Review Topics Flashcards

1
Q

Tay-Sachs disease is due to a deficiency of _________, leading to accumulation of __________

A

Hexosaminidase A; GM2 ganglioside

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

In the case of gas gangrene caused an organism that when cultured on blood agar shows a double zone of hemolysis, the causative organism is most likely ____________, which possesses ______ as a virulence factor responsible for erythema, necrosis, warmth, crepitus, and strong odor associated with the wound

A

Clostridium perfringens; alpha toxin

[MOA of alpha toxin is lysis of phospholipids]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What amino acid is essential in the diet of a patient with phenylketonuria?

A

Tyrosine

[treatment of PKU includes elimination of phenylalanine from the diet and supplementation of tyrosine because pts are no longer able to synthesize tyrosine]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

A mutation in the DNA mismatch repair system leads to increased risk of __________

A

Colon cancer

[Hereditary nonpolyposis colorectal cancer (HNCC)]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Karyotype of a partial mole vs. complete mole

A

Partial mole = 69,XXY or 69, XXX

Complete mole = 46, XX or 46 XY

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Vitamin that is an essential component of coenzyme A

A

Pantothenate (Vit B5)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

______ = bacteriophage mediated transfer of genetic material between 2 prokaryotes

A

Transduction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Morphology of HPV

A

Non-enveloped double-stranded circular DNA virus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Increased intracranial pressure, dry skin, alopecia, sore throat, and hepatomegaly are symptoms of vitamin _____ excess

A

Vitamin A (retinol)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Vitamin B12 deficiency can cause a macrocytic anemia. Lab results show increased _____ and _____

A

Homocysteine; methylmalonyl coA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Actinomyces israelii causes extensive fibrosis and eventually a lumpy jaw with a wooden consistency during its chronic phase. Describe the growth of this organism during the chronic phase

A

Forms communicating sinus tracts that release sulfur granules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Teratogenic antiseizure drug that interferes with folate absorption in the GI tract leading to potential neural tube defects

A

Valproic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Oral medication for MRSA and its MOA

A

Linezolid

Binds 23s RNA and interacts with the bacterial initiation complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

MOA of mirtazapine

A

Alpha-2 antagonist

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Acute colonic pseudo-obstruction with signs, symptoms, and radiographic appearance of a large bowel obstruction, but with no mechanical cause of obstruction; commonly seen in elderly males after major surgery

A

Ogilvie’s syndrome

[management = correction of underlying condition, anticholinesterases, and colonic decompression]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

IV medication used to decrease intracranial pressure in the setting of diffuse intracerebral hemorrhage; works through inhibition of the sodium channels and water reabsorption at the proximal tubule as well as both parts of the loop of henle

A

IV mannitol

[pulls water via osmosis from tissues such as cerebral parenchyma into the blood and then into the urine where it can be eliminated, resulting in a decreased intracerebral pressure]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Erb-Duchenne palsy clinical features and etiology

A

Etiology = damage to C5 and C6 nerve roots

Clinical features = paralysis of abductors, lateral rotators, and biceps brachii —> arm is pronated and medially rotated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Mechanism of resistance to methicillin in MRSA

A

Methicillin resistance in S.aureus is encoded by the mecA gene, which encodes the enzyme PBP2a, an ALTERED PENICILLIN BINDING PROTEIN that has reduced affinity for beta-lactam antibiotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

A popliteal artery aneurysm may cause symptoms due to a mass effect on other structures that lie in the popliteal fossa, such as the popliteal vein and the tibial nerve. If the tibial nerve is damaged, what 2 motor and 2 sensory areas would be affected?

A

Plantar flexion
Foot inversion

Sensation along posterior calf and sole of the foot

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

4 classical clinical features of minimal change disease (most common cause of childhood nephrotic syndrome)

A
  1. Nephrotic range proteinuria (>50mg/kg/day or 4+)
  2. Hypoalbuminemia (<30g/L)
  3. Edema
  4. Hyperlipidemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Biochemical process affected by G6PD deficiency

A

Fatty acid synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

MOA of bacillus anthracis toxin

A

Anthrax edema toxin is an adenylate cyclase that increases intracellular cAMP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Anthrax edema toxin is an adenylate cyclase that increases intracellular cAMP. What 2 other bacteria have a toxin that is most similar?

A

Bordetella pertussis
Vibrio cholerae

[MOA of their toxin is to activate adenylate cyclase]

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

MOA of toxin associated with Corynebacterium diphtheria and Pseudomonas aeruginosa

