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