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LCK STEP > Week 4 UWORLD Qs > Flashcards

Week 4 UWORLD Qs Flashcards

1
Q 
Adenomyosis
What is it
Presentation
Describe uterus on physical exam
Histo
A

Ectopic endometrium in the myometrium
Heavy periods b/c increase endometrial surface
Dysmenorrhea (pain) b/c endometrial tissue growth in the confined myometrial space
Uniformly enlarged uterus
Require biopsy: normal endo tissue

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2
Q

What drugs do you use to help opthalmopathy in Graves

A

Glucocorticoids since the eye disease is driven by lymphocyte infiltration - Th1 cells stimulate the fibroblasts to make GAGs increasing volume of retro-orbital tissues
Myositis of extra-occular muscles
Makes sense since anti-T drugs don’t directly help this symptom

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3
Q

Which diabetes drugs do you need to check kidney vs liver fxn for

A

Liver fxn = giltazones (bind PPAR gamma, also weight gain, edema HF risks)
Kidney fxn = SGLT2 Is (prox tubule glucose reabsorption) = canagliflozin, dapagliflozin
Increase glucose out in urine
Other SE: UTI, hypotension (osmotic diuresis)

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4
Q

Name what each nerve innervates

  1. Pudendal S2-4
  2. Lateral femoral cutaneous L2-3
  3. Inf gluteal L5-S2
  4. Genitofemoral L1-L2
  5. Obturator L3-4
  6. Illiohypogastric T12-L1
A
  1. Sensory to perineum, motor to urethral + anal sphincters (one you nerve block to suture perineal lacerations during childbirth, runs under sacrospinal ligament)
    If you go too medial to your landmark (ischial spine) may hit the int pud art + int gluteal art running w/ -> hematoma
  2. Sensory ant + lat thigh
  3. Motor glut max
  4. Sensory scrotum/labia majora, medial thigh
  5. Sensory: medial thigh, motor: adduction
  6. Sensory: suprapubic
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5
Q

Name the inheritance, defect, presentation, treatment MSUD

A

AR - can’t break down branched chain AAs b/c no BC alpha ketoacid deH complex (BCKDC)
Leucine, isoL (sweet smell), valine - restrict in diet
Accum in blood -> neurotox = how infant presents: seizure, stopped feeding, etc
Try to supplement the 5 cofactors of the enzyme: Tender Loving Care For Nancy
Thiamine
Lipoate
Coenzyme A
FAD
NAD

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6
Q

Why does a pt with pit hemorrhage die? What other disease could this be mistaken for

A

No ACTH -> CV collapse
Treat immediately w/ surg + glucocorticoids
Don’t confuse w/ rupture of saccular aneurysm at optic chiasm w/o specific CV collage or chronic history indicating hemm 2ary to pit adenoma

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7
Q

Why are CF pts sterile?

A

Congenital absence of vas deferens

Vs Kartagener syndrome = immotile sperm (similar presentation w/ recurrent pulm inf + digital clubbing)

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8
Q

What is the difference in mechanism between methimazole and propylthiouracil?

A

Both are thionamides aka block peroxidate rxns in thyroid colloid
PTU also X conversion of T4 -> T3 in the periphery

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9
Q

How does congenital rubella present? What type of vaccine is the MMR?

A

White pupils - congenital cataracts
Sensory neural hearing loss
PDA - continuous machine murmur over L infraclav
Live atten vaccine - think about it, you’ve never gotten an MMR booster so much do a good job by itself

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10
Q

Describe 4 major complications/SE if uterine leiomyomas

A

Aka FIBROIDs - think about how their size has local impact
1. Heavy/prolonged periods
2. Fertility challenges
3. Obstructs behind - constipation
4. Obstructs anteriorly - urine retention
Vs advanced cervical cancer - poop problems but also cervical bleeding
Vs rectrocele - poop problems w/o uterine enlargement

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11
Q

Walk through diagnostic steps if ACTH levels are inappropriately normal/elevated (not suppressed)

A

Do high dose dex (= potent glucocorticoid) suppression test
HIGH dose suppresses, LOW dose dose not - pit adenoma
Unchanged - ectopic ACTH prod
Both present w/ high cortisol b/c high ACTH

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12
Q

Where is C peptide separated from insulin?

