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USMLE Step 1 > Random First Aid Facts > Flashcards

Random First Aid Facts Flashcards

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USMLE® Step 1 flashcards
1
Q

Amino acids necessary for purine synthesis?

A

GAG = Glycine, Aspartate, Glutamine

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2
Q

Where is the action of Leflunomide?

A

It inhibits dihydroorotate dehydrogenase and interferes with synthesis of orotic acid from carbamoyl phosphate thereby inhibiting pyrimidine synthesis.

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3
Q

What is the effect of alpha-amanatin toxin and where does it come from?

A

Comes from the mushroom Amantia phalloides (death cap mushroom) and inhibits RNA pol II. Results in severe hepatotoxicity if ingested

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4
Q

What are P-bodies?

A

They are cytoplasmic bodies with exonucleases, decapping enzymes, and microRNAs that act as a quality control check point and storage reservoir for mRNAs.

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5
Q

Describe tRNA structure:

A

Amino acid bound to the 3’ end. Going 3’ to 5’ we have amino acid, T-arm (binds to ribosome), Variable arm, anti-codon loop, and D-arm (dihydrouracil residues for recognition by tRNA synthetase).

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6
Q

What is the pathophysiology of I-cell disease?

A

Defect in phosphotransferase so the Golgi can’t phosphorylate mannose residues and therefore nothing can be traficked to lysosomes. Get accumulation of lysosomal enzymes in the blood. Coarse facial features, clouded corneas, decreased joint mobility. Fatal in childhood.

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7
Q

Where is Collagen Type 1 found

A

Bone, Skin, Tendon, fascia, cornea, late wound repair

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8
Q

Collagen Type 2:

A

Cartillage, vitreous body, nucleus pulposus

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9
Q

Collagen Type 3:

A

Reticulin (Skin), blood vessels, uterus, fetal tissue, granulation tissue. Implicated in the rare vascular type of Ehlers-Danlos syndrome

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10
Q

Collagen Type 4:

A

Basement membrane, lens of the eye

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11
Q

What three processes happen to Collagen in the RER?

A

Synthesis, hydroxylation, and Glycosylation and formation of the triple helix. It is now procollagen. Defects in this point cause OI.

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12
Q

What disease do problems with collagen crosslinking lead to?

A

Ehlers-Danlos Syndrome

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13
Q

What is the pathophysiology of most common cause of OI?

A

Autosomal dominant with decreased production of otherwise normal Type 1 collagen.

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14
Q

What are the three types of Ehlers-Danlos Syndrome?

A

1) Hypermobility type (most common)
2) Classical Type (joint and skin symptoms); Type V collagen issues
3) Vascular type (vascular and organ rupture); Type III collagen.

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15
Q

What is Menkes disease?

A

It is due to impaired Cu absorption and transport thereby decreasing the activity of lysyl oxidase. Brittle, “kinky” hair, growth retardation, hypotonia

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16
Q

When should we consider Uniparental disomy?

A

When an individual is manifesting a recessive disorder and only one parent is a carrier! Note can be a meisosis 1 error (Heterodisomy) or meisosis 2 error (Homodisomy)

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17
Q

According to H-W equilibrium, what is the freqneyc of an X-linked recessive disease in Males and Females?

A

Males = q and females = q^2

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18
Q

What acid base abnormality can CF present with?

A

Contraction alkalosis and hypokalemia due to the decreased ECF H2O and Na+ content.

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19
Q

What is the treatment for CF?

A

N-Acetyl Cysteine can loosen mucous plugs and dornase alfa (DNAse) to clear leukocyte debris.

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20
Q

Why is the the dystrophin protein at an increased risk of spontaneous mutations?

A

It is the longest coding region of any human gene

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21
Q

What is the pathophysiology of Myotonic Dystrophy?

A

CTG trinucleotide repeat expansion in the DMPK gene (AD inheritance).
Sx: Myotonia ( slow relaxation!), wasting, frontal balding, cataracts, testicular atrophy, arrhythmia.
Type 1 fibers more affected

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22
Q

What is the various trinucleotide Repeat sequences:

A 
X-Girlfriends First Aid Helped Ace My Test
Fragile X: CGG
Friedreich ataxia: GAA
Huntingtons: CAG
Myotonic Dystrophy: CTG
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23
Q

What is the Leser-Trelat sign?

A

Sudden appearance of multiple seborrheic karatoses can indicate an underlying malignancy (e.g. GI, lymphoid)

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24
Q

What skin condition is associated with celiac disease?

A

Dermatitis Herpetiformis (IgA deposition at the tips of the dermal papillae). Pruritic papules, vesicles, and bullae.

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25
Q

What differentiates Toxic Epidermal Necrosis from Stevens-Johnson syndrome?

A

In TEN, >30% of the body surface area is involved

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26
Q

What are the 6 P’s of Lichen Planus?

A

Pruritic; Purple Polygonal Planar Papules and Plaques.
Also might see reticular white lines on mucosa (Wickham striae). Sawtooth infiltrate of lymphocytes at dermal-epidermal junction. HCV associated.

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27
Q

What does UVA vs. UVB cause?

