"High Yield or U-World Only" Step 1 Cards Flashcards

1
Q

IV Benzodiazepines

  1. Main one
  2. Indication
  3. MOA
A
  1. Lorazepam
  2. Intitial drug of choice for status epilepticus
  3. They work by enhancing the effect of GABA at GABA -A receptor, leading to increased chloride influx and suppression of AP firing
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2
Q

What is Vernet syndrome?

A

It is when you have lesions of the jugular foramen, thus leading to CN Ix, X, and XI dysfunction

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3
Q

What are lamellar bodies?

A

They are organelles which contain parallel stacks of membrane lamellae and are a component of type II pneumocytes. They function to store and release pulmonary surfactant.

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4
Q

Normal Vitamin E function

A

Protection of fatty acids against oxidation

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5
Q

What is responsible for swelling in myocardial cells during ischemia?

A

Ion pump failure due to ATP loss, leading to increased intracellular Na+ and Ca2+

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6
Q

Between (Na+, K+, Cl-, and Ca2+):

Which are mostly located intracellularly? Extracellularly?

A

Intracellular: K+

Extracellular: Na+, Cl-, Ca2+

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7
Q

What path does fetal blood take from the placenta to the heart?

A

Umbilical vein - liver - ductus venosus - IVC - heart

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8
Q

What type of Bacteria are capable of surviving boiling?

A

Spore forming

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9
Q

What are Hamartomas? How do they present? What are they composed of?

A

The most common benign lung tumor

Present as asymptomatic peripherally located “coin lesion” in patients 50-60 y/o

Composed of disorganized cartilage, fibrous and adipose tissue

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10
Q

What is responsible for the clinical variability of mitochondrial diseases?

A

HETEROPLASMY

The mixture of (2) types of genetic material

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11
Q

Lidocaine

  1. What class drug?
  2. MOA?
  3. Effect?
A
  1. IB antiarrhythmic
  2. Binds (mostly) to inactivated sodium channels and rapidly dissociates
  3. Effective in suppressing v.tach induced by rapidly depolarizing and ischemic myocardium.
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12
Q

Primase

How is it related to uracil showing up in partially replicated DNA strands?

A

A DNA-dependent RNA polymerase that incorporates short RNA primers into replicating DNA

Because it is composed of RNA, this primer could have uracil in it

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13
Q

Lung mass + hyponatremia, is suggestive of what?

A

SIADH (secondary to Small cell lung carcinoma)

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14
Q

Pertussis (Whooping cough)

  1. Who gets it?
  2. Describe the (3) phases
  3. What causes it?
A
  1. Was mostly in kids prior to vaccinations. Now usually adolescents/adults w/o a booster
  2. (phases)
    • Catarrhal stage- similar to many routine URI’s
    • Paroxysmal stage- severe coughing spells w/ classic whoop (post-tussive emesis)
    • Convalescent stage- during which cough improves
  3. Caused by gram-negative Bordetella pertussis.
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15
Q

Thiazide diuretics

  1. Indication
  2. MOA
  3. Effect on serum Ca2+
A
  1. Primary HTN
  2. Inhibit the Na+/Cl- cotransporter in the distal tubule, leading to inc. excretion of Na+ and H2O (as well as K+ and H+ ions)
  3. Increases distal tubular Ca2+ reabsorption, causing both hypercalcemia and hypocalciruria
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16
Q

What effect does left ventricular HF have on lung compliance?

A

Leads to fluid accumulation in the lung interstium resulting in decreased lung compliance.

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17
Q

What artery is responsible for supplying the occipital lobe?

A

Posterior Cerebral Artery

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18
Q

Linkage disequilibrium

A

When a pair of alleles are inherited together in the same gamete (haplotype) more or less often than would be expected given random chance

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19
Q

Which components of the skin drain to the superficial inguinal lymph nodes?

A

All skin from the umbilicus down, including the anus (below the pectinate line), excluding the testes, glans penis, and posterior calf

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20
Q

Name the muscles/areas that the musculocutaneous nerve innervates/provides sensory innervation to (3)

A
  1. Movement to Major forearm flexors
  2. Movement Corcobrachialis
  3. Sensory to lateral forearm
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21
Q

Theophylline

  1. Indication
  2. MOA
  3. Metabolization
  4. AE
A
  1. Used as an alternate therapy for asthma and COPD
  2. Adenosine receptor antagonist and phosphodiesterase inhibitor that causes bronchodilation by increasing cAMP levels and has mild anti-inflammatory effects
  3. Metabolized predominantly by hepatic cytochrome oxidase
  4. AE: Theophylline toxicity
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22
Q

After invading the nasopharnyx, how does N. meningitidis gain access to the brain?

A

Bloodstream followed by choroid plexus

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23
Q

Between positive/ negative predicative values, and sensitivity/ specificty, which are dependent on disease prevalence in the tested population?