A

ADP-ribosylation of EF-2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
MOA of toxin associated with C.botulinum
Inhibits release of ACh at synaptic vesicles
26
MOA of toxin associated with C.tetani
Inhibits release of GABA and glycine
27
What 2 bacteria have a toxin that inhibits protein synthesis by degrading ribosome subunit 60S?
Shigella dysenteriae E.coli O157:H7
28
MOA of the 2 toxins associated with S.aureus
Alpha toxin forms holes in membrane Beta toxin degrades sphingomyelin
29
________ is administered with chemotherapeutic agent Doxorubicin to prevent the worrisome side effect of _________
Dexrazoxane; cardiotoxicity [can cause CHF, dilated cardiomyopathy, and death in dose-dependent manner]
30
In the setting of carcinoid syndrome, patients are at risk of what vitamin deficiency?
Vitamin B3 (niacin) [this is because tryptophan (precursor to both niacin and serotonin) is shunted to serotonin synthesis, thus leading to a niacin deficiency]
31
Tapeworm that causes cysts in organs, especially the liver (hydatid disease)
Echinococcus granulosus
32
Dopamine agonists, such as __________ and ________, inhibit prolactin secretion and cause shrinkage of prolactin-producing adenomas
Cabergoline; bromocriptine
33
The presence of _______ and _______ in the urine are indicative of lead poisoning
Coproporphyrin; d-aminolevulinic acid [this is because ingested lead interferes with heme synthesis by blocking both ferrochelatase and aminolevulinic acid dehydratase]
34
_______ is a MAO-B inhibitor utilized in parkinsons that slows the metabolism of levodopa, enhancing its effect, but can increase side effects caused by levodopa
Selegiline
35
Irinotecan has been used either as monotherapy or in conjunction with 5-FU with or without leucovorin for treatment of advanced colorectal cancer. What is the MOA of irinotecan?
Inhibition of topoisomerase I
36
A primary lesion in left temporal lobe would cause what pattern of visual loss?
Right upper quadrantic anopia
37
Gaucher’s disease is due to a deficiency of ____________, leading to an accumulation of __________. Pts can have hepatosplenomegaly, anemia, thrombocytopenia, osteopenia, and avascular necrosis. Gaucher’s cells are macrophages with lipid accumulation that give appearance of wrinkled paper
Beta-glucocerebrosidase; glucocerebroside
38
Krabbe’s disease is caused by a deficiency of ______, leading to elevated levels of ________. Pts present with peripheral neuropathy, atrophy of the optic nerve, and developmental delay
Galactocerebrosidase; galactocerebroside
39
The long thoracic n. innervates the serratus anterior m. and may be injured during a mastectomy. PE findings generally include “winging” of the scapula and difficulty with what action?
Abduction of the arm past 90 degrees
40
First line treatment for Giardia infection
Metronidazole [can cause disulfuram reaction with alcohol; other drugs that do this include griseofulvin (antifungal), some of the cephalosporins, and sulfonylureas]
41
Side effect most commonly seen with medication used to treat mucormycosis (caused by Mucor, Rhizopus, or Absidia)
Nephrotoxicity [caused by Amphotericin B; other adverse effects include infusion reaction (fever, chills, and myalgias)]
42
Pain and inability to rotate the shoulder medially indicate injury to what rotator cuff muscle?
Subscapularis [subscapularis also does adduction]
43
Nephritic syndrome that presents as hematuria (gross or microscopic) discovered within 1-3 days of an upper respiratory or gastrointestinal infection
IgA nephropathy (aka Berger’s disease) [Note: key difference with acute poststreptococcal glomerulonephritis (another nephritic syndrome associated with hematuria following a URI) is TIMING. While Berger’s disease has hematuria within 1-3 days of URI, acute PSGN has hematuria 1-3 weeks after URI. Acute PSGN also appears as “lumpy bumpy” on light microscopy with low C3 levels]
44
_______ is an autosomal recessive genetic disorder that leads to hexagonal-shaped crystals in the urine, often causing bilateral renal stones at an early age. Treatment includes ______, which alkalizes the urine, thereby preventing precipitation of these crystals
Cystinuria; acetazolamide
45
Most likely location for aspirated foreign bodies while pt is upright/seated vs. supine
Seated = posterobasal segment of right lower lobe Supine = superior segment of right lower lobe
46
What cells release CCK?
I cells
47
____ cells release somatostatin hormone, responsible for decreasing gastric acid and pepsinogen secretion, pancreatic and small intestine fluid secretion, gallbladder contraction, and insulin and glucagon release
D
48
_____ cells are located in the antrum of the stomach and release gastrin, which is responsible for increasing ______ secretion, growth of gastric mucosa, and gastric motility
G; gastric H+
49
S cells are responsible for releasing ______ in response to increased levels of acid in the lumen of the duodenum; other functions include increasing bicarbonate and bile secretion, as well as decreasing gastric acid secretion
Secretin
50
How is renal reabsorption rate of a substance calculated?
Reabsorption rate = (Filtered Load) - (Excretion) Filtered load = GFR x plasma concentration Excretion = urine flow rate x urine concentration
51
What autonomic receptors are considered Gs-linked?
``` Beta 1 Beta 2 D1 H2 V2 ``` [alpha 1, M1, and M3 are Gq-linked. D2 and M2 are Gi-linked]
52
What are 3 options for prophylactic treatment for mycobacterium infections?
Isoniazid Azithromycin Clarithromycin
53
_______ is used for moderate-to-severe persistent asthma in pts who react to perennial allergens wherein symptoms are not controlled by inhaled corticosteroids
Omalizumab
54
Most common helminthic infection worldwide; usually asymptomatic, but some patients present with pulmonary and GI complaints. Stool microscopy shows characteristic thick shelled parasitic eggs.
Ascaris lumbricoides [nematode/roundworm]
55
Cold agglutinin disease is an autoimmune disease characterized by the presence of high concentrations of circulating _____ antibodies directed against RBCs. Patients will have low levels of circulating _____ due to consumption of that protein
IgM; C3b [Note: IgG is associated with warm agglutinins]
56
______ poisoning inhibits the ETC in the mitochondria, inhibiting ATP production via oxidative phosphorylation. Exposure can lead to altered mental status, new onset seizures, hepatic failure, and rhabdomyolysis leading to renal failure. Early in the course, pts have flushed skin with tachycardia, tachypnea, and HTN. Later, pts may have cyanosis, bradycardia, bradypnea, and hypotension. Treatment involvees IV administration of either a mixture of sodium nitrite and sodium thiosulfate, or hydroxocobalamin
Cyanide
57
Parasitic infection with clinical manifestations including periorbital swelling, myalgia, and myositis involving facial and neck muscles, conjuntival hemorrhage, and splinter hemorrhages. Muscle biopsy will show encysted larvae.
Trichinella spiralis
58
Interferon-alfa combined with ribavirin and a NS3/4A protease inhibitor is occasionally used to treat chronic hepatitis C if other drugs are not available. What are the 3 major side effects of this treatment?
Flu-like symptoms Neutropenia Depression
59
Where is the right mainstem bronchus in relation to the right pulmonary artery?
Posterior [mnemonic RALS to remember positions of pulmonary arteries in relation to their respective bronchi: Right pulmonary a. is Anterior, Left pulmonary a. is Superior]
60
How is renal potassium metabolism altered in the case of respiratory acidosis?
Potassium secretion in the distal tubules is decreased [explanation: High concentrations of H+ in the ECF cause H+ to enter cells in exchange for K+. This reduces the intracellular K+ concentration, resulting in hyperkalemia. Secretion in the distal tubules is reduced by any factor that reduces the intracellular K+ concentration]
61
T/F: anabolic steroid use may lead to prostatic hyperplasia
True [the prostate is a testosterone dependent gland and may increase in size due to steroid’s androgenic effects]
62
Four basic factors that cause a right-shift of oxygen-hemoglobin dissociation curve
Decrease in pH Increase in 2,3-BPG Increase in PCO2 Increase in temperature
63
Parkinsonian features, visual hallucinations, and waxing/waning mental status are characteristic of what type of dementia?
Lewy body dementia
64
Hematuria, mild proteinuria, fever, eosinophilia, nausea, vomiting, and rash are all features of __________________, which can occur within a few days of administration of an offending agent such as penicillin.
Acute interstitial nephritis [Note: difference in acute tubular necrosis is that there are muddy brown casts in the urine and eosinophilia is not a feature]
65
______________ is a paraneoplastic syndrome characterized by development of antibodies to presynaptic voltage gated calcium channels, leading to decreased release of ACh at the NMJ. Pts present with symmetric muscle weakness, most prominently in the proximal muscles of the lower extremities. It is associated with __________ cancer, so a ________ should be included in the workup
Lambert-Eaton Syndrome; Small cell lung cancer; CXR
66
___________ media is the culture media of choice for growth of Neisseria gonorrhea. It contains _____ to inhibit gram-positive bacterial growth, _______ to inhibit gram-negatives other than Neisseria, and ______ to inhibit fungal growth
Thayer-Martin Vancomycin; Polymyxin; Nystatin [Thayer-Martin = VPN media]
67
Vitamin B1 is essential for the synthesis of thiamine pyrophosphate (TPP), which is an active carrier of aldehydes and enzymes use it to transfer aldehydes to another molecule during the reaction. The enzyme ______ is involved in the hexose monophosphate shunt and uses TPP as its sole cofactor
Transketolase
68
During which phase of bacterial growth does spore formation begin — lag phase, log phase, stationary phase, or death phase?
Stationary phase [Lag phase = metabolic activity w/o division; log phase = rapid cell division; death phase = period of prolonged nutrient depletion and buildup of metabolic waste products leading to death]
69
Breakdown products of disaccharides lactose, sucrose, and maltose
Lactose = Glucose + Galactose Sucrose = Glucose + Fructose Maltose = Glucose + Glucose
70
Head lice is typically treated with topical _______. However, resistance to topical medications has grown. When resistance occurs, systemic treatment may be initiated, and ______ is the best option
Permethrin; ivermectin
71
N.meningitidis is a gram-negative diplococci that has a _______ capsule and ferments ______ and ______
Polysaccharide; glucose; maltose
72
3 features of irreversible cell injury
Severe membrane damage Calcium influx Release of intracellular enzymes (i.e., lysosomal rupture)
73
3 treatment options for heparin-induced thrombocytopenia (HIT) upon discontinuation of heparin
Lepirudin Argatroban Fondaparinux [Lepirudin and Argatroban are direct thrombin inhibitors, and Fondaparinux catalyzes the pt’s endogenous antithrombin to inhibit factor X (indirect factor X inhibitor)]
74
How do functional residual capacity (FRC), vital capacity, and total lung capacity (TLC) change in interstitial lung disease?
All decrease
75
Are acetyl-CoA carboxylase, arginase I, and PEP carboxykinase enzymes found in the mitochondria or the cytosol?
Cytosol
76
Are citrate synthase, carbamoyl phosphate synthetase I, and pyruvate carboxylase found in the mitochondria or the cytosol?
Mitochondria
77
When treating a patient with pheochromocytoma for hypertension, phenoxybenzamine may be used. What is the MOA?
Irreversible alpha adrenergic antagonist
78
Biliary complication associated with ulcerative colitis
Primary Sclerosing Cholangitis [Causes inflammation, fibrosis, and stricturing of medium and large-sized ducts in the intrahepatic and extrahepatic biliary tree. Clinically presents with fatigue and pruritis. Histology shows fibrous obliteration of epithelium and CT replacement in an onion-skin pattern]
79
Tumor marker for pancreatic cancer
CA 19-9
80
Tumor marker for colon cancer
CEA
81
Nontender testicular mass, elevated serum placental alkaline phosphatase, sheets of relatively uniform tumor cells with prominent nucleoli and clear cytoplasm — What type of tumor?
Seminoma
82
Wilson disease can affect the kidneys leading to what complication and subsequent electrolyte abnormality?
Renal tubular acidosis type II Results in hypokalemic, hyperchloremic nonanion gap metabolic acidosis
83
Diabetic cataracts are an end-organ consequence of uncontrolled DM. Glucose can enter cells via facilitated glucose transporters of GLUT family. Once in the cell it can be phosphorylated to glucose-6-phosphate by either glucokinase or hexokinase. Glucose-6-phosphate cannot leave the cell and can enter glycolysis. In the lens, nerve, and kidney cells glucose can also be converted to _________, a reaction accomplished by the enzyme _______ ______ using cofactor NADPH.
Sorbitol; aldose reductase
84
2 primary treatment options for bacterial vaginosis due to Gardnerella vaginalis infection
Metronidazole Clindamycin
85
Acid-base disturbance caused by hyperventilation
Respiratory alkalosis
86
T/F: the membrane potential of the postsynaptic membrane does not change as a result of rocuronium for anesthesia induction
True — Rocuronium is a non-depolarizing muscle relaxant that acts as a competitive antagonist at the NMJ causing muscle paralysis without membrane depolarization
87
MOA of Metformin
Decreases hepatic glucose production by inhibition of liver gluconeogenesis [can also decrease intestinal glucose absorption and increase insulin sensitivity]
88
Where is intrinsic factor produced?
Parietal cells within the gastric mucosa
89
CML is most commonly associated with a _______ translocation, usually resulting in the ______ fusion gene, which leads to constitutional activation of ________ _______ activity
t(9;22); BCR-ABL; Tyrosine kinase
90
Treatment agent of choice for CML
Imatinib (tyrosine kinase inhibitor) [this is because BCR-ABL fusion gene leads to constitutive activity of a tyrosine kinase!!]
91
Self-limited hypothyroidism that is typically seen following a viral, flu-like illness. Initially presents as transient hyperthyroidism as well as tender, enlarged thyroid gland with referred jaw pain
de Quervain thyroiditis
92
Spinal cord somatosensory pathway that conveys light touch, vibration, and proprioception
Posterior column
93
Spinal cord somatosensory pathway that conveys pain, temperature, and crude touch
Spinothalamic tract
94
The de novo synthesis of pyrimidines relies on the enzymatic conversion of orotic acid to _______ ________
Uridine monophosphate
95
In ________ palsy, pts have paralysis of the ipsilateral hand and Horner’s syndrome on the ipsilateral face. This is due to damage to _____ and _____ nerve roots
Klumpke; C8, T1
96
Cori’s disease is an inherited glycogen storage disease caused by a deficiency of __________, an enzyme responsible for debranching of glycogen during glycogenolysis. While not the most severe glycogen storage disease, pts still experience fasting hypoglycemia due to inability to use stored glycogen. As a result, gluconeogenesis is upregulated. The regulation of gluconeogenesis occurs at several key steps, but only ________ serves as an allosteric activator. This allosteric activation occurs with the reaction catalyzed by pyruvate carboxylase, which converts pyruvate to OAA. This reaction also requires ______ as a source of energy, and _____ as a cofactor, but these are not allosteric regulators of the enzyme.
Alpha 1,6-glucosidase Acetyl-CoA ATP; Biotin
97
Croup may present on X ray with narrowing of the trachea d/t inflammation, called “steeple sign”. What is the most common cause of croup?
Parainfluenza virus
98
Which of the following is not found in the outpouching of tissue associated with a Zenker’s diverticulum? ``` A. Lamina propria B. Muscularis mucosa C. Muscularis propria D. Submucosa E. This is a true diverticulum and contains all of the above layers ```
C. Muscularis propria [Zenker’s diverticulum is a pseudodiverticulum, so it does not contain all mucosal tissue layers. It has mucosal and submucosal tissue, but not the muscularis propria]
99
Mitochondrial disorder that causes painless progressive loss of central vision. Other clinical manifestations include dystonia, pseudobulbar palsy, intellectual deterioration, and muscle weakness. Many patients also have Wolff-Parkinson-White syndrome.
Leber hereditary optic neuropathy [Caused by mtDNA mutations that encode subunits of ETC complex I. Biopsy shows loss of retinal ganglion cells and degeneration of papillomacular bundle]
100
Dobutamine may be used to enhance contractile ability of the heart in pts with CHF exacerbation. It exerts its effect primarily on _____ receptors, which are GPCRs of the ______ subtype.
Beta-1; Gs
101
__________ presents in infancy with symptoms of lethargy, vomiting, jaundice, hepatomegaly, and bilateral cataracts while taking breast milk
Galactosemia [deficiency in galactose-1-phosphate uridyltransferase]
102
Disease and enzyme deficiency associated with the following clinical presentation: 11 year old girl of Ashkenazi Jewish descent presenting with hepatosplenomegaly, anemia, thrombocytopenia, osteopenia, and avascular necrosis
Gaucher’s diease Deficiency of beta-glucocerebrosidase [note: diagnostic histologic finding = lipid-filled macrophages with wrinkled-paper appearance]
103
What effect do competitive inhibitors have on Km and Vmax?
Km increases Vmax stays the same
104
Which of the following inhibits microtubule depolymerization? ``` A. 6-mercaptopurine B. Vincristine C. Cyclophosphamide D. Cisplatin E. Paclitaxel ```
E. Paclitaxel [note: vincristine inhibits microtubule POLYMERIZATION while paclitaxel inhibits microtubule DEPOLYMERIZATION]
105
The urea cycle is the pathway responsible for synthesis of what amino acid?
Arginine
106
Vertebral levels at which the celiac trunk, SMA, and gonadal artery arise from the abdominal aorta
Celiac trunk = T12 SMA = L1 Gonadal = L2
107
The inferior mesenteric artery arises from the abdominal aorta at vertebral level _______. The bifurcation of the aorta occurs at vertebral level ______.
L3; L4
108
Embryonic origin of prostate gland
Urogenital sinus
109
Brugada syndrome is a condition associated with a high risk of _____ _______ _____ in young adults
Sudden cardiac death
110
______ ______ presents on ECG as persistent ST elevations in leads V1-V3 with RBBB +/- terminal S waves in lateral leads (associated with typical RBBB); may also see prolongation of PR interval
Brugada syndrome [Note: Pts with Brugada syndrome may present with normal ECGs, with abnormalities appearing when pt has a fever, takes a Na-channel blocker, takes a TCA, or uses cocaine. Adrenergic stimulating decreases the ST elevation, while vagal stimulation worsens it]
111
__________ testicular tumors present in early childhood and secrete alpha-fetoprotein, but not hCG. They have a number of morphologic variants, but ___________ are diagnostic for these tumors, resembling primitive glomeruli
Yolk sac; Schiller-Duvall
112
What is the only apolipoprotein associated with LDL, binding LDL receptor on the liver?
Apolipoprotein B-100
113
Most abundant amino acid in collagen
Glycine
114
_______ ______ occurs due to deficiency of acid maltase. Thus, glycogen accumulates in lysosomes and cytoplasm, leading to cardiomyopathy, hypotonia, and hepatomegaly. Pts may present in the first few months of life or later in adulthood. When presenting in infancy, children commonly have respiratory distress, difficulty with feeds, and cardiomegaly
Pompe’s disease
115
Nitroprusside is a potent arterio- and venodilator indicated when rapid reduction of blood pressure is desired, as in the case of malignant hypertension. However, nitroprusside is converted to ______ in the bloodstream, and thus has the potential to cause toxicity, particularly in the setting of prolonged exposure.
Cyanide
116
3 serious adverse reactions that need to be monitored for in patients taking methimazole (antithyroid medication)
Agranulocytosis Aplastic anemia Hepatotoxicity
117
Benztropine is an _______ medication that is used to decrease the resting tremor and rigidity associated with Parkinson’s disease. It does not have any impact on bradykinesia.
Anticholinergic
118
Most common site of renal stone obstruction in children
Ureteropelvic junction
119
Abciximab functions by inhibiting a platelet integrin called ___________. This is the same molecule is defective in _______ _________ (disease)
GP IIb/IIIa Glanzmann Thrombasthenia
120
How does emphysema affect compliance and FRC?
Both are increased
121
What is Conn Syndrome?
Primary aldosteronism caused by hyperaldosteronism originating in the adrenal gland Characterized by HTN secondary to sodium retention, hypokalemia, and decreased renin secretion due to negative feedback of increased BP on renin secretion
122
Congenital condition affecting capillaries leading to port-wine stain, leptomeningeal angioma, seizures, glaucoma, and mental retardation