A

UVA - tanning and photoaging

UVB - sunburn

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28
Q

What is the ABCDE of melanoma?

A

Asymmetry, Border Irregularity, Color Variation, Diameter > 6 mm, Evolution over time

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29
Q

What amino acid is Vitamin B3 derived from?

A

Tryptophan, but it requires vitamin B2 and B6 in the process

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30
Q

Deficiency of what vitamin is associated with “Burning Feet Syndrome”?

A

Vitamin B5 (Pantothenate)

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31
Q

What does Arsenic due to glycolysis?

A

It causes glycolysis to produce zero net ATP. It also inhibits lipoic acid (i.e. inhibits Pyruvate Dehydrogenase and others).

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32
Q

What are the poisons of Oxidative Phosphorylation?

A 
Rotenone - Complex One
Antimycin A - Complex III
Cyanide - Complex IV
Oligomycin - Complex V
Aspirin, 2,3-DNP, Thermogenin - Uncoupling Agents
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33
Q

What is the mechanism of action of lactulose?

A

Acidifies the GI tract and traps NH4+ for excretion in the feces

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34
Q

What direction is lens subluxation in hyperhomocysteinemia?

A

Down and in = “Down to Home”

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35
Q

What is Cheyne-Stokes respiration?

A

It is periods of apnea and normal breathing due to slowed respiratory feedback loop. Seen in neurologic and cardiac disease and it is a poor prognostic sign.

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36
Q

What is an Osler node?

A

Tender raised lesions on finger or toe pad. Vs. Janeway lesions which are small painless erythematous lesions on palms and soles

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37
Q

What is Beck’s Triad of Cardiac Tamponade?

A

Hypotension, distended neck veins, and distant heart sounds

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38
Q

What is Kussmal’s Sign?

A

A paradoxical rise in JVP upon inspiration. Usually due to failure to transmit negative intrathoracic pressure to the heart (e.g. constrictive pericarditis, restrictive cardiomyopathies, tumors)

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39
Q

What is a pyogenic granuloma?

A

is a vascular lesion that occurs on both mucosa and skin, and appears as an overgrowth of tissue due to irritation, physical trauma or hormonal factors (e.g. pregnancy). Can ulcerate and bleed

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40
Q

What is a Glomus tumor?

A

Red-blue tumor under fingernails that is painful. From modified smooth muscle of the glomus body

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41
Q

What changes are seen in GnRH pulses with exercise-induced vs. ovarian defect ammenorrhea?

A

Exercise-induced: decreased amplitude and frequency

Ovarian defect: increased amplitude and frequency

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42
Q

What are the symptoms of glucagonoma?

A

Necrolytic migratory erythema, erythematous rash in groin, hyperglycemia, stomatitis, cheilosis, abd pain

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43
Q

What is a physical exam difference between neuroblastoma and Wilms tumor?

A

Wilms tumor is smooth and unilateral wheras neuroblastoma is irregular and can cross the midline

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44
Q

What is the Jod-Basedow phenomenon?

A

Thyrotoxicosis in a patient with iodine deficiency goiter when made iodine replete

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45
Q

How do you treat thyroid storm?

A

The 3 p’s = Propranolol, Propylthiouracil, Prednisolone (steroids)

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46
Q

What is Chvostek’s sign?

A

tapping of facial nerve leads to contraction of facial muscles (hypocalcemia sign)

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47
Q

What is Trousseau’s sign?

A

occlusion of brachial artery with BP cuff leads to carpal spasm (hypocalcemia sign)

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48
Q

What is the rule of 1/3s of carcinoid tumors?

A

1/3 metastasize, 1/3 present with 2nd malignancy, 1/3 are multiple.

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49
Q

What drug is prefered over metforim in renal failure patients?

A

Glipizide, as it undergoes hepatic clearance

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50
Q

What effects do glitazones have on blood lipids?

A

Decrease triglycerides and increase HDP, also decrease CRP

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51
Q

What enzyme catalyzes rate limiting step in bile synthesis?

A

Cholesterol 7-alpha hydroxylase

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52
Q

What is Menetrier disease?

A

Gastric hypertrophy with parietal cell atrophy. It is precancerous and the rugae of stomach are hypertrophied so they look like brain gyri

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53
Q

What is the Peutz-Jeghers syndrome?

A

Multiple non-malignant hamartomas along GI tract, hyperpigmented mouth, lips, hands, genitalia. Increased malignancy risk. AD

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54
Q

What is Gardner syndrome?

A

FAP + osseous and soft tissue tumors + congenital hypertrophy of retinal pigment epithelium

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55
Q

Turcot syndrome?

A

FAP + malignant CNS tumors

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56
Q

What are Mallory bodies?

A

Intracytoplasmic eosinophilic inclusions in hepatocytes due to long-term alcohol use

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57
Q

What are some triggers of hepatic encephalopathy due to increased NH3 production?

A

GI bleeding, constipation, high protein meal, infection

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58
Q

What are some triggers of hepatic encephalopathy due to decreased NH3 removal?

A

Renal failure, diuretics, post-TIPS

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59
Q

What is rifaximin and what is it used for?