A

Positive/ negative predictive values

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24
Q

Name the Glycogen Storage Diseases

A

Very Poor Carbohydrate Metabolism

  1. Von Gierke Disease
  2. Pompe Disease
  3. Cori Disease
  4. McArdle Disease
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25
Describe the Chvostek sign and the Trousseasu sign associated with DiGeorge Syndrome
The hypocalcemia assc. with DGS leads to increased neuromuscular excitability. _Chvostek sign_- tapping on the facial nerve elicits **twitching of the nose and lips** _Trousseau sign_- inflation of the BP cuff leads to carpal spasm
26
Beta Blocker MOA
They dec. AV nodal conduction, leading to an increased AV nodal refractory period
27
What is the common clinical presentation for a pneumothorax? What is often the cause of spontaneous pneumothorax?
1. Sudden unilateral chest pain 2. Hyperresonance 3. Absent breath sounds 4. Often seen in tall, thin males around age 20 Can be the spontaneous result of the rupture of an **apical subpleural bleb**
28
Describe the specific effect of the superantigens assc. with TSS
THey interact with MHC II on APCs (macrophages) and w/ T-cell receptor to widely _activate T cells_. (this leads to IL2 release from T cells, and TNF / IL1 release from macrophages, ultimately causing shock symptoms)
29
Effect of ACE-I on GFR
Decreases GFR (because AT-II can no longer constrict the efferent arteriole)
30
Following focal ischemia in an MI, how long until there is a **loss of cardiomyocyte contractility?**
Loss of focal contractility occurs within _60secs_!
31
_Polyarteritis nodosa_ 1. Pathology 2. What organ is normally spared? 3. Presentation 4. Potential consequences
1. Segmental, transmural, necrotizing nflammation of medium to small sized arteries in _any_ organ 2. **Lungs are spared** 3. Can be cutaneous manifestations, including livedo reticularis and palpable purpura 4. Inflammation can result in ischemia, infarcation, or hemorrhage and _bead-like aneurysm formation_
32
Presentation/ potential complications of femoral hernias
They can present with groin discomfort or manifest with a bulge on the upper thigh. Incarceration and strangulation are common complications of femoral hernias.
33
Describe the essential differences in **excitation-contraction coupling** in _cardiac vs skeletal muscle_
_Cardiac Muscle_: surface membrane depolarization → opening of voltage-sensing DHPR channel → Ca2+ dependent activatin of RyR2 channel **(calcium induced calcium release)** _Skeletal Muscle_: surface membrane depolarization → conformational change in voltage-sensing DHPR channel → allosteric activaton of RyR channel **(mechanical coupling)**
34
What is Trousseau sign? What does it indicate?
It is when superficial venous thromboses may appear in one site, resolve, and occur in another site. This often indicates _visceral cancer._
35
The proximal ureter receives its blood supply from what artery? The distal ureter?
_Proximal_: renal artery _Distal_: superior vesical artery
36
Consequences of chronic gout
**Tophi** (uric acid crystals in the soft tissues/joints), or **renal failure** due to uric acid deposition in tubules
37
Role of muscarinic agonists, in glaucoma tx
Increase trabecular outflow of aqueous humor
38
What drugs are/can be given with L-DOPA to reduce their peripheral metabolism?
_Carbidopa_- a DOPA decarboxylase inhibitor _Entacapone_- a COMT inhibitor These both increase levadopa bioavailability to the brain
39
What are the cutaneous neurofibromas assc. with NF1, derived from? What is its embryological derivation?
They are comprised mostly of _Schwann cells_, which are embryologically derived from the _neural crest_
40
Describe the main roles for _IL-1 through IL-6_ when secreted by macrophages
**Hot T-Bone** st**EAK** IL-1: fever (_hot_) IL-2: stimulates _T_ cells IL-3: stimulates _bone_ marrow IL-4: stimulates Ig**_E_** production IL-5: stimulates Ig**_A_** production IL-6: stimulates a**_K_**ute-phase protein production
41
_Fibrate medications_ 1. Function 2. MOA 3. AE
1. Dec. LDL and TG; Inc. HDL 2. _MOA_: Upregulate LPL, resulting in inc. oxidation of fatty acids; also, inhibit **cholesterol 7a-hydroxylase**, which catalyzes rate-limiting step of bile acid synthesis 3. Reduced bile acid production results in dec. cholesterol solubility, favoring **cholesterol stone formation**
42
Order the class one subcategories based on sodium-channel-binding strength (as measured by use dependence)
1C \> 1A \> 1B (least use dependent)
43
What receptors on the juxtaglomerular cells are responsible for stimulating renin release? What drugs take advantage of this fact, to decrease BP?
B-1 receptors (which is why B-blockers can help dec. blood pressure)
44
_Nitroprusside_ 1. MOA 2. Effect 3. Indication
1. Short-acting balanced **venous and arterial vasodilator** 2. Decreases both preload and afterload, thus maintaining stroke volume 3. Indicated for hypertensive HF
45
How can severe chronic rheumatoid arthritis lead to spinal cord injury?
It can cause joint destruction with **verterbal malalignment (subluxation)**, particularly at C1. Extension of neck (particularly during ET placement), can worsen the subluxation, leading to _acute compression of the spinal cord and/or vertebral arteries_
46
_Osgood-Schlatter Dz_ 1. Presentation 2. Etiology
1. Focal pain + swelling at the tibial tuberosity 2. Repetitive quadriceps contractions in adolescent (quads are attached to the tibial tuberositt, via the patella)
47
Describe the Sx of Digoxin Toxicity | (what ion is often elevated?)
* Typically presents w/ cardiac arrhythmias and nonspecific GI, neuro and visual (color change) sx * Elevated K+ is another sign of digoxin toxicity (due to inhibition of Na-K-ATPase)
48
1. What is the cofactor of phenylalanine hydroxylase? 2. What would be the presentation for a deficiency in either of these enzymes?
1. **Tetrahydrobiopterin** 2. Accumulation of phenylalanine in body fluids and the CNS. Homozygous infants are normal at birth but gradually develop **severe intellectual disabilities and seizures**. May also have **hypopigmentation** of skin, hair, eyes, and catecholaminergic brain nuclei
49
_Maturity-onset Diabetes of the Young_ 1. Presentation 2. What is the main pathogenesis? Describe in detail 3. Potential Consequences
1. MIld, nonprogressive hyperglycemia that often worsens with pregnancy-induced insulin resistance 2. Often a result of _mutation of the **glucokinase gene**_. Glucokinase = glucose sensor. Mutation leads to dec. beta cell metabolism of glucose, less ATP formation, and dec. insulin secretion 3. Could lead to fetal growth retardation and sever hyperglycemia at birth
50
What are the (3) tx for C.diff and in what situation would you use each?
1. **Metronidazole** (Initial mild/moderate C. Diff tx) 2. **Vancomycin** (Severe or recurrent C. Diff) 3. **Fidaxomicin** (recurrent C. Diff and inc. risk of recurrence)
51
Intraventricular Hemorrhage 1. Most frequent occurance is in what population? 2. Pathology? 3. Presentation?
1. Usually in **infants** born before 32 weeks gestation or with a low birth weight 2. IVH in premies usually originates in **germinal matrix**, where neurons and glial cells migrate out during brain development 3. Clinically silent or present with altered level of conciousness, hypotonia, and decreased movement. **Symptoms of catstrophic bleeding are the same as those of increased ICP**.
52
In Kallmann syndrome, there is an absence of GnRH secretory neurons in the \_\_\_\_\_\_\_\_\_\_\_\_\_
hypothalamus
53
What is the affect of occluson of the anterior cerebral artery?
**Disruption of sensory and motor function of the contralateral leg and foot**, while sparing the contralateral arm/face
54
Presentation of _Meckel diverticulum_
Most often presents with **painless melena** (gastric tissue can cause ulceration leading to lower GI bleed). The diverticulum may also become inflamed and simulate presentation of acute appendicitis.
55
_Treacher-Collins syndrome_ 1. Path? 2. What is the effect?
1. Genetic disorder resulting in abnormal development of the first and second pharyngeal arches 2. _Result_: (1) Craniofacial abnormalities which often compromise airway and feeding; (2) absent or abnormal ossicles leading to profound conductive hearing loss
56
What is the role of transketolase and transaldolase? What cells can use them and for what purpose?
They carry out the **nonoxidative reactions of the HMP shunt** (Pentose Phosphate Pathway) _All cells_ can synthesize ribose from fructose-6-phosphate using the nonoxidative rxns.
57
Path of _mitochondrial myopathy_ What does muscle biopsy show?
Myopathy + Lactic Acidosis due to failure of oxidative phosphorylation. Muscle biopsy often show **"ragged red fibers"**
58
What is the composition of fetal hemoglobin?
a2y2 (alpha 2, gamma 2)
59
Chloramphenicol AE
This antibiotic can lead to both dose-dependent (reversible) **cytopenias** and dose-independent (irreversible) **aplastic anemia**.
60
Th1 vs Th2 What do they secrete? What do they recruit/activate? How are they activated? How are they inhibited?
Both are CD4+ Helper T-cells **_Th1_** 1. Secretes IFN-y 2. Activates macrophages and cytotoxic T cells 3. Activated by INF-y and IL-12 4. Inhibited by IL-4 and IL-10 (from Th2) **_Th2_** 1. Secretes IL-4, 5, 10, and 13 2. Recruits eosinophils for parasite defense and promotes IgE production by B cells 3. Activated by IL-4 4. Inhibited by IFN-y (from Th1 cells)
61
What percentage of vessel must be blocked to qualify as stable angina?
\> or equal to 75%
62
What tumor type(s) is the following tumor marker assc. with?: ## Footnote **HCG**
- Choriocarcinoma - Germ cell tumors
63
How can malignant, nonseminomatous **germ cell** testicular tumors cause hyperthyroidism?
They secrete very high levels of hCG, which can bind the **TSH receptor** (leading to _paraneoplastic hyperthyroidism_)
64
_Ataxia-telangiectasia_ 1. Inheritance 2. Path 3. Sx/Presentation (4)
1. Autosomal-recessive 2. Result of a defect in DNA-repair genes, making the genes _hypersensitive to ionizing radiation_ 3. Presents as child w/ (1) **cerebellar ataxia,** (2) oculocutanoeous telangiectasias, (3) repeated sinopulmonary infxn, (4) inc. incidence of malignancy
65
What does the _candidal antigen test_ assess?
The activity of T cell-mediated immunity via recruitment of _macrophages, and CD4+ and CD8+ T lymphocytes_
66
What is the best non-surgical tx for cholesterol gallstones?
**Hydrophilic bile acids** (eg ursodeoxycholic acid). These decrease biliary cholesterol secretion and increase biliary bile acid concentration, improving cholesterol solubulity.
67
What enzyme is responsible for the conversion of NE to Epi? What upregulates this enzyme?
Phenylethanolamine-N-methyltransferase (**PNMT**) It is upregulated by cortisol
68
What tumor type(s) is the following tumor marker assc. with?: ## Footnote **CA 19-9**
Pancreatic
69
_Diabetic Mononeuropathy_ 1. Usually involves which CN? 2. Pathology 3. Sx (3)
1. CN III is most commonly involved 2. Caused predominantly by _central ischemia_ 3. **_Sx_**: ptosis, "down and out" gaze, normal light/accomodation reflexes
70
_Buproprion_ 1. Indication? 2. What key side-effect does it _NOT_ have? 3. Contraindications/ Adverse Effects?
1. First-line tx for **major deppression disorder** 2. Does _not_ cause sexual dysfunction (unlike the SSRIs) 3. Can lead to _seizures_ so contraindicated in any patient with **seizure disorders** or prior dx of _bulimia/anorexia_
71
Describe the _Chloride Shift_
* Carbonic Anhydrase activity w/in erythrocytes forms bicarb from CO2/water * Many of the bicarb ions diffuse from the RBC to the plasma * To maintain electroneutrality, chloride ions diffuse into the RBC
72
Which cells found in the lungs, contain elastase? How do they keep it controlled?
**Macrophages** (control w/ Tissue Inhibitos of Metalloproteinases- _TIMPs_) and **Neutrophils** (control w/ serum _a1 antitrypsin_)
73
Bisphonates have a chemical structure similar to \_\_\_\_\_\_\_\_\_\_\_\_\_
Pyrophosphate
74
_Paroxysmal Supraventricular Tachycardia_ 1. Presentation 2. Tx 3. Potential SEs of tx (4)
1. Sudden onset palpitations 2. Tx w/ adenosine 3. _SE_: (1) Flushing, (2) chest burning (from bronchospasm), (3) hypotension, (4) AV block
75
What is the indication for drugs which act on H1 receptors vs H2 receptors
H1 = allergies H2 = acid (GERD, ZE, etc.) Both are histamine receptors
76
Tx of Giant cell (temporal) arteritis
Tx with high dose corticosteroids (to prevent blindness), and _then_ get a biopsy
77
What are the (4) stages associated with Lobar Pneumonia? Describe the exudate.
1. **Congestion** (first 24 hours): Vascular dilation; exudate contains bacteria 2. **Red hepatization** (days 2-3): erythrocytes, PMNs and fibrin in exudate 3. **Gray hepatization** (days 4-6): RBCs disintegrate. Exudate contains PMNs and fibrin 4. **Resolution**: Enzymatic digestion of exudate
78
What is the presentation for Hemolytic Uremic Syndrome? (3) What is the etiology?
1. Microangiopathic hemolytic anemia 2. Thrombocytopenia 3. Renal insufficiency Result of **0157:H7** E.coli, usually in undercooked ground beef
79
Describe the pathogenesis of _Polymyositus_
Over-expression of MHC-I on the sarcolemma leads CD8+ infiltration, and subsequent myocyte damage
80
What molecule does insulin promote activation of, in order to increase glycogen synthesis?
**Protein phosphatase**, via the PI3K pathway
81
The KRAS gene is a member of what gene subfamily?
Ras
82
1. Tetrahydrobiopterin (BH4), serves as cofactor for which enzymes? 2. What enzyme is responsible for forming BH4? 3. What effect would a deficiency in BH4 have?
1. Important cofactor for the Phenylalanine hydroxylase and Tyrosine hydroxylase 2. **Dihydropteridine reductase** is responsible for converting BH2 to BH4 3. Deficiency would lead to inc. phenylalanine (can be corrected by diet) and def in tyrosine conversion to its downstream products such as dopamine, NE, Epi, and serotonin (leading to neuro deterioration)
83
Describe **Pompe Disease** 1. Deficient enzyme? 2. Pathology 3. Presentation
1. Acid a-glucosidase 2. Acid a-glucosidase is responsible for breakdown of glycogen w/in lysosomes. Its absence leads to glycogen accumulation w/in liver and muscle lysosomes 3. _Presentation:_ **Pompe** trashes the **Pump** (heart, liver, and muscle) -- cardiomegally, hypertrophic cardiomyopathy, excercise intolerance, hypotonia, etc.
84
What (2) drugs are selective vasodilators of coronary vessels? How can they affect the heart _negatively_ during ischemic events?
**Adenosine and dipyridamole** They can lead to _coronary steal_ because they vasodilate everything in the heart. The vessels going to ischemic areas are already maximumly dilated so they only lose out on blood.
85
Describe the hemodynamic profile of aortic regurg (3 key differences from normal)
1. Higher pressure peaks (more blood in LV) 2. Loss of dichrotic notch 3. Steeper fall in aortic pressure
86
What are the _only_ cells within atherosclerotic plaque which are capable of synthesizing collagen isoforms and ECM?
Vascular Smooth Muscle Cells (VSMCs)
87
The valgus stress test indicates injury where?
Injury with the MCL (test ability to passively abduct knee)
88
What is the most common location for acoustic schwannomas?
Cerebellopontine angle
89
Transudate vs exudate
_Transudate_- plasma only. Due to hemodynamic changes _Exudate_- plasma + ions. Due to structural damage
90
Permissiveness
**When one hormone allows another to exert its maximal effect** (ex. cortisol allows NE to work even better although cortisol has no direct vascular effect itself)
91
What is the clearence mechanism for inhaled particles which are... 1. 10-15 um? 2. 2.5-10 um? 3. less than 2um?
1. 10-15 um: Trapped in the upper respiratory tract 2. 2.5 - 10um: mucociliary transport 3. \<2.5um: phagocytized
92
_Nocardiosis_ (Nocarda asteroides infection) 1. Key micro characteristics 2. Epidemiology (where is it, how does it get to people and who does it affect?) 3. Clinical features (3) 4. Tx
1. Gram positive rod (**beading/branching**); acid-fast; Aerobic 2. Endemic in soil; get disease from spore inhalation/ trauma inoculation; **immunocomprimised/elderly** 3. Pneumonia (similar to TB); CNS (abscess); Cutaneous involvement 4. _Tx_: Bactrim and surgical drainage of abscess
93
What is an appendage, in the context of the heart?
It is a small saclike structure that is particularly susceptible to thrombus formation
94
Presentation of Left-sided Colon CA
1. Constipation 2. Sx of Intestinal Obstruction (Left sided colon CA tends to infiltrate the intestinal wall and encircle the lumen)
95
1. Most common cause of _homocystinuria_ 2. What is the result of this defect? 3. How might this present clinically?
1. Homocystinuria is most commonly caused by a defect in _cystathionine sythase_ 2. The result is an inability to form cysteine from homocysteine. Cysteine then becomes essential and buildup of homocysteine leads to elevated methionine 3. May present as _premature thromboembolic events_ (atherosclerosis, acute coronary syndrome, etc.), because homocysteine is **prothrombotic**
96
Sensitivity vs Specificity
**Sensitivity** = true-positive rate (the probability that a test detects disease when a disease is present) **Specificity**= true-negative rate (Probability that a test indicates no disease when disease is absent)
97
Role of prostaglandin agonists in glaucoma
Increase uveoscleral outflow of aqueous humor ## Footnote **PREFERED TX FOR OPEN ANGLE GLAUCOMA**
98
Name the microscopic and macroscopic changes associated with Ischemic Brain Injury, during the following times after injury: * 12-24 hours * 24-72 hours * 3-7 days * 1-2 weeks * \>2 weeks
99
_Clopidogrel_ 1. MOA 2. Indication
_MOA_: Irreversibly blocks the P2Y component of ADP receptors on the platelet surface and prevents platelet aggregation _Indication_: Just as effective as aspirin for prevention of CV events and should be used if patient has aspirin allergy.
100
Formula for half life
Vd x .7/CL
101
_Diazepam_ 1. Indications (4) 2. SEs (1 most common) 3. Contraindications
1. **Indications**: (1) anxiolytic, (2) sedative-hypnotic, (3) anticonvulsant, (4) muscle relaxant 2. **SE**: Sedation 3. **Contraindications**: Don't give to patients on other CNS depressants (ex. chlorpheniramine)
102
_Phenoxybenzamine_ 1. MOA 2. Indication 3. Effect
1. _Irreversible a1 and a2 antagonist_ that effectively reduces the arterial vasoconstriction induced by NE. 2. **Pheochromocytoma** 3. Because it is irreversible, even very high concentrations of NE (like those seen in pheochromocytoma) cannot overcome its effects
103
_Polymyositus_ Presentation
**Symmetrical** proximal _muscle weakness_
104
What is the relationship between CO2 and cerebral perfusion?
CO2 is a potent cerebral vasodilator and therefore a drop in CO2 (due to hyperventilation and subsequent hypocapnia for example), causes a linear decrease in cerebral perfusion.
105
_Niacin (nicotinic acid)_ 1. Indication 2. Adverse Effects 3. What is the mechanism for the adverse effects and how can they be prevented?
1. Used in the tx of hyperlipidemia. Effective in raising HDL cholesterol levels, and lowering LDLs and TGs 2. SEs include cutaneous flushing, warmth and itching 3. SEs are mediated by release of prostaglandins and can therefore be prevented by aspirin.
106
_Kawasaki Disease_ 1. What type of disease is this? 2. Presentation 3. Potential consequence 4. Tx
1. Medium-vessel vasculitis 2. **CRASH** and **burn** * **_C_**onjuctival injection, **_R_**ash, **_A_**denopathy, **_S_**trawberry tongue, **_H_**and/foot changes (edema and erythema) and _fever_ 3. Risk of **coronary artery aneurysm** 4. Tx w/ *IV and aspirin*
107
_Primary Myelofibrosis_ 1. Presentation 2. Mutation
1. **Bone marrow fibrosis,** severe fatigue, _splenomegaly_ (often causing early satiety/abdominal discomfort), hepatomegaly, and anemia 2. Mutation: JAK2 (of the JAK-STAT signaling pathway-- tyrosine kinase)
108
_Paradoxical Embolism_ 1. Pathology 2. Dx
1. When a thrombus from the venous system crosses into arterial circulation (as oppose to the pulmonary) via an abnormal connection between the right and left cardiac chambers 2. May see **fixed splitting of S2** if a shunt is present
109
Inheritance of CF
Autosomal recessive
110
What is the negative predictive value and how do we calculate it?
NPV represents the probability of not having a disease given a negative test result. NPV = true negatives/total negative tests
111
Following cleavage of pro-insulin, what is the fate of insulin and C-peptide?
They are both **stored** in islet cell secretory granules unti they are secreted in equimolar amounts
112
_ASD_ 1. What is the usual pathology? 2. What other cardiac condition is often also present? 3. Dx 4. Potential consequence 5. What genetic disease is also associated?
1. Failure of the _endocardial cushions_ of the atrioventricular canal to fuse completely during embryonic development can lead to a lower interatrial septum defect 2. **Malformation of mitral valve** leading to mitral regurg is often present 3. Wide, fixed splitting of the second heart sound (S2) 4. Pulmonary HTN 5. _Down syndrome_
113
What is the role of NAD+ in glycolysis? How is NAD+ regenerated after reduction to NADH?
NAD+ is needed to convert G3P to 1,3-BPG NADH then transfers electrons to pyruvate to form lactate and regenerate NAD+
114
Flattening of deltoid muscle + acromial prominence, suggests what type of injury?
Anterior humerous dislocation (most common type of shoulder dislocation)
115
What are the (3) options for Pyruvate metabolism and how do they occur?
1. **Lactic acid** (via lactate dehydrogenase in anaerobic conditions) 2. **Acetyl-CoA** (via pyruvate dehydrogenase in aerobic conditions) 3. **Oxaloacetate** (via pyruvate carboxylase)
116
Mycobacterium Avium Complex 1. What is it? 2. Risk factors? 3. Normal presentation? 4. How do you differentiate MAC from disseminated TB?
1. An infection caused by nontuberculous mycobacteria M avium and M intracellulare. 2. Risk factor: CD4 count 3. Presents w/ nonspecific symptoms- fever, weight loss, and diarrhea in HIV patient 4. Differs from TB due to _hepatosplenomegally_, _anemia_, and _elevated alk phosp/ LDH_
117
How does hypothermia lead to a left shift of the O2-dissociation curve?
Decreased temps help stabilize the bonds between O2 and hemoglobin
118
Formula for Loading dose
Vd x Cpss (steady state plasma concentration)/ [bioavailability fraction]
119
What is the most common CFTR mutation associated w/ CF?
**F508 mutation**. This causes impaired _postranslational processing_ (improper folding and glycosylation) of the CFTR. As a result, the abnomormal protein is targeted for degradation.
120
What key interleukin/cytokines are responsible for converting a helper T-cell into a Th1 cell? Th2 cell? Th17 cell?
**Helper T to...** Th1 (via IL-12) Th2 (via IL-4) Th17 (via TGF-B and IL-6)
121
*Embryologically*, the dorsal pancreatic bud forms what? The ventral pancreatic bud?
1. _Dorsal_: majority of the pancreatic tissue (body, tail, and most of the head) 2. _Ventral_: precursor of the uncinate process, inferior/posterior portion of the head, and the major pancreatic duct
122
What effect do Ach and adenosine have on cardiac pacemaker cells?
They reduce the rate of spontaneous depolarization of cardiac pacemaker cells by prolonging phase 4
123
_Chronic lung transplant rejection_ 1. Pathology/ histopathology 2. Presentation 3. Dx
1. Lymphocytix inflammation + epithelial destruction in **small airways**. Exudate and granulation tissue later found ultimately leading to fibrosis. Leads to obstructive lung disease **bronchiolitis obliterans**. 2. Dyspnea + dry cough 3. _Dx_: Spirometry shows airflow limitation w/ a drop in FEV1 and FEV1/FVC
124
_Cutaneous, strawberry-type capillary hemangiomas_ 1. Pathology 2. Prognosis
1. Benign congenital tumor of unencapsulated aggregates of closely packed, thin-walled capillaries 2. Initial growth followed by regression (excellent prognosis)
125
_Enteropeptidase_ 1. What secretes it? 2. What does it do? 3. What does its deficiency lead to?
1. Comes from the jejunal brush border 2. Activates trypsin from trypsinogen (needed for peptide breakdown and activation of other pancreatic enzymes) 3. Deficiency impairs protein and fat absorption
126
What are the main causes of pulsus paradoxus. Name one option for tx
**Pericardial dz**, asthma and COPD Beta-adrenergic agonisists are useful for the asthma/COPD etiologies
127
In an MI, when does loss of cardiomyocyte contractility occur?
Within the first _60 secs (1 minute)_ after the onset of total ischemia.
128
Tx of acute gout vs chronic gout
_acute_: NSAIDs (1st line), glucocorticoids, or colchicine (GI issues due to inhibition of microtubule formation) _chronic_: Xanthine oxidase inhibitors
129
Clostridium tetani blocks the release of what?
The inhibitors _Glycine_ and _GABA_
130
Prevention of Tumor Lysis Syndrome
Prevent w/ hydration + use of hypouricemic agents such as allopurinol or rasburicase
131
**Tension Pneumothorax** 1. Pathology? 2. Clinical presentation? 3. Dx? 4. Tx?
1. _Pathology_: As an increasing volume of air accumulates w/in the pleural space, the lungs and mediastinum shift to the opposite side and this pressure drops systemic venous return to the heart, leading to decreased cardiac output 2. _Presentation_: Tachycardia, hypotension, tachypnea, hypoxemia 3. _Dx_: Hx, mediastinal shift on CXR, absent breath sounds and hyperresonance to percussion on affected side 4. _Tx_: Emergency needle thoracostomy or chest tube
132
_Describe Cori Dz_ 1. Deficient enzyme 2. Which other GSD is it similar to?
1. Debranching enzyme 2. It is a milder version of Von Gierke (type I)
133
_Neurofibromatosis Type 1 (NF-1)_ 1. Sx (4) 2. Inheritance 3. What type of cells are the main compent of cutaneous neurofibromas?
1. (1) Cafe-au-lait spots, (2) Neurofibromas (short, sessile or pedunculated lesions), (3) Lisch nodules (pigmentated hamartomas of the iris), (4) pseudoarthritis 2. Autosomal-dominate inheritance 3. Schwann cells are the main component
134
How is carotid sinus massage useful for termination of paroxysmal SVT?
It leads to increased parasympathetic tone causing temporary inhibition of the SA node and the prolongation of AV node refractory period
135
What specifc joints are most commonly fused in Ankylosing Spondylitis? What are potential complications of AS? (3 systems)
**Fusion** of the sacroiliac and apophyseal joints of the spine are most commonly affected _Complications_: 1. Respiratory: limited chest wall expansion leading to hypoventilation 2. CV: \*\*\***most common!!!**-- aortitis, leading to dilation of the aortic ring and aortic insufficiency 3. Uveitis (blurred vision, photophobia, conjunctival erythema, etc.)
136
What type of RNA can be infective, what type cannot, and why?
1. Purified single-stranded positive-sense RNA can be infectious 2. Single-stranded negative sense or double stranded RNA cannot be infectious For a purified RNA molecule to induce viral protein synthesis in a host cell, it must be able to act **DIRECTLY AS mRNA USING HOST INTRACELLULAR MACHINERY** for translation.
137
How does the effect of Rheumatic Heart Disease on the mitral valve vary as the patient's age changes?
_First few decades of life_: MR _Middle-aged_: MS (most common cause of MS) _Elders_: Mixed mitral disease (S and R)
138
(Leukocyte Accumulation:) What receptors are involved in: 1. Rolling? 2. Tight Adhesion/Crawling? 3. Transmigration?
1. Rolling: Selectin 2. Adhesion/crawling: ICAM-1 and integrins 3. Transmigration: PECAM-1 and integrins
139
When given with a Statin, what drug is most likely to lead to myopathy or even rhabdomyolosis? Why?
Fibrates (like Gemfibrozil). They impair the hepatic clearance of Statins leading to excessive blood levels.
140
X-linked agammaglobulinemia Pathogenesis? Diagnostics? Presentation?
* A mutation in Bruton tyrosine kinase gene causes _failure of BM pre-B cells to mature_. * These patients have: 1. low B-cells in peripheral blood (CD19+, CD20+, CD21+) 2. pan-hypogammaglobulinemia (low Ig's) 3. Increased risk of infection w/ encapsulated bugs and certain viruses and parasites
141
_Allergic bronchopulmonary aspergillosis (ABPA)_ 1. History commonly associated (2)? 2. Chest imaging? 3. Dx?
1. **_A_**sthma and CF 2. Recurrent infiltrates and **_B_**ronchiectasis 3. Eosinophilia; **_P_**ositive skin test and Ig for **_A_**spergillus; Elevated IgE
142
Key presentation for cyanide poisoning
1. _Presentation_: Reddish skin discoloration, tachypnea, HA. Lab studies indicate severe lactic acidosis and dec. venous-arterial PO2 gradient
143
What are the key associations with Multiple Myeloma? (7)
**CRAB** (hyperCalcemia, Renal involvement, Anemia, Bone lytic lesions/Back Pain) + Primary amyloidosis, Rouleaux formation, and numerous plasma cells w/in bone marrow sample
144
_Familial Hypocalciuric Hypercalcemia (FHH)_ What is it? Describe the associated receptor?
A _benign_ autosomal dominant disorder casued by a defective calcium-sensing receptor (G-protein coupled), which is suppose to regulate PTH secretion. Defect raises the Ca2+ threshold
145
Most common site of injury during a traumatic aortic rupture
Aortic isthmus
146
Describe the pathogenesis of Sarcoidosis
Granuloma formation as a manifestation of cell-mediated immunity driven by _products_ of _Th1 type CD4 helper T-cells_, particularly _IL-2 and IFN-y_, which stimulate Th1 type **cell proliferation** and **macrophage activation**, respectively.
147
Name the (4) nerves/ vessels which enter the orbit via the _superior orbital fissure_
**OSATO** 1. **O**culomotor nerve 2. **S**uperior Ophthalamic vein 3. **A**bducens nerve 4. **T**rochlear nerve 5. **O**pthalmic nerve (CN V1) branches
148
_Right Ventricular MI_ 1. Presentation 2. Pathology 3. What does the hemodynamic assessment reveal? (CVP? Wedge pressure? CO?)
1. Presents with hypotension, elevated JV pressure, _clear lungs_ 2. Most often occurs in the setting of **acute inferior wall MI** 3. CVP= inc.; Wedge pressure = dec.; CO = dec.
149
What is the most common CV manifestation associated with SLE?
Pericarditis
150
Hypoglycemia with an elevated insulin and _low C-peptide level_, suggests what? _High C-peptide level_?
**Low**: exogenous insulin injection **High**: insulin secretagogue or insulin-secreting tumor
151
When amniocentesis is performed to check phospholipids, what are we really checking for?
Fetal lung maturity Phospholipids (such as lecithin aka phosphatidylcholine) are a majory component of pulmonary surfactant
152
Structural cardiac changes due to aging are generally not prominent before the age of...
65y/o
153
Describe the relationship between Bile acid-binding resins and Statins
BAB resins (ex. cholestyramine) Inc. stimulation of HMg-CoA Reductase, which will need to be compensated by giving a Statin. Synergistic effects with the statin further reduce plasma LDL level.
154
What is the effect of G6PD deficiency? What other enzyme deficiency might paint a similar picture?
_G6PD_ deficiency is a defect in the HMP shunt (aka the Pentose Phosphate Pathway), which leads to impairment of glutathione reduction due to failure to produce NADPH **Glutathione reductase deficiency** causes a similar clinical picture
155
What are the structural differences between MHC Class I and Class II?
**MHC Class I**: Heavy chain and B2-microglobulin **MHC Class II**: Alpha and beta polypeptide chains
156
What is the **most common** _congenital heart lesion_? 1. Dx/ Presentation? 2. Effect on blood oxygenation 3. Prognosis?
_Ventricular Septal Defects_ 1. Small VSDs have a loud, "blowing", holosystolic **murmur** at the mid/lower left sternal border (louder with handgrip). Murmur is usually inaudible _until_ 4-10 days when pulmonary vascular resistance declines enabling left-to-right shunt. 2. Right ventriclular blood has increased O2 content 3. Most are clinically insignficant and close spontaneously
157
New-onset odynophagia in the setting of chronic GERD usually indicates what? Dx?
Erosive esophagitis and the formation of an ulcer Dx via upper endoscopy
158
_Jervell and Lange-Nielsen Syndrome_ 1. Pathology 2. Clinical presentation 3. Mode of inheritance 4. What other disease is this similar to?
1. Congenital **long-QT syndrome**; thought to result from mutations in a K+ channel protein 2. Syncopal episodes; sudden cardiac death (*torsades de pointes*); 3. _Autosomal recessive condition_ 4. Similar to Romano-Ward syndrome
159
_Dihydropyridine CCBs (Anti-hypertensives)_ 1. Main examples 2. Indications 3. SEs
1. *Amlodipine and Nifedipine* 2. Effective for monotherapy or in combination with other agents for Tx of HTN 3. **_Peripheral Edema_** and _Dizziness/ Lightheadedness_
160
Trousseau syndrome
Migratory superficial thrombophlebitis assc. with visceral CA
161
What is pancreas divisum?
Failure of the dorsal and ventral pancreatic buds to fuse
162
Interstitial lung disease has what effect on lung volumes? Lung elastic recoil? How does this affect expiratory flow rates?
Decreased lung volumes Increased lung elastic recoil The increased recoil leads to _radial traction_ (outward pulling) of airways, leading to increased corrected expiratory flow rates.
163
What is the role of _Small nuclear RNA_ (snRNA)? What is the name of autoantibodies which work against the function of snRNA and what dz are they associated with?
snRNA is synthesized by RNA pol II and complexes w/ specific proteins to form small nuclear ribonucleoproteins (snRNPs). **These snRNPs are essential components fo the spliceosome and remove introns from to form mature mRNA.** Autoantibodies directed against snRNPs are called anti-Smith antibodies, and are assc. with SLE.
164
Type I Hypersensitivity Explain the mechanism. What mediates these reactions?
Mediated by the interaction of allergen w/ preexisting IgE bound to basophils and mast cells. Cross-linking occurs allowing for degranulation and immediate allergy signs.
165
Which **ligament** is most commonly involved in a _lateral ankle sprain_?
Anterior **talofibular ligament**
166
Main potential AE of neonatal oxygen therapy?
Retinal damage
167
Name the potential sequelae of Obstructive Sleep Apnea (3)
Systemic and Pulmonary HTN, and right heart failure
168
What type of gating does the CFTR channel have?
ATP-gated
169
_Cauda Equina Syndrome_ Presentation? Specific areas damaged?
Saddle anesthesia and loss of the anocutaneous reflex, assc. with damage to the S2 through S4 nerve roots
170
_Cystic Medial Degeneration_ 1. Characteristics 2. What does it predispose you for? 3. Who is it common in?
1. _Myxomatous_ (weakening of connective tissue) changes with pooling of proteoglycans in the _media layer of large arteries_ 2. **Aortic dissections/ Aneurysms** 3. Younger patients with _Marfan Syndrome_
171
_Walking pneumonia_ 1. What bug causes it? 2. Dx? 3. What type of medium is required for this bug to grow? 4. Sx?
1. **Mycoplasma pneumoniae** 2. CXR looks much worse than the clinical appearance of the patient indicates 3. Cholesterol medium 4. Nagging unproductive cough, low-grade fever, and malaise
172
Risks of secondhand smoke exposure (7)
1. SIDS 2. Low birth weight 3. Dental caries 4. Middle ear disease 5. Asthma 6. LRTI 7. Decreased GFR
173
_Ankylosing Spondylitis_ 1. What is it? 2. Presentation
1. Chronic inflammatory condition assc. wtih HLA-B27 serotype 2. Low back pain + stiffness in a young man. **Fusion** of axial joints (hence bamboo spine)
174
_Adenocarcinoma In-situ (of the lung)_ 1. Pathology 2. Microscopy 3. Presentation 4. Imaging
1. Arises from alveolar epithelium at the periphery of lung 2. _Microscopy_ shows well-differentiated, dysplastic **columnar cells** w/ or w/o mucin 3. Similar to other lung cancers (SOB, cough, hemoptysis) 4. Discrete mass or pneumonia-like consolidation on imaging
