Kawasaki
Typical Kawasakis
5days of fever and 4 of
Incomplete (aka Atypical) Kawasakis
5 days and 2-3 features
And CRP >= 30mg/L or ESR >=40mm/hr
And positive ECHO or 3 of
Incomplete KD <6mo
7d fever and 0-1criteria
And Positive ECHO OR
Hadley's Ix panel: Hb, WC, Neut, Plt, Na, AST, ALT, Alb, CRP, ESR, Ferritin, fibrinogen, fasting triglycerides, urine micro
Kobayashi score >4 consider steroids with initial IVIG
Other risk factors for refractory... update MAS, <6mo, asian, shock, enlarges coronary arteries at presentation
MAS: hepatosplenomegaly, cytopenia, fibrinogen <1.5g/L, high ferritin
HLH = 5 of,
5days of fever and 4 of
- Bilateral bulbar conjunctival injection without exudate
- Oral mucous membrane changes, including erythema and/or fissuring of lips, strawberry tongue, and/or erythema of oropharyngeal mucosa
- Palmar or planar erythema and/or oedema of hands and/or feet (acute phase) and/or periungual desquamation (subacute phase)
- Polymorphous rash (maculopapular, diffuse erythroderma, or erythema multiforme-like)
- Cervical lymph node ≥1.5 cm in diameter
Incomplete (aka Atypical) Kawasakis
5 days and 2-3 features
And CRP >= 30mg/L or ESR >=40mm/hr
And positive ECHO or 3 of
- Anemia for age
- WBC count ≥15x10^9
- Platelet count ≥450,000 after the seventh day of fever
- Albumin ≤30 g/L
- Elevated ALT level
- ≥10 WBC/hpf on urinalysis
Incomplete KD <6mo
7d fever and 0-1criteria
And Positive ECHO OR
Hadley's Ix panel: Hb, WC, Neut, Plt, Na, AST, ALT, Alb, CRP, ESR, Ferritin, fibrinogen, fasting triglycerides, urine micro
Kobayashi score >4 consider steroids with initial IVIG
Other risk factors for refractory... update MAS, <6mo, asian, shock, enlarges coronary arteries at presentation
MAS: hepatosplenomegaly, cytopenia, fibrinogen <1.5g/L, high ferritin
HLH = 5 of,
- fever > 38.5
- splenomegaly
- (2 of Hb <90 (100 in neonate), plt <100, Neut <1
- Ferritin > 500 (>3000 more indicative)
- hypertriglyceridemia (fasting >265mg/dL) or hypofibrinogenaemia (<150mg/dL)
- hemophagocytosis in biopsy (liver/spleen/marrow/lymphnode)
- low or absent NK cell activity
- Elevated CD25
- Elevated CXCL9