STEP1 Flashcards

1
Q

maternal/fetal hep B transmission

A

babies of HBeAg+ mothers have 90% chance of infection
infected babies are mostly asymptomatic b/c immune-tolerant (so immune cell doesn’t hurt infected liver cells), but have 90% chance of progression to chronic hep B; this can be prevented with hep B vaccine

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2
Q

pernicious anemia

A

pernicious anemia (vitamin B12 deficiency) is caused by autoimmune destruction of parietal cells, which live in the superficial gastric glands

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3
Q

hydroceles

A

hydroceles can be communicating, caused by incomplete obliteration of processus vaginalis, and this can also cause an indirect inguinal hernia

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4
Q

lab tests for osteoblasts and clasts

A

Osteoblast activity - bone-specific alkaline phosphatase

Osteoclast activity - 1) urinary deoxypyridinoline, 2) tartrate-resistant acid phosphatase and urinary hydroxyproline

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5
Q

TSS cause

A

TSS is due to toxic shock syndrome toxin from staph aureus. This is a superantigen which activates helper T cells and antigen-presenting cells (macrophages)

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6
Q

microbiology bile

A

Enterococci grow in bile and 6.5% NaCl
Strep viridans is bile-insoluble and pneumoniae is bile-soluble
enterococcal infection mostly after cystoscopy, colonoscopy, or OB procedures

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7
Q

Achalasia

A

Achalasia = motility disorder from decreased inhibitory ganglion cells in esophageal wall => normal contraction of UES but decreased peristalsis in mid esophagus and incomplete relaxation at LES => dysphagia, regurg, and chest pain
Barium swallow => bird’s beak sign

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8
Q

Gout tx

A

Acute: NSAIDS
Chronic: allopurinol (XO inhibitor), probenecit (uricosuric) etc.

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9
Q

how does food => insulin release?

A

glucose enters pancreatic beta cells via GLUT-2, then is metabolized to make ATP which binds K+ channels, causing them to close => membrane depolarization => v-gated Ca++ channel opening => increased intracellular Ca++ => insulin release

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10
Q

Filtration Fraction

A

Filtration Fraction = GFR/RPF (renal plasma flow)
mild efferent constriction => increased GFR and FF
severe efferent constriction => decreased GFR and increased FF
normal GFR = 125

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11
Q

action of fructose 2,6 bisphosphate

A

fructose 2,6-bisphosphate activates PFK-1, driving glycolysis and inhibiting gluconeogenesis

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12
Q

Pentose Phosphate Pathway

A

Pentose Phosphate Pathway makes NADPH (reductive power) and ribose-5-phosphate (nucleotide precursor)

irreversible oxidative steps and reversible nonoxidative steps
both make r-5-p, nonoxidative uses up excess r-5-p but kicks into reverse if need for r-6-p exceeds what oxidative can handle

oxidative makes NADPH and needs g-6-p deydrogenase
nonoxidative only makes r-5-p and need transketolase and transaldolase

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13
Q

Tumor Lysis Syndrome

A

chemo => cell death -> K, phosphorous, and uric acid release

uric acid precipitates in acid environments (eg: distal tubule and collecting ducts

tx: urine alkalinization, hydration, allopurinol

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14
Q

Traveler’s diarrhea cause

A

ETEC (E. coli) which makes heat labile (LT, cholera toxin-like) and heat stable (ST) toxins
LT => increased cAMP
ST => increased cGMP

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15
Q

DiGeorge Syndrome

A

CATCH 22 => no 3rd and 4th pharyngeal pouches => no thymus => no TCs => viral, fungal, and protozoal infections

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16
Q

Chediak-Higashi

A

AR trafficking deficit => no phagolysosones => pyogenic infections, neutropenia, granules in leukocytes, albinism, neuro defects (nystagmus), recurrent pyogenic staph and strep infections

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17
Q

e. Coli virulence factors + effects

A

LPS => bacteremia and septic shock
K1 capsular polysaccharide => neonatal meningitis
verotoxin (shiga-like toxin) => bloody diarrhea
heat-stable + -labile enterotoxins => watery diarrhea
P fimbriae => UTIs

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18
Q

adrenal cortex zones (out to in)

A

zona glomerulosa - mineralocorticoids (aldosterone)
zona fasciculata - glucocorticoids (cortisol)
zona reticularis - androgens

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19
Q

Cushing syndrome v. disease

A

Cushing syndrome = glucocorticoid excess
Cushing disease = Cushing syndrome caused by an ACTH-secreting pituitary adenoma, which => hyperplasia of zona fasciculata and reticularis

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20
Q

Post-operative urinary retention + tx

A

from decreased contractility of bladder detrusor muscle

treatment = Bethanechol (muscarinic agonist)

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21
Q

Incidence

A

incidence = new cases / population at risk

need to subtract out those already infected

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22
Q

Maple Syrup Urine Disease

A

failure to break down BRANCH(maple tree)ed- chain amino acids Leu, Ile, and Val 2/2 a mutation in BCKDComplex - may improve w/ high-dose thiamine treatment as this is a coenzyme required by BCKD

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23
Q

Bipolar treatment

A

lithium, valproate, quetiapine

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24
Q

hyperacusis

A

increased sound sensitivity, comes from stapedius muscle paralysis due to facial nerve injury

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25
Q

effects of AV shunt from tissue injury

A

AV shunt (fistula) => decreased resistance => increased preload (blood flow to heart) and decreased afterload (blood pressure) => increased peak volume (end-diastolic volume) and decreased peak pressure (afterload)

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26
Q

RBC storage

A

Whole and packed RBCs usually stored with citrate anticoagulant. this can chelate calcium and magnesium, which becomes a problem (hypocalcemia or hypomagnesemia) after 5-6 liters. RBCs can also lose intracellular potassium, which can cause hyperkalemia

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27
Q

1st and 2nd pharyngeal arch derivatives

A

1st pharyngeal arch: trigeminal n, mandible, maxilla, zygoma, incus, malleus
2nd pharyngeal arch: facian n, stpaes, styloid, lesser horn of hyoid

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28
Q

BC cytokines

A

CD19,20,21

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29
Q

TC cytokines

A

CD4(helper) or CD8 (cytotoxic)

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30
Q

Who expresses CD 15?

A

granulocytes and Reed-Sternberg cells (hodgkin lymphoma) express CD15

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31
Q

Who expresses CD16?

A

NK cells, neurophils, and macrophages express CD16

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32
Q

sepsis cytokines

A

TNF-alpha

IL-1 and IL-6 are involved too

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33
Q

cell that doesn’t use ketones

A

ketones are used by all cells with mitochondria, including brain (though it will be unhappy)
RBCs have no mitochondria

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34
Q

Vincristine MOA

A

inhibits MT formation by binding beta-tubulin - causes cytotoxicity during M phase (cell division) - side effects include neurotoxicity (peripheral neurophathy)

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35
Q

cell cycle phases + chemo agents at each stage

A

G1: prep for DNA synth
G0: resting
S: DNA replication (blocked by etoposide, irinotecan, MTX, 5-fluorouracil)
G2: error correction or apoptosis (bleomycin and doxorubicin act here)
M: cell division (vinca alkaloids and taxanes act here)

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36
Q

cell cycle nonspecific chemo agent

A

cyclophosohamide

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37
Q

effects of increased duodenal acidity on bile composition

A

increased duodenal H+ => secretin release from duodenal S-cells => bicarb secretion from pancreatic cells - K+ stays the same and Cl- decreases

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38
Q

hip muscles and actions

A

Flexion: iliopsoas, rectus femoris, tensor fascia lata
Extension: gluteus maximus, semitendinosus, semimemganosus, biceps femoris (long head)
Abduction: gluteus medius and minimus
Adduction: adductor brevis, longus, and magnus

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39
Q

iliopsoas components + attachments

A

psoas major +psoas minor + iliacus - go from pelvis/spine to femur

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40
Q

primary myelofibrosis pathophys

A

from megakaryocytic hyperplasia => fiberblast proliferation + collagen in bone marrow => pancytopenia over time and eventually => hepatosplemomegaly from compensatory extramedulaary hematopoiesis

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41
Q

primary myelofibrosis lab tests

A

teardrop-shaped and nucleated RBCs on smear

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42
Q

mutations causing myeloproliferative disorders

A

except for CMP, myeloprolifeative disorders (polycythemia vera, essential thrombocytosis, primary myelofibrosis) are caused by JAK2 mutation => increased activation of JAK/STAT pathway

CML is from BCR-ABL (9:22) fusion protein (Philadelphia chromosome)

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43
Q

Role of femoral n.