175
What is the most common condition predisposing a patient to infective endocarditis in... 1. Adults in wealthier nations? 2. Poorer nations/ children?
1. Mitral valve prolapse/ mechanical valves 2. Rheumatic fever
176
_Ramelteon_ 1. MOA 2. Indications 3. SEs
1. **MOA**: melatonin agonist 2. Initial insomnia treatment that demonstrates _high safety and efficacy_ in **older adults** 3. Very few SEs and no dosage adjustment needed
177
In what conditions do we see Cheyne-Stokes breathing? (2)
1. CHF 2. Neurologic disease
178
How do OC treat hirsutism?
They suppress pituitary LH secretion and subsequently decrease ovarian androgen production
179
Neural Tube Defects 1. Pathophysiology 2. Presentation 3. Dx
1. NTDs occur due to failure of fusion of the neural tube during the 4th week of development. This leaves an opening between the neurotube and the amniotic cavity. 2. Can present with encephalocele (herniation), anencephaly (no brain), or the spina bifadas 3. Leakage of alpha-fetpoprotein and AchE occurs, allowing for prenatal diagnosis
180
Idiopathic Pulmonary Artery Hypertension Pathogenesis/pathophys? Presentation? Treatment?
_Pathogenesis_: If familial form, result of inactivation of the pro-apoptotic BMPR2 gene. Results in increased **endothelial and smooth muscle cell proliferation and vascular remodeling** _Presentation_: Dyspnea, excercise intolerance in women 20-40 _Treatment_: Lung transplant and **bosentan** (endothelin-receptor antagonist) in the meantime
181
Formula for Maintenance dose
Cpss (steady-state plasma concentration) x CL/[bioavailability fraction]
182
What is the main difference in effect between unfractioned heparin and LMWH?
Both of them can bid to AT3 to increase its activity against factor Xa. _Only unfractionated heparin_ is able to bind to **both AT3 and thrombin**, thereby allowing antithrombin to inactivate thrombin.
183
PPO vs HMO vs POS (Point of Service) 1. Compare the monthly premiums 2. Compare copayments and deductibles 3. Is a PCP referral required for specialist visits? 4. Size of network 5. May go outside network?
184
_Statins_ 1. Indication 2. MOA 3. Side effects 4. What sort of things can help lead to adverse effects from Statins
1. _Indications_: Tx of hypercholesterolemia 2. Inhibit **HMG-CoA reductase**, thus blocking hepatic cholesterol synthesis. This forces the liver to _increase surface expression of LDL receptors_, thus pulling LDL from circulation 3. _SE_: Myopathy, rhabdomyolysis, and hepatoxicity 4. Drugs that interfere w/ statin metabolization, particuarly via cytochrome p450 enzymes (ex. fibrates)
185
_Abetalipoproteinemia_ 1. What is it? 2. Consequence? 3. Describe the histo
1. An inherited **inability to synthesize apoB**, which is critical for chylomicrons and VLDL 2. As a result, _lipids absorbed by the small intestine cannot be transported into the blood_ and accumulate in the intestinal epithelium. 3. Enterocytes w/ clear/ foamy cytoplasm
186
What is the effect of excercise on LV EDP? On systemic vascular resistance?
_LV EDP_ is increased due to the increased return _Systemic vascular resistance_ decreases due to large levels of vasodilation in muscles
187
_Isoproterenol_ 1. MOA 2. Effect
1. Non-selective Beta-_agonist_ 2. Increases myocardial contractility and decreases systemic vascular resistance
188
Isoproterenol | (MOA)
Nonselective B-adrenergicc **_agonist_** (leads to inc. vasodilation, inc. cardiac rate, and inc. contractility)
189
What are the (3) main dopaminergic systems? What are their functions? What diseases are they associated with?
190
Presentation of Right-Sided Colon CA
1. Occult bleeding 2. Sx of iron deficient anemia
191
_Myasthenia Gravis_ 1. Sx 2. Disease associated with 3. Pathogenesis 4. Tensilin test result 5. Nerve stimulation studies
1. **Sx**: Weakenss that is worse at the end of the day/ with exertion; extraocular muscles are affected first (ptosis/diplopia) 2. **Assc. with** risk of _thymoma_ 3. **Path**: antibodies against Ach receptors 4. Improvement/ resolution w/ tensilon test 5. Decremental response to nerve stim. test
192
Length constant
A measure of how far along an axon an electrical impulse can propagate This is _increased_ by **myelination**
193
Name the (2) classes of dopamine agonists (and examples). What dz are they most often used for?
1. **Ergot compounds**- Bromocriptine 2. **Nonergot compounds**- Pramipexole, ropinirole
194
*Histoplasma Capsulatum* 1. What is it/where is it found? 2. How is it transmitted? 3. Pathology? 4. Presentation? 5. Dx?
1. A mold found in soils of **Mississippi and the Ohio River Valley** 2. It is transmitted by the respiratory route when bird/rat droppings containing fungal spores are inhaled 3. _Pathology_: In the lungs, the fungus is ingested by macrophages and a granuloma forms, similar to TB 4. _Presentation_: Asymptomatic if immunocompetent. May develop acute pulmonary disease, or develop chronic pulmonary histoplasmosis (**looks like TB**). May see lymphadenopathy and hepatosplenomegally 5. _Dx._: **small oval bodies w/in macrophages** 6.
195
What type of drug is ipratropium and what type of bronchoconstriction is affected by it?
It is an antimuscarinic agent Only reverses vagally-mediated bronchoconstriction
196
**Varicocele** 1. Pathophysiology? 2. Presentation?
1. Increased pressure in the left gonadal vein resulting in valve leaflet failure and varices of the testicular pampiniform plexus 2. Presents with flank/abdominal pain and gross or microscopic hematuria
197
_Bile acid-binding resins_ 1. Give one major example 2. MOA
1. Cholestyramine 2. Binds bile acids, forcing the liver to inc. uptake of LDL in order to create more bile.
198
At the FRC, the airway pressure is? alveolar pressure is? Intrapleural pressure is?
Airway pressure = 0 Alveolar pressure = 0 Intrapleural pressure = Negative, with a value of -5cm H2O (prevents pneumothorax)
199
1. A subset of patients stricken by influenze got on to develop secondary... 2. What types of patients are these? 3. What are the most common causes for this secondary disease state?
1. ...Bacterial pneumonia 2. Elderly 3. S. Pneumo, S. Aureus, H. Flu
200
_Maple Syrup Urine Disease (MSUD)_ 1. Pathology 2. Presentation 3. Treatment 4.
1. _Path_: defect in *a-keto dehydrogenase*, leading to inability to degrade branched chain amino acids (**leucine, isoleucine, valine**) beyond deaminated a-keto acid state 2. _Sx_: Dystonia; poor feeding; maple syrup scent urine in first few days of life 3. _Tx_: *Dietary restriction* of branched-chain AA's
201
_Hartnup Dz_ 1. Path 2. Sx 3. Possible Consequences 4. Tx
1. Defective intestinal and renal tubular absorption of _dietary tryptophan._ This can result in **Niacin** deficiency, as Niacin is synthesized from tryptophan 2. Sx: Often asymptomatic but may result in photosensitvity or pellegra-like skin rashes 3. Tx w/ nicotinic acid/ nicotinamide and a high-protein diet
202
Tricuspid Valve Endocarditis 1. Most common bug associated? 2. Usual patient population? 3. Potential complications?
1. Staph aureus is #1. Pseudomonas is #2 2. IV drug users 3. These patients can develop multiple septic emboli in lungs. Resulting pulmonary infarcts will be hemorrhagic due to dual blood supply.
203
What are the key effects of uncontrolled gestational diabetes on the fetus?
1. Beta cell hyperplasia due to increased trans-placental glucose delivery to the fetus (leads to **TRANSIENT** hypoglycemia at birth) 2. Fetal macrosomia (large size at birth!)
204
When is _anovulation_ most common?
In the first several years after menarche (immature H-P-Ovarian axis), and in menopause.
205
_Blastomyces dermatitidis_ 1. Endemic to where? 2. Characterisitics? 3. Presentation?
1. _Endemic_ to southeastern US (east of mississippi river) 2. _Characteristics_: Dimorphic fungus which is a large yeast (in the human body) with a single, **broad-based bud**. 3. Infxn follows inhalation and may present as a _lung infection_ (with GRANULOMA) or cause _flu-like illness/pneumonia_ in the *immunocompetent*. Can present as systemic disease in *immunocomprimised patients.*
206
What is the main side effect which limits the long-term efficacy of arteriolar vasodilators. Give (2) main examples of these drugs
Ex: Hydralazine and minoxidil There can be reflex sympathetic stimulation (leading to **inc. HR**, contractility, and CO) and also stimulation of the RAA system leading to **sodium and fluid retention/edema**
207
What are the afferent and efferent limbs leading to/away the carotid bodies?
_Afferent_: **G****lossopharyngeal nerve** _Efferent_: **Vagus nerve**
208
Tetralogy of Fallot Pathophysiology and clinical presentation
**VOIR**: 1. VSD 2. Overiding aorta 3. Infundibular pulmonary stenosis 4. RVH _Presentation_: Cyanosis; Improvement w/ squatting (increases SVR)
209
What types of procedures are associated with enterococcus endocarditis?
Genitourinary instrumentation or catheterization
210
Injury to the _lower trunk of the brachial plexus_ 1. What mechanism of injury which causes this? 2. Consequence of this injury?
1. Result of **sudden upward jerking** of the arm at the shoulder 2. May causes paralysis of all **intrinsic hand muscles (Klumpke's palsy)** w/ sparing to the extensors, leading to **total claw hand deformity**. Can also see sensory loss and weakness.
211
Blood flow is directly proportional to the vessel radius raised to what power?
The **_4th_** power
212
Pulmonary Embolism 1. Presentation 2. Lab data (blood gases?acidosis or alkalosis? 3. Other diagnostic tests
1. Acute-onset dyspnea, calf-swelling (indicative of DVT), obesity, Hx of prolonged immobility 2. Hypoxemia and respiratory alkalosis 3. CT pulm angiography is image testing of choice
213
Name the (5) regions of the secondary structure of tRNA
1. **Acceptor stem** (mediates corrct tRNA recognition) 2. **3' CCA tail** (used as recognition sequence by proteins) 3. **D loop** (facilitates correct tRNA recognition) 4. **Anticodon loop** (used by the ribosome complex to select the right tRNA) 5. **T loop** (facilitates binding of tRNA to ribosomes)
214
Describe the functions of each of the (3) domains of G protein-coupled receptors. Describe composition of the transmembrane domain in detail.
1. _Extracellular domain_- responsible for ligand binding 2. _Intracellular domain_- coupled with heterotrimeric G proteins 3. _Transmembrane domain_- composed of **nonpolar, hydrophobic amino acids** (alanine, valine, leucine, etc.), arranged in an **alpha-helical fashion**. Serve to **anchor** the proteinto the **phospholipid bilayer.** May also play role in cellular signaling and transport.
215
What dz is associated with incorrect assembly of snRNPs? What is the specific mutation associated?
**Spinal Muscular Atrophy** (delayed motor development + flaccid paralysis) SMN1 gene
216
Thin (actin) filaments of the I band are bound to structural proteins, where? Thick (myosin) filaments?
Thin: Z-line Thick: M-line
217
What is the most superficial layer in which you can appreciate an absence of ganglion cells in Hirschsprung dz?
The submucosa of the narrowed area
218
The anterior cerebral arteries supply which portions of the brain? (2) What is the affect that occlusion can have?
1. They supply the medial portions of the 2 hemispheres (frontal and parietal) 2. Occlusion can cause contralateral motor and sensory deficits of the lower extremities, behavorial changes, and urinary incontinence
219
Name the a-ketoglutarate dehydrogenase co-enzymes which may be defective in Maple Syrup Urine Dz (MSUD)
_**T**ender **L**oving **C**are **F**or **N**ancy_ **T**hiamine pyrophosphate **L**ipoate **C**oenzyme A **F**AD **N**AD
220
Why is raloxifene usually a better choice than tamoxifen for tx of osteoporosis in women?
Unlike tamoxifen, raloxifene has does not have agonist activity in the uterus (which inc. risk of endometrial hyperplasia/CA)
221
What is the most common location on the aorta for a traumatic aortic rupture (blunt aortic injury)?
The **aortic _isthmus_** (located just past the aortic arch)
222
_Niemann-Pick Dz_ 1. What type of dz is this? 2. Deficient enzyme 3. Inheritance 4. Presentation
1. LSD 2. **Sphingomyelinase** 3. AR (common in Ashkenazi Jew population) 4. Sphingomyelin accumulation, leading to _hepatosplenomegally_ , "Cherry-red" spot on macula, and _progressive neurodegeneration_
223
_Hormone Sensitive Lipase (HSL)_ 1. Where is it found? 2. What is its function?
1. Found in adipose tissue 2. Functions to **drive the breakdown of stored triglycerides** into free fatty acids and glycerol. During times of starvation, this enzyme _provides substrates for hepatic gluconeogenesis and ketone body formation_
224
_McArdle dz_ 1. Deficient enzyme 2. Path 3. Presentation
1. Skeletal muscle glycogen phosphorylase 2. Muscles can't breakdown glycogen in muscle (McArdle = Muscle) 3. Present with painful muscle cramps, myoglobinuria w/ strenuous excercise, and arhytmia from electrolye abnormalities.
225
_Kartagener_ _Syndrome_ 1. Pathology 2. Presentation (3)
1. Form of primary ciliary dyskinesia, that is autosomal recessive in nature 2. _Presentation_ * Recurrent respiratory infections (e.g., sinusitis, bronchiectasis) * Situs inversus (reverse organ positioning) * Infertility
226
Describe the work that _activated Ras_ does.
It begins a phosphorylation cascare that results in the activation of **mitogen-activated protein kinase**, which enters the nucleus to influence gene transcription.
227
SE profile for _ACE-Inhibitors_ (2)
1. Bradykinin induced cough and angioedema if there is accumulation 2. First dose hypotension due to volume depletion
228
Legionella pneumophila 1. Description of bug 2. Symptoms 3. Dx info 4. Pathogenesis 5. Tx
1. Facultative intracellular gram-negative bacillus 2. _Symptoms_- high fever, elevated transaminases, cough, confusion, and diarrhea, 3. _Dx_- Hyponatremia and sputum gram stain showing many neutrophils but few to no organisms 4. Passed from contaminated water 5. Respiratory fluroquinolones or newer macrolides
229
Length constant | (How is it affected by myelination?)
A measure of how far along an axon an electrical impulse can propagate. Myelination increases this constant.
230
Name the genes involved in each of the (3) steps of the "adenoma-to-carcinoma sequence"
1. **Progression from normal mucosa to a small polyp**: mutation of the _APC tumor suppressor gene_ 2. **Inc. in the size of the polyps**: mutation of the _K-ras protoncogene_ 3. **Malignant transformation**: requires mutation of *both* _p53_ and _DCC_ (Deleted in Colorectal Carcinoma)
231
What is rT3 and what is it created from?
An inactive form of T3, that is generated almost entirely from peripheral conversion of T4
232
_Pure Red Cell Aplasia_ 1. What is it? 2. What dz's is it associated with?
1. Rare form of marrow failure that is characterized by severe hypoplasia of marrow erythroid elements in the setting of normal granulopoiesis and thrombopoiesis 2. Assc. with **Thymoma**, lymphocytic leukemias and Parvovirus B19
233
_Vitamin E deficiency_ 1. Who does it occur in? 2. What is defiency in this vitamin associated with? 3. Clinical manifestations
1. Can occur in individuals with fat malabsorption 2. Assc. w/ inc. susceptibility of the **neuronal** and **erythrocyte** membranes to oxidative stress 3. _Clinical manifestations_: ataxia, impaired proprioception and vibratory sensation, and hemolytic anemia
234
Why might patients with signficant renal dysfunction demonstrate prolonged bleeding time?
Accumulation of uremic toxins impair platelet aggregation and adhesion (no effect on platelet count, PT or aPTT)
235
Other than the small joints of the hand, what other joints/bones are often affected by RA?
The **cervical spine**
236
Function of the _Posterior Cruciate Ligament_ How is its integrity tested?
Prevents posterior displacement of the tibia relative to the femur. Tested via the posterior draw test.
237
_Pulmonary Fibrosis_ 1. Microscopic view 2. Clinical presentation 3. Dx 4. What disease can help cause it?
1. _Microscopic_: Progresive fibrosis an lead to cystically-dilated bronchioles that later coalesce to from "honeycomb" appearance 2. Presents with **gradual-onset dyspnea**, first w/ exertion and then eventually even at rest 3. Physical exam can show _end-inspiratory crackles_ and _PFTs show restriction_ (decreased FEV1 and FVC w/ normal ratio)
238
Why do patients with _Antiphospholipid antibody syndrome_ often get false positive syphillus exams?
Presence of anticardiolipin antibody (which is tested for in Treponema pallidum, but is also present in this dz)
239
Almost 100% of cases of Hypertrophic Cardiomyopathy result from mutations in genes encoding what?
Beta-myosin heavy chain | (A cardiac sarcomere protein)
240
Why do you normally do cardiac catherization via the femoral artery, _below_ the inguinal ligament and not above?
There is signficant risk for a **_retroperitoneal hemorrhage_** if you do it above the inguinal
241
_Acute bacterial parotitis_ 1. Occurs most commonly in who? 2. Bacteria most commonly involved? 3. Dx
1. Common in elderly, _postoperative patients_ who are **intubated or dehydrated** 2. *S. Aureus* 3. Elevated serum amylase (in the presence of normal serum lipase/ no evidence of pancreatitis)
242
Elastase from what cells, are inhibited by alpha-1 antitripsin?
Neutrophils
243
Which cell types, when metabolizing a single glucose molecule, will always ueild pyruvate but sometimes generate _NO_ net ATP? (Explain this mechanism)
**Erythrocytes** 2, 3- BPG dec. hemoglobin affinity for O2. When 2,3-BPG needs to be synthesized, the RBC bypasses the step which converts 1,3 BPG to 3-phosphoglycerate, instead **forming 2,3 BPG in a process which does _not_ create ATP.**
244
Sx of _trapezius_ weakness vs _deltoid_ weakness
_Trapezius_- drooping of shoulder, impaired abduction **above horizontal,** winging of the scapula _Deltoid_- impaired abduction at angles below the horizontal plane
245
Finasteride MOA and indication
_MOA_: 5-alpha-reductase inhibitor that suppresses conversion of testosterone to DHT _Ind_: BPH, Androgenetic alopecia
246
When is an aortic stenosis murmur at its loudest?
The intensity is proportional to the magnitude of the LV to aorta pressure gradient during systole, (i.e. whenever aortic pressure is at its strongest)
247
1. Propionyl CoA is derived from what? 2. What enzyme is responsible for its conversion to methylmalonyl CoA? 3. What would deficiency of this enzyme lead to?
1. It is derived from amino acids (Val, Ile, Met, and Thr), as well as odd-numbered fatty acids and cholesterol side chains 2. **Propionyl CoA carboxylase** is the enzyme responsible 3. Deficient enzyme leads to development of proprionic acidemia
248
What are the (2) key functions of _type II pneumocytes_?
1. Regeneration of the alveolar lining 2. Surfactant production
249
_Tabes Dorsalis_ 1. Epidemiology? 2. Pathogenesis? 3. Clinical findings?
1. _Epidemiology_: Increased incidence of syphilis in MSM/ HIV-infected patients 2. _Pathology_: Treponema pallidum spirochetes directly damage the dorsal sensory roots 3. _Clinical findings_: sensory ataxia, lancinating pains, neurogenic urinary incontinence, Argyll Robertson pupils
250
Which ventricle forms the apex of the heart?
The left ventricle (All other chambers lie medial to the midclavicular line)
251
Pancoast tumors and Horner's syndrome
Among other symptoms, Pancoast tumors can lead to Horner's syndrome due to involvement of the cervical sympathetic ganglia.
252
How do PPIs affect osteoporosis?
Longterm PPI use may be associated with increased osteoporosis risk, most likely do to a decreased absorption of Ca2+ (acidic environment is needed)
253
_T-test_ versus _analysis of variance (ANOVA)_
A **t-test** is used to compare the difference between the means of (2) groups. **ANOVA** compares bbetween the means of 2 or more groups.
254
What rxns does _Thyroid peroxidase_ catalyze? (3)
1. Oxidation of iodide 2. Iodination of thyroglobuin 3. Coupling rxn between 2 iodized-tyrosine residues
255
Which blood vessel carries blood with the _lowest content of oxygen_ in the body and why?
**Coronary sinus** Myocardial oxygen extraction is very high and has a high demand
256
What are the effects of occlusion of the MCA?
1. Contralateral hemiplegia of the face/ upper limb 2. Relative preservation of the lower limb 3. Aphasia (if occlusion is in dominant hemisphere -- usually left)
257
What is the cause of _Myotonic Dystrophy_?
An autosomal dominant disorder, leading to increased number of trinucleotide repeats on the myotonia-protein kinase gene
258
Why does listening to heart sounds at end expiration make them more audible?
Decreased lung volume, bringing the heart closer to the chest wall.
259
_Fenoldopam_ 1. MOA 2. Indication 3. Effects (3)
1. _MOA_: selective peripheral D1 receptor agonist 2. _Indication_: given IV ti lower BP in HTN crisis, especially in patients with **renal insufficiency** 3. _Effects_: (1)Arteriolar dilation, (2) increased renal perfusion, and (3) promotion of diuresis/ natriuresis
260
Superior Vena Cava Syndrome 1. Pathogenesis? 2. Presentation?
1. Path: Compression of SVC (often by an intrathoracic carcinoma-- eg. mediastinal mass) leading to impaired venous return 2. Presentation: Dyspnea, facial swelling, and dilated collateral veins in upper trunk
261
Inapparopriate activation of what enzyme, leads to pancreatitis?
Trypsinogen (to trypsin)
262
_17a-hydroxylase def._ Presentation?
**Boy appear phenotypically femaile at birth, but girls develop normally.** (Result of dec. androgen, estrogen, and cortisol synthesis) Patients present with hypogonadism, HTN, and hypokalemia
263
Why is it that calcium channel blockers affect smooth and cardiac muscle, but not skeletal?
**Cardiac and smooth muscle** cells depend on extracellular _calcium influx_ into cells via L-type calcium channels. **Skeletal muscle** cells do not, because they have a _direct mechanical coupling_ of the L-type channel and the RyR.
264
Lesions on the medial foot cause, what type of lymphadenopathy? On the lateral foot?
_Medial_: **Inguinal** lympadenopathy _Lateral_: **popliteal** and **inguinal** lymphadenopathy
265
Causes of oral thrush (3 main)
1. Candida albicans 2. Albuterol inhaler 3. HIV
266
_Coronary Sinus_ 1. What does it serve as? 2. Under what condition might it be dilated?
1. Serves as the endpoint of venous drainage from the coronary blood supply, _draining directly into the right atrium_ 2. Will be dilated by any factor that dilates the right atrium (most commonly **pulmonary HTN**), because it freely communicates with the RA.
267
What is usually the most key component of the pathogenesis of AAAs?
Transmural aortic wall inflammation
268
What are the effects of nitrate? (3 general categories of effects) Side Effects?
* Primarily **venodilators** (but also vaso) that increase **peripheralvenous capacitance** * Reduce cardiac preload and afterload and LVEDP and volume, reducing work of heart * Modest effect on arteriolar dilation _Side Effects_: HA, cutaneous flushing, hypotension
269
_Aspergillus fumigatus_ 1. Describe its structure 2. How do people get it? 3. Describe associated disease processes
1. Mold that froms septate hyphae that branch at 45-degree angles 2. Spores are inhaled and particularly impact those in immunocompromised states 3. Wide spectrum of diseases: * **Invasive pulmonary aspergillosis** (for the immunosuppressed/ neutropenic) * **Aspergillus colonization** in preexisting lung cavities * **Allergic bronchopulmonary aspergillosis** (lung hypersensitivity reaction) for patients w/ asthma
270
"Red ragged" muscle fibers on _Gomori_ trichome are seen in what kinds of disease...?
Mitochondrial diseases The mitochondria accumulate under sarcolemma
271
Of all major vascular beds, which (2) are most susceptible to athersclerosis?
1. **Lower abdominal aorta** 2. Coronary arteries
272
_Aortic Regurgitation_ 1. Presentation 2. Describe how the pulses may feel 3. Describe the murmur and the best way to hear it 4. What is the state of the pulse pressure?
1. _Presentation_: progressive fatigue + dypsnea 2. _Pulses_: "water-hammer" pulses (bounding femoral and carotids) and head-bobbing with each heartbeat (de Musset sign) 3. _Murmur_: * Decrescendo murmur after A2 * High-pitched, blowing quality * Best heard at left sternal border, at 3rd/4th intercostal space, with the patient _sitting up and leaning forward_ 4. The pulse pressure is widened
273
How does _sepsis_ lead to ARDS? Describe step by step.
During sepsis, cytokines circulate in response to infection, activate pulmonary epithelium and provoke an inflammatory response mediated by **neutrophils**. This leads to _capillary damage_ and the leakage of protein/fluid into the alveolar space.
274
What is the marker for hematopoietic stem cells?
CD34+
275
Function of the suprachiasmatic nucleus (of the hypothalamus)
Circadian rhythm regulation and pineal gland function
276
Blockage of what vein causes symptoms similar to those in SVC syndrome, except only on one side of the body? What are those symptoms?
Blocking of the Brachiocephalic vein Shows one sided face-swelling, arm swelling and engorgement of subcutaneous veins .
277
What types of tissues are comonly found in a Meckel diverticulum? Which is most common? The presence of these tissues is an example of what?
**Gastric (most common),** pancreatic, colonic, endometrial, small bowell, etc. This is an example of _ectopy_
278
MOA of class 3 antiarrhytmic agents
They block K+ efflux from cardiac myocytes and prolong phase 3 of the myocyte AP
279
_Describe Von Gierke Dz_ 1. Deficient enzyme 2. Presentation 3. Tx
Type I Glycogen Storage Dz 1. Glucose 6- phosphatase 2. Severe fasting hypoglycemia (can't convert G6P to glucose); hepatomegaly 3. Tx: frequent oral glcose/cornstarch; avoidance of fructose and galactose
280
What is NE extravasation and what is the treatment?
Blanching of vein into which NE is being infused due to NE leakage causing major a1 receptor activation and subsequent constriction. Use _phentolamine_ (alpha-receptor blocker)
281
Major SEs of Corticosteroids
1. Immunosuppression (except with neutrophilia!!) 2. Corticosteroid-induced psychosis
282
_Fibrates_ 1. MOA 2. Effect 3. Compare this to fish oil
1. _Activates_ peroxisome proliferator-activated receptor alpha **(PPAR-a)**. 2. This leads to _decreased hepatic VLDL_ production and increased _Lipoprotein lipase_ activity, thus **lowering triglyceride levels** 3. Fish oil supplements w/ O3FA dec. VLDL and ApoB production
283
If a patient with HIV has rin-enhancing lesions, and treated for toxoplasmosis is negative, what dz should you suspect? What cell typically compose this dz?
Suspect _Primary Central Nervous System Lymphom__a_ It is typically composed of B-lymphocytes, and is associated with EBV.
284
_G protein-couple receptors_ 1. What do they bind? 2. What are the domains which make them up? (3)
1. Bind glycoprotein hormones (eg TSH, LH, FSH) 2. **3 major domains**: Extacellular domain, transmembrane domain, intracellular domain
285
1. Name the anthracycline chemotherapeutic agents. 2. What is their most severe side effect 3. How does it present? 4. How is this SE prevented?
1. Doxorubicin, daunorubicin, epirubicin and idarubicin 2. Their most severe side effect is a cumalitive dose-related _dilated cardiomyopathy_ due to free-radical formation 3. Presents w/ right and left ventricular CHF 4. Prevented via **dexrazoxane**- iron-chelating drug which reduces free radical formation
286
_X-linked (Bruton) agammaglobulinemia_ 1. Caused by what? 2. Effect on germinal center formation 3. Presentation?
1. Caused by a **defect in B cell maturation**, resulting in the absence of mature B cells with severe defiency of all Ig types. T cells remain fxnal and intact! 2. No B cells = _no_ germinal center formation 3. Recurrent infxn in setting of intact T Lymphocyte function
287
_Narcolepsy_ 1. Pathology 2. Presentation 3. Tx
1. _Pathology_: Due to low levels o stimulatory neurotransmitter **orexin** (hypocretin) which is involved in maintaining wakefulness and suppressing REM sleep 2. Episodes of refreshing sleep during the daytime + at least one REM sleep-related phenomena (cataplexy, hypogogic/hyponopompic halluciantions, and sleep paralysis) 3. Psychostimulants: **Modafinil**
288
_Retinal Artery Occlusion_ Presentation? Pathogenesis? What is the path most likely taken to occlude the artery?
_Presentation_: Acute, painless, monocular vision loss _Pathogenesis_: Thromboembolic complications of athersclerosis in the **internal carotid**. _Path_: Internal carotid --\> Ophthalmic artery --\> retinal artery
289
Other than trauma, what is one major cause for cardiac tamponade?
Viral pericarditis w/ signficant pericardial fluid accumulation (following a respiratory infection)
290
_Sarcoidosis_ Pathophys? Presentation? Dx? Treatment?
1. _Path_:Immune mediated, widespread noncaseating granulomas 2. _Pres_: Black women w/ possible erythema nodusum, arthralgia, elevated ACE levels, etc. 3. _Dx_: Bilateral hilar adenopathy on CXR 4. _Tx_: Steroids
291
Myocardial hibernation
A state of _chronic myocardial ischemia_ in which myocardial metabolism and function is **reversibly** reduced in order to match a reduction in coronary blood flow, thus _preventing myocardial necrosis_
292
What does _Prussian blue stain_ detect?
Intracellular iron
293
_Antiarrhythmic drugs_ Drugs + Predominant actions **Class IA, IB, IC**
1. _IA_: **Dispyramide, Procainamide, Quinidine**- *slows AP conduction velocity; prolongs APD* 2. _1B_: **Lidocaine, Mexiletine, Phenytoin**- *Shortens APD* (no effect on AP conduction velocity) 3. _1C_: **Flecainide, Propafenone**- *Slows AP conduction velocity; minimal effect on APD* "Double Quarter Pounder; Light Mayo and Pickles; Fries Please"
294
What illnesses can result in cold agglutinins (3)
1. Mycoplasma pneumoniae 2. EBV infection 3. Hematologic malignancy
295
What role does NFK-B play in osteoclast differentiation?
RANK-L is the receptor for activated NFK-B
296
What is the most effective preventative intervention in almost all patients?
Smoking cessation
297
Describe the structure of the mealtime insulin analogs
AA substitution at the C terminal end of the B chain
298
Name the A's of small cell (oat cell) carcinoma
1. May produce **A**CTH 2. SI**A**DH 3. **A**ntibodies against presynaptic Ca2+ channels (Lambert-Eaton) 4. **A**mplification of *myc* oncogenes
299
What receptors is epinephrine capable of affecting?
a1, B1, and B2
300
Where does the gastroduodenal artery sit? How can it be affected by ulceration?
It lies along the posterior wall of the duodenal bulb. It is likely to be eroded by posterior duodenal ulcers, leading to potential life-threatening hemorrhage.
301
Xanthomas are found where? What are they associated with?
These yellowish macules/papules are found on the medial eyelids. They are accumulations of macrophages + cholesterol/ TG and are assc. with hyperlipidemia/ dyslipidemia
302
_Hairy Cell Leukemia_ 1. What type of neoplasm? 2. Presentation?
1. Indolent B-cell neoplasm 2. MIddle-aged men w/ **bone marrow /fibrosis** (leading to dry tap) + massive **splenomegaly** (due to red pulp infilitration) + cytoplasmic projections on lymphocytes
303
_Diphteria_ 1. Epidemiology 2. Micro/pathology 3. Clinical Sx (4) 4. Complications (3)
1. Endemic in developing countries 2. ***Corynebacterium diphtheriae*** colonize repiratory tract and secrete _diphtheria toxin_ (inhibits protein synthesis via **ribosylation** of EF-2) 3. (1) Pseudomembrane; (2) cervical adenopathy (3)sore throat (4) fever 4. (1) Suffocation due to edema/pseudomembrane aspiration; (2) Heart failure/ (3) neuro toxicity from the toxin
304
Paroxysmal nocurnal hemoglobinuria is result of an acquired mutation in what gene? What inhibitor proteins is absent and/or deficient as a result?
Mutation in the ***PIGA*** gene. This leads to absence of the GPI anchor that normally protects the RBC from _complement_, and associated deficiency of CD55/CD59 complement inhibitor proteins.
305
What is the difference in the response generated by inactivated vs live-attenuated viral vaccines?
_Inactivated_: Generate a humoral response, inducing neutralizing antibodies which **keep virus from entering the cell membrane** _Live-attenuated_: Generate a strong cell-mediated response, _in addition_ to a humoral response
306
What is the MOA for _ANP and BNP_? How is this similar to sildenafil?
ANP and BNP are similar to NO. They activate _guanyl cyclase_ which increases the levels of _cGMP_. cGMP leads to relaxation of vascular smooth muscle and vasodilation, via myosin light-chain dephosphorylation _Sildenafil_ is a phosphodiesterase inhibitor and therefore decreases degradation of cGMP, ultimately causing the same result.
307
Role of the musculocutaneous nerve (2)
1. Innervates the major forearm flexors and the coracobrachialis 2. Provides sensory innervation to the lateral forearm
308
Complications of Paget dz (2)
1. High Output cardiac failure 2. Osteosarcoma
309
Thiazide diuretics 1. MOA 2. Effects 3. AEs
1. Inhibit Na+/Cl- co-transporter in the DCT, thereby decreasing reabsorption of NaCl 2. Lower BP by decreasing intravascular volume, reduce CO, and lower systemic vascular resistance 3. Dec. insulin secretion and glucose uptake, and increase LDL cholesterol and TG levels
310
What is the (4) step route that N. meningitidis uses to cause meningitis?
Pharynx --\> blood --\> choroid plexus --\> meninges
311
_Lesch-Nyhan Syndrome_ 1. Inheritance 2. Specific defect 3. What are the results of this defect?
1. X-linked recessive 2. Defect in hypoxanthine-guanine phosphoribosyltransferase (**HGPRT**) 3. Results in failure of the purine salvage pathway leading to inc. _degradation of hypoxanthine and guanine to uric acid._ De novo purine synthesis must inc. to compensate via inc. **PRPP amidotransferase**
312
_Tay-Sachs Dz_ 1. Inheritance 2. Enzyme that is deficient 3. What accumulates? 4. Presentation
1. Autosomal recessive disorder 2. B-hexosaminidase A deficiency 3. GM2 ganglioside accumulation 4. _Presentation_: progressive neurodegeneration and cherry-red macular spot, **NO HEPATOSPLENOMEGALY**!!! (unlike Niemann-Pick dz)
313
How does eithanol block gluconeogenesis?
Ethanol metabolism reduces NAD+ to NADH and **increases the NADH/NAD+ ratio.** This inhibits all other pathways requiring NAD+, including reactions required for _gluconeogenesis_.
314
What bones meet at the pterion? What vessel lies underneath it?
Frontal, parietal, temporal, and sphenoid bones **Middle meningeal artery** (branch of the maxillary artery)
315
Most severe AE of the anthracyclines (rubicins)
Cumulative dose-related dilated cardiomyopathy, due to formation of free radicals in the myocardium
316
_Subthalamic nucleus_ 1. What pathology can cause damage to this structure? 2. What is the consequence of this damage?
1. **Lacunar stroke** can cause damage 2. Damage may result in **contralateral hemiballism**, characterized by wild, involuntary, large-amplitude, flinging movements of proximal limbs on one side of the body
317
What is ARDS? 1. Pathology? 2. Presentation? 3. Histo? 4. What main diseases/issues can lead to ARDS?
**Acute Respiratory Distress Syndrome** 1. Characterized by diffuse injury to the pulmonary microvascular endothelium and alveolar epithelium, resulting in increased pulmonary capillary permability and a leaky alveolocapillary membrane 2. Presents as progressive hypoxemia refractory to O2 therapy and diffuse interstitial edema w/o cardiogenic cause. 3. Hyaline membranes on histo 4. **Pancreatitis**
318
What (3) types of drugs disrupt the peptidoglycan cell wall of gram-positive/ gram-negative organisms?
