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uWorld 19 Flashcards

1
Q

what is train-of-four (TOF) stimulation

A

used during anesthesia to assess the degree of paralysis induced by NMJ-blocking agents
a peripheral nerve is stimulated 4 times in quick succession and the muscular response is recorded
the height of each bar represents the strength of each twitch; higher bars indicate the activation of increasing numbers of individual muscle fibers (myocytes)

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2
Q

what is seen on TOF stimulation when a NON depolarizing NMJ blocker (vercuronium) is administered

A

competitive inhibition of postsynaptic acetylcholine receptors at the motor endplate prevents some of these fibers from activating, decreasing the strength of the twitch
PROGRESSIVE REDUCTION in each of the 4 responses (FADING PATTERN) as a result of less acetylcholine being released with each subsequent impulse (due to additional effect of presynaptic acetylcholine receptor blockade)

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3
Q

what is seen on TOF stimulation when a depolarizing NMJ blocker (vercuronium) is administered

A

initially function by preventing depolarization of the motor endplate and show EQUAL REDUCTION of all 4 twitches during TOF stimulation (PHASE I BLOCKADE)
the responses remain equal b/c presynaptic acetylcholine receptor stimulation helps to mobilize presynaptic acetylcholine vesicles for release
persistent exposure to succinylcholine results in eventual transition to PHASE II BLOCKADE as the acetylcholine receptors become desensitized and inactivated (functionally similar to nondeplolarizing blockade)

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4
Q

succinyl choline is commonly administered for what

A

RAPID-SEQUENCE INTUBATION due to its rapid onset (less than 1 minute)

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5
Q

twisting injury or blow t the lateral knee while the foot is planted (VALGUS STRESS INJURY) to the knee results in what

A

MEDIAL COLLATERAL LIGAMENT (MCL) injury

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6
Q

what is the valgus stress test

A

performed when the knee is extended by placing one hand along the lateral thigh and pressing inward while the other hand is placed not eh medial aspect of the ankle and pushed outward
laxity of the knee and/or medial joint line widening indicate MCL injury

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7
Q

how is meniscal injury diagnosed

A

joint line tenderness while the knee is flexed

medial meniscal injury can occur with twisting moment of the knee and often accompanies MCL injury

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8
Q

what does the act do and what tests are there for its injury

A

resits anterior movement and the medial rotation of the tibia
injury indicated by increased anterior movement of the tibia in relation to the femur
anterior draw test
Lachman test

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9
Q

excessive backward movement of the tibia in relation to the femur indicates what

A

posterior draw test

PCL injury

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10
Q

what is ATTRITION BIAS

A

the loss of subjects differ in their rest of developing the outcome compared to the raining subjects
is a form of SELECTION BIAS

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11
Q

what is misclassification bias

A

occurs when exposure or the outcome is not identified correctly
random (or nondiffernetial) misclassification affects all the groups the same
Ex: pediatric cuff used on adults in a study but the control and the treatment used the same cuff = random missclassificaiton

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12
Q

NADH methemoglobin reductase does what and a deficiency results in what

A 
reduces ferric (fe3+) iron to ferrous (Fe2+) iron, regenerating hemoglobin from methemoglobin
deficiency results in methemoglobinemia
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13
Q

goblin chain denaturation occurs in what disorder

A

G6PD deficiency

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14
Q

what cytokine causes cachexia

A

TUMOR NECROSIS FACTOR-ALPHA (aka cachectin) by suppressing appetite and increases basal metabolic rate
along with IL-1beta and IL-6 are main mediators of paraneopalsic cachexia

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15
Q

TNF-alpha is produced by what and mediates what

A 
macrophages
fever (along with IL-1), mediates many of symptoms of septic shock, causes hepatic release of ACUTE PHASE REACTANTS (c-reactive protein, fibrinogen, hepcidin)
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16
Q

what does IL-3 do

A

produced by activated CD4+ Th cells

stimulates growth and fifernetiaiton of myeloid cells

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17
Q

what is the main function of TGF-beta

A

inhibition of inflammatory response

decreases T-cell proliferation and cytokine prodcution

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18
Q

what is seen histologically in the kidneys from the damage that calcineurin inhibitors (cyclosporine, tacrolimus) do

