Kawasaki Disease

Objectives:

1. Recognize the clinical features associated with classic and incomplete Kawasaki Disease (KD).
2. Formulate a differential diagnosis for patients with suspected KD.
3. Understand the evaluation and management of classic and incomplete KD.
4. Recognize the complications of KD and understand the need for cardiac monitoring and follow-up.

Articles:

• For a comprehensive overview of KD presentation, evaluation, treatment, and complications: 2018 Review Article
• Review the 2017 AHA Guidelines for the latest, evidence-based recommendations in KD diagnosis, treatment, and long-term management. 

Additional Resources:

• Explore this CHOP algorithm on the evaluation and treatment of KD. Don’t miss the embedded hyperlinks, which contain key clinical information, like IVIG dosing and side effects and discharge instructions!
• For audiovisual learners, OPENPediatrics has two great videos: Kawasaki Overview (10m) and Developments in Diagnosis and Management with Dr. Jane Newburger (50m)   
• Check out the Cribsiders podcast Episode #16 Kawasaki Disease with Recrudescent Guest Dr. Tremoulet 

Self-Assessment:

1. A 2-year-old African American boy presents to your clinic for evaluation of fever. His mother states that he has had fever up to 39°C that started 5 days ago and his eyes and lips are red. The boy had 1 episode of vomiting yesterday and 1 watery stool today. He is eating and drinking less than usual, but urinating normally. The boy has a sibling that is currently well. On physical examination, the boy is irritable. His temperature is 39.2°C, heart rate is 120 beats/min, and his blood pressure is 80/50 mm Hg. He has bilateral, nonpurulent conjunctivitis. His lips and tongue are red with hypertrophied papillae. He has a morbilliform rash on his chest. The dorsum of his hands and feet appear edematous. The remainder of his physical examination is unremarkable. You order laboratory studies. His erythrocyte sedimentation rate is 40 mm/h and his white blood cell count is 15,200/µL (15.2 x 10⁹ /L). His other laboratory results are normal. Of the following, the MOST concerning feature of the boy’s presentation that warrants further evaluation is his:

1. duration of fever
2. erythrocyte sedimentation rate
3. tachycardia
4. vomiting and diarrhea
5. white blood cell count

2. A 3-year-old boy develops a rash and fever. He has been febrile for 7 days, in association with decreased appetite and energy. He continues to drink well and has normal urine output. A red rash has been present for a few days, and this morning the skin on his fingers started to peel. The boy’s heart rate is 130 beats/min, respiratory rate is 30 breaths/min, blood pressure is 100/60 mm Hg, and pulse oximetry is 99% on room air. He is irritable but consolable. His conjunctiva are injected without exudate (Item Q107A). His tongue looks like a strawberry, and his hands and feet are slightly swollen with peeling skin on his fingers (Item Q107B). The child is admitted to the local hospital. Of the following, the BEST next step in management is to administer:

1. aspirin orally and immunoglobulin intravenously
2. aspirin orally and methylprednisolone intravenously
3. immunoglobulin and methylprednisolone intravenously
4. oxacillin and methylprednisolone intravenously

3. A 3-year-old girl has had fever for 6 days. Her father states that for the past 3 days her temperature has been at least 38.9°C, and it is not improving. He indicates that her eyes are red and she has a rash. He says she has been very cranky and that her appetite is decreased. He reports no vomiting, diarrhea, dysuria, cough, or trouble breathing. Her immunizations are up-to-date. The family was on vacation at a crowded amusement park for the first 3 days of her illness. She was seen at an urgent care center near the park where no etiology for her fever was found and no testing was done. Her temperature is now 38.9°C. She is irritable but interactive and cooperates with the examination. She has injected conjunctiva bilaterally without exudate, erythematous and cracked lips, and a polymorphous rash on her trunk and legs. The remainder of the physical examination findings are normal. Of the following, the MOST appropriate next steps in the diagnostic approach for this child include:

1. chest radiography, complete blood cell count with differential, blood culture, and urine culture
2. complete blood cell count with differential, comprehensive metabolic panel, C-reactive protein level, and urinalysis
3. nasopharyngeal swab for influenza A and B, rapid streptococcal antigen test, and heterophile antibody test
4. serum measles IgM antibody, complete blood cell count with differential, and nasopharyngeal swab for viral culture

4. A 6-month-old uncircumcised male infant presents to the emergency department with a 7-day history of fever, with a temperature of up to 39.2°C. Five days ago, he was seen at urgent care and diagnosed with a “viral illness”. Two days later, he was seen by his pediatrician, who obtained screening labs and performed a suprapubic aspiration for urine. The CBC and urinalysis were normal, and the blood and urine cultures were negative. On physical examination, the infant is alert, but very fussy. He has bilateral nonpurulent conjunctival injection and a scarlatiniform rash on his trunk. His heart rate is 160 beats/min and his blood pressure is 80/55 mm Hg. His parents do not consent to lumbar puncture or urine catheterization, but a venous blood sample is obtained and a bagged urine specimen was sent for urinalysis. Laboratory results are as follows:

• White blood cells, 10,500/µL (10.5 x 10⁹ /L) with 65% neutrophils, 25% lymphocytes, 10% atypical lymphocytes
• Platelets, 452,000 x 10³/µL (452 x 10⁹ /L)
• Erythrocyte sedimentation rate, 60 mm/h
• C-reactive protein, 4.5 mg/L
• Urinalysis shows 12 WBC/hpf
• Alanine aminotransferase, 20 U/L
• Aspartate aminotransferase, 25 U/L

All of the following features support the diagnosis of “incomplete” KD, EXCEPT:

1. Echocardiography showing an ejection fraction of 65%
2. Alanine aminotransferase, 20 U/L
3. Erythrocyte sedimentation rate, 60 mm/h
4. Infant with unexplained fever for ≥7 days
5. Urinalysis with 12 WBC/hpf

5. A 15-month-old girl with a history of epilepsy treated with phenobarbital is being evaluated for a rash. For the past several days she has had a low-grade fever, decreased energy and appetite, and an increase in her baseline seizure frequency. Her eyes have been slightly red and itchy, and she developed several small sores inside her mouth the day before the visit. This morning, she awoke with a widespread, red rash. She is fussy and refusing to eat or drink. She had 1 wet diaper overnight, but has not voided yet today. She appears ill. She has a temperature of 38.4°C, heart rate of 185 beats/min, and respiratory rate of 16 breaths/min. She has bilateral nonpurulent conjunctival injection, cracking of her lips, multiple shallow ulcerations on her buccal and gingival mucosa, and a diffuse rash (Item Q55). Of the following, the intervention MOST highly associated with an improved clinical outcome for this patient is prompt:

1. administration of broad-spectrum antibiotics
2. administration of high-dose corticosteroids
3. administration of intravenous immunoglobulin
4. discontinuation of phenobarbital

6. A 10-month-old Asian American male infant is brought to the office with 6 days of fever and a new rash. His mother states that he has been difficult to feed for 2 days because he is so irritable. He has had 2 wet diapers in the last 24 hours. Vital signs show a temperature of 39.5°C rectally, respiratory rate of 30 breaths/min, heart rate of 160 beats/min, and a blood pressure of 90/65 mm Hg. Physical examination shows an alert, but very fussy infant with dry, cracked lips, erythematous hands and feet, unilateral cervical lymphadenopathy, and nonexudative bulbar conjunctivitis. There is an erythematous maculopapular rash on his trunk and arms. Laboratory results are notable for white blood cells in the urine, elevated ESR, anemia, and elevated ALT. The patient is admitted to the hospitalist service. Echocardiography shows bilateral coronary artery dilatation. High-dose aspirin and intravenous immunoglobulin are administered. Over the next 48 hours, he remains febrile but his oral intake improves. Of the following, the BEST next step in management is:

1. administration of broad-spectrum antibiotics
2. administration of intravenous immunoglobulin
3. discharge home on low-dose aspirin with close PCP and cardiology follow-up
4. discharge on high-dose aspirin with close PCP and cardiology follow-up

7. A 4-year-old girl was admitted to the pediatric inpatient floor from the emergency department for a constellation of symptoms, including a high fever for six days in conjunction with dry, cracked lips, erythematous hands and feet, unilateral cervical lymphadenopathy, and nonexudative bulbar conjunctivitis. Her fever resolved with the standard treatment and no vascular changes were seen on echocardiography. She has been fever free for 24 hours and is now ready for discharge. Of the following, the MOST appropriate discharge recommendation for this patient is:

1. Return to the emergency department if her fever returns within 12 hours
2. Continue high-dose aspirin for 6 weeks
3. Delay live virus vaccines for 11 months
4. Follow-up with cardiology in 6 weeks for repeat echocardiography

8. A 2-year-old generally healthy boy comes into the Emergency Department with a chief complaint of fever, rash and lethargy.  This is his fourth day of fever, which has been spiking as high as 40°C between doses of acetaminophen.  He was seen yesterday by his primary care physician who prescribed amoxicillin after a physical exam revealed a possible otitis media.  This morning, he developed a rash and he has been lethargic.  His lips have been dry and cracked but the parents attributed this to his decreased PO intake.  He has not produced a wet diaper today.  On exam, he is febrile to 39°C, with HR 165, RR 24, BP 70/40, O2 Sat 98% on room air.  He appears lethargic and irritable.  He has non-purulent, injected conjunctiva bilaterally.  His tympanic membranes appear red with some retained fluid.  His lips are red, dry and cracked.  Oropharynx is dry, without oral or pharyngeal lesions.  He has prominent unilateral lymphadenopathy.  His chest is clear to auscultation, but he is tachypneic.  He is tachycardic with a regular rhythm, and soft systolic murmur is heard at the left lower sternal border.  Abdominal exam is benign.  He has 2+ distal pulses, and capillary refill is about 3 seconds.  His hands and feet appear edematous and erythematous.  He has a diffuse morbilliform rash over his abdomen and back.  Of the following, what is the MOST appropriate management strategy?

1. Order an echocardiogram and begin treatment for Kawasaki disease if there is coronary dilation.
2. Draw blood cultures and begin intravenous dopamine infusion.
3. Obtain blood cultures, bladder catheterization for urinalysis/culture, and lumbar puncture.
4. Place an IV, administer 10 cc/kg of isotonic crystalloid, and order 2 g/kg IVIG.

Find the answers here