A

Antibiotic that kills intestinal bacteria, used to treat hepatic encaphalopathy by reducing NH3 load (also Rx travelers diarrhea)

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60
Q

What is the mechanism of NH3 induced encephalopathy?

A

Increases glutamine in astrocytes thereby decreasing the total pool of glutmate stores in the brain and depleting alpha-ketoglutarate needed for Kreb’s cycle. It also causes mitochondrial swelling and dysfunction in astrocytes.

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61
Q

What liver tumor to OCPs commonly cause?

A

Hepatic Adenoma, rupture can lead to shock

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62
Q

What is a notable physical exam finding in Budd-Chiari syndrome?

A

No JVD! since the obstruction is in the hepatic vein.

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63
Q

What are some causes of Budd-Chiari syndrome?

A

Hypercoagulable states, polycythemia vera, pregnancy, HCC

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64
Q

What is a hepatoblastoma?

A

It is a liver tumor seen in Kids that is associated with FAP and Beckwith-Wiedman syndrome. Fatal if not resected

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65
Q

What two diseases should PAS + globules make you think of?

A

Whipple’s disease and alpha-1 antitrypsin deficiency

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66
Q

What vitamin should infants receiving phototherapy be treated with?

A

Vitamin B2

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67
Q

What drug can be used to treat type II Criegler-Najjar?

A

Phenobarbital

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68
Q

Does Rotor or Dubin-Johnson syndrome cause a black liver?

A

Dubin-Johnson syndrome causes a black liver

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69
Q

What can the motor symptoms of Wilson’s disease be characterized as?

A

Parkinsonian symptoms due to degeneration of the Basal Ganglia

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70
Q

What is the genetic associations of hemochromatosis?

A

C282Y or H63D mutation on the HFE gene which senses iron levels and is involved inregulation. HLA-A3 association

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71
Q

What are the characteristics of Beckwith-Wiedmann Syndrome?

A

macroglossia, macrosomia (birth weight and length greater than the 90th percentile), midline abdominal wall defects (omphalocele/exomphalos, umbilical hernia, diastasis recti), ear creases or ear pits, and neonatal hypoglycemia (low blood sugar after birth)

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72
Q

What is the histologic pattern of bile duct fibrosis in PSC?

A

Onion Skin pattern is seen. Also see alternating strictures and dilation with “beading” of both extra and intra hepatic bile ducts on ERCP

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73
Q

What color are the stones in biliary infection?

A

brown, radio-lucent

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74
Q

What is the Charcot triad of cholangitis?

A

Jaundice
Fever
RUQ Pain

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75
Q

What are the causes of acute pancreatitis?

A

GET SMASHED
Gallstones, Ethanol, Trauma, Steroids, Mumps, Autoimmune, Scorpion, Hypercalcemia/Hypertriglyceridemia, ERCP, Drugs (e.g. sulfa)

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76
Q

What is the lining of a pancreatic pseudocyst?

A

Granulation tissue, not epithelium, hence not true cyst

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77
Q

What is Courvoisier’s sign?

A

Obstructive jaundice with a palpable non-tender gallbladder (sign of Pancreatic CA)

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78
Q

What population usually has cis deletions in alpha thalasemia?

A

Asians.

Africans have trans

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79
Q

What is the difference between Hb Barts and HbH?

A

Hb Barts = 4-gamma globins

HbH = 4 beta globins

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80
Q

What is the basophilic stippling seen in lead poisoning caused by?

A

rRNA aggregates that form due to inhibition of their breakdwon

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81
Q

What are some features of Lead poisoning?

A

Lead Lines on Gingivae and metaphysis of long bones
Encephalopathy
Abdominal Colic
Basophilic stipling and Sideroblastic Anemia
Wrist and foot Drops

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82
Q

What do labs shows in sideroblastic anemia?

A

Increased Iron
Normal TIBC
Increased ferritin

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83
Q

What is the treatment for sideroblastic anemia?

A

pyridoxine to improve heme synthesis

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84
Q

What is the Eosin-5-maleimide binding test used for?

A

Screening for Hereditary Spherocytosis

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85
Q

What is eculizumab?

A

It is a terminal complement inhibitor used for the rx of PNH (binds C5 and inhibits cleavage)

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86
Q

What two hematologic conditions are associated with a “crew cut” skull on X-ray?

A

Sickle Cell and Thalasemias

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87
Q

What is transferrin saturation?

A

= serum iron / (TIBC or Transferrin levels)

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88
Q

What medication and conditions causes increased transferrin production?

A

OCPs and Pregnancy, this in turn results in decreased % saturation

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89
Q

What effect do corticosteroids have on blood counts?

A

Neutrophilia (decreased migration), Lymphopenia (apoptosis of lymphs), Eosinopenia (sequester in LNs)

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90
Q

What is the defect in X-linked sideroblastic anemia?

A

delta-ALA synthase deficiency

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91
Q

What are the two precusors of ALA?

A

Glycine and Succinyl-CoA

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92
Q

True or false, a 1:1 dilution of patients blood will fix a clotting factor deficiency?