A

early arteriolar hyalinization and TUBULAR VACUOLIZATION

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19
Q

what is seen in wischott aldrich

A

WATER
thrombocytopenia (petechia, purpura, hematemesis and epistaxis)
eczema
recurrent infections (combined B and T-lyphocyte disorder)

increased risk of PYOGENIC INFECTIONS b/c they can’t mount immune response to things with polysaccharide capsule (N. meningitis, H flu, step pneumo)
t-cell defect they get fucked by PCP and HSV

becomes apparent at 6 months
treated with HLA-matched bone marrow

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20
Q

what are the important clinical findings of Chediak higashi

A

ocular albinism
peripheral neuropathy
immunodeficiency related to dysfunction of phagolysosome formation

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21
Q

what is seen in Krebbe disease

A

galactocerebroside accumulation

progressive neurodegeneration, peripheral neuropathy, optic atrophy

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22
Q

what is seen in Fabry disease

A

globotriaosylceramide accumulation

causes angiokeratomas, peripheral neuropathy, and glomerulopathy that typically present in adulthood

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23
Q

what is seen in von Gierke disease

A 
glucose-6-phosphatase deficiency
hepatomegaly
hypoglycemia
seizures
lactic acidosis
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24
Q

what is seen in pompe

A

lysosomal alpha-1,4-glucosidase deficiency
cardiomegaly
severe hypotonia

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25
blood supply to the femoral head derives mainly from where
ascending cervical and retinaclular branches of the MEDIAL CIRCUMFLEX ARTERY (branch off deep femoral)
26
what arteries form the trochanteric anastomosis
branches of the lateral circumflex and superior and inferior gluteal arteries join with the medial circumflex artery
27
what vessel is important in children because it supplies the region of the femoral head proximal to the epiphyseal growth plate
artery of ligamentum heres (form the obturator artery) minimal clinical significance in adults
28
abdominal and flank bruits are specific for what
renal artery stenosis | but found in minority of patients
29
what is a root cause analysis
quality improvement measure that aims to identify what, how and why an undesirable outcome occurred first step is collecting data to obtain complete information about the event or events
30
RB tumor suppressor gene (anti-oncogene) is located where
Ch 13
31
a blood flow curve showing cyclical variation in flow during the cardiac cycle, with MAXIMUM FLOW occurring during DIASTOLE and minimal flow occurring during ventricular systole is unique for what tissue
LEFT VENTRICULAR MYOCARDIUM majority of blood flows through the vascular beds when the myocardium is relaxed and not comprising the vessels systolic reduction in flow is greatest in subendocardial myocardium (where wall pressure is the highest)- region is prone to ischemia and MI
32
the right ventricle blood flow appears how throughout the cardiac cycle
relatively constant pressures in right ventricle are much lower than the left, and as a result the increase in systemic blood pressure during diastole is able to compensate for the rise in right ventricular wall pressure
33
what are markers of osteoclast activity and what is the most reliable of the three
``` tartrate-resistant acid phosphatase urinary hydroxyproline (also found in meat products) urinary DEOXYPYRIDINOLINE (most reliable) ```
34
what is a reflects osteoblastic activity
bone-specific alkaline phosphatase
35
what does pyridinoline do
covalently cross-links collagen fibers | these cross-links are the most commonly used method in assessing osteoclastic activity
36
whats the difference between urticaria and angioedema
urticaria is due to increased permeability of the micro vasculature leading to edema of the SUPERFICIAL DEMRIS angioedema is involvement of the deep semis and subcutaneous tissue
37
what is dyskeratosis
abnormal, premature keratinization of individual keratinocytes strongly eosinophilic and may have small, basophilic nuclear remnant found in squamous cell carcinoma
38
what is acantholyisis
