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Capstone Test 1 > Leg Pain Cases > Flashcards

Leg Pain Cases Flashcards

(67 cards)

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1
Q

what genetic mutation results in sickle cell anemia?

A

replacement of glutamic acid (hydrophilic) with valine (hydrophobic) in the Beta chain

chromosome 11

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2
Q

sickle cell disease arises when two abnormal […] chains are present

A

beta

(results in >90% HbS)

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3
Q

HbS […] when deoxygenated, resulting in needle-like structures

A

polymerizes

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4
Q

increased risk of sickling occurs with [3]

A

hypoxemia
dehydration
acidosis

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5
Q

Hb[…] protects against sickling

A

HbF

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6
Q

high [….] at birth is protective against sickling for the first few months of life

A

HbF

(no need for hydroxyurea in newborns, consider PCN prophylaxis against capsular bacteria)

had an Amboss question that said PCN ppx for first five years of live

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7
Q

treatment of SCD with […] increases levels of HbF

A

hydroxyurea

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8
Q

patients with SCD have a high risk of aplastic crisis with [….] infection

A

Parvovirus B19

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9
Q

patients with SCD have an increased risk of […] osteomyelitis

A

salmonella paratyphii

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10
Q

autosplenectomy in patients with SCD will result in […] on blood smear

A

Howell-Jolly

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11
Q

what is the most common cause of death in children with SCD?

A

sepsis by encapsulated bacteria

(S. pneumo, H. flu)

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12
Q

what is the most common cause of death in adults with SCD?

A

acute chest syndrome

(vaso-occlusion of pulmonary microcirculation; often precipitated by pneumonia)

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13
Q

one mutated and one normal B chain results in

A

sickle cell trait

(HbA and HbS on electrophoresis)

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14
Q

RBCs with less than 50% HbS do not sickle, EXCEPT for in the […]

A

renal medulla

(seen in sickle cell trait)

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15
Q

what would you expect to see on blood smear of a patient with SCD? [2]

A

sickle cells
target cells

(would not be seen in sickle cell trait)

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16
Q

[…] screen causes cells with any amount of HbS to sickle
will be positive in both SCD and trait

A

metabisulfate

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17
Q

massive […] hyperplasia in patients with SCD results in “crewcut” skull and “chipmunk facies”

A

erythroid

(extramedullary hematopoiesis)

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18
Q

patients with SCD often present with […] due to vaso-occlusive infarcts in bones

A

dactylitis

(often first sign seen in infants0

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19
Q

does SCD present with extravascular hemolysis or intravascular hemolysis?

A

Both - but mostly extravascular

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20
Q

how would you differentiate SCD from beta thalassemia?

A

beta thalassemia is microcytic, SCD is normocytic

electrophoresis would have HbA2 in beta thalassemia

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21
Q

how would you differentiate SCD from hereditary spherocytosis?

A

cell shape

spherocytosis is diagnosed via osmotic fragility test (fragility in hypOtonic solution)

spherocytosis has high RDW and high MCHC

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22
Q

how would you differentiate SCD from hereditary pyruvate kinase deficiency?

A

PKD presents in newborns with jaundice

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23
Q

what are the adverse effects of hydroxyurea? [3]

A

myelosuppression
mastocytosis

darkening of skin/nail beds

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24
Q

Sickle cells lack elasticity and adhere to vascular endothelium, which disrupts microcirculation and causes vascular […] and subsequent tissue […]