Sturge-Weber syndrome
123
The ganglionic destruction in achalasia begins with irreversible of the esophageal myenteric neruons, ultimately leading to fibrosis and scarring of Auerbach’s plexus. In particular, the neurons destroyed are important in secretion of ______ ______, thus secretion of this substance is decreased in achalasia
Nitric oxide
124
Which class of anti-asthma drugs exhibits hepatotoxicity?
Antileukotrienes (e.g., Zileuton)
125
A lesion in the ______ ______ will lead to a right or left upper quadrantic anopia
Temporal lobe
126
Anticoagulant treatment for atrial fibrillation that does not require monitoring through routine blood draws
Dabigatran (direct thrombin inhibitor)
127
Inheritance of Marfan syndrome
Autosomal dominant
128
Is the IVC intraperitoneal or retroperitoneal?
Retroperitoneal
129
Is the pancreas intraperitoneal or retroperitoneal?
The head, neck, and body are retroperitoneal The tail is intraperitoneal (located in splenorenal ligament)
130
Of the 4-drug regimen used to treat mycobacterium tuberculosis infection, _________ frequently produces visual problems due to retrobulbar neuritis, including laack of acuity and color blindness. The mechanism of action of this medication is to inhibit _______ _______, interfering with the mycobacterial cell wall
Ethambutol; arabinosyl transferase
131
3 types of antibody associated with antiphospholipid antibody syndrome
Lupus anticoagulant Anti-cardiolipin Anti-beta2-glycoprotein
132
Lesions within the ______ ______ cause contralateral hemiballismus
Subthalamic nucleus
133
MOA of heparin
Potentiates pt’s endogenous antithrombin [thus inactivating thrombin and factor Xa]
134
What causes impaired vision in the case of giant cell arteritis?
Occlusion of the ophthalmic artery
135
Triad associated with Plummer Vinson syndrome
Esophageal webs Glossitis Iron deficiency anemia
136
Pyruvate kinase is responsible for conversion of phosphoenolpyruvate to pyruvate, a reaction that produces 2 ATP molecules. Without functioning enzymes (e.g., Pyruvate Kinase Deficiency), phosphoenolpyruvate builds up and is shunted to an alternative pathway, generating ________________ which results in increased tendency for hemoglobin to release oxygen
2,3-bisphosphoglycerate (2,3-BPG)
137
________ is a chemotherapeutic that may cause myelosuppression due to inhibition of thymidylate synthase. The rescue for this myelosuppression is _______
5-FU; thymidine
138
The head of the pancreas is supplied by the __________ artery, which is a primary branch of the _______ ______ artery
Pancreaticoduodenal; superior mesenteric
139
Nerve roots associated with radial nerve
C5, C6, C7, C8, T1
140
Apolipoprotein B-48 is found in ______ and helps facilitate their secretion
Chylomicrons
141
Hydralazine may increase the risk of drug-induced lupus, which presents with myalgias, arthralgias, and red scaly rash. What genetic abnormality may predispose pts to drug-induced lupus with lipid soluble drugs?
Polymorphisms in the genes for phase II reactions of lipid-soluble drugs — like glucuronidation, sulfurization, or acetylation
142
Hereditary or acquired defect in the PCT that occurs almost exclusively in caucasian males which results in losses of glucose, sodium, phosphate, protein, and other solutes in the urine
Fanconi syndrome
143
What complication of hyperparathyroidism is characterized by “brown tumors” that form in bone leading to increased risk of fracture; the lesions are cysts filled with blood, fibrous tissue, and osteoclasts
Osteitis fibrosa cystica
144
The ______ ______ contains the primary motor cortex and so damage to this region would cause major motor deficits such as hemiparesis
Precentral gyrus
145
Side effect most likely to limit patient’s ability to tolerate a fibrate for hyperlipidemia
Myalgia [Fibrates activate PPARs and can cause muscle damage, especially when combined with statins]
146
The scleroderma variant associated with CREST syndrome has a positive ____________ antibody
Anti-centromere
147
Depletion of ________ is responsible for the reduction of glycolysis in the setting of lactate dehydrogenase deficiency
NAD+ [if skeletal muscle lacks lactate dehydrogenase, NADH can not be oxidized to NAD+ and the cori cycle cannot provide the glucose input. Thus ATP production via anaerobic fermentation is reduced, ATP production drops, and muscle activity decreases]
148
Yellow fever virus is a type of _________ found in the saliva of the Aedes aeqypti mosquito typically found in South America and sub-Saharan Africa. These viruses are enveloped, single-stranded, positive-sense, linear RNA viruses.
Flavivirus
149
______ is a recognized tumor-suppressor gene and has been shown to be down-regulated in extranodal NK/T cell lymphoma. When expressed, it inhibits cell growth through activation of apoptosis as well as arrest of the cell cycle. When it is down-regulated, there is decreased apoptosis of the malignant cells
FOXO3
150
Acute severe lead intoxication can be treated by _______ or CaNa2EDTA
Dimercaprol
151
Drug of choice for absence seizures
Ethosuximide [blocks T-type Ca++ channels in the thalamus]
152
Major side effects of the chemotherapeutic _________ include myelosuppression, GI upset, and hepatotoxicity. Allopurinol inhibits xanthine oxidase, the enzyme that metabolizes this drug, so concurrent allopurinol therapy increases the risk of side effects.
6-mercaptopurine
153
________ _______ can mimic acute coronary syndrome, including ST elevation in precordial leads (V1-V4) and ballooning of the heart’s apex and midventricular hypokinesis on echocardiography, and often follows a severe physical or psychosocial stressor
Takotsubo cardiomyopathy
154
_______, _______, and _______ all stimulate parietal cells within the stomach to produce acid to decrease the stomach’s pH
Histamine, Acetylcholine, and Gastrin
155
What organelle are the CYP450 enzymes found in?
Smooth ER
156
Most patients with anticholinergic toxicity can be treated with supportive care only until the effects of the drug wear off; however, ________ is indicated when one of the following is present: (1) tachydysrrhythmias with hemodynamic compromise, (2) intractable seizures, or (3) severe agitation or psychosis
Physostigmine
157
What organ is affected most in mercury exposure?
Kidneys [Mercury exposure can cause membranous glomerulonephritis, leading to NEPHROTIC syndrome]
158
Heme, which is synthesized from porphyrin, is dependent upon sufficient quantities of amino acid _______ for its synthesis. Deficiency would result in a non-iron deficiency anemia
Glycine
159
To avoid chronic lactic acidosis in pyruvate dehydrogenase complex deficiency, a diet rich in what 2 amino acids is recommended?
Lysine Leucine [these are the 2 purely ketogenic amino acids]
160
______________ artery aneurysms can lead to visual disturbances due to compression of the optic nerve
Anterior communicating artery
161
The inability to rotate the shoulder medially indicates injury to what rotator cuff muscle?
Subscapularis [Infraspinatus does lateral rotation, Teres minor adducts and laterally rotates, supraspinatus helps deltoid abduct]
162
What type of heart murmur would you expect in a pt with longstanding ankylosing spondylitis?
Aortic regurgitation (soft, high-pitched early diastolic murmur)
163
Autosomal recessive enzyme deficiency in which diagnosis is established by identifying lab abnormalities, including anion gap metabolic acidosis, hypoketotic hypoglycemia, and the presence of dicarboxylic acids in the urine
Acyl-CoA dehydrogenase deficiency
164
4 most common primary tumor sites that metastasize to the lungs
Colon Breast Prostate Bladder
165
Typical antipsychotics primarily block the _____ receptor. Atypical antipsychotics offer a more favorable side effect profile than typical antipsychotics due to additional antagonism at the _____ receptor
D2; 5-HT2
166
Blood supply to the proximal 1/3, middle, and distal 1/3 of the ureter
Proximal 1/3 = Renal a. Middle = Small branches from common iliac a. Distal 1/3 = Superior vesical a.
167
In a patient with a history of transplant over 100 days ago, typical findings of _____________ include erythema, hyper/hypopigmentation, induration, atrophy, and skin thickening. Patients also complain of ocular involvement, including aqueous tear deficiency, cataracts, conjunctival hyperemia, and corneal epithelial staining. Hair and nail dystrophy can also occur. The liver is affected in about half of patients, most commonly manifesting as liver function test abnormalities
Chronic graft-versus-host disease
168
The most commonly used prophylactic agent in the prevention of chronic graft-vs-host disease is _________. This is a potent immunosuppressive that can lead to severe renal insufficiency and hyperbilirubinemia. In patients with significant side effects, ________ can be given as a rescue regimen
Methotrexate; leucovorin [leucovorin is a reduced form of folic acid]
169
_________ are tumors that are most commonly found within the cerebellum, arising from the vasculature. Patients may present with symptoms of cerebellar ataxia (i.e., wide based gait). Most are sporadic, but they can be seen in association with __________ syndrome, in which case there may be multiple throughout the CNS. Surgical resection is warranted because bleeding from these tumors can be fatal
Hemangioblastomas; VHL
170
Treatment for Tourette’s syndrome
Atypical and typical neuroleptics, like haloperidol
171
_______ _______ _______ is characterized by repetitive seizures consisting of brief, often symmetric, muscular contractions with loss of body tone causing the patient to fall or slump forward. Seizures usually occur in the morning and are precipitated by stress and sleep deprivation. ________ ______ is often used to treat these seizures
Juvenile myoclonic epilepsy; Valproic acid
172
What characteristic of HCV leads to its high genetic heterogeneity?
HCV lacks RNA polymerase proofreading exonuclease
173
When used in the treatment of HIV, maraviroc antagonizes the ______ receptor on white blood cells, preventing its interaction with the ______ protein of HIV.
CCR5; gp120
174
________ ________ is due to a deficiency in galactocerebrosidase, leading to accumulation of galactocerebroside. Clinical features include peripheral neuropathy, optic atrophy, and developmental delay in infancy. ______ cells can be seen in the white matter of the CNS; these cells are macrophages clustered near blood vessels that have numerous nuclei and enlarged cytoplasm
Krabbe’s disease; Globoid [Krabbe’s disease = Globoid cell leukodystrophy]
175
Chronic carriers of HBV infection have increased chances of malignant transformation to hepatocellular carcinoma. This develops independently of the progression to cirrhosis, so what is the mechanism of carcinogenesis?
HBV viral incorporation into the hepatocellular genome, leading to genomic instability
176
Parasitic roundworm found in tropical climates (such as Southeast Asia) that can present with vague GI complaints as well as pulmonary and dermatologic problems. With chronic infection, dermal migration of larvae becomes apparent as serpiginous eruptions in the skin
Strongyloides stercoralis
177
Inheritance of Hemophilia A and B
X-linked recessive
178
Local anesthetics include the ester family (benzocaine, cocaine, procaine, and tetracaine) and the amide family (bupivacaine, lidocaine, mepivacaine, prilocaine). The local anesthetics in the _______ family are more allergenic because they are metabolized by the plasma pseudocholinesterases while _______ drugs are metabolized by the CYP450 system
Ester; amide
179
Symptoms of chronic infection with ________ _________ (parasitic infection) are typically isolated to the heart and GI systems. In the heart, the disease can cause arrhythmias and 3rd degree BBB. Symptoms include syncope, lightheadedness, and palpitations. It can also cause CHF and stroke. In the GI tract, it typically causes achalasia and megacolon.
Trypanosoma cruzi (Chagas disease)
180
Etiology of Rocky Mountain Spotted Fever and how this organism causes the rash
Rickettsia rickettsii R.rickettsii is an obligate intracellular organism that causes rash by invading vascular endothelial cells, resulting in increased vascular permeability, hypovolemia, and hypotension
181
_______ cells have Reinke crystalloids (hexagonal prisms by EM) and produce testosterone in response to LH. ________ cells have Charcot-Bottcher crystalloids (bundles of microfilament) and form the blood-testis barrier
Leydig Sertoli
182
________ are a class of drugs that increase insulin release from beta cells of the pancreas. Hypoglycemia is a prominent side effect due to possibility of overstimulation of insulin release
Sulfonylureas
183
Amantadine was originally developed to treat ________, but recently has been used to treat Parkinson’s disease
Influenza
184
Proteus mirabilis is a gram-negative bacterium that produces urease, which alkalinizes the urine. This can lead to what complication specific to UTI caused by this organism?
Alkalinization causes precipitation of magnesium ammonium phosphate crystals, which can lead to formation of struvite kidney stones
185
Broca’s aphasia is typically due to a lesion located where?
Dominant frontal lobe, affecting the inferior frontal gyrus
186
Ito cells are lipid-containing stellate cells in the liver which store ________ in the quiescent state and secrete _______ in the activated state
Vitamin A; collagen
187
Patients with ____________ typically have circulating antinuclear and anti-smooth muscle antibodies, jaundice, hepatosplenomegaly, and elevated transaminases
Autoimmune hepatitis
188
_______ _____ tumors lead to blockage of the aqueduct, leading to damage to CN III, CN IV, and Edinger-Westphal nucleus. As a result, pts have impaired pupillary response to light but intact accommodation. Mass effect on the superior colliculi can lead to impaired conjugate vertical gaze as well as deviation of the eyes downward. This is also known as _______ syndrome
Pineal gland; Parinaud’s
189
The abducens nerve begins at the junction of the pons and medulla. From there, it moves anteriorly through the skull, going through the ________________ with the oculomotor nerve, trochlear nerve, opthalmic and maxillary branches of the trigeminal nerves, and the internal carotid artery. It then passes through the ____________ before innervating the lateral rectus m.
Cavernous sinus; superior orbital fissure
190
By what mechanism do beta blockers reduce blood pressure?
Beta blockers inhibit release of renin from renal JG cells through antagonism of beta-1 receptors on these cells
191
Niacin can be obtained through dietary intake or synthesized endogenously from _______
Tryptophan
192
Cystic fibrosis is most commonly due to a 3-base pair deletion in the CFTR gene at amino acid position F508. How does this mutation affect CFTR?
This mutation IMPAIRS POST-TRANSLATIONAL PROCESSING of CFTR, resulting in shunting of CFTR toward the proteasome, with complete absence of the protein on the cell surface
193
Meningeal signs, confusion, and trophozoites in the CSF raise strong suspicion for primary amebic meningoencephalitis, which is caused by ______ ______, a free-living, motile protozoan parasite that lives in warm water and soil worldwide. Exposure primarily occurs when humans inadvertently inhale the organism during recreational water activities. Most individuals do not become ill, but a minority develop fatal meningoencephalitis after the organism penetrates the olfactory mucosa, crosses the cribriform plate, and invades the olfactory bulb
Naegleria fowleri
194
For the embryologic development of the kidney, the _____ _____ ultimately gives rise to the collecting system - including the collecting tubules and ducts, major and minor calyces, renal pelvis, and the ureters. The ______ ______ gives rise to the glomeruli, Bowman’s space, proximal tubules, loop of henle, and distal convoluted tubules
Ureteric bud; metanephric mesoderm (blastema)
195
Heme synthesis occurs partly in the _________ and partly in the cytoplasm of erythrocytes
Mitochondria
196
Excess ________ secretion due to a pancreatic islet cell tumor can result in watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome aka pancreatic cholera)
VIP [this is a VIPoma]
197
A positive tuberculin skin test is characterized by significant skin induration around the injection site at 48 hours. What 2 immune effector cells are most important for control of the tuberculosis?
CD4 T cells Macrophages
198
T/F: failed gallbladder visualization on radionuclide biliary scan is specific to acute cholecystitis
True — acute cholecystitis is most often caused by gallstones obstructing the cystic duct. In acute or chronic cholecystitis, the radiotracer will be taken up by the liver with progressive excretion into the common bile duct and proximal small bowel, but the gallbladder will NOT be visualized due to the obstruction
199
Viruses with segmented genomes are capable of genetic shifts through reassortment. What 4 viral classes have segmented genomes?
Bunyavirus Orthomyxovirus Arenavirus Reovirus
200
________ is a potent immunosuppressive agent that works via inhibition of calcineurin activation, resulting in decreased IL-2 production; often used in transplant pts. Serum levels should be monitored closely, and patients should be routinely checked for _____ toxicity
Cyclosporin; renal
201
AR or X-linked disorder affecting several genes that alter a multi-protein complex required for DNA repair; Typically presents in early childhood with signs of pancytopenia in addition to congenital malformations. Short stature, cafe-au-lait spots or hypopigmentation, dysplastic thumbs, microcephaly, and hypogonadism may be seen
Fanconi anemia
202
Malignant ovarian tumors with “fried egg” appearance, normal serum AFP, but beta-hCG and LDH are commonly elevated; frequently seen on streak gonads, which can occur in Turner’s syndrome
Dysgerminomas
203
_________ is a zoonotic illness that is transmitted directly to humans through contact with infected animals (like veteranarians or farmers) or via ingestion of unpasteurized milk or cheese products. Symptoms include persistent, undulating fever and joint pain
Brucellosis
204
_______ is a chemotherapeutic primarily used in NHL, breast, and ovarian cancers that carries the risk of hemorrhagic cystitis due to irritation of the bladder by its metabolite acrolein. MOA is alkylation of nitrogenous bases in nucleic acids. A few medications have been used with efficacy for preventing this damage, and each of these bind to acrolein. The most frequently used is _______, because it does not decrease the efficacy of the chemotherapeutic. Other agents used include N-acetylcysteine, amifostine, and pentosan polysulfate
Cyclophosphamide Mesna (2-mercaptoethanesulfonate sodium)
205
A pt presenting with hypertension, hematuria, proteinuria, and edema likely has a _________ syndrome. One potential cause is ____________, an immune-complex mediated disease that occurs 2-4 weeks after exposure to group A beta hemolytic streptococcus.
Nephritic PSGN
206
The most highly oxygenated blood in the fetus is carried by the ___________, which flows directly into the __________ via the ___________
Umbilical vein; IVC; ductus venosus
207
The strain phase of valsalva, abrupt standing, and nitroglycerin administration will _________ the intensity of the murmur associated with hypertrophic cardiomyopathy. Sustained hand grip, squatting, and passive leg raise will ________ the intensity of the murmur associated with hypertrophic cardiomyopathy
Increase Decrease
208
Most common cause of viral gastroenteritis
Norovirus
209
Neurophysins are carrier proteins for _______ and _________. Thus, point mutations in neurophysin II underlie most cases of hereditary hypothalamic __________ __________
Oxytocin; ADH; diabetes insipidus
210
Sitagliptin, an oral antidiabetic agent, functions by inhibiting the enzyme ______, which is responsible for the inactivation of incretins. This results in increased active incretin levels, leading to increased secretion of insulin and decreased secretion of glucagon
Dipeptidyl peptidase IV (DDP-IV)
211
______ is an inhaled anesthetic associated with mild hepatotoxicity and more fulminant hepatitis
Halothane
212
Cyanide is an inhibitor of what enzyme affecting oxidative phosphorylation?
Cyanide is an inhibitor of cytochrome c oxidase (part of 4th complex of ETC) It increases venous O2 saturation by preventing the body from using O2
213
A deficiency in vitamin C would most affect the synthesis of which of the following neurotransmitters? ``` A. ACh B. Dopamine C. Glutamate D. Histamine E. Norepinephrine ```
E. Norepinephrine [Vitamin C is required for the conversion of dopamine to NE by the enzyme dopamine-beta-hydroxylase]
214
Autosomal recessive disorder that affects the metabolism of methionine; manifests clinically with mental retardation, osteoporosis, tall stature, kyphosis, and atherosclerosis leading to stroke and MI at a young age