A

Pre-pro-insulin - made on the RER
Proinsulin moves through RER -> golgi
In secretory granules, C cleaved from insulin - both excreted in granules - why you can detect C peptide in the blood stream

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13
Q

Which thyroid drugs work to stop

  1. I uptake
  2. Peroxidase rxns in the colloid
  3. Secretion T4 > T3
  4. Conversion/use at periphery
A
  1. Percholate/pertechnetate, K iodide - competitively block Na/I co-transporter (that is working against normal [I] gradient)
  2. Propylthiouracil/methimazole
  3. K iodide
  4. Propylthiouracil, BBers, ipodate
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14
Q

In males, what are the -FB molecules for FSH + LH

A

FSH -> Sertoli -> inhibin B @ ant pit

LH -> Leydig -> T @ ant pit + hypothal

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15
Q

Describe the inherited hyper-lips-proteinemia that has defective ApoE

A

AR, T3 = familial dysbetalipoproteinemia
B/c Apo E3/4 normally found on chylo + VLDL –> elevated VLDL, chylomicrons, remnants
Liver can’t removed these w/o Apo E3/4 = high cholesterol + Tgs
Premature atherosclerosis - palmar xanthomas

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16
Q

Describe type 1, 2a, 4 inherited hyperlipoproteinemias

A 
1 = familial chylomicronemia = defective lipoprotein lipase, ApoC 2 -> high chylomicrons = acute pancreatitis, lipid in retina, xanthomas
2a = familial hypercholesterolemia = defective LDL R, Apo 100 -> high LDL = premature atherosclerosis w/ xanthomas
4 = familial hyper TGs = multiple proteins problematic = high VLDL - coronary disease, pancreatitis, diabetes
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17
Q

What is the difference between iodo-tyrosine deiodinase and iodo-thyronine deiodinase?

A

Iodo-tyrosine - removes I from MIT + DIT for recycling as T3/4 are secreted from the thyroid
Iodo-thyronine - @ periphery, remove I from T4 -> T3 = active

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18
Q

Describe who glucagon works

A

Mainly @ liver
Gs -> increase cAMP -> PKA
1. Activate glycogen phosphorylase (glycogenolysis)
2. Activate rate limiting gluconeogenesis enzymes
3. Increase F 2,6 bisphos - inhibit glycolysis
Sustained low BG -> the renal cortex becomes most impt glucose source (why E is more impt for renal gluconeo)

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19
Q

Mechanism of glitazones

A

Upreg PPAR gamma = TF that up regulates the genes for:
1. GLUT4 (induced by insulin @ peripheral tissues)
2. Adiponectin = cytokine from fat to stim FA ox
Fibrates activate PPARg to lower TGs

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20
Q

Describe 3 defective enzymes and presentations of cong adrenal hyperplasia

A

X 17 alpha - can’t go across - all aldo, no cortisol or sex - F normal genitals, M under developed, no puberty, develop aldo excess (HTN + hypoK)
X 11 B - can’t go down (further) - no cortisol, but weak aldo, more sex - no salt wasting, high ACTH b/c no -FB causes more androgen syn - why girls have ambig genitals
X 21 - can’t go down - no aldo, cort, excess androgens - salt wasting at birth, F w/ ambiguous, M w/ precocious pub

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21
Q

Sucrose is broken down into fructose - name the 2 enzymes needed for fructose breakdown and the diseases associated

A

Detect fructose w/ Cu reduction test (not urine dipstick)
Fructose -> F 1 P (fructokinase)
1. Essential fructosuria - no symptoms, benign but positive lab results
F 1 P -> DHAP or glyceraldehyde (aldolase B)
1. Hereditary fructose intol
HypoG + vomit after eat fructose
Fail 2 thrive, liver + renal fail

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22
Q

Presentation of glucagonoma (tumor of alpha cells)

A

Diabetes like… duh hyperG
Necrolytic migratory erythema - plaques the come together w/ bronze central area blistering + scaling
+/- normocytic normochromatic anemia

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23
Q

What is the difference between gestational HTN vs pre-eclampsia vs eclampsia

A

Gest - new onset BP > 20 (aka new HTN not pre-existing)
Pre - + proteinuria/end organ damage
Eclamp - + seizures

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24
Q

Fxns of the SER + tissues with a lot of them

A

Detox @ hepatocytes
Steroid syn @ adrenals, liver, gonads (ovaries + testes)
Specialized SER @ skeletal muscle

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25
Q

Describe the B cell leukemia that will give you a dry tap (and thus splenomegaly)

A

HAIRY CELL LEUKEMIA
B cells with cytoplasmic projections (“hairy”)
Splenomeg b/c accumulating in the red pulp
TRAP stain
Treat cladribine (2 CDA) = adenosine deaminase I

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26
Q

Describe AML
Histo
RFs
Presentation

A

+ MPO, Auer rods (azurophilic rod like granules)
RF: h/o alkylating chemo, myeloprolif disorders, DOWNS
DIC common presentation

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27
Q

What diseases causes joint laxity, hyper-extensible skin, tissue fragility and aortic aneurysm/organ rupture?

