UWorld 1 & 2 Flashcards

1
Q

What affect does estrogen have on HMG-CoA reductase?

A

Upregulates

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2
Q

Why do pregnant women present more with gallstones?

A

Estrogen upregulates HMG-CoA reductase, reusling in bile to become supersaturated with cholesterol

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3
Q

What effect does progesterone have on bile acid secretion and gallbladder emptying?

A

Progesterone reduces bile acid secretion and slows gallbladder emptying

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4
Q

What is used to treat patients with recurrent calcium-based nephrolithiasis?

A

Thiazide diuretics - increase Ca reabsorption

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5
Q

What drug can be used to treat narcolepsy?

A

Modafinil

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6
Q

How do you calculate maintenance dose?

A

(Steady state plasma concentration x clearance) / bioavailability fraction

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7
Q

What is the MOA of ribavirin?

A

Interfering with the duplication of viral genetic material - it is a nucleoside antimetabolite

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8
Q

Adrenal crisis requires what for immediate treatment?

A

Fluid resuscitation and glucocorticoid supplementation (hydrocortisone or dexamethasone)

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9
Q

What is a patients greatest risk from dying following complications for acute rheumatic fever?

A

Pancarditis

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10
Q

DRESS syndrome is seen in what drugs? What timeframe does it present at?

A

Presents 2-8 weeks after exposure; egs: phenytoin, carbamazepine, allopurinol, sulfonamides, antibiotics (minocycline, vanco)

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11
Q

How does DRESS present

A

2-8 wks after exposure - fever generalized lymphadenopathy, facial edema, diffuse morbilliform skin rash; labs show eosinophilia

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12
Q

Drug induced antineutrophil cytoplasmic antibodies associated vasculitis presents with what drugs?

A

Commonly medications for hyperthyroidism (PTU, methimazole) and hydralazine

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13
Q

How does drug induced antineutrophil cytoplasmic antibodies associated vasculitis present?

A

Constitutional symptoms, arthralgias/arthritis, cutaneous vasculitis

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14
Q

What is mixed cryoglobulinemia?

A

Small to medium vessel vasculitis caused by circulating immunoglobulin-complement complexes that precipitate on refigeration

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15
Q

What is mixed cryoglobulinemia associated with?

A

Chronic inflammatory states - SLE, HCV

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16
Q

How does mixed cryoglobulinemia present?

A

Systemic findings and palpable purpura in the lower extremities due to cutaneous vasculitis

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17
Q

What are the sertoli cells responsible for and where are they found?

A

Found in the testes; responsible for producing anti-mullerian hormone which causes mullerian duct involution; they also produced androgen-binding protein which concentrates testosterone in the seminiferous tubules to enable spermatogenesis

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18
Q

What do the Leydig cells do and what are they responsible for?

A

Secrete testosterone and stimulate the Wolffian duct to develop into internal male reproductive organs (epididymides, vas deferens, ejaculatory ducts, seminal vesicles)

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19
Q

What forms the external male reproductive organs? What hormone is responsible?

A

Genital tuberlce, urogenital folds and labioscrotal swelling; dihydrotestosterone

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20
Q

What is septic abortion?

A

Abortion resulting in retained products of conception

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21
Q

How does septic abortion present?

A

Fever, chills, lower abd pain and blood or purulent discharge; boggy, tender uterus with dilated cervix. Pelvic US: retained products of conception, increased vascularity, echogenic material in cavity, thick endometrial stripe

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22
Q

What microorganisms are responsible for septic abortion?

A

S aureus is very common; others: gram neg bacilli (E coli) and GBS

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23
Q

What is Asherman syndrome?

A

Long term complication from septic abortion - adhesions in uterine cavity that can lead to secondary amenorrhea and infertility

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24
Q

What organisms are resistant to cephalosporins?

A

L monocytogenes, MRSA, enterococci, atypicals (mycoplasma and chlamydia)

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25
Q

What type of immunity is required to clear Listeria infection?