1. Penicillans 2. Cephalosporins 3. Vancomycin
319
For neonates infected with Hep B, what is their... 1. level of HBV replication? 2. level of liver enzymes? 3. risk of chronic infection?
1. High HBV replication 2. mildly elevated liver enzymes 3. very high (90%) risk of chronic infection
320
Metabolism of 1g of protein or carbohydrate, produces ______ Calories of energy. Metabolism of 1g of fat produces _____ Calories of energy.
P/C: 4 C F: 9 C
321
_Naltrexone_ 1. MOA 2. Indication
1. Mu-opiod receptor 2. First line tx for moderate to severe alcohol use disorder (blocks the rewarding and reinforcing effects)
322
Main Side Effect difference between ACE-I's and ARBs
ACE-I's raise the level of Bradykinin causing non-productive cough. ARBs do not. The two drugs are very similar otherwise
323
Myotonic dystrophy presentation (5)
Sustained muscle contraction (can't release doorknob) + weakness/atrophy (often also see cataracts, frontal balding and gonadal atrophy)
324
What is moa of mineralocorticoid receptor antagonists? What diseases are they good for and who should they not be used for? Give (2) examples
(Spironolactone and Eplerenone) They prevent aldosterone from binding to its receptor in the distal renal tubules, leading to increased sodium excretion. Indications: CHF and reduced left ventricular EF Contraindications: Hyperkalemia and renal failure
325
**Kussmaul sign**
Paradoxical rise in JVP during inspiration, because volume-restricted right ventricle is unable to accomadte the inspiratory increase in venous return. Associated with **constrictive pericarditis**
326
Name the key characteristics of which distinguish Slicosis from other forms of pneumoconiosis (2) How can silicosis impair immune function?
1. Eggshell calcification at the hilar nodes 2. _Birefringent_ silica particles surround by fibrous tissue on histology Silicosis impairs the **_macrophages_**, by disrupting them with internalized silica particles
327
Where are dietary lipids digested? Where are they absorbed?
1. _Digestion_: in the **duodenum** via pancreatic enzymes 2. _Absorbed_: in the **jejunum** in the form of water-soluble micelles
328
What are the (2) _main_ effects of **nitrates**?
1. **Venodilation** (as well as general vasodilation) 2. **Decreased preload** (i.e. decreased LV EDV and EDP) All told, this results in decreased left ventricular systolic wall stress and myocardial oxygen demand
329
What is the neurological effect of vitamin B12 deficiency?
**Subacute combined degeneration!!!** _demyelination_ of dorsal columns, lateral CST, and spinocerebellar tracts, leading to: ataxic gait, paresthesia, and impaired position/vibration sense
330
Why is _N-myc_ capable of being detected by DNA probes?
It is a transcription factor and thus is capable of _binding DNA_.
331
Describe Type IV Hypersensitivity How are antibodies involved? What are the (4) T's of this hypersensitivity?
Aka delayed (T-cell mediated) type Sensitized T cells encounter antigen and then release cytokines (leading to macrophage activation) **No antibodies involved** _4 T's_: T cells Transplant rejections TB skin tests Touching (contact dermatitis)
332
What is the relationship between Cystic Fibrosis, ADEK, and squamous metaplasia?
* CF can lead to pancreatic insufficency, fat malabsorption, and an ADEK deficiency. * Vitamin A maintains orderly differentiation of specialized epithelia. * Avitaminosis A can cause squamous metaplasia of such epithelia to keratinizing epithelium
333
What tumor type(s) is the following tumor marker assc. with?: ## Footnote **Alpha fetoprotein**
- Hepatocellular carcinoma - Germ cell tumors
334
Conversion disorder
**Neurological** symptoms that are incompatible with a neurological disease. Often stressed associated.
335
At what point in the respiratory cycle is the total pulmonary vascular resistance at its lowest?
The functional residual capacity (the trough of the tidal volume)
336
Frontal Lobe Syndromes 1. Cause 2. Clinical manifestation 3. Left sided vs Right sided 4. Dx
1. _Cause_: Damage to frontal lobe 2. _Presentation_: Issues with executive fxn (complex tasks, motivation, organization, etc.) and personality 3. **Left sided**: apathy/depression | **Right sided**: disinhibtion 4. Dx: info from fam/friends, neuropsych testing and structural brain imaging
337
What effect might fibrinolytics have on heart rhythm?
They may cause a repurfusion arrhythmia on arterial reopening. These arrhytmias are usually benign.
338
_AV Shunts_ 1. What affect do they have on preload/afterload? 2. What might the physical exam reveal?
1. AV shunt _increases_ the **preload** and _decreases_ the **afterload** by routing blood directly from the arterial system to the venous system 2. Physical exam may reveal pulsatile mass w/ thrill on palpation. Ausculation reveals a constant bruit over the site.
339
Stimulation of the vagus nerve would have what effect on the lungs?
It would cause bronchoconstriction and increased bronchial mucus secretion via Ach binding to M3 receptors. This ultimately increases the work of breathing.
340
Ulcers in which of these locations are NOT associated with an increased risk of carcinoma in that location? * Duodenum * Esophagus * Stomach * Colon/Rectum
Of these, _only Duodenal ulcers_ are not associated with an inc. risk of CA
341
What organelle serves as the primary site of ribosome synthesis and assembl?
The _nucleolus_ (All ribosomal RNA except 5S RNA is transcribed here)
342
CREST Syndrome 1. What is this disease a subset of? 2. Pathology? 3. What are the sx?
1. Limited **scleroderma** 2. Increased proliferation and accumulation of monoclonal T-cells in affected tissues. Leads to increased TGF-B release and thus increased production of collagen and ECM 3. Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia
343
Ortner syndrome
When mitral stenosis causes left atrial dilatation sufficient to impinge on the left laryngeal nerve
344
_Wilson Dz_ 1. Sx (3) 2. Dx
1. **Sx**: _Liver_ injury (sometimes asymptomatic), _psych_ sx's (impulsive and moody), _Basal ganglia_ injury (leading to parkinsonism) 2. **Dx**: Kayser-Fleischer rings; labs show increased transaminases (liver injury) and low **serum ceruloplasmin**
345
1. Acid-fast stans ID organisms that have ______ \_\_\_\_\_\_ (2) in their cell walls. 2. How do acid fast stains specifically work?
1. **Mycolic Acid** (like mycobacterium and some Nocardia species) 2. Aniline dye is applied to a smear and then is decolorized w/ acid alcohol
346
What are the two ways in which Beta-blokers ameliorate the sx of thyrotoxicosis?
1. Decreasing the effect of adrenergic impulses on target organs 2. Decreasing the rate of peripheral T4 to T3 conversion
347
What type of injury does Carbon tetrachloride (CCL4) cause, and how?
It causes free radical injury, after being oxidized by the P450 system. The result is CCl3 which causes lipid degradation and formation of H2O2
348
PaO2
A measurement of the partial pressure of oxygen dissolved in the **_plasma only_**! (not in the RBCs)
349
_Lesch-Nyhan Syndrome_ 1. Inheritance? 2. Presentation? 3. Pathology? 4. The [] of what enzyme increases as a result?
1. X-linked recessive 2. _Presentation_: **self-mutilation, hyperuricemia**, dystonia, and choreoathetosis in the _first few years of life_ 3. _Pathology_: HGPRT deficiency (used in purine salvage pathway). Leads to degradation of hypoxanthine bases into uric acid. 4. Increased demand for de novo purine synthesis leads to **i****ncreased _PRPP_** activity
350
_Hepcidin_ 1. What is it/ what does it do? 2. When is it released?
1. Hepcidin is an **acute phase reactant** synthesized by the liver that acts as the **central regulator of iron homeostasis.** It _prevents release of iron_ bound by **ferritin** 2. High iron levels/ inflammatory conditions increase hepcidin synthesis. Hypoxia and increased EPO lower hepcidin levels
351
_Subarachnoid Hemorrhage_ (SAH) 1. Presentation 2. Pathology and associated diseases (3) 3. Dx
1. **Sx**: "Worst headache of my life" + fever/nuchal rigidity 2. **Path**: Saccular (berry) aneurysms are most common cause. Associated with Ehlers-Danlos syndrome, ADPKD, and AV malformations 3. **Dx**: Non-contrast CT showing hyperdensity in cisterns/sulci; LP showing gross blood or xanthochromia (yellow discoloration of the CSF)
352
What is the main dz Primary Biliary Cirrhosis (PBC) is associated with?
Sjrogren's syndrome (and other autoimmune disorders)
353
What are the normal adult pressures in the cardiac chambers, the pulmonary artery and the aorta (minimum and maximum)
**RA**: 0 and 8 mmHg **RV**: 4 and 25 mmHg **PA**: 9 and 25 mmHg **LA**: 2 and 12 mmHg **LV**: 9 and 130 mmHg **Aorta**: systolic BP
354
What is responsible for the green discolortion of pus or sputum during bacterial infections?
**_Myeloperoxidase (MPO)_** from neutrophil azurophilic granules. MPO is a heme-containing pigmented molecule.
355
What type of polyps are most likely to undergo malignant transformation?
Villous adenomas
356
What type of epithelium makes up the true vocal cords?
Stratified squamous
357
_Prostacyclin vs Thromboxane A2_ Compare their effects
**_Prostacyclin_**: vasodilates, *inhibiting platelet aggregation* and increasing vascular permeability **_Thromboxane A2_**: prostaglandin which enhances platelet aggregation and causes vasoconstriction
358
_McCune-Albright Syndrome_ Name the triad of clinical features
1. **Fibrous dysplasia** (multiple osteolytic-appearing lesions of the hip/ pelvis) 2. **Cafe-au-lait spots** 3. **Endocrine abnormalities** (precocious puberty, hyperthyroidism, etc.)
359
What is responsible for the clinical variability of mitochondrial dz?
The mixture of two types of genetic material, known as heteroplasmy. In other words, during mitosis there is a random distribution of mitochondria, some containing lots of damaged mitochondria, others containing very few.
360
What key structures form from the _third_ pharyngeal pouch?
Thymus and **inferior** parathyroid glands
361
The venous component of **internal hemorrhoids** drain where? **External hemorrhoids**?
_Internal_: drain into the middle and **superior rectal veins**, which communicate with the internal iliac and **inferior mesenteric veins** _External_: drain via the **inferior rectal vein** into the internal pudendal vein, which communicates with the internal iliac veins
362
Describe the echocardiogram assc. with Transposition of the Great Arteries (TGA)
Aorta lying anterior to the pulmonary artery
363
Where is ADH synthesized? Is this the same location, or a different location from where it is stored?
In the hypothalamus. It is then _transported_, to the posterior pituitary for storage and later release into the circulation. Only hypothalamic damage will be enough to cause central Diabetes insipidus
364
1. Pernicious anemia (Vit B12 def.) is caused by autoimmune destruction of what cells? 2. What do these cells secrete and where are they located?
1. Destruction of **parietal cells** (chronic atrophic gastritis). 2. They secrete HCL and IF, and are found primarily in the **superficial region of the gastric glands**
365
Deletions or additions of a number of base pairs which are not a multiple of three, indicate that a ___________ mutation has occured.
Frameshift
366
What prompt eosinophils to release their cytoplasmic granules to kill a parasite?
When parasites enter the body they are coated by IgG and IgE which bind to the Fc receptor on the eosinophil, prompting it to degranulate. (**Antibody-dependent cell-mediated cytotoxicity**) Eos also contribute to _late stage_ hypersensitivity
367
If a pneumonia patient has a CD4+ count greater than 400cells/uL, what bug do you expect to be the cause?
\>400?: **strep pneumo** pneumo jiroveci (PCP)
368
What effect does expgenous steroid abuse have on hematocrit?
Increases hematocrit
369
_Meckel diverticulum_ 1. What is it/ How common is it? 2. What causes it?
1. The most common congenital anomaly of the small intestine 2. It forms due to incomplete obliteration of the omphalomesenteric duct that connects the midgut lumen and yolk sac cavity
370
For an otherwise health child, what is the most common cause of osteomyelitis?
Staph aureus
371
How is the pathogenesis of Non-bacterial thrombotic endocarditis (_NBTE_) related to that of _Trousseau's_?
They can both be induced by disseminated cancers, usually due to some hypercoagulable state.
372
Location of femoral hernias
Inferior to the inguinal ligament, lateral to the pubic tubercle, and medial to the femoral vein
373
Coordinate each artery with the leads associated with them 1. LAD 2. LCX 3. RCA
1. Anterior and Septal 2. Left lateral 3. Inferior and Right
374
_Aschoff bodies_ What are they and with what dz process are they found? What is another name for the cellular component?
They are interstitial myocardial granulomas, found in myocarditis due to acute rheumatic fever The macrophages are aka as caterpillar cells
375
Phosphorylation of what residues (and via what?), leads to insulin resistance?
Phosph. of serine and threonine residues of insulin receptor and insulin receptor substrate by serine kinase, leads to insulin resistance
376
_Prinzmetal's (variant) Angina_ 1. Pathology? 2. When does it occur? 3. Dx? 4. Tx? 5. What drug can make it worse?
1. Episodic, transient attacks of **coronary vasospasm** 2. Usually occurs at rest and during late night/early morning 3. Temporary transumral MI w/ ST elevation on EKG 4. Tx with vasodilators and CCBs 5. **_Ergonovine_** can provoke vasospasm and can *aid in diagnosis*
377
_Aortic stenosis_ 1. Common murmur 2. Sx 3. Main causes
1. Crescendo-decrescendo murmur **right sternal border** 2. Typically asymptomatic. If advanced can present w/ exertion and inlcude syncope, dizzyness, angina or even HF 3. **Main causes**: abnormal valve w/ calcification (e.g. bicuspid aortic); calcified normal valve; or rhemuatic heart disease
378
_Adult type Aortic Coarctation_ 1. Key triad 2. View on imaging
1. (1) upper body hypertension; (2) diminished lower extremity pulses; (3) enlarged intercostal artery _collaterals_ 2. **Notching of ribs** as a result of the enlarged, tortuous intercostal arteries
379
What drug group is responsible for most of the overdoses in the country?
Opioids
380
_Chronic Granulomatous Disease_ 1. Pathogenesis? 2. Presentation?
1. _Pathogenesis_: A *genetic* defect in the **NADPH oxidase complex** decreases the formation of reactive oxygen species which have a direct _microbicidal_ activity and also _activate granule proteases_. 2. _Presentation_: Child with recurrent bacterial and fungal infections
381
_Coccidioides immitis_ 1. Describe the (2) forms 2. Where is it endemic to? 3. How is it transmitted? 4. How does it present (5)
1. Mold in the cold, endospore in the heat 2. Southwest US, northern Mexico, regions of Central and South America 3. Transmitted via spore inhalation which form endospores that rupture and disseminate 4. _Presentation_: * acute pneumonia (most common) * chronic pneumonia * pulmonary nodules and cavities * extrapulmonary nonmeningeal disease * meningitis
382
_Colonic Diverticula_ 1. Involve what part of the colon? 2. What process leads to their development?
1. Usually involve the sigmoid colon 2. The result of increased intraluminal pressure (**pulsion**) created during strained bowel movements
383
What is the MOA and Indication for both Cromolyn and Nedocromil? How effective are they compared to drugs with a similar indication?
They are mast cell stabilizing agents which inhibit mast cell degranulation independent of stimuli. They are a second-line treatment for _allergic rhinitis_ and _bronchial asthma_. Glucocorticoids are the first line prophylactic and are a superior drug.
384
_Primary Carnitine Deficiency_ 1. What is the effect at the level of the cell? 2. Presentation
1. Deficiency in carnitine impairs fatty acid transport from the cytoplasm into mitochondria, **preventing B-oxidation of fatty acids into acetyl CoA** 2. Sx: _Cardiac_ and _skeletal myocyte_ injury (lack of ATP from Citric acid cycle) and impaired ketone body production by the liver during fasting periods
385
Describe the process of _Infective Endocarditis_ (4)
1. Disruption of normal endocardial surface (usually in areas of maximum turbulence) 2. Focal adherence of **fibrin and platelets**, forming _sterile fibrin-platelet nidus_ 3. Colonization by microorganisms (*strep* to damaged areas; *staph* to damaged or normal areas) 4. Formation of macroscopic vegetations made of debris
386
_Aortic Dissection_ 1. Most important risk factor 2. What specific layer tears? 3. What genetic issue predisposes for aortic dissection?
1. HTN is most important risk factor 2. Tunica intima is the layer which tears away 3. Marfan's which leads to _cystic medial degeneration_
387
What is the most abundant AA in collagen?
Glycine (occupies every thrid AA position)
388
_Osler-Weber-Rendu Syndrome_ 1. What is the other name? 2. Genetics 3. Clinical Presentation 4. Possible consequences
1. Hereditary hemorrhagic **_telangiectasia_** 2. Autosomal dominat 3. Presence of telangiectasias in the skin and mucous membraes of the lips, oropharynx, respiratory tract, GI tract and urinary tract 4. Rupture of these vessels may cause epistaxis (**_nosebleeds_**), GI bleed, or hematuria
389
What is the effect of low hepcidin levels?
Low hepcidin levels increase intestinal iron absorptin and stimulate iron release by macrophages
390
_Lambort-Eaton syndrome_ 1. Sx 2. Disease associated with 3. Pathogenesis 4. Tensilin test result 5. Nerve stimulation studies
1. **Sx:** weakness improves during the day and with excercise. Weakness of proximal muscles 2. **Assc**. with pre-existing malignancy 3. **Path**: antibodies against pre-synaptic calcium channels 4. No improvement from tensilon test 5. Incremental response from nerve stim. test
391
How is *Campylobacter* infection spread?
Via **domestic animals** or from contaminated food
392
_Tumor lysis syndrome_ 1. When can it develop? 2. What characterizes it?
1. Develops during chemo for CAs with rapid cell turnover, high tumor burden, or high chemo sensitivity 2. Characterized by a **leak of intracellular ions** (such as hyperphosphatemia, hypocalcemia, hyperkalemia, and hyperuricemia)
393
What is the most common cause of bacterial meningitis in adults of all ages?
Strep Pneumo
394
_Serotonin syndrome_ 1. How do we get this syndrome? 2. Sx (3) 3. What is the antidote? 4. What is the precursor to serotonin?
1. The result of either serotonin overdose or co-prescription with an MAOI or triptan 2. (1) neuromuscular excitation, (2) autonomic stimulation, (3) altered mental status 3. _Antidote_- **Cyproheptadine** ( a first generation histamine antagonist) 4. **Tryptophan** is the precursor to serotonin
395
_Dopamine:_ What is the effect of... 1. Low doses? 2. Medium doses? 3. Very high doses?
1. _Low dose_: stimulate D1 receptors in the renal and mesenteric vasculature 2. _Medium dose_: stimulate B-1 receptors, increasing cardiac contractility 3. _High dose_: stimulate a-1 receptors, producing generalized vasoconstriction
396
_First generation anti-histamines_ 1. Name them 2. In addition to blocking histamine receptors, what other affects? 3. How can the eye be affected?
1. Ex. **Chlorpheniramine, diphenhydramine** 2. Also have antimuscarinic, anti-alpha adrenergic, and anti-serotonergic properties 3. Anticholinergic effects on occular ciliary muscles impair accomdation and cause **blurring of vision for close obects**
397
_Vitamin A toxicity_ 1. Acute affects (3) 2. Chronic affects (5) 3. Teratogenic affects (3)
1. _Acute_: N/V, vertigo, blurred vision 2. _Chronic_: alopecia, dry skin, hyperlipidemia, hepatotoxicity/ hepatosplenomegaly, and visual difficulties 3. _Teratogenic_: Microcephaly, cardiac anomalies, fetal death
398
MOA of B-Blockers
B-Blockers decrease AV nodal conduction, leading to an increased AV nodal refractory period
399
What are the (5) general dz types associated with _clubbing_?
1. Lung dzs (oft associated with hypoxia) 2. Heart dzs (especially cynotic congenitals dzs and bacterial endocarditis) 3. IBD 4. Hyperthyroidism 5. Malabsorption
400
Main phase "0" difference between regular cardiac cell and a pacemaker cell
Regular cardiac cell: 0 = Na+ rush Pacemaker: 0 = Ca2+ rush
401
_Vascular and Immunologic Manifestations of Infective Endocarditis_ (3) Vascular (2) Immunological
_Vascular_: 1. Systemic emboli 2. Mycotic aneurysm 3. Janewy lesions (**nontender**- palms/soles) _Immunologic_: 1. Osler nodes (**Painful**- toes/fingertips) 2. Roth spots
402
_Viral Laryngotracheitis_ 1. Also known as? 2. Presentation? 3. Most common virus responsible?
1. Croup 2. Brassy, barking cough; Dyspnea; Recent history of upper respiratory infection (URI) 3. **Parainfluenza virus** is the most common cause
403
Elevated prolactin levels (from a prolactinoma), directly suppress what?
GnRH
404
Capsaicin causes depletion of what neurotransmitter? What does this lead to?
_Substance P_ An intitial burning/stinging sensation, but chronic exposure leads to **reduced pain transmission**
405
Omalizumab MOA/ indication
Anti-IgE antibody Add-on therapy for patients with severe allergic asthma
406
What are the (2) major effects of *Clostridium perfingens?*
1. Late-onset food poisoning (consisting of transient watery diarrhea) 2. Clostridial myonecrosis (gas gangrene)
407
Which blood vessels does the third part of the duodenum interact with?
It crosses horizontally acros the abdominal aorta and the IVC at the level of L3. The Superior mesenteric vessels lie anterior to the duodenum at this location.
408
What are the (4) major causes of hypoxemia? Which has a normal A-a gradient?
1. Alveolar hypotension (**normal A-a gradient**) 2. V/Q mismatch 3. Diffusion impairment 4. Right-to-left shunting
409
What kind of drug are theophylline and aminophylline? What is their MOA?
They are Methylxanthines They cause bronchial dilatation by decreasing phosphodiesterase enzyme activity, thereby increasing intracellular camp.
410
Compare cardiac tissue conduction velocity between the: AV node, purkinje system, ventricular muscle, and atrial system
**_P**_ark _**At**_ _**Vent**_ure _**Av_**enue *Fastest*- Purkinje system, Atrial system, Ventricular system, AV node- *slowest*
411
_Wilson Disease_ 1. Inheritance 2. Path
1. Autosomal recessive mutation of the ATP7B gene 2. _Path:_ Mutation leads to a decrease in copper incorporation into ceruloplasmin and reduced biliary copper excretion leading to copper accumulation in liver, brain, and eye
412
_Hyperacute rejection_ vs _acute rejection_ vs _chronic rejection_ (particularly in the lung) Time-frame? What part of the lung is affected?
_Hyperacute_: Occurs in minutess. Preformed antibodies lead to graft blood vessel spasm and diffuse intravascular coagulation (white graft rejection) _Acute_: 1-2 weeks. Due to recipients rection to the HLA of the graft. Causes **vascular damage and bronchial infiltration**. Perihilar and lower lobeopacities on CXR. _Chronic_: months to years after transplant. Inflmmation of the small bronchioles (lungs) or vessels (kidneys) leading to narrowing and obstruction
413
Which cell types have B1 receptors?
Cardiac tissue and renal juxtaglomerular cells
414
Name the main organisms that can cause diarrhea with only a small inoculum (4)
1. Shigella 2. Entamoeba histolytica 3. Giardia lamblia 4. Campylobacter jejuni
415
_Gallstone ileus_ 1. What is it? 2. Presentation 3. Dx
1. _Obstruction_ in the **_ileum_** due to passage of a large gallstone through a cholecystenteric fistula in the small bowel 2. SBO 3. _Dx_: Ab XR may reveal gas within the gallbladder and biliary tree
416
Mechanisms of bacterial transfer: Transformation vs Conjugation vs Transduction
_Transformation_: **Direct uptake** of naked DNA form the environment _Conjugation_: One way transfer of chromosomal or plasmid DNA between bacteria via direct physical contact (one of them must have F factor which codes for a _sex pilus_). _Transduction_: Transfer via **bacteriophage**
417
Name the pentad for _Thrombotic thrombocytopenic purpura_
1. Neurologic symptoms 2. Renal failure 3. Fever 4. Thrombocytopenia 5. Microangiopathic hemolytic anemia
418
What is the most common genetic cause for: Hypertrophic cardiomyopathy? Dilated cardiomyopathy?
1. _Hypertrophic_: Autosomal dominant mutations in cardiac sarcomere proteins (usually **beta-myosin heavy chain**) 2. _Dilated_: Autosomal dominant mutations of myocyte cytoskeleton (**dystrophin**) or **mitochondrial enzymes**
419
_Migraine_ 1. Sx 2. Abortive therapy (1) 3. Prophylactic therapy (3)
1. Unilateral HAs, with pulsating/throbbing quality, associated with photophobia, phonophobia, and nausea 2. **Abortive**: Triptans (serotonin agonists) 3. **Prophylactic**: Beta-blockers, antidepressantts, and anti-convulsants
420
How is tolerance to nitrates avoided?
You must provide a nitrate-free interval every day in patients w/ long acting nitrates.
421
_Fat Embolism_ 1. Presentation? 2. Staining?
1. Severe resp. distress + diffuse neurological impairment + upperbody petechial rash, following long bone injury 2. Fat emboli turns block from stain w/ **osmium tetroxide**
422
The 22q11.2 microdeletion associated with DiGeorge, causes what embryological component(s) not to develop?
3rd and 4th branchial pouches
423
What cranial nerves and what aortic arch derivatives are associated with each pharyngeal/aortic arch (1-6)
1. CN V; Maxillary artery 2. CN VII; Stapedial artery (regresses) 3. CN IX; Common carotid and prox. internal carotid 4. CN X (superior laryngeal); True aortic arch and Subcalvian arteries 5. Obliterated 6. CN X (recurrent branch); Pulmonary arteries and Ductus arteriosus
424
In patients with mitral regurg, what is the most reliable ausculatory finding to assess severity
The presence of a left-sided **_S3 gallop_**. This indicates high regurgitant volume and left ventricular volume overload
425
_Isotretinoin_ Indication and Contraindication
_Indication_: Used to tx severe acne w/ significant scarring _Contraindication_: Pregnancy, due to high risk of teratogenicity
426
Describe the biopsy specimen associated _giant cell arteritis_. What other dz is this identical to?
Scattered, focal granulomatous inflammation centered on the media with : 1. intimal thickening 2. elastic lamina fragmentation 3. giant cell formation (not granulomatous) Same histo as **Takayasu arteritis**
427
Describe the process of staphylococcal food poisoning
It often occurs after a food handler inoculates food **(usually a _mayonnaise_ containing product),** w/ *S.aureus* that is allowed to incubate at room temp, producing heat-stable _exotoxin_ that causes rapid-onset N/V and abdominal cramping
428
Describe the mechanism with which _Sarcoidosis_ can lead to hypercalcemia
1-a hydroxylase expression in activated macrophages, leads to **PTH independent Vit D activation** and subsequent inc. intestinal absorption of Ca2+
429
1. Who should be vaccinated against _Strep Pneumo_? 2. What are the (2) main vaccines?
1. Elderly/ young children are most at risk 2. Both affect the _outer polysaccharide covering_: * **Pneumococcal polysacharide vaccine** * **Pneumococcal conjugate vaccine**
430
What type of drug is useful for nausea assc. with GI insults? For nausea assc. with migraine?
**GI**: 5-HT3 receptor antagonists **Migraine**: Dopamine antagonists
431
What disease are caused by "exotoxin" release by S. aureus?
1. Toxic shock syndrome 2. Staphylococcal scalded syndrome 3. Gastroenteritis
432
What is the role of protein A in *staph aureus*?
Protein A is a virulence factor found in the peptidoglycan wall of Staph Aureus that **_binds the Fc portion of IgG_**, leading to impaired complement activation, opsonization, and phagocytosis.
433
_Ehlers-Danlos syndrome_ 1. What is it? 2. Usual cause? 3. Presentation
1. Group of rare hereditary disorders charcterized by defective collagen synthesis 2. The result of procollagen peptidase deficiency, which results in _impaired cleavage of terminal propeptides_ in the extracellular space 3. Patients present with joint laxity, hyperextensible skin, and tissue fragility, due to collagen which does not crosslink
434
What is the indication for an ace-inhibitor? For a thiazide?
**ACE-inhibitors**: inhibit chronic angiotensin II-mediated remodeling that occurs in association w/ MI and CHF **Thiazides**: useful as an initial treatment for essential HTN w/o CHF or diabetes
435
What cell type composes the wall of the cyst in the years after a stroke?
Astrocytic processes (glial scar)
436
_Selective IgA Deficiency_ 1. Pathology 2. Presentation 3. What are these patients at risk for?
1. _Pathology_: Failure of B-cells to switch from IgM to IgA production. Very common 2. _Presentation_: Usually asymptomatic but can have recurrent sinopulmonary and GI tract infections 3. _Risk_: Many of these patients form IgG antibodies directed against IgA so risk anaphylaxis during transfusion
437
What does the smooth ER contain? What type of cells contain lots of smooth ER?
(In contrast to the RER,) the smooth ER contains **enzymes for steroid and phospholipid biosynthesis**. _ALL_ steroid-producing cells (adrenals, gonads, liver, etc.) contain a well-developed smooth ER.
438
_Mesothelioma_ What is it? Risk factor(s)? Presentation? Dx factors? (3)
* Rare malignant neoplasm of mesothelial cells. * Primary risk factor is consistent _Asbestos_ exposure * Symptoms include: dyspnea and chest pain * _Dx_ tests will show: 1. Hemorrhagic pleural effusion 2. Pleural thickening on radiographic study 3. Long slender microvilli and abundunt tonofilaments on histo.
439
Dystrophic vs Metastatic calcification What conditions do they each occur in?
_Dystrophic_: Occurs normally, w/ age, in **damaged** or **necrotic** tissues in the setting of **normal calcium levels**. _Metastatic_: Occurs in normal tissue in the setting of **hypercalcemia**
440
_Klebsiella_ Encapsulated or not? Lactose-fermenting or not? Usual presentation/ patients affected/characteristics?
1. Encapsulated 2. Lactose fermenting (grows pink on MacConkey agar) 3. It causes pneumonia in subjects with impaired host defenses, especially alcoholics 4. Characterized by tissue necrosis, early abscess formation and currant jelly sputum
441
How can Isoniazid lead to sideroblastic anemia? Describe the process in detail (3 main steps)
1. Isoniazid (TB drug) inhibits pyridoxine phosphokinase, leading to a **vitamin B6 deficiency** (pyridoxine). 2. The active form of pyridoxine is a cofactor for **delta-aminolevulinate synthase**, an enzyme which catalyzes the rate-limiting step of _heme synthesis_. 3. Inhibition leads to **sideroblastic anemia**.
442
What drugs do you give to reverse heparin effects? Warfarin effects?
_Heparin_: protamine sulfate _Warfarin_: Vitamin K and Fresh Frozen Plasma (FFP)
443
Describe the pathogenesis of centriacinar emphysema
1. Oxidative injury to the respiratory bronchioles by _smoking_ 2. Macrophage activation 3. Inflammatory recruitment of neutrophils 4. Release of _proteases_ by _neutrophils_ and _macrophages_
444
What sort of procedure is associated with _enterococcal endocarditis_
Genitourinary instrumentation or catherization (enterococcus is a component of normal colonic and GU flora)
445
_Antiarrhythmic drugs_ 1. Drugs + Predominant actions 2. Potential SEs **Class IV**
**Verapamil; Dilitiazem** 1. *Slows sinus node discharge rate; slows AV nodal conduction and prolongs refractoriness* 2. Can lead to severe bradycardia and hypotension (especially in combo with a beta-blocker)
446
_Hexokinase vs Glucokinase_ 1. Location 2. Affinity vs Capacity? 3. Induced by insulin? 4. Mutation assc. with Maturity-onset diabetes of the young?
1. **H**: Everywhere but liver and pancreatic B cells; **G**: Liver and pancreatic B cells 2. **H**: High affinity, low capicity; **G**: low affinity, high capacity 3. **H**: Not induced by insulin; **G**: induced by insulin 4. **H**: Not assc. with MODY; **G**: Assc. w/ MODY
447
What components are needed to do PCR? (4)
1. Primers that are complementary to the regions of DNA flanking the segment of interest 2. Thermostable DNA polymerase 3. Deoxynucleotide triphosphates 4. A source DNA template strand
448
Ghon Complex and TB reactivation (who is it in and what characterizes it?)
This complex characterizes the initial stages of _M. tuberculosis_ infection. It consists of a lower **lobe lung lesion** (Ghon focus) and **ipsilateral hilar adenopathy**. Reactivation of TB occurs most often in immunosuppressed patients and is characterized by apical cavitary lesions and hemoptysis.
449
What sometimes occurs between days 2-4 following a transmural MI? Why?
**Early-onset pericarditis** develops in 10-20% of patients. It represents an inflammatory reaction to adjacent cardiac muscle necrosis.
450
Define _Preventable Adverse Effect_
An injury to a patient due to failure to follow evidence-based best practice guidlines. (Not to be confused with *malpractice,* which is a legal determination)
451
What is the usual clinical presentation for Hyper-IgM syndrome? (2)
1. Lymphoid hyperplasia 2. Recurrent sinopulmonary infections
452
Patient presents due to lightheadedness/ passing out while buttoning a tight shirt collar. 1. Most likely dz process 2. What nerve is most assc. with this dz process?
1. Carotid sinus hypersensitivity (triggered by pressure from the shirt) 2. The afferent limb of the carotid sinus reflex is a branch of the **glossopharyngeal nerve**
453
_McCune-Albright Syndrome_ Pathogenesis
Mutation in the GNAS gene which leads to constitutive activation of the G protein/cAMP/ adenylate cyclase signaling cascade
454
Digoxin MOA
Digoxin directly blocks the Na/K+ pump in myocardial cells, leading to increased intracellular Na. This slows functioning of Na+/Ca+ exchanger, thereby keeping Ca+ trapped in the the myocardial cell as well, increasing contractility.
455
_Huntington disease_ 1. Describe the genetic/cellular level cause of this disease. 2. How are histones involved?
1. Increased CAG trinucleotide repeats in the gene that codes for the huntingtin protein, leading to gain-of-fxn mutation. 2. Transcriptinal repression (silencing), via histone _**de**acetylation,_ is thought to be one of the main mechanisms in which this mutation causes disease
456
What is the most common viral cause for aplastic crisis for sickle cell patients?
Parvovirus B19
457
_Tetrodotoxin_ 1. Where does it come from? 2. What effect does it have?
1. Comes from pufferfish 2. Binds to voltage-gated sodium channels in nerve and cardiac tissue, preventing sodium influx and depolarization -- leads to paralysis
458
_Pulsus Paradoxus_ 1. What is it? 2. How is it detected? 3. Why does it happen? 4. What disease processes is it associated with? (4)
1. An exaggerated drop (\>10mmHg) in systolic BP during inspiration 2. Detected when taking the BP, by listening to the difference between when korotkoff sounds are first heard during expiration and the pressure at which they are heard throughout all phases of respiration 3. Inspiration inc. venous return and normally this expands RV into pericardium but if this expansion can't happen, the interventricular septum pushes into the LV, dropping LV EDV and subsquently the stroke volume 4. Pericardial disease, acute cardiac tamponade, asthma, COPD
459
What tumor type(s) is the following tumor marker assc. with?: ## Footnote **Carcinoembryonic antigen**
GI (colorectal)
460
_Achalasia_ 1. Cause 2. What is seen on esophageal manometry?
1. Caused by reduced numbers of inhibitory ganglion cells in the esophageal wall 2. Manometry shows dec. amplitude of peristalsis in the mid esophagus, w/ increased tone and incomplete relaxation at the LES
461
What pathology does this histological image indicate?
Coccidioides immitis
462
List the (6) steps for Ggucose-induced insulin release from beta cells