A

quad motion, patellar reflex, and sensation over anteromedial thigh and leg

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44
Q

collagen types

A

Be (So Totally) Cool, Read Books
I - Bone, Skin, Tendons, ligaments, corena, vessels, scars - mut => osteogenesis imperfecta (most common)
II - Cartilage, vitreous humor, nucleus pulposus
III - Reticulin, vessels, skin, lungs, guts, bone marrow, lymph, granulation tissue - mut => vascular Ehlers-Danlos
IV - Basement membranes - mut => Alport syndome

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45
Q

nec fasc causes in order

A

strep pyogenes (GAS), staph aureus, c. perfringens, polymicrobial

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46
Q

Group A Strep lab tests + virulence factors

A

Strep pyogenes is G+, coag-, catalase-, PYR+, B-hemolytic, and bacitracin-sensitive
virulence factors: hyaluroonidase, M protein, toxins O and S

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47
Q

acoustic neuroma

A

schwann cell tumor from vestibulocochlear nerve (cerebellopontine angle => unilateral sensorineural hearing loss

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48
Q

antiemetic categories and examples

A

antimuscarinis (anticholiergics) - scopolamine
antihistamines - diphehydramine, promethazine, meclizine
Dopa R antagonists - prochlorperazine, metoclopramide
5-HT R antagonists - ondansetron, granisetron
neurokinin 1 R antagonists - aprepitant, fosaprepitant

last 3 rows good for chemo-induces vom

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49
Q

vomiting pathophys

A

vomiting reflex mediated by M1, D2, H1, 5-HT, and NK1 receptors

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50
Q

Hardy-Weinberg

A

p + q = 1
p^2 + 2pq + q^2 = 1
square root of prevalence = mutant allele frequency

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51
Q

eukaryotic TSS

A

CAAT and TATA

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52
Q

Pellagra

A

niacin (B3) deficiency => photosensitive dermatitis, diarrhea, dementia

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53
Q

What enzymes is vit B3 a cofactor for

A

pyruvate dehydrogenase
malate dehydrogenase
isocitrate dehydrogenase
alpha-ketoglutarate dehydrogenase complex

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54
Q

how long after fertilization is hCG positive in serum and urine?

A

8 days; 14 days

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55
Q

elastase

A

elastases are proteases in alveolar fluid produced by alveolar macrophages and by neutrophils; if excess => centriacinal or panacinar emphysema

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56
Q

increased affinity of Hgb for O2

A

Hgb mutations causing increased O2 affinity (left shift) => decreased O2 release in tissues => kidney hypoxia => epo release => erythrocytosis

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57
Q

regulation of glycogenolysis in liver and muscle

A

glycogenolysis in muscle cells increases during contraction b/c increased Ca from contraction => phosphorylase kinase (PK) activation => glycogen phosphorylase activation => glycogenolysis

In liver, PK is activated through epi and glucagon binding to GPCRs=> increased cAMP => PKA activation => PK activation

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58
Q

Diabetes Insipidus

A

From decreased ADH production (central) or resistance (nephrogenic)

If respond to exogenous ADH, it’s central. ADH is made in the paraventricular and supraoptic nuclei of the hypothalamus and stored in the post. pituitary.

Damage to post. pit. only causes temporary DI, while hypothalamus damage is permanent.

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59
Q

Rifaximin

A

Rifaximin is an antibiotic used in hepatic encephalopathy to decrease intestinal production and absorption of ammonia.

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60
Q

Lactulose

A

Used in hepatic encephalopathy to lower colonic pH and thus increase conversion of ammonia to ammonium.

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61
Q

Varenicline

A

Used in smoking cessation. Partial nAchR agonist which reduces withdrawal cravings and reduces the rewarding effects of nicotine.

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62
Q

alpha-1 antitrypsin deficiency

A

alpha-1 antitrypsin deficiency (AD) - made in liver, blocks proteolytic enzymes (like elastase)

deficinecy => increased tissue damage during inflammation => panacinar emphysema (worse with smoking)

also => intrahepatocyte accumulation of misfolded AAT => cirrhosis and HCC - on liver histology with periodic acid-Schiff see reddish-pink intracellular granules.

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63
Q

EHEC

A

Enterohemorrhagic e. Coli => bloody diarrhea - O157:H7 - makes Shiga-like toxin, which inactivate 60s ribosomal subunit

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64
Q

How long does depression have to last to qualify as Major Depressive Disorder?

A

> 2 weeks

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65
Q

RPGN immunofluorescence

A

RPGN => crescent formation (fibrin deposits) - IF shows these deposits

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66
Q

Langerhans giant cells

A

TB => caseating granuloma with Langerhans giant cells (not Langerhans cells) which are macrophages activated by CD4+ Th1 cells. They have multiple nuclei organized peripherally in a horseshoe shape

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67
Q

Stop codons

A

UAA, UAG, UGA

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68
Q

M pneumoniae

A

causes walking pneumonia: low-grade fever, malaise, chronic dry cough, CXR much worse than clinical appearance, need cholesterol to grow b/c only have cholesterol-rich cell membrane

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69
Q

Pharyngeal arch - Aortic arch - CN - key derivatives

A

1-1-Trigeminal (CN V) - maxillary artery
2-2-Facial (CN VII) - stapedial artery (regresses)
3-3- Glossopharyngeal (CN IX) - common carotid and proximal internal carotid arteries
4-4- Superior laryngeal branch of Vagus (CN X) - aortic arch, subclavian arteries
5-5-obliterated-obliterated
6-6-recurrent laryngeal branch of Vagus (CN X) - pulmonary arteries and ductus arteriosus

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70
Q

PSGN microscopy findings

A

2-4 wks post strep infection (skin or pharyngitis) => RBCs, proteinuria on UA

light microscopy: large, hypercellular glomeruli
IF: granualr deposits IgG, IgM, C3 along BM - lumpy bumpy
EM: IC depositions on epithelial side of BM

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71
Q

Digoxin

A

rate control in afib, slows AV node conduction via increased parasympathetic tone 2/2 sensitized arterial baroreceptors and increased efferent ganglionic transmission

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72
Q

statins and bile sequestrants

A
statins inhibit HMG CoA redutase, preventing hepatic cholesterol synth
bile sequestrants (cholestyramine) bind bile acids in GI tract => increased synth of new bile acids, depletion of hepatic cholesterol, and increased HMG CoA reductase => increased hepatic cholesterol synth
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73
Q

ovulation trigger

A

LH surge

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74
Q

gonadotropin feedback in males

A

inhibin B from Sertoli cells inhibits FSH

testosterone from Leydig cells inhibits LH

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75
Q

TB granuloma formation

A

multinucleated giant cells and epitheliod macrophages develop due to Th1 cells release Inf-y, IL-12, and TNF-alpha

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76
Q

true and false diverticula

A

Meckel’s diverticulum: connects ileum to umbilicus; true diverticulum, so contains mucosa, submucosa, and smooth muscle

false diverticula (herniations through muscular layer) only have mucosa and submucosa

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77
Q

DRESS Sy

A

Drug Reaction with Eosinophilia and Systemic Symptoms - from anticonvulsants, allopurinol, sulfa, abx => fever, lymphadenopathy, facial edema, skin rash, hepato, renal, and pulmonary sx

Labs: eosinophilia, lymphocytosis, elevated ALT

Tx: remove drug

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78
Q

catecholamine synth

A

tyrosine -(tyrosine hydroxylase)-> DOPA -(dopa decarboxylase)-> Dopanime -(dopaine beta-hydroxylase)-> Norepinephrine -(PNMT)-> Epinephrine

last step is upregulated by Cortisol

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79
Q

protein A

A

Staph Aureus VF that’s part of cell wall, binds Fc portion of IgG Abs and prevents complement activation => decreased C3b and impaired opsonization and phagocytosis

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80
Q

mannitol toxicity and RFs

A

pulmonary edema - volume redistribution from tissues to plasma => increased hydrostatic pressure => leakage

RFs: CHF, hx of pulmonary edema

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81
Q

renal artery stenosis

A

occurs in elderly 2/2 atherosclerosis or women of childbearing age 2/2 fibromuscular displasia, => asymmetrical kidneys, pain after eating from ischemia, HTN, abdominal bruit

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82
Q

acute interstitial nephritis

A

from beta lactams, NSAIDs, sulfa, rifampin, and diuretics => type IV hypersensitivity (1-3 wks) => increased IgE, eosinophilia, eosinophiluria

Sx: fever, maculopaupular rash, acute renal fail, from renal interstitial edema, leukocyte inflammation

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83
Q

Ductal Carcinoma in Situ

A

pleomorphic cells with prominent central necrosis that don’t penetrate the BM; precursor to invasive ductal carcinoma (breast cancer)

