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Vasculitis > Ch. 35 KD > Flashcards

Ch. 35 KD Flashcards

1
Q

MC clinical feature of KD

A

Polymorphous rash, primarily truncal, nonvesicular

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2
Q

Least common clinical feature of KD

A

CLAD with at least one node >1.5cm

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3
Q

In the presence of these 2 criteria, fewer than 4 criteria suffice to make the diagnosis of KD

A

Fever and coronary artery changes

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4
Q

Incomplete KD

A

Fever of at least 5 days + 2 or 3 compatible criteria OR infants with fever of at least 7 days without other explanation + compatible lab tests

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5
Q

Lab tests that support incomplete KD

A

CRP at least 3mg/dL and/or ESR at least 40mm/hr + 3 or more of the ff:
1. Anemia for age
2. PC ≥450,000 after 7th day of fever
3. Alb <3 g/dL
4. Elevated ALT
5. WBC ≥15,000
6. Urine ≥10 WBC/hpf
OR (+) Echo

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6
Q

T/F Older patients are least likely to meet the classic criteria for KD

A

F, youngest patients

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7
Q

T/F Younger patients have the highest risk of developing coronary artery abnormalities

A

T

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8
Q

This age group has the highest risk for developing giant coronary aneurysms hence echo assessment is recommended if with fever for at least 7 days without other explanation

A

Infants 6 months or younger

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9
Q

Countries with highest and 2nd highest incidence of KD

A
  1. Japan 2. South Korea
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10
Q

77% of Kd consists of children in this age group

A

<5 years

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11
Q

Gender predilection of KD

A

Males > females

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12
Q

Untreated, the clinical signs of KD subside after an average of ___

A

12 days

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13
Q

T/F During the subacute phase of KD, inflamm markers are still markedly elevated

A

T

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14
Q

T/F During the subacute phase, pericarditis, abdominal pain, ascites, and hydrops of the gallbladder may occur

A

T

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15
Q

Hallmark of KD

A

Fever exceeding 40C, remaining above 38.5C for most of the acute phase

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16
Q

T/F Limbal sparing is required to qualify conjunctivitis as a criteria in KD

A

F

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17
Q

T/F Patients with KD may present with photophobia

A

T, consequence of anterior uveitis, BUT not a criterion

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18
Q

T/F KD rash may present as psoriasiform rash

A

T

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19
Q

Least common KD feature

A

Anterior CLAD

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20
Q

T/F Diffuse LAD and splenomegaly is highly suspicious of KD

A

F, should raise suspicions of a viral or other illness

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21
Q

Useful in making a retrospective diagnosis of KD

A

Periungual peeling (2-3w after fever onset)
Transverse grooves across the nails (Beau’s lines, 4-6w after fever onset)

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22
Q

Tachycardia not commensurate with fever in KD is suspicious of

A

KD myocarditis

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23
Q

T/F KD myocarditis tends to respond quickly to IVIg treatment

A

T

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24
Q

T/F Long-term abnormalities in cardiac contractility is very uncommon in KD

A

T

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25
Q

T/F IVIg resistance has been shown to be more common in patients with KDSS

A

T

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26
Q

Most significant and characteristic complication of KD

A

Development of coronary artery dilation

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27
Q

T/F Coronary artery dilation is common even in the 1st week of KD

A

T

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28
Q

JMH criteria to define coronary artery abnormality in KD

A

> 3mm in children <5y
4mm in children >5y

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29
Q

AHA coronary artery aneurysm classification

A

Small aneurysm: z=2.5 to <5
Medium: z=5 to <10 and absolute dimension <8mm
Large or giant: z=10 or greater or absolute dimension ≥8mm

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30
Q

Leading cause of acquired heart disease among children in the developed world

A

KD

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31
Q

MCC of morbidity and mortality in patients with KD

A

Large/giant coronary aneurysms

32
Q

MCC of mortality in KD

A

Coronary thrombosis (leading to ischemia, MI, and sudden death)

33
Q

T/F Visceral arteries are usually spared in KD

A

T

34
Q

Group of arteries that are also involved in KD besides the coronaries

A

Muscular arteries

35
Q

Most consistent clinical observation in patients with KD

A

Extreme irritability

36
Q

Characteristics of arthritis in KD

A

Synovial fluid: Inflammatory with neutrophil predom
Oligoarticular (MC to least: knees, ankles, wrists, elbows, hips)
Responds to IVIG and high-dose aspirin
No residua

37
Q

Phase at which arthritis is MC observed in KD

A

Subacute

38
Q

GI manifestations of KD

A

Abdominal pain, profuse watery diarrhea, hydrops of the gallbladder

39
Q

KD vs other viral illnesses

A

Other viral illnesses (e.g. measles, EBV, adenovirus) typically have less evidence of systemic inflamm and generally lack extremity changes of KD

40
Q

KD vs toxin-mediated infection (scarlet, TSS, SSSS)

