karyotype has what syndrome that may present with webbed neck, delayed growth, low set ears, arched palate and cubitus valgus.

A

Turner syndrome

262
Q

What can be used to treat patients with Turner syndrome in order to normalize height

A

Growth hormone

263
Q

Growth hormone receptor is what class of receptor

A

JAK STAT

264
Q

_____ is secreted by intestinal L cells in response to food intake and regulaes glucose levels by slowing gastric emptying, suppressing glucagon secretion, and increasing glucose-dependent insulin release

A

GLP-1 (glucagon-like peptide-1)

265
Q

GLP-1 is degraded by _____

A

DDP-4 (dipeptidyl peptidase 4)

266
Q

What test should be run prior to starting a patient on SGLT2 inhibitors

A

Serum ceatinine to determine the GFR (the effectiveness of SGLT2 inhibitors is dependent on GFR)

267
Q

What is the most common cause of death in patients with diabetes mellitus

A

Coronary heart disease

268
Q

What is reverse T3

A

An inactive form of thyroid hormone that is generated almost entirely from peripheral conversion of T4

269
Q

What is xanthelasma?

A

A type of xanthoma usually found on the medial eyelid

Yellowish macules/papules

270
Q

What is xanthoma

A

Dermal accululations of benign apparing macrophages with abundant, finelyvacuolated (foamy)cytoplasm containing cholesterol, phospholipids, and triglycerides

271
Q

What can cause xanthomas, including xanthlasmas

A

Hyperlipidemia and/or dyslipidemia

272
Q

After an obese patient loses 15 pounds what are the likely changes to be seen in grhelin, leptin, and insulin

A

Ghrelin: increased
Leptin: decreased
Insulin: decreased

273
Q

What receptor/second messanger system does glucagon use to exert its effects

A

Adenulate cyclase: Gs –> adenylate cyclase –> cAMP –> PKA

274
Q

What 2 tests should be monitored when administering testosterone therapy

A

PSA (prostate specific antigen) indicates increased prostate volume which increases risk of prostate cancer
Hematocrit: should not be started on patients with hematocrit >50% because testosterone can induce erythrocytosis –> increased blood viscocity –> increased risk of thromboembolism

275
Q

Parasympathetic stimulation of ____ receptors promotes insulin secretion and is induced by the smell and/or sight of food

A

M3

276
Q

Stimulation of ___ adrenergic receptors stimulates insulin release and stimulation of ___ adrenergic receptors inhibits insulin release

A

Beta 2

Alpha 2

277
Q

Which adrenergic receptor mediated response is predominant in beta cells

A

Alpha 2 (sympathetic stimulation leads to overall inhibition of insulin secretion)

278
Q

What is the likely diagnosis of a 7 yr old boy with 4 cm multiloculated, cystic, supracella lesion with calcifications

A

Craniopharyngioma

279
Q

Craniopharyngiomass are tumors arisingfrom remnants of ___

A

Rathkes pouch

280
Q

How does glucagon restore blood glucose

A

Inducing glycogenolysis and gluconeogenesis in the liver

281
Q

What are the most common hormonally active adenomas

A

Prolactinoma

282
Q

How will male patients present with prolactinomas

A

Mass effect: headaches, bitemporal heminanopsia

Supression of GnRH: hypogonadism (impotence, decreased libido, infertility)

283
Q

Hypertension and muscle weakness may be due to overactivity of what area in the adrenal gland

A

Zona glomerulosa

284
Q

Overactivity of the adrenal zona glomerulosa will result in increased secretion of what

A

Mineralocorticoids (aldosterone)

285
Q

What is the function of aldosterone

A

Sodium retention

Decreased potassium and H+ reabsorption

286
Q

What crosses the placenta and allows newborns with hypohyroidism to appear asymptomatic

A

T4

287
Q

What is vitilization

A

Occurs with very high andreogen levels

Characterized by clitoromegaly, increased muscularity, and voice deepending in addition to hirsutism

288
Q

What is the most common cause of hirsutism

A

Polycystic ovary syndrome (PCOS)

289
Q

Where is somatostatin released from

A

Pancreatic delta cells

290
Q

Somatostatin decreases the release of what? (6)

A 
Secretin
Cholecystokinin
Glucagon
Insulin
Gastrin
Growth hormone
291
Q

Gallbladder stones in a somatostatinoma is due to the inhibition of what?