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84
Q

alpha agonists

A

increase systolic and diastolic BP, via peripheral vasoconstriction => reflexive increased vagal tone => decreased HR, slowed AV node conduction

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85
Q

hand bones

A

lunate and scaphoid bones articulate with radius

straight line to pinky, here comes the thumb
scaphoid lunate triquetrum pisiform hamate capitate trapezoid trapezium

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86
Q

altitude

A

altitude => hypoxemia => hyperventilation => respiratory alkalosis (low PaCO2)

after a few days => chronic respiratory alkalosis and compensatory decreased serum bicarb (metabolic acidosis)

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87
Q

DNA binding protein examples

A

TFs (Myc, CREB), steroid Rs (cortisol, aldosterone, pregesterone), thyroid hormone R, fat-soluble vitamin Rs (vit D, retinoic acid), and DNA transcription and replication proteins

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88
Q

CN III palsy

A

ptosis, down and out gaze, fixed dilated pupil, loss of accommodation

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89
Q

strongyloides stercoralis

A

strongyloides stercoralis: roundworm filariform larvae penetrate skin, move to lungs via blood, get coughed up and swallowed, develop in intestine - eggs hatch to rhabditiform larvae which are pooped out

diagnose via rhabditiform larvae in stool

can cause autoinfection cycle => hyperinfection syndrome with multiorgan dysfcn and sepsis

tx = ivermectin

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90
Q

Acromegaly tests

A

Screen by checking for high IGF-1

Confirm with oral glucose tolerance test (hyperglycemia should suppress GH)

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91
Q

Hormones made in ant pit

A
Flat p(I)g
FSH LH ACTH prolactin GH
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92
Q

Prolactinoma tx

A

Cabergaline
Bromocriptine
Don’t need surgery

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93
Q

Nonfunctional pituitary adenoma

A
Go find the adenoma
GH
FSH and LH
TSH
ACTH
Lost in this order
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94
Q

herpetic gingivastomatitis

A

primary HSV-1 Tzank smear with multinucleated giant cells

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95
Q

mycoplasma pneumoniae lab

A

cold agglutination - Abs which are anti-RBC but only when cold, not at physiologic temp (cold agglutinins)

Other things that cause these are EBV and hematologic malignancies

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96
Q

Dengue

A

4 serotypes of Dengue virus - 2nd infection by a different serotypes => Abs worsen infection => Dengue hemorrhagic fever

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97
Q

Legionella RFs and labs

A

travel, nursing homes

hyponatremia, increased neutrophils

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98
Q

sporothrix schenckii

A

subcutateous mycosis from a thorn prick which spreads along lymphatics

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99
Q

congenital rubella

A

cataracts, sensorineural deabness, PDA

prevent by giving mom MMR vaccine weeks before delivery but some time after first few weeks gestation (live attenuated)

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100
Q

Aspergillus complications

A

can cause allergic bronchopulmonary aspergillosis (a type I hypersensitivity reaction) in people with asthma or CF; this leads to eosinophilia

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101
Q

influenza A superinfections

A

staph aureus, strep pneumoniae, haemophilus influenzae

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102
Q

cat scratch fever

A

bartonella hensleae - primary erythema and rash => lymphadenopathy

can also cause bacillary angiomatosis in immunocompromised people (red/purple vascular papules)

and culture-negative endocarditis

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103
Q

Th2 cell role

A

sensitized Th2 cells secrete IL-4 and IL-13, which drive BC class switching to IgE. They also make IL-5 (recruits eosinophils) and IL-10

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104
Q

candida immune response

A

purely TC-mediated (like PPD - type IV hypersensitivity)

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105
Q

opsonins

A

IgG, complement; IgM doesn’t opsonize but rather activates complement which does

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106
Q

Type III hypersensitivity

A

IC deposition which causes vasculitis and can use up complement (causing drop in blood levels)

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107
Q

n. meningitidis VF

A

LOS => shock; blood levels correlate with morbidity and mortality

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108
Q

strawberry tongue

A

scarlet fever

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109
Q

top causes of osteomyelitis

A

staph aureus and GAS, after bacteremia

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110
Q

scabies

A

rapidly spreading rash, more itchy at night, from hands up arms and torso

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111
Q

cryptococcus

A

CNS sx in HIV, non-enhancing lesions, positive latex agglutination test, budding yeast on india ink and light microscopy

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112
Q

obligate intracellular bacteria

A

Rickettsia, Chlamydia, Coxiella (need host ATP)

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113
Q

facultative intracellular bacteria

A

salmonella, neisseria, brucella, mycobacterium, listeria, francisella, legionella, yersinia pestis

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114
Q

parasite cause of biliary tract disease, cholangiocarcinoma

A

clonorchis sinensis

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115
Q

parasite cause of brain cysts, sz

A

taenia solium (neurocysticercosis)

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116
Q

parasite that causes hematuria, bladder SCC

A

schistosoma haematobium

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117
Q

parasite cause of liver cycsts

A

echinococcus granulosus (hydatid cyst)

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118
Q

parasite that causes microcytic anemia

A

ancylostoma, necator

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119
Q

parasite that causes myalgias and periorbital edema

A

trichinella spiralis

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120
Q

perianal pruritus

A

enterobius (tape test)

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121
Q

parasites that cause portal HTN

A

schistosoma mansoni and japonicum

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122
Q

parasite that causes vB12 deficiency

A

diphillobothrium latum (cobalamin)

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123
Q

B vitamin names

A
B1 - Thiamine - The
B2 - Riboflavin - Responsible
B3 - Niacin - Nutritionist
B5 - Pantothenic acid - Puts
B6 - Pyridoxine - Pineapple
B7 - Biotin - Before
B9 - Folate - Fried - least reserves
B12 - Cobalamin - Chicken
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124
Q

DNA virus rules and exceptions

A

all ds except for parvovirus
all replicate in nucleus except for poxvirus
all icosahedral except for poxvirus

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125
Q

RNA virus rules and exceptions

A

all ss except for reoviruses

all replicate in cytoplasm except influenza (orthomyxovirus) and retroviruses

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126
Q

herpesvirus membrane origin

A

nuclear membrane (other enveloped viruses all cytoplasm)

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127
Q

reoviruses and main relevance

A

coltivirus - colorado tick fever

rotavirus - fatal diarrhea in kids

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128
Q

picornaviruses and main relevance

A

PERCH
Poliovirus - salk/sabin vaccines - IPV/OPV
Echovirus - aseptic meningitis
Rhinovirus - common cold
Coxsackievirus - aseptic meningitis, herpangina (mouth blisters, fever), hand/foot/mouth disease, myocarditis and peridarditis
Hepatitis A virus - acute hepatitis, smoking aversion

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129
Q

Hepevirus and main relevance

A

Hepatitis E virus

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130
Q

calicivirus and main relevance

A

norovirus - gastroenteritis

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131
Q

flaviviruses and main relevance

A

HCV, Yellow fever, Dengue, St. Louis encephalitis, West Nile (meningoencephalitis), Zika

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132
Q

togaviruses and main relevance

A

rubella, W and E equine encephalitis, Chikungunya virus

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133
Q

retroviruses and main relevance

A

HTLV - TC leukemia

HIV - AIDS

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134
Q

coronaviruses

A

SARS, MERS, common cold

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135
Q

orthomyxovirus

A

influenza virus

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136
Q

paramyxoviruses and main relevance

A

parainfluenza - croup
RSV - bronchiolitis in babies; tx = ribavirin
Measles (rubeola)
Mumps

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137
Q

rhabdovirus

A

rabies

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138
Q

filoviruses and main relevance

A

Ebola - rapidly fatal hemorrhagic fever

Margurg - same

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139
Q

arenaviruses and main relevance

A

LCMV - lymphocytic choriomeningitis virus

Lassa fever encephalitis - spread by rodents

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140
Q

bunyaviruses and main relevance

A

California encephalitis - arthropod borne
Sandfly/Rift Valley fever - arthropod borne
Crimean-Congo hemorrhagic fever - arthropod borne
Hantavirus - hemorrhagic fever, pneumonia - rodent poop

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141
Q

delta virus and main relevance

A

Hepatitis D virus - needs Hep B to replicate

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142
Q

aspergillus fumigatus manifestations

A

Invasive pulmonary aspergillosis - oportunistic infection in immunosuppressed and neutropenic pts
Aspergilloma - colonizing - fungus ball within preexisting lung cavity
Allergic bronchopulmonary aspergillosis - type I hypersensitivity reaction in pts with asthma or CF

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143
Q

c. diff treatment

A

Oral metronidazole or vancomycin

If recurrent, give fidaxomicin (RNA polymerase inhibitor)