1. Glucose enters the beta cell through GLUT-2 2. Glucose is metabolized by glucokinase to glucose-6-phosphate 3. Glucose 6-phosphate is further metabolized by glycolysis and the Krebs cycle to produce ATP 4. High ATP to ADP ratio causes closure of ATP-sensitive K+ channels 5. Subsequently, depolarization of beta cells results in opening of voltage-dependent Ca2+ channels 6. High intracellular Ca2+ leads to insulin release
463
Patients with Down Syndrome are at an increased risk for what CA types?
ALL and AML
464
_Torsades de Pointes_ 1. Pathology 2. What are the most common precipitants? (3 categories + examples)
1. Polymorphic v.tach that occurs in the setting of a congenital or acquired _prolonged QT interval_ 2. **Medications** such as certain (1) _antiarrhythmics_ (sotalol, quinidine); (2) _antipsychotics_ (haloperidol); (3) _antibiotics_ (macrolides, fluoroquinolones)
465
When providing a nerve block of the brachial plexus between scalene muscles, what must you be weary of?
Transient ipsilateral diaphragmatic paralysis due to involvement of the phrenic nerve roots as they pass through the interscalene sheath.
466
What is the site of lowest osmolarity in the nephron?
DCT
467
Tx of **A****cute Adrenal insufficiency**
Hydrocortisone or dexamethasone
468
What tumor type(s) is the following tumor marker assc. with?: ## Footnote **PSA**
Prostate
469
Describe the peripheral blood smear associated with lead poisoning
Coarse erythrocyte basophilic stippling and microcytic hypochromic anemia
470
Attributable Risk Percent (definition + calculation)
The excess risk in the exposed population that can be attributed to the risk factor. ARPexposed = 100 x [(RR-1)/RR], where RR = relative risk
471
MOA of SSRIs
Blockade of the serotonin transporter
472
**Patent Ductus Arteriosus (PDA)** 1. Pathology 2. Why is it patent 3. Clinical manifestations 4. Typical age at presentation 5. Tx.
1. Vascular connection between the main pulmonary artery and the aorta still remains after birth 2. Patent due to prostaglandin E2 production by the placenta 3. Clinical features vary by size: _Small_: continuous machine-like murmur w/ no other symptoms. _Large_: progressive pulmonary HTN, reversal of shunt (now right-to-left), ultimately leading to **HF** and **cyanosis** (Eisenmenger syndrome), particularly in lower extemities. 4. Childhood 5. Tx: **Indomethacin** (prostaglandin E@ inhibitors)
473
Name the mealtime insulin analogs Describe their onset/ offset
**Glulisine, Aspart, Lispro** They have rapid onset and offset of action, mimicking physiologic postprandial insulin secretion
474
_Maple Syrup Urine Disease_ 1. Pathogenesis 2. Sx 3. Dx 4. Tx 5.
1. Blocked degradation of **_branched_** amino acids-- **_I_**soleucine, **_L_**eucine, and **_V_**aline (**I L**ove **V**ermont) due to decreased a-ketoacid dehydrogenase 2. Sx: severe CNS defect, intellectual disabiity, and death 3. Urine smells like maple syrup/ burnt sugar 4. Tx: restriction from branched amino acids in diet, and **thiamine supplementation**
475
Describe the key presentations associated with _thiamine deficiency_?
Beriberi and Werknicke-Korsakoff syndrome 1. **Wernicke-Korsakoff**- Confusion + occulomotor abnormalities + memory deficita + ataxia (**COMA**) 2. **Dry beriberi**- characterized by symmetrical peripheral neuropathy 3. **Wet beriberi**- includes the addition of high-output CHF
476
Tissue damage and resultant abscess formation is primarily caused by...
Lyosomal enzyme release from neutrophils
477
Where do the ureters sit in respect to the common, external, and internal iliac?
Anterior (right on top of them)
478
Describe _Aspergillus fumigatus **colonization**_ 1. What is it? 2. How does it look on imaging? 3. Sx
1. Aspergillus develops in old lung cavities (from TB, emphysema, sarcoidosis, etc) and it forms a **fungus ball** w/o tissue invasion 2. Appears on CXR as radioopaque structure which shifts when Pt changes postion 3. Can cause hemoptysis (otherwise asymptomatic)
479
_POMC_, a polypeptide precursor, goes through enzymatic cleavage and modification to produce what (3) proteins?
1. Beta-endorphins (endogenous opioid peptide) 2. ACTH 3. MSH
480
Ethosuximide MOA
Blocks T-type Ca2+ channels in thalamic neurons (used to treat absence seizures)
481
What is _Kinesin_ and what does it do?
A _microtubule-associated_, ATP-powered motor protein that **facilitates the anterograde** transport of neurotranmsitter-containing *secretory vesicles* down axons to synaptic terminals.
482
Describe the activation of Ras proteins (2 steps)
1. Growth factor ligand binds to a **receptor tyrosine kinase** on the cell membrane, causing _autophosphorylation_ 2. Proteins interact with Ras, promoting **GDP removal and GTP binding!**
483
What are the (2) mechanisms by which beta-blockers lower blood pressure?
1. Reducing myocardial contractility and HR 2. Decreasing renin release by the kidney
484
_Antiarrhythmic drugs_ Drugs + Predominant actions **Class III**
**Amiodarone, Dronedarone, Dofetilide, Sotalol** (also class II) *Prolongs APD by blocking K+ channels* (no effect on AP conduction velocity)
485
What effect does sickling (in SCD) have on labs?
Leads to 1. Increased indirect bilirubin 2. Increased lactate dehydrogenase 3. Decreased **haptoglobin** (binds free hemoglobin and is then removed by the spleen)
486
Buerger's disease (thromboangiitis oblierans) 1. Main population demographics 2. Pathophysiology 3. Presentation 4. Tx
1. Heavy smokers, males 2. **Path**- Segmental thromboding vasculitis 3. **Presentation**- Intermittent claudication which can lead to gangrene, autoamputation, and superficial nodular phlebitis. Also associated w/ Raynauds 4. **Tx**: smoking cessation
487
How is nitric oxide synthesized?
It is synthesized from arginine by NO synthase
488
_Ariginase_ 1. Fxn 2. Sx of Arginase deficiency 3. Tx of Arginase deficiency
1. _Fxn_: urea cycle enzyme that produces urea and ornithine from arginine 2. _Sx_: (1) Progressive spastic diplegia, (2) growth delay, (3) abnormal movements 3. _Tx_: Arginine-free, low protein diet
489
Propionyl CoA is derived from which amino acids? (4)
Val, Ile, Met, and Thr
490
What types of drugs are not effective against organisms of the mycoplasma species? What type are? Why?
All of these organisms _lack peptidoglycan cell walls_ and are therefore **resistant to agents that attack this wall**, uch as penicllins, cephalosporins, carbapenems, and vanco. Must treat with _anti-ribosomal agents_ (tetracycline and macrolides)
491
Describe themost important biochemical abnormality in Alzheimer dz Where is this decline most notable?
A dec. in Ach level due to the **deficiency of choline acetyltransferase**, needed to synthesize Ach. Most notable in the _basal nucleus of Meynert_ or the _hippocampus_
492
Why doesn't _Wallerian_ degeneration occur in the CNS?
Due to the persistence of **myelin debris**, secretion of neuronal inhibitory factors, and development of dense glial scarring.
493
What is the best determinate of oral bioavailability of a drug?
AUC (Oral)/ AUC (IV) This assumes they've been administered at equal doses
494
Lipofuscin
Yellow-brown, granular product of lipid peroxidation and considered to be a sign of "wear and tear"/aging.
495
Key AE associated with lamotrigine
SJS (30% of body surface)
496
Relationship between blood flow and radius
Blood flow is directly proportional to vessel radius raised to the fourth power.
497
What virus is the pictured biopsy associated with? What is the normal type of patient who has this virus? Describe the virus?
This biopsy is associated with cytomegalovirus (CMV). This is particuarly common among patients who have recently had _lung transplants_ or are generally immunocompromised. It is an enveloped double-stranded DNA virus
498
_Familial chylomicronemia syndrome_ 1. Which protein is defected? (2) 2. Which lipoprotein is elevated? 3. Major manifestations?
1. _Defected_: Lipoprotein lipase; ApoC-II 2. _Elevated_: Chylomicrons 3. _Manifestations_: **Acute pancreatitis**; lipemia retinalis (milky lipids in retinal vasculature); eruptive skin xanthomas; hepatosplenomegaly
499
a-1 blockers are useful for the treatment of ... (2 conditions) What natural secretion has these same effects?
HTN and benign prostatic hyperplasia (relaxes the bladder) **ANP/BNP** has the same effects
500
What drug is used as abortive therapy during an acute migraine? What is its MOA?
_Triptans_ They are **serotonin 5-HT1B/5-HT1D agonists**, and thus inhibit the release of vasoactive peptides, promoting vasoconstriction, and blocking pain pathways in the brainstem.
501
Fick Principle
An alternative means for calculating cardiac output. CO = O2 consumption/ AV O2 difference
502
What is the role of atypical T-cells in assc. with EBV?
They function to destroy virally-infected B-lymphocytes
503
What is the foundation of management for RA? Why are anti-inflammatory drugs often used?
**DMARDs** (Dz-modifying antirheumatic drugs) such as methotrexate and sulfasalazine are the most key tx. DMARDs take weeks to kick in so short-term tx with anti-inflammatory tx is often used in the meantime.
504
MOA of _Sotalol_
It has both beta-adrenergic blocking properties and class 3 antiarrhtmic (K+ channel blocking) properties. It prolongs the PR interval and the QT interval.
505
1. What do _Central Chemoreceptors_ sense? 2. What do _Peripheral Chemoreceptors_ sense? 3. What do _Pulmonary Stretch Receptors_ sense?
1. _Central_: Increased PaCO2 2. _Peripheral_ (carotid and aortic bodies): PaO2 -- stimulated by hypoxemia 3. _Pulm Stretch:_ Regulates duration of inspiration based on degree of lung distension
506
You see a large cell with multiple nuclei in the shape of a horshoe. What is it called and what disease process is it associated with?
_Langhans Giant Cells._ Formed from macrophages which are activated in response to pulmonary TB.
507
_Drug-induced Lupus Erythematosus_ 1. Presentation 2. Dx 3. What is almost never seen? 4. Drugs normally implicated
1. New onset of lupus symptoms 2. Anti-nuclear antibodies (ANA) and anti-histone antibodies 3. Anti-dsDNA **_ALMOST NEVER SEEN_** 4. _HIP_: **_H_**ydralazine, **_I_**soniazid, **_P_**rocainamide
508
_Amatoxins_ 1. Where are they found? 2. What is there MOA?
1. Found ina variety of posionous mushrooms 2. Potent inhibitors of RNA pol, thus halting mRNA synthesis
509
What (3) parts of the body does the **_iliohypogastric nerve_** innervate?
_Sensation_ to the **suprapubic** and **gluteal** regions _Motor function_ to the **anterolateral abdominal wall muscles**
510
Potential complications from hereditary spherocytosis (2)
1. Pigmented gallstones 2. Aplastic crises from parvovirus B19 infection
511
What type of cells are seen in the pulmonary vasculature in cases of an Amniotic fluid embolism?
**Fetal squamous cells**
512
Path and Presentation of patients with 21-hydroxylase deficiency
Deficient cortisol and aldosterone synthesis + adrenal androgen overproduction _Males_: normal genitalia + vomiting, hypoTN, hyponatremia, hyperkalemia _Females_: ambiguous genitalia (+ sx above)
513
_Meningiomas_ 1. Pathophysiology? 2. Who is usually affected? 3. Where do these tumors occur? 4. Presentation?
1. Common, slow-growing (benign), intracranial tumors 2. Typically adults 3. Arise in regions of dural reflection (falx cerenri, tentorium cerebelli, etc.) 4. Patients are often asymptomatic but may present w/ HA, seizure or focal neurologic deficits depending on size/ location of tumor
514
What molecules take on a trple helical conformation?
Collagen, due to repetitive AA sequences within each alpha chain
515
1. Name the non-selective beta-blockers 2. Name the selective beta-blockers 3. Why would you choose selective over non-selective?
1. _Non-Selective_- Propanolol, timolol, and nadolol 2. _Selective_- metoprolol, atenolol, acebutolol and esmolol 3. Selective only target B1, so if you have patients w/ COPD/asthma, you want to only use these.
516
What are the type II Anti-arrhythmics?
Beta-blockers
517
The (2) most signficant risk factors for development of esophageal SCC in the US
Smoking tobacco and drinking alcohol
518
What is Annular Pancreas? What might it cause?
The _ventral pancreatic bud_ abnormally encircles the 2nd part of the duodenum, forming ring of pancreatic tissue. May cause **duodenal narrowing.**
519
_Amyotrophic Lateral Sclerosis_ 1. Sx 2. Gross anatomy 3. Histo/ microscopic 4. Genetics 5. Tx
1. **Sx**: _LMN_- muscle weakness, atrophy, fasiculations; _UMN_- spasticity, hypereflexia, pathologic reflexes 2. **Gross**: (1)Thin anterior roots; (2) mild atrophy of precentral gyrus 3. **Histo/micro**: loss of anterior horn neurons, lateral CSTs, motor nuclei neurons, and denervation atrophy of muscles 4. **Genetics**: mutation of gene for superoxide dismutatse (SOD1) may be implicated 5. **Tx**: _Riluzole_ (decreases glutamate release)
520
1. _Peroxisomes_ are responsible for metabolizing what? 2. What does _Peroxisomal disease_ commonly lead to?
1. Metabolizes very long chain fatty acids or fatty acids with branch points at odd-numbered carbons since they cannot undergo mitochondrial beta-oxidation 2. Can lead to neurologic defects from _improper CNS myelination_
521
Name the muscles of the _rotator cuff_ and what each one does
**SITS** **_S_**upraspinatus- abducts arm initially (before deltoid takes over) **_I_**nfraspinatus (laterally rotates arm) **_T_**eres minor (adducts and laterally rotates arm) **_S_**ubscapularis (medially rotates and adducts arm)
522
_Ataxia Telangiectasia_ 1. Classic triad 2. Specific defect
1. Cerebellar ataxia + telangiectasias + inc. risk of sinopulmonary infections 2. Defect in the gene that encodes for the ATM gene, which plays a role in DNA break repair
523
_Cryptococcus neoformans_ 1. How do they look? 2. Who do they affect/ how is it passed? 3. Most common disease processes caused 4. Dx
1. Budding yeasts w/ thick capsules 2. Passed via pigeons/soil and affect only **immunocompromised** 3. _Meningoencephalitis_ is the most common disesase caused 4. India Ink/ Mucarmine
524
_Hypertrophic Cardiomyopathy_ 1. Key potential clinical consequences (2)? 2. Histo 3. Pathology 4. Dx of this consequence? 5. What actions make those sx better or worse?
1. Left ventricular outflow obstruction; sudden death in stressful situation 2. Extreme myofiber disarray w/ interstitial fibrosis 3. Mutations in genes encoding cardiac sarcomere proteins 4. Harsh crescendo-decrescendo systolic ejection-type murmur best heard along left sternal border and apex 5. * Mechanisms dec. preload or afterload _increase obstruction_ -- such as sudden standing or nitro. * Mechanisms inc. preload or afterload _decrease obstruction_ -- such as squatting, sustained hand grip, or passive leg raise
525
Describe the pathology of _Good Pasture Syndrome_
It is a Type II hypersensitivity, in which there are antibodies to the glomerulus basement membrane and alveolar basement membrae
526
_Asbestos exposure_ 1. Potential clinical manifestations? (3) 2. What two cancer types can Asbestos lead to? Which is most common?
1. (1) Pleural thickening + (2) calcified lesins of the posterolateral midlung zones/diaphragm + (3) occasional pleural effusions 2. **Bronchogenic carcinoma** (most common and can occur w/o asbestos) and **malignant mesothelioma** (rare but more specific to heavy asbestos exposure)
527
What antibodies have a high specificity for Rhematoid arthritis?
Antibodies to citrullinated peptides/ proteins (anti-CCP)
528
1. _Inhibition of uterine contractons_ is a result of stimulation of which adrenergic receptor? 2. _Pupillary dilation_ is the result of stimulation of which adrenergic receptor?
1. B2 (inhibits uterus) 2. a2 (dilates pupils)
529
Dactylitis (What is it and what is it a common presentation of?)
Painful swelling of the hands and feet It is a common presentation of sickle cell in young children.
530
What does the radial nerve do? Damage to it may result in what?
1. Innervation to the skin of the posterior arm, forearm, and dorsal lateral hand 2. Motor innervation to all of the extensors of the upper limb (below the shoulder) Damage leads to _wrist drop_
531
What phase of the AP corresponds with the QRS of the EKG? QT?
QRS = Phase 0 QT = Phase 3
532
Inherited defects involving what signaling pathway, result in _disseminated mycobacterial dz_ in infancy/ early childhood?
**Interferon-gamma** | (needed to activate macrophages)
533
What hormone is often increased in liver cirrhosis? What can this lead to?
**Estrogen** (leading to gynecomastia + spider angiomas + hair loss + testiclar atrophy)
534
Which neural structure is most affected by the thiamine deficiency associated with Wernicke encephalopathy?
Mamillary bodies
535
What specific component of the H. Flu capsule is the vaccine set against?
**Polyribosyl-ribitol-phosphate** (PRP)
536
What is the "Number Needed to Treat", and how is it calculated?
It's the number of patients that need to be treated with a medicatin to avoid a negative outcome. It is 1 divided by the percent difference in outcome between the control and the experiment
537
What are the main effects of a glossopharyngeal nerve lesion?
1. Loss of **gag reflex** 2. Loss of **general sensation** of the upper pharynx, posterior tongue, tonsils and middle ear cavity 3. Loss of **taste** on posterior third of tongue
538
Diffuse muscle pain + fatigue +neuropsych disturbances, w/ negative labs = what dz?
Fibromyalgia
539
What is a pancoast tumor? Where is the most likely location for a pancoast tumor to occur? What is the most common presentation?
Carcinoma in the apex of the lung. These usually arise in the superior sulcus (groove fromed by the subcalvian vessels) _Common presentation_: 1. Pt. w/ extensive smoking history 2. **Shoulder pain-** _most common symptom_ 3. Horner syndrome 4. Pain in distribution of C8-T2 roots (spinal cord compression)
540
What is the stabilizing force for the secondary structure of proteins?
Hydrogen bonds
541
_Lesch-Nyhan syndrome_ Key presentation components (2)
1. Self-mutilation 2. Hyperuricemia
542
What are the classic sputum findings for Extrinsic Allergic Asthma
Eosinophils and Charcot-Leyden crystals (crystalloid bodies containing eosinophil membrane proteins).
543
_H. flu_ 1. What does it require in order to grow? 2. What does its pathogenicity depend on? 3. Which strain is most invasive? 4. How does it present in unvaccinated individuals?
1. Requires both **X factor** (hematin) and **V factor** (NAD+) to grow 2. Pathogenicity depends on the presence of a _capsule_ 3. Type B strain = most invasive and virulent 4. _Type B causes_- severe epiglottitis, meningitis, and bacteremia; _Non-type B_ causes noninvasive sinusitis, bronchitis, otitis media and conjunctivitis
544
*Streptococcus gallolyticus* (formely S. Bovis) can cause **endocarditis** and **bactermia**. When this bug is cultured in the blood, workup for _what_, is absolutely essential?
**_Colonic malignancy_** with colonoscopy
545
Explain the rapid onset and short duration of action for drugs similar to propofol
They are **_lipophilic_** and thus readily diffuse across membranes. First accumulate in tissues with _high blood flow_ (like brain, hence rapid onset), then dubsequently redistributed to organs receiving less blood flow, like fat or muscles (hence short duration)
546
What substances are capable of inactivating _enveloped_ viruses?
**Organic solvents** (ex. _Ether_) [Non-enveloped viruses are generally resistant to the action of ether]
547
_Aspirin_ MOA
Aspirin impairs prostaglandin synthesis by irreversibly inhibiting COX. Inhibition of COX-1 in platelets prevents synthesis of thromboxane A2, a potent stimulator of platelet aggregation and vasoconstriction.
548
Under normal cricumstances, how does Ras protein become inactivated?
It has intrinsic GTPase activity , that allows it to hydrolyze the attached GTP
549
_Verrucous endocarditis_ 1. Who does it occur in? 2. What is it? 3. Potential consequence?
1. Occurs in up to 25% of patients with SLE 2. Can cause small cardiac valvular vegetations on either side of a valve, resulting in fibrotic valve thickening and deformity 3. May cause acute coronary syndrom in young patients with **normal coronary arteries**
550
Abciximab MOA
It blocks the GP IIb/IIIa receptor, which normally promots platelet binding to fibrinogen
551
_Antiphospholipid Antibody Syndrome_ 1. Type/ cause of antibodies 2. Sx (2)
1. Antiphospholipid antibodies (either primary or due to **_SLE_**) 2. Venous or arterial thromboembolism (in the presence of **paradoxical inc. PTT)** + recurrent pregnancy loss
552
_Carbamazepine_ 1. Indication 2. MOA 3. Adverse Effects
1. Seizure tx 2. Blockage of _voltage-gated sodium channels_ in neuronal membranes 3. Can cause **bone marrow suppression**
553
What is the pathognomnic presentation for a large patent ductus arteriosus complicated by Eisenmenger syndrome?
Differential clubbing and cyanosis w/o blood pressure or pulse discrepancy
554
_Giant Cell Arteritis_ 1. Also known as? 2. Main Sxs (4) 3. What does the artery look like on biopsy?
1. aka _Temporal_ arteritis 2. **Jaw Claudication**, HA, facial pain, and vision loss 3. Temporal artery biopsy demonstrates granulomatous inflammation of the media
555
Leuprolide MOA
Continuous GnRH agonist
556
What is the specific deficiency in patients with Glanzmann thrombasthenia?
(GP) IIb/IIIa, needed for platelet aggregation
557
Name the (4) key _neutrophil_ chemotactic agents during inflammation
1. Leukotriene B4 2. 5-HETE (leukotriene precursor) 3. C5a (complement component) 4. IL-8
558
Which bug is associated with right-sided endocarditis in IV drug users?
Staph aureus
559
What is the best _ausculatory_ indicator of the severity of a patient's **mitral regurgitation**?
**Presence of an audible S3** (indicating a large volume of regurgitant flow re-entering the ventricle during mid-diastole)
560
Describe the histo of myxomas
Scattered cells within a mucopolysaccharide stroma, with abnormal blood vessels and hemorrhaging
561
Name the precursor protein/peptide responsible for localized amyloidosis for each of the following organs: 1. Cardiac atria 2. Thyroid gland 3. Pancreatic islets 4. Cerebrum/cerebral blood vessels 5. Pituitary gland 6. _Multi-organ_ amyloid deposition?
1. Cardiac atria: **atrial natriuretic peptide** 2. Thyroid gland: **calcitonin** 3. Pancreatic islets: **islet amyloid protein** 4. Cerebrum/cerebral blood vessels: **B-amyloid protein** 5. Pituitary gland: **prolactin** 6. Multi-organ amyloid deposition? **Immune globulin light chains**
562
What do RAS genes code for?
A family of small G-proteins involved in **signal transduction** in the Ras-MAPK pathway
563
Distinguish Precursor B-ALL from precursor T-ALL
This can only be done via immunophenotyping **B-ALL**: TdT+, _CD10+_ and _CD19+_ **T-ALL**: TdT+, CD1a+, and + for T-cell markers (CD2 , 3, 4, 5, 7, and 8)
564
_Chronic Granulomatous Disease_ 1. Pathogenesis? 2. Clinical Manifestations?
1. _Pathogenesis_: Inactivating mutation affecting **NADPH oxidase.** Impaired respiratory burst inhibits phagocytic _intracellular killing_ 2. _Clinical Manifestation_: **Recurrent infections** in child w/ catalase (+) bacteria/fungi (commonly in lungs, skin, LNs and liver). Diffuse **granuloma formation**
565
_Acute Arsenic Posioning_ 1. Presentation 2. Source 3. Antidote
1. **Garlic odor breath** + diarrhea + N/V 2. Insecticides 3. Cure = Dimercaprol
566
What is the most common cause of death in patients who have been struck by lightning?
Cardiac arrhythmias/ arrest
567
Oligodendrocytes Vs Schwann Cells
_Oligodendrocytes_- CNS myelin (oligarchy means central power) _Schwann_- Peripheral Myelin
568
_Pigment gallstones_ 1. Composition 2. Appearence 3. Cause (including key enzyme) 4. What organism is often associated
1. Composed of Ca2+ salts of unconjugated bilirubin 2. Soft, dark brown or black 3. Typically arise secondary to **bacterial** or **helminthic infection** of the biliary tract. **_Beta-glucuronidase_**, released by injured hepatocytes and bacteria, hydrolyzes bilirubin glucorinides to unconjugated bilirubin 4. The liver fluke *Clonorchis sinensis* (high prevalence in East Asian countries), is a common cause
569
How do chronically elevated FFA levels contribute to insulin resistance?
By impairing insulin-dependent glucose uptake and increasing hepatic gluconeogenesis
570
What is the most frequent cause of lung abscess and what type of conditions increase their risk. Name (1) key example of this cause. Tx (2)?
**Anaerobic bacteria** normally found in the oral cavity are the most common cause. Risk factors include anything that increases _aspiration risk_ (seizure, alcholism, drug abuse, stroke, dementia, etc.) Ex: **Actinomycosis** Tx: Penicillin or Clindamycin (if hypersensitive to Penicillin)
571
What is the primary site for complement production?
The liver
572
Kehr sign
**Referred shoulder pain** due to irritation of the phrenic nerve sensory fibers around the diaphragm as the **result of some abdominal process** (ruptured spleen, peritonitis, hemoperitoneum)
573
_Mitral Stenosis_ 1. What is the best/most reliable asuculatory indicator of the severity?
1. The interval between A2 and the opening snap (OS). **Shorter interval = more severe stenosis**
574
_Germinoma_ 1. What type of mass is it? 2. Classical manifestations (2) and the Sx associated with each (3) 3. Dx
1. Pineal gland mass 2. (1) **Obstructive hydrocephalus** due to aqueductal stenosis - Sx: papilledema, HA, vomiting (2) **Dorsal midbrain (Parinaud) syndrome** due to direct compression of the pretectal region of the midbrain- Sx: paralysis of upward gaze, ptosis, pupillary abnormalities 3. Dx via _MRI_
575
Define accuracy vs reliability
_Accuracy_: the degree to which the aerage measurment value matches that of the gold standard _Reliability_: reproducibility of a result
576
What is hypocapnia and what does it imply for a patient?
A state of reduced CO2 in the blood. Always implies alveolar hyperventilation.
577
_Meconium Ileus_ What is it and what disease is it associated w/?
Small bowel obstruction due to abnormally dehydrated **meconium** in a patient w/ _cystic fibrosis_
578
What are "Lichtenberg figures" and what are they associated with?
Erythematous cutaneous marks in a fern-leaf pattern. They are pathognomonic of **_lightning strikes_**
579
_Primary Biliary Cirrhosis/ Cholangitis_ 1. What characterizes this dz? 2. Who is it common in ? 3. Presentation?
1. Chronic liver dz characterized by autoimmune destruction of the intrahepatic bile ducts and cholestasis (elevated alk phos) 2. Most common in middle aged women 3. _Sx_: Severe pruritis (especially at night)
580
How does _A-fib_ look on an EKG? In this condition, what ultimately regulates the number of atrial pulses which reach the ventricle?
* On _EKG_, it is characterized by: **absent P waves, irregularly irregular R-R intervals,** and **narrow QRS** * Ventricular response is based on transmission of the abnormal impulses through the AV node. _The AV node refractory period regulates the number of impulses._
581
_Naltrexone_ 1. Indication 2. MOA 3. Effect
1. 1st line treatment for moderate to severe alcohol use disorder 2. Blocks the mu-opiod receptor 3. **Effect**: blocks the "rewarding and reinforcing" effects of alcohol, thus reducing cravings
582
What type of MHC do APCs express?
MHC Class II so that they can present antigen to CD4 cells
583
_Zolpidem_ 1. Indication 2. SImilar to what drug? How does it differ?
1. Short-acting hypnotic used for **short-term insomnia** 2. Same MOA as benzos but a much lower risk of tolerance and dependence
584
_Alkaptonuria_ 1. Inheritance 2. Enzyme that is deficient 3. Presentation (3)
1. Autosomal-recessive disorder 2. Deficiency of **homogentisic acid dioxygenase** (an enzyme involved in tyrosine metabolism) 3. _Presentation_: (1) diffuse blue-black deposits in connective tissues, (2) adults have slerae and ear cartilage hyperpigmentation, (3) osteoarthropathy of spine and large joints
585
1. What is the cause of _umbilical hernias_? 2. What conditions are they associated with?
1. Incomplete closure of the umbilical ring 2. **D**at **B**ig **H**ernia --\> assc. w/ **_D_**own Syndrome, **_B_**eckwith-Wiedmann syndrome, **_H_**ypothyroidism
586
What tumor type(s) is the following tumor marker assc. with?: ## Footnote **CA 125**
Ovarian
587
_Candida Albicans_ 1. What does it give rise to when heated? 2. Where is it found normally in humans?
1. Gives rise to true hyphae when incubated at 37 degrees C 2. Normally found in the **GI tract** (including the oral cavity) and thus is a common contaminant of sputum cultures
588
Positive Predictive Value
The proportion of indivudals with positive test results who actually have the dz PPV = TP/ (TP + FP)
589
_Dobutamine_ 1. MOA 2. Indication 3. Effect (3) 4. AE
1. B-agonist (predominant activity on B1 receptors) 2. Management of refractory HF w/ severe LV systolic dysfunction/ cardiogenic shock 3. (1) Positive **inotropic** effect (2) weakly positive **chronotropic** effect (3) mild **vasodilation** 4. Some increased O2 consumption due to increased chronotropy
590
Role of B-blockers and a2 agonists in tx of glaucoma
Decrease **secretion** of aqueous humor via the _ciliary epithelium_
591
In the _pathogenesis of atherotic plaques_, What is responsible for promoting migration of smooth muscle cells from the media into the intima, and subsequent proliferation?
The release of _Platelet-derived growth factor (PDGF)_ by locally adherrent platelets, endothelial cells, and macrophages.
592
Describe the common presentation for sarcoidosis
1. Hilar adenopathy 2. Pulmonary infiltrates 3. Non-caseating granuloma 4. AA Woman
593
What are the (3) main sx of _vitamin A_ overuse?
1. Intracranial HTN 2. skin changes 3. Hepatosplenomegaly
594
Describe the process of granuloma formation for TB
1. Macrophages eat m. tuberculosis 2. **Macrophages** present to T cells and also secrete **IL-12** 3. T-cells differentiate to **Th1** 4. Th1 produces **IFN-y** which activates macrophages 5. Activated macrophages produce **TNF-a**, increasing recruitment, allowing for walling off of infection
595
Resistance in the airway is maximal between which bronchial generations?
Generations 2-5 (then drops off due to high cross-sectional area)
596
Main difference between MDD and Persistent depressive disorder (dysthymia)
Persistent DD is \> 2y, while MDD is greater than 2 weeks (usually 6-12mo) Also Pers. DD tends to be milder
597
After cutting through fascia, what else is cut during a cricothyrotomy?
The **cricothyroid membrane**
598
Achlorydia
Lack of HCl in gastric secretions
599
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ is almost always needed in order to develop acute pyelonephritis (as opposed to regular UTI)
Vesicoureteral reflux
600
What key structure is assc. with ovarian torsion (other than the ovary)?
The **infundibulopelvic ligament** (IP). It suspends the ovaries and has blood vessels/nerves for the ovary running through it.
601
How can untreated hydrocephalus in an infant lead to UMN issues?
Stretching of the periventricular pyramidal tracts
602
_Acute acalculous cholecystitis_ 1. What is it? 2. Who does it occur in?
1. Acute inflammation of GB in **_absence_** of *gallstones* 2. Occurs in critically ill patients (and thus has very poor prognosis)
603
Why is Piperacillin-tazobactam such an effective tx for *Bacteroides*?
Bacteroides (gram-negative, anaerobic rods) produce **_B-lactamase_**, an enzyme that breaks down penicillins. Tazo inhibits the B-lactamase, so that piperacillin can do its job.
604
What type of brain damage leads to decerebrate (extensor) posturing?
Damage at or below the midbrain tegmentum, pons (below the red nucleus)
605
What type of brain damage leads to decorticate (flexor) posturing?
Damage w/in the cerebral hemispheres (above the red nucleus)
606
What hormone is ultimately responsible for causing gestational diabetes?
**Human placental lactogen (hPL)** It decreases maternal fatty acid stores and increases maternal serum glucose, but also increases insulin resistance
607
Describe the path the median nerve takes prior to the carpal tunnel
1. Courses between the humeral and ulnar heads of the pronator teres muscle 2. Runs between the flexor digitorum superficialis and the flexor digitorum profundus muscles (like meat in a sandwich) 3. Crosses wrist w/n carpal tunnel
608
What is the most sensitive screening test for dx od primary hypothyroidism?
Serum TSH | (even more sensitive than T3/T4)
609
The production of what cytokine is a very important driver of Giant Cell Arteritis?
IL-6 (cell mediated immunity is the primary mechanism underlying this dz)
610
Why should protein be restricted in an ornithine transport deficiency?
Ornithine is a crucial part of the **_urea cycle_**, needed to breakdown protein. Defects can lead to neurological damage, due to ammonia accumulation.
611
Which antibiotic groups disrupt the peptidoglycan cell wall of bacteria? (3)
1. Penicillins 2. Cephalosporins 3. Vancomycin
612
What compensatory pathway can help convert _fructose_ to a useable form, in cases of _essential fructosuria_?
The **hexokinase pathway** can be used, if the _fructokinase pathway_ is broken (as it is in the case of essential frucosuria)
613
First-order kinetics vs zero-order kinetics
_First_: a constant proportion of the drug is metabolized per unit time (based on serum levels) _Zero_: a constant amount of drug is metabolized per unit time (independent of serum levels)
614
(2) key histo findings in _Glioblastoma_ Multiforme
1. Pseudopalisading 2. Central areas of necrosis + hemorrhage (hence, multiforme)
615
_Cholesteatoma_ 1. What is it? 2. Etiology 3. Presentation
1. Collection of squamous cell debris that forms a pearly mass behind the tympanic membrane 2. Congenital or acquired following infxn, trauma or middle ear surgery 3. Can cause hearing loss
616
What are the (2) normal arrest points for oocytes?
1. **Prophase** of _meiosis I_ (prior to ovulation) 2. **Metaphase** of _meiosis II_ (prior to fertilization)
617
What tissue types have B1 receptors?
Cardiac and Renal JGA cells
618
What is the major immune mechanism against *Giardia*?
secretory **_IgA production_**
619
_Viral bronchiolitis_ 1. Cause 2. Presentation (4) 3. Dx
1. **RSV** 2. Low-grade fever, cough, tachypnea, inc. work of breathing 3. Wheezing + crackles
620
What type of bacteria is *Gardenella vaginalis*?
An anaerobic gram-variable rod
621
Describe the morphology of *cryptococcus neoformans*
Budding yeast
622
What nerve innervates the _cricothyroid muscle_?
The external branch of the superior laryngal nerve
623
What is the optimal site for a _femoral nerve block_ and what becomes anesthetized?
1. Inguinal crease = optimal site 2. Skin and muscles of the anterior thigh (e.g. quads), femur, and knee
624
Why can _PAH_ be used to estimate Effective Renal Plasma Flow (eRPF)?
Because it is both filtered and secreted in the proximal collecting tube (PCT), resulting in nearly 100% excretion of PAH by the kidneys.
625
Progressive onset of heart failure in the setting of recent viral infection should raise suspicion for what?
Dilated cardiomyopathy caused by viral myocarditis
626
What are the values for serum Ca2+, phosphorous, and PTH in _primary osteoporosis_? (low, normal, high-- for each)
All normal
627
What is the most efficient way to decrease cerebral blood flow/ ICP in a patient?
Hyperventilate. This will blow off CO2, leading to a vasoconstriction.
628
Presentation of _Porphyria cutanea tarda_
Blistering cutaneous photosensitivity
629
Cardiac defect assc. with Turner's syndrome
Bicuspid aortic valve + coarctation of the aorta
630
What is the triad of sx assc. with _Serotonin Syndrome_?