A

Yes, b/c most factors can decrease by 60% w/o changing coag studies at all

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93
Q

What are the symptoms of acute intermittent porphyria?

A 
5P's:
Painful abdomen
Port wine colored urine
Polyneuropathy
Psychological disturbacnes
Precipitated by drugs, EtOH, starvation
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94
Q

What is the treatment for acute intermittent porphyria?

A

Give glucose and heme which inhibit ALA synthase

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95
Q

What is the color of urine in porphyria cutanea tarda?

A

Tea-colored

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96
Q

What is the result of a ristocetin cofactor assay on someone with vWD?

A

There is decreased agglutination of platelets as the vWF is not present or not functional. Note that in Type 2B, there is increased agglutination on this assay and it is diagnostic

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97
Q

How do you differentiate a leukemoid reaction from CLL?

A

In a normal leukemoid reaction, ALP is increased, whereas it is decreased in CML

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98
Q

What differentiates waldenstrom macroglobulinemia from Multiple Myeloma?

A

The M spike is IgM instead of IgG or IgA and there are no lytic bone lesions. Hyperviscosity symptoms predominate

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99
Q

What is the pseudo-pelger-huet anomaly?

A

Neutrophils with bilobed nuclei connected with thin chromatin filament. Seen after chemo

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100
Q

What is the treatment of Hairy Cell Leukemia?

A

Cladribine, an adenosine analog

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101
Q

What are common presentations of Langerhans Cell Histiocytosis?

A

1) Lytic bone lesion and skin rash

2) recurrent otitis media with mass involving mastoid

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102
Q

What markers are expressed by langerhans cells?

A

S-100 and CD1a

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103
Q

What are the symptoms of essential thrombocytosis?

A

Bleeding and thrombosis due tot he overproduction of abnormal platelets

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104
Q

True or false, LMWH binds thrombin?

A

False! LMWH binds factor Xa only! They hve a longer half life and better bioavailability

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105
Q

What is a Housemaids Knee?

A

Due to prepatellar bursitis

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106
Q

What causes Anserine Buristis?

A

Commonly obesity or athletic overuse. Medial knee pain

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107
Q

What is the most common location of rib fracture?

A

Anterior to the angle of the rib - we need to anasthetize it lateral to the angle of the rib

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108
Q

Why is clawing seen best with distal lesions of the median or ulnar nerve?

A

The remaining extrinsic flexors of the wrist exagerrate the loss of the lumbricals. For proximal lesions, they are more pronounced during voluntary flexion

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109
Q

What compartments does the common peroneal nerve innervate?

A

The anterior (deep peroneal) and lateral (superficial peroneal) compartments. Injured in a fibular neck fracture

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110
Q

Where is it safest to give a gluteal injection?

A

The superolateral quadrant to avoid the superior gluteal (superomedial) and sciatic (inferomedial)

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111
Q

Is the lesion contra or ipsilateral to the side that drops in a trandelenburg sign?

A

It is contralateral to the hip that drops

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112
Q

What nerve roots are the achiles reflex and patellar reflex testing?

A

Achiles - S1

Patellar - L4

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113
Q

What are the polriazed light findings in gout and pseudogout?

A

Gout - yellow when parallel to the light (negative)

Pseudogout - blue when parallel to the light (positive)

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114
Q

What is the classic x-ray finding in psoriatic arthritis?

A

A pencil-in-cup deformity

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115
Q

What are the two types of lupus nephritis?

A

Nephritis - diffuse proliferative glomerulonephritis

Nephrotic - membranous glomerulonephritis

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116
Q

How does inflammation in polymyositis differ from dermatomyositis?

A

Polymyositis - Endomysial, CD8+ T-Cells

Dermatomyositis - Perimysial inflamm + atrophy, CD4+ T Cells

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117
Q

What is myositis ossificans?

A

Metaplasia of skeletal muscle to bone following muscular trauma

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118
Q

Are melanocyte numbers normal or abnormal in albinism?

A

Normal number with decreased melanin production (unless potentially caused by failure of cell migration in development)

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119
Q

What is Melasma/Chloasma?

A

Hyperpigmentation associated with pregnancy (Mask of Pregnancy)

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120
Q

In Urticaria, is edema in the dermis or epidermis?

A

Dermis

vs. the epidermis (spongiosis) in Eczema

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121
Q

What causes increased coloration in an Ephelis/Frekel?

A

Increased melanin production in a normal number of melanocytes

122
Q

What is Auspitz sign?

A

Pinpoint bleeding spots from exposure of the dermal papillae when scales are scraped off in psoriasis

123
Q

What is the difference microscopically between staph scalded skin syndrome and TEN?

A

In scalded skin syndrome, attachments only destroyed in stratum granulosum vs. TEN which destroys dermal-epidermal junction

124
Q

What infectious disease is Lichen Planus associated with?

A

HCV

125
Q

What is pityriasis rosea?

A

It presents with a herald path followed by christmas tree distribution and self-resolves in 6-8 weeks

126
Q

What is keratoacanthoma?

A

A variant of SCC that grows rapidly and may regress spontaneously over months

127
Q

What are the drivatives of the 5 secondary brain vesicles?