A

Cell mediated immunity; humor responses do not play a role

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26
Q

Why is listeria resistant to cephalosporins?

A

Altered penicillin binding proteins

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27
Q

What is listeria treated with?

A

Ampicillin - ampicillin is generally added to empiric treatment for meningitis in young infants or immunocompromised

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28
Q

What cephalosporin covers Listeria, MRSA, enterococcus faecalis?

A

5th gen - ceftaroline

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29
Q

Down syndrome presenting with 46,XX t(14;21) karyotype is an example of what?

A

Unbalance robertsonian translocation

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30
Q

Unbalanced translocations resulting in Down Syndrome is most commonly between what two chromosomes?

A

14 and 21

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31
Q

What causes dry beriberi? What are the symptoms?

A

B1 def. symmetrical peripheral neuropathy of the distal extremities with resulting sensory and motor impairments

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32
Q

What is Wet beriberi?

A

Includes symptoms of dry berberi (peripheral neuropathy) with the addition of cardiac involvement (cardiomyopathy, high output CHF)

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33
Q

B1 def resulting in Wernick-Korsakoff damages what regions of the CNS?

A

Medial dorsal nucleus of the thalamus, mammillary bodies

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34
Q

What enzymes are affected by B1 def?

A

Pyruvate dehydrogenase, alpha-ketogluturate dehydrogenase, transketolase, and branched-chain ketoacid dehydrogenase

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35
Q

An anion gap metabolic acidosis results in what process in the kidneys?

A

Renal ammoniagenesis - renal epithelial cells metabolize glutamine, generation ammonium and bicarb; ammonium ions are transported into the tubular fluid and excreted, bicarb functions to buffer acids in the blood

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36
Q

What is renal ammoniagenesis? When do you see it?

A

Acidosis; results in renal tubular epithelial cells to metabolize glutamine, generating ammonium and bicarb. Bicarb is absorbed into the blood.

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37
Q

What are the clinical features of Fabry disease?

A

Neuropathic pain (burning sensation in palms and soles), angiokeratomas, telangiectasias, glomerular disease, TIA, stroke, cardiac disease

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38
Q

What is the inheritance pattern of Fabry disease?

A

Xlinked recessive

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39
Q

What enzyme is deficient in fabry disease?

A

Alpha galactosidase A - results in accumulation of globotriaosylceramide

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40
Q

Deficiency in alpha galactosidase A results in accumulation of what? What disease is this?

A

Accumulation of ceramide trihexoside (globotriaosylceramide); fabry disease

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41
Q

What type of murmur is consistent with hypertrophic cardiomyopathy?

A

Systolic murmur that becomes pronounced with standing from supine position

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42
Q

Hypertrophic cardiomyopathy results in what cardiac flow issue?

A

Anterior mitral leaflet shfits toward aortic valve, thickened septum further obstructs LV outflow = LVOT obstruction

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43
Q

LVOT obstruction is seen in what disease? What makes it worse?

A

Seen in HCM; worse with decreased LV volume (also caused by decreased preload or reduced systemic vascular resistance)

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44
Q

What medications should be avoided in patients with HCM?

A

Vasodilators and diuretics

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45
Q

What is verapamil?

A

Nondihydropyridine calcium channel blocker

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46
Q

What is disopyramide?

A

Call IA antiarrhythmic

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47
Q

Subserosal leiomyomas cause what?

A

Irregular uterine enlargement - causing pelvic pressure

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48
Q

The nucleolus is the primary site for what?

A

Ribosomal RNA transcription

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49
Q

RNA polymerase I is responsible for what?

A

Ribosomal RNA

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50
Q

RNA polymerase II is responsible for what RNA production?

A

Messenger RNA, snRNA, microRNA

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51
Q

RNA polymerase III is responsible for production of what RNA?

A

Transfer RNA, 5s ribosomal RNA

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52
Q

Where does RNA polymerase I function?

A

Nucleolus

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53
Q

What lab values are consistent with primary hyperaldosteronism?

A

Increased aldosterone, low renin

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54
Q

What lab values are associated with secondary hyperaldosteronism?

