#cardiology
see: [RCH Kawasaki disease](https://www.rch.org.au/clinicalguide/guideline_index/Kawasaki_disease/)
see also: [[paediatric sepsis]], [[Febrile child]]
- **Autoimmune vasculitis** of small and medium blood vessels
- unknown cause
- peak age 1-2 years; 80% < 5 years
- Usually 6 mo to 5 years, but can also be in infants and older children
- 2nd most common childhood vasculitis after [[Henoch-Schonlein Purpura|HSP]]
- ==most common cause of acquired heart disease in children== in high income countries
- ==Single dose **IVIG** 2g/kg reduces coronary artery abnormalities== from 20-25% to 3-5%. Ideally given in first 10 days of illness
- Infants and adolescents with [[#incomplete Kawasaki disease]] are at risk of developing coronary artery aneurysms
>[!key points] Diagnostic criteria
>- fever > 5 days + 4/5 criteria
>1. rash
>2. oral changes (strawberry tongue)
>3. lymphadenopathy
>4. extremity changes
>5. conjunctival injection
**mnemonic**:: ROLLE
- **rash** - not itchy, measles like
- **oral** - red cracked lips, red mouth
- **limb** - swelling of hands and feet with marginated erythema of palms/soles
- periungual desquamation late finding
- **lymphadenopathy** 1.5cm or greater in neck
- **eye signs** - conjunctivitis
|**Criterion**|**Features**|
|---|---|
|Conjunctival injection <br>|Bilateral, non-exudative, painless. Often with limbic sparing (zone around the iris is clear)|
|Rash <br>|Erythematous polymorphous rash occurs in the first few days, involving trunk and extremities <br>Variable presentations, most commonly maculopapular, erythema multiforme-like or scarlatiniform <br>Bullous, vesicular, or petechial rashes are not typical in KD|
|Oral Changes <br> <br>|Strawberry tongue<br><br>Erythema, dryness, cracking and bleeding of the lips<br><br>Diffuse oropharyngeal erythema <br>Exudates are not typical of KD|
|Extremity changes <br>|Hyperaemia and painful oedema of hands and feet that progresses to desquamation from the second week of illness|
|Lymphadenopathy <br>|Cervical, most commonly unilateral, tender. At least one node >1.5cm. Less common feature and seen in older children|
# DDx
- [[measles]] or other viral exanthem
- saleded skin syndrome (staph)
- scarlet fever (strep)
- steven's johnson syndrome
- juvenile rheumatoid arthritis
- drug reaction/erythema multiforme
# Complications
- coronary artery aneurysms
- myocardial infarction
- arrhythmias
- cardiac failure
- myocarditis/pericarditis
- pericardial effusions
- valve regurgitation (MR > AR, but aortic root dilation is common)
- shock - with consumptive coagulopathy
- mild GI manifestations common (gallbladder hydrops, cholestasis, paralytic ileus)
# management
- **no definitive blood test**, but do all the things to screen for sepsis:
- bloods + CRP/ESR
- blood culture
- store serum
- urinalysis
- COVID swab
- ECG