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144
Q

Staph scalded skin syndrome

A

Widespread epidermal sloughing with gentle pressure (NIkolsky’s sign), plus fever, in infants and children; due to exfoliatin (exotoxin which cleaves desmoglein - basically a toxic pemphigus vulgaris) production by staph species; not usually fatal

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145
Q

lactose-fermenting G- rods

A

entereobacter cloacae, e. coli, klebsiella pneumoniae (pseudomonas aeruginosa does not ferment lactose)
all 4 cause UTIs in ppl with catheters

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146
Q

strep gallolyticus (formerly bovis) bacteremia

A

colon cancer eval

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147
Q

HSV-1 encephalitis

A

Most common cause of fatal sporadic encephalitis
Primary oropharyngeal infection -> brain via nose or reactivation of virus from trigeminal ganglion
Causes edema and hemorrhagic necrosis of the temporal lobe (uni- or bilateral)

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148
Q

Diseases associated with HLA B27

A

seronegative arthropathies: psoriatic arthritis, ankylosing spondylitis, IBD-associated arthritis, and reactive arthritis

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149
Q

Diseases associated with HLA DQ2/DQ8

A

celiac disease

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150
Q

Diseases associated with HLA DR2

A

MS, hay fever, SLE, Goodpasture syndrome

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151
Q

Diseases associated with HLA DR3

A

DM1, SLE, Graves, Hashimoto, Addison disease

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152
Q

Diseases associated with HLA DR4

A

RA, DM1, Addison disease

153
Q

cytokines secreted by macrophages

A

IL-1 - fever, inflamm, activates endothelium to express adhesion molecules, induces chemokine secretion to recruit WBCs
IL-6 - fever, acute phase proteins from liver
IL-8 - neutrophil chemotaxis “clean up on aisle 8”
IL-12 - TCs-> Th1 cells, activates NK cells
TNF-a - activates endothelium, causes WVC recruitment and vascular leak; cachexia in ca, granulomas in TB

154
Q

cytokine mediators of sepsis

A

IL-1, IL-6, TNF-a

155
Q

Cytokines secreted by all TCs

A

IL-2 - stimulates Th cells, CD8+ TCs, regulatory TCs, and NK cells
IL-3 - supports growth of BM stem cells; like GM-CSF

156
Q

IL1-6 effects

A
Hot T-bone stEAK
IL-1 - hot - fever
IL-2 - T - stimulates TCs
IL-3 - bone - stimulates BM
IL-4 - E - stimulates IgE
IL-5 - A - stimuates IgA
IL-6 - K - stimulates aKute phase proteins
157
Q

Cytokines secreted by Th1 cells

A

Inf-y - stimulates macrophages to kill phagocytosed pathogens, inhibits Th2 cells
plus:
IL-2 - stimulates Th cells, CD8+ TCs, regulatory TCs, and NK cells
IL-3 - supports growth of BM stem cells; like GM-CSF

158
Q

Cytokines secreted by Th2 cells

A

IL-4 - TC -> Th2 cells, promotes BC growth, IgE->IgG
IL-5 - promotes BC growth and differentiation, IgA, eosinophils
IL-10 - attenuated inflammatory response, inhibits Th1 cells, macrophages, and DCs
plus:
IL-2 - stimulates Th cells, CD8+ TCs, regulatory TCs, and NK cells
IL-3 - supports growth of BM stem cells; like GM-CSF

159
Q

immunomodulatory cytokines

A

TGF-B, IL-10

160
Q

alemtuzumab

A

targets CD52, used for CLL, MS

161
Q

bevacizumab

A

targets VEGF, used for CRC, RCC, non-small cell lung ca (also macular degeneration)

162
Q

cetuximab

A

targets EGFR, used for stage IV CRC, head and neck ca

163
Q

rituximab

A

targets CD20, used for BC non-Hodgkin lymphoma, CLL, rneumatoid arthritis, ITP

164
Q

trastuzumab

A

targets HER2/neu, used for breast and gastric ca

165
Q

adalimumab, certolizumab, golimumab, infliximab

A

targets soluble TNF-a, used for IBD, RA, ankylosing spondylitis, and psoriasis

166
Q

daclizumab

A

targets IL-2 R (CD25), used for relapsing MS

167
Q

eculizumab

A

targets C5, used for paroxysmal nocturnal hemoglobinuria

168
Q

natalizumab

A

targets a4-integrin, used for MS, Crohn

169
Q

ustekinumab

A

targets IL-12 and IL-23, used in psoriasis and psoriatic arthritis

170
Q

abciximab

A

targets plt G IIB/IIIa, used to prevent clots in pts undergoing percutaneous coronary intervention

171
Q

denosumab

A

targets RANKL, used for osteoporosis

172
Q

Digoxin immune Fab

A

targets digoxin, used in digoxin toxicity

173
Q

omalizumab

A

targets IgE, used in asthma

174
Q

palivizumab

A

targets RSV F protein, used for RSV prophylaxis in premies

175
Q

ranibizumab, bevacizumab

A

target VEGF, used in macular degeneration, diabetic retinopathy, and macular edema (also CRC for beva)

176
Q

GTP-making step in CAC

A

made by succinyl-CoA sythetase while turning succinyl-CoA into succinate

177
Q

GTP use in gluconeogenesis

A

used by PEP carboxykinase to make PEP from oxaloacetate

178
Q

urea cycle defects

A

cause hyperammonemia => lethargy, vom, sz, and cerebral edema in infancy;

treat with careful protein restriction matching intake to requirement

also give phenylacetate to increase ammonia excretion

179
Q

alkaptonuria

A

AR deficiency of homogentisic acid oxidase=> buildup of homogentic acid and inability to make fumarate from tyrosine

black urine if left to oxidize, bluish skin, arthropaty

180
Q

riboflavin deficiency

A

B2 - used to make FAD, electron acceptor for succinate dehydrogenase, which convers succinate to fumarate

181
Q

tyramine hypertensive crisis

A

MAO inhibitors (tranylcypromine, phenelzine) prevent metabolism of tyramine (indirect sympathomimetic) and ingestion of dietary tyramine in cheese, meat, and beer can cause HTN, HA, blurred vision, tachycardia, diaphoresis, and tremors

182
Q

polycistrionic

A

bacterial (e. coli) mRNA can encode multiple genes (eg: lac operon)

183
Q

pompe disease

A

glycogen storage disease type II - lack of lysosomal acid a-glucosidase (acid maltase, a1,4,1,6-glucosidase)

=> accumulation of glycogen in liver and muscle lysosomes => cardiomegaly, hypotonia, macroglossia, hepatomegaly, nl blood glucose

184
Q

fructose metabolism

A

phosphorylated in liver to fructose-1-phosphate; bypasses phosphofructokinase, so metabolized faster than other monosaccharides

185
Q

galactose-1-phosphate uridyl transferase deficiency

A

GALT; catalized galactose 1-phosphate -> glucose-1-phosphate

def => galactosemia => jaundice, vom, hepatomegaly, kidney failure, e. coli sepsis, cataract, hemolytic anemia

tx: avoid lactose, replace with soy milk (sucrose)

186
Q

galactokinase deficiency

A

GALK; catalyzes galactose -> galactose-1-phosphate

def=>cataracts, rarely pseudotumor cerebri

187
Q

renal ammoniagenesis

A

response by renal tubular epithelial cells to chronic acidosis, metabolize glutamine to glutamate, generating ammonium to excrete into urine and bicarb which is absorbed into blood

188
Q

Lesch-Nyhan syndrome

A

XR deficiency of HGPRT => distonia, choreoathetosis, self-mutilation, and hyperuricemia from hypoxanthine and guanine buildup and degreation to uric acid

HGPRT is part of purine salvage pathway, so compensate via de novo purine synthesis (uses PRPP amidotransferase)

189
Q

Ehlers-Danlos syndrome

A

defects in collagen synthesis: procollagen peptidase def => impaired extracellular cleavage of terminal propeptides so can’t crosslink right => joint laxity, hyperextensible skin, tissue fragility

190
Q

NAD+ synthesis

A

3 pathways, using tryptophan, niacin (b3), or nicotinamide

191
Q

pellagra

A

niacin (B3) deficiency => dermatitis, diarrhea, and dementia

192
Q

Aschoff body

A

interstitial fibrosis with central lymphocytes and Anitschkow cells (caterpillar cells) - found in myocarditis from ARF (GAS) and => vitral valve stenosis and regurgitation

193
Q

central line pt safety

A
hand hygiene
barrier precautions
chlorhexidine prep
don't use femoral
remove catheter when no longer needed
194
Q