1. **Autonomic instability** (hyperthermia, HTN, tachycardia) 2. **AMS** 3. **Neuromuscular hyperactivity**
631
How is Linezolid able to cause Serotonin syndrome?
It has MAOI activity, so combined with an SSRI, it can precipitate serotonin syndrome
632
Why are thiazides effective for some patients with nephrolithiasis?
They _increase Ca2+ reabsorption from the nephron_, so are effective for calcium based nephroliths
633
What does **arteriovenous concentration gradient** tell us about the _tissue solubility_ and _onset of action_ of an anesthetic?
Anesthetics with _high tissue solubility_ are characterized by **large** AV concentration gradients and **slower** onsets of action.
634
Ultimately, what are _vegetations_ assc. with infective endocarditis, composed of?
(1) sterile fibrin-platelet nidus + (2) bacterial colonization
635
Craniopharyngiomas commonly show what?
Calcification
636
CN III courses between which 2 vessels?
SCA and PCA
637
Put Alcohol w/drawal symptoms in order (4)
Mild w/drawal (tremors, diaphoresis, etc.) --\> seizures --\> alcoholic hallucinosis --\> delirium tremens
638
Describe the breakdown of _heme_ to _unconjugated bilirubin_
**Heme --\> Biliverdin** (via heme oxygenase) **Biliverdin --\> unconjugated bilirubin** (via biliverdin reductase)
639
A patient has hypoxemia, in the setting of a normal A-a gradient. What (2) differentials should you consider?
1. Alveolar hypoventilation 2. Inspiration of air at high altitude
640
Which vessels are most likely to be penetrated by gastric ulcers? Why?
**The left and right gastric arteries.** They run along the lesser curvature of the stomach, where the great majority of ulcers occur.
641
Why do individuals who've experienced excessive weight loss or who excercise strenously, often miss menstruation?
Decreased _leptin_, leads to decreased _GnRH_ secretion via the **_hypothalamus_**
642
What is most responsible for the increase in nosocomial bloodstream infections?
Intravascular devices (indwelling catheters)
643
Where does fluid accumulate in cases of _communicating hydrocele?_
W/in the **_tunica vaginalis_**
644
Is *S pyogenes* _PYR-positive or PYR-negative?_
(PYR)-positive
645
In a pregnant woman, which drugs are the best tx choice for for DVTs?
Low Molecular Weight Heparins
646
What substances allow for a prenatal dx of NTDs?
The presence of alpha fetoprotein and AchE in the amniotic fluid (or even serum for AFP)
647
For what reason does Tamoxifen rely on CYP2D?
Tamoxifen is a prodrug and relies on CYP2d to become activated, as _endoxifen_
648
What region of the LN is populated primarily by T lymphocytes (as well as dendritic cells)?
The paracortex region
649
The transverse portion of the duodenum (the bottom part of the C), runs between which (2) vessels?
Aorta and SMA
650
Why is their a major increase in the density of _connexins_ in the uterus just before delivery?
Connexins assemble into **gap junctions** which are needed to facilitate communication and coordination between cells preparing for labor contractions. They increase in response to rising estrogen levels.
651
What component of the tRNA serves as the AA binding site?
3'CCA tail (loaded by aminoacyl tRNA synthetase)
652
Where does lymph from the testes drain? The scrotum?
_Testes_: para-aortic LNs _Scrotum_: superficial inguinal LNs
653
Dissection of what nerve leads to "winged scapula", and why?
The _long thoracic nerve_. The results in weakness of the **serratus anterior muscle.** (leading to winged scapula and impaired abduction of the shoulder beyond the horizonal)
654
What types of renal injury can be caused by NSAIDs? (2)
Chronic interstitial nephritis + papillary necrosis
655
What role do _neurophysins_ play for oxytocin and vasopressin?
They are involved in posttranslational processing and stabilization w/in neurosecretory vesicles during transport to the posterior pituitary.
656
What are the (2) main roles that NADPH plays?
1. Reduction of glutathione (i.e. prevention of oxidative damage) 2. Biosynthesis of cholesterol, fatty acids, and steroids
657
Why are patients who've experienced organophosphate poisoning still at risk for _muscle paralysis_, even after being given atropine?
Atropine only reverses _muscarinic_ _sx_ of the poisoning. It has no effect on the **nicotinic receptors**, which cause the muscle paralysis (need pralidoxime→**PAM**)
658
Why must you be careful not to overuse a-adrenergic agonists as decongestants?
Overuse causes negative feedback, resulting in dec. NE synthesis and release from nerve endings, diminishing their effect
659
*Candida albicans* is a normal inhabitant of what?
The GI tract (including the oral cavity)
660
Morphine MOA
Binds to mu receptors, resulting in G-protein activation of potassium conductance. This hyperpolarizes the postsynaptic neurons, blocking pain transmission
661
Most common cause of _galactosemia_
Deficiency in galactose-1-phosphate uridyl transferase (GALT)
662
Describe the characteristic histopathological findings assc. with _HIV-associated dementia_
**Microglial nodules** (activated macrophages/giant cells around center of necrosis)
663
_Thoracic outlet syndrome_ 1. Pathlogy 2. Presentation 3. What is it associated with?
1. Compression of lower trunk of brachial plexus in the scalene triangle (bordered by anterior/ medial scalene and first rib) 2. **Sx**: uper extremity numbness, tingling, and weakness 3. Having an extra rib
664
**Gout or pseudogout:** associated with myeloproliferative disorders
Gout
665
Tx of _Wilson Dz_
D-penicillamine (copper chelating agent)
666
In females, the paramesonephric ducts fuse to form, what? (4 things)
1. Fallopian tubes 2. Uterus 3. Cervix 4. Upper vagina
667
What does the liver do in attempts to compensate for protein loss in nephrotic syndromes?
Increase its synthesis of a bunch of other proteins (including VLDL, LDL, and HDL). This often leads to lipiduria.
668
_P bodies_ play an important role in protein synthesis. Where do they operate/ what is their general role?
They work in the _cytoplasm_. Play an important role in mRNA translation regulation and mRNA degradation
669
If a patient presents with mono, but has a negative agglutination to horse erythrocytes, what is the likely dx?
CMV (agglutination to horse erythrocytes = **_monospot test_**)
670
Tx for *Chlamydia trachomatis*
Doxycycline or azithromycin
671
IV tx to lower BP in hypertensive emergency. What is its effect (3)
_Fenoldopam_ (dopamine-1 receptor agonist) 1. Arteriolar dilation 2. Inc. renal perfusion 3. Promotes diuresis and natriuresis
672
_Neuroleptic Malignant Syndrome_ 1. Etiology 2. Sx 3. Tx
1. Rxn to antipsychotic drugs **FEVER** (**F**ever, **E**ncephalopathy, **V**itals unstable, **E**nzyme inc., **R**igidity of muscles) Tx w/ _Dantrolene_
673
Rapid correction of chronic hyponatremia, may lead to what?
**Central pontine myelinolysis** (osmotic demyelination of the axons in the central pons). Can cause quadriplegia.
674
How do you reduce the risk of "wrong-site surgery"?
Dual identifiers (usually a nurse and physician)
675
What is the appropriate attire for C-dif?
Nonsterile gloves + gown
676
Describe the morphology of _acute transplant rejection_
It can have a _humoral_ and _cellular_ component * **Humoral**- necrotizing vasculitis with a neutrophilic infiltrate * **Cellular**- lymphocytic interstitial infiltrate and endotheliitis
677
What does it mean when we say that the _lac operon_ is polycistronic?
It means that the one mRNA codes for several proteins
678
What is the specific defect associated with _Fragile X syndrome_?
An X-linked trinucleotide repeat disorder (**CGG**)n which affects _methylation_ and expression of the _FMR1 gene_
679
Explain how _A fib_, with subsequent _abdominal pain_, can lead to **acidic urine**.
1. A fib can lead to acute ischemic colitis 2. Ischemia forces bowel to use anaerobic respiration 3. Anion gap acidosis occurs 4. Kidneys generate **ammonia** and bicarb (via conversion of _glutamide to glutamine_), so **bicarb** can be used to buffer blood 5. **Ammonia is urinated out** (hence acidic urine!!!)
680
What is the difference in fxn between free and attached ribosomes?
**_Free_** = produce proteins for the cytosol **_Attached_** = produce proteins for cell membrane/ secretion
681
Which diuretics are best for improving survival in patients with CHF and decreased EF?
Mineralocorticoids (eg, spironolactone, eplerenone, etc.)
682
If the rectus abdominis muscle is incised laterally, what vessel is at greatest risk of injury?
The _inferior epigastric artery_
683
How can left-sided heart dz, lead to pulmonary _vasoconstriction_?
The backflow of blood causes damage to the endothelium, which causes impaired NO function and endothelin release.
684
Sx associated with hep C
Hep C is typically _asymptomatic_! There may be skin manifestations in chronic Hep C.
685
What determines which side of the coronary circulation is dominant?
Attachment to the **Posterior descending/interventricular artery** **(PDA)**
686
The PDA (posterior descending/interventricular artery) supplies blood to what key structure?
The AV node (via the AV nodal artery)
687
Osteocytes send signals and nutrients/waste to one another via what structure?
Gap junctions
688
What is the major cause of morbidity and mortality in patients recovering from SAH? How do you prevent this?
Severe vasospasm Tx w/ nimodipine to prevent
689
_Biliary Atresia_ 1. Presentation 2. Dx 3. What is seen on Biopsy?
1. dark urine + jaundice + clay colored stools in first months of life 2. Dx: conjugated hyperbilirubinemia and take biopsy 3. Biopsy reveals duct prolifeation, portal tract edema, and fibrosis
690
Name the only medication which selectively decreases HR but has _no effect_ on myocardial contractility or relaxation. What is its MOA?
**Ivabradine** Selective inhibition of funny sodium channels (If)
691
Name the toxicities associated with _aminoglycosides_ (2)
1. nephrotoxicity 2. ototoxicity
692
a2 receptor effect on insulin secretion? B2 receptor effect? Which predominates
**a2**: inhibits insulin secretion **B2**: promotes insulin secretion a2 predominates when both are activated
693
Tx of _Restless Leg Syndrome_
Dopamine agonists
694
Cytokeratin is a commonly-used immunohistochemical marker for what?
Epithelial-derived tissues
695
What symptoms characterize _Atypical Depression_? Tx?
1. **Mood reactivity** (ex. feeling better n response to positive events) 2. **Leaden paralysis** (heavy arms/legs) 3. **Rejection sensitivity** 4. **Increased sleep/appetite** _Tx_: MAOIs
696
What is one of the rare tissues to have an anabolic _increase_ in protein synthesis in response to longterm glucocorticoid tx?
The **liver**, due to increased hepatic gluconeogenesis and glycogenesis (contributes to the hyperglycemia seen).
697
What is the main MOA of all hormone containing Oral Contraceptives?
Inhibition of ovulation by dec. FSH and LH synthesis
698
Ristocetin aggregation test is used to test for what?
von Willebrand dz (should show decreased aggregation of platelets)
699
A rxn to Poison Ivy, is a form of what?
Allergic contact dermatitis (type IV hypersensitivity)
700
A patient has weakness during finger and thumb extension (w/o wrist drop or sensory deficits). ## Footnote **Most likely site of injury?**
_Radial nerve_ during its passage through the supinator canal
701
Most important tx for Diptheria?
Diphtheria antitoxin (passive immunization)
702
Nitroglycerin acts primarily on which vessels?
**LARGE _VEINS_**!!! (It's a venodilator, which allows it to decrease preload)
703
Best mechanism for Dx of vertebral osteomyelitis
MRI of the spine
704
What type of drugs are preferentially metabolized and eliminated by the liver?
Drugs w/ high _lipophilicity_
705
What connects the 3rd ventricle to the 4th ventricle?
Cerebral aqueduct
706
What drains the 4th ventricle?
Lateral foramina of Luschka and Median foramen of Magendie
707
_DRESS Syndrome_ 1. What does it stand for? 2. When does it occur?
1. Drug rxn with eosinophilia and systemic symptoms 2. **2-8 weeks after exposure to high risk drugs** (ex. anticonvulsants, allopurinol, sulfonamides, and antibiotics)
708
_DRESS Syndrome_ presentation
1. **F**ever 2. **E**osinophilia 3. **D**iffuse rash 4. **I**nternal organ Dysfunction 5. **F**acial edema You will be **FED IF** you get **_DRESS_**ed
709
Increased intake of what substances, helps to prevent renal calculi formation?
Citrate and fluids
710
Effect of DM on urinary continence
Can cause _overflow incontinence_ due to inability to sense a full bladder and due to incomplete bladder emptying.
711
**SE**: *Hyponatremia, Hypercalcemia, Hypocalcemia* _Thiazides_ are more likely to cause which of these? _Loop Diuretics_ are more likely to cause which of these?
_Thiazides_: Hyponatremia and hypercalcemia _Loop diuretics_: Hypocalcemia
712
Presentation of _Intestinal Malrotation_
1. **Intestinal obstruction** (due to compression by the adhesive bands) 2. **Midgut volvulus** (intestinal ischemia due to twisiting around the blood vessels)
713
What aspect of oral administration of a drug is responsible for its decreased bioavailability?
First-pass metabolism by the liver
714
_Ezetimibe_ action
Reduces intestinal absorption of **_cholesterol_** at the brush border (this leads to inc. LDL expression pulling more cholesterol out of the circulation)
715
Name the organisms most often involved in **Chronic Granulomatous Dz** (5)
_Catalase-postive bugs_ which destroy their own hydrogen peroxide **Specifically**: 1. S. aureus 2. Burkholderia cepacia 3. Serratia marcescens 4. Nocardia 5. Aspergillus
716
Which checkpoint does Rb regulate?
G1 → S
717
What causes _Humoral Hypercalcemia of Malignancy_?
Secretion of _Parathyroid hormone-**related** protein_ (PTHrP). It closely resembles parathyroid hormone and causes increased bone resorption and decreased renal excretion of calcium.
718
_Lynch Syndrome (HNPCC)_ 1. What does it lead to? 2. What is responsible? 3. What is the key gene affliated?
1. Leads to occurence of colonic adenocarcinomas at a young age ( 2. Mutations in DNA mismatch repair is responsible 3. **MSH** = key gene
719
What is responsible for the outflow block associated with hypertrophic cardiomyopathy?
The septum touches the mitral valve leaflets, and blocks the aortic valve
720
Other than iodide, what other ions are absorbed into the thyroid gland via the sodium-iodide symporter?
Perchlorate and pertechnetate ions
721
Injury of what nerve, leads to foot drop and weak eversion/ toe extension?
Common peroneal nerve
722
In what part of the cell does Vitamin C function?
The **RER**, where it is a cofactor for hydroxylation of proline and lysine.
723
What is the most common COD in patients with TCA overdose, and why?
Cardiac arrhythmias and refractory hypotension. Due to Inhibition of fast sodium channels in cardiac myocytes.
724
**KRAS** Proto- oncogene or tumor suppressor?
Proto-oncogene
725
What is used for prevention of hemorrhagic cystitis, during tx with systemic chemo (w/ cyclophosphamide or ifosfamide)?
Mesna
726
In addition to looking completely different from their tissue of origin, what other characteristics might _anaplastic cells_ possess?
1. abnormal mitoses 2. giant, multinucleated tumor cells
727
In addition to trisomy 21, what else can lead to Down syndrome?
Unbalanced _Robertsonian Translocations_ [46, XX, t(14;21)] or Mosaicism (some cells have extra copy of chromosome 21)
728
What is the specific mechanism implicated in the pathophysiology of _cataracts_ and _peripheral neuropathy_ in DM?
During hyperglycemia, excess plasma glucose is converted to sorbitol by _aldose reductase_. Sorbitol accumulates w/in some cells and attracts water, leading to osmotic cellular injury.
729
Dx of Chronic Granulomatous Dz
Diagnosis: DHR **flow cytometry** (prefered); **Nitroblue** tetrazolium (NBT) testing
730
What is the role of short non-coding RNA sequences (microRNA and small interfering RNA)?
Induce posttranscriptional gene silencing by base-pairing w/ complementary sequences w/in target mRNA molecules
731
What causes appendicitis pain to eventually localize?
Irritation of the parietal peritoneum
732
What is the inheritance for _classical galactosemia_?
Autosomal recessive
733
What bug/virus causes encephalitis that can lead to hemorrhagic necrosis of the inferior and medial temporal lobes?
Herpes simplex virus
734
What happens if obliteration of the _omphalomesenteric (vitelline) duct_ fails?
1. Persistent vitelline duct 2. Meckel diverticulum 3. Vitelline sinus 4. Vitelline duct cyst
735
Classic triad of sx for RCC?
1. Hematuria 2. Flank pain 3. Palpable mass
736
Sx of angioedema (and what drug can cause it?)
1. Swelling of tongue, lips, eyelids, or sometimes larynx 2. Caused by bradykinin accumulation from ACE-I's
737
Equation for absolute risk reduction
ARR= (event rate in the control group) - (event rate in the tx group)
738
What structure is cut in tx of _carpal tunnel syndrome_?
Transverse carpal ligament
739
Aliskiren MOA
Direct _renin inhibitor_ (Blocks conversion of angiotensinogen to angiotensin I)
740
What can creatinine be used to measure?
Approximate measure of GFR (secreted 10%)
741
What can inulin be used to measure?
**GFR** (freely filtered and neither reabsorbed or secreted) (can also estimate extracellular volume)
742
What would be the result of, loss of **CYP21A2 (21-hydroxylase)** on: Cortisol Aldosterone Androgens
Loss of cortisol and aldosterone, increased androgens (most common casued of CAH)
743
What would be the result of, loss of **CYP11B1 or B2 (11-hydroxylase)** on: Cortisol Aldosterone Androgens
Loss of cortisol and aldosterone, increased androgen **and** increased aldosterone precursor, **_11DOC_** (binds strongly to mineralocorticoid receptor, *simulating* increased aldosterone)
744
What would be the result of, loss of **CYP17 (17-hydroxylase)** on: Cortisol Aldosterone Androgens
Loss of Cortisol and androgens. Increased aldosterone.
745
Non-rhytmic conjugate eye movements + myoclonus (opsoclonus-myoclonus syndrome) is assc. with what dx?
Neuroblastoma
746
_Orotic aciduria_ 1. What is it? 2. What causes it?
1. Rare autosomal recessive disorder of de novo pyrimidine synthesis 2. Defect in _uridine 5'-monophosphate (UMP) synthase_
747
Presentation of individuals with _Orotic aciduria_ Tx?
Children with 1. Physical/mental retardation 2. Megaloblastic anemia 3. Large amounts of urinary orotic acid Tx w/ **uridine**
748
What are the _dendritic cells_ of the skin called? Describe them? (cell line? structure?)
**Langerhans cells** (derived from myeloid cell line and possess racquet-shaped intracytoplasmic granules called _Birbeck granules_)
749
Which _Carcinoid tumor types_ are capable of causing carcinoid syndrome?
**Metastatic** (The cytokines of local carcinoid tumors _confined to the intestine_, are metabolized by the liver before reaching systemic circulation)
750
How do _cancers_ of the _pelvis_ spread to the **lumboscral spine**?
Via the vertebral venous plexus
751
Attachement point for greater and lesser omentum
Greater = Greater curvature of stomach Lesser = lesser curvature of stomach (to liver/duodenum)
752
In cases of Small Intestinal Bacterial Overgrowth (SIBO), what vitamins/minerals... ... become deficient? (5) ...increase in production? (2)
_Deficient_: vitamins B12, A, D, E, and iron _Increased_: folic acid and vitamin K
753
What is the most important virulence factor for E. Coli?
**Fimbrae** (allows it to stick to uroepithelial cells)
754
Prophalictic procedures for _Group B Strep_ (*S. agalactia*)
1. **Prenatal screening** (35-37 weeks) 2. **_INTRAPARTUM_ antibiotics**
755
_Colchicine_ 1. Indication 2. MOA 3. SE
1. Tx of **acute gouty arthritis** in patients who can't take NSAIDs 2. **Blocks tubulin polymerization**, thus inhibiting leukocyte migration/phagocytosis 3. N/Dia
756
Main AE of _Phenytoin_ (3)
1. Ataxia 2. Nystagmus 3. Gingival hyperplasia
757
_Hematogenous osteomyelitis_ 1. Most common in who? 2. What part of the bone is affected?
1. Most common in **children** 2. The **_metaphysis_** of **long bones** (slower blood flow)
758
Drug of choice for _B-blocker overdose_
**Glucagon** (causes inc. of intracellular cAMP)
759
What is _carboxyhemoglobin_?
Carbon monoxide (CO) -bound hemoglobin
760
What's the worst long-term prognostic factor for PSGN?
Adult onset
761
What hormone stimulates release of _Bi-carb rich secretions_ from the exocrine pancreas?
**Secretin**, produced in the S cells of the duodenum
762
What happens to the ratio of Lecithin to sphingomyelin during the third trimester?
**Increases** Lecithin is a component of surfactant (greatly inc. during third trimester), while sphingomyelin is a general amniotic fluid component which should remain constant.
763
What is the cause of _Acute hemolytic transfusion Rxn_?
Type II HSN rxn, w/ subsequent complement activation
764
What differentiates eccrine and adrenal medulla neural input, from the rest of the sympathetic responding glands/organs?
No adrenergic postganglionic neurons
765
If bone conduction is greater than air conduction, what type of hearing loss is it?
Conduction hearing loss
766
Innervation of _external hemorrhoids_
_Inferior rectal_ nerve (branch of the **pudendal**)
767
What volume counts as low Vd?
3-5L
768
What protein is most involved in the transport process, leading to _HSV-1_ recurrence?
Kinesin
769
What is the _Sudan III stain_, and what is it the best test for?
It is a stain for **fat** and it is the most sensitive strategy for malabsorptive disorders
770
What is a **_homeobox_** and what do they code for?
It is an **180 nucleotide**, highly conserved **gene**, which codes for _transcription factors_
771
What does acid-fast stain specifically identify?
Organisms with _mycolic acid_ present in their cell walls
772
What (4) CAs are associated with _dermatomyositis_?
1. Ovarian 2. Lung 3. Colorectal 4. non-Hodgkin
773
_Piriformis Syndrome_
_Sciatica-like_ sx as a result of **compression** of the sciatic nerve, due to muscle injury or hypertrophy of the **piriformis muscle**
774
What type of gland is assc. with _inflammatory acne_?
**Sebaceous** (a _holocrine_ gland)
775
Most frequent mechanism of sudden death in the first 48h after acute MI?
**V. Fib** (due to electrical instability in the ischemic myocardium)
776
What is given to women at risk of premature delivery, to prevent NRDS?
Steroids (ex. dexamethasone)
777
What occurs in a crossover study?
Subjects are randomly allocated to a sequence of 2 or more tx given consecutively
778
What do sertoli cells produce and what does it do?
Inhibin B, which feedsback to inhibit FSH secretion
779
What causes _Familial dysbetalipoproteinemia_? What does it lead to?
Defects in ApoE2 and ApoE4. Leads to dec. clearance of chylomicrons and VLDL remnants
780
The enzymes responsible for glycolysis, fatty acid synthesis, and the PPP, all reside where?
The cytosol
781
How far down into the airway do _cilia_ persist?
Through the length of the respiratory bronchioles
782
_Pleiotropy_ definition
Instances where multiple phenotypic manifestations result from a single genetic mutation
783
Which histone type is outside of the histone core?
H1
784
What can injury of the prostatic plexus (often during a prostatectomy) lead to?
Erectile dysfunction (it innervates the corpus cavernosa of the penis)
785
What cells do _melanocytes_ derive from?
Neural crest cells
786
What is the "Thayer-Martin" selective medium, and what is it used for?
aka _VPN_ (vanco, polymyxin, and nystatin + trimethoprim) Kills everything except *_Neisseria_*
787
Most common cause of aseptic meningitis
Enteroviruses (polio/ coxsackie)
788
What are the (3) main chemotactic agents for neutrophils?
1. Leukotriene (and its precursor) 2. C5a 3. IL-8
789
Most common cause of cystathionine synthase deficiency? How do you tx this issue?
Homocystinuria (responds dramatically to vit B6-- pyridoxine)
790
**HBsAg and HBeAg** Which is the marker for high infectivity?
HBeAg
791
Drug of Choice for Tx of resistant schizophrenia?
Clozapine
792
RF for _C__andidemia_
Presence of a central vascular catheter + receipt of parenteral nutrition
793
Lymphatic drainage of the rectum proximal to the anal dentate line? Distal?
_Proximal_: inferior mesenteric and internal iliac LNs _Distal_: inguinal nodes
794
Describe the anemia assc. with _Mycoplasma pneumoniae_
Autoimmune hemolytic anemia (it looks like human erythrocytes)
795
What is the mechanism of liver injury for Hep B Pts?
The presence of viral HBsAg and HBcAg on the cell surface stimulates the host's cytotoxic CD8+ cells to destroy infected hepatocytes.
796
When is glucose excreted?
When it gets beyond the "Transport Maximum of Glucose", which is around 200 mg/dL. Prior to this value, it is completely reabsorbed.
797
What complicates the long term tx medicating of Parkinson's patients?
Unpredictable fluctuations in motor function
798
How do neutrophils often look in _Leukemoid rxn_?
Granulated (Dohle bodies)
799
Conn's Syndrome? Tx?
Primary Hyperaldosteronism | (tx w/ eplerenone or spironolactone)
800
What (3) drugs work via inhibition of _dihydrofolate_?
1. Trimethoprim 2. Methotrexate 3. Pyrimethamine
801
What makes up the superficial ring?
Openings in the external abdominal oblique
802
What makes up the deep inguinal ring?
Physiologic openings in the transversalis fascia
803
_Rituximab_ MOA
Monoclonal antibody directed against the CD20 antigen
804
_Valproate_ indication
Bipolar maintenance drug
805
What causes malignant hyperthermia? Tx?
Caused by admin of inhalation anesthetics/ succinylcholine to genetically susceptible individuals. Tx w/ **Dantrolene** (Ryr receptor blocker)
806
What effect can HPV have on newborns?
Respiratory papillomatosis, w/ infection of larynx (true vocal cords)
807
Patients w/ familial retinoblastoma have increased risk for what type of tumor?
Secondary tumors, especially **_osteosarcomas_**
808
What is phencyclidine?
PCP (angeldust)
809
How does schizophrenia differ from schizoaffective disorder?
In schizoaffective disorder, there _are_ signficant mood symptoms (ie mania or depression) in conjunction with the hallucinations.
810
Equation for _true positives_
(Sensitivity) \* (number of patients actually with the dz)
811
Rifampin MOA
Blocks the action of bacterial DNA-dependent RNA polymerase
812
False negatives
(1-sensitivity) \* number of patients actually with the dz
813
Most common cause of bloody nipple discharge?
Intraductal papilloma (typically presents _w/o_ breast mass or skin changes)
814
What is responsible for the phosphorylation of Rb?
**CDK4**, after exposure to proliferation signals
815
**Riboflavin** is a precursor of what coenzymes? What are they used for?
FMN and FAD (electron acceptor for _succinate dehydrogenase_)
816
What effect does sarcoidosis have on calcium levels? How?
**_Hypercalcemia_** Activated macrophages produce excess 1,25 dihydroxyvitamin D
817
Name the gene mutations assc. w/ _early onset Alzheimer_
1. APP (chromosome 21) 2. Presenilin 1 3. Presenilin 2
818
Name the gene mutations assc. with late-onset Alzheimer dz
Apoliporotein E4
819
Best drugs for _alcholol_ w/drawal
Benzos
820
What forms the inferior surface of the heart? What vessel supplies it?
The inferior wall of the left ventricle. Fed by the **_PDA_**, which usually derives from the right heart
821
Primary cause of morbidity in acute rheumatic fever?
Severe **_pancarditis_** | (inflammation of the entire heart)
822
How can total parenteral nutrtion lead to gallstones?
Because there is no normal enteral stimulation, there is dec. CCK release and subsequent billiary stasis.
823
Presentation of primary HSV-1 infection in children
Gingivostomatitis
824
Systematic desensitization is an example of what type of therapy?
Behavioral therapy
825
Listeriosis Sx (3)
1. Gastroenteritis 2. Septicemia 3. Meningoencephalitis
826
Key AE of _Niacin_
It can decrease renal excretion of **uric acid**, precipitating _acute gouty arthritis_
827
What are the (2) most important buffers in settings of diabetic ketoacidosis?
NH3 and HPO42- | (are urinated out as NH4 and H2PO4)
828
Penicillin is structurally similar to what part of the pepidoglycan?
D-alanine-D-alanine (penicillin looks like it, vanco directly binds to it)
829
Effects of a _Pudendal Nerve Injury_ When does it usually occur?
1. Fecal/urinary incontinence 2. Perineal pain 3. Sexual Dysfunction Often occurs as a stretch injury during labor
830
Name the common complications of Psoriasis (3)
1. Psoriatic arthritis 2. Nail pitting 3. Uveitis
831
What does Hep B look like on liver Biopsy?
Large hepatocytes folled with finely granular, homogenous, pale pink cytoplasm (eosinophilic inclusions), often described as **ground glass**. This is from the accumulation of _Hep B surface antigen_ w/in affected hepatocytes.
832
Pathogenesis of _Hirschsprung dz_ What organ is always involved?
The result of _abnormal_ migration of **neural crest cells** during embryogenesis. These cells are ganglion precursors, and always involve the **rectum** because they migrate caudally
833
Folate deficiency can lead to erythroid precursor cell apoptosis. What can be given to help allieviate this?
**_Thymidine_** supplementation
834
Who should you avoid giving first gen. antihistamines to, and why?
Older patients, with cognitive or fxnal impairments
835
Other than histamine, what is the best marker for _mast cell activation_?
**_Tryptase_** (it is a preformed inflammatory mediator released by the mast cell)
836
On what part of the collecting duct, does ADH work?
The medullary segment
837
Differentiate between the following: 1. Western Blot 2. Northern Blot 3. Southern Blot
1. **Western**: id's proteins 2. **Northern**: id's RNA 3. **Southern**: id's DNA
838
_Cocaine_ MOA
Inhibits synaptic _reuptake_ of NE, DA, and Ser
839
What is the prefered longterm tx for panic disorder?
SSRIs
840
Describe the hormone status of patients w/ Klinefelter Sperm count?
Elevated FSH and LH (no feedback), and decreased testosterone due to Leydig damage Azoospermia (no sperm)
841
What parameter correlates with the potency of an inhaled anesthetic?
The _minimal alveolar concentration_. This is the ED50 and is inversly proportional to the MAC (the lower the MAC, the higher the potency)
842
Where are the juxtaglomerular cells located? What is the effect of chronic renal hypoperfusion on them?
They are modifed SM cells of the _afferent glomerular arteries_. They secrete renin in response to the Macular Densa. Chronic hypoperfusion will cause hypertrophy and hyperplasia.
843
How does Hep B facilitate the infectivity of Hep D
The _hep B surface antigen_ of the hep B virus **_must coat the hep D antigen_** of the hep D virus, before it can infect hepatocytes and multiply.
844
How does prolonged exposure to loud noises cause hearing loss?
Damage to the _sterociliiated hair cells_ of the **O****rgan of Corti**
845
What are mutations in the **KIT** receptor tyrosine kinase, often assc. with?
Mast cell proliferation
846
_Systemic mastocytosis_ 1. Path 2. Presentation
1. Abnormal proliferation of mast cells and inc. histamine release 2. Histamine causes _hypersecretion_ of gastric acid by _parietal cells_ of the stomach, as well as a variety of other symptoms (hypotension, flushing, pruritus, etc.)
847
Which (2) drugs have the highest risk of causing drug-induced lupus erythematosus (DILE)?
1. Procainamide 2. Hydralazine
848
_Baclofen_ 1. MOA 2. Indication
1. Agonist at the **GABA-B receptor** 2. Effective monotherapy for _spasticity_ secondary to both _brain and spinal cord dz_, including MS
849
What mutation is assc. with medullary thyroid CA?
Activating mutations of the **_RET proto-oncogene_**
850
Inactivating mutations of p53 are fairly common in what typeof thyroid CA?
Anaplastic thyroid CA
851
RAS mutations are common in what type of thyroid CA?
Follicular thyroid CA (and some follicular adenomas)
852
What is _Congenital Pyloric Stenosis_? How does it present?
Smooth muscle hypertrohy of the pyloric muscularis mucosae _Presentation_ 1. projectile nonbilious vomit 2. olive-sized mass in distal stomach
853
Interaction with what drugs can lead to _chronic lithium toxicity_?
1. Thiazides 2. ACE inhibitors 3. NSAIDs
854
Potential serious complication of antithyroid drugs What is the _presentation_ and how should you respond?
**Agranulocytosis** Sudden onset of fever and sore throat (discontinue drug and take white count)
855
Tx of CAH
Low doses of corticosteroids to suppress excessive ACTH secretion
856
What does hemoglobin electrophoresis show for B-Thalassemia minor?
Slightly dec. HbA with inc. HbA2 and HbF
857
Identify this microorganism
*N. gonorrhea* (gram-negative intracellular diplococcus)
858
What factors does *H. flu* require to grow, and what other microorganism can provide them?
1. Factor X (hematin) 2. Factor V (NAD+) ## Footnote *S. aureus*
859
What organism?
Histoplasma capsulatum (in the form of oval bodies w/in a macrophage)
860
**Pt presents w/ foot drop + inability to evert** Name the damaged nerve
Common peroneal
861
COX-1 vs COX-2
**_COX-1_**: House keeping fxns such as platelet aggregation, gastric mucosal protection, etc. (consitutively expressed) **_COX-2_**: *Inducible* enzyme only expressed by **inflammatory tissues**
862
Celecoxib MOA
Selective COX-2 inhibitor
863
What type of muscle fibers compose _postural skeletal muscles_? (Type I or Type II)
**Type I (slow twitch, aerobic, lots of mitochondria)** [this differs from type II, fast twitch, which is anaerobic, and is for rapid forceful pulses of movement]
864
Describe the relationship between BMI and Leptin
**Directly correlated** Leptin is produced by adipocytes in proportion to the quantity of fat stored. Increased leptin leads to _decreased_ appetite and increased satiety
865
Tryptophan is a precursor for what chemical?
Serotonin
866
What is the antidote for serotonin syndrome?
**Cyproheptadine** (an antihistamine)
867
Compare the embryological derivations of the anterior and posterior pituitary
_Anterior_: Surface ectoderm _Posterior_: Neural tube (Both are derivations of the ectoderm)
868
_Mesothelioma_ 1. Main finding on radiographic studies 2. Histopathology
1. **_CT/MRI_**: pleural thickening 2. **_Histo_**: long, slender microvilli and abundant tonofilaments
869
Kids with _Xeroderma pigmentosum_ are very sensitive to, what?
UV-light (due to an endonuclease deficiency)
870
Why is dietary fructose rapidly metabolized?
It bypasses PFK-1, the major rate-limiting enzyme of glycolysis
871
_Caudal regression syndrome_ 1. What is it? 2. What condition is it commonly associated with?
1. Sacral agenesis causing lower extremity paralysis and urinary incontinence in a newborn 2. Poorly controlled maternal diabetes
872
_Pudenal nerve block_ Blocks sensory to what area? Motor to what area?
1. _Sensory_: Perineum 2. _Motor_: Urethral and anal sphincters
873
MOA of _inactivated_ viral vaccines MOA of _live-attenuated_ viral vaccines
_Inactivated_: humoral response (antibodies against hemagglutin, thus preventing **viral entry into the cell**) _Live-attenuated_: cell-mediated + humoral
874
What nerve runs along the _orbital floor_? What would be the result of damage to this nerve?
_Infraorbital nerve_ Damage can result in paresthesia of the upper cheek, upper lip, and uper gingiva.
875
What does the oxygen-dissociation curve of myoglobin look like?
Hyperbolic (not sigmoid)
876
The common _cardinal veins_ of the developing embryo ultimately give rise to what?
The **superior vena cava**, and other constituents of the systemic venous circulation
877
What effect do almost all volatile (inhaled) anesthetics have on cerebral blood flow? What potential risk does this hold?
They increase cerebral blood flow, which can sometimes lead to increased ICP.
878
What is the classic triad assc. with _Parocysmal nocturnal hemoglobinuria_?
1. Hemolytic anemia 2. Pancytopenia 3. Thrombosis at atypical sites