Homocysteinuria
215
Diffuse esophageal spasm can cause retrosternal chest pain that is typically precipitated by cold or hot food; the pain can be intermittent and is not situation-dependent. It can show ______ pattern on barium swallow (but barium swallow can also be negative). This can ultimately lead to functional obstruction due to increased esophageal wall stress. What is first line treatment for diffuse esophageal spasm?
Corkscrew First line is CCBs like nifedipine, which act on L-type calcium channels of both cardiac and smooth muscle
216
Transthyretin is a protein responsible for transporting vitamin A and thyroxine. Transthyretin ______ is an autosomal dominant condition, and an important subtype affects the heart, causing a restrictive cardiomyopathy
Amyloidosis
217
Adenocarcinoma of the lung (NSCLC) is associated with mutation on the _____gene
K-ras
218
Metachromatic leukodystrophy is due to a deficiency of __________, leading to accumulation of __________, which causes demyelination. Patients present with cognitive impairment, ataxia, and optic atrophy
Arylsulfatase A; cerebroside sulfate
219
In essential fructosuria, _______ is able to phosphorylate fructose to fructose-6-phosphate, shunting it to glycolysis
Hexokinase
220
Long-term uncontrolled diabetes can lead to diabetic hyporeninism, which causes RTA type ____, which presents with hyperkalemia.
RTA type IV [Other causes of RTA type IV include ACE inhibitors, ARBs, NSAIDs, and adrenal insufficiency. RTA types I and II present with hypokalemia. Causes of RTA type I include analgesic nephropathy and amphotericin toxicity in severe fungal infections. Causes of RTA type II include Fanconi syndrome and Acetazolamide]
221
An intention tremor in the right arm indicates a lesion in which of the following? ``` A. Cerebellar vermis B. Left cerebellar hemisphere C. Left subthalamic nucleus D. Right cerebellar hemisphere E. Right subthalamic nucleus ```
D. Right cerebellar hemisphere [Lesions in cerebellar hemispheres can cause ataxia and intention tremors in ipsilateral limbs. Pts with lesions in the subthalamic nuclei present with contralateral hemiballismus]
222
WAGR syndrome is due to a chromosomal deletion of the WT1 gene. What are the clinical features?
Wilms tumor Aniridia Genitourinary abnormalities Mental retardation
223
Most effective class of medications to lower triglycerides
Fibrates
224
______ ______ = description of anomalies including pulmonary hypoplasia, clubbed feet, and cranial abnormalities due to oligohydramnios
Potter sequence [classically due to bilateral renal agenesis; other causes include polycystic kidney disease, hydronephrosis, and any other cause of ureter obstruction]
225
Most common cardiac defect seen in DiGeorge syndrome patients
Tetralogy of Fallot
226
Disease characterized by rheumatoid nodules surrounded by pigmented cells in the lungs. It is specifically seen in rheumatoid arthritis patients who also have one of the pneumoconiosis
Caplan’s syndrome
227
Beck’s triad of cardiac tamponade
1. Jugular venous distention 2. Hypotension (with pulsus paradoxus) 3. Diminished heart sounds
228
Ehlers-Danlos syndrome is a group of CT disorders involving a defect in _____ synthesis
Collagen
229
Small intestinal bacterial overgrowth (SIBO) is characterized by overproduction of _______ and _______, associated with nausea, bloating, abdominal discomfort, and malabsorption
Vitamin K; Folate
230
Light microscopy revealing several small, ovoid bodies within a macrophage raise strong suspicion for ______ ________, a dimorphic fungus transmitted by inhalation. Patients with impaired immunity (e.g., advanced AIDS) are at risk for dissemination through the ___________ system; common manifestations include hepatosplenomegaly, lymphadenopathy, pancytopenia, and ulcerated lesions on the tongue
Histoplasma capsulatum; reticuloendothelial
231
Microtubule-associated, ATP-powered motor protein that facilitates the anterograde transport of neurotransmitter-containing secretory vesicles down axons to synaptic terminals
Kinesin
232
Which one causes transcriptional repression: acetylation of histones or deacetylation of histones?
Deacetylation of histones [This is the mechanism behind Huntington disease — abnormal huntingtin gene causes increased histone deacetylation, silencing the genes necessary for neuron survival]
233
Pulmonary artery catheters are used to diagnose pulmonary HTN and occasionally for management of critically ill pts. The pressure measured at the catheter tip is called the pulmonary artery occlusion pressure (aka pulmonary capillary wedge pressure), and closely reflects the end-diastolic pressure in what heart chamber(s)?
Left atrium Left ventricle
234
What triad of changes are seen on ECG with Wolff-Parkinson-White syndrome?
Shortened PR interval (<0.12s) Early upslope of QRS (delta wave) Widened QRS complex
235
Treatment of choice for CMV
IV Ganciclovir [Works by competitively inhibiting incorporation of dGTP by viral DNA polymerase. Other options to tx CMV include oral valganciclovir, IV foscarnet, or IV cidofovir]
236
2 most common causes of pulsus paradoxus without pericardial effusion
Asthma | COPD
237
Farmer’s lung is a hypersensitivity pneumonitis caused by inhalation of thermophylic Actinomyces found in the dust from moldy hay. Initial treatment involves giving _______ to decrease the inflammatory response. Afterwards, avoiding exposure to offending agent and wearing a mask when exposure is possible
Corticosteroids
238
_______ _______ are calcifications found in several benign or malignant tumors including papillary thyroid carcinoma, serous cystadenocarcinoma of the ovary, meningioma, and mesothelioma
Psammoma bodies
239
Nerve roots associated with long thoracic n.
C5-7
240
What is the most common enzyme deficiency associated with congenital adrenal hyperplasia, manifesting with hyponatremia, hyperkalemia, hypotension, and ambiguous external genitalia?
21-beta-hydroxylase deficiency
241
What ligament prevents posterior translation of the tibia relative to the femur, and when ruptured the tibia rests in a more posterior position than usual?
Posterior cruciate ligament
242
Which of the following is the treatment for carcinoid syndrome? ``` A. Hydroxyurea B. Leuprolide C. Octreotide D. Phentolamine E. Somatropin ```
C. Octreotide
243
________ herniations result in ptosis, mydriasis, lateral strabismus, and contralateral hemiparesis of the extremities
Uncal
244
Brown-Sequard syndrome results from a hemisection of the spinal cord. It is characterized by _________ spastic paralysis below the lesion, ________ loss of touch and vibration sensations below the lesion, and _________ loss of pain and temperature sensation below the lesion. [ipsilateral/contralateral/bilateral]
Ipsilateral; ipsilateral; contralateral
245
Henoch-Schonlein purpura, a _____ (small/medium/large) vasculitis that occurs in children. It is a type _____ hypersensitivity characterized by ___________ on vessel walls
Small; III; IgA immune complexes
246
CLL is associated with _______ about 10% of the time, which is an anemia characterized by an elevated reticulocyte count, a reduced haptoglobin level, and a positive Coombs test, as well as the presence of ______ on peripheral smear
Autoimmune hemolytic anemia (AIHA); spherocytes [Note: the differential diagnosis for spherocytosis includes hereditary spherocytosis and autoimmune hemolytic anemia]
247
New-onset seizures in adults justify MRI to exclude the possibility of an intracranial neoplasm. __________ are most commonly located in the frontal lobes, and examination will reveal the presence of rounded cells with perinuclear halos giving them “fried egg” appearance. They also have the presence of finely branching capillaries, which looks like “chicken wire”
Oligodendrogliomas
248
A lesion in which hypothalamic nucleus would lead to difficulty lowering body temperature?
Anterior [lesion in dorsomedial nucleus leads to decreased aggression and feeding, lesion in lateral nucleus leads to decreased feeding, lesion in supraoptic nucleus leads to DI, lesion in ventromedial nucleus leads to increased feeding
249
Pronator syndrome occurs when the _____ nerve is compressed
Median
250
A biopsy of a breast lesion shows large, atypical adenocarcinoma cells with pale to clear cytoplasm that infiltrate below the epidermis; when in the context of scaly, raw, and sometimes ulcerated lesions of the nipple associated with pain, burning, and pruritis, this biopsy is indicative of what condition?
Paget disease
251
What is the etiology of hypercortisolism if a low-dose dexamethasone suppression test fails to suppress cortisol levels, but a high-dose dexamethasone suppression test decreases cortisol levels?
Cushing disease (pituitary ACTH-secreting adenoma)
252
Marfan syndrome is an autosomal dominant disease caused by point mutations in the FBN1 gene on chromosome _____
15
253
How does asbestosis show up on lung biopsy?
Interstitial fibrosis with occasional ferruginous bodies (arise from iron and protein coating with inhaled fibers)
254
Which of the following is associated with AE of galactorrhea? ``` A. Fluoxetine B. Haloperidol C. Lithium D. Nortriptyline E. Phenelzine ```
B. Haloperidol [This drug is a dopamine antagonist]
255
Autosomal dominant genetic condition characterized by the constellation of mucosal neuromas, marfanoid body habitus, medullary thyroid carcinoma, and pheochromocytoma
MEN2B
256
3 findings in MEN1
Pituitary adenoma Parathyroid hyperplasia Pancreatic tumors (e.g., gastrinoma, insulinoma, vipoma, glucagonoma)
257
3 findings in MEN2A
Parathyroid hyperplasia Medullary thyroid carcinoma Pheochromocytoma
258
Most common non-epithelial benign neoplasm of the GI tract; symptoms include early satiety and abdominal bloating. When these tumors contain KIT mutations, they almost always display spindle-shaped cells on biopsy
Gastrointestinal stromal tumor (GIST)
259
Gene mutation associated with Peutz-Jegher’s syndrome
STK11
260
Which of the following presents as painful vaginal bleeding in the 2nd or 3rd trimester and is associated with increased risk due to HTN, smoking, and use of cocaine? ``` A. Ectopic pregnancy B. Incomplete abortion C. Placental abruption D. Placenta accreta E. Placenta previa ```
C. Placental abruption [note that placenta previa presents with PAINLESS vaginal bleeding during 2nd or 3rd trimester with risk factors of previous C section and multiparity]
261
The levator scapulae muscle is innervated by a nerve supply from the roots of ______
C3-5
262
In persons with schizophrenia resistant to multiple trials of both 2nd and 1st generation antipsychotics, the atypical antipsychotic ________ has been found to be an effective treatment. The most serious and potentially life threatening side effect of this medication is ________
Clozapine; agranulocytosis [Other AEs of clozapine include weight gain, diabetes, hyperlipidemia, seizure, myocarditis, salivation, and sedation]
263
How do aldosterone levels change in pregnancy?
Aldosterone levels increase due to increased activation of RAAS
264
Polycythemia vera is associated with _____ serum erythropoietin concentrations
Low — due to negative feedback effect
265
Artery involved in stroke leading to ipsilateral face analgesia, contralateral body analgesia, dysarthria, dysphagia, and ataxia
Posterior inferior cerebellar artery [note that stroke affecting anterior inferior cerebellar artery also presents with ipsilateral face analgesia and contralateral body analgesia, but instead of dysarthria/dysphagia/ataxia, the additional findings are ipsilateral face hemiparesis and ipsilateral deafness]
266
Artery involved in stroke leading to contralateral hemianesthesia, contralateral hemiplegia, contralateral homonymous hemianopia, ipsilateral horizontal gaze preference
Middle cerebral artery
267
Innervation of the short head and long head of biceps femoris
Short head = common fibular branch of the sciatic n. (L5-S2) Long head = tibial branch of the sciatic n. (L5-S3)
268
Lateral medullary syndrome typically includes ipsilateral ______ syndrome
Horner’s
269
Condition characterized by abnormal proliferation of mast cells (often associated with mutations in the KIT receptor tyrosine kinase) and increased histamine release. Histamine causes hypersecretion of gastric acid by parietal cells in the stomach as well as a variety of other symptoms (e.g., hypotension, flushing, pruritis)
Systemic mastocytosis
270
The brachial artery, median nerve, and radial nerve all run anterior to the elbow and may be injured in supracondylar humeral fractures. Of these, the ___________ is the structure most likely to be injured with lateral displacement of the proximal fracture fragment
Radial n
271
What 2 classes of medications should be avoided in pts with hypertrophic cardiomyopathy?
Vasodilators (eg, dihydropyridine CCBs, nitroglycerin, ACE-Is) Diuretics [Pts with HCM have dynamic left ventricular outflow tract obstruction that worsens with decreased LV volume — so any meds that decrease preload and/or reduce systemic vascular resistance should be avoided]
272
Obligate intracellular protozoa that mature in macrophages and can be identified on biopsy by the presence of rod-shaped kinetoplasts
Leishmania spp. [Transmission via infected sand flies —> syndrome of cutaneous leishmaniasis, characterized by chronic pinkish papule that evolves into nodule or plaque]
273
Tay-Sachs and Niemann-Pick disease are very similar in that they both present with neurologic regression and cherry-red macular spots. The differentiating factor is the presence or absence of hepatosplenomegaly. Of the two, which one is associated with hepatosplenomegaly as an additional finding?
Niemann Pick
274
CKD can cause hyperphosphatemia due to decreased renal excretion of phosphorus. Dietary phosphorus restriction is recommended, but oral phosphate binders are often needed. _______ is a nonabsorbable anion-exchange resin that binds intestinal phosphate to reduce absorption
Sevelamer
275
T lymphocytes are important for the prevention of superficial candida infection, while ________ are the immune cells responsible for preventing its hematogenous spread
Neutrophils
276
Which of the following causes the form of SCID in which flow cytometry demonstrates reduced levels of B-, T-, and NK-lymphocytes? A. Defect in DNA double-strand break repair enzymes B. Lack of NADPH oxidase C. Adenosine deaminase deficiency D. Defective CD40L signaling E. Defective neutrophil phagosome-lysosome fusion
C. Adenosine deaminase deficiency
277
Thumb adduction is a function of the ______ nerve, and is the only thumb movement not controlled by the ______ nerve.
Ulnar; median
278
Genetic testing techniques utilized in chromosome analysis include ___________ which detects presence/absence of specific DNA sequences in a specific chromosome location, and _________ which detects gross abnormalities in chromosomes
FISH; karyotyping
279
ABCDE of Sarcoidosis
ABCDE of sarcoid = elevated ACE, bilateral hilar LAD, elevated Calcium, elevated vitamin D, Erythema nodosum
280
Trazodone is an atypical antidepressant that also causes sedation and aids in weight loss. It is a weak inhibitor of the 5-HT2A receptor, as well as inhibitor of H1-receptor. It is commonly associated with what adverse effect?
Priapism
281
Treatment for Actinomyces israelii
Penicillin [Since it is a gram-positive rod, A.israelii is very sensitive to beta-lactam drugs, such as penicillin, which inhibit peptidoglycan synthesis]
282
What are the “ABC’s of anaerobes” to remember?
Actinomyces Bacteroides Clostridium
283
Which of the following is associated with increased uric acid levels and potential to precipitate a gout attack? ``` A. Lisinopril B. Losartan C. Metformin D. Niacin E. Sulfonylurea ```
D. Niacin
284
Which of the following is affected in an ovarian torsion? ``` A. Broad ligament B. Cardinal ligament C. Infundibulopelvic ligament D. Ovarian ligament E. Round ligament ```
C. Infundibulopelvic ligament [this is analagous to suspensory ligament of the ovary]
285
__________ is the enzyme that converts testosterone to dihydrotestosterone. Deficiency is autosomal recessive and limited to genotypical males. When the enzyme is deficient, males have ambiguous genitalia until puberty because the development of male genitalia is dependent on high levels of androgens
5-alpha-reductase
286
Alport syndrome presents with renal failure and hearing loss. It is caused by a defect in _____________, the type of collagen found in basement membranes
Type IV collagen
287
___________ is an X-linked congenital immunodeficiency characterized by recurrent pyogenic infections and eczema due to IgM deficiency and IgA excess. It is associated with a mutation of the ______ gene on the X chromosome
Wiskott-Aldrich syndrome; WASp
288
Drug of choice in treating acute Chagas disease
Nifurtimox
289
Which of the following is an H2 inhibitor utilized to decrease gastric acidity? ``` A. Loratadine B. Misoprostol C. Odansetron D. Rabeprazole E. Ranitidine ```
E. Ranitidine [H2 blockers include cimetidine, ranitidine, nizatidine, and famotidine. Note that loratadine is an H1 blocker utilized for allergic rhinitis]
290
Which of the following is most likely associated with a 21-hydroxylase deficiency? ``` A. Elevated angiotensin II B. Elevated BP C. Elevated glucocorticoids D. Hyperplasia of adrenal medulla E. Hypersecretion from the zona fasciculata ```
A. Elevated angiotensin II [21-hydroxylase deficiency leads to the inability of the adrenal cortex to produce glucocorticoids and mineralocorticoids. This is the most common form of CAH. This leads to hypotension (salt-wasting) and thus activation of the RAAS]
291
The second-generation tetracyclines (doxycycline) and macrolides are the drugs of choice for Mycoplasma pneumoniae causing community-acquired pneumonia. Note that tetracyclines are teratogenic and thus contraindicated in pregnancy, in which case macrolide antibiotics such as Azithromycin would be preferred. What is the MOA of macrolides?
Binds 50S ribosomal subunit and blocks dissociation of peptidyl tRNA from ribosomes, ultimately arresting RNA-dependent protein synthesis
292
What class of antibiotics can be used for outpatient tx of uncomplicated UTI, and are associated with the AE of QT prolongation and Torsade de pointes?
Fluoroquinolones
293
_________ is a class IB antiarrhythmic that blocks sodium channels and preferentially slows depolarization in ischemic tissue. The most common adverse effects involve the CNS - including drowsiness, numbness, speech disturbance, and dizziness.
Lidocaine
294
______ is a class III (blocks potassium channels) antiarrhythmic medication that is unique in that it also has powerful class I activity (blocks sodium channels), and ancillary class II (blocks beta-receptors) and class IV (blocks calcium channels). It is utilized for ventricular arrhythmias and works by prolonging the cardiac action potential
Amiodarone
295
Which of the following is utilized as a part of HAART in HIV therapy and inhibits reverse transcriptase without incorporation into viral DNA? ``` A. Acyclovir B. Efavirenz C. Nelfinavir D. Saquinavir E. Zidovudine ```
B. Efavirenz [Other NNRTIs include delavirdine, nevirapine, and rilpivirine]
296
The ______ is the primary rotator cuff muscle involved in external rotation of the upper extremity, assisted by another rotator cuff muscle _________, which is innervated by the _______ nerve
Infraspinatus; teres minor; axillary
297
Which of the following is most likely to cause tardive dyskinesia? ``` A. Clozapine B. Fluphenazine C. Lithium D. Risperidone E. Selegiline ```
B. Fluphenazine [Long-term treatment with typical antipsychotics, such as haloperidol, trifluoperazine, fluphenazine, thioridazine, and chlorpromazine, can cause tardive dyskinesia]
298
The mechanism of action of cyclosporine is that it binds to cyclophilins inhibiting ________ and thereby preventing the production of IL-2 and its receptors. This drug is used to suppress organ rejection after transplant surgeries. It can also cause nephrotoxicity, hirsutism, and gingival hyperplasia
Calcineurin
299
Donepezil is utilized in the tx of Alzheimers disease. What is its MOA?
Acetylcholinesterase inhibitor
300
Persistent inflammatory muscle disease that causes weakness of the skeletal muscles via cytotoxic T-cell mediated process directed against muscle fascicles. Presents with onset of proximal muscle weakness and possible complaints of difficulty with kneeling, climbing or descending stairs, raising arms, lifting objects, combing hair, and arising from seated position
Polymyositis [Note that lab studies reveal elevated serum creatine kinase, lactate dehydrogenase, and aldolase, as well as anti-Jo-1 antibodies in serum]
301
Which of the following would most likely be positive in a pt with atypical pneumonia due to Mycoplasma pneumoniae? ``` A. Catalase test B. Coagulase test C. Cold agglutinin titer D. Maltose test E. Warm agglutinin titer ```
C. Cold agglutinin titer
302