A

Cholecystokinin (normally causes gallbladder contraction)

292
Q

MOA glyburide

A

Sulfonylurea

293
Q

How can hypothyroidism cause elevated prolactin levels

A

Increasedd TRH –> prolactin release

294
Q

Diabetes insipidus leads to increased osmolarity and contracted volumes in ICF, ECF, or both?

A

Both

295
Q

In diabetes insipidus, is the volume contraction isosmotic, hyposmotic, or hyperosmotic

A

Hyperosmotic (loss of free water with retention of electrolytes)

296
Q

What causes amenorrhea in a female with low body weight

A

Low leptin –> inhibits pulsatile GnRH from hypothalamus -> low FSH and LH release –> low estrogen –> emenorrhea

297
Q

What is Kallmann syndrome

A

Absence of GnRH secretory neurons in hypothalamus due to defective migration from the olfactory placode
Presentation: hypogonadism and anosmia that often present with delayed puberty

298
Q

What is the primary source of ATP at the begining (first 10 sec) of exercise

A

Phosphocreatine shuttle

299
Q

How does SGLT2 inhibitor cause decreased sodium levels

A

SGLT2 is a sodium/glucose symptor, when inhibitted it increases both Na and glucose secretion

300
Q

Why would a patient with MEN type 2B have episodic headaches

A

Pheochromocytoma –> catecholamines -> paroxysmal hypertension and tachycardia –> increase in BP –> headache

301
Q

What can cause paresthesias and muscle weakeness in patients with primary hyepraldosteronism

A

Excess urinary K and H secretion

302
Q

Do patients with primary hyperaldosteronism have significant fluid volume expansion? Why?

A

No

Aldosterone escape

303
Q

What 2 hormones are dependent on neurophysins

A

Oxytocin

Vasopressin

304
Q

Niemann-pick disease is an autosomal recessive lysosomal storage disorder caused by a deficiency in ____

A

Sphingomyelinase

305
Q

What are the key features of Niemann-Pick disease

A

Macular cherry red spot
Progressive neurodegeneration
Hepatosplenomegaly

306
Q

How will radioactive iodine uptake change in subactute (de Qyervain) thyroiditis

A

Decrease ( decreased thyroid metabolic activity and organification of iodine due to loss of TSH)

307
Q

What is the pathophysiology of postpardum thyroiditis

A

Autoimmune distrution of thyroid follicles and release of preformed thyroid horone
Lymphocytic infiltrates and sometimes germinal centers present

308
Q

How does cystic fibrosis cause increased serum glucose during glucose tolerance testing

A

Thick, inspissated secretions blok the lumen of pancreatic ducts –> destruction of pancreatic islet cells –> decreased insulin production –> CF related diabetes

309
Q

How does estrogen tratment effect thyroid hormone levels

A

Estrogen increases thyroglobulin –> increased bound T4 and transient decrease in fre T4 and T3 until TBG is saturated andT3 and T4 normalize –> increased total T4

310
Q

What test should be done before begining metformin treatment

A

Serum creatinine measurement (contraindicated in renal dysfucntion due to increased risk of lactic acidosis)

311
Q

What are tamoxifen’s effects in breast, bone and endometrial tissue

A

Breast: estrogen antagonist
Bone: estrogen agonist
Endometrium: estrogen agonist

312
Q

What are raloxifene’s effects in the breast, bone, and endometrial ltissue

A

Breast: antagonist
Bone: agonist
Endometrium: antagonist

313
Q

How does chronic glucocorticoid use accelerate osteoperosis

A

Inhibit proliferation and differentiations of osteoblast precursosrs
Increased expression of RANK and RANK-L
Supressed calcium absorption and reabsorption –> increased clacium release from bone

314
Q

What type of receptor is the insulin receptor

A

Transmembrane tyrosine kinase receptor –> phosphorylation of insulin receptor substrate 1

315
Q

Activation of what kinase inducs the metabolic functions of insulin

A

Phosphatidylinositol 3 kinase (PI3K)

Stimulates GLUT4 translocation, glycogen synthesis, fat synthesis

316
Q

Insulin activates phosphatidylinositol-3-kinase which activates _____ which dephosphorylates glycogen synthase, leading to its activation and promoting glycogen synthesis

A

Protein phosphatase

317
Q

What is the likely diagnosis of a patient presenting with a history of hypothyroidism, weight loss, hyperpigmentation, abdominal pain, vomiting, weakness, fever, severe hypotension and refractory shock

A

Adrenal crisis (with chronic primary adrenal insufficiency)

318
Q

What is the treatment fo acute adrenal crisis

A 
Agressive fluid resuscitation
Glucocorticoid supplementation (hydrocortisone or dexamethasone)
319
Q

In a non medical setting (without venous access), sever hypoglycemia, causing loss of consciousness can be treated how?

