Kawasaki disease: Difference between revisions

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== Background ==
==Background==
 
*Also known as: mucocutaneous lymph node syndrome
*Mucocutaneous lymph node syndrome
*[[Vasculitis]] of unknown etiology
*Vasculitis of unknown etiology
*Peaks at 18-24 months
*Peaks at 18-24 months
**Rare in <4mo, >5yr
**Rare in <4mo, >5yr
*Leading cause of acquired heart disease in children
*Leading cause of acquired heart disease in children
*Coronary aneurysm more common in incomplete than in classic KD


 
==Clinical Features==
{| cellspacing="1" cellpadding="3" border="0" bgcolor="#666666" width="100%"
[[File:1200px-Kawasaki.png|thumb|Kawasaki disease signs]]
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
*[[Fever]] that is high, abrupt. Anyone with a fever >5 days should get considered for a Kawasaki workup.
! valign="top" bgcolor="#ffffff" align="left" rowspan="0" | Classic Kawasaki Disease
*[[Rash]] often seen in perineum; accompanies onset of fever
! valign="top" bgcolor="#ffffff" align="left" rowspan="0" | Incomplete Kawasaki Disease
**Maculopapular most common; vesicles not seen
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | Fever for 5 d or more plus four of the following symptoms
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | Fever for 5 d and two to three clinical criteria of classic Kawasaki disease
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;1. Bilateral nonexudative conjunctivitis
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | ''plus''&nbsp;
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;2. Mucous membrane changes (erythema, peeling, cracking of lips, "strawberry tongue," or diffuse oropharyngeal mucosae)
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | C-reactive protein&nbsp;[[Image:]]3.0 milligrams/L and/or erythrocyte sedimentation rate&nbsp;[[Image:]]40 mm/h plus three or more of the following supplemental labs or positive echo
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" rowspan="4" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;3. Changes of the extremities (erythema or swelling of hands/feet, peeling of finger tips/toes in the convalescent stage)
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;1. Albumin <3 grams/dL
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;2. Anemia for age
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;3. Elevated alanine aminotransferase
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" rowspan="2" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;4. Platelets >450,000/mm<sup>3</sup>&nbsp;after 7 d of fever onset<br/>&nbsp;
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;4. Rash
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" rowspan="2" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;5. Cervical adenopathy (more than one node >1.5 cm unusually unilateral anterior cervical)
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;5. White blood cell count >12,000/mm<sup>3</sup><br/>&nbsp;
|- class="font12" style="font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; "
| valign="top" bgcolor="#ffffff" class="font12" align="left" style="color: rgb(51, 51, 51); font-size: 12px; line-height: 17px; margin-top: 0px; margin-bottom: 9px; margin-left: 0px; margin-right: 0px; " | &nbsp;&nbsp;6. Presence of pyuria
|}
 
 
 
 
== Diagnosis ==
 
=== CDC Definition ===
 
*Fever >5d and 4 of the following:
 
#Rash
#Cervical LAD (>1.5cm diam, usually unilat)
#Bilateral nonexudative conjunctivitis
#Oral mucosal changes
#Extremity edema/erythema/desquamation
 
=== Presentation ===
 
*Fever that is high, abrupt
*Rash often seen in perineum; accompanies onset of fever
*Cardiac complications develop early on
*Cardiac complications develop early on
**Coronary artery aneurysm development most prevalent as fever lessens
**Coronary artery aneurysm development most prevalent as fever lessens


===Associated Symptoms===
*Cardiac
**Coronary aneurysm
***Most develop during 3-4th week of illness
***May lead to MI (leading cause of death)
**[[Myocarditis]]/[[pericarditis]]
**[[Pericardial effusion]]
**LV dysfunction
**Valvular dysfunction
**[[Dysrhythmias]]
*Aseptic [[meningitis]]
*[[Urethritis]]
*[[Anemia]]
*[[RUQ pain]], large gallbladder (hydrops on US)


==Differential Diagnosis==
{{Pediatric fever DDX}}
{{Erythematous rash DDX}}


=== Associated Sx ===
==Evaluation==
===Work-Up===
*CBC
**[[Leukocytosis]]
**Elevated platelets
*Labs
*[[LFTs]]- elevated
*ESR, CRP- elevated
*[[Blood culture]]
*[[Urinalysis]]
*[[ECG]]
*[[Echocardiography|TTE]] (Coronaries, LV, valves)
**Consider if f ESR≥ 40, CRP≥ 3, or if desquamation occurs
*Red Top "Kawasaki Serum to CBR"