879
What is the role of beta-lactamase inhibitors? Give (3) examples.
Given _with_ penicillin family antibiotics, so that they can work against beta-lactamase inhibitor secreting bacteria. **Ex**: Clavulanic acid, sulbactam, and tazobactam
880
Bleeding after a tooth extraction is suggestive of what? What do you give to stop the acute bleeding?
Hemophilia (dec. factor 8 or 9) Give thrombin
881
What accounts for the fistulas and strictures associated with Crohn's Dz?
Transmural inflammation
882
_Acute Dystonic Reaction_ 1. What is it? 2. What causes it?
1. Sudden, involuntary contraction of a major muscle group 2. Recent initiation of a first gen. _antipsychotic medication_, leading to **_D2 antagonism_** in he nigrostriatal pathway
883
What is _Patent Foramen Ovale_? What can it lead to?
**Incomplete fusion of atrial septum primum and secundum** Can lead to paradoxical embolization of venous clots into the systemic arterial circulation
884
Scaphoid fractures most frequently result from what? What is the largest risk associated with them?
Fall onto an outstretched hand Large risk of **_avascular necrosis_** due to their weak blood supply
885
How does _chronic bronchitis_ appear on **biopsy**? (4)
1. Thickened bronchial walls 2. Leukocyte infilitrate 3. Mucus gland enlargement 4. Squamous metaplasia
886
What is the most common benign liver tumor? (include description) Should you biopsy?
_Cavernous hemangioma_ Cavernous, blood-filled vascular spaces of variable size lined by single epithelial layer. DON'T BIOPSY! (likely to bleed)
887
What type of residue is attached to lysosome-bound proteins?
Mannose-residue
888
What specific serotype is _Ankylosing Spondylitis_ associated with?
HLA B27 (a specific human leulocyte antigen _class **II**_ serotype) *Emphasis on class _TWO_!!*
889
What other atopic dzs is eczema associated with? (2)
Allergic rhinitis and asthma
890
Ca-125 serves as a marker for what CA type(s)
Epithelial ovarian CA
891
Characteristic CXR for bronchial obstructive lesion
Unilateral pulmonary opacification and deviation of the mediastinum _toward_ the opacificed lung
892
What vessel of the portal circulation is associated with _esophgeal varices_?
Left gastric vein
893
Give the layers of the stomach, in order. Which layer does an erosion not go beyond?
1. Mucosa 2. Muscularis mucosa (erosion doesn't go past this point) 3. Submucosa 4. Muscularis propria 5. Serosa
894
Name the autoantibodies assc. w/ SLE
1. Anti-smith (snRNPs) 2. Anti-DS-DNA 3. Anti-nuclear
895
How does _Lead_ cause anemia?
Inhibition of the heme synthesis pathway, leading to a microcytic, hypochromic anemia
896
What is one of the main pathological causes for a uniformly enlarged uterus? Presentation?
**Adenomyosis** The presence of endometrial glandular tissue within the myometrium (presents w/ menorrhagia and dysmenorrhea)
897
What test is used to confirm dx of menopause?
Elevated serum FSH (due to loss of feedback)
898
How can opiods lead to increased pressures in the commmon bile duct/ gallbladder?
They can cause contraction of smooth muscle in the sphincter of Oddi
899
Where does iron absorption primarily occur?
In the duodenum and the proximal jejunum
900
Etiology for _Mallory-Weiss Tears_
Increased intraluminal gastric pressure due to retching/vomiting/other abdominal strain
901
Which ovarian tumor secrtes testosterone?
Sertoli-Leydig
902
_Crigler-Najjar syndrome_ 1. Inheritance 2. Path
1. Aut. recessive 2. Genetic lack of UGT enzyme, leads to inability to conjugate bilirubin via glucuronidation (infants often die early)
903
t(15;17) What CA?
**APL** (M3 AML variant)
904
_Methylmalonic acidemia_ 1. Etiology 2. Presentation
1. Deficiency of methylmalonyl-CoA mutase 2. **Sx**: lethargy + vomiting + tachypnea
905
_Methylnalonic Acidemia_ 1. Labs 2. Dx
1. _Labs_: hyperammonemia, ketotic hypoglycemia, metabolic acidosis 2. _Dx_: elevated urine methylmalonic acid and proprionic acid
906
Most common cause of _unilateral fetal hydronephrosis_
Inadequalte canalization of the _Utereropelvic Jxn_
907
What mediates the glomerulonephritis assc. with PSGN?
Type III HSNT (IC deposition)
908
In _Cystinuria_, there a defective transporter causes build up of what AAs in the intestinal lumen/kidneys?
**COLA** Cysteine, Ornithine, Lysine, Arginine (leads to nephrolithiasis)
909
What does the following suffix indicate about a medication? 1. -cept 2. -nib
1. _-cept_: receptor molecule 2. _-nib_: kinase inhibitor
910
Patients with hemochromatosis are at incrased risk for what?
Liver cirrhosis + hepatocellular carcinoma
911
What is the substitution associated with Sickle Cell?
_Valine_ for _glutamic acid_
912
What is the triad associated with Wiskott-Aldrich syndrome?
1. Eczema 2. Thrombocytopenia 3. B-cell/T-cell deficiency
913
_C1 inhibitor deficiency (C1INH)_ What happens?
* Inappropriate activation of the complement cascade (due to increased C2 and C4 cleavage) * Also leads to inc. Bradykinin because C1INH blocks its formation
914
Somatostatin Function
Inhibits release of many other hormones/enzymes (Secretin, CCK, glucagon, insulin, and **g****astrin**)
915
Phentolamine vs Phenoxybenzamine
Both are a-antagonists _Phentolamine_: reversible, competitive (effects can be overcome by dose) _Phenoxybenzamine_: irreversible (effects cannot be overcome by dose)
916
_Pilocytic Astrocytoma_ 1. View on biopsy 2. View on gross
1. _Biopsy_: rosenthal fibers (eosinophilic, corkscrew fibers) 2. _Gross_: cystic + solid CHILDREN ONLY!
917
Tx of _Drug-induced Parkisonism_
1. Decrease/discontinue drug 2. Give **anticholinergic**
918
3 tx for cyanide toxicity
1. Direct binding of cyanide (via hydroxocobalamin) 2. Use of detoxifying sulfur donors (via sodium thiosulfate) 3. Induction of methemoglobinemia (via sodium nitrate) **DUI**
919
_Glomangioma_ 1. Etiology 2. Presentation
1. Originates from modified smooth muscle cells that control thermoregulatory functions 2. Very tender, small, red-blue lesion under the nail bed
920
A decrease in what component of collagen is responsible for wrinkling?
Collagen _fibril_ production
921
Common tx for _bradycardia_? Associated side effect?
Atropine May increase introcular pressure, precipitating closed-angle glaucoma
922
Why must alcohol be avoided when on _metronidazole_?
Can cause _disulfiram-like_ effects, such as **abdominal cramps, nausea, HA** due to acetaldehyde accumulation
923
Acetyl-CoA serves as an allosteric activator of what?
Gluconeogenesis (increases activity of pyruvate carboxylase)
924
Porcelain gallbladder is associated with an increased risk of what?
Adenocarcinoma of the gallbladder
925
What effect might an ASD have on pulmonary circulation?
Can lead to chronic pulmonary hypertension due to left-to-right intracardiac shunting
926
What is _Niacin_ (vit B3) synthesized from? What other cofactors are needed?
Synthesized from _Tryptophan_ Requires vitamins B2 and B6 as cofactors (B6/B2 = B3)
927
High levels of **what**, serve to increase cholesterol solubility and decrease the risk of gallstones? (2)
1. Bile salts 2. Phosphatidylcholine
928
What does FSH stimulate in males? What does LH stimulate in males?
_FSH_: Sertoli to release **Inhibin** (feedsback) _LH_: Leydig to release **Testosterone** (feedsback)
929
Key differences between the _pneumococcal conjugate_ and _pneumococcal polysaccharide_ vaccines
_Conjugate:_ Good for infants; more robust immune response via B and T cell activation _Polysaccaride_: Good for adults; more serotypes covered (23 vs 13)
930
A child falls while holding someone's hand. On presentation, refuses to move elbow. **What is the injury?**
The _annular ligament_ is torn and displaced
931
What arteries supply the head and neck of the femur?
The **medial femoral circumflex** and its branches
932
Describe the alteration in resting _respiratory_ function in obesity
Extrinsic restrictive pulmonary function tests are seen **(everything decreases except RV which remains normal)** Less capacity + air gets out faster
933
What are cavernous hemangiomas (neuronal)? What increased risk is associated with them?
1. Vascular malformations mostly in the brain parenchyma 2. Inc. risk of intracerebral hemorrhage and seizure
934
_Physostigmine_ 1. MOA 2. Indication
1. Cholinesterase inhibitor 2. Atropine poisoning (ex. via jimson weed)
935
_Neurofibromatosis Type 1_ 1. Inheritance 2. Mutation 3. Presentation (3)
1. Autosomal-**dominant** 2. Mutation to NF1 (Ch. 17) 3. Cafe-au-lait spots + multiple neurofibromas + Lisch nodules
936
Where is the cortisol receptor located?
**Within the cytoplasm** It then translocates to the nucleus after binding its substrate.
937
How does vWF lead to increased PTT and bleeding time?
vWF deficiency leads to impaired platelet function (prolonged bleeding time) and coagulation pathway abnormalities because it is a **carrier for _factor VIII_** (prolonged PTT)
938
Define _Statistical Power_
**(1-Beta)** A study's ability to detect a difference when one exists
939
What is the relationship between _transpeptidases_ and _peniclllins/cephalosporins_
Transpeptidases are an example of a **"penicillin-binding protein",** which penicillins/cephalosporins bind to
940
What artery/nerve run along the midshact of the humerus?
Radial nerve + deep brachial artery
941
Name the triad associated with _Hemochromatosis_
1. Hyperpigmentation (bronze diabetes) 2. DM 3. Pigment cirrhosis leading to hepatomegally
942
_Follicular Lymphoma_ 1. Presentation 2. Associated translocation
1. Painless waxing and waning lymphadenopathy 2. t(14;18) w/ bcl-2 overexpression
943
Efficacy
Max effect achievable
944
During what portion of the TCA is GTP synthesized?
It is synthesized by _succinyl-CoA synthetase_ during conversion of **succinyl-CoA → succinate**
945
Key GI manifestation of lead poisoning
Constipation
946
What is the result of non-fusion of the urethral folds in a male?
**Hypospadia** | (opening along ventral shaft of penis)
947
Most common adverse effects of ADHD meds
Dec. appetite and insomnia
948
What enzyme is most commonly deficient in impaired B-oxidation of fatty acids?
Acyl-CoA dehydrogenase (catalyzes the first step)
949
Best tx for patients w/ PCOS who wish to become pregnant Explain the MOA
**Clomiphene** (estrogen receptor modulator which decreases negative feedback inhibition on the hypothalamus, thus inc. GnRH)
950
Patients w/ CD4 counts
Prophylactic _azithromycin_ to prevent **Mycobacterium Avium Complex** (MAC)
951
What tx can be used to partially overcome food poisoning with *C. botulinum*?
Rapid stimulation
952
Individual presents with _vertical and torsional diplopia_ and an eye that is _deviated upward_. What muscle is the issue?
Superior oblique
953
The presence of which serologic marker is most likely to increase the risk of vertical Hep B transmission?
HBeAg (a marker of viral replication and increased infectivity)
954
What process is directly stimulated by the NF-kB pathway?
NF-kB is a _proinflammatory transcription factor_ that increases **cytokine production**
955
What is the etiology for _trendelenburg gait_ (hip drop)?
Injury to the _contralateral_ superior gluteal nerve (superomedial quandrant)
956
Where does bicarb to neutralize gastric acid come from?
Bicarb comes from the submucosal glands of the duodenum (**Brunner glands**) and from the _pancreatic duct secretions_
957
What cell type composes the _ovaries_?
Simple cuboidal
958
What female repro organs have simple columnar epithelium?
1. Fallopian 2. Uterus 3. Endocervix
959
What female repro components have stratified squamous non-keratinized epithelium?
1. Ectocervix 2. Vagina
960
What is the major virulence factor for *S. pyogenes*? What does it do?
**Protein M** (Inhibits phagocytosis and complement activation)
961
Streptomycin MOA
Inhibits protein synthesis by inactivating the 30S (small) ribosomal subunit
962
Define _spongiosus_
An accumulation of edema fluid in the intercellular spaces of the epidermis
963
What (3) organs/systems does stimulation of B2 receptors, relax?
1. Peripheral vasculature 2. Bronchi 3. **Uterus**
964
Common COD for patients w/ adult-type coarctation of the aorta (3)
1. LV failure 2. Ruptured aortic aneurysm 3. intracranial hemorrhage (**HTN** **associated complications**)
965
RNA produced by _RNA Pol I_
rRNA | (forms ribosome components)
966
RNA produced by _RNA Pol II_
mRNA, snRNA, microRNA (gene silencing)
967
RNA produced by _RNA Pol III_
1. tRNA 2. 5s rRNA
968
Result of early antibiotics on preventing PSGN
Ineffective (though useful for prevention of Acute RF)
969
Inheritance of G6P dehydrogenase deficiency
X-linked recessive
970
Other than IL-1, and IL-6, what other cytokine is critical in inducing a systemic inflammatory response?
TNF-a
971
What is the effect of _Phospholipase C activation_?
Leads to activation of **_protein kinase C_**, via *direct* **DAG** activation or *indirect* **IP3** induced, Ca2+ (Phospholipase C breaks PIP2 into DAG and IP3)
972
Why does silicosis lead to an increased risk of TB?
Silicosis impairs **_macrophage function_** (disrupted by internalized silica particles)
973
Name the key E.coli virulence factor assc. w/ neonatal menigitis
K1 capsular polysaccharide
974
Name the stop codons
UAA UAG UGA
975
In _polymyositis_, what autoantibodies are common? (2)
ANA and Anti-Jo-1 (Histidyl-tRNA synthetase)
976
Name the only sx of _grave's dz_ which is not improved via B-Blockers
Exophtalmos
977
Equation for _Odds Ratio_
OR = ad/bc
978
What is the first step of _base excision repair_?
Removal of the defective base via _glycosylase_ (the empty spot on the sugar backbone is then cleaved and removed by endonuclease + lyase)
979
Explain the _Haldane Effect_
In the lungs, binding of oxygen drives hemoglobin to release H+ (protons) and CO2
980
1. Where in the lungs do supine patients tend to aspirate? 2. Upright patients?
1. _Supine_: posterior segment of upper lobes (and superior segment of lower lobes) 2. _Upright_: Base RIGHT SIDE FOR BOTH
981
What keeps a PDA open? What closes it?
_Open_: Prostaglandin E2 (E, k**EE**ps it open) _Closes_: Indomethacin
982
In cases of *Echinococcus granulosus*, what can be the result of spilling of cyst contents during surgery?
Anaphylactic shock
983
_Li-Fraumeni Syndrome_ 1. Inheritance 2. Mutation 3. Family Hx
1. Autosomal dominant 2. Mutated TP53 3. Family Hx of many CA types (sarcoma, leukemia, breast, adrenal)
984
What phenomenon facilitates dischare of urine from the umbilicus?
Failure of the **urachus** (allantois remnant), _to obliterate_ at birth
985
_Methotrexate_ 1. What is it? 2. Indications
1. A folic acid analog that competitively inhibits dihydrofolate reductase (normally converts folic acid to THF). 2. Serves as a chemotherapeutic and anti-psoriasis agent
986
Role of _metalloproteinases_ during wound healing
Contracture (along with myofibroblasts)
987
What is the most common cause of spontaneous lobar hemorrhage in the elderly? What are the most common sites of this hemorrhage?
**CEREBRAL AMYLOID ANGIOPATHY** (Commonly in the occipital and parietal lobes)
988
What dz's are Lewy bodies assc. w/?
Parkinson dz and Lewy body dementia
989
What conditions lead to _polyhydramnios_ (excessive amniotic fluid)?
1. GI obstruction 2. Anencephaly
990
What kind of receptors contain zinc-finger binding domains?
Intracellular (ex. steroids, thyroid hormone, fat-soluble)
991
What is the role of _releasing factors_?
Recognize _stop codons_ and terminate protein synthesis
992
What nerve handles plantar foot sensory?
Tibial
993
**Factitious Disorder**
Intentional falsification or inducement of sx w/ goal to assume sick role
994
_Etanercept_ 1. What is it? 2. Indication 3. What must you do first?
1. Decoy receptor for TNF-a 2. Tx for extreme RA First must eval for TB
995
Metyrapone MOA
Blocks 11-B-hydroxylase (steroid pathway)
996
How do most enveloped nucleocapsid viruses acquire their lipid bilayer envelope? (exception?)
By budding through the plasma membrane of the host cell. (except for herpesviruses)
997
What commonly occurs following an SAH? How can it be prevented?
Vasospasm (prevent with CCB)
998
Patients w/ Actinic keratoses are at inc. risk for what?
Invasive squamous cell carcinoma
999
Lab tests show no CD18 on leukocyte surfaces. Dx?
Leukocyte Adhesion Deficiency | (They can't cross into tissues)
1000
Classic presentation for _Phenylketonuria_
1. **_S_**eizures 2. **_L_**ight pigmentation 3. **_I_**ntellectual disability 4. "**_M_**usty" odor
1001
_Phenylketonuria_ Pathology What becomes an essential AA as a result?
Results from the inability to convert **phenylalanine** to **tyrosine** (via the phenylalanine hydroxylase system). Tyrosine becomes an essential AA
1002
_Glucagonoma_ 1. Presentation 2. Dx
1. _Sx_: (1) **Necrolytic migratory erythema** - bronze-colored, central indurated area w/ peripheral blistering/scaling around face, perineum, and extremities (2) **DM** 2. _Dx_: Elevated glucagon levels
1003
TOC for OCD
SSRIs
1004
_Congenital torticollis_
Congenital contraction of unilateral SCM, usually due to malposition in utero, or birth trauma.
1005
How does VZV look on light microscopy?
Intranuclear inclusions in keratinocytes + multinucleated giant cells
1006
Tx of Graves' ophthalmopathy
High-dose glucocorticoids (ex. prednisone)
1007
What mold has spherules packed with endosphores?
*Coccidioides immites*
1008
What viruses does the *Aedes aegypti* mosquito carry?
Dengue + Chikungunya
1009
A mass in the _parotid gland_, risks compressing what nerve?
**Facial** | (leading to ipsillateral facial droop)
1010
Transmission of _congenital toxoplasmosis_
Transplacental (in utero)
1011
_Kiesselbach Plexus_
Point at the nasal septum where the **anterior ethmoidal, sphenopalatine,** and **superior labbial arteries** all meet. (most common location for epistaxis)
1012
What type of kidney stones are assc. w/ _Crohn dz_?
**Oxalate** (bowel damage leads to dec. Ca2+ absorption, and thus inc. free Oxlatate excretion in the urine)
1013
Key pathogenesis for _Alcohol-induced Hepatic Steatosis_
Dec. **Free fatty acid oxidation** secondary to excess NADH production by _alcohol dehydrogenase_ and _aldehyde dehydrogenase_
1014
What drug is useful for tx of both BPH and HTN?
a1-blockers
1015
Where do the intercostal nerves/vessels lie?
Lower border of the ribs (subcostal)
1016
What structural change indicates _irreversible_ myocardial cell injury?
**Mitochondrial vacuolization** (loss of ability to further generate ATP)
1017
Reid index
Ratio of thickness of the mucous gland layer in the bronchial wall, compared to everything in the wall above the level of the cartilage (measures bronchitis progression)
1018
Mechanism of resistance against _aminoglycosides_
**Methylation** of the aminoglycosidee-binding portion of the 30S ribosome
1019
When does _cleft lip occur_?
When the maxillary prominence fails to fuse with the intermaxillary segment
1020
When does _cleft palate_ occur?
When the palatine shelves fail to fuse w/ one another or w/ the primary palate
1021
Clinical manifestations of *Babesiosis*
Flulike symptoms (And labs show anemia + thrombocytopenia)
1022
Dx of _Babesiosis_
Blood smear shows **Intraerythrocytic** _pleimorphic ring forms_ (maltese cross)
1023
Echinocandin MOA
Antifungal which inhibits synthesis of the plysaccharide _glucan_, an essential component of the fungal cell wall.
1024
_Schizophrenia_ 1. What is sx are required for dx 2. Duration?
1. At least (2) of the following (5): **delusions**, **hallucinations**, **disorganized speech**, grossly disorganized behavior, and negative sx (at least _one_ of the bolded must be present) 2. \>6 months w/ \>1 month of active sx's
1025
What is the role of IgA on mucosal surfaces?
It **binds/inhibits the action of pili** as well as other cell surface antigens that normally mediate _mucosal adherence and subsequent penetration_ by bacteria
1026
What order of IV medications should be given to alcoholics/malnourished patients? Why?
IV thiamine supplementation prior to IV dextrose (or else risk precipitating Wernicke encephalopathy)
1027
Genetic _Recombination_ vs _Reassortment_
_Recombination_: **Crossover** between 2 chromosomes from different viruses _Reassortment_: Exchange of entire genome **segments** (segmented viruses only)
1028
How is vision affected by age?
Loss of structural proteins in the lens leads to _presbyopia_ (far-sightedness), but also tends to **slightly correct pre-existing myopia**
1029
What are the (5) P's of _Acute Intermittent Porpyria_?
1. Painful abdomen 2. Port wine-colored urine 3. Polyneuropathy 4. Psych disturbances 5. Precipitated by drugs (CYP450), alcohol, starvation
1030
Tx for _Acute Intermittent Porphyria_
Glucose + heme (Inhibits ALA synthase, halting build-up of PBG and ALA)
1031
_Jujenual/Ileal Atresia_ 1. Path 2. Sx 3. Gross
1. **Vascular occlusion** in utero leads to ischemia of segments of bowel, and subsequent narrowing/obliteration 2. Bilious emesis 3. Absence of large portions of the gut, potential winding of gut remnants winding around vascular stalk
1032
What does PAS staining, stain for?
Glycoprotein
1033
What role does _desmopressin_ play in clotting?
Increases circulating factor VIII and endothelial secretion of vWF
1034
_Arginase Deficiency_ Presentation
1. Spastic diplegia 2. Growth delay 3. Abnormal movements
1035
Injury to what portion of the male genitalia is assc. with pelvic fractures?
Posterior urethra
1036
_Sirolimus MOA_
Immunosuppressant (binds to FKBP in cytoplasm, inhibiting mTOR thus blocking IL-2)
1037
What is PPAR-gamma?
An **_intracellular nuclear receptor_** that acts as a transcriptiona regulator of many genes involved in glucose and lipid metabolism
1038
Adhesion of cells to the ECM involves _integrin-mediated_ binding to what?
1. Fibronectin 2. Collagen 3. Laminin
1039
Tissue damage and resultant abscess formation is primarily caused by...
**Lysosomal enzyme** release from _neutrophils_ and _macrophages_
1040
What tissue type is unable to use _ketones_ and why?
Erythrocytes | (lack mitchondria)
1041
_Zenker (false) diverticulum_ 1. How does it form? 2. Presentation?
1. _Diminished relaxation_ of **cricopharyngeal muscles** during swallowing, leading to increased intraluminal pressure in the oropharynx (forming outpouching) 2. Dysphagia + halitosis (food stuck) + regurg + recurrent aspiration
1042
Why must you be cautious when using B-blockers for patients w/ DM?
It excarcebates and masks the NE/epi mediated sx of hypoglycemia
1043
_Budd-Chiari Syndrome_ Path
Occlusion of hepatic vein
1044
What happens if a tRNA is mischarged w/ an incorrect AA?
It is either corrected by the aminoacyl-tRNA synthetase proofreader, or that incorrect AA is incorporated into the growing chain.
1045
First line tx for general anxiety disorder
SSRIs
1046
What defines _Major Depressive Disorder w/ Psych Features_?
MDD psych sx which are **only present during the episode of MDD**
1047
How does *Trypanosoma cruzi* cause its effects?
It can destroy the myenteric plexi in the esophagus, intestines, and ureters (leading to achalasia, megacolon, and megaureter)
1048
What effect do muscarinic agents have on the detrusor muscle?
They _constrict it_!! This pushes urine out.
1049
What effect does estrogen have on the H-P-Thyroid axis?
Estrogen inc. the level of _thyroxine-binding globulin_ which leads to a corrective, transient increase in **TSH and T4**
1050
What effect does prolonged ACTH stimulation have on the adrenal glands?
HYPER**_PLASIA_** of the Zona fasiculata and reticularis
1051
Describe the teratogenicity assc. w/ **valproate**
Decreases levels of folic acid, thus _increasing risk of NTDs_
1052
Describe the relationship between the solubility of gas, and the onset of action
The more soluble a gas, the _slower_ its onset of action (less soluble gases saturate the brain quicker)
1053
Name the (2) watershed ares of the colon, which are most susceptible to ischemic damage in hypotensive states
1. _Splenic_ flexure 2. Rectosigmoid colon
1054
_Stress-related Mucosal Dz_ 1. What is it? 2. Sx?
* Acute gastric mucosal defects that develop in response to severe physiological stress * _Sx_: multiple, small gastric erosions/**_ulcers_**, which may **perforate or bleed**
1055
_Friedreich ataxia_ 1. Sx 2. Path
1. Cerebellar ataxia + dorsal column issues + scoliosis/foot abnormalities + hypertrophic cardiomyopathy 2. AR disorder caused by mutation of the **FXN gene**
1056
What dz is described as _spotty necrosis_ on histo? What else does the histo show?
**_Hep A_** (also shows **ballooning degeneration** and _mononuclear_ infiltrates)
1057
What makes up the walls of _Pancreatic Pseudocysts_? How is this different from true cysts?
Formed from **granulation tissue** and **fibrosis** (true cysts are lined by epithelium)
1058
Long term effect of *Vibrio cholerae* on cells of the colon
None | (does not cause cell death at all)
1059
**Effect modification** vs **Confounding**
_Effect modification_: When the effect of the main exposure on the outcome is modified by the presence of another variable _Confounding_: It looks similar to effect modification but appropriate stratification reveals there is actually no true difference that exists
1060
**CD14** is the surface marker for what?
Monocyte-macrophage cell lineage
1061
Where does the _left ovarian vein_ drain? The _right ovarian vein_?
_Left_: drains into the **left renal vein** _Right_: drains directly into the **IVC**
1062
Digoxin Clearence
**Renal**
1063
Describe the "preparation" stage in the stages of change model
Patient is in the process of planning to make a change in the near future, and mapping out how to do that
1064
Which Benzos are preferred for patients w/ _impaired hepatic metabolism_?
**LOT** **_L_**orazepam **_O_**xazepam **_T_**emazepam
1065
Relationship between _Azathioprine_ and _Xanthine oxidase_
Xanthine oxidase catalyzes one of the main mechanisms for **azathioprine** metabolism (Any drug blocking XO will thus inc. the level of **Aza**)
1066
How do you dx _acute cholecystitis_?
Ultrasound or otherwise nuclear medicine hepatobiliary scanning
1067
Identify #8
Psoas muscle
1068
_Methotrexate AE_
Bone marrow suppression + liver function abnormalities
1069
What mediates _paraneoplastic cachexia_
TNF-a
1070
_Costosternal Syndrome (Costochondritis)_ 1. What causes it? 2. Sx
1. Caused by repetitive activity, leading to inflammation of the upper costal cartilage at the costochondral or costosternal jxn 2. Pain is **reproducable w/ palpation** and _worsened w/ movement or changes in position_
1071
What group does "reactive arthritis" from chlamydia, belong to? What does this mean?
It belongs to the HLA-B27 assc., seronagtive spondyloarthropathies (thus there is risk for _sacroiliitis_)
1072
What organs synthesize heme?
Virtually every organ (just need mitochondria). But principally in the **BM** (erythrocyte precursor cells) and **hepatocytes**
1073
What is responsible for the hypercoagulative state of nephrotic syndrome?
The loss of anticoagulant factors in the urine (especially antithrombin III)
1074
_Thombotic Thrombocytopenic Purpura_ 1. What is missing? 2. What happens as a result?
1. Loss of **ADAMTS13** (aka vWF cleaving protease), which is normally responsible for breaking vWF into smaller multimers 2. Inc. large vWF multimers leads to inc. platelet adhesion and subsequent aggregation/thrombosis
1075
_TTP_ 1. Presentation 2. Tx
1. Hemolytic anemia (schistocytes, inc. LDH, dec. haptoglobin) 2. Give plasmapherisis + steroids
1076
Stimulation of what _adrenergic_ receptor causes increased IP3 release?
a-1
1077
Male born w/ feminized external genitalia (small phallus and hypospadia) that masculinizes at puberty. _Dx_?
**5a-reductase deficiency** (converts testosterone to dihydrotestosterone, which mediates development of the external genitalia in the male fetus)
1078
What must we be careful of early in tx w/ an ACE-Inhibitor?
First dose hypotension
1079
Presentation of patients w/ _von Willebrand dz_
Lifelong Hx of mucosal bleeding (gingival, epistaxis, and/or menorrhagia) Platelet levels are normal but they don't fxn well
1080
_Hydroxyurea_ MOA + Indication
For sickle cell patients w/ frequent pain crises **Increases fetal hemoglobin** (mechanism unknown)
1081
_Gardos channel blocker_ 1. Indication 2. MOA
1. Sickle cell 2. Hinder the efflux of potassium and water from the cell, **thus preventing dehydration** of erythrocytes and subsequent polymerization of Hb S
1082
Which ribs overlie the spleen?
Ribs 9, 10, and 11
1083
Which ribs overlie the left kidney? Right kidney?
_Left_: Ribs 11 and 12 _Right_: 12
1084
_Hemolytic uremic syndrome_ Etiology/pathology
Shiga toxin (*E. Coli* O157:H7 or *Shigella* ) induces capillary endotheial damage, resulting in platelet activation and formation of **microthrombi**
1085
_Hemolytic Uremic Syndrome_ Clinical features
**_H_**emolytic anemia (w/ schistocytes) **_A_**cute kidney injury **_A_**ntecedent diarrheal illness **_T_**hrombocytopenia (HUS- **HAAT**)
1086
What gene is abnormal w/ inherited Pulmonary Arterial HTN?
BMPR2
1087
What charaterizes Drug-induced lupus erythematosus?
Abrupt onset of lupus sx w/ positive anti-histone antibodies
1088
What type of individuals are genetically predisposed for Drug-induced lupus srythematosus (DILE)?
Patients w/ expression of slow hepatic acetylators
1089
In general, what is a _Chiari malformation_?
Congenital disorder that results from the underdevelopment of the posterior fossa. Causes parts of the cerebellum and medulla to herniate through the foramen magnum.
1090
Describe _Chiari type I_ | (prognosis and sx)
Relatively benign and presents in adulthood w/ occipital HA and cerebellar dysfxn (type of Chiari malformation)
1091
Describe _Chiari type II_ | (prognosis and sx)
Severe form. Affects neonates and often assc. w/ myelomeningocele and hydrocephalus (Type of Chiari malformation)
1092
Describe the onset of action and risk of dependence for Buspirone
Slow onset and no risk of dependence
1093
Tx of _somatic symptom disorder_
Regularly schedule medical visits w/ focus on fxnal improvement. Avoid unecessary dx testing.
1094
**What is the dx?** Frontal bossing Delayed fontanel closure Femoral/tibial bowing
_Rickets_
1095
What is required to activate 6-MP? To deactivate it?
_Activate_: HGPRT _Deactivate_ Xanthine Oxidase
1096
Describe the biopsy findings of Subacute granulomatous (de Quervain) thyroiditis
MIxed, cellular infilitration w/ occasional multinucleated giant cells
1097
Describe the clinical features of Subacute granulomatous thyroiditis
Painful thyroid enlargement and transient hyperthyroid sx, following a _viral illness_
1098
Typical genotype of a complete mole and why
46, XX (haploid sperm enters empty egg and duplicates. At least one X required for survival-- hence no 46, YY)
1099
What is responsible for destruction of cells w/ decreased or absent MhC class I proteins on their surface?
NK cells
1100
What is _Delusional disorder_? How does this differ from **paranoid personality disorder**?
_Delusional disorder_: \> or = 1 delusion in **absence** of other psychotic sx _Paranoid_: general suspiciousness or distrust of *everyone*, w/o odd fixed belief
1101
1. Potential consequences of CREST syndrome 2. Assc. antibodies
1. Can have sclerosis of renal, **pulm**, CV, and GI systems (due to small vessel deposits) 2. Antibodies: (anti-centromere)
1102
Describe the pathogenesis of _prion dz_ Give the (2) major examples
Prion protein (**PrP**) is normally _a-helical_ in neurons. If converted to _B-pleated sheets_, it becomes resistant to proteases and accumulates in the brain matter creating vacuoles (spongiform encephalopathy) ex. Creutzfeldt-Jakob and Bovine Spongiform
1103
How (time-frame) does pheochromocytoma present?
Sx are often **_episodic_** in nature, coming and going with fluctuations in catecholamine synthesis
1104
_Anal fissures_ 1. What are they/ what causes them? 2. Most common location (be specific)?
1. _Longitudinal tears_ in the mucosa, usually due to passage of hard stool in patients w/ _chronic constipation_ 2. Usually at the **posterior midline** (low blood flow)
1105
_Obturator nerve_ 1. What path does it follow? 2. How is it injured? 3. How does injury present?
1. Only major nerve that exits the pelvis via **obturator foramen** 2. Injury via compression 3. _Sx_: weakness in thigh **adduction** + sensory loss over distal medial thigh
1106
Where do the arteries going to the testicles/ovaries originate from?
The _gonadal arteries_ originate from the AA
1107
_Septic abortion_ 1. Presentation 2. Common pathogens assc.
1. Fever, uterine tenderness, and foul-smelling discharge after a pregnancy 2. *S. aureus* or *E.coli*
1108
What is the targetoid skin disorder assc. w/ HSV?
Erythema multiforme
1109
Key AE of dihydropiridine CCBs (2)
**Peripheral Edema** + Dizziness/lightheadedness
1110
_Rabies_ 1. Main source in the US 2. Prevention
1. Bats 2. Killed vaccine
1111
_Rabies_ Presentation
Agitation + spasms progressing to coma
1112
What is the most characteritic biochemical change in _Huntington dz_?
Dec. **GABA** in the brain
1113
1. What role does TGF-B play in the healing process? 2. What happens in the body if there is too much TGF-B
1. It is critical for fibroblast migration, proliferation, and connective tissue synthesis 2. Increase is responsible for **hypertrophic/keloid scarring** (as well as _fibrosis_ of the lung, liver and kidney in chronic inflammation)
1114
How do patients w/ familial chylomicroenmia syndrome frequently present?
In childhood w/ recurrent episodes of acute pancreatitis
1115
High levels of dietary _aflatoxin_ exposure (produced by aspergillus) leads to what? What does this increase risk for?
G:C → T:A transversion in codon 249 of the p53 gene (Greatly increases risk for **HEPATOCELLULAR CARCINOMA**)
1116
What is AA transamination and what serves as the cofactor for it?
An amino group from an AA, is transfered to an a-keto acid, which in turn becomes an AA Catalyzed by vitamin B6 (**pyridoxal phosphate**)
1117
What kind of diet must you place a patient on who has _Pyruvate Dehydrogenase Comlex Deficiency_ and why?
A **ketogenic diet** (high fat, low carbs) This near-absence of glucose in the diet decreases the amount of pyruvate enerated, thereby decreasing lactate levels
1118
Name the (2) exclusively ketogenic AA's (can't be metabolized to pyruvate)
Lysine and Leucine
1119
First line tx of ADHD
**Stimulant medications!** (inc. NE and Epi to prefrontal cortex)
1120
Local cutaneous AE of chronic topical corticosteroid administration (2 major ones)
1. Atrophy/ thinning of dermis 2. Tightening of skin
1121
When a nerve is severed, what happens to the segment of axon still connectd to the cell body?
**Axonal reaction** (sets the stage for regeneration) * Swelling * Nuclei displaced to periphery * Nissl substance becomes fine, granular, and dispersed
1122
Who do _Ovarian teratomas_ most comonly occur in?
Females age 10-30
1123
Describe the histology of _Schwannomas_ (2 key components)
* Biphasic cellularity * S-100 positivity (neural crest derived protein)
1124
What runs through the _hepatoduodenal ligament_?
**The portal triad** (hepatic artery, portal vein, common bile duct)
1125
Where in the peritoneum does the bladder sit?
The bladder is _extraperitoneal_
1126
_Mucormycosis_ 1. Presentation 2. Dx
1. Patient w/ _diabetic ketoacidosis_, presenting w/ facial pain, HA, and **black necrotic eschar** 2. Biopsy needed for dx
1127
Where do the aspiration contents come from that cause lung abscesses?
They are oropharyngeal contents (**NOT STOMACH!**)
1128
Key translocation for _Burkitt lymphoma_
t(8;14) (c-Myc to Ig heavy chain)
1129
_c-Myc_ fxn
Transcription activator
1130
Tx for oral/esophageal candidiasis
Nystatin
1131
How do RBCs look in G6PD deficiency?
_Heinz bodies_ (Hb precipitation), which can be "bitten" from the cell forming **bite cells**
1132