A

Emergency glucagon kit: intranasal or subcutaneous/intramuscular formulations

320
Q

In an emergency setting, subcutaneous administration of glucagon corrects hypoglycemia how?

A

Increasing hepatic glycogenolysis

321
Q

How does hypothyroidism cause elevated total cholesterol

A

Decreased expression of LDL receptor expression –> decreased clearance of LDL
(May also decrease LDL receptor activity and biliary excretion of cholesterol)

322
Q

How can hypothyroidism cause hypertriglyceridemia?

A

Decreased expression of lipoprotein lipase

323
Q

What causes hypoglycemia in neonates born from mothers with diabetes

A

Hyperfuctioning of pancreatic beta cells

324
Q

Patients with long standing type 1 diabetes can have increased risk of hypoglycemia due to decreased ___ secretion

A

Glucagon (long standing diabetes patients frequently also have alpha cell failure)

325
Q

21 hydroxylase is responsible for conversion of ____ to _____

A

Progesterone –> 11 deoxycorticosterone
And
17 hydroxyprogesterone –> 11 deoxycortisol

326
Q

Deficiency of 21 hydroxylase deficieny causes a build up of 17 hydroxyprgesterone which is then shunted toward ____ production

A

Androgen

327
Q

Labratory studies in all forms of 21 hydroxylase deficiency reveal elevated _____ and ____ levels

A

17 hydroxyprogesterone

Testosterone

328
Q

FSH stimulates ____ release and ____ production from Sertoli cells in the seminiferous tubules

A

Inhibin B

Androgen binding protein

329
Q

What is the most commno inborn error of methionin metabolism leading to ectopia lentis (dislocated lens), intellectual disability, and marfanoid habitus?

A

Homocystinuria

330
Q

Homocystinuria is most frequently caused by an autosomal recessive deficiency of cystathionine beta synthase, an enzyme that requires _____ as a cofactor

A

Pyridoxine (vitamin B6)

(50% patients repsond to pyrdoxine supplementation_

331
Q

Homocystinuria is most commonly caused by a deficiency of cystathionine beta synthase and can be treated by dietary restriction of _____

A

Methionine

332
Q

Excessive vitamin D can cause symptomatic ______

A

Hypercalcemia

333
Q

Presentation of hypercalcemia

A

(Due to impaired depolarization of neuromuscular membranes)
Weakness
Constipation
Confusion
(Impaired concentration of urine in distal tubule)
Polyuria
Polydipsia

334
Q

How do granulomatous diseases such as sarcoidosis and tuberculosis caues hypercalcemia

A

They can have PTH independent conversion of 25 hydroxyvitamin D to 1,25 hydroxyvitamin D due to expresion of 1-alpha-hydroxylase in activated macrophages

335
Q

How can hyperparathyrdism cause constipation

A

High PTH –> hypercalcemia –> inhibitted nerve depolarization –> impaired smooth muscle contraction and reduced colonic motility

336
Q

____ is secreted by intestinal L cells in response to food intake and regulates glucose by slowing gastric emptying, supressing glucagon secretion, and increasing glucose-dependent insulin release

A

GLP-1 (glucagon-like peptide)

337
Q

Deposits of ___ are universally seen in the pancreatic islets of patients with type 2 diabetes

A

Amylin

338
Q

How do 5 alpha reductase inhibitos cause gynecomastia

A

By blocking the conversion of testosterone to DHT, te excess testosterone is availble for conversion to gynecomastia

339
Q

A patient presenting with infertility, gynecomastia, and long lower extremeties is suggestive of what diagnosis

A

Klinefeler syndrome

340
Q

What is klinefelter syndrome cause by

A

Chromosomal disorder: 47, XXY

341
Q

Klinefelter syndrome is the most common cause of male hypogonadism. How does it cause male hypogonadism?