A 
Telencephalon - Cerebral Hemispheres
Diencephalon - Thalamus
Mesencephalon - Midbrain
Metencephalon - Pons and cerebellum
Myelencephalon - Medulla
128
Q

What maternal condition is Anencephaly associated with?

A

Type 1 Diabetes

129
Q

What is a Dandy-Walker malformation?

A

Agenesis of cerebellar vermis with cystic enlargement of 4th ventricle

130
Q

What is Wallerian degeneration?

A

Degeneration of a PNS axon distal to the injury and retraction proximally

131
Q

What effect does myelin have on nerve conduction constants?

A

Increases the space constant and conduction velocity

132
Q

What do Meissner corpuscles sense?

A

Dynamic fine/light touch, position sense on Glabrous skin

133
Q

What do pacinian corpuscles sense?

A

Vibration and pressure in the deep skin layers, ligaments, joints

134
Q

What do Merkel discs sense?

A

They are slow adapting. Pressure, deep static touch, position sense

135
Q

What two areas of the brain are not protected by the BBB?

A

OVLT (osmolality sensor) and Area Postrema (Vomiting Center)

136
Q

What is the lateral are of the hypothalamus for?

A

Controls hunger sensation, inhibited by leptin

137
Q

What is the ventromedial area for?

A

Controls satiety, stimulated by leptin

138
Q

What happens if you destroy the posterior hypoathalmus?

A

You become cold-blooded (poikilotherm) like a snake

139
Q

What is a sign of a lateral cerebellar lesion?

A

Propensity to fall towards the ipsilateral/injured side

140
Q

What is the genetics of early onset Parkinson’s?

A

Autosomal Recessive disease with defects in the ubiquitin-proteasome pathway genes (e.g. Parkin, PINK-1, DJ-1)

141
Q

What does the abnormal huntigtin protein cause?

A

Decreased transcription of other genes via histone hypermethylation

142
Q

When is myoclonus commonly seen?

A

With metabolic abnormalities like renal or liver failure

143
Q

What is the treatment of essential/postural tremor?

A

Beta blockers, primidone, (Self-medicate with EtOH)

144
Q

Are Broca’s and Wernicke’s areas found in the dominant or non-dominant hemisphere?

A

Dominant

145
Q

What is Gerstmann Syndrome?

A

Lesion of the Left (dominant) parietal-temporal cortex causing agraphia, acalculia, finger agnosia, and left-right disorientation

146
Q

What results from correcting sodium to fast?

A

From low to high your pons will die

From high to low your brain will blow (cerebral edema/herniation)

147
Q

Is repetition good or poor in transcortical aphasias?

A

It is good

148
Q

What nerve runs between the PCA and superior cerebellar artery?

A

CN III

149
Q

What is the artery of percheron?

A

It is a variant artery from the PCA that can supply thalami and dorsal midbrain

150
Q

What main artery supplies the thalamus?

A

The PCA

151
Q

What are patients with acute cerebral edema hyperventilated?

A

Because this drops PCO2 and causes cerebral vasoconstriction and decreased ICP

152
Q

Strokes of which artery are associated wtih dysphagia and hoarsness?

A

PICA due to involvement of the nucleus ambiguus

153
Q

What symptoms are specific to an AICA lesion?

A

Facial nucleus affects

154
Q

Which aneurysms are associated with focal deficits and which with headache?

A

Headache - Berry Aneurysm

Focal Deficits - Charcot-Bouchard

155
Q

What is seen on histology of lacunar infarcts?

A

Lipohyalinosis and microatheromas

156
Q

What is Ataxia-Hemiplegia syndrome?

A

Caused by infarcts at the base of the pons usually due to HTN

157
Q

What is Dysarthria-Clumsy Hand Syndrome?

A

Caused by infarcts at the base of pons or genu of internal capsule, usually due to HTN

158
Q

What is the pterion?

A

It is the meeting place of the frontal, temporal, parietal, and sphenoid bones. Middle meningeal is deep to the pterion

159
Q

What do you treat a vasospasm with due to subarachnoid hemhorrage?

A

Nimodipine

160
Q

What is the most common cause of lobar intraparenchymal hemhorage?

A

Amyloid angiopathy

161
Q

What is the progression of histologic features of a stroke?

A

12-48 hrs: Red Neurons
24-72 hrs: Necrosis + Neutrophils
3-5 Days: Macrophages
1-2 wks: Reactive gliosis + vascular proliferation

162
Q

What is a clue towards an embolic stroke?

A

Multiple vascular territories can be affected

163
Q

What enzyme defect can lead to ALS?

A

Superoxide dismutase 1

164
Q

What drug can be used to treat ALS?

A

Riluzole (decreases glutamate release)

165
Q

Which spinal cord region is the ASA water shed territory?

A

The upper thoracic territory

166
Q

What muscle disorder should pes cavus and kyphoscoliosis point you towards?

A

Friedrichs Ataxia

167
Q

Does uvula deviate to same or opposite side for a CN X lesion?

A

The opposite side

168
Q

What are the signs of cavernous sinus syndrome?