What role do _metalloproteinases_ of inflammatory macrophages, play in plaque stability?
These _zinc-containing enzymes_ (**Metalloproteinases)** degrade ECM, making plaques more unstable
1133
**Reaction Formation**
Immature defense mechanism that involves replacing unacceptable feelings and impulses w/ their extreme opposite.
1134
**Renal Angiomyolipoma** 1. What is it composed of? 2. What dz is it associated with?
1. Benign tumor composed of blood vessels, SM, and fat 2. Assc. w/ **tuberous sclerosis**
1135
**Tuberous sclerosis** Presentation
1. Hamartomas in the brain 2. Facial angiofibroma 3. Leaf-shaped patches of skin w/o pigment 4. Seizures/ Cognitive disability
1136
How does excess _ammonia_ disrupt excitatory neurotransmission in the brain?
It crosses the **BBB** and is taken up by **astrocytes**, inc. _glutamine production_. This ultimately leads to inc. intracellular osmolarity, swelling, and impaired glutamine release for conversion to **glutamate**
1137
What drugs for herpes virus do not require viral _phosphorylation_?
Cidofavir and Foscarnet
1138
What characterizes _Borderline Personality Disorder_?
1. Peristent pattern of unstable relationships 2. Mood lability (possible suicidal ideation) 3. Impulsivity
1139
_Achondroplasia_ **Path** (including key mutation)
Exaggerated inhibition of **_chondrocyte_** proliferation in the growth plates of long bones, due to _FGFR3_ mutation.
1140
What side of the body does each _cerebellar_ hemisphere coincide with?
Ipsilateral
1141
Type I (alpha) error
Falsely concludes there is a stastical difference
1142
Type II (Beta) error
Falsely concludes that there is NO statistical difference
1143
Berkson's bias
Selection bias that can be created by selecting hospitalized patients as the control group
1144
A high mitotic index (Ki-67 fraction) approaching 100% is typical for what CA type?
Burkitt lymphoma
1145
Where does Burkitt lymphoma happen in adults and what adults are most likely to have it?
Pelvis/ abdomen (HIV+ or generally immunocomprimsed adults)
1146
Most common cause of Hyper-IgM syndrome
Absence of the **CD40 ligand**, resulting in defective immunoglobulin class switching
1147
Which vessels come together to form the IVC
The right and left common iliac veins
1148
Foreign bodies lodged in the piriform recess, put what nerve/reflex at risk?
The internal laryngeal nerve, which mediates the afferent limb of the _cough reflex_
1149
_Dubin-Johnson Syndrome_ Path
Defective hepatic excretion of **bilirubin glucuronides** across the canaicular membrane, resulting in direct _hyperbilirubinemia_ and _jaundice_
1150
_Dubin-Johnson Syndrome_ Gross and Histo
_Gross_: liver appears black due to impaired excretion of epinephrine metabolites _Histo_: Epi metabolites apprear as dense pigments w/in lysosomes
1151
What is the most specific test for *S. pyogenes*?
The **pyrrolidonyl arylamidase test (PYR)**
1152
_Hydralazine_ 1. MOA 2. Indication
1. _MOA_: Increases cGMP, leading to vasodilation (A \> V) and afterload reduction 2. _Indication_: severe HTN
1153
What is the most common _congenital heart defect_ associated w/ **Down Syndrome**
A complete _atrioventricular septal defect_
1155
What messenger system does _Glucgaon_ use to exert its effects?
Adenylate cyclase second messenger system (Gs, cAMP, PKA)
1156
What hormone receptors use PKA?
TSH, glucagon, and PTH receptors
1157
_Uniparental disomy_ 1. What is it? 2. Give (2) examples
1. When an individual inherits 2 coies of a chromosome from one parent and none from the other 2. Ex. Prader-WIlli syndrome or Angelman syndrome
1158
What is the most common cause of Turner syndrome?
Paternal **meiotic nondisjuntion**
1159
What z-score represents 95% of the statistical distribution? 99%?
_95%_: z-score = 1.96 _99%_: z-score= 2.58
1160
What does a liver biopsy w/ green-brown plugs and pigment accumulation, signify?
Cholestasis
1161
First line tx for _narcolepsy_
**Modafinil** (or other psychostimulants)
1162
_Rett Syndrome_ Presentation
1. Loss of speech and motor skills 2. deceleration of head growth 3. stereotypic hand movements (Usually in girls, following period of normal development) **Rett syndrome** = **REGRESS syndrome**
1163
Most common mutation assc. w/ _Rett Syndrome_
*MECP2* gene
1164
Describe the histo and immunohisto for _Small Cell Lung Carcinoma_
_Histo_: clusters of small, ovoid cells w/ scant cytoplasm and high mitotic count _Immunohisto_: stains positive for neuroendocrine markers, especially **chromagranin**
1165
Describe the murmur assc. w/ ASD
**Fixed splitting of S2** (because right side of heart now ALWAYS gets more blood, not just during respiration)
1166
Metabolic effect of vomiting
Metabolic _acidosis_
1167
**What is this dx?** Necrotizing vasculitis of upper/lower resp tract (causing ulcers, sinusitis, hemoptysis) + renal issues
Wegener's
1168
_HSP_ Path
It is an IgA-mediated type III HSN Rxn (which is why it often follows an URT infxn)
1169
What is the role of sunlight exposure in vitamin D activation?
It catalyzes conversion of 7-dehydrocholesterol to _cholecalciferol_ (vitamin D3)
1170
_Benzos_ MOA
Positive allosteric modulation of GABAA
1171
Common eye-related injury w/ congenital CMV
Chorioretinitis
1172
What effect does _pyruvate kinase deficiency_ have on RBCs and the spleen?
Leads to a **hemolytic anemia** due to loss of ATP and subsequent loss of cation transport. Spleen must dispose of these parts, leading to red pulp hyperplasia.
1173
_Oseltamivir_ MOA
Neuraminidase inhibitor
1174
What causes hypersensitivity to intradermally injected tobacco extract?
Being a heavy smoker
1175
What type of vaculitis is _Buerger dz_?
Segmenting (and medium sized vessels)
1176
Decribe the (2) morphological variants of _gastric adenocarcinoma_
1. **Intestinal**- solid mass that projects into stomach (glandular cuboidal/columnar cells) 2. **Diffuse**- linitis plastica. Infiltrates stomach wall + displays _signet-ring cells_
1177
Most prominent organisms associated w/ intraabdominal infxns
*Bacteroides fragilis* and *E. coli*
1178
Major SE of _ganciclovir_, especially in combination w/ bactrim or zidovudine
Bone marrow suppression
1179
What sort of antibodies can be expected in _Pauci-immune RPGN_?
**p-ANCA/c-ANCA** (will not show up on light micro or IF, unlike IC's or antiglomerular BM antibodies)
1180
_Axillary Nerve_ 1. How is it injured? 2. How does injury present?
1. Injured via **shoulder trauma** 2. _Presentation_: sensory loss over lateral shoulder + **deltoid** weakness
1181
How does synchronization of glycogen degradation w/ skeletal muscle contraction occur?
Release of sarcoplasmic **_calcium_** following neuromuscular stimulation, causes activation of _phosphorylase kinase_, stimulating glycogen phosphorylase to inc. glycogenolysis
1182
Neprilysin inhibitors (MOA)
Prevent degradation of ANP (Neprilysin normally breaks down natriuretic peptides)
1183
In addition to the eosinophilia and respiratory issues, what is a major sx assc. w/ Churg-Strauss?
Peripheral neuropathy
1184
Capitate
When a payor (often an employer) pays a **fixed, predetermined fee** (to an insurance company, or physician group), to provide all services required by a patient (often employees) (payment structure of HMOs)
1185
DNA _gyrase_ is aka...?
Topoisomerase II
1186
Fxn of _DNA polymerase I_
The removal of RNA primers and replacement w/ DNA
1187
What is responsible for "dimpling" that can occur in breast CA?
Malignant infiltration of the **_suspensory ligaments_**, leading to fibrosis, shortening, and thus skin traction.
1188
Affect of inhaled anesthetics on the liver? What lab values are affected?
Can cause highly lethal _fulminant hepatitis_. Elevated AST and PT
1189
What predisposes individuals for _angiosarcoma_?
**Axillary LN dissection which leads to chronic lymphedema.** (angiosarcoma is a misnomer, as it is technically a *lymphangiosarcoma*)
1190
Best tx for severe hypoglycemia in a nonmedical setting
**Intramuscular** glucagon | (IV dextrose in-hospital)
1191
Effect of gallbladder _hypomotility_
Bile concentration (since gallbladder absorbs water), leading to formation of **BILE SLUDGE**
1192
What is the major determinant of cyanosis severity in tetralogy of fallot?
The degree of _obstruction_ of the **right ventricular outflow tract**
1193
Daptomycin MOA
Depolarizes the bacterial cell membrane
1194
What is the most common site for _acute compartment syndrome_? What is housed in this site? (2)
The anterior comportment of the leg, which contains: 1. Deep peroneal nerve 2. Anterior tibial artery/veins
1195
Idenitifying characteristics of a _craniopharyngioma_ (4)
1. Suprasellar (rathke's pouch) 2. Calcified Cysts 3. Cholesterol rich fluid w/in cysts 4. Children
1196
During continous infusion, how long does it take to reach steady state?
4-5 half-lives
1197
What is the translocation assc. w/ follicular lymphoma? What does it lead to?
t (14;18) BCL-2
1198
Most likely AE of SSRIs?
Sexual dysfunction
1199
Other name for "mole"
Nevus
1200
Osteoblastic lesions are also known as...? Osteolytic?
_Osteblastic_: sclerotic _Osteolytic_: lucent (assc. w/ osteoclasts)
1201
_Elastin_ What gives it its rubber-like properties?
Extensive _cross-linking_ between elastin monomers, which is facilitated by **lysyl oxidase** (related to lysine)
1202
_Tennis Elbow_ 1. What is injured? 2. What attaches to this injured point?
1. It is also known as **lateral epicondylitis** (the lateral epicondyle is injured) 2. Attach point for _extensor carpi radialis brevis_ and extensor digitorum (both involved in wrist extension)
1203
At what point does coagulative necrosis begin after an acute MI?
4 hours
1204
_Multiple Myeloma_ Sx/ presentation
**CRAB** eating a **Roll** of **M** & **M**'s hyper**C**alcemia **R**enal involvement **A**nemia **B**one lytic lesions **Roul**eaux formation **M**onoclonal **M** spike
1205
How does the glu → val change in HbS lead to sickling?
_Glu_ has a _negative_ charge and **val** is **neutral**. Changing out the charge for neutral leads to _hydrophobic interactions_ between deoxygentated Hb, causing the sickling shape
1206
(+) VDRL of the spinal fluid indicates, what?
neurosyphilis
1207
_Medullablastoma_ 1. Location 2. Cell types 3. Who?
1. Cerebellum 2. Primitive, blue cells (homer-wright rosettes) 3. Children
1208
If uncorrected, what can vesicoureteral reflux lead to?
Loss of nephrons and secondary HTN (due to all the inflammation)
1209
What does EBV bind on the B-cell to gain entry?
CD21
1210
Effect of lactose intolerance on stool pH
Bacterial fermentation of lactose **lowers stool pH**
1211
_Serum sickness_ 1. What is it? 2. What causes it?
1. Type III HSN rxn to non human proteins, resulting in IC deposition 2. Fever, pruritis, skin rash, arthralgia, dec. complement levels
1212
For patients w/ IBD, how are mutations leading to colorectal cancer different from the ones which lead to sporodic CRC?
IBC patients develo early p53 and late APC mutations
1213
Characteristic histo assc. w/ _Reye syndrome_
Microvesicular steatosis of hepatocytes (no inflammation or edema)
1214
What dz is this associated with?
Gaucher | (Most common LSD)
1216
What portions of the nephron are most suceptible to ATN and why?
The straight _proximal tubule_ and _thick ascending Loop of Henle_ (higher oxygen demands due to active transport of ions)
1217
Where are indirect and direct hernias located (relative to the inferior epigastric)?
_Indirect_: lateral _Direct_: medial
1218
Prevention of neonatal tetanus
**Maternal vaccine** (passive immunity via transplacental IgG)
1219
Describe the ulcers assc. w/ chlamydia
small and shallow + large, painful LNs
1220
Describe the ulcers assc. w/ syphillus
single, well-circumbscribed
1221
What is this?
**Candida albicans** | (germ tube formation at 37 degrees C)
1222
_Calcipotriene_ MOA and indication
Psoriasis med that is a **vitamin D** analog, which binds to the vit D receptor and _inhibits keratinocyte proliferation_
1224
What medications should be avoided for individuals with hypertrophic cardiomyopathy?
Any that decrease preload and/or **_systemic vascular resistance_** (you want both to be relatively high)
1225
Where does the majority of water reabsorption occur (regardless of hydration status)?
PCT
1226
Differentiate pilocytic astrocytoma and medullablastoma
Both are in kids 1. Pilo is more common and imaging shows both solid and cystic components 2. Medullo is always solid
1227
What is the job of **fructose 2,6 bisphosphonate**?
Helps control the balance between gluconeogenesis and glycolysis
1228
_Temporomandibular Disorder_ 1. What is wrong? 2. Presentation
1. Damage/issue with the _mandibular division_ of the **trigeminal nerve** 2. Pain/sx within the middle ear issues and muscles of mastication
1229
Why do patients w/ MS develop urinary incontinence?
Loss of inhibition of detrusor contraction in the bladder
1230
What is the main way in which UV rays cause DNA damage? (How is it corrected?)
Formation of abnormal pyrimidine-pyrimidine covalent bonds (**pyrimidine dimers**) Corrected via nucleotide excision repair
1231
_Lynch Syndrome_ 1. What is it? 2. What genes are mutated?
1. Autosomal dominant dz caused by nucleotide mismatch repair 2. Mutation in MSH2 and MLH1
1232
_Calcineurin_ Essential for what? What (2) drugs block it?
Essential for _IL-2_ activation Blocked by the immunosuppressants **cyclosporine** and **tacrolimus**
1233
Effect of high altitude exposure on blood gases
Hypoxemia + respiratory alkalosis
1234
What is HER2?
Tyrosine kinase receptor
1235
Most important environmental risk factor for pancreatic CA
Smoking
1236
_Name this psych disorder_: Eccentric, odd beliefs/magical thinking, interpersonal awkwardness/anxiety (despite familiarity)
Schizotypal
1237
How might alpha-1 antitrypsin deficiency appear on histo?
PAS (+) granules of unsecreted, polymerized AAT in the hepatocytes
1238
What is responsible for local defense against *Candida*? Systemic defense?
_Local_: T cells _Systemic_: Neutrophils
1239
What role can CYP450 play in carcinogenesis?
Many pro-carcinogens rely on **CYP450 monoxygenase** metabolization to become activated (and thus dangerous)
1240
_Rifaximin_ 1. MOA 2. Indication
1. Antibiotic which alters GI flora to decrease intestinal production and absorption of ammonia 2. **Hepatic Encephalopathy** (too much ammonia due to liver damage)
1241
TOC for trigeminal neuralgia
Carbamazepine
1242
What values characterize the dyslipidemia seen in insulin resistance?
**High TGs and low HDL** | (no LDL involvement)
1243
What kind of receptor is JAK2?
**_Cytoplasmic_** tyrosine kinase receptor
1244
Path of HbC dz
_Missence mutation_ leading to **glutamate → lysine**, change
1245
What nerve provides sensation to the _external auditory canal?_
Vagus branch (auricular branch)
1246
Name the (3) muscles/ muscle groups involved in sitting up
1. **_h_**ip flexors (**iliopsoas**) 2. **_e_**xternal abdominal oblliques 3. **_r_**ectus abdominus (I got up for **HER**)
1247
**Achondraplasia** inheritance
Autosomal dominant
1248
Where do most RCCs originate from?
The **epithelial cells** of the _proximal renal tubules_
1249
_Factor V Leiden_ 1. What is it? 2. Consequences?
1. Inherited mutation causing factor Va resistance to inactivation by **Protein C** 2. Thrombosis in young person (usually pulmonary)
1250
Role of *N. meningitidis* pili (fimbriae)
Adherence to nasopharynx epithelium
1251
What parts of the brain are most susceptible to "global ischemia"?
Pyramidal cells of the neocortex or **Hippocampus (#1!!!)** and _Purkinje cells_ of the cerebellum
1252
1253
PKU inheritance
Autosomal recessive
1254
What is responsible for the presentation assc. w/ _Pemphigus Vulgaris_?
IgG autoantibodies against **desmosomal proteins** (eg, desmoglein-- connects epithelial cells to epithelial cells)
1255
What is responsible for the presentation assc. w/ _bullous pemphigoid_?
IgG antibodies against hemidesmosomes (connect epithelium to BM)
1256
Describe the potassium levels in patients w/ DKA
Most have normal/high _serum_ levels of potassium, despite a total body (i.e. intracellular) deficit. d**K**a = dec. **K+** (you must replace potassium as you manage these patients)
1257
What accounts for the bimodal distribution of isoniazid metabolism speed in the population?
The presence of genetically "fast" and "slow" acetylators, as INH is metabolized by acetylation. (slow acetylators = inc. risk of AE)
1258
What are metalloproteinases composed of?
Zinc
1259
Which SITS muscle is most vulnerable in a rotator cuff injury and why?
**Supraspinatus** (Due to impingement between the acromion and the head of the humerus)
1260
_*BRAF* V600E_ mutation is associated w/ dz?
Melanoma
1261
_Dengue Fever_ Presentation
1. Retro-orbital pain 2. Break-bone fever (hemorrhagic) 3. Rash 4. Thrombocytopenia
1262
Primary vs secondary infxn of _Dengue_
_Primary_: often asymptomatic _Secondary_: often much worse (**different serotype**)
1263
Shiga-like toxin MOA
Inactivates 60S ribosomal subunit
1264
What is often seen in renal tubular lumen for patients with _multiple myeloma?_
**Bence-Jones proteins** (large eosinophilic casts in tubular lumen)
1266
Why does Hep C not integrate into the host genome?
It lacks reverse transcriptase
1267
What are the (2) key growth factors that promote angiogenesis in neoplastic and granulation tissues?
1. VEGF 2. **Fibroblast growth factor**
1268
Why is the H-flu vaccine conjugated to a tetanus toxoid or n. menigitidis membrane protein?
Needed to elicit Th2 response
1269
In addition to its anti-seizure effects, what other use does _valproate_ have?
It can tx manic episodes assc. w/ bi-polar dz
1270
What is the common mechanism for the protective factors against ovarian CA?
Anything htat **decreases the amount of repair** at the ovarian surface which ust take place as a result of lifetime ovulatory frequency (ex. oral contraceptives, multiparity, breastfeeding)
1271
How does the effect of exogenous secretin differ between normal gastric G cells and ZE gastrinomas?
Secretin (in addition to secreting biochem), _normally_ serves to **decrease gastrin** secretion from G cells. In _ZE_, it paradoxically causes an **increase in gastrin**
1272
_Interferons a and B_ 1. What produces them? 2. What is their role?
1. Produced by most human cells in response to viral infections 2. Halts viral protein synthesis and promotes apoptosis of infected cels
1273
_Splenic Vein Thrombosis_ 1. How might it occur? 2. How might it present in imaging?
1. Chronic pancreatitis can push a clot into the **splenic vein** 2. It would show varices belonging to only the veins of the gastric fundus
1274
What do you give to treat TCA overdose?
Sodium bicarb
1275
The toxin for traveler's diarrhea is similar to what other toxin?
Cholera toxin
1276
What enzymes require thiamine as a cofactor? (2)
Pyruvate dehydrogenase and a-ketoglutarate dehydrogenase
1277
What route does shigella use for invasion?
Microfold (M) cells of the Peyer patches
1278
_Superoxide dismutase_ Function
Neutralizes ROS, preventing cell injury
1279
What is _myoedema_ and what is it associated with?
Focal "mounding" of muscle (lump formation) following percussion. Assc. w/ **hypothyroid myopathy**
1280
How do you distinguish between *_tuberculoid leprosy_* and _*lepromatous leprosy*?_
Give the **lepromin skin test** (similar to a PPD) (+) in patients with tuberculoid leprosy (strong Th1 cell-mediated response to *mycobacterium leprae*) (-) in patients w/ lepromatous leprosy (they have a weak Th1 response and a strong Th2 response to *m. leprae)*
1281
What compound tends to be low in patients w/ _narcolepsy_?
Orexin (**hypocretin**)
1282
_Ondansetron_ MOA/indication
Inhibits serotonin receptors and is used to treat N/V following chemo
1283
Subclavian Steal Syndrome
Severe stenosis of proximal subclavian artery, leading to reversal in blood flow from the contralateral vetebral artery to the ipsilateral vertebral artery
1285
Damage to what structure leads to a pure sensory stroke?
Thalamus
1286
Main cause for _Lacunar infarcts_
Small vessel occlusion/lipohyalinosis in the deep brain vessels, usually due to DM or HTN
1287
**Which fungi?** Mississippi and Ohio River Basins
Histoplasma capsulatum
1288
Common cause of acute bacterial arthritis in sexually active young adults.
*N. gonorrhoeae*
1289
Auer rods are chrystalized, what?
**PEROXIDASE!!! (MPO+)**
1290
How do the particles look in _silicosis_
**Birefringent** _silica particles_ surrounded by fibrous tissue
1291
**Transitional Cell Carcinoma (of the bladder)** Presentation
Painless, gross hematuria in an older male (Usually do to smoking or occupation)
1292
_Goodpasture syndrome_ Pathology? Presentation?
Anti GBM antibodies and anti-alveolar antibodies (Present w/ hemotysis and renal failure)
1293
What does _Aldolase B deficiency cause_?
Hereditary fructose intolerance
1294
_Milirinone/inamrinone_ 1. Drug type 2. Drug effect
1. Phosphodiesterase-3 inhibitor 2. Increases cAMP concentration leading to inc. **cardiac contractility** and **vasodilation**
1295
Why is **Primaquine** added to the tx regimen w/ **Chloroquine**?
Chloroquine only kills malaria in the blood stream, but has no effect on the latent hepatic infxns assc. w/ *P vivax* and *P ovale. Primaquine* can eradicate these hypnozoites, preventing relapse.
1296
In _sarcoidosis_ what enzyme is found to be increased in the serum?
ACE
1297
Name the _Retroperitoneal Abdominal Organs_
SAD PUCKER **S**uprarenal glands **A**orta/IVC **D**uodenum (except 1st part) **P**ancreas (head/body) **U**reters and bladder **C**olon (ascending and descending) **K**idneys **E**sophagus **R**ectum
1298
Damage to what side of the brain can lead to neglect?
The **non-dominant** side
1299
What is the status of sodium in the blood for CF patients?
Hyponatremia (they lose sodium via sweat because it is normally reabsorbed with Cl-)
1300
What's the main difference in symptoms between low-potency and high-potency anti-psychotics?
_Low_: non-neurological (sedation, anticholinergic, orthostatic hypotension) _High_: extrapyramidal
1301
What component of *n*. *meningitidis* assist it in resisting phagocytosis? What part is responsible for its toxic effects?
_anti-phagocytosis_: polysacharide capsule _toxicity_: outer membrane lipooligosaccharide (LOS), [analogous to LPS of enteric rods]
1302
What parts of the spinal cord can be affected by syringomyelia?
Commonly begins w/ the STT, but later stages will also compromise the DCT and/or the CST
1303
Serum antibodies to the _phospholipase A2 receptor_ is indicative of what?
**Membranous nephropathy** | (PLA2R is found in the podocytes)
1304
How can _Chron's lead to gallstones_?
Bile acids are normalaly reabsorbed in the terminal ileum, which is often inflamed in Crohn dz. Dec. bile acids = inc. cholesterol precipitation and stone formation.
1305
Why doesn't axonal regeneration occur in the CNS?
Persistence of myelin debris, secretion of neuroinhibitory factors, and development of dense glial scars.
1306
_Psoas abscess_ 1. Presentation 2. Dx
1. Fever, back pain, inguinal mass, difficulty walking 2. (+) psoas sign-- pain when hip is extended
1307
(2) key vascular signs of abusive head trauma
1. Subdural hemorrhage 2. Retinal hemorrhage
1308
What is the triad associated w/ _Normal pressure hydrocephalus_?
Wet, Wobbly, Wacky (urinary incontinence, ataxia, cognitive dysfunction)
1309
Synovial fluid showing leukocyte count \> 100,000 and no crystals **Dx**?
Septic arthritis
1310
How can _hypertriglyceridemia_ lead to acute pancreatitis?
If a patient has _\>1000 mg/dL of TGs_, the [FFA] exceeds the binding capacity of albumin and leads to **direct tissue injury**
1311
What (3) enzymes/rxns require _Biotin_ (vit. B7) as a cofactor? What can often block the fxning of biotin?
1. **Pyruvate carboxylase**: pyruvate → oxaloacetate 2. **Acetyl-CoA carboxylase**: acetyl-CoA → malonyl-CoA 3. **Propionyl-CoA carboxylase**: propionyl-CoA → methylmalonyl-CoA (Blocked by _Avidin_ found in **egg whites**)
1312
_Pick's Dz_ 1. Gross presentation 2. Microscopic presentation
1. Pronounced atrophy of frontal and temporal lobes 2. **Pick bodies** (cytoplasmic inclusions of microtubule-associated protein tau)-- silver staining
1313
Mutation of what gene leads to _Bilateral acoustic Neuroma_?
NF2 | (NF**_2_** = **_bi_**lateral acoustic neuroma)
1314
AE of Ethambutol
Optic neuropathy
1315
Presentation assc. w/ weakness in the _trapezius muscle_
1. Impaired abduction above the horizontal 2. Shoulder drooping
1316
_Central Retinal Artery Occlusion_ 1. Presentation 2. Funduscopic exam
1. Sudden, painless, monocular blindness 2. "Cherry-red macula" (has its own blood supply) and pale retina
1317
Why is hypernatremia not seen in patients w/ hyperaldosteronism?
**Aldosterone escape**: Inc. sodium → inc. GFR → filtration → dec. sodium retention (this prevents volume overload)
1318
Loss of which neurons is characteristic of _Huntington Dz_?
Caudate/Putamen | (indirect path of the motor circuit)
1319
Most common disorder of the urea cycle
Ornithine transcarbamylase deficiency (hyperammonemia and elevated urinary orotic acid)
1320
Where does the _great saphenous vein_ originate? Where does it travel up?
Originates at the **medial** side of the foot, courses anterior to the medial malleolus and then travels up the medial leg/thigh
1321
_Hepatic Angiosarcoma_ 1. Associated with what? 2. What do the tumor cells express?
1. Assc. w/ carcinogens such as **arsenic, thorotrast,** and **polyvinyl chloride** 2. Express CD31 (PECAM)
1322
What is nonbacterial thrombotic endocarditis most commonly associated with?
Advanced malignancy or inflammatory dz/sepsis
1323
What effect might carcinoid syndrome have on the heart?
**Plaque like deposits of fibrous tissue** on the right heart endocardium, which may progress to pulmonic stenosis and/or restrictive cardiomyopathy
1324
What is the job of _arachnoid granulations_?
To absorb CSF, to remove it from the subarachnoid space. (A malfunction here could cause communicating [normal pressure] hydrocephalus)
1325
What predisposes patients to _lymphangiosarcoma_?
**Persistent lymphedema** (due to chronic dilatation of lymphatic channels)
1327
What is an interscalene nerve block used for? (what must you be careful of?)
To anesthetize the _brachial plexus_ for shoulder/upper arm procedure. (Must be careful because the phrenic is almost always affected)
1328
**Anti-Yo, Anti-P/Q, and anti-Hu** are the most common antibodies associated with what dz?
**Paraneoplastic Cerebellar Degeneration** (an autoimmune attack of the cerebellum for CA patients)
1329
Bell's palsy
Idiopathic paresis of the facial nerve
1330
What is _Hypoxic-ischemic Encephalopathy_ and what would it show on autopsy?
It is global cerebral ischemia, due to profound cerebral hypoperfusion. (Shows **triangular watershed infarcts** at locations between the zones of perfusion of the major cerebral arteries)
1331
First line tx of essential tremor
Propranolol
1332
What cells stain (+) for **synaptophysin**?
Neurons | (and thus tumors of neuronal origin)
1333
Is ulnar deviation wrist a**_B_**duction or wrist a**_D_**duction?
a**_D_**duction
1334
_Buprenorphine_ MOA and potential effect
Partial opiod agonist w/ low activity but high affinity to mu-receptor-- thus it can precipitate w/drawal
1335
More than 1/2 of patients w/ Temporal Arteritis, have what sx associated?
**Polymyalgia Rheumatica!!**
1336
Most common COD in _Friedreich Ataxia_
**Hypertrophic cardiomyopathy**
1337
Phrenic nerve arises from which spinal cord segments?
C3-C5
1338
Options to assist patients in quiting smoking
1. **Varenicline** (partial agonist of **_nic_**otinic Ach receptors 2. Bupropion
1339
What characterizes Adenocarcinoma in situ of the lung?
This preinvasive lesion is characterized by _growth along intact alveolar septa_, w/o vascular or stromal invasion. Can spread aerogenously and be invasive!
1340
What do _Granulosa Cell Tumors_ of the Ovary, secrete?
Estrogen
1341
_Cavernous Sinus Thrombosis_ Presentation
Deficits in CN's III, IV, V and VI, following an infection
1342
Primary mechanism for excess copper removal in the healthy human body?
**Hepatic excretion** in the _bile_
1343
Role of _glycerol kinase_
Breakdown glycerol (from TGs) to eventually create energy/glucose
1344
_Prostacyclin_ action
1. Vasodilates 2. Inhibits platelet aggreggation 3. Increases vascular permeability (All the opposite of thromboxane A2)
1345
**Diphenoxylate** MOA and indication
**Anti-diarrheal** Binds mu-opiod receptor to slow motility of the gut
1346
What role can proteosomes play in tx of _multiple myeloma_?
_Plasma cells_ in MM are producing lots of protein so they are particlular susceptible to proteosomes. The result is **apoptosis**.
1347
How does INH lead to peripheral neuropathy?
By decreasing vitamin B6 levels. It is **_NOT_** DIRECTLY TOXIC ITSELF!
1348
The PI3K/Akt/mTOR pathway, is an intracellular pathway for what?
1. Anti-apoptosis 2. Pro-cell proliferation 3. Pro-angiogenesis (All play a role in CA)
1349
What is the best follow-up test for an elevated alk phosph of unknown etiology?
y-glutamyl transpeptidase
1350
Anti-mitochondrial antibodies are typically found in what disease?
Primary biliary cirrhosis
1351
What is the most common trigger for DIC in pregnancy?
The release of tissue factor (thromboplastin) from an injured placenta, into maternal circulation
1352
Prolonged QT + Neurosensory deafness ## Footnote **What Dz?**
Jervell and Lange-Nielsen Syndrome
1353
Where is H. pyolori found at the greatest concentration?
The prepyloric area of the gastric antrum (just before the pyloric sphincter)
1354
What are the (2) types of _Chronic Gastritis_?
_Type 1_: (fundus/body) **A**utoantibodies to **_parietal_** cells → pernicious anemia + achloridia _Type 2_: (antrum) H. pylori **B**acteria → inc. CA risk (MALT or adenocarcinoma) "A" comes before "B"
1355
Toxic megacolon is mostly associated with which IBD? Sx + Dx?
**Ulcerative colitis** Abdominal pain/distension, bloody diarrhea, signs of shock. **Abdominal XR** (colonoscopy and barium contrast study are contraindicated due to perforation risk)
1356
What are the (2) distinct regulatory mechanisms of the *lac* operon?
1. _**N**egative_ via binding of the repressor protein to the **O**perator locus 2. _**P**ositively_ via binding by cAMP-CAP binding upstream from the **p**romoter region N-O = NO **_p_**ositive = **_p_**romoter
1357
_Chronic Mesenteric Ischemia_
Atherosclerosis involving the mesenteric arteries, which leads to bowel hypoperfusion during meals and subsequent pain.
1358
What causes the _esophageal dysmotility_ associated w/ CREST syndrome?
Atrophy and **fibrous replacement** of the muscularis in the lower esophagus. Leads to the LES becoming atonic and dilated (can result in GERD)
1359
_CEA_ levels are useful for the monitoring of what dz?
Colon CA
1360
_Necrotizing enterocolitis_ 1. Path 2. Pt demographic
1. Bacterial invasion + ischemic necrosis of the bowel wall 2. Asc. w/ premature newborns and enteral feeding (tube to stomach)
1361
_Necrotizing enterocolitis_ Dx
Abdominal XR showing air in the bowel wall
1362
The presence of _erythroid precursors_ in organs such as the liver and spleen is indicative of what?
Extramedullary hematopoiesis (oft assc. w/ severe chronic hemolytic anemia, such as B-thalassemia)
1363
What compound is added to stored blood and what effect might it have on a patient?
**Citrate** It chelates Ca2+ and Mg, and thus may reduce their plasma levels
1364
Lipid A
The toxic component of _LPS_ (Causes macrophage activation leading to sx of septic shock)
1365
Autism spectrum disorder is typically recognized by what age?
2y
1366
What determines whether or not an enveloped virus can attach to a specific host cell?
Whether or not a viral envelope glycoprotein w/ a high binding affinity for a host cell surface **_glycoprotein_** is present
1367
EBV commonly stimulates B cells to do what?
**Proliferate continously** | (ie immortalization)
1368
Monospot test is aka what?
**Heterophile Antibody Test**
1369
**Pseudomonas** Motile or non-motile?
**MOTILE!!**
1370
Key difference between B-ALL and T-ALL
T-ALL has **t****hymic mass**
1371
What are the (2) major causes of concern w/ _theophylline intoxication_?
1. Seizures 2. Tachyarrhythmias
1372
What are the (2) most important steps in workup of metabolic alkalosis?
Ascertaining the patient's _volume status_ and _urine Cl-_
1373
Longterm risk w/ PCOS (and why?)
**Endometrial hyperplasia/carcinoma** (anovulation leads to progesterone-estrogen misbalance)
1374
Meglitinides MOA
Close the ATP-dependent _K+ channel_ in the pancreatic beta cell membrane, inducing depolarization and thus stimulating insulin release
1375
What drugs tx N/V from... Chemo? GI issues?
_Chemo_: dopamine receptor antagonists, serotonin antagonists, Neurokinin 1 (nK1) receptor antagonists _GI_: antimuscarinics, antihistamines
1376
Activating mutations of the KRAS gene lead to what?
Constitutive activation of the epidermal growth factor (EGFR) pathway, promoting increased cell proliferation and growth.
1377
_Drug-Induced Interstitial Nephritis_ Presentation
**Fever, rash, and acute renal failure** following administration of certain drugs (ex. penicillin derivatives). Also see pus (often eosinophils) in the urine
1378
Wet vs Dry _Age-related Macular Degeneration_
_Dry_: basic gradual vision loss in one or both eyes _Wet_: Inc. VEGF, in response to Dry AMD
1379
Dx of Wet Age-related Macular Degeneration
Fundoscopy showing grayish-green subretinal discoloration w/ adjacent fluid/hemorrhage
1380
Key difference between dysplasia and carcinoma
Dysplasia is _reversible_
1381
What does **bone-specific alkaline phosphatase** reflect?
Osteoblastic activity
1382
What do **TRAP**, **u****rinary hydroxyproline**, and**urinary deoxypyridinoline** reflect?
_Osteoclastic_ activity (**urinary hydroxyproline** is the most reliable)
1383
Phencyclidine MOA
_PCP_ is an NMDA receptor **antagonist**
1384
Why do reticulocytes stain blue w/ Wright-Giemsa stain?
Residual ribosomal RNA
1385
How does PSGN look on light microscopy? On IF? On EM?
_light_: enlarged, hypercellular glomeruli _IF_: "lumpy-bumpy" granular deposits of **IgG** and **C3** _EM_: electron dense deposits on the epithelial side of the BM (camel hump)
1386
What is the role of T-tubules?
They bring AP into the interior of the muscle fibers
1387
Effect of severe htN in the retinal precapillary arterioles
Endothelial disruption, necrosis and subsequent hemorrhage
1388
Main dose-limiting AE of vincristine?
Neurotoxicity
1389
What happens to phosphate levels in kidney dz?
They increase, due to a decreased filtered load
1390
What does a _stool guiac test_ screen for?
Occult blood
1391
Rat poison has the same toxicities as what drug? How do you tx?
**WARFARIN** (so tx w/ FFP)
1392
Terbinafine MOA
Antifungal which inhibits squalene epoxidase
1393
What can reverse the effects of methotrexate?
N-f-THF (folinic acid, leucovorin) supplementation
1394
_Essential fructosuria_ 1. What is it?
_Benign_ autosomal recessive disorder which causes some dietary fructose load to be secreted in the urine unchanged (due to defective fructokinase metabolism)
1395
Testing for fructose in the urine
(+) copper reduction test (-) urine dipstick
1396
What is one of the most serious complications of the *recovery phase* of ATN?
Hypokalemia (can cause arrhythmia)
1397
What are the (2) most characteristic signs of _dermatomyositus_?
Gottron papules (pictured) and heliotrope rash (rash on eyelids)
1398
Presentation of _IgA nephropathy_ (Berger dz)
_Recurrent_, self-limited, **painless hematuria** w/in 5 days after a _URI_
1399
List the cephalosporin-resistant organisms
**LAME** Listeria, MRSA, Enterococci, Atypicals
1401
Placenta accreta
When the placenta adheres to the myometrium as opposed to the endometrium (can cause severe postpartum hemmorhage)