A

occlusion

infarction

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25
painless gross hematuria in a patient with sickle cell trait may indicate
renal papillary necrosis
26
inhibits xanthine oxidase, preventing the formation of uric acid
allopurinol
27
what type of crystals precipitate in gout?
monosodium urate
28
in gout, crystals are [...] shaped
needle
29
in gout, crystals are [...] birefringent
negatively
30
in pseudogout, crystals are [...] shaped
rhomboid
31
in pseudogout, crystals are [...] birefringent
weakly positive
32
what type of crystals precipitate in pseudogout?
calcium pyrophosphate
33
treatment of acute gout [3]
NSAIDs colchicine corticosteroids
34
treatment of chronic gout [2]
allopurinol febuxostat
35
mechanism of action of colchicine
prevents microtubule assembly --> disrupts neutrophil chemotaxis
36
mechanism of action of probenecid
URAT inhibitor prevents reabsorption of uric acid from PCT --> increases excretion
37
[...] syndrome is characterized by a hypercoaguable state antibodies such as anti-lipid coagulant and anti-cardiolipin
anti-phospholipid syndrome
38
history red flags for anti-phospholipid syndrome in SLE
DVT hepatic vein thrombosis stroke recurrent pregnancy loss
39
what serum antibody is sensitive, but not specific for SLE?
ANA
40
what serum antibodies are highly specific for SLE? [2]
Anti-dsDNA anti-Sm
41
deficiency of [...] complement proteins is associated with SLE
early C1q, C4, C2
42
deficiency of early complement proteins in SLE causes decreased clearance of [...]
immune complexes
43
describe some characteristics of a classic presentation of SLE
female of reproductive age facial rash joint pain fever oral ulcers photosensitivity
44
what would you expect to see on skin biopsy in SLE?
-DEJ "smudging" -holes in the basal layer "vacuolization" -melanin dropout -dead reds
45
Mesangial and/or subendothelial deposition of immune complexes (C3 and C1q) → expansion and thickening of mesangium, capillary walls, and/or glomerular basement membrane
lupus nephritis
46
most common nephritis syndrome associated with SLE
diffuse proliferative glomerulonephritis | aka lupus nephritis
47
SLE is a type [...] HSR
III antigen-antibody complexes damage tissues
48
MOA inhibits microtubule polymerization by binding to the cytoskeleton
colchine (impairs phagocytosis, chemotaxis, and migration of neutrophils)
49
side effects of colchicine
GI upset myelosuppression hair loss
50
Sickle cell trait causes [...] secondary to transient sickling of RBCs in the renal capillaries
renal papillary necrosis
51
complications associated with sickle cell [...] include recurrent urinary tract infections, chronic kidney disease, and renal medullary carcinoma
trait
52
most common pathogen in SCD-associated osteomyelitis in the US/Europe
S. typhi (S. aureus is most common in Africa/ME)
53
Infarction of trabecular bone is characteristic of [...]
avascular necrosis (subchondral lucency secondary to microfractures, sclerosis, joint space narrowing on Xray)
54
characterized by a moth-eaten appearance of the bones on x-ray
dactylitis
55
Microhematuria and macrohematuria can occur in SCD due to sickling of RBCs in the [...]
renal papilla
56
Hydroxyurea inhibits ribonucleotide reductase, which inhibits DNA replication and causes cell cycle arrest during the [...] phase.
S
57
Hydroxyurea inhibits [...], which inhibits DNA replication and causes cell cycle arrest during the S phase.
ribonucelotide reductase
58
hydroxyurea can also be used to treat [...] disorders due to inhibition of DNA synthesis
myeloproliferative
59
mnemonic for remembering how far hemoglobin types travel on electrophoresis
A Fat Santa Claus (A travels the farthest, C travels the least)
60
highly specific antibody markers for SLE [2]
Anti-Smith anti-ds DNA (correlated to lupus nephritis)
61
[...] is a nonspecific screening test for syphilis that detects anticardiolipin antibodies
rapid plasma reagin (RPR)
62
patients with antiphospholipid syndrome will have false [...] testing for infection with Treponema pallidum
positive (due to anticardiolipin antibodies)
63
Medications that can act as haptens (e.g., allopurinol, cephalosporins, penicillin) can precipitate a [...] reaction
serum sickness like
64
fever, rash on flexor surfaces with central clearing, arthralgia, and lymphadenopathy occuring 1-3 weeks after starting antibiotics
serum sickness like reaction (vs erythema multiforme: rash has dark center, and starts on extensor surfaces like back of hands/top of feet)
65
target lesions with dusky center on back of hands/feet
erythema multiforme
66
erythema multiforme is a type [...] HSR
IV
67
decrease prostaglandin synthesis by reversibly inhibiting the enzymes COX-1 and COX-2
NSAIDs

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