A

Ophthalmoplegia and decreased corneal and maxillary sensation. Normal visual acuity

CN 6 most commonly affected

169
Q

What nerve is involved in coughing that lies in the piriform recess?

A

The internal laryngeal nerve

170
Q

What is Amarosis Fugax?

A

Painless transient monocular vision loss due to embolus to ophthalmic artery

171
Q

What does the directional term for INO refer to?

A

To the eye which is paralyzed (i.e. not the nystagmus eye)

172
Q

What protein is defective in Lewy Body Dementia?

A

alpha-synuclein

173
Q

What is a striking feature of CJD?

A

Startle Myoclonus

174
Q

What are the symptoms of Progressive Supranuclear Palsy?

A

EPS and dystonia of neck with paralysis of downward gaze

175
Q

What happens to nerve impulses in MS?

A

Decreased length constant, increased time constant = slower conduction

176
Q

Can autonomic function be affected in GBS?

A

YES

177
Q

What is the classic CSF finding in GBS?

A

albuminocytologic dissociation

178
Q

What is the most common site of inflammation in ADEM?

A

Periventricular

179
Q

What are two musculoskeletal association of Charcot-Marie-Tooth?

A

Scoliosis and foot deformities (arch problems) (Note ddx would include Friedrich’s Ataxia)

180
Q

What is the inheritence pattern and metabolic defect in Adrenoleukodystrophy?

A

X-Linked. Impaired VLCFA metabolism

181
Q

What is the most common cause of seizure by age group?

A

Children: Genetic
Adults: Tumors
Elderly: Stroke

182
Q

What does positional testing reveal for peripheral vertigo?

A

Delayed horizontal nystagmus

183
Q

What does positional testing reveal for central vertigo?

A

Immediate nystagmus in any direction (may CHANGE directions) + focal neuro findings

184
Q

What gene is mutated in Sturge-Weber Syndrome?

A

GNAQ (activating mutation), usually somatic/non-inherited

185
Q

What is the brain tumor seen in Sturge-WEber Syndrome?

A

Leptomeningeal angioma

186
Q

What is the brain tumor seen in Tuberous Sclerosis?

A

Subependymal Hamartomas

187
Q

What cells do meningiomas arise from?

A

Arachnoid cells

188
Q

What brain tumors can result in secondary polycythemia?

A

Hemangioblastoma (produce EPO)

189
Q

What is the capillary pattern of an oligodendroglioma?

A

Chicken-wire capillaries

190
Q

What is the gross structure of a pilocytic astrocytoma?

A

It is cystic + solid

191
Q

What is the classic histologic finding for Medulloblastomas?

A

Homer-Wright Rosettes (small round blue cells, vs. ependymoma)

192
Q

What is the classic histologic finding for ependymomas?

A

Perivascular rosetttes

193
Q

What is the “false localization sign”

A

Uncal herniation compresses contralateral crus cerebri leading to ipsilateral paralysis

194
Q

What are the ultimate symptoms of Metachromatic Leukodystrophy?

A

Ataxia and Dementia

195
Q

What are prophylactic strategies for migranes?

A

Propranolol, Topiramate, CCB, Amitryptyline

196
Q

What differentiates negative reinforcement from punishment?

A

Negative Reinforcement - Behavior produces removal of aversive stimulus
Punishment - Unwanted behavior produces aversive stimulus to extinguish it

197
Q

What differentiates sublimation from reaction formation?

A

Reaction formation is unconsciously derived

198
Q

In cases of physical child abuse, who is the most frequent abuser?

A

Biological Mother

199
Q

Are the tics in Tourette syndrome rhythmic?

A

No

200
Q

Is the EEG abnormal in Delerium?

A

Yes

201
Q

What class of drugs can often cause delerium?

A

Anticholinergics

202
Q

What are the reversible causes of dementia?

A

NPH, B12 deficiency, hypothyroidism, neurosyphilis, HIV

203
Q

What are two situations where tactile halluciations are common?

A

1) EtOH withdrawal

2) Cocaine abuse

204
Q

What is bipolar I disorder?

A

At least 1 manic episode with or without a hypomanic or depressive episode

205
Q

What is bipolar II disorder?

A

Defined by the presence of a hypomanic and a depressive episode

206
Q

What are the sleep changes in depression?

A

Increased total REM
Decreased slow-ave sleep
Early morning awakening

207
Q

Can hallucinations be part of normal bereavement?

A

Yes, simple hallucinations can

208
Q

What is required in addition to a panic attack to diagnose panic disorder?

A

Presistent concern about an attack, worrying about consequences, or changes in behavior related to attack

209
Q

In specific phobia, does the person recognize that the fear is excessive?

A

Yes

210
Q

How can you remember the three clusters of personality disorders?

A

Weird, Wild, and Worried

211
Q

How do you differentiate dellusional disorder and paranoid personality disorder?

A

In paranoid, all of the interactions of the person are permeated by the paranoia

212
Q

What disorder is lanugo (fine body hair) associated with?

A

Anorexia nervosa

213
Q

What phase of sleep do terrors occur in?

A

Slow-wave sleep so no memories of arousal (vs. REM)

214
Q

What causes narcolepsy?