- Treatment below
- paeds consultation
# Treatment
> [!Treatment]
> 1. IVIG 2g/kg single IV infusion
> 2. Aspirin 3-5mg/kg daily until normal ECHO on follow up
> 3. +/- steroids -- consider in high risk groups <12 months, asian, bloods abnormal, coronary involvement
# incomplete Kawasaki disease
![[Pasted image 20230823004301.png]]
# Related Questions
## conjunctivitis
- [x] 1Q: [Conjunctivitis](x-devonthink-item://D6654B01-83AA-4553-94F0-791FD96A636D?page=21) -- [Answer](x-devonthink-item://00427DF6-6D28-4FEB-A0CA-DF96DBBBCE97?page=11)
## erythema multiforme
- [ ] 73Q: [18 month old with rash](x-devonthink-item://09493372-578D-4C97-972A-EEC617B38B53?page=10) -- [Answer](x-devonthink-item://A0D348CE-FCD4-4ECD-BE21-6CA73F6DE8CD?page=4)
- [ ] 74Q: [Rash](x-devonthink-item://5DC0999B-D537-4002-86AF-FD7B54B45E2E?page=23) -- [Answer](x-devonthink-item://406AF611-5CD4-4B3B-9795-327E8F4E3626?page=8)
- [ ] 75Q: [Target Lesions](x-devonthink-item://A8F2D7A0-9A26-4012-AF42-19D640D674B4?page=10) -- [Answer](x-devonthink-item://A10DE51E-92FA-42D1-8AA0-7AE68C2FA743?page=5) -- [prop](x-devonthink-item://B864660E-6598-4555-ACA9-B87F41ED4C3A?page=3)
- [ ] 76Q: [Erythema Multiforme](x-devonthink-item://EE0B8625-5F19-46CF-8208-56D79DC48BC5?page=18) -- [Answer](x-devonthink-item://6F751245-A36C-447A-8AE7-599AD5871C71?page=17) -- [prop](x-devonthink-item://308BBEF4-A83E-4972-8F63-9249898FC8E8?page=12)
## kawasaki disease
- [x] 80Q: [18 month old with fever and rash](x-devonthink-item://5DD7C01C-AB9B-4F0E-9CC4-266700BD94E8?page=12) -- [Answer](x-devonthink-item://7CE81625-E4A6-40C1-BAEB-7E44C75B1E60?page=14)
- [x] 81Q: [Kawasaki Disease](x-devonthink-item://84CE632D-3DD4-4F41-A900-23F41BE57287?page=1) -- [Answer](x-devonthink-item://D25C2F83-6C30-4F45-8B5B-6E7E57BD24A3?page=19)
- [x] 82Q: [Unwell Child](x-devonthink-item://C88FD92C-E0CB-48A1-8D73-F20489FA4E6C?page=15) -- [Answer](x-devonthink-item://98D17FA0-225B-4E94-B21C-4E36D5C76A7C?page=46)
## lymphadenopathy
- [ ] 83Q: [Question](x-devonthink-item://1EA9311E-0B9E-49F7-8D6E-4C4187A838C4?page=55) -- [Answer](x-devonthink-item://B1CB2E8F-5D04-49EE-8274-043871389D28?page=35)
## measles
- [ ] 84Q: [Measles](x-devonthink-item://1A2C485F-D4AD-4821-AFF2-452BA753717F?page=52) -- [Answer](x-devonthink-item://FD716379-1A77-4B5B-B257-1154995ECA6E?page=33) -- [prop](x-devonthink-item://1A2C485F-D4AD-4821-AFF2-452BA753717F?page=66)
- [ ] 85Q: [Unwell Child with Rash](x-devonthink-item://73409C77-B2FA-4E0A-AEB3-5EB284457F0C?page=55) -- [Answer](x-devonthink-item://5A848952-80E3-4184-B553-368412A69917?page=36) -- [prop](x-devonthink-item://2A1F4A99-92AE-4C8E-B325-AE448BD46AC6?page=13)
## petechial rash
- [ ] 87Q: [Child with fever and rash](x-devonthink-item://4BE7EDE1-1843-4BA0-B8D2-0DCEF50784D4?page=17) -- [Answer](x-devonthink-item://15F8F701-8EC8-4F9A-8DEC-5220C8561C8A?page=13) -- [prop](x-devonthink-item://281EC7A9-8E5A-461A-AA34-3FF490AA0EC2?page=7)