A 
EHLERS DANLOS
Skin symptoms (T4 collagen) vascular symptoms (T3 collagen)
Procollagen peptidase def - after the pro collagen triple helix is exocytosed, you can't remove the C + N term to form tropocollagen 
Net collagen doesn't cross link
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28
Q

Presentation and cause of scurvy

A

Vit C def (malnutrition)
Can’t hydroxylate lysine and proline in the RER (step 2 of synthesis) b/c it is cofactor for hydroxylase enzymes
Bleeding gums, bruising, heme-arthrosis
Redness around hair follicle (perifollicular hemm) + corkscrew hairs

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29
Q

What is paraneoplastic cerebellar degeneration

A

Immune response to tumor elsewhere cross reacts with Purkinje neurons (AI) -> degen cerebellum
Anti Yo, P/Q, Hu Abs
Progressive dizziness, limb = truncal ataxia, dysarthria, visual disturbances

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30
Q

Describe the effects of atenolol: where does it work and what does it do to cAMP levels at these sites

A

B1 selective - qiSs - Gs so normally on AC -> increase cAMP, BBer will decrease cAMP
Heart: heart fire hydrant
JGA: girl w/ yellow umbrella
No B1 on VSM: no one with dilated sleeves
VS propranolol - nonselective B1 + 2, decrease cAMP at VSM, JGA and heart
A-BEAM are B1 = atenolol, betaxolol, esmolol, acebutolol, metoprolol

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31
Q

What metalloprotease cleaves natriuretic peptides, glucagon, oxytocin, bradykinin

A

Neprilysin

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32
Q 
Describe the sensory and motor regions of 
L2
L3
L4
L5
S1
A

L2: upper ant-med thigh (think follow below inguinal lig), iliopsoas (hip flex)
L3: below that same wrap touches medial knee, iliopsoas, hip adduction, quads (knee extension)
L4: wrap middle thigh, anterior knee, medial calf + ankle/foot, patellar reflex hip add, quads
L5: outer butt, lateral leg, middle shin, middle top foot, tib ant (dorsiflex + invert), peroneus (foot evert), ext dig braves (toe extension)
S1: butt, back lateral leg, lateral foot, achilles reflex grastrox (plantar flex) hamstrings (knee flex) glut max (hip extend)

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33
Q

What is piriformis syndrome

A

Origin: anterior aspect sacrum
Insert: greater trochanter femur
Occupies most greater sciatic foramen
Above performs: sup gluteal vessels + nerve
Below: inf gluteal vessels, internal pudendal vessels, nerves including sciatic
IF you hypertrophy or injury piriformis, compression of sciatic -> sciatic like symptoms
Vs obturator internus - similar fxn but from the obturator membrane, ischium and pubic rim through the less sciatic foramen to the greater trochanter

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34
Q

Make the jump between prolactimona and bone loss

A

Increase PRL -> suppress GnRN -> less LH/FSH
Ovarian atrophy -> less E -> bone loss
1. Increased RANKL -> more osteoclast

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35
Q

What disease would present w/ RBC lacking CD55?

A

PNH
GPI = protein that connect membrane to DAF (CD55) + MIRL = factors that inactivate complement
W/o this, RBC subject to complement destruction -> hemolysis -> Hgbemia -> Hgburia -> hemosiderosis in kidney
Thrombosis if platelets also attacked by complement
Increase risk AML

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36
Q

If you’re going to give an alcoholic sugar (dextrose IV) what must you add first and why?