A

Decreased orexin production in lateral hypothalamus

215
Q

What is used to treat narcolepsy at night?

A

sodium oxybate (GHB)

216
Q

What are the stages of overcoming substance addiction?

A
  1. Pre-contemplation
  2. Contemplation
  3. Preparation/Determination
  4. Action/willpower
  5. Maintenance
  6. Relapse
217
Q

What are serum markers of EtOH abuse?

A

GGT and AST>ALT

218
Q

What is buprenorphine?

A

It is a partail opioid agonist used to help treat addiction. It has less abuse potential than methadone. Mixed with naloxone in pill to prevent IV administration

219
Q

What is the treatment of cocaine overdose?

A

Benzodiazepines

220
Q

What are some key features of PCP intoxication?

A

Vertical and Horizontal Nystagmus, psychosis, delerium, homicidality. “Acute Brain Syndrome” of disorientation and poor judgement

221
Q

What can treat PCP intoxication?

A

Benzodiazepines and rapid-acting antipsychotics

222
Q

What is the order of symptom progression in delerium tremens?

A

ANS -> psychotic symptoms -> confusion

223
Q

What is the most frequent site of urinary obstruction in a fetus?

A

The Ureteropelvic junction (last to canalize)

224
Q

What causes multicystic dysplastic kidney?

A

Abnormal interaction between ureteric bud and metanephric mesenchyme

225
Q

What structures does the ureter pass under?

A

The uterine artery and the vas deferens

226
Q

Does the ureter pass under or over the gonadal vessels?

A

Passes under them ontop of the psoas

227
Q

Does the ureter run over or under the common illiac?

A

Over

228
Q

What percentage of extracellular fluid is plasma volume?

A

1/4 of it

229
Q

What compound can be used to measure the extra-cellular volume?

A

Inulin

230
Q

What is a normal GFR?

A

100 mL/min

231
Q

Does creatinine clearance over or under estimate GFR?

A

Overestimates it b/c of moderate secretion

232
Q

Does the ERPF underestimate the true renal plasma flow?

A

Yes, by about 10%

233
Q

What happens to the clerance of a secreted substance as the plasma [ ] continues to increase?

A

It approaches that of inulin b/c the transporters become saturated

234
Q

Do NSAIDs cause a decrease in FF?

A

No b/c the prostaglandins normally increase both RPF and GFR

235
Q

Is glucosuria and aminoaciduria normal in pregnancy?

A

Yes

236
Q

What happens to renal arterioles when MAP increases?

A

Macula dense constricts afferent and decreases renin dilates efferent thus the GFR and FF are maintained

237
Q

What drives Mg and Ca reabsorption in Thick ascending limb?

A

Lumen + due to K backleak

238
Q

Which nephron segment generates and excretes NH3?

A

PCT

239
Q

What acid base abnormality does Bartter and Gitelman syndrome result in?

A

Hypokalemia and metabolic alkalosis

240
Q

What is the inheritance pattern of Liddle syndrome?

A

AD

241
Q

Does Cl get absorbed at the same rate as Na early in the proximal tubule?

A

No

242
Q

Is there a reflex bradycardia with AT II pressor effect?

A

No, it limits this by affecting barroreceptor function

243
Q

What drugs shift K out of cells?

A

Digoxin and B-adrenergic antagonists

244
Q

what can happen with hypomagenesemia?

A

Tetany, Torsades

245
Q

What is Winter’s formula?

A

PCo2 = 1.5 [Bicarb] + 8 +/- 2 (Resp compensation for met acidosis)

246
Q

What are the causes of normal gap acidosis?

A 
HARD-ASS
Hyperalimentation
Addison disease
RTA
Diarrhea
Acetazolamide
Spironolactone
Saline infusion
247
Q

Other than diabetic glomerulonephropathy, what glomerular disease can have PAS+ staining?

A

Membranous nephropathy

248
Q

What autoantibody is associated with membranous nephropathy?

A

Anti-PLA2 receptor

249
Q

What is tubular proteinuria?

A

LMW proteins (B2, Ig light chain) in urine that appear when PCT function is disrupted

250
Q

What is the most common cause of death in SLE?

A

Diffuse proliferative glomerulonephritis (“Wire looping” of capillaries)

251
Q

What type of stones precipitate at increased pH?

A

Ca Phosphate, Struvite

252
Q

What type of stones precipitate at decreased pH?

A

Ca Oxalate, Uric Acid, Cystine

253
Q

What are some urease positive bugs?

A

Proteus, Staphylococcus, Klebsiella

254
Q

What type of tumor is described as eosinophilic cells with lots of mito and no perinuclear clearing?

A

Renal oncocytoma - benign

255
Q

What are risk factors for SCC of the bladder?

A

Schistosoma haematobium, chronic cystitis, smoking, chronic nephrolithiasis

256
Q

What bug is associated with UTI in sexually active young women?

A

E. Coli > S. Saprophyticus

257
Q

What is the likely etiology of diffuse cortical necrosis?

A

DIC + Vasospasm

258
Q

What is the goal of a “renal failure diet”?

A

Control nitrogen production (E.g. gluconeogenesis prevention) and phosphate balance

259
Q

What is medullary cystic disease?