Infection with the nematode Loa loa may be asymptomatic, or may present with local hypersensitivity reaction causing episodic subcutaneous angioedema of the extremities with local pain and pruritis. Adult filarial worms may migrate into subconjunctival tissues of the eye where they can be easily observed. Loa loa infection is treated with ________, which is an antihelminthic agent that acts by inhibiting arachidonic acid metabolism in filarial microfilaria, which disrupts their membranes and makes them more susceptible to immune attack
Diethylcarbamazine
303
Primary hyperaldosteronism (Conn syndrome) may show increased _____ levels which will help maintain normal serum sodium levels chronically, i.e., the “sodium escape” phenomenon, even in the face of increased aldosterone levels. Other features may include hypokalemia, lack of edema, hypomagnesemia, and metabolic alkalosis
ANP
304
________, the most common cause of gastritis with watery diarrhea in infants, has a unique genome consisting of dsRNA
Rotavirus
305
Sympathetic innervation of the testicles
T10-11
306
One treatment option for BPH is tamsulosin, which is an _______________ that will inhibit smooth muscle contraction of the prostatic urethra, making urination more normal and improving pt quality of life. AEs include hypotension and dizziness
Alpha-1 antagonist
307
_________ is a non-enveloped linear dsDNA virus that is stable in chlorinated water, and therefore can infect patients in swimming pools via contaminated respiratory secretions in the water
Adenovirus
308
Which of the following is an adverse effect of amiodarone? ``` A. Bronchoconstriction B. Lower extremity edema C. Pulmonary fibrosis D. Reflex tachycardia E. Torsades de pointes ```
C. Pulmonary fibrosis [Others include thyroid dysfunction, hepatotoxicity, ocular changes, and skin reactions]
309
Autosomal dominant trinucleotide expansion disease exhibiting repeats of CTG within DMPK gene, leading to myotonia (noted with sustained grasp), muscle wasting, frontal balding, cataracts, testicular atrophy, and arrhythmias.
Myotonic muscular dystrophy
310
What is the advantage of using amoxicillin over ampicillin?
They are similar, but amoxicillin has better bioavailability from the GI tract due to greater stability in gastric acid — thus it has greater oral absorption
311
A major side effect of phenytoin is a ________ anemia due to _______ deficiency, specifically due to inhibition of intestinal conjugase needed to convert this nutrient into the absorbable form
Megaloblastic; folate
312
A patient with megaloblastic anemia due to folate deficiency caused by phenytoin therapy is at increased risk for which of the following: ``` A. Achlorhydria B. Demyelination of the spinal cord C. Direct myopathy D. Nystagmus E. Vessel thrombosis ```
E. Vessel thrombosis [pts with folate deficiency present with an elevated level of homocysteine — which leads to a state of hypercoagulability as well as destruction of arteries]
313
Autosomal dominant condition characterized by the triad of eczema, eosinophilia, and recurrent skin and pulmonary infection; also associated with multiple connective tissue and skeletal abnormalities, including scoliosis, hyperextensibility, pathologic fractures, retained primary dentition, craniosynostosis, and vascular abnormalities
Hyperimmunoglobulinemia E (aka Job syndrome)
314
Antibodies associated with Hashimoto thyroiditis
Anti-microsomal
315
Of the rotator cuff muscles, the _____ and _____ externally rotate the arm, while the _______ acts to internally rotate the arm
Infraspinatus; teres minor; subscapularis
316
The ulnar nerve arises from the ____ cord of the brachial plexus. The medial and lateral cords of the brachial plexus combine to give rise to the ______ nerve. The posterior cord of the brachial plexus give rise to the _____ and _____ nerves
Medial Median Axillary; radial
317
Genetics associated with Wilms tumor
Deletion of WT1 gene on chromosome 11
318
What water-soluble vitamin deficiency can occur secondary to poor diet, excessive raw egg white consumption, and congenital disorders of metabolism?
Biotin (vitamin B7) [Note that biotin is an important cofactor for several carboxylase enzymes. In the liver, the conversion of pyruvate to oxaloacetate for gluconeogenesis requires pyruvate carboxylase and biotin]
319
The ______ of the cell is home to enzymes necessary for glycolysis, fatty acid synthesis, and the pentose phosphate pathway
Cytosol [Contrast with beta-oxidation of fatty acids, TCA cycle, and gluconeogenesis which occur in the mitochondria]
320
Chronic lung transplant rejection is due primarily to progressive scarring of the small airways, leading to _______ _______. Manifestations occur months or years after transplantation and include obstructive lung disease (e.g., reduced FEV1) with dyspnea and dry cough
Bronchiolitis obliterans
321
The ureters originate bilaterally at the renal pelvis and course inferiorly toward the bladder within the retroperitoneum just anterior to the psoas muscles. Midway from the kidney to the pelvic inlet, the ureters cross _______ to the gonadal artery and vein. The ureters then gain access to the pelvis by crossing _______ to the external iliac artery at, or just after, the bifurcation of the common iliac artery. At this point, the ureter lies ______ to the ovarian vessels, and ______ to the internal iliac artery.
Posterior; anterior Medial; anterior
322
Key findings include fetal growth retardation, hypertonia (clenched hands with overlapping fingers), rocker bottom feet, and cardiac/GI/renal defects; most commonly the result of meiotic nondisjunction due to advanced maternal age
Trisomy 18 (Edwards syndrome) [Note that trisomy 13 (patau syndrome) presents with midline facial defects (e.g., holoprosencephaly, cleft lip/palate), MSK abnormalities (polydactyly), and GI anomalies (omphalocele, umbilical hernia); it is not associated with overlapping fingers]
323
With Plasmodium (malaria) infection, erythrocytic forms of the parasite may be treated with antimalarials such as ________ (in non-resistant areas), _________, and _________. _______ is required to kill P vivax and P ovale liver hypnozoites
Chloroquine; atovaquone-proguanil; artemisinins Primaquine
324
Deficiency of what vitamin is associated with complications from Measles virus (rubeola)?
Vitamin A [acute measles depletes vitamin A stores, resulting in risk of keratitis and corneal ulceration]
325
Which of the following embryological events is most likely responsible for tetralogy of fallot? a. Anomalous pulmonary venous return b. Aortic arch constriction c. Deviation of the infundibular septum d. Endocardial cushion defect e. Linear development of the aorticopulmonary septum
c. Deviation of the infundibular septum [Tetralogy of Fallot results from anterior and cephalad deviation of the infundibular septum during embryologic development, resulting in a malaligned VSD with an overriding aorta]
326
The Kozak consensus sequence occurs on eukaryotic mRNA and is defined by the following sequence: (gcc)gccRccAUGG, in which R is either ______ or ______. This sequence helps initiate translation at the methionine start codon (AUG), thus a substitution for one of the above nucleotides would impair initiation of translation
adenine; guanine
327
Enzyme found in adipose tissue, where it functions to drive the breakdown of stored triglycerides into FFAs and glycerol. During times of starvation, this enzyme provides substrates for hepatic gluconeogenesis and ketone body formation
Hormone-sensitive lipase
328
_________ is a cofactor used by hydroxyls enzymes in the synthesis of tyrosine, dopamine, and serotonin. PKU can result from a deficiency in this cofactor, and intellectual disability is the hallmark.
Tetrahydrobiopterin (BH4)
329
Common fibular nerve injuries may result in what motor and sensory defects?
Weakness of dorsiflexion (deep fibular n) Weakness of eversion (superficial fibular n) Loss of sensation over the dorsum of the foot
330
Autosomal recessive inherited defect in lipoprotein lipase (or ApoC-II) resulting in acute pancreatitis, lipemia retinalis (milky-appearing retinal vasculature), eruptive skin xanthomas, and HSM
Familial Chylomicronemia Syndrome (Type I hyperlipoproteinemia)
331
How do the pupils react to light in the right vs. left eye when the left optic nerve is damaged?
When light is shined in the left eye (the eye with the damaged optic nerve), neither pupil will constrict because the nerve can’t sense the light. However, light in the right eye will cause both pupils to constrict because the motor pathways are intact
332
Neurologic damage associated with vitamin B12 deficiency classically includes subacute combined degeneration of what 3 spinal cord tracts?
Dorsal columns (loss of position and vibration sense, sensory ataxia) Lateral corticospinal tracts (spastic paresis) Spinocerebellar tracts (contributes to ataxia)
333
HIV attaches to host cells using the viral surface protein ______, which binds the CD4 molecule as the primary receptor and the chemokine receptor _______ as a coreceptor. Binding of the primary receptor and coreceptor induces a conformational change in the viral surface protein that exposes underlying transmembrane protein _____, which mediates viral fusion to the host cell and release of viral capsid into the cytoplasm
gp120; CCR5 gp41
334
Congenital condition that is usually asymptomatic at birth but later infants present with constipation, lethargy, hypotonia, macroglossia, an umbilical hernia, and large anterior fontanelle
Congenital hypothyroidism (cretinism)
335
T/F: nucleotide excision repair involves removal of deaminated bases by glycosylase
False — Nucleotide excision repair involves endonuclease nicking of the damaged DNA strand and typically occurs in the case of sunburn. Base excision repair involves removal of deaminated bases by glycosylase — this process may occur spontaneously or secondary to chemical exposure
336
mRNA splicing is performed by spliceosomes, which remove introns containing _____ at the 5’ splice site, and _____ at the 3’ splice site. Splice site mutations may result in inappropriate removal of exons and retention of introns, leading to the formation of dysfunctional proteins
GU; AG
337
Most common type of placentation in dizygotic vs. monozygotic twins
Dizygotic = dichorionic/diamnionic Monozygotic = monochorionic/diamnionic
338
5 catalase positive organisms
Staphylococcus aureus Burkholderia cepacia Serratia marcescens Nocardia Aspergillus
339
Beta-endorphin is one endogenous opioid peptide that is derived from proopiomelanocortin (POMC). POMC is a polypeptide precursor that goes through enzymatic cleavage and modification to produce not only beta-endorphins, but also _____ and ______.
ACTH; MSH
340
_______ is a peptide that is structurally similar to insulin. Also referred to as insulin-like growth factor, this peptide is released in response to growth hormone and stimulates growth in target cells
Somatomedin C
341
What syndrome typically occurs 2-8 weeks after exposure to high-risk drugs such as anticonvulsants (e.g., phenytoin, carbamazepine), allopurinol, sulfonamides (e.g., sulfasalazine), and antibiotics (e.g., minocycline, vancomycin). Patients typically develop fever, generalized LAD, facial edema, diffuse skin rash, eosinophilia, and internal organ dysfunction
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome
342
The most classic clinical triad of congenital ______ includes congenital cataracts (white pupils), sensory-neural deafness, and a patent ductus arteriosus
Rubella [prevented with live attenuated rubella virus vaccine pre-conception]
343
The bilateral _______ veins combine to form the SVC, and obstruction causes symptoms similar to those seen in SVC syndrome, but only on one side of the body
Brachiocephalic
344
_______ converts androgens into estrogens in the ovaries, testes, placenta, and other peripheral tissues. Placental deficiency of this enzyme causes accumulation of androgens during pregnancy, resulting in ambiguous external genitalia in female infants and maternal virilization
Aromatase
345
The __________ arteries supply the medial portions of the 2 hemispheres (frontal and parietal lobes). Occlusion can cause contralateral motor and sensory deficits of the lower extremities, behavioral changes, and urinary incontinence
Anterior cerebral aa.
346
Which of the following has the lowest ID50 (requires the lowest number of bacteria to cause infection)? ``` A. Clostridium perfringens B. Enterotoxigenic E.coli C. Shigella flexneri D. Vibrio cholerae E. Vibrio parahaemolyticus ```
C. Shigella flexneri
347
Activating mutations of the _____ gene lead to constitutive activation of the EGFR pathway, promoting increased cell proliferation and growth. Tumors harboring these mutations are resistant to treatment with anti-EGFR drugs (e.g., cetuximab, panitumumab)
KRAS
348
IV drug use is a major risk factor for hepatitis B and C viral infection. Acute viral hepatitis is marked by panlobular inflammation and hepatocyte necrosis and ballooning. ________-mediated signals also cause hepatocyte apoptosis with the formation of intensely eosinophilic _______ bodies
CTL; Councilman
349
In prokaryotes, ___________ has 5’ to 3’ exonuclease activity in addition to 5’ to 3’ polymerase and 3’ to 5’ exonuclease activities. This 5’ to 3’ exonuclease activity functions to remove the RNA primer created by RNA primase and repair damaged DNA sequences
DNA polymerase I
350
G6PD deficiency is an X-linked recessive disorder resulting in episodic bouts of hemolysis when RBCs experience increased oxidative stress. G6PD is the rate-limiting step of _________________. It catalyzes the conversion of glucose-6-phosphate to 6-phosphogluconolactone, which is subsequently converted to _____________. The purpose of this pathway is to generate NADPH and ribose-5-phosphate. NADPH can be used to maintain a stead supply of glutathione.
Pentose phosphate pathway; 6-phosphogluconate
351
Bullous impetigo is a superficial infection by Staphylococcus aureus that is most common in young children. It is characterized by a blistering skin rash with tan- to honey-colored crusts. The blistering in bullous impetigo is caused by __________, which targets _________ in epidermal cellular junctions and causes a loss of cell adhesion
Exfoliative toxin A; desmoglein 1 [Pemphigus vulgaris involves autoantibodies against desmosomes (desmogleins 1 and 3). Bullous pemphigoid involves autoantibodies against hemidesmosomes]
352
_______ pneumonia is common in pts with advanced HIV and usually presents with slowly worsening cough, dyspnea, hypoxia, bilateral interstitial infiltrates, fever, and weight loss. Diagnosis requires visualizing the organism in respiratory secretions using special stains (e.g., methanamine silver stain). First-line treatment is __________
Pneumocystis; TMP-SMX
353
What 2 stool tests are utilized in the diagnoses of Clostridium difficile infection? Which one is most sensitive and specific?
Nucleic acid amplification test (NAAT) — uses PCR to detect genes present in toxigenic strains (e.g., toxin B-encoding gene). This test is highly sensitive and specific for toxigenic strains but does not distinguish active toxin production and may lead to overdiagnosis in asymptomatic carriers Enzyme immunoassays — use Abs to detect C diff antigens or toxins. Highly specific but poor sensitivity.
354
What class of HIV antiretrovirals carry the side effect profile including hyperglycemia, lipodystrophy, and drug-drug interactions due to inhibition of CYP450?
Protease inhibitors
355
Tumor cell lines can become resistant to therapy after exposure to various anticancer agents. These cells express a P-glycoprotein encoded for by the ____________ gene. This P-glycoprotein is a transmembrane ATP-dependent efflux pump protein that has a broad specificity for hydrophobic compounds. It can both reduce the influx of drugs into the cytosol and can increase efflux from the cytosol, thereby preventing the action of chemotherapeutic agents
MDR1 (human multidrug resistance gene)
356
Familial dysbetalipoproteinemia (type III hyperlipoproteinemia) is an autosomal recessive disorder characterized by elevated cholesterol and triglyceride levels. It is caused by defects in ____ and _____, leading to decreased clearance of chylomicrons and VLDL remnants. Patients can develop eruptive and palmar xanthomas and premature atherosclerosis
ApoE3; ApoE4
357
Blanching of a vein into which norepinephrine is being infused together with induration and pallor of the tissues surrounding the IV site are signs of NE extravasation and resulting vasoconstriction. Tissue necrosis is best prevented by local injection of a _______-blocking drug, such as _______
Alpha 1; phentolamine
358
Nontypeable strains of Haemophilus influenzae are part of the normal upper respiratory tract flora and are a common cause of acute otitis media, sinusitis, and bronchitis. Because nontypeable strains do not form a _______________, immunity is not conferred by vaccination with the H influenzae type b vaccine
Polysaccharide capsule
359
MOA of DPP-4 inhibitors (e.g., sitagliptin, saxagliptin) utilized in diabetes
Inhibit the degradation of GLP-1, prolonging its effects and leading to improved glycemic control [GLP-1 acts to slow gastric emptying, suppress glucagon secretion, and increase glucose-dependent insulin release] Major side effects include nasopharyngitis and other upper respiratory symptoms
360
The candidal antigen skin test is used to determine the presence of cellular immunity through the detection of a delayed-type hypersensitivity reaction (type IV). What are the 3 key cells involved in the response with a positive test?
Macrophages CD4+ T cells CD8+ T cells
361
The _______ _______ is a paired brainstem nucleus located in the posterior rostral pons near the lateral floor of the 4th ventricle. It projects to virtually all parts of the CNS and helps control mood, arousal (reticular activating system), sleep-wake states, cognition, and autonomic function. It functions as the principal site for _______ synthesis in the brain
Locus ceruleus; norepinephrine
362
Hereditary pulmonary arterial hypertension is most often due to autosomal dominant inactivating mutation in BMPR2. How does this lead to the condition of Hereditary PAH?
Patients with this mutation have a predisposition for dysfunctional endothelial and smooth muscle cell proliferation An insult (e.g., infection, drugs) is thought to then activate the disease process. The end result is vasoconstriction with vascular smooth muscle proliferation, intimal thickening and fibrosis, increased pulmonary vascular resistance, and progressive pulmonary HTN.
363
Lactose intolerance presents with flatulence, crampy abdominal pain, and watery diarrhea after dairy product consumption. Studies can show _______ breath hydrogen content, _______ stool pH, and _______ stool osmolality
Increased; decreased; increased
364
What structures are associated with the first and second pharyngeal arches? What syndrome is associated with abnormal development of these 2 arches?
First pharyngeal arch = CN V, maxilla, zygoma, mandible, incus, malleus, masseter m., temporalis m. Second pharyngeal arch = CN VII, styloid process of temporal bone, lesser horn of hyoid, stapes, muscles of facial expression Abnormal development of the first and second arches —> Treacher-Collins syndrome, characterized by craniofacial abnormalities and conductive hearing loss
365
Which of the following is characteristic of NSAID-induced kidney injury? A. Impaired afferent arteriolar vasodilation B. Impaired efferent arteriolar vasodilation C. Interstitial inflammation D. Toxic injury to the proximal tubules E. Vasculitis of the glomerular capillaries
A. Impaired afferent arteriolar vasodilation [NSAIDs inhibit prostaglandin synthesis, which can cause prerenal azotemia in at-risk patients. Patients with intravascular volume depletion (e.g., CHF, diarrhea, excess diuresis) and CKD depend on renal prostaglandin production to dilate the afferent glomerular arteriole and maintain GFR]
366
How does pyruvate kinase deficiency cause splenomegaly?
Pyruvate kinase deficiency causes hemolytic anemia due to failure of glycolysis and resultant failure to generate sufficient ATP to maintain erythrocyte structure As reticuloendothelial cells in the splenic red pulp are involved in removal of damaged RBCs, their increased activity in the setting of pyruvate kinase deficiency causes them to undergo hyperplasia —> splenomegaly
367
Occlusion of the __________ artery typically results in contralateral hemiparesis and hemisensory loss of the face and upper limb with relative preservation of lower limb function. If the occluded artery is in the dominant (usually left) hemisphere, aphasia may also occur.
Middle cerebral a.
368
______________ is an autosomal dominant condition caused by porphobilinogen deaminase deficiency. Most patients remain asymptomatic, but a minority present with acute attacks characterized by abdominal pain and vomiting, peripheral neuropathy, neuropsychological symptoms, and reddish-brown urine. Treatment consists of IV glucose or heme preparations, which downregulate ____________ enzyme activity
Acute intermittent porphyria; ALA synthase