A

B1 - thiamine - likely deficient and required for glucose metabolism
W/o you risk
1. Sudden drop in whatever thiamine there is -> Wernicke encephalopathy = ataxia, nystagmus, opthalmoplegia, anterograde amnesia
2. Can progress to Korsakoff psychosis
Think about maxillary body necrosis in this setting when lose control of emotion and memory

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37
Q

Drugs for acute treatment gout attack

A
  1. NSAIDS

2. Colchicine if CI to NSAIDs

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38
Q

What vitamin do you supplement measles treatment with

A

Vit A since measles (paramyxovirus) depletes stores

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39
Q

Treat psoriasis

A

Vit D analogs: calcipotriene, calcitriol, tacalcitol
Bind R -> act as TF
Decrease keratinocyte prolif
Stim keratinocyte differentiation

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40
Q

What drug do you use for complex and simple partial seizures that decreases NA current in cortical neurons

A 
Complex = lose memory, simple = not, partial = one area body
Carbamazepine - also for tonic clonic 
Stabilize Na channels in inactive state 
SE
1. Bone marrow suppression
2. Hepatotoxic
3. SIADH
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41
Q

Describe internal capsule stroke

A

Pure motor weakness of CL arm, leg, lower face

W/ clasp knife spasticity (UMN lesion), + Babinski, hyperreflex, and increase muscle tone

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42
Q

Describe Vit E def

A

Neurodysfxn due to free radical damage of cell membranes
Mimics Friedrich:
Ataxia - degen spino-cerebellar
Lose position/vibration - dorsal columns
Lose DTR - peripheral nerve degen

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43
Q 
What is:
Acanthosis
Hyperkeratosis
Spongiosis 
Dyskeratosis
Hypergranulosis
A

Basalis -> spinosum -> granulosum -> lucidum -> corneum
Acan = thickening of stratum spinosum
Hyperkeratosis = thickening of stratum corneum
Spongiosis = accum of edema in intercell spaces of epidermis, intercell bridges more visible in edematous cells, “spongey” epidermis, may/may not form vesicles
Dyskeratosis = abnormal, premature keratinization of individual keratinocytes below granulosum, genetic or disease (SCC)
Hypergran = increased granulosum (lichen planus)

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44
Q

Name the 3 GP 2b 3a and 3 ADP-R inhibitors

A 
ADP binds R on platelets which up regulates GP2b3a Rs that combine platelets via fibrinogen (converted by coag cascade)
1. ADP R Is 
Clopidogrel - effective ASA alt to prevent CV events 
Prasugrel
Ticlopidine 
GP2b3a Is = no platelet aggregation: 
Abciximab 
Eptifibatide
Tirofiban
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45
Q

DMD mutation and inheritance and histo

A

Delete dystrophin gene - X chromosome
Frameshift mutation b/c not a multiple of 3 -> mutated protein
Dystrophin = pt cytoskeleton of muscle fibers
Histo = muscle necrosis - later gets replaced w/ fat + CT

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46
Q

Describe AVN causes + presentation

A

Pain at rest + gets worse with use - not like any other bone thing you know
Causes:
1. **Glucocorticoid use
2. Sickle cell or the Bends - cause occlusion
3. Alcoholism
4. Vasculitis

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47
Q

What happens in skeletal muscle when ATP binds

A

Myosin head detaches from actin filament - bind ATP “resets” the system
Cleave the ATP to form cross bridge

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48
Q

Vit E def presentation

A

Vit E normally protects cell membranes from ox damage
1. Hemolytic anemia
2. Neuromuscular disease: muscle pain and maybe:
Dorsal column - lose proprioception + vibration
Spinocerebellar - ataxia and hyporeflexia (peripheral nerve dysfxn)

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49
Q

Calculate the [serum] of a drug 8 hours after admin if t/12 = 4, 490mg was started w/ 70L vol dist

A

490/70 = 7mg/L = drug concentration
4 hrs later - 3.5
8 hrs later - 1.75 mg/L = answer

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50
Q

Presentation of acute RF

A

JONES criteria
Joints: polyarthritis
Heart: mitral regurg murmur
Nodules: subcutaneous
Erythema marginatum = red, circular lesions w/ central clearing
Sydenham corhea - also changes in voice
M protein molecular mimicry attacks the heart AND neuronal proteins

51
Q

Describe muscles that V3 innervates, where out of skull, and how injury would present

A 
Muscles of mastication: master, medial pterygoid, temporalis (that close jaw) + lateral pterygoid (opens jaw)
V1 ophthalmic - superior orbital fissue
V2 maxillary - foramen rotudum 
V3 mandibular - foramen ovale 
Injury = jaw towards affected side
52
Q

Weirdest nerve that innervates external ear

A

Vagus - auricular branch

@ post part ext auditory canal - aka inner part of out ear on your head

53
Q

Symptoms + enzymes involved in lead poisoning

A

Inhibit ALA - aminolevulinic acid deH in heme synthesis
Neuro: cog impaired, behavioral problems
GI: constipation, ab pain
Renal: interstitial nephritis, elevated urine delta aminolevulinic acid
Anemia (pale)

54
Q

2 mechanism of digitialis

A
  1. Block Na/K ATPase - increases intracell Na + Ca -> more exctiation contraction coupling -> increase contractility
  2. Enhancing vagal tone to decrease HR
55
Q

SE theophylline

A

Asthma med - decrease cAMP = bronchodilation
Seizures
Arrhythmia
SE impt to note b/c small therapeutic index
If OD - activated charcoal, BBers

56
Q

You can use leucovorine to rescue what medication?