A

Increased renin and aldosterone

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55
Q

Inactivated (killed or component) viral vaccines predominantly generate what type of immune response?

A

Humoral

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56
Q

Live attenuated viral vaccines generate what type of immune response?

A

Cell mediated in addition to providing humoral immunity

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57
Q

Wilson’s disease can cause cystic degeneration to what?

A

Putamen, as well as damage to other basal ganglia structures

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58
Q

Where is the putamen located?

A

Medial to the insula and lateral to the globus pallidus on a coronal section

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59
Q

Interventricular foramina of Monro is located where?

A

Between lateral and third ventricle

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60
Q

Cerebral aqueduct is located where?

A

Between third and fourth ventricle

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61
Q

How does CSF enter the subarachnoid space?

A

Via one of 3 foramina: 2 lateral foramina of Luschka and a single midline foramen of Magendie

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62
Q

Obstruction at the level of the cerebral aqueduct causes increased pressures where?

A

Third and lateral ventricles

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63
Q

What are the key features of schizotypal PD?

A

Eccentric; odd thoughts, perceptions and behavior

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64
Q

Schizoid PD is characterized by what?

A

Prefers to be a loner, detached, unemotional

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65
Q

When the head of the humerous is displace anteriorly, what might you see on PE? What nerve is at risk?

A

Flattening of the deltoid prominence, protrusion of acromion; axillary n.

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66
Q

How do you calculate sensitivity?

A

TP/TP+FN

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67
Q

How do you calculate specificity?

A

TN/(TN + FP)

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68
Q

People who have suffered an AE are more likely to recall previous risk factors than are people who have not experience a poor outcome. This is an example of what?

A

Recall bias

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69
Q

What is recall bias?

A

Inaccurate recall of past exposure by people; applies to most retrospective studies such as case control studies

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70
Q

What organism is likely to cause infective endocarditis after a GU procedure?

A

Enterococci

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71
Q

Patient recently underwent genitourinary procedure comes in with a UTI that results in gram positive cocci. Likely organism?

A

Enterococci

72
Q

Are enteroocci aerobic or anaerobic?

A

Aerobic

73
Q

Explain the morphology and gram staining and any other important characteristics of enterococcus spp.

A

Gram + cocci in pairs and chains, gamma hemolysis, PYR +, grow in bile and in 6.5% sodium chloride; they are unable to convert nitrates to nitrites

74
Q

How does RSV present?

A

Usually < 2 years old; antecedent nasal congestion/discharge, cough, followed by wheezing/crackles and potentially resp distress

75
Q

How does thrombotic thrombocytopenic purpura present?

A

Pentad of symptoms: neurologic and renal symptoms, fever, thrombocytopenia, and MAHA

76
Q

Bicuspid aortic valves commonly result in what?

A

Aortic stenosis around 50 years old (10 years prior to the average onset of senile calcific aortic stenosis)

77
Q

Koilocyte is a sign of infection with what?

A

Human papillomavirus

78
Q

What does a koilocyte look like?

A

Immature squamous cell with dense, irregularly staining cytoplasm and perinuclear clearing, resulting in a halo; nucleus has raisinoid appearance

79
Q

What affect do thiazide diuretics have on calcium absorption?

A

Increase calcium absorptions, resulting in decreased calcium excretion

80
Q

The etiology of narcolepsy is believed to be due to low levels of what?

A

Orexin (hypocretin)

81
Q

A neurologic disorder characterized by episodes of irresistible, refreshing sleep during the daytime and usually one or more REM sleep-related phenomena describes what?

A

Narcolepsy

82
Q

What is train of four stimulation?

A

Used during anesthesia to assess the degree of paralysis induced by NMJ-blacking agents; peripheral nerve is stimulated 4 times in a quick succession the the muscular response is recordded

83
Q

How is maintenance dose calculated?

A

(Steady state plasma concentration x clearance) / [bioavailability fraction]

84
Q

What is the MOA of ribavirin? What is it used for?