- [x] 88Q: [Petechial rash](x-devonthink-item://B9C99BB4-DAF8-4D15-BBD3-40E82B279902?page=6) -- [Answer](x-devonthink-item://DF848F67-27AB-450A-988B-159784B72957?page=6)
- [ ] 89Q: [Teenager with Rash](x-devonthink-item://7061D1B4-0AB9-4963-B3B0-23BDD975B2CD?page=16) -- [Answer](x-devonthink-item://3F30C77E-E23E-4200-89FE-48A41618E0C2?page=11)
## rash
- [x] DUPLICATE Q: [18 month old with rash](x-devonthink-item://09493372-578D-4C97-972A-EEC617B38B53?page=10) -- [Answer](x-devonthink-item://A0D348CE-FCD4-4ECD-BE21-6CA73F6DE8CD?page=4)
- [ ] 90Q: [Painful rash](x-devonthink-item://80040649-2DDB-4266-9A7C-4DE4E6DD4AE6?page=9) -- [Answer](x-devonthink-item://C96EDD2F-137A-43E5-80EE-5F42C5971D55?page=13) -- [prop](x-devonthink-item://51B63B5B-D684-4BF3-8B62-95FCA5EF7503?page=11)
- [x] DUPLICATE Q: [Measles](x-devonthink-item://1A2C485F-D4AD-4821-AFF2-452BA753717F?page=52) -- [Answer](x-devonthink-item://FD716379-1A77-4B5B-B257-1154995ECA6E?page=33)
- [ ] 91Q: [Rash](x-devonthink-item://F0498813-9350-484C-AD5B-6FF7C3AE9015?page=12) -- [Answer](x-devonthink-item://A491A3F6-FD6D-492F-BCBE-7F7BAE101EDF?page=9)
- [x] DUPLICATE Q: [Rash](x-devonthink-item://5DC0999B-D537-4002-86AF-FD7B54B45E2E?page=23) -- [Answer](x-devonthink-item://406AF611-5CD4-4B3B-9795-327E8F4E3626?page=8)
- [ ] 92Q: [Child with Rash](x-devonthink-item://7FCD3940-4BB4-45FE-86A6-E5707E82D5B5?page=25) -- [Answer](x-devonthink-item://3263A68A-96A6-43EC-985B-43260C3509BF?page=8)
- [x] DUPLICATE Q: [Kawasaki Disease](x-devonthink-item://84CE632D-3DD4-4F41-A900-23F41BE57287?page=1) -- [Answer](x-devonthink-item://D25C2F83-6C30-4F45-8B5B-6E7E57BD24A3?page=19)
- [x] DUPLICATE Q: [Erythema Multiforme](x-devonthink-item://EE0B8625-5F19-46CF-8208-56D79DC48BC5?page=18) -- [Answer](x-devonthink-item://6F751245-A36C-447A-8AE7-599AD5871C71?page=17)
- [ ] 93Q: [Child with Rash](x-devonthink-item://D6654B01-83AA-4553-94F0-791FD96A636D?page=15) -- [Answer](x-devonthink-item://00427DF6-6D28-4FEB-A0CA-DF96DBBBCE97?page=8) -- [prop](x-devonthink-item://AB9BDA6D-9CA8-4E73-9D15-B6105225A1B4?page=4)
- [x] DUPLICATE Q: [Unwell Child with Rash](x-devonthink-item://73409C77-B2FA-4E0A-AEB3-5EB284457F0C?page=55) -- [Answer](x-devonthink-item://5A848952-80E3-4184-B553-368412A69917?page=36)
- [ ] 94Q: [Unwell Infant with Rash](x-devonthink-item://8993893C-07A9-47B0-A68B-F8ED4C37C83D?page=16) -- [Answer](x-devonthink-item://C6A8980F-CAE5-458C-AC1E-FEBE553FDDE2?page=12) -- [prop](x-devonthink-item://3124817F-4CDF-46FB-993B-1501587D1E75?page=5)
## unwell infant
- [ ] 98Q: [Unwell infant](x-devonthink-item://85167CB5-A7B5-4BF3-9BC7-AC46D5538A42?page=10) -- [Answer](x-devonthink-item://5B03E66C-E043-4EB7-A5F6-7389CB927BD7?page=14) -- [prop](x-devonthink-item://D0A460D5-938B-4002-A684-EFD9189B08C1?page=12)