A

MTX not 5 FU

Leucovorin doesn’t require reduction by THF reductase

57
Q

Describe spleen of a CF pt as a kid vs adult

A

Kid: congested

Adult - repeated infarct -> fibrosis + atrophy = fxnal asplenia

58
Q

If you have viral meningitis, what will the pattern be on CSF for glucose, protein, and immune cells present

A

Viral
Normal glucose, increased protein, high % lymphocytes
Vs bacteria - low G, high P, neutrophils

59
Q

Narcolepsy vs hypersolmnolence disorder

A 
Narc = "sleep attacks" direct into REM +/- hallucinations before or after, feel refreshed after (see low hypocretin in CSF b/c lose these neurons in the lat hypothal to mediate wakefulness)
Hypersolm = persistent daytime sleepiness that impairs life w/o known cause, don't feel refreshed after voluntary naps
60
Q

Enzymes involved in nucleotide excision repair

A 
Think DNA damage -> pyrimidine dimers 
*Endonuclease* removes damage
DNA pol make replacement
DNA ligase joins 
Defect - xeroderma pigmentosum 
Vs HNPCC is defective DNA mismatch repair via 3->5' exonucleases (proof reading capabilities of DNA pol)
61
Q

Describe the EKG for hypokalemia

A 
T wave flat
ST depress
U waves
Premature atrial + ventricular contractions -> could cause VTach or VFib
Remember hypoK + hypoMg SEs of amphoB
62
Q

What rash are you thinking of with white/blue/gray specks on a red base on the buccal mucosa?

A

RUBEOLA caused by measles
- enveloped, non-seg RNA (paramyxo)
Triad: conjunctivits + coryza (runny nose) + Koplik spots
Followed by red spotty rash starts on face spreads to trunk/limbs

63
Q

5 symptoms that present for TTP

A 
Fever
Neuro symptoms
Renal fail (HUS)
Anemia w/ schistocytes
TCP
W/ normal PT/PTT unlike DIC
64
Q

What’s going to happen to you if you eat puffer fish w/ tetrodotoxin

A

No Na influx into nerves and cardiac muscle

Death b/c resp fail + hypotension

65
Q

Describe motor innervations of deep vs superficial perineal nerve

A

Deep: ant compartment - dorsiflex, toe extend

Superficial - evert + sensory to lateral leg + foot

66
Q

Malignant hyperthermia - inheritance, pathophys, how treat if this happens

A

AD mutation in ryanodine receptor - screen for this before ORs
Releases large acts Ca after exposure to anesthesia
Stim ATP dep re-uptake by SR -> excessive ATP consumption -> make heat/high temp -> muscle damage
Present: fever + muscle rigidity after general anesthesia
High BP, HR, hyperK
+ Dantrolene = muscle relaxant = X Ca release

67
Q

Xray findings for necrotizing enterocolitis

A

Think babies!
As bowel progress to gangrene -> form intramural gas collections
“Curving lucencies that parallel bowel wall lumen” - makes sense since the gas is in the bowel wall

68
Q

What predicts bad prognosis for post-strep GN?

A

ADULT AGE

69
Q

CN for motor innervation of the tongue

A 
12 = hypoglossus 
10 = vagus only for palatoglossus
70
Q

CN for sensory and TASTE of the tongue

A 
Ant 2/3
Sens = V3
Taste = 7 via chorda tympani
Post 1/3, sen + taste CN 9
Post tongue root = vagus
71
Q

What pts should you avoid using SCh in for induction anesthesia? Which should you use instead

A

Crash or burn injuries
De-innervating injuries/diseases - quadriplegia, Guillain Barre
States up-reg nACH Rs or have increase risk rhabdo
Large of arts K release w/ SCh (this is normal, the amt released is abnormally high in these states)
HyperK -> cardiac arrest
Use curoniums instead b/c non-depol agents

72
Q

What heart defect causes wide, fixed splitting of S2 that does NOT vary w/ respiration

A

ASD

73
Q

What type of healing process are matrix metalloproteinases involved in?