369
________ syndrome results from damage to the angular gyrus of the dominant parietal lobe and is characterized by agraphia, acalculia, finger agnosia, and left-right disorientation
Gerstmann
370
Histologically, GISTs are characterized by a proliferation of stromal or epithelioid type cells and stain positive for _______, an oncogene involved in the pathogenesis of the tumor. This gene encodes a tyrosine kinase that is involved in cell proliferation and differentiation
c-kit
371
Test utilized in pts with malabsorption after an abnormal fecal fat test; reflects intestinal transport function and surface area, making it useful to differentiate between pancreatic disease and mucosal disease
D-xylose test [patients with pancreatic disease will have normal test because problem is with digestion, not absorption. Patients with mucosal disease will have abnormal test]
372
What type of anemia is associated with SLE — cold hemolytic anemia or warm hemolytic anemia?
Warm hemolytic anemia — in which there is a positive Coombs test at 37 C but not at 4 C
373
3rd gen cephalosporin used to treat Pseudomonas aeruginosa
Ceftazidime [other tx options include extended-spectrum penicillins (piperacillin, ticarcillin) and 4th gen cephalosporin Cefepime]
374
antibodies present in CREST syndrome
Anti-centromere
375
CN and pharyngeal arch associated with mandible, maxilla, zygoma, incus, and malleus; portion of maxillary a.
Arch 1; CN V
376
CN and pharyngeal arch associated with stapes, styloid, lesser horn of hyoid
Arch 2; CN VII
377
CN and pharyngeal arch associated with greater horn of hyoid, common carotid a., and proximal internal carotid a.
Arch 3; CN IX
378
Milrinone is a _____ inhibitor that leads to decreased degradation of cAMP which results in increased contractility, vasodilation, _____ preload, and ____ afterload
PDE-3 inhibitor; decreased preload; decreased afterload
379
________ is an inborn error of methionine metabolism, usually autosomal recessive deficiency in __________ enzyme (requires B6 cofactor) resulting in ectopia lentis, intellectual disability, marfanoid habitus, and risk of thromboembolic events
Homocystinuria; cystathionine beta synthase
380
Vitamin B1 deficiency characterized by muscle wasting, hyporeflexia, decreased vibration and proprioception, and signs of heart failure
Beriberi
381
Vasculitide associated with polymyalgia rheumatica
Giant cell arteritis
382
Most common heart abnormality with fragile X syndrome
MVP
383
Enzyme that breaks down triglycerides in chylomicrons and VLDL
Lipoprotein lipase
384
Enzyme that breaks down triglycerides in adipose
Hormone-sensitive lipase
385
Apolipoprotein that activates lecithin acyltransferase; found on HDL and chylomicrons
A-I
386
Apolipoprotein that facilitates chylomicron secretion
ApoB48
387
Apolipoprotein that facilitates uptake of chylomicron remnants; found on IDL but not LDL
ApoE
388
Cystinuria is associated with defective reabsorption of ____, _____, _____, and ______ leading to hexagonal urine crystals and staghorn calculi
COAL Cystine, ornithine, arginine, lysine
389
Vitamin deficiency with angular stomatitis, palpebritis, cheilosis, glossitis
Vitamin B2 def
390
Mycoplasma pneumoniae has a triple-layered bacterial membrane containing sterols; what is the tx?
Ethambutol | Macrolide
391
In utero exposure to lithium classically causes what fetal congenital heart defect?
Ebstein anomaly — tricuspid valve insufficiency
392
________ syndrome affects capillary-sized blood vessels leading to port wine stain, leptomeningeal angiomatosis, seizures, and glaucoma
Sturge-weber
393
3 major maninfestations of chronic chagas disease
Secondary achalasia Megacolon Dilated cardiomyopathy
394
Hyperacute transplant rejection is what type of HSR?
Type II — due to preformed antidonor antibodies
395
PDE inhibitor that inhibits platelet aggregation and acts as a direct arterial vasodilator; used to treat symptomatic peripheral artery disease (e.g., claudication)
Cilostazol
396
Which antifungal is associated with renal toxicity manifesting as electrolyte abnormalities — hypokalemia, hypomagnesemia — as well as decreased EPO and n/n anemia?
Amphotericin B
397
The concentrations of PAH, creatinine, inulin, and urea ______ as you travel along the kidney tubule
Increase [the concentrations of bicarb, glucose, and amino acids decrease]
398
Metabolic acidosis leads to renal ammoniagenesis — in which renal epithelial cells metabolize ______ to generate ammonium and bicarb. The ammonium is then excreted in urine and bicarb is reabsorbed to buffer the blood
Glutamine
399
Budding yeast with polysaccharide capsule
Cryptococcus neoformans
400
Thick walled spherules packed with endospores
Coccidioides immitis
401
Percentage of data included in 1, 2, and 3 standard deviations
1 SD = 68% 2 SD = 95% 3 SD = 99.7%
402
Condition characterized by segmental, transmural inflammation of arterial wall with fibrinoid necrosis; associated with hepatitis B
Polyarteritis nodosa
403
Differentiate topoisomerase I vs. topoisomerase II and give examples of chemotherapeutic inhibitors
Topoisomerase I induces single stranded breaks — inhibited by irenotecan and topotecan Topoisomerase II induces double stranded breaks — inhibited by etoposide (used in testicular and small cell lung ca)
404
Thioamides methimazole and PTU inhibit thyroid peroxidase with what effects?
Decreased iodine organification and coupling of iodotyrosinases [PTU also inhibits peripheral conversion]
405
Antibiotic that depolarizes cell membrane by creating transmembrane channels; primary adverse effect is myopathy (increased CPK)
Daptomycin
406
Protozoan cause of watery (secretory) diarrhea that can be life-threatening in immunocompromised pts; dx by acid-fast stain showing cystic organisms and histological appearance of villous blunting, inflammatory infiltrate in LP, and basophilic cysts lining brush border of mucosal surface
Cryptosporidium parvum
407
NRTI that is a thymidine analog that does not have a 3’OH group, so once added into DNA, synthesis is terminated
Zidovudine
408
Mydriasis, abd pain, diarrhea, piloerection, lacrimation, yawning = _____ withdrawal
Opioid
409
Young obese woman with daily headache that worsens with valsalva; b/l papilledema and transient visual disturbance d/t optic n. compression and impaired axoplasmic flow
Idiopathic intracranial HTN (pseudotumor cerebri)
410
Rubella is in what family of viruses?
Togavirus [both rubella and rubeola (paramyxovirus) cause a rash that starts on the face and spreads down, but RUBELLA spreads faster and does not coalesce; also RUBELLA is more commonly associated with postauricular/occipital LAD]
411
Rubeola is in what family of viruses?
Paramyxovirus
412
Difference between concentric and eccentric LVH
Concentric = caused by pressure overload (chronic HTN, aortic stenosis); sarcomeres added in parallel Eccentric = caused by volume overload (AR, MR, ischemia, dilated cardiomyopathy); sarcomeres added in series
413
Insulin resistance may occur d/t TNF alpha, catecholamines, glucocorticoids, and glucagon via phosphorylation of ____ or _____ residues of the insulin receptor
Serine; threonine
414
Aortic arch associated with true aortic arch and subclavian aa.
Arch 4
415
Aortic arch associated with pulmonary aa. and ductus arteriosus
Arch 6
416
Selective peripheral D1 agonist used in hypertensive emergency, especially in pts with renal insufficiency; causes arteriolar dilation, increases renal perfusion, and promotes diuresis and natriuresis
Fenoldopam
417
Segmented dsRNA virus that causes watery diarrhea with villous blunting, increased secretory crypt cells, and loss of brush border enzymes
Rotavirus
418
Pharmacologic tx for portal HTN
Somatostatin and octreotide inhibit release of glucagon and VIP —> indirectly reduce portal blood flow Vasopressin —> direct splanchnnic vasoconstriction
419
Lowest osmolarity (aka most dilute) segment of nephron
DCT [CD is most concentrated]
420
Psychiatric condition in which neurologic symptoms are incompatible with known disease
Conversion disorder
421
2nd gen antipsychotic that is the drug of choice for tx-resistant schizophrenia; AEs include agranulocytosis, seizures, myocarditis, and metabolic syndrome
Clozapine
422
A stroke affecting which of the following arteries is associated with upper extremity problems, homonymous hemianopia, aphasia, and hemineglect? ``` A. ACA B. PCA C. PICA D. MCA E. AICA ```
D. MCA
423
A stroke affecting which of the following arteries is associated with UMN signs and weakness affecting contralateral LE? ``` A. ACA B. PCA C. PICA D. MCA E. AICA ```
A. ACA
424
A stroke affecting which of the following arteries is associated with contralateral hemiparesis (weakness in face, arm, leg) ``` A. ACA B. PCA C. PICA D. MCA E. AICA ```
B. PCA
425
A stroke affecting which of the following arteries is associated with dysphagia, hoarseness, vestibulocerebellar symptoms? ``` A. ACA B. PCA C. PICA D. MCA E. AICA ```
C. PICA
426
Women < 55 presenting with resistant HTN and CNS involvement (HA, TIA, stroke, aneurysm), fibromusclar webs alternating with areas of aneurysmal dilation, loss of internal elastic lamina, and renal artery stenosis
Fibromuscular dysplasia
427
Antibodies associated with polymyositis
Anti-Jo-1 [anti-tRNA synthetase]
428
Decorticate vs. decerebrate posturing
Decorticate = flexor — damage to neural structures above red nucleus (cerebral hemispheres, internal capsule) Decerebrate = extensor — damage to brainstem at or below level of red nucleus (midbrain tegmentum, pons)
429
________ syndrome presents with infertility, gynecomastia, long lower extremities, and 47,XXY genotype. It is the most common cause of male hypogonadism and is characterized by progressive destruction and hyalinization of the seminiferous tubules, leading to small, firm testes. Serum inhibin levels are ______, testosterone levels are ______, FSH levels are ______, and LH levels are ______
Klinefelter Decreased; decreased; increased; increased
430
Regardless of the patient’s hydration status, the majority of water reabsorption in the nephron occurs in the _________
Proximal tubule
431
Serum albumin levels, serum bilirubin levels, and _________ are lab values reflective of liver function and are of greatest prognostic significance in patients with cirrhosis
Prothrombin time
432
Large, sporulating gram-positive rod that is encased in an antiphagocytic polypeptide capsule composed of D-glutamic acid
Bacillus anthracis
433
How does digoxin cause a reduction in heart rate?
By increasing parasympathetic tone, which leads to inhibition of AV nodal conduction
434
Alpha 2 adrenergic receptors ______ insulin secretion, and beta 2 adrenergic receptors _______ insulin secretion. The _________ effect is generally predominant
Inhibit; stimulate Alpha 2
435
Autosomal recessive disorder resulting from a defect in DNA-repair genes, making them hypersensitive to ionizing radiations. Manifestations include cerebellar ataxia, oculocutaneous telangiectasias, repeated sinopulmonary infections, and increased incidence of malignancy
Ataxia-telangiectasia
436
Alzheimer disease has a strong genetic predisposition. What 3 gene mutations are associated with early-onset Alzheimers, and what genotype is associated with late-onset Alzheimers?
Early onset = APP (Chr21), presenilin 1 (Chr14), and presenilin 2 (Chr1) — thought to promote the production of A-beta-amyloid Late onset = apolipoprotein E4 genotype
437
Renal clearance of which of the following would DECREASE following a dose of desmopressin? ``` A. Calcium B. Creatinine C. Glucose D. PAH E. Urea ```
E. Urea [Vasopressin and Desmopressin cause a V2 receptor-mediated increase in water and urea permeability at the inner medullary collecting duct. The resulting rise in urea reabsorption (decreased urea clearance) enhances the medullary osmotic gradient, allowing the production of maximally concentrated urine]
438
Protein defect, elevated lipoproteins, and major manifestations for familial chylomycronemia (hyperlipoproteinemia type I)
Defect in lipoprotein lipase and ApoC-II Elevated chylomicrons Major manifestations = acute pancreatitis, lipemia retinalis, eruptive skin xanthomas, hepatosplenomegaly
439
Protein defect, elevated lipoproteins, and major manifestations for familial cholesterolemia (hyperlipoproteinemia type II A)
Defect in LDL receptor and ApoB100 Elevated LDL Major manifestations = premature coronary artery disease, corneal arcus, TENDON xanthomas, and XANTHELASMAS
440
Protein defect, elevated lipoproteins, and major manifestations for familial dysbetalipoproteinemia (hyperlipoproteinemia type III)
Defect in ApoE Elevated Chylomicron and VLDL remnants Major manifestations = premature coronary artery disease and peripheral vascular disease, tuboeruptive and PALMAR xanthomas
441
Protein defect, elevated lipoproteins, and major manifestations for familial hypertriglyceridemia (hyperlipoproteinemia type IV)
Defect in ApoA-V Elevated VLDL Major manifestations = increased pancreatitis risk, associated with obesity and insulin resistance
442
Autosomal dominant disease characterized by pigmented mucocutaneous macules and numerous hamartomatous polyps in the GI tract; usually benign but can present with abdominal pain due to intussusception, obstruction, or malignant transformation
Peutz-Jeghers syndrome [mutations in serine/threonine kinase 11 (STK11) gene on Chr 19]
443
Wernicke encephalopathy is primarily associated with atrophy of the mammillary bodies. What is the classic triad it presents with?
Encephalopathy Oculomotor dysfunction (i.e., nystagmus) Ataia [Contrast with alcoholic cerebellar degeneration, which is due to loss of purkinje cells in the cerebellar vermis, leading to gait ataxia, truncal instability, and a rhythmic postural tremor of the fingers and hands]
444
What type of diabetes insipidus is associated with Lithium use?
Nephrogenic diabetes insipidus [Lithium impairs aquaporin-2 water channels and reduces responsiveness to endogenous and exogenous vasopressin]
445
A systolic murmur that increases in intensity with maneuvers that DECREASE LV blood volume is highly suggestive of hypertrophic cardiomyopathy. HCM is characterized by LV outflow obstruction that increases afterload and decreases cardiac output. The hypertrophy also leads to a small LV cavity and impaired relaxation of the LV walls with consequent diastolic dysfunction. The hypertrophied LV walls have high O2 demand that can exceed O2 supply and lead to exertional angina. T/F: HCM is associated with normal coronary arteries
True
446
Concomitant family history of colon and endometrial cancer in first-degree relatives strongly suggests ________ syndrome, a ______ ______ inherited cancer syndrome that markedly increases the risk of colorectal, endometrial, ovarian, and other cancers. It is the second most common cause of hereditary ovarian cancer (after BRCA gene mutations), and it is the most common inherited cause of colon cancer. It is caused by a germline mutation involving mismatch repair genes, leading to _______ instability
Lynch; autosomal dominant; microsatellite
447
Alkaptonuria is an autosomal recessive disorder due to a defect in homogentisic dioxygenase. Clinical features include darkening of urine after exposure to air, blue-black discoloration of cartilage and skin, and debilitating ____________
Osteoarthropathy
448
Which of the following is most likely increased in the extracellular space of a region affected by ischemia? ``` A. Na+ B. Cl- C. K+ D. Ca++ E. HCO3- ```
C. K+ [all others decrease in extracellular concentration; intracellular accumulation of Ca++ is a hallmark of ischemic injury]
449
In a normal mammalian kidney, densely immunostaining, protein-containing cells adjacent to the renal glomerulus are most likely ____________
JG cells in the JG apparatus
450
A test is performed in which multiple wells containing mixtures of ethanol and amniotic fluid are shaken and then examined to find the highest value well that contains a ring of stable foam. What does this ring of stable foam represent?
Surfactant
451
Marfanoid body habitus is defined by pectus deformity, tall stature, arachnodactyly, joint hyperlaxity, skin hyperelasticity, and scoliosis. The differential diagnosis includes Marfan syndrome, which is an autosomal dominant condition with distinguishing features including normal intellect, aortic root dilation, and upward lens dislocation. Also on the differential diagnosis is ________, an autosomal RECESSIVE mutation in the gene encoding _____________, resulting in intellectual disability, hypercoagulability, DOWNWARD lens dislocation, megaloblastic anemia, and a fair complexion
Homocystinuria; cystathionine-beta-synthase (CBS) [CBS is essential for degradation of methionine, and deficiency leads to buidup of both methionine and homocysteine]
452
Heparin is a safer choice than warfarin in pregnancy because of: ``` A. High lipid solubility B. High water solubility C. Higher albumin binding D. Higher Vd E. Slow onset of action ```
B. High water solubility [rule of thumb — water soluble materials do not readily cross the placenta while lipophilic materials do!]
453
________ describes the mixing of genomic segments in segmented viruses that infect the same host cell. This results in major antigenic shifts are are responsible for pandemics and epidemics of Influenza A. ________ refers to gene exchange that occurs through the crossing over of 2 dsDNA molecules. The resulting progeny can have recombined genomes with traits not present simultaneously in either parent virus
Reassortment Recombination
454
Lesions causing cyanotic congenital heart disease (5 Ts)
``` Tetralogy of Fallot Truncus arteriosus Transposition of the great arteries Total anomalous pulmonary venous connection Tricuspid atresia ```
455
New mucosal intestinal epithelial cells differentiate from stem cells residing in what histological layer?
Crypts of Lieberkuhn
456
A pt with chronic hepatitis B has a liver biopsy which shows spherical nodules within the confines of fibrous septae. The nodules are composed of what proliferating cell type?
Hepatocytes
457
What is the mechanism causing tardive dyskinesia in the sitting of long-term use of antipsychotics?
Dopamine receptor upregulation due to long-term blockade of these receptors in the nigrostriatal dopamine pathway
458
A congenital absence of dopamine beta-hydroxylase will impair synthesis of ______ and _______. Infants and small children may present with hypotension, ptosis, and hypothermia. Adults often present with postural/orthostatic hypotension, exercise intolerance, nasal congestion, and ejaculatory difficulties
Norepinephrine; epinephrine
459
Massive pulmonary emboli (e.g. saddle embolus) with significant occlusion of both pulmonary arteries causes _______ shock. Thus in the setting of shock due to saddle embolus, central venous pressure will ________, cardiac output will ________, and systemic vascular resistance will _________
Obstructive Increase; decrease; increase
460
Morphology and mechanism for adenovirus replication
dsDNA Utilize DNA-dependent DNA polymerase to replicate in the nucleus
461
A major cause of postural hypotension is insufficient stimulation of __________ receptors upon standing
Alpha1 adrenergic
462
Psoriasis is characterized by hyperkeratosis and epidermal hyperplasia. In predisposed pts, disruption of the normal skin barrier leads to activation of ________ cells, which induces recruitment and differentiation of _______ cells. The resulting inflammatory response stimulates local keratinocyte proliferation and production of antimicrobial peptides and inflammatory cytokines, leading to a self-amplifying inflammatory loop
Dendritic; CD4+ T cells (primarily Th17 and Th1)
463
Medical therapy for weight loss is recommended for pts who are unable to lose weight with dietary modification and exercise and are obese, or overweight with weight-related complications. One potential medication is _______, which is an intestinal lipase inhibitor that reduces absorption of dietary fat. It has significant GI side effects including diarrhea, fecal incontinence, and oily spotting.
Orlistat
464
Cancer of the rectum would spread hematogenously to the liver via what veinous pathway?
Rectum —> superior rectal v. —> inferior mesenteric v. —> portal venous system [note that the rectum ends at the dentate line; after the dentate line is the anus, which is drained by middle and inferior rectal vv. which drain into systemic circulation via the internal iliac v.]
465
Insulin increases the intracellular concentration of which of the following? ``` A. 2,3 BPG B. 3-hydroxybutyrate C. Fructose-1-phosphate D. Fructose-2,6-bisphosphate E. Lactate ```
D. Fructose-2,6-bisphosphate Insulin and glucagon regulate glucose metabolism via the bifunctional PFK-2/F-2,6-BPase enzyme complex. Insulin activates the PFK-2 domain, which converts F-6P to F-2,6-BP, which then activates PFK-1, increasing the production of F-1,6-BP and driving the cell toward glycolysis. Glucagon activates F-2,6-BPase, which degrades F-2,6-BP, favoring gluconeogenesis