Thiamine deficiency

A

B1 deb => beriberi (peripheral neuropathy, CHF) and Wernicke-Korsakokff (confusion, ataxia, oculomotor abnormalities, memory deficits)

infantile beriberi: cardiomegaly, tachycardia, cyanosis, dyspnea, and vomiting

dry beriberi: peripheral neuropathy

wet beriber: peripheral neuropathy and cardiac involvement)

195
Q

acute graft rejection

A

cell mediated (type IV H), 40% of heart transplants, 1-4wks post-transplant

histo shows dense infiltrate of mononuclear T-lymphocytes

this is why we give immunosuppressants

196
Q

myocyte relaxation

A

due to calcium efflux via Na/Ca exchange pump and sarcoplasmic reticulum Ca-ATPase pump

197
Q

blotting procedures

A
SNoW  DRoP
Southern = DNA
Northern = RNA
Western = protein
Southwestern = DNA-binding proteins
198
Q

mitochondrial inheritance

A

all offspring of affected females may show disease; no offspring of affected males will habe it

199
Q

genetic disorders on chromosome 3

A

von Hippel-Lindau, renal cell carcinoma

200
Q

genetic disorders on chromosome 4

A

ADPKD (PKD2), achondroplasia, Huntington

201
Q

genetic disorders on chromosome 5

A

cri-du-chat, FAP

202
Q

genetic disorders on chromosome 6

A

Hemochromatosis, ARPKD

203
Q

genetic disorders on chromosome 7

A

Williams Sy, CF

204
Q

genetic disorders on chromosome 9

A

Friedrich ataxia

205
Q

genetic disorders on chromosome 11

A

Wilms tumor, SCD, B-thalassemia, MEN1 (B-globulin gene defects)

206
Q

genetic disorders on chromosome 13

A

Patau Sy, Wilson disease, retinoblastoma (RB1), BRCA2`

207
Q

genetic disorders on chromosome 15

A

Prader-Willi, Angelman, Marfan

208
Q

genetic disorders on chromosome 16

A

ADPKD (PKD1), a-globulin gene defects (a-thalassemia)

209
Q

genetic disorders on chromosome 17

A

neurofibromatosis type 1, BRCA1, p53

210
Q

genetic disorders on chromosome 18

A

Edwards Sy

211
Q

genetic disorders on chromosome 21

A

Down Sy

212
Q

genetic disorders on chromosome 22

A

Neurofibromatosis type 2, DiGeorge Sy (22q11)

213
Q

genetic disorders on X chromosome

A

Fragile X, X-linked agammaglobulinemia, Klinefelter (XxY)

214
Q

vitamin A def + tox

A

(retinol), def => night blindness, dry sclay skin, corneal degeneration (squamous metaplasia), bitot spots on conjunctiva, immunosuppression

acute tox => nausea, vomiting, vertigo, blurred vision

chronic tox => alopecia, dry skin, hepatomegaly, arthralgias, pseudotumor cerebri

also teratogenic (cleft palate, cardiac problems)

215
Q

vitamin B1 def

A

(thiamine)
Wernicke-Korsakoff => confusion, ataxia, confabulation, memory loss
Dry Beriberi => polyneuritis, muscle wasting
Wet Beriberi => Dry Beriberi + CHF

216
Q

vitamin B2 def

A

(riboflavin)

chelosis (lip scaling and fissures), corneal vascularization

217
Q

vitamin B3 def

A

(niacin)
Pellagra (can be from Hartnup disease - AR W def => inability to make niacin, ca, or B6 def) => diarrhea, dementia, dermatitis

218
Q

vitamin B5 def

A

(pantothenic acid)

dermatitis, enteritis, alopecia, adrenal insufficiency

219
Q

vitamin B6 def

A

(pyridoxine), isoniazid can cause def

convulsions, irritability, peripheral neuropathy, sideroblastic anemia (Fe excess)

220
Q

vitamin B7 def

A

(biotin) avidin can cause def (body builders)

dermatitis, alopecia, enteritis

221
Q

vitamin B9 def

A

(folate) from EtOH, pregnancy
small reserve, check B12 levels
megaloblastic anemia, PMNs, glossitis
increased homocysteine and normal methylmalonic acid

222
Q

vitamin B12 def

A

(cobalamin)
megaloblastic anemia, PMNs, paresthesias, subacute combined degeneration
increased homocysteine and methylmalonic acid
can => secondary B9 def

223
Q

vitamin C def

A

(ascorbic acid) - tx for methemoblobulinemia b/c reduces Fe3+ to Fe2+
def => scurvy - swollen gums, bruising, petechiae, anemia, poor healing, corkscrew hair, immunosuppression
tox => n,v,d, fatigue, nephrolithiasis

224
Q

vitamin D def

A

1,25-(OH)2 D3 (calcitriol) = active form
kids - rickets - bow legs (give breastfed infants oral vitamin D)
adults - osteomalacia - bone pain, muscle weakness, hypocalcemic tetany

225
Q

vitamin E def

A

(tocopherol)
hemolytic anemia, acanthosis, muscle weakness, posterior column and spinocerebellar tract demyelination - looks like vitB12 but without megaloblastic anemia, PMNs, or increased methylmalonic acid

226
Q

vitamink K def

A

hemorrhagic disease of the newborn; all neonates given K injection at birth

227
Q

zinc def

A

delayed wound healing, hypogonadism, decreased pubic hair, anosmia, acrodermatitis, enteropathy

228
Q

catecholamine level tests

A

dopamine - measure homovanillic acid
norepinephrine - measure normetanephrine
epinephrine - measure metanephrine

229
Q

phenylketonuria

A

PKU phenylanaline hydroxylase def (or tetrahydrobiopterin/BH4 - cofactor), so can’t make tyrosine from phenylalanine - need tyrosine and BH4replacement and low phenylalanine diet; no aspartame

phenyl ketones in urine, causes sz, fair skin, lack of pigmentation in brain eczema, musty body odor, growth and intellectual retardation

230
Q

albinism

A

tyrosinase def so can’t make melanin from DOPA

231
Q

homocysteinuria

A

increased homocysteine, osteoporosis, marfanoid habitus, down+in eyes, clots, kyphosis, intellectual disability

cystathione synthase def - treat with decreased methionine, increased cysteine, B6, B9, B12

wimpy cystathione synthase - treat with increased cysteine and B6

methionine synthase deficiency - treat with increased methionine

232
Q

von gierke disease

A

glycogen storage disease type I - Glucose-6-phosphatase deficiency

glycogen buildup in liver, lactic acidosis, increased TGs, gout, hepatomegaly, hypoglycemia
tx = frequent oral glucose, avoid fructose and galactose

233
Q

Cori disease

A

glycogen storage disease type III - debranching enzyme def (mild von Gierke)

glycogen buildup in liver but no lactic acidosis or hypoglycemia; limit dextrins in cytosol, intact gluconeogenesis

234
Q

McArdle disease

A

glycogen storage disease type IV - glycogen phosphorylase (myophosphorylase) def in skeletal muscle

muscle can break down glycogen=> cramps, rhabdo, myoglobinuria, arrhythmias, second-wind phenomenon during exercise; no hypoglycemia

235
Q

Tay-Sachs

A

AR hexosaminidase A def => accumulation of GM2 ganglioside in lysosomes

neurodegeneration, cherry red spor on macula, onion skin lysosomes, no hepatosplenomegaly

236
Q

Fabry disease

A

XR a-galactosidase A def => accumulation of ceramide trihexoside

early: episodic peripheral neuropathy, angiokeratomas, and hypohidrosis (not sweating)
late: renal fail and heart disease

237
Q

metachromatic leukodystrophy

A

AR arylsulfatase A def => accumulation of cerebroside sulfate

central and peripheral demyelination with ataxia and dementia

238
Q

krabbe disease

A

AR galactocerebrosidase def => accumulation of galactocerebroside and psychosine

peripheral neuropathy, loss of oligodendrocystes,developmental delay, optic atrophy

239
Q

gaucher disease

A

most common lysosomal storage disease - AR glucocerebrosidase def; treat with recombinant enzyme;

hepatosplenomegaly, pancytopenia, osteoporosis, avascular necrosis, bone crices, gaucher cells (lipid-laden macrophages that look like crumpled tissue paper

240
Q

niemann-pick

A

AR sphingomyelinase def => sphingomyelin accumulation

neurodegeneration, hepatosplenomegaly, foam cells, cherry-red spot on macula

241
Q

hurler syndrome

A

AR a-L-iduronidase mut => developmental delay, “gargoylism”, airway obstruction, corneal clouding, hepatoslenomegaly

242
Q

hunter syndrome

A

XR iduronate sulfatase def => mild hurler (developmental delay, gargoylism, hepatosplenomegaly - no corneal clouding)