A

Nucleoside antimetabolite that is phosphorylated intracellularly and interferes with duplication of viral genetic material; used in HCV and RSV

85
Q

Ribavirin has multifactorial actions/affects, what are they?

A

Induces hypermutation, inhibits RNA pol and inosine monophosphate dehydrogenase (resulting in defect 5’-cap formation) and modulating more effective Th1 immune response

86
Q

How does acute adrenal insufficiency present?

A

Hypotension, shock, n/v, abdominal pain, weakness, fever

87
Q

How is acute adrenal insufficiency treated?

A

Fluid resuscitation and immediate glucocorticoid supplementation

88
Q

Patient with acute endocarditis is most likely to die from what complication?

A

Pancarditis

89
Q

What is the difference in genetic mutation between duchenne and beckers muscular dystrophy?

A

Duchenne is due to a frameshift mutation and Beckers is due to a non-frameshift mutation (somewhat functional protein)

90
Q

Dystrophin gene is on what chromosome?

A

X chromosome

91
Q

What is the function of dystrophin?

A

Structural component of skeletal muscle fibers that provide mechanical stability to the sarcolemma; it links component of the cytoskeleton to transmembrane proteins

92
Q

Polymyositis is associated with inflammation of what?

A

Endomysial inflammatory infiltration

93
Q

Dermatomyositis is associated with inflammation of what?

A

Perifascicular inflammation

94
Q

What are the results of nitroblue tetrazolium test in a patient with chronic granulomatous disease?

A

The neutrophils fail to turn blue

95
Q

What serologic tests can be used to follow disease progression of syphillis?

A

VDRL and RPR - evaluate for the presence of antibody against cadiolipin (byproduct of treponemal infection)

96
Q

Wht serologic tests remain positive for life after a syphillis infection?

A

FTA-ABS and microhemagglutination assay for T pallidum (MHA-TP)

97
Q

How is visualization of T pllidum done?

A

Dark field micrscopy - reveals helical motile spirochetes

98
Q

What is the NPV?

A

Probability of not having a disease given a negative test result

99
Q

Punch biopsy with normal appearing epidermis reveals stellar cells with characteristic intracytoplasmic granules having the shape of a tennis racquet describes what type of cells?

A

Langerhans cells

100
Q

What is a Birbeck granule?

A

Characteristic racquet-shaped intracytoplasmic granule seen in Langerhans cells

101
Q

What cell line are Langerhans cells derived from?

A

Myeloid

102
Q

The immune complexes seen in PSGN are composed of what?

A

IgG, IgM, and C3

103
Q

What is the function of pyruvate carboxylase?

A

Converts pyruvate to oxaloacetate

104
Q

How does acetyl-CoA stimulate gluconeogenesis?

A

Increases the activity of pyruvate carboxylase when acetyl-CoA is abundant

105
Q

The cytosol has enzymes necessary for what biochemical pathways?

A

Glycolysis, fatty acid synthesis, pentose phosphate pathway

106
Q

Mitochondria are the site for what biochemical pathways?

A

Beta-oxidation of fatty acids, citric acid cycle, and carboxylation of pyruvate

107
Q

HMG-CoA is important for what biochemical pathway?

A

Ketogenesis; found in the mitochondria

108
Q

What is the function of ornithine transcarbamylase? Where is it found?

A

Combines ornithine and carbamoyl phosphate to form citrulline; reaction occurs within the mitochondria

109
Q

What is a comon measure of fetal lung maturity?

A

Lecithin/sphingomyelin ratio

110
Q

Lecithin approximately equals sphingomyelin until what time of the pregnancy?

A

Middle of the 3rd trimester

111
Q

What is the treatment for gonorrhea?

A

Ceftriaxone and azithromycin (for resistance and potential chlamydia co-infection)

112
Q

Gram negative intracellular diplococcus describes what organism? What other important characteristics?

A

N gonorrhea; pili and non-motile

113
Q

What happens after decreasing PaCO2 in a mechanically ventilated patient?