loss of cohesion b/w keratinocytes in the epidermis | seen in pemphigus family of disorders
39
what is acanthosis
diffuse increase in thickness of the status sinus (prickle cell layer) b/w the granular cell layer and the basal layer of the epidermis psoriasis, seborrheic dermatitis, acanthosis nigricans
40
what is spongiosis
intercellular epidermal edema that histologically appears as an increase in the width of places b/w cells often associated with spongiotic dermatitis, which encompasses a number of conditions (eczematous dermatitis)
41
URTICARIA a common transient HYPERSENSITIVITY disorder is characterized by intensely pruritic, raised, erythematous plaques (WHEALS) that arise suddenly and resolve over several hours, what causes this
IgE-meidated degranulation of mast cells can also be due to non-IgE-mediated degranulation (opiates, radiocontrast agents, physical urticaria) or mast cell independent mechanisms (aspirin, hereditary andgioeurotic edema)
42
what does somatostatin do
secreted from "delta" cells in the pancreatic islet decreases secretion of secretin, cholecystokinin, glucagon, insulin, and gastrin inhibits production of growth hormone from anterior pituitary
43
what is a somatostatinoma
rare pancreatic islet cell tumor arising from delta cells present with: hyperglycemia steatorrhea (dec secretin and GI motility) galbladder stones (dec CCK) hypochlorhydria (dec gastrin)
44
what is a derivative of the 1st aortic arch
maxillary artery
45
what is a derivative of the 2nd aortic arch
stapedial artery (typically regresses in humans)
46
what is a derivative of the 4th aortic arch
part of true aortic arch and the subclavian arteries
47
what is a derivative of the 6th aortic arch
pulmonary arteries and ductus arteriosus
48
serum IgG4 antibodies to the M-type phospholipase A2 receptor (PLA2R), a transmembrane protein abundant on podocytes is indicative of what
idiopathic membranous nephropathy
49
minimal change disease is possibly due to what
abnormal T cell production of glomerular permeability factor that affects the glomerular capillary wall, leading to fusion of the foot processes and marked proteinuria
50
mixed cryoglobulinemia is seen in patients with what
hepatitis C renal disease likely due to IgM deposition in the glomerulus that leads to basement membrane thickening and cellular proliferation
51
what is the MOA of buspirone
partial agonist fo the 5HT1a receptor no muscle relaxant or anticonvulsant properties no risk of dependence slow onset of action
52
what is seen on water deprivation test in patients with partial nephrogenic diabetes insipidus
slow but steady rise in urine osmolality with increasing serum osmolality after water deprivation no further increase in urine osmolality with DDAVP, and the urine osmolality remains low
53
what is seen in patients with primary polydipsia
increase in serum and urine osmolality on water deprivation that is similar to partial nephrogenic DI, however the correction in primary polydipsia is more rapid, and the urine osmolality returns t a level closer to normal (but still sub maximal due to washout of the medullar osmotic gradient) history of psychiatric disorders or medication-induces xerostomia is usually present
54
when and why does one see EUVOLEMIC HYPONATREMIA
SIADH mild increase in extracellular fluid volume surpasses the renin-aldosterone axis and stimulates the production of the natriuretic peptides, leading to excretion of sodium in the urine (natriuresis)- normal body fluid volume and low plasma osmolality
55
anti-A IgG and anti-B IgG is seen with what blood group(s)
O blood group (OO) | no antigen on RBC
56
anti-A IgM is seen with what blood group(s) | anti-B IgM is seen with what blood group(s)
anti-A IgM: blood group B (B antigen on RBC) | anti-B IgM: blood group A (A antigen on RBC)
57
what blood group has no antibodies in the serum
AB | but has A and B antigen on red blood cells
58
what blood type mother can cause ABO hemolysis in a fetus
O because they have IgG anti-A and anti-B antibodies in serum which can cross the placenta causing hemolysis in the fetus can occur in first pregnancy unlike Rh disease which requires a previous pregnancy
59
what is Eptifibatide
inhibits platelet aggregation and thrombosis by blocking the glycoprotein IIb/IIIa receptor (binding site for fibrinogen) typically used in acute coronary syndrome and in patients undergoing percutaneous coronary intervention