243
Q

citrate shuttle

A

in liver, citrate shifted out to cytoplasm to allow fatty acid synthesis (citrate = synthesis)

ATP citrate lyase in traps in cytoplasm by converting citrate to acteryl0CoA

244
Q

Carnitine shuttle

A

in muscle, carnitine shuttle transports fatty acids into mitochondria to be broken into ketone boedies via beta oxydation
used fatty acyl-CoA synthetase and Acyl CoA dehydrogenase

245
Q

primary carnitine deficiency

A

inherited carnitine shuttle defect => toxic accumulation of fatty acids in cytoplasm

weakness, hypotonia, hypoketotic hypoglycemia

246
Q

medium-chain acyl-CoA dehydrogenase deficiency

A

can’t break down fatty acids => accumulation of FAs in blood

hypoketotic hypoglycemia, vom, lethargy, sz, coma, liver failure, SIDS

tx=frequent feedings

247
Q

fed, fasting<1d, starvation 1-3d and >3d

A

fed - energy from glycolysis and aerobic respiration - insulin => storage of lipids, protein, and glycogen

fasting <1day - liver does glycogenolysis and gluconeogenesis; small amt of FA release from adipose - glucagon and epi => use of fuel reserves

<3days - liver uses up glycogen reserved after day 1; it makes glucose from lactate, alanine, glycerol, and propionyl CoA, adipose releases FAs, muscle and liver use FAs, brain uses ketones, and RBCs use glucose

> 3daysbrain uses ketones from adipose stores (this determines survival) and when that runs out vital proteins are degraded => organ failure and death

248
Q

chylomicron

A

delivers dietary TGs to tissues; remnants deliver cholesterol to liver
ApoE, A, C, and B-48 (remnants only have E and B-48_

249
Q

VLDL

A

delivers hepatic TG to tissue

ApoE, C, B-100

250
Q

IDL

A

from degredation of VLDL; delivers Tgs and cholesterol to liver
ApoE and B-100

251
Q

LDL

A

delivers hepatic cholesterol to tissue, from modification of IDL by hepatic lipase
Apo B-100 only

252
Q

HDL

A

transports cholesterol from tissues to liver, stores apo C and E, increased synth in alcoholics
has LCAT, which allows esterification of cholesterol
Apo-E, A, C

253
Q

abetalipoproteinemia

A

AR apoB48 and B100 def => fat malabsortption, steatorrhea, and later retinitis pigmentosa, spinocerebellar deeneration (vitE def), ataxia, and acanthocytosis
tx by restricting LCFAs and supplemental vitE

254
Q

familial hyperchylomicronemia

A

AR LPL or ApoC def => increased chylomicrons, TGs, cholesterol

pancreatitis, hepatosplenomegaly, xanthomas

255
Q

familial hypercholesterolemia

A

AD LPL R def => increased LDL, VLDL, cholesterol

heterozygotes: cholesterol = 300mg/dL, MIs in 30s
homozygotes: cholesterol > 700mg/dL, MIs as kids
accelerated atherosclerosis, achilles xanthomas, corneal arcus

256
Q

familial dybetalipoproteinemia

A

AR ApoE def => increased VLDL and chylomicrons

atherosclerosis, xanthomas, xanthoma striatum palmare

257
Q

familial hypertriglyceridemia

A

AD hepatic VLDL overproduction => increased VLDL, TG

TG>1000mg/dL, acute pancreatitis

258
Q

cryptococcus infection

A

soil and pigeon droppings -> lungs (asymptomatic) -> brain if immunosuppressed

259
Q

MEN

A

RET mutation
MEN1: hyperparaT (hyperCa), pituitary ca (prolactin, visual sx), pancreatic ca
MEN2a: medullary thyroid ca (calcitonin), pheochromocytoma, parathyroid hyperplasia (hyerCa)
MEN2b: medullary thyroid ca (calcitonin), pheochromocytoma, marfanoid habitus and mucosal neuromas

260
Q

acute intermitten porphyria

A

AD porphobilinogen deaminase deficiency => precursor buildup when heme synth pathway (ALA synthase) upregulated due to meds, EtOH, smoking, progesterone, or low-cal diet

causes acute AP and neuro sx, treat with heme, which => feedback inhibition of ALA synthase

261
Q

CGD most common infections

A

cat+ organisms: s. aureus, burkholderia cepacia, serratia, nocardia, and aspergillus

262
Q

scaphoid fx complications

A

nonunion, avascular necrosis

263
Q

hamartoma CXR and path

A

CSR: well-defined coin lesion with popcorn calcifications
path: disorganized cartilage, fibrous, and adipose tissue

264
Q

bladder anatomy

A

extraperitoneal, behind aponeurosis of abdominal wall muscles

265
Q

UV DNA damage

A

thymine dimers - repaired via nucleotide excision repair by UV-specific endonuclease; def in xeroderma pigmentosum

266
Q

VSD murmur

A

large: no murmur
small: harsh holosystolic murmur best heard at the lower left sternal border; increases with increased afterload (handgrip)

267
Q

influenza vaccine function

A

killed vaccine - causes neutralizing anti-HA Abs which prevent infection of cells in the first place

268
Q

salmonella typhi progression

A

typhoid fever
week 1: rising fever, bacteremia, relative bradycardia
week 2: AP, rose spots on trunk and abdomen, watery diarrhea or constipation
week 3: hepatosplenomegaly
intestinal bleeding +perforation

269
Q

JAK-STAT pathway

A

JAK dimerization => STAT dimerization (dimer is TF)

used by growth hormones, cytokines (inf), and hematopoietic growth factors (epo, G-CSF)

270
Q

primary hyperaldosteronism

A

HTN, hypoK, metabolic alkalosis, renin suppression

tx: surgery of aldosterone antagonist (spironolactone, eplerenone)
SE=gynecomastia

271
Q

antifungal targets

A

mitosis - griseofulvin
DNA/RNA synth - flucytosine
cell wass - caspofungin
cell membrane - amphoteracin B + nystatin,\ bind ergosterole, azoles block ergosterole synth

272
Q

holosystolic murmurs

A

Mitral regurg: loudest at apex, radiates to axilla
Tricuspid regurg: best heard at left upper sternal border, increases with inspiration
VSD: best heard at left lower sternal border, accompanied by thrill

273
Q

loop diuretics

A

(furosemide) block NaK2Cl channel in ascending loop of henle - most potent, 1st line in uncomplicated HTN

274
Q

surfactant

A

lecithin (phosphatidylcholine) is main component of surfactant. lecithin:sphingomyelin ratio in amniotic fluid should be 2:1 by week 35

275
Q

chronic granulomatous disease

A

NADPH oxidase mutation => no respiratory burst

test for this with DHR flow cytometry, which looks for superoxide production

276
Q

central and peripheral chemoreceptors

A

central - sees CO2, H+
peripheral (carotid) - sees CO2, H+, and O2
people with COPD no longer respond to high CO2 so their respiratory drive comes from O2; give O2 and they stop breathing

277
Q

SCID

A

ADA def => no T or B cells -> absent thymus, infections, candida, decreased gammaglobulins

278
Q

infection post-lung transplant

A

CMV (enveloped dsDNA) with owls-eye cells

279
Q

elastin production

A

like collagen but elasticity comes from desmosine cross-linking between monomers formed by Cu-dependent lysyl oxidase

280
Q

Ipatropium

A

anticholinergic, used in asthma
blocks ACh action at muscarinic Rs => bronchodilation

SNS activates muscarinic ACh Rs, while PNS suppresses them

281
Q

sheehan syndrome

A

ischemic necrosis of anterior pituitary after hemorrhage in childbirth

282
Q

adrenal medulla

A

center of adrenal gland - chromaffin cells make epinephrine and norepinephrine - activated by ACh from SNS (only adrenals and sweat glands use ACH; rest of SNS uses NE)

283
Q

Waterhouse-Friedrichson Syndrome

A

n. meningitidis make LOS => septic shock and adrenal hemorrhage

284
Q

ADH production

A

ADH is made in the paraventricular and supraoptic nuclei of the hypothalamus and stored in the posterior pituitary gland

Injury to the post. pit. => transient central DI, while injury to the hypothalamus => permanent central DI

285
Q

Hashimoto’s thyroiditis

A

middle aged women with anti-thyroid antibodies => lymphocyte infiltration and germinal centers in thyroid

286
Q

SGLT2 inhibitors

A

canaglifozin, dapagliflozin - used in DM2; cause urinary glucose loss (increased risk of UTI as side effect); avoid in renal failure - check creatinine before starting