A

Low PaCO2 results in vasoconstriction, which increases resistance and therefore decreases cerebral blood flow. The decreased cerebral blood flow decreases ICP

114
Q

What influences cerebral circulation?

A

Systemic blood pressure and arterial blood gas levels; systemic pressures of 60-140mmHg have little effect on cerebral blood volume

115
Q

What blood pressure values causes increased cerebral vascular volume?

A

BP >150 mmHg increases vascular volume and blood flow

116
Q

Blood pressures below what threshold cause cerebral hypoperfusion?

A

< 50 mmHg

117
Q

What is the most important regulator of cerebral blood flow?

A

Arterial blood gases, especially PaCO2

118
Q

What effect does carbon dioxide have on vasculature?

A

Vasodilator

119
Q

What are the important landmarks for pudendal nerve block?

A

Ischial spines and sacrospinous ligament

120
Q

Pudendal nerve serves sensory of what?

A

Perineum

121
Q

Pudendal provides motor innervation to what?

A

Urethral and anal sphincters

122
Q

Pudendal nerve is derived from what nerve roots?

A

S2-S4

123
Q

The sacrospinal ligament connects what?

A

Attached to the ischial spine and sacrum

124
Q

What runs medial to the pudendal nerve?

A

Internal pudendal artery and inferior gluteal artery

125
Q

Injury during closure of a pfannenstiel skin incision can result in damage to what nerve?

A

This is a c section; injury to iliohypogastric nerve

126
Q

The obturator provides motor and sensory innervation to what?

A

Medial thigh

127
Q

During what procedure can the obturator nerve be injured?

A

Retroperitoneal pelvic lymph node dissection

128
Q

How is a pudendal nerve block performed?

A

Injecting anesthetic intravaginally, medial to ischial spine through sacrospinous ligament

129
Q

What is the function of hepcidin? Where is it secreted from?

A

Acts as a central regulator of iron homeostasis-binds ferroportin and causes degradation; released by liver

130
Q

How is iron absorbed?

A

From the proximal small intestine facilitated by the divalent metal transporter-1 (DMT-1)

131
Q

What increases the synthesis of hepcidin?

A

High iron levels and inflammatory conditions

132
Q

What lowers hepcidin levels?

A

Hypoxia and increased erythropoiesis

133
Q

What is the cause of an imperofrate hymen?

A

Incomplete degeneration of the central portion of the fibrous tissue band connecting the walls of the vagina

134
Q

16 yo F presents with abdominal pain that seems to occur at the beginning of each month for a few days, then reside. She has never menstruated, is not sexually active, and has normal secondary sex characteristics. Exam reveals a palpable mass anterior to the rectum; B-hCG is negative. What is the most likely diagnosis?

A

Imperforate hymen

135
Q

What is Kallmann syndrome?

A

Impaired synthesis of GnRH by hypothalamus; presents with primary amenorrhea, absent secondary sex characteristics, and an olfactory sensory defect

136
Q

What is androgen insensitivity syndrome?

A

Genetically male, but have female phenotype due to resistance to testosterone

137
Q

How does androgen insensitivity syndrome present?

A

In adolescence, breast development results from peripheral conversion of testosterone to estrogen, but axillary and pubic hair are absent; primary amenorrhea occurs due to complete absence of mullerian structures

138
Q

If endometrial implants occur in the pouch of Douglas, what symptoms will result?

A

Painful defecation, dyspareunia

139
Q

Ankle sprain presenting with ecchymosis at the anterolateral aspect of the ankle may have injured what ligament?

A

Anterior talofibular ligament

140
Q

How do beta-blockers contribue to the therapy of thyrotoxicosis?

A

Reduce HR and anxiety; also decreases peripheral conversion of T4 to T3 by inhibiting iodothyronine deiodinase

141
Q

What are the indications for clozapine treatment?

A

Treatment resistant schizophrenia; schizophrenia associated with suicidality

142
Q

What are the AEs of clozapine?

A

Agranulocytosis, seizures, myocarditis, metabolic syndrome

143
Q

What is a significant AE of ganciclovir? What can increase the incidence of this AE?