#Cardiac
===Evaluation===
##Coronary aneurysm
*Clinical diagnosis
###Most develop during 3-4th week of illness
{| class="wikitable"
###May lead to MI (leading cause of death)
| align="center" style="background:#f0f0f0;"|'''Classic Kawasaki Disease'''
##Myo/pericarditis
| align="left" style="background:#f0f0f0;"|Fever for 5 days or more '''plus''' four of the following symptoms
##Pericardial effusion
|-
##LV dysnfunction
| ||1. Bilateral nonexudative [[conjunctivitis]]
##Valvular dysfunction
|-
##Dysrhythmias
| ||2. Mucous membrane changes (erythema, peeling, cracking of lips, "strawberry tongue," or diffuse oropharyngeal mucosae)
#Labs
|-
##Elevated ESR/WBC/LFTs/Plts
| ||3. Changes of the extremities (erythema or swelling of hands/feet, peeling of finger tips/toes in the convalescent stage)
#Aseptic meningitis
|-
#Urethritis, Anemia
| ||4. [[Rash]]
#RUQ pain, large GB (hydrops)
|-
 
| ||5. Cervical adenopathy (more than one node >1.5 cm unusually unilateral anterior cervical)
== Work-Up ==
|-
 
| '''Incomplete Kawasaki Disease'''||Fever for 5 days '''and''' two to three clinical criteria of classic Kawasaki disease '''plus'''
#CBC/Diff/SPA/ALT/TBili
|-
#Blood Cx and UA
| ||C-reactive protein 3.0 milligrams/L and/or erythrocyte sedimentation rate 40 mm/h '''plus''' positive echocardiogram '''or''' three or more of the following:
#ECG
|-
#Echo (Coronaries, LV, Valves)
| ||1. Albumin <3 grams/dL
#Red Top "Kawasaki Serum to CBR"
|-
 
| ||2. [[Anemia]]
== Treatment ==
|-
 
| ||3. Elevated alanine aminotransferase (ALT)
#Immunoglobulin
|-
##IVIG 2gm/kg over 12hr
| ||4. Platelets >450,000/mm3 7d after fever onset
#ASA 20mg/kg/dose q6h
|-
 
| ||5. White blood cell count >12,000/mm3
== Disposition ==
|-
| ||6. Pyuria
|-
|}


#Cardiology f/u
==Management==
#Cont ASA at high dose
*[[IVIG]] 2gm/kg over 12hr
##Switch to ASA 3-5mg/kg/day once afebrile x48h
*[[Aspirin]] 20mg/kg/dose q6h


== Source ==
==Disposition==
*Admit
*Follow-up cardiac evaluation for coronary aneurysm screening


Tintinalli
==References==
<References/>


<br/>[[Category:Peds]] <br/><br/>
[[Category:Pediatrics]]

Revision as of 01:05, 15 September 2019

Background

  • Also known as: mucocutaneous lymph node syndrome
  • Vasculitis of unknown etiology
  • Peaks at 18-24 months
    • Rare in <4mo, >5yr
  • Leading cause of acquired heart disease in children
  • Coronary aneurysm more common in incomplete than in classic KD

Clinical Features

Kawasaki disease signs
  • Fever that is high, abrupt. Anyone with a fever >5 days should get considered for a Kawasaki workup.
  • Rash often seen in perineum; accompanies onset of fever
    • Maculopapular most common; vesicles not seen
  • Cardiac complications develop early on
    • Coronary artery aneurysm development most prevalent as fever lessens

Associated Symptoms

Differential Diagnosis

Pediatric fever

Erythematous rash

Evaluation

Work-Up

  • CBC
  • Labs
  • LFTs- elevated
  • ESR, CRP- elevated
  • Blood culture
  • Urinalysis
  • ECG
  • TTE (Coronaries, LV, valves)
    • Consider if f ESR≥ 40, CRP≥ 3, or if desquamation occurs
  • Red Top "Kawasaki Serum to CBR"

Evaluation

  • Clinical diagnosis
Classic Kawasaki Disease Fever for 5 days or more plus four of the following symptoms
1. Bilateral nonexudative conjunctivitis
2. Mucous membrane changes (erythema, peeling, cracking of lips, "strawberry tongue," or diffuse oropharyngeal mucosae)
3. Changes of the extremities (erythema or swelling of hands/feet, peeling of finger tips/toes in the convalescent stage)
4. Rash
5. Cervical adenopathy (more than one node >1.5 cm unusually unilateral anterior cervical)
Incomplete Kawasaki Disease Fever for 5 days and two to three clinical criteria of classic Kawasaki disease plus
C-reactive protein 3.0 milligrams/L and/or erythrocyte sedimentation rate 40 mm/h plus positive echocardiogram or three or more of the following:
1. Albumin <3 grams/dL
2. Anemia
3. Elevated alanine aminotransferase (ALT)
4. Platelets >450,000/mm3 7d after fever onset
5. White blood cell count >12,000/mm3
6. Pyuria

Management

Disposition

  • Admit
  • Follow-up cardiac evaluation for coronary aneurysm screening

References