287
Q

hypothyroid myopathy

A

fatigue, weight loss, myoedema (focal mound of muscle after percussion, with increase CK from slowed Ca reabsorption by sarcoplasmic reticulum - measure TSH in all people in increased CK because this can precede other symptoms of hypothyroidism

288
Q

carcinoid syndrome

A

flushing, watery diarrhea, and bronchospasm - diagnosis: increased 24 hr urary 5-HIAA, imaging
treatment: surgery, octreotide (somatostatin analog)

if in gut has to be from metasthasis to escape first-pass metabolism by the liver

289
Q

shiga-like toxin

A

O:157:H7 EHEC VF - inactivates human 60s ribosomal subunit (ribosylation)

290
Q

herpes reactivation

A

vesicle => ulcer => crust

291
Q

splitting

A

normal: slightly delayed P2 closure with increased RV filling during inspiration => split S2 during inspiration but not expiration
wide: pulmonic stenosis or RBB => delayed P2 => exaggeration of normal splitting
fixed: ASD => increased RV volume => delayed P2 in both inspiration and expiration
paradoxical: aortic stenosis or LBBB=> delayed aortic valve closure, P2 occurs before A2, so splitting is greater during expiration than inspiration

292
Q

heart ausculatation areas

A

All Physicians Try Marijuana
Aortic - aortic stenosis or slcerosis, flow murmur (physiologic)
Pulmonic - pulmonic stenosis, flow murmur
Triscuspid - tricuspid regurg or stenosis, VSD or ASD
Mitral - mitral regurg, prolapse, stenosis
Left sternal border - aortic and pulmonic regurg, hypertrophic cardiomyopathy

293
Q

normal cardiac pressures

A

RAP <5, RVP 25/5, PAP 25/10, PCWP (LAP) <12, LVP 120/10, AAP 120/80
nickel, quarter over nickle, quarter over dime, dime, systolic/dime, systolic/diastolic

294
Q

cuases of R->L shunts

A

Truncus arteriosus (fails to divide)
Transposition of the great vessels
Tricuspid atresia (needs ASD and VSD for viability)
Tetralogy of Fallot
TAPVR (total anomalous pulmonary venous return)

295
Q

cardiac defects caused by fetal acohol syndrome

A

VSD, PDA, ASK, tetralogy of Fallot

296
Q

cardiac defects caused by congenital rubella

A

PDA, pulmonary artery stenosis, septal defects

297
Q

cardiac defects caused by Down syndrome

A

AV septal defect, VSD, ASD

298
Q

cardiac defects caused by maternal diabetes

A

transposition of the great vessels

299
Q

cardiac defects caused by Marfan syndrome

A

mitral prolapse, thoracic aortic aneurysm/dissection, aortic regurg

300
Q

cardiac defects caused by Li

A

Ebstein anomaly (downward displacement of the tricuspid)

301
Q

cardiac defects caused by Turner syndrome

A

Bicuspid aortic valve, aortic coarctation

302
Q

cardiac defects caused by Williams syndrome

A

aortic stenosis

303
Q

cardiac defects caused by DiGeorge

A

truncus arteriosus, tetralogy of Fallot

304
Q

causes of culture negative endocarditis

A

HACEK - haemophilus, aggregatibacter, cardiobacterium, eikenella, kingella

305
Q

symptoms of bacterila endocarditis

A

FROM JANE

Fever, Roth spots, Osler nodes (ouch), Murmur, Janeway lesions, Anemia, Nail-bed hemorrhage, Emboli

306
Q

normal distribution standard deviations

A

68% within one SD of the mean
95% within two SDs
99.7% within 3 SDs

307
Q

fanconi syndrome

A

reabsorptive defect in the PCT due to Wilson disease, glycogen storage disease, ischemia, ca, drugs, or Pb => metabolic acidosis (proximal renal tubular acidosis)

308
Q

bartter syndrome

A

AR reabsorptive defect in thick ascending loop of Henle (like taking a loop diuretic) affecting the NK2Cl cotransporter => hypokalemia, hypercalciuria, and metabolic alkalosis

309
Q

Gitelman syndrome

A

AR reabsorptive defect of NaCl in DCT (like taking thiazide diuretic) => hypokalemia, hypomagnesemia, metabolic alkalosis, and hypocalciuria

310
Q

Liddle syndrome

A

AD mut => increased Na reabsoprtion in collecting tubules => HTN, hypokalemia, metabolic alkalosis, decreased aldosterone (looks like hyperaldosteronism but with undetectable aldo)

311
Q

SAME

A

syndrome of apparent mineralocorticoid excess - hereditary 11B-hydroxysteroid dehydrogenase deficiency - turns cortisol into cortisone (used in cells with mineralocorticoid Rs to prevent crossreactivity
without 11B, get HTN, hypokalemia, metabolic alkalosis, and low aldosterone (feedback)

glycerrhetinic acid in locorice also blocks 11B

tx: exogenous corticosteroids (don’t act on mineralocorticoid R and feedback to reduce endogenous cortisol production

312
Q

Winters formula

A

predicted respiratory compensation ofr metabolic acidosis
if measured >predicted; respiratory acidosis
if measured

313
Q

anion gap

A

Na-(Cl+HCO3)

314
Q

anion gap acidoses

A

MUDPILES

Methanol, Uremia, DKA, Propylene glycol, Iron/INH, Lactic acidosis, Ethylene glycol, Salicylates

315
Q

non-gap acidoses

A

HARDASS

Hyperalimentation, Addison disease, Renal tubular acidosis, Diarrhea, Acetazolamide, Spironolactone, Saline infusion

316
Q

Renal Cell Carcinoma

A

VHL or other chromosome 3 mutation
polygonal clear cells from proximal convoluted tubule - grossly yellow from lipids - elderly male smokers => hematuria, mass, polycythemia, flak pain, fever, weight loss
paraneoplastic syndromes (EPO, ACTH, PTHrP, renin)
invades renal vein to IVC, mets to lung and bone

tx: surgery, immunotherapy

317
Q

renal oncocytoma

A

benign well-circumscribed mass with central scar from collecting ducts - large eosinophilic cells => painless hematuria, flaink pain, mass
tx: surgery

318
Q

nephroblastoma

A

Wilms tumor - early childhood - embryonic glomerular structures, due to WT1or2 KO (chromosome 11)
large palpable unilateral flank mass + hematuria

319
Q

WAGR complex

A

Wilms tumor, Aniridia ,Genitourinary malformations, mental Retardation (WT1 deletion)

320
Q

Denys-Drash Syndrome

A

Wilms tumor, early nephrotic syndrome, male pseudohermaphroditism (WT1 mut)

321
Q

Beckwith-Wiedemann Syndrome

A

Wilms tumor, macroglossia, organomegaly, hemihyperplasia (WT2 mut)

322
Q

Transitional cell carcinoma

A

urinary tract system tumor (renal calyces - bladder) => painless hematuria
associated with phenacetin, smoking, aniline dyes, and cyclophosphamide (Pee SAC)

323
Q

Squamous cell carcinoma of the bladder

A

due to chronic irritation -> squamous metaplasia -> dysplasia and ca=> painless hematuria
RFs: schistosoma haematobium, chronic cystitis, smoking, chronic nephroplithiasis

324
Q

structures perforating the diaphragm

A

I ate ten eggs at twelve

IVC - 8, esophagus + vagus - 10, aorta - 12

325
Q

bifurcations

A

biFOURcations
common carotid - C4
trachea - T4
abdominal aorta - L4

326
Q

physiologic deas space

A

tidal volume * (PaCO2-PeCO2)/PaCO2

327
Q

blood O2 content

A

(1.34Hb levelSaO2) + (0.003 * PaO2)

328
Q

pulmonary vascular resistance

A

PVR = (Ppulm a - Pl atrium)/CO

329
Q

alveolar gas equation

A

PAO2=PIO2-PaCO2/R = 150mmHg-PaCO2/0.8 at sea level on room air

330
Q

A-a gradient

A

PAO2-PaO2 - normally between 10 and 15 mmHg

331
Q

hypoxia v hypoxemia

A

hypoxia is decreased O2 deliver to tissues

hypoxemia is decreased PaO2

332
Q

asbestosis

A

shipbuilding roofing, plumbing
ivory white, calcified, supradiaphragmatic and pleural plaques in lowe lobes
ferruginous bodies on prussian blue stain
causes bronchogenic carcinoma and mesothelioma

333
Q

berylliosis

A

aerospace, manufacturing

granulomatous (noncaseating on histology), affects upper lobes - tx with steroids

334
Q

coal worker’s pneumoconiosis

A

black lung disease
macrophages laden with carbon => inflammation and fibrosis, upper lobes
asymptomatic version in cities is called anthracosis