A

Inherited disease causing tubulointerstitial fibrosis and shrunken kidneys

260
Q

Why do complex cysts require removal?

A

Risk of RCC development

261
Q

What is a frequent iatrogenic cause of metabolic alkalosis?

A

Loop or thiazide diuretics

262
Q

Which type of diuretic can cause hyponatremia?

A

Thiazides

263
Q

Which diuretic is contraindicated in renal failure?

A

Spironolactone

264
Q

What causes paradoxical aciduria in diuretic use?

A

In a low K state, H is exchanged for Na in cortical collecting tubule

265
Q

What is bradykinins vasoactive effect?

A

Vasodilator, hence risk of angioedema on ACE inhibitors

266
Q

What drug should be avoided in bilateral renal artery stenosis?

A

ACE inhibitors b/c these patients are depending on AT II to maintain GFR

267
Q

Which type of beta blockers increase the risk for hyperkalemia?

A

Non-selective beta blockers (recall, shifts K out of cells)

268
Q

Where is airway resistance the lowest?

A

At the small airways/bronchioles due to large number in parallel

269
Q

Where dose airway smooth muscle and cili extend to?

A

To the terminal bronchioles

270
Q

What type of epithelial cell is found in the respiratory bronchioles?

A

Cuboidal cells

271
Q

Where do aspirated foreign bodies lodge when supine?

A

Superior portion of right inferior lobe

272
Q

What is the relation of the pulmonary artery to the bronchus?

A

RALS - Right Anterior Left Superior

273
Q

What is the largest contributor to functional deadspace?

A

Apex of a healthy lung

274
Q

What is alveolar ventilation?

A

Minute Ventilation - Dead Space Ventilation

275
Q

What two drugs are given in cyanide toxicity?

A

Nitrites are used first followed by thiosulfate

276
Q

Why might newborns develop methemoglobinemia?

A

Due to NO treatment to treat pulmonary HTN

277
Q

What direction does CO shift the Hemoglobin curve?

A

Left shift (hence bright red appearance)

278
Q

What is the typical O2 binding capacity of blood?

A

20.1 mL O2/dL

279
Q

How do emphysema and fibrosis affect diffusion capacity?

A

Emphysema - decreased SA > decreased DLCO

Fibrosis - Increased Thickness > decreased DLCO

280
Q

What happens to the apical V/Q in exercise?

A

It approaches 1 due to capillary dilation

281
Q

Does 100% O2 improve PO2 in a shunt or dead space?

A

In dead space only

282
Q

What is the Haldane Effect?

A

Dissociation of H+ from Hb in lungs promotes CO2 formation and Hb loading. Opposite of Bohr effect

283
Q

What changes occur to blood gas during exercise?

A

None! There is an increase in venous CO2 content and decrease in venous O2 content though

284
Q

What is the most common causes of sinusitis?

A

Viral URI and superimposed infection with S. Pneumo, H. Flu, and M. Catarrhalis

285
Q

What is Homan’s sign?

A

Dorsiflexion of the foot leading to calf pain, sign of DVT

286
Q

What is the classic triad of fat embolus?

A

Hypoxemia, Neurological Abormalities, Petechial rash

287
Q

What distinguishes pre and post-mortem thrombi?

A

Lines of Zahn. Interdigitated fibrin/platelets and RBCs. Only pre-mortem thrombi

288
Q

What leads to cor pulmonale in COPD?

A

Chronic hypoxic vasoconstriction

289
Q

What is the diagnostic criteria for chronic bronchitis?

A

Productive cough > 3 months per year for > 2 years

290
Q

What type of reaction is hypersensitivity pneumonitis?

A

Mixed Type III/IV. Think farmers and birds

291
Q

What is Caplan’s Syndrome?

A

Rheumatoid arthritis, pneumoconioses, intrapulmonary nodules

292
Q

Histologically what does berylliosis resemble?

A

Sarcoidosis

293
Q

What is the breathing rate patterns for restrictive and obstructive disease?

A

Restrictive - high rate low volume (minimizes work)

Obstructive - low rate high volume (minimizes work)

294
Q

What differentiates OSA and Obesity hypoventilation syndrome?

A

In OSA, blood gas is normal during the day, but not in OHS

295
Q

What is the only situation with increased fremitus?

A

Consolidation (lobar pneumonia or pulmonary edema). NOT pleural effusion (which moves lung away from chest wall)

296
Q

What is the most common type of cancer in the lung?

A

Metastases (breast, colon, prostate, bladder)

297
Q

What lung cancer is associated with hypercalcemia?

A

SCC

298
Q

What oncogene is amplified in small cell lung cancer?

A

L-myc

299
Q

What is the cell of origin of small cell lung cancer?

A

Kulchitsky cells

300
Q

What do bronchial carcinoid tumors stain + for?

A

Chromogranin

301
Q

What structures are seen in mesothelioma histology?

A

Psammoma bodies

302
Q

Which type of pneumonia has a patchy distribution and involves bronchioles and adjacent alveoli?

A

Bronchopneumonia

USMLE Step 1 (4 decks)

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