A

Progressive destruction and hyalinization of the sminiferous tubules, leading to small, firm testes
Sertoli cells are damaged –> decreased inhibin levels
Leydig cells damaged –> decreased testosterone
Loss of feeback inhibition –> increased FSH and LH

342
Q

Cholestyramine MOA

A

Bile acid-binding resin

343
Q

How do bile acid binding resins such as cholestyramine cause hypertriglyceridemia

A

Increase hepatic production of triglycerides and increase the release of triglyceride heavy VLDL particles into circulation

344
Q

How does low cortisol contribute to hyponatremia

A

Cortisol usually inhibits ADH

345
Q

Primary adrenal insufficiency affects what portions of the adrenal gland

A

All 3 layers of the cortex

346
Q

Normal aging causes what changes to free testosterone, total testosterone, LH, and serum sex hormone-binding globulin

A

Free Testosterone: decreased
Total testosterone: decreased
LH: increased (due to decreased testosterone feedback)
Sex hormone binding globulin: increased

347
Q

Niemann-Pick disease is characterized _______ deficiency

A

Sphingomyelinase

348
Q

What fundoscopic finding is consistant with Niemann-Pick disease

A

Cherry-red macular spot

349
Q

What are 3 clinical features of Niemann-Pick disease

A

Hepatosplenomegaly
Neurological regression
Cherry-red macular spot

350
Q

What behavior often causes weight gain in patients who initiate insulin therapy for type 2 diabetes

A

Patients may eat more to supress feelings of hypoglycemia

Should inquire about paitents perspective on appetite, dietary patterns, and hypoglycemic symptoms

351
Q

Pheochromocytoma is a tumor of ___ cells in the _____ characterized by increased production of ____

A

Chromaffin
Adrenl medulla
Catecholamines

352
Q

Pheochromocytoma stains positive for what 3 stains?

A

Synaptophysin
Chromogranin
Neuron-specific enolase

353
Q

Failure of serum 11-deoxycortisol and urinary 17-hydroxycorticosteroid levels to rise in response to metyrapone indicate what?

A

Either primary or secondary adrenal insufficiency

354
Q

Metyrapone blocks ___ synthesis by inhibiting 11 beta hydroxylase

A

Cortisol

355
Q

List the 3 characteristics of MEN type 1

A

Primary hyperparathyroidism
Pituitary tumors
Pancreatic tumors

356
Q

In SIADH, what is the volume status and sodium status

A

Euvolemic

Hyponatremia

357
Q

In congenital adrenal hyperplasia due to 21 hydroxylse deficiency, how will aldosterone, cortisol, and androgen production be affected

A

Decreased aldosterone and cortisol production

Increased androgen production

358
Q

How does 21 hydroxylase deficiency present in male patients

A

1-2 weeks after birth with vomiting, hypotension, hyponatremia, hyperkalemia due to salt wasting, genitalia are normal

359
Q

What cellular mechanism causes hypoglycemia in patients with type 2 diabetes

A

Increased transocation of GLUT4 induced by muscle contraction –> increased glucose uptake during exercise

360
Q

What is the most common cause of secondary hyperthyroidism

A

TSH secreting pituitary adenoma

361
Q

Hypertension, hypokalemia, and absent secondary sexual characteristics, and a blind puch vagina in a person with X,Y chromosome indicate a deficiency in what enzyme

A

17 alpha hydroxylase

362
Q

Elevated TSH and low T4 in a newborn who has normal length and weight likely has what diagnosis

A

Thyroid dysgenesis

363
Q

In the polyol pathway, glucose is converted into ____ by aldose reductase, which is trapped inside the cell until it is converted into ____ to facilitate excretion

A

Sorbitol

Fructose

364
Q

Build up of ____ leads to cataracts in patients with diabetes

A

Sorbitol (and advanced glycosylation end products)

365
Q

How does sorbitol lead to cell injury

A

Itt accumulates in certain cells and causes an influx of water –> osmotic cellular injury
Also aldose reductase (converts glucose to sorbitol) depletes NADPH which increases oxidative strewss

366
Q

What are signs that Cushing syndrome is ACTH dependent

A

Hyperpigmentation (MSH is derived from POMC also)
Androgenization (ACTH stimualtes production of adrenal androgens –> increased DHEAS and hirtutism and menstrual abnormalities)

367
Q

Why might a patient with lupus show signs of Cushing syndrome

A

Iatrogenic Cushing syndrome from chronic glucocorticoid use

368
Q

Phentermine MOA

A

Stimulates the release and inhibits the reuptake of Norepinephrine

369
Q

What mechanism is responsible for hyponatremia following a subarachnoid hemorrhage