A

Contractures
MMPs degrade collagen + ECM
Increases myofibroblasts to contract wound
Excess MMP leads to excessive contraction
Vs keloid = excessive collagenous scar from fibroblasts

74
Q

What hormone secreted in pregnancy (and some tumors) can cause hyperthyroidism?

A

B hCG - alpha subunit homology w/ TSH, LH, and FSH

75
Q

If a woman is hemorrhaging after delivery, how would you differentiate between uterine atony and placenta accreta?

A

Placenta accreta won’t respond to massage or uterotonic agents - plus you might see that the placenta was removed in pieces instead of all together

76
Q

What changes to coag panel is associated with uremia in RF pts?

A

Toxins lead to qualitative platelets disorder
Aka platelet ct is normal (quantity) plus PT/PTT normal
Bleeding time will be prolonged b/c can’t interact appropriately due to toxins

77
Q

What 2 syndromes can lead to congenital long QT?

A

“Episodes of unprovoked fainting”

  1. Romano Ward = AD, cardiac only
  2. Jervell + Lange Nielsen = AR, w/ sensorineural def
78
Q

What is Hartnup disease?

A 
AR defect in transporters for neutral AA in SI + kidney
Instead of absorbing, lose neural AAs in urine and stool
Esp lose tryptophan = pre-cursor of:
1. V3 - niacin
2. Serotonin
3. Melatonin 
B3 deficiency = no NAD/NADH = pellagra
Diarrhea
Dementia
Dermatitis
79
Q

VHL vs TS

A

VHL = hemagioblastomas @ retina or cerebellum
Also look for cysts/cancer in liver, pancreas, kidney (uni or BILAT RCC which will be lumpy
PHEO
TS = hamartomas = tumor that looks like tissue of origin, in similar places - skin, renal angiomyo, cardiac rhabdo

80
Q

Describe visual field defects with MCA (2) and PCA (1)

A

MCA:
1. Parietal lobe = higher lobe = lower homonymous quadrant-opia
2. Temporal lobe = lower = upper homony quad
PCA: homony hemianopia w/ macular sparing (vs w/o = optic tract before LGN aka MCA)

81
Q

Fxn of TATA box

A

= promotor
Start transcription here via RNA pol 2
TATA not in the mRNA sequence, but must here change how the gene is expressed
Transcription = DNA -> mRNA
Translation = mRNA -> protein, ribosome starts @ start codon

82
Q

When would you chose valproate over ethosuximide for treatment of absence seizures? (Aka what else might be going on?)

A

If absence w/ tonic clonic

83
Q

Describe mechanism, faults, and SE of daptomycin

A

MSSA + MRSA infections
Doesn’t penetrate GN cell wall - can’t use here
NOT FOR PNA b/c inactivated by pulm surfactant
Creates transmemb channels - intracell leaks out
SE: increased CPK + myopathy (watch out using w/ statins) - reg assess for muscle weakness + pain

84
Q

What gene mutation is associated with Crohn’s? How does this make sense with the pathophys

A

NOD2 gene
Activates NKfB (TF to produce cytokines, think IL 2 T cell activation) when epi cells sense LS
Mutate -> decrease NFkB -> impaired immune response
Now back can colonize GI -> exagg adaptive immune response -> chronic GI inflam

85
Q

What are you thinking:

Pt presents with impaired balance, tremor, difficulty speaking + elevated serum transaminases w/o hepatitis

A

WILSON’S DISEASE
AR mutate ATP7B
Can’t get Cu out of hepatocytes -> free rad -> hepatocyte damage (elevated transam)
**Low serum ceruloplasmin + increase Ucu
Deposit of Cu elsewhere in body: eyes, brain

86
Q 
Name symptoms of 
NF 1
MEN 1
MEN 2a
MEN 2b
A

NF 1 = mutate NF 1 TSG, cafe au lait, cutaneous neurofibromas, increased risk CNS tumors
MEN 1 = pancreas, ant pit, paraT (presents gastric ulcers due to ZE or kidney stones b/c high Pca)
MEN 2a = mutate RET, pheo, paraT tumor, medullary thyroid cancer secreting calcitonin
MEN 2b = “ “ muco-cutaneous neurofibroma + marfan-ish

87
Q

Name 3 steps of adenocarcinoma sequence

A

Why colon adenocarcinoma arises from polyps
APC -> early adenoma
KRAS -> late adenoma
p53 + upreg COX (ASA preventative) -> adenocarcinoma

88
Q

What is creatine phosphokinase?