466
Name the nonpolar/hydrophobic amino acids
``` Alanine Valine Leucine Isoleucine Phenylalanine Tryptophan Methionine Proline Glycine ``` [hydrophilic include arginine, histidine, lysine, aspartate, glutamate]
467
Probenecid can be used to enhance penillin’s efficacy in certain clinical settings like penicillin resistance, gonorrhea, or neurosyphilis. What is the MOA of probenecid?
Blocks organic anion transporters on renal tubular cells to inhibit tubular secretion of certain antibiotics (e.g., penicillin)
468
Cough syncope typically occurs in overweight male patients with COPD. What causes “cough syncope”?
Increased intrathoracic pressure during a coughing episode —> decreased venous return to the heart —> transient decrease in cardiac output and cerebral perfusion
469
Morphology and location of replication of orthomyxovirus
Segmented, negative-sense, ssRNA Unique among RNA viruses in that it is the only one (other than retroviruses) that replicates in the nucleus
470
A newborn with normal blood glucose, hyperammonemia, and increased urinary orotic acid most likely has a ________________ deficiency, the most common urea cycle disorder. Because of its location in the pathway, defects in this enzyme result in accumulation of orotic acid (an alternate metabolite of carbamoyl phosphate produced by the pyrimidine biosynthetic pathway) and decreased ________ production
Ornithin transcarbamylase (OTC) deficiency Citrulline
471
Patients with 17-alpha hydroxylase deficiency present with lack of pubertal development and HTN due to insufficient production of sex hormones and cortisol, and overproduction of mineralocorticoids (deoxycorticosterone and corticosterone). What changes would be seen in LH, ACTH, and renin?
LH increases ACTH increases Renin decreases
472
Inheritance pattern of medium-chain acyl-coA dehydrogenase (MCAD) deficiency
Autosomal recessive [note that heterozygotes have reduced enzyme function, while full disease manifestations of hypoketotic hypoglycemia are present in homozygotes]
473
In acute respiratory distress syndrome, hypoxemia results from ventilation-perfusion mismatch; this is largely due to __________ physiologic shunting as the fluid-filled, nonventilated alveoli continue to receive blood. In addition, formation of microthrombi results in inadequate perfusion of the remaining well-ventilated alveoli, causing _______ physiologic dead space
Right-to-left; increased
474
2 HIV antiretrovirals that act as allosteric RT inhibitors, inhibiting HIV DNA synthesis from RNA template by terminating DNA chain elongation
Efavirenz Nevirapine [NNRTIs]
475
5 HIV antiretrovirals that act as competitive nucleoside/nucleotide RT inhibitors, thus inhibiting HIV DNA synthesis from RNA template by terminating DNA chain elongation
``` Tenofovir Abacavir Emtricitabine Lamivudine Zidovudine ``` [NRTIs]
476
4 HIV antiretrovirals that inhibit HIV polyprotein cleavage
Atazanavir Darunavir Indinavir Ritonavir [protease inhibitors]
477
2 HIV antiretrovirals that inhibit HIV DNA integration into host genome
Dolutegravir Raltegravir [integrase inhibitors]
478
HIV antiretroviral that inhibits HIV fusion with target cell membrane by binding to HIV gp41
Enfuvirtide [fusion inhibitor]
479
What is the difference between fibroadenoma and fibrocystic change in the breast?
Fibroadenoma = common estrogen-sensitive benign breast tumor in young women; typically presents as solitary nontender, firm, well-demarcated, spherical, and mobile nodule corresponding to an encapsulated proliferation of stromal and ductal elements Fibrocystic changes = most common cause of palpable nodular changes in breast; generally present as poorly defined, diffuse increase in cysts and fibrotic tissue THROUGHOUT; lesions are often numerous and PAINFUL, with maximal tenderness during the premenstrual phase
480
Biopsy of a skin lesion demonstrates dermoepidermal junction aggregates of large cells that fuse with adjacent nests. The individual atypical cells have irregular nuclear contours, hyperchromasia, and are round or spindle shaped. A mutation of chromosome 9p21 is identified in atypical cells. This patient is most likely predisposed to which of the following? ``` A. Dermatofibrosarcoma protruberans B. Malignant fibrous histiocytoma C. Basal cell carcinoma D. Melanoma E. Neurofibroma ```
D. Melanoma [Histology described is a dysplastic nevus. Although most dysplastic nevi are clinically stable, some have the potential to evolve into melanoma. Mutations in the CDKN2A gene on chromosome 9p21 are associated with Dysplastic Nevi Syndrome]
481
Embryologic brain precursor of cerebral hemispheres and lateral ventricles
Telencephalon [Prosencephalon divides into telencephalon and diencephalon]
482
Embryologic brain precursor of midbrain and aqueduct
Mesencephalon
483
During embryologic development, the rhombencephalon divides into the ___________ (which later forms the pons, cerebellum, and upper 4th ventricle) and the __________ (which later forms the medulla and lower 4th ventricle)
Metencephalon; myelencephalon
484
Carbon dioxide is utilized in the de novo synthesis of pyrimidine and purine nucleobases. The first reaction for both of these processes occurs in the ______
Cytosol [for pyrimidines this invovles glutamine, CO2, and 2 molecules of ATP used to form carbamoyl phosphate via carbamoyl phosphate synthetase II. After initial synthesis in the cytosol, purine and pyrimidine nucleotides are utilized for DNA synthesis in the nucleus]
485
Some antifungals act at the cell membrane, while some act at the cell wall. Cell membrane active agents include amphotericin B, nystatin, and azoles. What class of antifungals function by inhibiting CELL WALL synthesis?
Echinocandins (caspofungin)
486
Complete molar pregnancy is an abnormal proliferation of ______ and ________ without fetal tissue. Due to paternal imprinting, staining with p57 is _________
Cytotrophoblasts; syncytiotrophoblasts; negative
487
_________ is preferentially expressed in the liver and pancreatic beta cells, where it plays an important regulatory role in glycogen synthesis and insulin secretion, respectively. Mutations that impair expression of this enzyme raise the glucose threshold required for insulin release, leading to maturity-onset diabetes of the young (MODY)
Glukokinase
488
CYP450 inhibitors
``` Acetaminophen/NSAIDs Antibiotics/antifungals (azoles) Amiodarone Cimetidine Cranberry juice, Gingko biloba, Vit E Omeprazole Thyroid hormone SSRIs (fluoxetine) ```
489
CYP450 inducers
``` Carbamazepine, phenytoin Ginseng, St. John’s wort Oral contraceptives Phenobarbital Rifampin ```
490
Gram negative rods may be cultured on MacConkey agar. Lactose fermenters form pink colonies, while non-lactose fermenters form white colonies. The lactose fermenters can be categorized based on fermentation speed. What are the fast fermenters vs. slow fermenters?
Fast = Klebsiella, E.coli, Enterobacter Slow = Citrobacter, Serratia
491
Gram negative rods may be cultured on MacConkey agar. Lactose fermenters form pink colonies, while non-lactose fermenters form white colonies. The non-lactose fermenters can be further categorized based on whether they are oxidase positive or negative. If oxidase positive, it is ____________. If oxidase negative and there is no H2S production, it is _________. If oxidase negative and there is H2S production (black color), it is either _______ or ________
Pseudomonas; Shigella; Salmonella; Proteus
492
How would the following parameters change in hypovolemic shock: arterial baroreceptor firing rate, systemic vascular resistance, pulmonary vascular resistance, and systemic capillary fluid transfer (filtration vs absorption)?
Decrease in arterial baroreceptor firing rate Increase in systemic vascular resistance Increase in pulmonary vascular resistance Systemic capillary fluid transfer favors absorption
493
A young female presents with intermittent diarrhea, nausea, and a 4lb weight loss over the past month. Physical exam reveals a migrating serpiginous pruritic rash around the anus. WBC is 8000 with 19% eosinophils. Which of the following will most likely reveal the diagnosis? ``` A. KOH prep B. Skin biopsy C. Bone marrow biopsy D. Peripheral smear E. Stool microscopy ```
E. Stool microscopy
494
A female presents with an ovarian mass and signs of virulization including hirsutism, male pattern baldness, breast atrophy, clitoral enlargement, and amenorrhea (last period was 5 months ago). What type of ovarian tumor is most likely?
Sertoli-Leydig cell tumor
495
A patient who has overdosed on aspirin would most likely show what changes in their serum HCO3, arterial pH, and blood PCO2?
All decreased
496
Vasopressin signaling occurs via _________
GPCR [V1 is Gq linked, and results in increased vascular smooth muscle contraction. V2 is Gs linked and results in increased H2O permeability and reabsorption via upregulation of aquaporin-2 in the collecting tubules of the kidney, as well as increased release of vWF]
497
2 y/o male presents with developmental delay, small stature with large head, irritability, poor eye contact, and flapping of the hands. Which of the following is the most likely cause? ``` A. Creation of alternative splice site B. Trinucleotide repeat expansion C. Frameshift mutation D. Missense mutation E. Nonsense mutation ```
B. Trinucleotide repeate expansion
498
Which of the following is true of RSV? A. DNA genome B. Double-stranded genome C. Mature virion lacking viral polymerase D. Mediation of cell entry via a fusion protein E. Viability on surfaces for several weeks
D. Mediation of cell entry via a fusion protein RSV (and all of the paramyxovirus family) contain surface F protein which causes respiratory epithelial cells to fuse and form multinucleated cells. Pavilizumab is a preventative monoclonal antibody against this fusion, used in premature infants
499
T/F: administration of Rho(D) immune globulin prevents formation of antibodies to RhD in a Rh-negative mother
True
500
A pt with a history of HTN and CKD on dialysis presents with SOB and confusion. EKG shows low voltage and electrical alternans, indicating possible pericardial effusion. What changes would be seen in BP, pulse, JVP, and pulsus paradoxus?
BP decreased Pulse increased (low voltage, electrical alternans, and tachycardia are classic triad of pericardial effusion) JVP increased Pulsus paradoxus increased
501
Shoulder pain is reproduced with external rotation against resistance. Along with teres minor, what other muscle tendon is likely inflamed?
Infraspinatus
502
Which of the following is used in the treatment of idiopathic chronic constipation? ``` A. Azathioprine B. Infliximab C. Lubiprostone D. Mesalamine E. Sulfasalazine ```
C. Lubiprostone [PGE1 derivative; activates chloride channels on the apical side of GI epithelial cells]
503
Inheritance of DMD
X-linked disorder typically due to frameshift deletions or nonsense mutations [female carrier status is uncertain due to random X inactivation]
504
A week old breast-fed male presents with poor weight gain, irritability, vomiting, jaundice, and hepatomegaly. A reducing substance test of the urine is positive, and a glucose oxidase result is negative. Which of the following is most likely present at increased concentrations in the liver? A. Fructose 1,6-bisphosphate B. Galactose 1-phosphate C. Glucose 1-phosphate D. Glucose 6-phosphate
B. Galactose 1-phosphate
505
Circular dsDNA virus that affects transplant patients (potentially leading to allograft rejection), and commonly targets the kidneys
BK virus (a polyomavirus)
506
Inheritance of cystic fibrosis
Autosomal recessive
507
Squamous cell carcinoma at the apex of the tongue is most likely to spread to what lymph nodes first?
Submental
508
A malignant anal mass below the dentate line is discovered. Which of the following lymph nodes should be evaluated first? ``` A. Internal iliac B. Popliteal C. Sacral D. Superficial inguinal E. Superior rectal ```
D. Superficial inguinal
509
Abrupt onset gross hematuria in an otherwise healthy patient with a family history of sickle cell disease suggests a diagnosis of ___________________ due to underlying sickle cell trait.
Renal papillary necrosis [Conditions associated with RPN include SCD/trait, analgesic nephropathy, diabetes, pyelonephritis, and UTI]
510
Viral cause of meningitis that may present with neuroinvasive disease characterized by asymmetric flaccid paralysis and/or parkinsonian features
West Nile Virus (ssRNA flavivirus)
511
A patient sustains an injury to the arm when tackled mid-throw during a football game. On PE, there is flattening of the deltoid muscle and insensitivity of the overlying skin to pinprick. Peripheral pulses are intact. Which of the following is responsible? ``` A. AC joint subluxation B. Anterior dislocation of the humerus C. Posterior dislocation of the humerus D. Clavicular fracture E. Fracture of the coracoid process ```
B. Anterior dislocation of the humerus [Anterior dislocations are the most common, classically following a blow to an externally rotated and abducted arm (e.g., throwing a football). Results in flattening of deltoid prominence, protrusion of the acromion, and anterior axillary fullness]
512
Absence of CD55 and CD59 on RBCs, hemolytic anemia, pancytopenia, thrombosis at atypical sites, and hemosiderosis (iron deposition in kidneys) indicate what diagnosis?
Paroxysmal nocturnal hemoglobinuria
513
________ leprosy is associated with cell-mediated immunity (Th1 response) and a positive lepromin skin test _________ leprosy is associated with humoral immunity (Th2 response) and a negative lepromin skin test
Tuberculoid Lepromatous
514
First-line pharmacotherapy for moderate-to-severe alcohol use disorder; works by preventing the reinforcing effects of alcohol use
Naltrexone — mu opioid antagonist [Another option is Acamprosate, which modulates glutamate neurotransmission at the NMDA receptor. Disulfuram is considered second-line due to poorer efficacy and dependence on supervised administration]
515
Propionic acidemia is an autosomal recessive congenital deficiency in propionyl-CoA carboxylase, the enzyme responsible for conversion of propionyl-CoA to methylmalonyl-CoA. Deficiency results in excess propionic acid accumulation in the blood, causing severe metabolic acidosis. Hypoglycemia and ketosis frequently develop secondary to the acidosis. Affected patients present 1-2 weeks after birth with lethargy, poor feeding, vomiting, and hypotonia. Treatment involves starting a low-protein diet containing minimal amounts of what 4 amino acids?
Valine Isoleucine Methionine Threonine
516
Most common cause of spontaneous lobar hemorrhage, particularly in the elderly; most common sites of hemorrhage include occipital and parietal lobes
Cerebral amyloid angiopathy
517
Nonrhythmic conjugate eye movements and involuntary jerking movements of the trunk and limbs are features of opsoclonus-myoclonus syndrome, a paraneoplastic syndrome associated with ________, which is the most common extracranial solid neoplasm in children. The tumor typically arises from the _________ cells of the adrenal medulla and presents with an abdominal mass and elevated catecholamine breakdown products
Neuroblastoma; neural crest cells
518
Major virulence factor of haemophilus influenzae type b is the polysaccharide capsule composed of ______ _______, which protects against phagocytosis and complement-mediated lysis by binding _________, a circulating regulator protein that normally prevents complement (C3b) deposition on host cells
Polyribosylribitol phosphate (PRP); factor H [the Hib vaccine is composed of PRP conjugated to a protein toxoid]
519
Initiation of allopurinol would increase activity of which of the following? ``` A. Azathioprine B. Ganciclovir C. Prednisone D. Tacrolimus E. Trimethoprim ```
A. Azathioprine [allopurinol competitively inhibits xanthine oxidase, resulting in increased conversion of azathioprine to its active metabolite via HGPRT]
520
What location is pacing the ventricles in a third degree AV block?
The AV node [in third degree block, the atria and ventricles are paced independently. The SA node paces the atria, while the AV node paces the ventricles]
521
What 2 classes of antibiotics cause QT prolongation?
Macrolides Fluoroquinolones [other medication-induced causes include odansetron, azoles, antipsychotics, TCAs, class IA antiarrhythmics, class III antiarrhythmics]
522
How does Crohn disease cause oxalate kidney stones?
Impaired bile absorption in the terminal ileum leads to loss of bile acids in stool with subsequent fat malabsorption. Intestinal lipids then bind calcium ions, and the resulting soap complex is excreted. Free oxalate (normally bound by calcium to form an unabsorbable complex) is absorbed and forms urinary calculi
523
Electrolyte abnormality associated with Legionella
Hyponatremia
524
A normal alveolar to arterial oxygen gradient is 5-15 mm Hg. What are 2 major causes of hypoxemia in the setting of a normal A-a gradient?
Hypoventilation (obesity hypoventilation syndrome, neuromuscular disorders) Low inspired fraction of O2 (high altitude)
525
A normal alveolar to arterial oxygen gradient is 5-15 mm Hg. What are 3 major causes of hypoxemia in the setting of an elevated A-a gradient?
Right-to-left shunt (cardiac septal defects, pulmonary edema) V/P mismatch (pulmonary embolism, COPD) Impaired diffusion (interstitial lung disease)
526
Streptococcus pyogenes is differentiated from other beta-hemolytic strep in that it is _______-positive and ________-sensitive
Pyrrolidonyl arylamidase (PYR); bacitracin
527
What is the mechanism behind inhaled amyl nitrite as the antidote for cyanide poisoning?
Nitrites are oxidizing agents that induce methemoglobinemia, which contains ferric (Fe3+) rather than ferrous (Fe2+) iron Cyanide binds to ferric iron more avidly than to mitochondrial cytochrome enzymes, diminishing cyanide’s toxic effect
528
Glomerulonephritis, a photosensitive skin rash, and arthralgias in a young woman are suggestive of SLE. Up to 30% of pts with SLE have _______ antibodies, which cause paradoxical aPTT prolongation and a false positive RPR/VDRL. These pts are at increased risk for venous and arterial thromboembolism and unexplained, recurrent pregnancy loss
Antiphospholipid antibodies
529
What virulence factor of E.coli is most important for its ability to cause UTI?
P fimbriae [other virulence factors it has are: LPS leading to shock, K1 capsular polysaccharide leading to neonatal meningitis, Verotoxin leading to bloody gastroenteritis, and heat-stable/heat labile enterotoxins leading to watery gastroenteritis]
530
Diabetic neuropathy may involve CN III, leading to ptosis, down-and-out gaze, and normal light and accommodation reflexes. Which of the following describes the mechanism by which this occurs? A. Nerve compression B. Nerve ischemia C. Nerve inflammation
B. Nerve ischemia [central ischemia affects somatic nerve fibers but spares peripheral parasympathetic fibers, which is why the pupillary size and reactivity is normal in diabetic CN III mononeuropathy]
531
What type of vaccine protects against tetanus, and what type of immunity does it generate?
Tetanus toxoid vaccine (formaldehyde-inactivated tetanus toxin) Stimulates a humoral antibody response (antitoxin antibodies)
532
The major virulence factor of Streptococcus pyogenes is M protein, an alpha helical protein that shares structural homology with tropomyosin and myosin. What is the purpose of this virulence factor?
Inhibits phagocytosis, prevents complement binding, and aids in epithelial attachment [Antibodies against M protein form shortly after acute infection, and may cross react with epitopes on myosin, leading to rheumatic carditis]
533
What are the Light criteria defining an EXUDATIVE effusion regarding pleural fluid protein:serum protein ratio AND pleural fluid LDH:serum LDH ratio?
Pleural fluid protein:serum protein ratio >0.5 Pleural fluid LDH:serum LDH ratio > 0.6 [Or pleural fluid LDH > 2/3 upper limit of normal serum LDH; causes of exudative infusion include infection like PNA, malignancy, and rheumatologic disease]
534
Lesions of the macula result in which of the following? ``` A. Homonymous hemianopsia B. Binasal hemianopsia C. Bitemporal hemianopsia D. Arcuate scotomas E. Central scotomas ```
E. Central scotomas
535
Congenital ________ presents with chorioretinitis, hydrocephalus, jaundice, hepatosplenomegaly, and a placenta infiltrated with crescent-shaped organisms. Most cases in pregnant women arise due to consumption of undercooked, contaminated meat
Toxoplasmosis [other mechanism of acquiring infection is incidental ingestion of oocytes from cat feces (felines are definitive host)]
536
P.aeruginosa is a gram-negative, opportunistic pathogen with multiple mechanisms of antibiotic resistance, including beta-lactamases, efflux pumps, and antibiotic-modifying enzymes. Antibiotic-modifying enzymes add chemical groups (e.g., acetyl, adenyl, phosphate) to a target drug and are the most common mechanisms of resistance of what class of antimicrobial?
