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

Basic information Name: 邱X豪 Patient ID: 17545XXX Gender: Boy Age: 1y1m Underlying disease: Nil Admission date: 2016/4/23 邱國豪 17545659

Chief complaints Fever on and off for about 5 days

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

Skin lesions

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

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)

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

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

Lab data

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.

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

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

Kawasaki disease

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

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: 335-336

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 ≥450 000/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;114:1708-1733 ©2004 by American Academy of Pediatrics

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: 2747-2771

Others… Circulation. 2004; 110: 2747-2771

Laboratory findings Circulation. 2004; 110: 2747-2771

Treatment Aspirin Reye syndrome High dose: anti-inflammatory ; Low dose: anti-platelate Acute phase: 30-50 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: 2747-2771

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: 2747-2771

-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: 2747-2771

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

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