60
what is ticagrelor
binds DP P2Y12 receptor on platelets, which prevent platelet aggregation by blocking ADP-mediated activation of GP IIb/IIIa receptor complex
61
what are echinocandin antifungals
caspofungin- block gluten synthesis
62
how does flu cytosine work
inhibits the synthesis of DNA (replication) and RNA (protein synthesis) in fungal cells
63
how does griseofulvin work
enters fungal cells and binds microtubules, inhibiting mitosis CPY450 inducer
64
how does gabapentin work
blocking voltage-gated (presynaptic) calcium channels, thereby attenuating excitatory neurotransmitter release
65
basement membrane splitting is seen on light microscopy using silver or PAS stains in what kidney problem
membrnaoproliferative glomerulonephritis (MPGN) glomeruli have lobular appearance with proliferate mesangial cells and increased mesangial matrix GRANULAR DEPOSITS seen on immunoflourescnece
66
uniform, diffuse capillary wall thickening on LM is a sign of what kidney problem
membranous glomerulopathy granular deposits of IgG and C3 on immunofluorescence typical nephrotic syndrome presentation
67
what is seen on immunofluorescence of focal segmental glomerulosclerosis (FSGN)
IgM and C3 deposits may be seen in the sclerotic areas due to nonspecific trapping of these components
68
what are the deposits made of in good pastures
``` IgG and C3 linear deposits from anti-GBM antibodies targeting type IV collagen glomerular crescents (RPGN) on LM (proliferating parietal cells with an infiltration of monocytes and macrophages are seen on LM ```
69
viral RNase H does what
removes the RNA primary, allowing for completion of newly synthesized DNA important pharmaceutical targets for the treatment of retroviruses such as HIV
70
what is seen on the CSF of herpes simplex encephalitis
HEMORRHAGIC LYMPHOCYTIC PLEOCYTOSIS with INCREASED protein and normal glucose also abnormal MRI signal in the bilateral TEMPORAL LOBES TX: IV acyclovir
71
what kind of receptor is Human epidermal growth factor receptor 2 (HER2)
TYROSINE KINASE | blockade of HER2 w/ trastuzumab downregulates CELLULAR PROLIFERATION and PROMOTES APOPTOSIS
72
what is bevacizumab
monoclonal antibody to vascular endothelial growth factor (VEGF) which binds a TYROSINE KINASE treats METASTATIC cancers (colon, lung)
73
what is denosumab
human monoclonal antibody against RANKL used to reduce skeletal-related events if bets to bone occurs
74
when are aromatase inhibitors (anastrozole, letrozole) used for
adjunct therapy for postmenopausal patients who have breast cancer with ER and/or PR tumor expression
75
changes in the concentration and ratios of components such as ______ can shift the balance of urine to cause salt precipitation and stone formation
``` calcium phosphate oxalate uric acid citrate ``` increased concentrations of all (BUT CITRATE) promote formation of stones
76
high urine concentrations of what has stone-preventing effect by binding free (ionized) calcium, preventing its precipitation and facilitating its excretion
CITRATE potassium citrate is often prescribed to prevent recurrent calcium stones in adults when dietary modifications are unsuccessful
77
what is required for the formation of uric acid and cysteine stones, and also promotes calcium oxalate crystal formation
acidic pH
78
hyperuricosuria is associated with what conditions
gout myeloproliferative disorders situations of high purine and alcohol intake can be idiopathic
79
what is a pancreatic pseudocyst
common compilation of pancreatitis collection of fluid rich in enzymes and inflammatory debris inflammatory reaction in the walls of the surrounding organs
80
what makes up a pancreatic pseudocyst
walls consist of granulation tissue and NO epithelial lining fibrosis, thickening and organization of the walls occur with tie formation of firm fibrotic pseudocyst walls ("maturation" of pseudocyst) takes about 4 to 6 weeks following the episode of acute pancreatitis
81
what is the most common location for a pseudocyst
the lesser peritoneal sac, bordered by the stomach, duodenum and transverse colon walls are closely adherent to the surrounding hollow organs
82
when are glycogen-rich cuboidal epithelium seen in the pancreas