Aminoglycosides (e.g., gentamicin) [Altered aminoglycosides have a reduced ability to bind to the 16S ribosomal RNA within the 30S ribosomal subunit]
537
The most susceptible vascular regions to atherosclerosis involve bends and branch points that encourage turbulent blood flow, which disrupts vascular wall integrity and leads to endothelial cell dysfunction. The hemodynamics of what 2 vascular beds make them most susceptible to atherosclerosis of all the major vascular beds in the body?
Lower abdominal aorta Coronary arteries
538
Recovery from a subarachnoid hemorrhage may be complicated by delayed cerebral ischemia due to cerebral vasospasm, typically manifesting within 3-12 days after the initial incident with altered mental status and/or focal neurologic deficits. What pharmacologic agent improves outcomes by preventing or reducing the extent of these deficits?
Nimodipine, a selective calcium-channel blocker [neuroprotective effect is due to combo of increased cerebral vasodilation and decreased calcium-dependent excitotoxicity]
539
Glucose transport protein GLUT-4 is an insulin-sensitive glucose transporter expressed in _________ and ___________ that translocates the plasma membrane in response to increasing insulin levels
Skeletal muscle cells; adipocytes [In contrast, GLUT-1, 2, 3, and 5 are always present in the plasma membrane and constitutively transport glucose in an insulin-independent manner]
540
What type of colon cancer can produce large quantities of prostaglandin E2, resulting in increased mucin production and a secretory diarrhea characterized by watery, mucinous stools?
Villous adenomas [mucin is a potassium-rich glycoprotein, note that excess production can result in hypoproteinemia and hypokalemia]
541
When comparing Th1 vs. Th2 differentiation: _______ are involved in the pathogenesis of UC. They produce IL-5 and IL-13 which contribute to inflammation and damage of intestinal mucosa. ______ are involved in Crohns disease. They produce IL-2 and IFN-y, promoting further T cell response, activation of macrophages, and differentiation of macrophages into giant cells. Th17 are a subset of this type, and contribute to cell injury in CD
Th2 Th1
542
Most common trigger for DIC in pregnancy
Release of tissue factor from injured placenta (e.g., placental abruption) into maternal circulation
543
AEs of mood stabilizers in bipolar disorder: Diabetes insipidus, hypothyroidism, tremor, and ebstein anomaly are associated with ________ Aplastic anemia, SIADH, and neural tube defects are associated with _____
Lithium Carbamazepine
544
Excessive bleeding in a patient with ESRD is due in part to uremic toxins in circulation. How would the following parameters change in this bleeding disorder: PT, aPTT, platelet count, bleeding time
PT normal aPTT normal Platelet count normal Bleeding time prolonged
545
Most common infectious agent causing fetal hydrops; mom often complains of transient arthralgias during pregnancy
Parvovirus B19 [naked ssDNA virus]
546
Innervation of gluteus medius
Superior gluteal n
547
What 2 features are required for a diagnosis of TTP?
MAHA | Thrombocytopenia
548
What effects do ACE inhibitors have on GFR and serum K+?
Decreased GFR Hyperkalemia
549
A 65 y/o man suffers an acute anterior MI. How would the following parameters change compared to a healthy man of the same age: systemic vascular resistance, pulmonary vascular reistance, pulmonary capillary wedge pressure
SVR increases PVR decreases PCWP increases
550
The parotid duct passes through which of the following muscles to enter the oral cavity: ``` A. Buccinator B. Masseter C. Orbicularis oris D. Temporalis E. Zygomaticus major ```
A. Buccinator
551
Commonly injured bones in a FOOSH include fracture of the ______ and/or dislocation of the ________ (which can present with acute carpal tunnel syndrome)
Scaphoid; lunate
552
What class of HIV antiretrovirals is associated with lactic acidosis?
Nucleoside reverse transcriptase inhibitors
553
Patients with osteogenesis imperfecta have an issue with what type of wound healing? ``` A. Cell migration B. Clot formation C. Granulation tissue deposition D. Inflammation E. Scar formation ```
E. Scar formation [Osteogenesis imperfecta have decreased production of type 1 collagen, which is important in late wound repair (scar formation)]
554
A positive acidified serum test in the setting of anemia and “dark urine in the morning” indicates PNH. This is characterized by which of the following? ``` A. Abnormal cell morphology B. Decreased G6PD activity C. Defect in a cell membrane anchor protein D. Destabilization of cytoskeleton E. Underproduction of globin proteins ```
C. Defect in a cell membrane anchor protein [impaired synthesis of GPI ancho fro decay-accelerating factor (CD55) and membrane inhibitor of reactive lysis (CD59) that protects RBC from complement]
555
What are the fibrous proteins that form the 2-dimensional network on the inner surface of the nuclear membrane?
Lamins
556
Which of the following is a virus that forms plaques on human laryngeal cells but rapidly loses its ability to form plaques when dried or exposed to acidic pH? ``` A. Coronavirus B. Coxsackievirus C. Epstein Barr virus D. Norovirus E. Rotavirus ```
A. Coronavirus
557
Male with gradually progressive weakness, anorexia, and weight loss over period of 6 months, BP is 75/41, skin is hyperpigmented, and morning cortisol is low. Which of the following? ``` A. Adrenocortical carcinoma B. Amyloidosis C. Autoimmune adrenalitis D. Basophilic pituitary adenoma E. Metastasis to adrenal gland ```
C. Autoimmune adrenalitis [aka Addisons disease]
558
Total occlusion of blood flow by a tourniquet followed by removal of occlusion results in 50% increase in flow, then a return to baseline control values. What substance is responsible?
Adenosine
559
HOX genes play a role in embryogenesis through regulation of ______
Transcription
560
What serum marker is most likely elevated with a biliary tract obstruction, specifically a common bile duct obstruction?
Alkaline phosphatase
561
What ion channel is blocked by tetradotoxin from a puffer fish?
Sodium channels
562
Infectious mononucleosis results in atypical lymphocytes seen on peripheral smear. What type of cells are these?
Reactive CD8+ T lymphocytes [Not infected B cells!!!]
563
Young pt with lethargy and mild hepatomegaly, hypoglycemia, lactic acidemia, ketonemia, and metabolic acidosis. IV administration of glycerol or fructose does not increase serum glucose. IV administration of galactose DOES increase serum glucose. What liver metabolic pathway is the cause of these findings?
Gluconeogenesis
564
A 3 week period of bed rest results in what physiologic change? ``` A. Decreased blood volume B. Decreased plasma sodium C. Increased plasma aldosterone D. Increased plasma volume E. Increased sympathetic tone ```
A. Decreased blood volume
565
A 55 y/o male with an acute MI, hypotension, elevated JVP, and pleural effusion results in what changes in PCWP, CO, and SVR?
PCWP increases CO decreases SVR increases
566
In neonatal respiratory distress syndrome, supplemental O2 can result in complications of retinopathy of prematurity, intraventricular hemorrhage, and _____ _____
Bronchopulmonary dysplasia [lungs firm and poorly aerated, prominent interstitial fibrosis surrounding irregular, dilated airspaces with bronchiolar metaplasia]
567
What veins allow for tumor cells in the breast to obtain access to vertebral bodies for metastasis?
Intercostal vv.
568
What causes the increase in total serum T3/T4 in pregnancy?
Increased hepatic production of T4-binding globulin
569
Sudden onset dysarthria, dysphagia, and dizziness; exam shows decreased pain sensation on right side of face and complete loss of pain sensation on left side of body. What blood vessel is affected?
Anterior inferior cerebellar artery (on right side)
570
Which of the following explains hypokalemia of alcohol withdrawal? ``` A. Catecholamine-mediated intracellular shift of K+ B. Decreased dietary intake of K+ C. Decreased renin release D. Decreased urine flow rate E. Increased muscle breakdown ```
A. Catecholamine-mediated intracellular shift of K+
571
Deficiency of vitamin ____ appears similar to Vit B12 deficiency with neurologic presentation including decreased position/vibration sense, but it does not show megaloblastic anemia or hypersegmented neutrophils
Vitamin E
572
What stage of the cell cycle are cyclins made?
G2
573
Loss of pain and temp on the right side of the face and the left side of the body, paralysis of vocal cord on the right, and absence of gag reflex on the right. Which of the folowing is damaged? ``` A. Left ventromedial medulla B. Right ventromedial medulla C. Left dorsolateral medulla D. Right dorsolateral medulla E. Left ventrolateral pons F. Right ventrolateral pons G. Left ventromedial midbrain E. Right ventromedial midbrain ```
D. Right dorsolateral medulla
574
Breakdown of dipeptides and tripeptides into free amino acids takes place primarily in what area in the GI tract?
Intestinal mucosa
575
Which of the following is the most likely cause of drug-induced bone marrow suppression in HAART? ``` A. Azithromycin B. Lamivudine C. Nelfinavir D. Pentamidine E. Zidovudine ```
E. Zidovudine
576
17 y/o F who has never had a period, BMI is 15, breast development stage 5, pubic/axillary hair stage 1, pelvic exam shows blind vaginal pouch. Which of the following is most likely? ``` A. Decreased serum estrogen B. Decreased serum LH C. Hyperplastic adrenal cortices D. Increased serum testosterone E. Streak ovaries F. Uterus ```
D. Increased serum testosterone
577
What artery maintains anastomotic supply to testicle if the testicular artery is ligated?
Artery of the ductus deferens
578
19 y/o M reports 1 week of weakness in neck and extremities; position sense is also impaired. Bowel and bladder function normal. Which is most likely? ``` A. ALS B. Parkinson C. Poliomyelitis D. Polyneuropathy E. Syringomyelia ```
D. Polyneuropathy
579
Which of the following are connected by desmosomes: ``` A. Basal keratinocyte:lamina lucida B. Basal keratinocyte:suprabasal keratinocyte C. Granular keratinocyte:stratum corneum D. Lamina lucida:lamina densa E. Melanocyte:basal keratinocyte ```
B. Basal keratinocyte:suprabasal keratinocyte
580
A strain of bacteria sensitive to ampicillin is cultured with another type of bacteria that is resistant to ampicillin. After co-culturing, the initial bacteria is now resistant to ampicillin. What mode of bacterial genetic transfer occurred?
Conjugation
581
2 initial substrates for heme synthesis
Glycine | Succinyl coA
582
Inability to recognize objects unless touching them or hearing them (alexia) represents a lesion supplied by what artery?
Posterior cerebral a. (Affecting occipital lobe)
583
Inability of alpha intercalated cells to secrete H+, so no new HCO3 is generated, resulting in metabolic acidosis Urine pH > 5.5 Serum K+ decreased
RTA type 1 (distal renal tubule) [causes: amphotericin B toxicity, analgesic nephropathy, congenital anomalies/obstruction, autoimmune disease like SLE]
584
Defect in PCT HCO3 reabsorption resulting in metabolic acidosis Urine pH < 5.5 Serum K+ decreased
RTA type 2 (proximal renal tubule) [causes: fanconi syndrome, multiple myeloma, carbonic anhydrase inhibitors]
585
Hypoaldosteronism or aldosterone resistance leading to hypokalemia, which results in decreased NH3 synthesis in PCT and subsequent decrease in NH4 secretion Urine pH <5.5 (or variable) Serum K+ increased
Type 4 RTA (hyperkalemic RTA) [causes: decreased aldosterone production due to diabetic hyporeninism, ACEIs, ARBs, NSAIDs, heparin, cyclosporin, adrenal insufficiency; or aldosterone resistance due to K+ sparing diuretics, nephropathy d/t obstruction, or TMPSMX]
586
Herniation of abdominal contents into the left pleural cavity in a full term infant indicates maldevelopment of what structure
Left pleuroperitoneal membrane
587
A genetic abnormality in what enzyme results in slow recovery from paralysis induced by a short-acting neuromuscular blocking agent?
Pseudocholinesterase
588
Female infant with fever of 102, vomiting, diarrhea, and dehydration, exam of a stool suspension by negative stain electron microscopy shows viral particles with a wheel-like shape. What is the morphology of the involved virus?
Naked dsRNA, segmented icosahedral
589
35 y/o man with severe midline back pain due to compression fractures at T10 and L1, 8 lb weight gain, and imaging shows a pituitary macroadenoma. The most likely cause of his symptoms is a tumor producing what hormone?
ACTH
590
A prosthetic endograft is inserted in the femoral artery, threaded up the aorta, then expanded when in place. It extends from just below the renal arteries to the bifurcation of the aorta. Which of the following organs is most likely to lose its primary blood supply and now must rely on collateral circulation: ``` A. Adrenal gland B. Descending colon C. Pancreas D. Spleen E. Stomach ```
B. Descending colon
591
A patient on alendronate, calcium carbonate, ezetimibe, HCTZ, and simvastatin is placed on ciprofloxacin for a UTI. Which of her medications most likely interfered with the efficacy of the antibiotic?
Calcium carbonate
592
After a chemical attack, a patient is given atropine. What other drug is most appropriate to add?
Pralidoxime
593
A 17 y/o male is short and thin. His FSH is 5 mlU/mL (N=4-25) and his testosterone is 8 nmol/L (N=10-35). Which of the following findings is most likely? A. Enlarged testes B. Excess body hair C. Gynecomastia D. Low pitched voice
C. Gynecomastia
594
If all secondary causes of pericarditis are ruled out, which of the following is the most likely cause of a primary pericarditis? ``` A. Bacterium B. Fungus C. Parasite D. Tumor E. Virus ```
E. Virus
595
5 y/o female with chronic cough worse at night and productive of thick green sputum. History of intermittent cramps in RLQ and frequent respiratory tract infections. Her 8 y/o bro is healthy. PE shows clubbing of the fingers and hyperresonance to percussion. Diffuse inspiratory crackles and pulmonary wheezing on expiration. CXR shows hyperinflation especially prominent in the right upper lobe. Which of the following represents the likelihood of her brother being a carrier? ``` A. 1/1 B. 1/2 C. 1/3 D. 1/4 E. 2/3 ```
E. 2/3
596
If mom is taking valproic acid, the fetus would be at greatest risk for teratogenic effects at which stage of pregnancy? ``` A. 0-2 weeks B. 3-8 weeks C. 9-12 weeks D. 12-18 weeks E. 18-24 weeks ```
B. 3-8 weeks
597
Following an operation, a 68 y/o patient has a lung region that is underventilated but well perfused. This would lead to an increase in which of the following: ``` A. Alveolar dead space B. Anatomic dead space C. Physiologic dead space D. Physiologic shunt E. PO2 ```
D. Physiologic shunt
598
A pt who recently returned from Africa is started on oral chloroquine therapy for malaria caused by plasmodium vivax. His initial response is good, but he develops recurrent parasitemia 2 months later. Which of the following explains the recurrence? A. Chloroquine is ineffective on P.vivax B. Chloroquine is ineffective on the exoerythrocytic malarial tissue stages C. Chloroquine is only effective when combined with metronidazole D. The patient has a second previously occult malaria infection
B. Chloroquine is ineffective on the exoerythrocytic malarial tissue stages
599
As an incidental finding, only 3 parathyroid glands are found on the posterior side of the thyroid gland, but one is missing from its usual superior location on the right. What embryological event led to this finding?
Abnormal migration of endoderm from the 4th pharyngeal pouch
600
Following a 4 week course of radiation therapy for breast cancer, a patient develops bilateral patches of atelectasis in the upper lung fields. The atelectasis in this patient most likely developed because of which of the following: ``` A. Compression B. Consolidation C. Contraction D. Obstruction E. Resorption ```
C. Contraction
601
Total cholesterol of 200, HDL of 50, and triglycerides of 550. Which lipid-lowering agent should be added?
Fenofibrate
602
The presence of a lysine (k487) for a glutamate (E487) codes for the oriental variant of aldehyde dehydrogenase. What effect does this have on the enzyme?
Decreased catalytic efficiency of K487
603
X linked recessive disease resulting in defective __________ leads to deafness, hematuria, and progressive renal failure
Type IV collagen
604
Patient with group A strep pyogenes has a widespread, red, sandpaper-like rash over the extremities. What strep component is the cause of these skin findings?
Erythrogenic toxin
605
A study designed to test effectiveness of a treatment divides 100 women into 2 groups. 48 receive the new drug, 52 receive standard therapy. The primary purpose of assigning patients to different groups is to create which of the following: A. Double blind study B. Single blind study C. 2 groups with similar sample size D. 2 groups with similar underlying characteristics
D. 2 groups with similar underlying characteristics
606
Secretion of hormones from which cells causes contraction of the gallbladder?
Enteroendocrine cells of the small intestine
607
6 month history of increased forearm bruising, extensive wrinkling, scaly erythematous patches on the face, and irregularly shaped brown macules on the face and forearms. Ecchymoses are of various stages of healing and more numerous on the right side. Labs are all normal. There is no gum bleeding noted. Which of the following is the most likely cause of ecchymoses? A. Exocytosis of lymphocytes B. Extensive solar elastosis C. Impaired platelet function D. UV destruction of langerhans cells
B. Extensive solar elastosis
608
A man with normal renal function receives a heart transplant. One year later he has a BP of 170/110 and a serum creatinine of 2.1. What immunosuppressive drug most likely caused these findings?
Cyclosporine
609
A pt with a PE is put on IV heparin and 24 hrs later warfarin is added. On day 2, PTT is prolonged and PT is WNL. Which of the following best explains the normal PT and INR? A. Heparin-warfarin interaction B. Long half life of factor II (prothrombin) C. Too low of a dose of warfarin D. Undetected liver disease
B. Long half life of factor II
610
1 day of fever, chills, confusion, and memory loss after returning from the Gulf coast where pt walked barefoot on the beach. Hx of severe cirrhosis and portal HTN. Temp 102, BP 90/40, PE shows cellulitis of right LE with early blister formation. One day later cultures grow gram-negative lactose fermenting organism. What is the most likely causal organism?
Vibrio vulnificus
611
Which of the following should pts with Raynauds avoid? ``` A. Acetaminophen B. Dextromethorphan C. Diphenhydramine D. Ibuprofen E. Phenylephrine ```
E. Phenylephrine
612
During a study a pt with normal calcium, PO4, and PTH is given infusion of 2 g calcium chloride over 2 hours. His calcium concentration is now 11.5 mg/dL. Compared with pre-infusion level, which of the following substances is most likely increased? ``` A. 7-dehydrocholesterol B. 1,25-dihydroxycholecalciferol C. 24,25-dihydroxycholecalciferol D. Previtamin D3 E. Vitamin D3 ```
C. 24,25-dihydroxycholecalciferol
613
10 y/o M with 4 day hx of nosebleeds and easy bruising. 3 weeks ago he had a URI. PE shows ecchymoses over upper and lower extremities. Labs show normal Hb and leukocyte count with decreased platelet count. Bone marrow smear shows increased megakaryocytes. Which of the following is most likely? A. Abs against gp IIb/IIIa complex B. Decreased binding of GpIa/IIa to collagen C. Decreased concentration of GpIa/IIa D. Decreased concentration of gp IIb/IIIa E. Decreased synthesis of thromboxane A2
A. Abs against gp IIb/IIIa complex
614
Pt has severe hemorrhage and shock, xray shows fracture of left 9th and 10th ribs. What organ is injured?
Spleen
615
Pt has 47, XXY karyotype and molecular genetic studies show one of the X chromosomes was inherited from the pts father. An error of chromosome segregation most likely occurred during anaphase at which stage of spermatogenesis in the pts father?
Primary spermatocyte
616
39 y/o male with 1 week hx of red spots on shins, joint pain, and fatigue. PE shows purpura over lower extremities. Liver edge is 4 cm below R costal margin. Labs show elevated LFTs, positive Hep C Abs, cyroglobulins, decreased C4, proteinuria 4+, and numerous RBCs in urine. What type of HSR?
Type III
617
Pt is fearful of being stuck with needle while awake. The most appropriate anesthesia administered by mask to anesthetize the pt quickly would have which of the following characteristics? ``` A. High blood solubility B. High CSF solubility C. High lipid solubility D. Low blood solublity E. Low lipid solubility ```
D. Low blood solubility
618
Which of the following is the genetic mechanism of antigenic shift? ``` A. Amplification B. Frameshift C. Point mutation D. Reassortment E. Recombination ```
D. Reassortment