A

Toxin-mediated infections require a focus of infection

41
Q

KD vs SJS

A

SJS: Exudative conjunctivitis, mucosal surface ulcerations

42
Q

KD vs PAN

A

PAN: Htn, post-prandial abdominal pain
KD: Mucocutaneous changes

43
Q

Pathology of KD

A

Necrotizing vasculitis of the medium-sized muscular arteries

44
Q

Inflammatory Infiltrates in KD

A

Macrophages and IgA-secreting plasma cells

45
Q

3 phases of arteriopathy in KD

A

1: neutrophilic necrotizing arteritis that begins in the endothelium resulting in a saccular aneurysm
2: subacute/chronic vasculitis driven by lymphocytes, plasma cells, and eosinophils
3: smooth muscle conversion into myofibroblasts causing progressive stenosing lesions and thrombosis

46
Q

Hematologic abnormalities in KD

A

Anemia, leukocytosis with left shift, thrombocytosis

47
Q

Suspected in a patient with KD but with anemia, thrombocytopenia, and only a modest elevation in ESR

A

MAS

48
Q

CSF in KD

A

Mononuclear pleocytosis, normal glucose and protein

49
Q

Synovial fluid in Kd

A

WBC 50-300K primarily neutrophils

50
Q

Elevation of this factor indicates presence of damaged endothelium

A

vWF

51
Q

Hypotension in Kd is associated with

A

Peripheral vasodilation (warm shock), decrease LV function, and depressed EF

52
Q

Define IVIg resistance in KD

A

Fever without other explanation after 36 hrs from end of 1st IVIg

53
Q

Aspirin may be d/c in KD once lab studies return to normal unless CA abnormalities are detected. When does this usually happen?

A

Within 2 months of disease onset

54
Q

JMH coronary artery aneurysm classification

A

Small: ≤4mm internal diameter OR internal diameter <1.5x of adjacent segment in children at least 5y
Medium: >4 to <8mm internal diameter or 1.5-4x of adjacent segment in children at least 5y
Large/Giant: ≥8mm internal diameter or >4x adjacent segment in children at least 5y

55
Q

AHA warns against prescribing this drug in children requiring protection from thrombosis because it antagonizes antiplatelet effect of low-dose aspirin

A

Ibuprofen

56
Q

IVIg reduces frequency of CA abnormalities from 20-25% to 5% as long as the ff are met

A

Dose 2g/kg, given within 10 days of illness

57
Q

Infusion reactions that occasionally accompany IVIg infusion

A

Fever and hypotension

58
Q

Fever and hypotension from IVIg are best managed by

A

Slowing down rate and administration of diphen

59
Q

Headache can occur up to ___ from administration of IVIg

A

72 hr

60
Q

IVIg-related hemolysis can be seen in what population of patients with KD

A

15% of non-O blood types

61
Q

Why are patients with KD at high risk for IVIg-related hemolysis

A

Risk factor for IVIg-related hemolysis includes administration of high-dose IVIg in an inflammatory disease

62
Q

T/F IVIG-associated hemolysis can be severe and require transfusion

A

T

63
Q

T/F Risk for IVIg-related hemolysis is decreased with subsequent doses of IVIg

A

F, frequently seen when a second dose of IVIg is given for persistent fever in non-O blood type patients

64
Q

AHA cautiously recommends IVIG beyond 10th day of illness if patients has

A

1) “Manifestations of continuous inflamm”: Fever, mucocutaneous signs
2) Elevated ESR/CRP
3) Abn echo

65
Q

DEspite IVIg, ___ % of children and ___ % of infants<6m develop coronary art abn in KD

A

> 25, >50;
~5% by JMH criteria, more by z score criteria

66
Q

Very strong predictor of subsequent aneurysm development in KD

A

Z ≥2.5 of the LAD and or proximal RCA

67
Q

Most definitive trial re: CS combined with IVIg in primary treatment of KD

A

RAISE trial by Kobayashi

68
Q

Kobayashi score considered high risk for IVIg resistance

A

≥5

69
Q

Level of this cytokine is markedly elevated in KD, rationalizing rescue therapy with biologic

A

TNF-a, Infliximab

70
Q

Can be given for children with KD who failed all available medical interventions

A

Plasmapheresis

71
Q

REsistance to IVIg occurs in ___% of children with KD

A

10-20%

72
Q

T/F IVIg resistance = higher risk for cor artery aneurysm

A

T

73
Q

Treatment protocols for IVIg resistance

A

1) 2nd dose of IVIg at 2g/kg
2) MPPT at 30mkday x 3 days OR PRed at 2mkday
3) Inflix single dose at 5-10mkday

74
Q

Recommended echo for KD

A

At diagnosis
2 weeks after onset

75
Q

It is highly unlikely that new onset coronary dilation will present by this time in KD if initial echo did not show dilation

A

6 weeks

76
Q

T/F single small- to medium-sized aneurysms usually resolve in KD

A

Y

Vasculitis (8 decks)

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