1. Case discussion Pediatrics Reporter : Intern 周昀澤 Supervisor : CR 賴馥蘋
Date : 2016/5/3

2. Basic information Name: 邱X豪 Patient ID: 17545XXX Gender: Boy Age: 1y1m
Underlying disease: Nil
Admission date: 2016/4/23
邱國豪

3. Chief complaints
Fever on and off for about 5 days

4. Present illness Irritation before going to sleep -> BT ~ 39’C
5 days ago
Irritation before going to sleep -> BT ~ 39’C
URI symptoms: running nose and cough with whitish sputum
Peripheral extremity changes
erythema of palms or soles
edema of hands or feet
Polymorphous rashes over his body
Bilateral bulbar conjunctival injection
No periungual desquamation
2 days ago

5. Skin lesions

6. Present illness
He was brought to 麻豆新樓醫院 for persistent fever despite going to LMD
Referred to our hospital

7. Birth history
G1P1, GA: 39+4 weeks, BBW: 3176 gm, NSD, PROM(-); Apgar score:8->9
BH: 81cm = 85~97th percentile, BW: 10.4kg =85~97th percentile,
Feeding: On full diet
NO developmental delay
Vaccination: On schedule (MMR and Varicella on 2016/4/12)

8. Past history and family history
Current medications: Nil
Family history: Nil

9. Physical examinations
Conscious: Clear, irritation
Head and neck
Bilateral bulbar conjunctival injection
Injected throat (+), strawberry tongue (-), red lips
Cervical lymphadenopathy (+), around 1.6 cm, left near SCM (anterior triangle)
Heart: no murmur, regular heart beat
Limbs: Erythema on all fingers, edema for four limbs especially on hands and feet
Skin: Polymorphous blanchable rashes over trunk and four limbs, BCG redness

10. Lab data

11. Tentative diagnosis Kawasaki disease Streptococcal scarlet fever
Adenovirus infection
Toxic shock syndrome
KD is most commonly confused with infectious exanthems of childhood
Children with adenovirus typically have exudative pharyngitis and exudative conjunctivitis
Patients with scarlet fever typically have a rapid clinical response to appropriate antibiotic therapy. Such treatment for 24-48 hr with clinical reassessment generally clarifies the diagnosis. Furthermore, ocular findings are quite rare in group A streptococcal pharyngitis and may assist in the diagnosis of KD.

13. Course of admission Cardaic echo Cardaic echo Discharge IVIG 12g+9g
Aspirin 50mg

14. Cardiac ultrasonography, 4/25
Situs solitus, levocardia
No chamber enlargement
Good LV systolic function (LVEF: 66.8%)
Normal proximal coronary artery size, RCA: 0.182cm, LCA: 0.231cm
Trivial mitral regurgitation
No PDA, no coarctation
Gallbladder distention, hydrops gallbladder

15. Kawasaki disease

16. Introduction
Mucocutaneous lymph node syndrome or infantile polyarteritis nodosa
Highest incidence occurring in Asian children
Vasculitis with a predilection for the coronary arteries
20-25% develop coronary artery abnormalities of untreated children
< 5% for treated by IVIG
Nelson Textbook of Pediatrics, 20th Edition

17. Diagnostic criteria
Fever persisting at least 5 days (at 4 of 5 features)
Changes in extremities
Acute: Erythema and edema of hands and feet
Convalescent: Membranous desquamation of fingertips
Polymorphous exanthema
Bilateral, painless bulbar conjunctival injection without exudate
Changes in lips and oral cavity: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
Cervical lymphadenopathy (>1.5 cm in diameter), usually unilateral
Circulation. 2001; 103:

18. Evaluation of suspected incomplete Kawasaki disease.
Exudative conjunctivitis Exudative pharyngitis Discrete intraoral lesions
Bullous or vesicular rash Generalized adenopathy
Albumin ≤3.0 g/dL
Anemia for age
Elevation of ALT
Platelets after 7 days ≥450k
WBC ≥15k
U/A ≥10 WBC/HPF
Evaluation of suspected incomplete Kawasaki disease. (1) In the absence of gold standard for diagnosis, this algorithm cannot be evidence based but rather represents the informed opinion of the expert committee. Consultation with an expert should be sought anytime assistance is needed. (2) Infants ≤6 months old on day ≥7 of fever without other explanation should undergo laboratory testing and, if evidence of systemic inflammation is found, an echocardiogram, even if the infants have no clinical criteria. (3) Patient characteristics suggesting Kawasaki disease are listed in Table 1.
Characteristics suggesting disease other than Kawasaki disease include exudative conjunctivitis, exudative pharyngitis, discrete intraoral lesions, bullous or vesicular rash, or generalized adenopathy.
Consider alternative diagnoses (see Table 2). (4) Supplemental laboratory criteria include albumin ≤3.0 g/dL, anemia for age, elevation of alanine aminotransferase, platelets after 7 days ≥ /mm3, white blood cell count ≥15 000/mm3, and urine ≥10 white blood cells/high-power field. (5) Can treat before performing echocardiogram. (6) Echocardiogram is considered positive for purposes of this algorithm if any of 3 conditions are met: z score of LAD or RCA ≥2.5, coronary arteries meet Japanese Ministry of Health criteria for aneurysms, or ≥3 other suggestive features exist, including perivascular brightness, lack of tapering, decreased LV function, mitral regurgitation, pericardial effusion, or z scores in LAD or RCA of 2–2.5. (7) If the echocardiogram is positive, treatment should be given to children within 10 days of fever onset and those beyond day 10 with clinical and laboratory signs (CRP, ESR) of ongoing inflammation. (8) Typical peeling begins under nail bed of fingers and then toes.
Jane W. Newburger et al. Pediatrics 2004;114:
©2004 by American Academy of Pediatrics

19. Other clinical findings
Cardiovascular
CHF, myocarditis, pericarditis, valvular regurgitation
Coronary artery abnormalities
Aneurysms of medium-size noncoronary arteries
Raynaud’s phenomenon
Peripheral gangrene
Circulation. 2004; 110:

20. Others…
Circulation. 2004; 110:

21. Laboratory findings
Circulation. 2004; 110:

22. Treatment Aspirin Reye syndrome
High dose: anti-inflammatory ; Low dose: anti-platelate
Acute phase: mg/kg per day in four divided dose
No fever for 48 hours: 3-5mg/Kg per day
Low dose maintain until no evidence of coronary changes by 6 to 8 weeks after the onset of illness
Reye syndrome
active infection with varicella or influenza
high-dose aspirin for a prolonged period after KD
Suggested 6 weeks after varicella vaccine
Still unclear results for low dose Aspirin
Circulation. 2004; 110:

23. Treatment Immunoglobin
Reducing the prevalence of coronary artery abnormalities in acute phase
higher doses given in a single infusion having the greatest efficacy.
2 g/kg in a single infusion
Best timing : 5-7 days of illness
Circulation. 2004; 110:

24. -With careful clinical follow-up 10 to 20 years after the onset of Kawasaki disease, patients with no coronary artery changes on echocardiography at any stage of the illness seem to demonstrate a risk for clinical cardiac events that is similar to that in the population without Kawasaki disease,2 but research studies suggest subclinical abnormalities of endothelial function and myocardial flow reserve.
Circulation. 2004; 110:

25. Prognosis Mortality rate of is low (0.1 to 0.3 percent)
Long-term morbidity depends upon the severity of CA involvement
Without: clinically asymptomatic 10 to 21 years later
CA dilatation <8 mm generally regresses
≥8 mm: greatest risk for myocardial infarction
Kawasaki disease: Initial treatment and prognosis, Uptodate

26. Thanks for your attention !