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Capstone Test 1 > Leg Pain Cases > Flashcards

Leg Pain Cases Flashcards

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
Q

painless gross hematuria in a patient with sickle cell trait may indicate

A

renal papillary necrosis

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26
Q

inhibits xanthine oxidase, preventing the formation of uric acid

A

allopurinol

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27
Q

what type of crystals precipitate in gout?

A

monosodium urate

28
Q

in gout, crystals are […] shaped

29
Q

in gout, crystals are […] birefringent

A

negatively

30
Q

in pseudogout, crystals are […] shaped

31
Q

in pseudogout, crystals are […] birefringent

A

weakly positive

32
Q

what type of crystals precipitate in pseudogout?

A

calcium pyrophosphate

33
Q

treatment of acute gout [3]

A

NSAIDs
colchicine
corticosteroids

34
Q

treatment of chronic gout [2]

A

allopurinol
febuxostat

35
Q

mechanism of action of colchicine

A

prevents microtubule assembly –> disrupts neutrophil chemotaxis

36
Q

mechanism of action of probenecid

A

URAT inhibitor

prevents reabsorption of uric acid from PCT –> increases excretion

37
Q

[…] syndrome is characterized by a hypercoaguable state antibodies such as anti-lipid coagulant and anti-cardiolipin

A

anti-phospholipid syndrome

38
Q

history red flags for anti-phospholipid syndrome in SLE

A

DVT
hepatic vein thrombosis
stroke
recurrent pregnancy loss

39
Q

what serum antibody is sensitive, but not specific for SLE?

40
Q

what serum antibodies are highly specific for SLE? [2]

A

Anti-dsDNA
anti-Sm

41
Q

deficiency of […] complement proteins is associated with SLE

A

early

C1q, C4, C2

42
Q

deficiency of early complement proteins in SLE causes decreased clearance of […]

A

immune complexes

43
Q

describe some characteristics of a classic presentation of SLE

A

female of reproductive age
facial rash
joint pain
fever
oral ulcers
photosensitivity

44
Q

what would you expect to see on skin biopsy in SLE?

A

-DEJ “smudging”
-holes in the basal layer “vacuolization”
-melanin dropout
-dead reds

45
Q

Mesangial and/or subendothelial deposition of immune complexes (C3 and C1q) → expansion and thickening of mesangium, capillary walls, and/or glomerular basement membrane

A

lupus nephritis

46
Q

most common nephritis syndrome associated with SLE

A

diffuse proliferative glomerulonephritis

aka lupus nephritis

47
Q

SLE is a type […] HSR

A

III

antigen-antibody complexes damage tissues

48
Q

MOA inhibits microtubule polymerization by binding to the cytoskeleton

A

colchine

(impairs phagocytosis, chemotaxis, and migration of neutrophils)

49
Q

side effects of colchicine

A

GI upset
myelosuppression
hair loss

50
Q

Sickle cell trait causes […] secondary to transient sickling of RBCs in the renal capillaries

A

renal papillary necrosis

51
Q

complications associated with sickle cell […] include recurrent urinary tract infections, chronic kidney disease, and renal medullary carcinoma

52
Q

most common pathogen in SCD-associated osteomyelitis in the US/Europe

A

S. typhi

(S. aureus is most common in Africa/ME)

53
Q

Infarction of trabecular bone is characteristic of […]

A

avascular necrosis

(subchondral lucency secondary to microfractures, sclerosis, joint space narrowing on Xray)

54
Q

characterized by a moth-eaten appearance of the bones on x-ray

A

dactylitis

55
Q

Microhematuria and macrohematuria can occur in SCD due to sickling of RBCs in the […]

A

renal papilla

56
Q

Hydroxyurea inhibits ribonucleotide reductase, which inhibits DNA replication and causes cell cycle arrest during the […] phase.

57
Q

Hydroxyurea inhibits […], which inhibits DNA replication and causes cell cycle arrest during the S phase.

A

ribonucelotide reductase

58
Q

hydroxyurea can also be used to treat […] disorders due to inhibition of DNA synthesis

A

myeloproliferative

59
Q

mnemonic for remembering how far hemoglobin types travel on electrophoresis

A

A
Fat
Santa
Claus

(A travels the farthest, C travels the least)

60
Q

highly specific antibody markers for SLE [2]

A

Anti-Smith
anti-ds DNA (correlated to lupus nephritis)

61
Q

[…] is a nonspecific screening test for syphilis that detects anticardiolipin antibodies

A

rapid plasma reagin (RPR)

62
Q

patients with antiphospholipid syndrome will have false […] testing for infection with Treponema pallidum

A

positive

(due to anticardiolipin antibodies)

63
Q

Medications that can act as haptens (e.g., allopurinol, cephalosporins, penicillin) can precipitate a […] reaction

A

serum sickness like

64
Q

fever, rash on flexor surfaces with central clearing, arthralgia, and lymphadenopathy occuring 1-3 weeks after starting antibiotics

A

serum sickness like reaction

(vs erythema multiforme: rash has dark center, and starts on extensor surfaces like back of hands/top of feet)

65
Q

target lesions with dusky center on back of hands/feet

A

erythema multiforme

66
Q

erythema multiforme is a type […] HSR

67
Q

decrease prostaglandin synthesis by reversibly inhibiting the enzymes COX-1 and COX-2

Capstone Test 1 (5 decks)

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