serous pancreatic neoplasms
83
when is columnar mucinous epithelium found in the pancrease
mucinous cystic neoplasms of the pancreas
84
when are atypical cells forming papillary projections seen in the pancreas
papillary variant of pancreatic adenocarcinoma
85
the blood supply tot he PROXIMAL URETER comes from where | what about the distal ureter
proximal ureter: the RENAL ARTERY distal ureter: superior vesical artery in between: anastomotic and highly variable, with possible afferent branches form the gonadal, common iliac, aorta, and uterine arteries
86
how is the blood supply established in a kidney transplant
anastomosing the DONOR RENAL ARTERY with the RECIPIENT EXTERNAL ILIAC ARTERY proximal 1/3 of donor ureter is preserved and used to establish continuity from the collecting system of the kidney to the recipients bladder distal part is susceptible to ischemia due to lack of anastomotic connections
87
what is a recognized complication of renal transplant and causes leakage of urine 5-10 days following transplant
distal ureteral ischemia
88
what is an aspergilloma (mycetoma)
aspergillus COLONIZATION of a PREEXISTING LUNG CAVITY forming a FUNGUS BALL may be asymptomatic or may cause cough and HEMOPTYSIS not contagious
89
when does one see invasive aspergillosis
invasive pulmonary aspergillosis occurs in severely immunosuppressed and neutropenic patients and is characterized by primary lung involvement with cough, hemoptysis, pleuritic chest pain, fever necrotizing pneumonia and granuloma formation also occur extra pulmonary involvement is common
90
what is a EUNUCHOID BODY HABITUS and when is it seen
result of testosterone deficiency tall stature and gynecomastia facial and body hair is sparse or absent and muscle mass is decreased seen in KLINEFELTER
91
when does one see short stature, hypotonia, intellectual disability and obesity
prader-willi | microdeletion of paternal ch 15p11-13
92
when does one see macroorchidism, large jaw, and intellectual disability
FRAGILE X SYNDROME | X-linked disorder w/ mutations in FMR1 gene
93
arachnodactylyl, scoliosis, and aortic rood dilation are signs of what
Marfan | fibrillar defect
94
when are necrotizing inflammation and pulmonary hemorrhage seen
granulomatosis with polyangitis | presents sub acutely with cough, dyspnea, and hemoptysis and neuritic syndrome
95
fevers, hemodynamic instability, tachypnea, and evidence of urinary tract infection with urosepsis is likely complicated by what
ACUTE RESPIRATORY DISTRESS SYNDROME (ARDS) sepsis and pulmonary infections are the two most common risk factors for ARDS
96
what is AR ORGANIC ACIDEMIA
deficiency of enzyme METHYLMALONYL-CoA MUTASE result is a build up of methylmalonic acid and propionic acid leading to METABOLIC ACIDOSIS HYPOGLYCEMIA results from overall increased metabolic rate leading to increased glucose utilization and direct toxic inhibition of gluconeogenesis by the organic acids KETONES are produced in preens of hypoglycemia HYPERAMMONEMIA occurs from organic acids directly inhibiting the urea cycle
97
what are the signs of organic acidemia (methylmalonaly-CoA mutase deficiency)
anion gap metabolic acidosis hypoglycemia ketosis hyperammonemia evaluated during METHYLMALONIC ACID AND PROPIONIC ACID
98
wha is propionic acidemia
deficiency in propionyl-CoA carboxylase hyperammonemia hypoglycemia metabolic acidosis NO elevated METHYMALONIC ACID
99
production of ketones in the presence of hypoglycemia is the normal response of what
fasting state (as keynotes provide fuel for brain)
100
fatty acid oxidation disorder, like medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, can present with what
hypoglycemia hyperammonemia metabolic acidosis
101
urea cycle defects typically present with what
hyperammonemia without hypoglycemia or ketosis
102
nutritional deficiencies can result in what kind of myopathy
dilated Thiamine (B1) (wet beriberi heart disease) or chronic anemia
103
what is seen histologically in viral myocarditis
lymphocytic interstitial inflammatory infiltrate, w/ focal across of myocytes adjacent to areas of inflammatory cells
104
toxic agents like alcohol, cocaine, iron (in hemochromatosis), or doxorubicin may cause what heart problem
dilated cardiomyopathy

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