A

Neutropenia; increased with co-administration of zidovudine or TMP-SMX

144
Q

What is the genome of parvovirus?

A

SsDNA virus

145
Q

What are the autopsy results of fetal hydrops?

A

Pleural effusion with secondary pulmonary hypoplasia and ascites

146
Q

What are the effects of an in utero infection of parvovirus?

A

Profound anemia and CHF; resulting in hydrops fetalis

147
Q

What is the genome of rubella?

A

Enveloped ssRNA virus

148
Q

How is coronary dominance determined?

A

By the coronary artery supplying the posterior descending artery (which also supplies blood to the AV node)

149
Q

The posterior descending artery originates from what?

A

Right coronary artery (70%), both right coronary and left circumflex artery (20%), and the left circumflex artery (10%)

150
Q

What is the cause of Graves disease?

A

Type II HSR - IgG stimulating TSH receptors

151
Q

On jugular venous pressure tracings, what is the first peak?

A

The a wave - generated by atrial contraction

152
Q

The a wave on a jugular venous pressure tracing is absent when?

A

Atrial fibrillation

153
Q

The y descent on jugular venous tracings does what in constrictive pericarditis?

A

Becomes more prominent - steeper and deeper during inspiration

154
Q

What happens to the a wave on a jugular venous tracing in hypertrophic cardiomyopathy?

A

Prominent a wave

155
Q

What are common features on CT in constrictive pericarditis?

A

Calcification and thickening

156
Q

That are the effects of tetrodotoxin?

A

Binds Na channels, inhibiting influx and preventing action potential conduction; results in paresthesias, weakness, dizziness, nausea

157
Q

What determines the ventricular contraction rate in conditions where the atria undergo rapid depolarization?

A

The AV node refractory period

158
Q

How is cyclosporine metabolized?

A

By the liver and GI tract CYP450 enzymes (CYP3A specifically)

159
Q

What is the most signifiant SE of cyclosporine?

A

Nephrotoxicity

160
Q

What compromises the dorsal columns of the spinal cord?

A

Gracile and cuneate fasciculus

161
Q

Gracile fasciculus and cuneate fasciculus are important for what?

A

Position and vibration senses

162
Q

What are the lateral corticospinal tracts important for? Damage results in what?

A

Upper motor neuron pathways;spastic paresis, hyperreflexia, pathologic reflexes

163
Q

Poliomyelitis primarily affects what part of the spinal cord?

A

Anterior horns

164
Q

What genes are associated with Lynch syndrome?

A

MSH2, MLH1, MSH6, PMS2

165
Q

What genes are associated with familial adenomatous polyposis?

A

APC

166
Q

What cancers are associated with lynch syndrome?

A

CRC, endometrial cancer, ovarian cancer

167
Q

In HNPCC, what is the genetic defect?

A

Mutation in gene responsible for DNA mismatch repair

168
Q

How do nitrates decrease LV wall stress?

A

Reduced preload (decreased LVEDV); decreases myocardial oxygen demand

169
Q

Bisphosphontes have a chemical structure similar to what?

A

Pyrophosphate - attache to hydroxyapatite binding sites

170
Q

What is the MOA of bisphosphonates?

A

Bind to hydroxyapatite binding site, osteoclasts take up the bisphosphonate and are unable to continue resorption, so the osteoclasts undergo apoptosis

171
Q

What is the MOA of bosentan? When is it used?

A

Endothelin-receptor antagonist; used in pulmonary arterial hypertension

172
Q

What is the gene mutation seen in familial pulmonary artery hypertension?

A

BMPR2

173
Q

What direction does DNA synthesis occur in?

A

5’ to 3’ direction

174
Q

What enzyme has 5’ to 3’ exonuclease activity?

A

DNA poly I in prokaryotes

175
Q

What type of necrosis is seen after irreversible ischemic injury in most tissues?

A

Coagulative necrosis

176
Q

When is fat necrosis seen?

A

Acute pancreatitis