Ch. 35 KD Flashcards
MC clinical feature of KD
Polymorphous rash, primarily truncal, nonvesicular
Least common clinical feature of KD
CLAD with at least one node >1.5cm
In the presence of these 2 criteria, fewer than 4 criteria suffice to make the diagnosis of KD
Fever and coronary artery changes
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
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
T/F Older patients are least likely to meet the classic criteria for KD
F, youngest patients
T/F Younger patients have the highest risk of developing coronary artery abnormalities
T
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
Countries with highest and 2nd highest incidence of KD
- Japan 2. South Korea
77% of Kd consists of children in this age group
<5 years
Gender predilection of KD
Males > females
Untreated, the clinical signs of KD subside after an average of ___
12 days
T/F During the subacute phase of KD, inflamm markers are still markedly elevated
T
T/F During the subacute phase, pericarditis, abdominal pain, ascites, and hydrops of the gallbladder may occur
T
Hallmark of KD
Fever exceeding 40C, remaining above 38.5C for most of the acute phase
T/F Limbal sparing is required to qualify conjunctivitis as a criteria in KD
F
T/F Patients with KD may present with photophobia
T, consequence of anterior uveitis, BUT not a criterion
T/F KD rash may present as psoriasiform rash
T
Least common KD feature
Anterior CLAD
T/F Diffuse LAD and splenomegaly is highly suspicious of KD
F, should raise suspicions of a viral or other illness
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)
Tachycardia not commensurate with fever in KD is suspicious of
KD myocarditis
T/F KD myocarditis tends to respond quickly to IVIg treatment
T
T/F Long-term abnormalities in cardiac contractility is very uncommon in KD
T
T/F IVIg resistance has been shown to be more common in patients with KDSS
T
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
T
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
KD
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
T
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
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
T
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
Y