A

Damage to hypothalamus –> excesive production of ADH

370
Q

Beta thalassemia minor causes what hemoglobin changes

A

Decreased hemoglobin A

Inceased hemoglobin A2

371
Q

Beta thalassemia major causes what hemoglobin changes

A

Hemoglobin A absent
Hemoglobin A2 increased
Hemoglobin F increased

372
Q

How can hemolytic anemia give a misleadingly low hemoglobin A1C

A

Rapid erythrocyte turnover –> misleadingy low A1C

373
Q

Diagnostic riteria for diabetes includes fasting plasma glucose _____, hemoglobin A1C ____, or random glucose _____ in a patient with symptoms of hyperglycemia, or oral glucose tolerance test ith plasma glucose _____

A

Fasting: > or equal to 126
A1C: > or equal to 6.5%
Random: > or equal to 200
Glucose tolerance: > or equal to 200 (2 hrs after glucose ingestion)

374
Q

what is acanthosis nigricans and what does it indicate

A

hyperpigmentation is skin folds

insulin resistance

375
Q

how do chronically elevated free fatty acids contribute to insulin resistance

A

they impair insulin dependent glucose uptake and increase hepatic gluconeogenensis

376
Q

what amino acids should be restricted from the diet of a patient with maple syrup urine disease

A

leucine
isoleucine
valine

377
Q

what enzyme is deficient in patients with maple syrup urine disease

A

branched chain alpha ketoacid dehydrogenase complex

378
Q

In a patient presenting with hyperthyroid symptoms, low TSH, elevated T4 and low thyroglobulin indicates what diagnosis

A

thyrotoxicosis from exogenous source (ie. levothyroxine tablets)

379
Q

thyrotropin receptor antibodies are present in what disease

A

Graves disease

380
Q

thyroid dermopathy is caused by stimulation of ____ by TRAb and activated T cells, leading to excess production of ____ and adipogenesis

A

fibroblasts

glycosaminoglycans

381
Q

what is a common adverse side effect of highly active antiretroviral therapy (HAART)

A

medication induced body fat redistribution (lipoatrophy or lipodystrophy)

382
Q

advanced atherosclerosis at a young age seen in both a father and a son causing early onset MI is likely due to decreased expression of what receptor

A

LDL receptor in the liver (familial hypercholesterolemia)

383
Q

fibrates MOA

A

activate peroxisome proliferator activated receptor alpha (PPAR alpha) –> increased synthesis of lipoprotein lipase

384
Q

what are the most effective agents for the treatment of hypertriglyceridemia

A

fibrates (fenofibrate, gemfibrozil)

385
Q

what is the most common cause of inadequate thyroid hormone level in infants with treated hypothyroidism

A 
rapid growth (which increases the physiologic need for thyroid hormone)
another common reason is decreased absorption
386
Q

what can cause poor intestinal absorption of levothyroxine

A

co administration with various foods (soy products) and certain medications (iron, calcium, antacids)

387
Q

Growth hormone’s growth promoting effects are primarily mediated by ____ which is released from the liver

A

IGF-1

388
Q

besides rapid linear growth, what are other manifestations of gigantism in children

A 
large hands and feet
thickening of calvarium
protrusion of jaw
excessive sweating
oily skin
389
Q

if metoclopramide is not tolerated, what antibiotic can be used to treat gastroparesis

A

erythromycin

390
Q

how is erythromycin prokinetic

A

it activates the motilin receptor in smooth muscle of the upper GI tract which results in persistaltic contractions

391
Q

what changes in GnRH, LH, and testosterone are seen in a patient with a prolactinoma

A

GnRH, LH, and testosterone are all decreased

392
Q

what vitamin D supplementation should be chosen for patients with hypoparathyroidism

A

calcitriol

calcidiol is dependent on PTH for conversion into calcitriol

393
Q

high serum levels of _____ confirms diagnosis of infants with 21-hydroxylase deficiency

A

17-hydroxyprogesterone

394
Q

21 hydroxylase is responsible for converting ____ into 11-deoxycorticosterone and 17-hydroxyprogesterone to _______

A

progesterone

11-deoxycortisol

395
Q

what does a TSH level

A

their current dose is too high

396
Q

what is the most common risk of thyrotoxicosis due to thyroid replacement therapy

A

cardiovascular complications due to increased beta adrenergic receptor expression (A fib, angina, high output heart failure)

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