A

CPK = muscle breakdown measure

Don’t confuse for creatinine!!!!

89
Q

What nerve exits through obturator foramen - fxn?

A

Obturator nerve
Thigh adduction
Sensation for distal medial thigh

90
Q

Describe how an AV shunt changes the PV loop

A

Gets fatter + wider:
Increase volume back to heart b/c not going through high R arterioles - increase preload AND rate
TPR reduced b/c taken out high R arterioles - decrease afterload

91
Q

What is Kallman syndrome?

A

X GnRH from hypothal
Both: decreased smell + no body hair
More common in M: small testes
W: 1ary amen + no vary sex characteristics

92
Q

Describe Mullerian aplasia

A

Mullerian ducts for fallopian tubes + uterus + upper vagina
So have:
- Variable uterus + 1ary amen
- No upper vagina
*Normal ovaries - regular 2ary sex characteristics b/c still get E

93
Q

Describe the fxn of TGF beta in the healing process

A

CT synthesis
Remodel ECM
Excess TGF beta: large scars w/ too much fibroblast activity

94
Q

Pompe: mutation, problem, presentation

A

Pompe = T2 glycogen storage disease
Def acid alpha glucosidase - can’t break down glycogen in lysosomes - so accum in lysosomes in liver + muscle
*Cardiomeg, hypotonia, hepatmeg
Normal BG - unique to this glycogen storage disease

95
Q

Describe T1, 3, 5 glycogen storage diseases

A 
T1 = von Gierke (glucose 6 phosphatase) - hepatomeg, lactic acidosis, fasting hypoG, hyperuricemia, hyperlipidemia
T3 = Cori (debranching enzymes) hepatomeg, ketotic hypoG, hypotonia + weakness, abnormal glycogen w/ short outer chains
T5 = McArdle (glycogen phosphorylase) - weakness + fatigue w/ exercise, no rise in blood lactate after exercise
96
Q

What form of insulin do you use IV to treat DKA?

A

Regular

97
Q

Describe the 3 types of moles (nevi)

A
  1. Jxnal nevi - along D/E jxn but only in the epidermis, glat pigment lesions
  2. Compound nevi - extend into dermis - raised on the skin
  3. Intradermal nevi - only dermal component
98
Q

How does ACoA impact the rate of gluconeogen

A

Increase activity of pyruvate carboxylase

It is regulatory - allows more pyruvate for ACoA prod when ACoA levels low prevent cell rom becoming E depleted

99
Q

What does skin dimpling w/ breast cancer indicate?

A

Involvement of suspensory ligaments (Cooper’s)

Vs nipple changes in the lactiferous sinus is involved

100
Q

Why give clavulanate w/ amox?

A

Beta lactamase I - expands amox to cover strains of bacteria that produce beta lactamase

101
Q

Which BB do you use for acute MI in normal pts vs pts w/ pulm history?

A

Non-selective BB in normal pts - propranolol, nadolol

Use B1 selective in pulm pts to avoid bronchospasm - metoprolol

102
Q

Equation for half life

A

t1/2 = (0.7 x Vd) / CL

103
Q

What 2 tumors secrete AFP?

A

Germ cell tumors

HCC

104
Q

What are the effects of isoproterenol?

A

B1 + B2 agonist
Increased heart contractility
Increase TPR

105
Q

What is in the clear cells of clear cell RCC?

A

High glycogen or lipid content

106
Q

What type of transmembrane protein is CFTR?

A

Chr 7
ATP gated - needs ATP b/c pumping Cl against [ ] grad
Creates the extract gradient to pull Na + H2O creating sweat
1. Makes you can’t put NaCl into mucous secretions @ lung + pancreas = too think
2. Means you can’t REABSORB NaCl at sweat = salty sweat

107
Q

Why use CA Is for acute angle closure?

A

Pull off water in the kidney (PCT)
Also CA in the eye - HCO3- in aqueous humor
X CA -> decrease humor formation

108
Q

Which disease presents in kids with vascular deposits of IgA + C3?

A

Henoch Schonelin purpura
HST rxn
Palpable purpura + ab pain + joint pains

109
Q

How does a mitral stenosis murmur change as the disease progresses - aka what change dets severity of the murmur?

