15.2.2.2 Flashcards
General about kawasaki disease
- Diagnosis made in setting of acute febrile disease and certain clinical features
- Acute self-limiting vasculitis affecting small and medium arterioles
- Multisystem vasculitis
- No causative agent
- Fever not settling within 5 days treatment
- purely paediatric disease
- M>F
- seasonal
- temperate climate
Pathogenesis
- Infectious agent
- Genetically predisposed
- Inflammatory response
Three phases
- Acute
- Subacute
- Convalescent
Clinical features
- fever of at least 5 days (without treatment persists for 3-4 weeks)
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4 of following: - mucositis (tongue red + ulcers+ cracked lips)
- conjunctivitis (non exudative) -> most common
- rash (any kind except fascicular rash)
- ertrheme and oedema (extremity changes(
- cervical lymphadenopathy (unilateral)
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Differential diagnosis
- Viral infections
- Rheumatological
- Drug reactions
- Toxin mediated
Incomplete Kawasaki disease
- More common in young infants
- > 6 months and fever > 7 days
- Lab results in keeping with Classical Kawasaki disease
- only with 2/3 of other symptoms
Why is the diagnosis of Kawasaki important?
Risk of coronary aneurysms
Pathophysiology of Kawasaki coronary aneurysms
Edema of the walls with inflammatory infiltration
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Vessel weakens and looses it’s structural integrity > Dilation and aneurysm
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Heals with fibroses and stenosis > decreased blood flow with thrombosis
Groups of people with higher risk to develop aneurysms
- Male
- < 6 months, > 5 years
- Persistent fever
- Aneamia
- High CRP
- Hypoalbuminaemia
MIS-C
Def - WHO case definition of multisystem inflammatory syndrome in children (MIS-C)
All 6 criteria must be met:
1. Agelto19years
2. 2. Fever for 23 days
3. Clinical signs of multisystem involvement (at least 2 of the following):
- Rash, bilateral nonpurulent conjunctivitis, or mucocutaneous inflammation signs (oral, hands, or feet),
- Hypotension or shock,
- Cardiac dysfunction, pericarditis, valvulitis, or coronary abnormalities (including echocardiographic findings or elevated troponin/BNP),
- Evidence of coagulopathy (prolonged PT or PTT; elevated D-dimer),
- Acute gastrointestinal symptoms (diarrhea, vomiting, or abdominal pain)
4. Elevated markers of inflammation (eg, ESR, CRP, or procalcitonin)
5. No other abvious microbial cause of inflammation, Including bacterial sepsis and staphylococcal/streptococcal toxic shock syndromes
6. Evidence of SARS-CoV-2 infection (any of the following):
- Positive SARS-CoV-2 RT-PCR (reverse transcription real-time PR);
- Positive antibody test;
- Positive antigen test;
- Likely contact with an individual with COVID-19