335
Q

silicosis

A

foundries, sandblasting, mines
macrophages => fibrosis, silical impairs function => increased TB susceptibility
affects upper lobes
eggshell calcifications of hilar LNs on CXR

336
Q

mesothelioma

A

pleural malignancy, from asbestosis
psammoma bodies, cytokeratin and calretinin +
smoking isn’t an RF

337
Q

airway cell types

A

bromchi: ciliated pseudostratified columnar epithelium with mucin-secreting goblet cells, cartilage, and submucosal mucoserous glands => ciliated simple cuboidal at terminal bronchioles (no glands or cartilage)+> no goblet cells by terminal bronchiole

cilia stay until respiratory bronchioles

338
Q

charcot-bouchard aneurysm

A

<1mm aneurysm in basal ganglia, cerebellum, thalamus, or pons, associated with HTN, => intracerebral hemorrhage which causes progressive neuro deficits and then HA

339
Q

Saccular aneurysms

A

(berry aneurysm) 2-25 mm aneurysm in circle of Willis associated with ADPKD, Ehlers-Danlos, and HTN, => subarachnoid hemorrhace which causes sudden severe HA

340
Q

myocardial hibernation

A

response to ischemia in which cells decrease metabolism and function to match reduced blood flow, => decreased contractility which improves with revascularization

341
Q

G neurotransmitters

A

GABA and glycine are inhibitory

glutamate is activating

342
Q

length constant

A

distance along axon at which AP decreases to 37% of original amplitude

decreased in demyelinating processes

343
Q

time constant

A

time it takes for an change in membrant potential to achieve 63% of the new value

increased in demyelinating processes

344
Q

dobutamine

A

B agonist B1>B2 and a1

stimulation of B1 => increased cAMP => increased cyctosolic Ca2+ => increased myocardial contractility

stress test, decompensated heart failure

345
Q

iron homeostacic

A

regulated by hepcidin, an acute phase reactant made by the liver

high Fe, inflamm => increased hepcidin => ferroportin degredation => decreased intestinal Fe absorption and decreased release from macrophages

346
Q

hemoglobin electrophoresis

A

HbA (nl) moves quickly toward + end(anode) b/c negatively charged

HbS has a missnce mut => replacementof a - AA with a neutral AA, so moves less quickly

HbC has a missense mut => replacement of a - AA with a + AA, so moves even more slowly

347
Q

serum heterophile antibodies

A

agglutinate RBCs from other species - caused by EBV

heterophile-negative mono-like syndrome is most likely CMV - can get CMV from blood transfusion

348
Q

Rubella

A

togavirus => maculopapular rash spreading from face to body (also in measles/rubeola) but fast and comes with postauricular lymphadenopathy

349
Q

acne

A

caused by sebaceous glands, which are holocrine exocrine glands (cell lysis)

apocrine glands release vesicles and merocrine glands release watery product via exocytosis

350
Q

anti-RH(D) IG

A

IgG anti-D Abs which opsonize fetal RBCs and prevent maternal sensitization

IgM wouldn’t work b/c IgM isn’t an opsonin

351
Q

Mullerian aplasia v. androgen insensitivity syndrome

A

Mullerian aplasia - XX, with short vagina +/- uterus; normal ovaries and secondary sexual characteristics; likely to have renal abnormalities as well

androgen insensitivity - XY => no uterus or ovaries; minimal axillary and pubic hair

352
Q

iatrogenic Cushing Sy

A

from long-term glucocorticoids (eg for SLE) => decreased ACTH and corisol; low ACTH => bilateral adrenal cortical atrophy

sudden cessation of glucocorticoids after long-tern use can => adrenal insufficiency (adrenal crisis)

353
Q

severe hyertriglyceridemia

A

> 1,000 => acute pancreatitis
treat with lipid-lowering agent such as fenofibrates (fibrates cause bigger drop than other drugs like statins) which activates PPAR-a => increased LPL synth

354
Q

causes of orthostatic hypertension

A

a1 antagonists (terazosin), hypovolemia, autonomic dysfunction (Parkinson, DM2)

355
Q

leukocyte adhesion deficiency

A

leukocytes lack CD18 which is needed for integrin formation and adhesion to endothelium

=> recurrent nonpurulent skin + mucosal infections (often staph), impaired wound healing and late umbilical cord separation, as well as persistent leukocytosis

356
Q

carvedilol

A

beta blocker

357
Q

most common cause of anemia in alcoholism

A

folate (B9) deficiency - can develop within weeks => macrocytic anemia and neutrophils with hypersegmented nuclei

358
Q

vision from left hemiretina

A

passes through left: optic n., lateral optic chasm, optic tract, lateral geniculate, optic radiations, and primary visual cortex

359
Q

Renal Cell Carcinoma histo

A

clear cells filled with accumulated glycogen and lipids

360
Q

temperal lobe lesions

A

injury to meyer’s loop in the temporal lobe causes a contralateral pie in the sky lesion (opposite quadrant gone, both eyes)

361
Q

adult hep b infection course

A

95% develop mild acute hepatitis which then resolves completely

362
Q

stapedius musc. fcn and innervation

A

stabilizes the stapes; lesion causes hyperacusis (can accompany Bell’s palsy)

innervated by the stapedius n. which is a branch of the facial n. (CN VII)

363
Q

glioblastoma gross path

A

very aggressive, single tumor which may cross the midline and has areas of necrosis and hemorrhage - edges may be less well defined than with a met

364
Q

Gilbert syndrome

A

mild reduction in UGT1; causes unconjugated hyperbilirubinemia with stress

365
Q

neurosyphilis spine complication

A

tertiary neurosyphilis causes damage to dorsal columns

366
Q

prevention of colon polyps

A

low dose ASA - reduces endothelial proliferation

367
Q

lung cancer locations

A

squamous and small cell cacinomas are Sentral and often caused by Smoking
SmallCC - central, very aggressive
Squamous CC - central, hilar mass, hypercalcemia
Adenocarcinoma - peripheral - common, clubbing
Bronchial carcinoid is subtype of adeno - less smoking association, causes flushing, diarrhea, wheezing
Borncheoalveolar is subtype of adeno - better prognosis, hazy infiltrates on CXR
Large CC - peripheral - poor prognosis

368
Q

adrenal anatomy

A

cortex - from mesoderm
-G makes aldosterone when stim by angiotensin II
-F makes cortisol when stim by ACTH
-R makes DHEA when stim by ACTH
medulla - from neural crest - chromaffin cells - makes catecholamines when stimulated by preganglionic SNS

369
Q

pancreas endocrine cells

A

Islets of Langerhans - collections of a, B, and d cells
a - peripheral - glucagon
B - central - insulin
d - interspersed - somatostatin

370
Q

hashimoto thyroiditis

A

antithyroid peroxidase and antithyroglobulin Ab => early hyperthyroid 2/2 thyrotoxicosis from follicular rupture => hypothyroid
enlarged, nontender thyroid
increased risk of non-Hodgkin lymphoma
Histo: Hurthle cells, lymphoid aggregates + germianl centers

371
Q

congenital hypothyroidism

A

can be 2/2 maternal or fetal hypothyroidism or I2 deficiency

pot-belly, pale, puffy face, protruding umbilicus and tongue, poor brain development

372
Q

subacute granulomatous thyroiditis

A

(de Quervain) self-limited thyroid cell destruction following viral infection => initial hyperthyroid then hypothyroid, with pain in thyroid and jaw and elevated ESR
histo shows granulomatous inflammation

373
Q

medication causes of hypothyroidism

A

amiodarone, Li

374
Q

Riedel thyroiditis

A

thyroid replaced by fibrous tissue with inflammatory infiltrate - only hypothyroid in 1/3 of cases
fixed, hard, painless goiter

375
Q

graves diseas

A

thyroid-stimulatin gIG (IgG- type II H) => hyperthyroidism, pretibial myxedema, and infiltration of retroorbital space by activated TCs => exophthalamos

376
Q

toxic multinodular goiter

A

focal patchtes of constitutively hyperfunctioning follicular cells - these “hot” nodules are rarely malignant

377
Q

thyroid storm

A

incompletely treated hyperthyroidism worsens in acute stress => agitation, delirium, fever, diarrhea, coma, and tachyarrythmias; possibly elevated liver enzymes
tx: propranolol, propylthiouracil, prednisolone, potassium iodide

378
Q

Jod-Basedow phenomenon

A

thyrotoxicosis (hyperthyroidism) when pt with iodine def and some autonomous thyroud tissure is made iodine replete

379
Q

Wolff-Chaikoff effect

A

thyroid gland downregulation in response to increased iodide