Ch. 35 KD Flashcards by Butterfly Rash

MC clinical feature of KD

Polymorphous rash, primarily truncal, nonvesicular

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Least common clinical feature of KD

CLAD with at least one node >1.5cm

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

Fever and coronary artery changes

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Incomplete KD

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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Lab tests that support incomplete KD

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

F, youngest patients

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

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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

Infants 6 months or younger

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Countries with highest and 2nd highest incidence of KD

Japan 2. South Korea

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77% of Kd consists of children in this age group

<5 years

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Gender predilection of KD

Males > females

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

12 days

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

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

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Hallmark of KD

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

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

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T/F Patients with KD may present with photophobia

T, consequence of anterior uveitis, BUT not a criterion

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T/F KD rash may present as psoriasiform rash

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Least common KD feature

Anterior CLAD

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

F, should raise suspicions of a viral or other illness

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Useful in making a retrospective diagnosis of KD

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

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Tachycardia not commensurate with fever in KD is suspicious of

KD myocarditis

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

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

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

Most significant and characteristic complication of KD

Development of coronary artery dilation

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

JMH criteria to define coronary artery abnormality in KD

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

AHA coronary artery aneurysm classification

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

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

MCC of morbidity and mortality in patients with KD

Large/giant coronary aneurysms

MCC of mortality in KD

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

T/F Visceral arteries are usually spared in KD

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

Muscular arteries

Most consistent clinical observation in patients with KD

Extreme irritability

Characteristics of arthritis in KD

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

Phase at which arthritis is MC observed in KD

Subacute

GI manifestations of KD

Abdominal pain, profuse watery diarrhea, hydrops of the gallbladder

KD vs other viral illnesses

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

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

Toxin-mediated infections require a focus of infection

KD vs SJS

SJS: Exudative conjunctivitis, mucosal surface ulcerations

KD vs PAN

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

Pathology of KD

Necrotizing vasculitis of the medium-sized muscular arteries

Inflammatory Infiltrates in KD

Macrophages and IgA-secreting plasma cells

3 phases of arteriopathy in KD

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

Hematologic abnormalities in KD

Anemia, leukocytosis with left shift, thrombocytosis

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

MAS

CSF in KD

Mononuclear pleocytosis, normal glucose and protein

Synovial fluid in Kd

WBC 50-300K primarily neutrophils

Elevation of this factor indicates presence of damaged endothelium

vWF

Hypotension in Kd is associated with

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

Define IVIg resistance in KD

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

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

Within 2 months of disease onset

JMH coronary artery aneurysm classification

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

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

Ibuprofen

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

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

Infusion reactions that occasionally accompany IVIg infusion

Fever and hypotension

Fever and hypotension from IVIg are best managed by

Slowing down rate and administration of diphen

Headache can occur up to ___ from administration of IVIg

72 hr

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

15% of non-O blood types

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

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

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

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

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

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

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

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

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

Very strong predictor of subsequent aneurysm development in KD

Z ≥2.5 of the LAD and or proximal RCA

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

RAISE trial by Kobayashi

Kobayashi score considered high risk for IVIg resistance

≥5

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

TNF-a, Infliximab

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

Plasmapheresis

REsistance to IVIg occurs in ___% of children with KD

10-20%

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

Treatment protocols for IVIg resistance

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

Recommended echo for KD

At diagnosis 2 weeks after onset

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

6 weeks

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