A

Time between S2 (specifically A2) + OS
Progression = higher LA P b/c blood not getting into LV
Higher LA P will force mitral valve open EARLIER
Shorter A2-OS interval = worse indication of disease

110
Q

All 3 of these anti-arrhythmics can cause heart block, what are the differences between them: non-dihydro Ca CB, propranolol, lidocaine

A

Non-DHP Ca CB = atria rhythm control, SE constipation
Prop = atria rhythm control but can’t use non-selectives for lung pts b/c worsen bronchoconstriction
Lidocaine = ventricular rhythm control

111
Q

3 steps of cancer progress to mets

A
  1. Downreg E-cad
  2. Adhere BM (increase laminins + other adhesion mol)
  3. Invade BM via metalloproteinases or proteases
112
Q

Mechanism and use of leuprolide

A

GnRH analog
1. Pulsatile -> agonist: infertility
2. Continuous -> antagonist: early puberty
Use: uterine fibroids, endometriosis
Prostate cancer - continuous to decrease synthesis of DHT
**Watch out for transient rise in LH/T at start

113
Q

What is thoracic outlet syndrome

A 
Compress lower trunk brachial plexus
If compress subclavian artery -> pain
If compress subclavian vein -> swelling 
In the scalene triangle: scalenes run from the C spine to the ribs
UE numbness, tingling, weakness 
Causes
1. Extra rib
2. Scalene muscle abnormalities
3. Injury: repeated overhead throwing (baseball)
114
Q

Describe how sepsis + pulm infection can lead to ARDS

A

Increase cytokines responding to infection
Activate pulm epi
Increase recruitment + extravasation of neutrophils into lungs
Inflam -> capillary damage -> leak protein + fluid
Fluid filled lungs = hypoxia + resp failure

115
Q

What are the 3 dopamine agonists you can use for parkinsons - why?

A

Direct D agonists don’t have to be metab - directly active, prolong need to start levodopa

  1. Bromocriptine
  2. Pramipexole
  3. Ropinirole
116
Q

What are the 4 cases of renal papillary necrosis - how does it present?

A 
Acute gross hematuria
Gray white/yellow necrosis of distal 2/3 of renal pyramids -> coag necrosis 
1. Sickle cell
2. Analgesic (NSAIDs)
3. Diabetes
4. Pyelonephritis
117
Q

Hgb + Hct won’t tell you whether pt has absolute or relative high RBCs - what measure do you need for this?

A

RBC mass

Diff bet whether fluid volume in body is diluting/concentrating RBC numbers or if there are actually a ton of RBCs

118
Q

Name the skin lesions of 1ary, 2ary, and 3ary syphilis

A

1ary - painless chancre (ulcer)
2ary - condyloma latum (warty), also look for systemic maculopapular rash (including hands + feet)
3ary - gumma (indurated and ulcered) , also look for aortic tree barking + prostitute pupils

119
Q

What is Fabry disease

A

X-R - lysosomal storage disease
alpha-galactosidase A def - lysosomal accum of ceramide trihexoside
Triad:
1. Peripheral neuropathy - small nerves mean pain/burning and loss hot/cold
2. Angiokeratomas - dark red, non blanching macules in clusters over groin, butt, belly button
3. Decreased sweating = hypohidrosis

120
Q

What molecule is a good marker for GFR vs RPF?

A

GFR: inulin or creatinine, freely filtered and not really secreted or absorbed - you get what you get
RPF: PAH - mostly excreted, so you have to measure how much flows by prox tubule

121
Q

Three scenarios of the same disease - what is the disease”

  1. Mom has increased acne and a deeper voice during preg (increased T) whose baby girl has ambiguous genitalia and clitromegaly
  2. F @ puberty doesn’t get her period, has osteoporosis and is tall
  3. M who is tall and has osteoporosis
A

Aromatase def
Makes sense - low E = delays fusion of epiphyses + increase risk osteoporosis (think meno)
In utero, the excess T will go back into mom’s circulation too

122
Q

What is subclavian steal syndrome?

A

Key point: vertebral art off subclavian art
Somehow stenosis right before the L subclavian only
Means less BF down the aorta
Now blood coming up R vertebral doesn’t go into the brain - cross over via basilar art - down L verebral art -> feeds the downstream aorta
Symptoms due to insufficient BF
Pain in L arm
Dizziness, vertigo, drop attacks

123
Q

How does drug induced SLE present

A

W/o the cutaneous findings
Jt pain, pleuritic chest pain
Anti-histone Abs

LCK STEP (27 decks)

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