Good Morning  Morning Report July 2, 2013

Problem Characteristics Semantic Qualifiers Symptoms Acute /subacute Chronic Localized Diffuse Single Multiple Static Progressive Constant Intermittent Single Episode Recurrent Abrupt Gradual Severe Mild Painful Nonpainful Bilious Nonbilious Sharp/Stabbing Dull/Vague Problem Characteristics Ill-appearing/ Toxic Well-appearing/ Non-toxic Localized problem Systemic problem Acquired Congenital New problem Recurrence of old problem He has done research on transitions in care

Illness Script Predisposing Conditions Pathophysiological Insult Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc) Pathophysiological Insult What is physically happening in the body, organisms involved, etc. Clinical Manifestations Signs and symptoms Labs and imaging 3

Differential Diagnosis** What other diagnoses would you consider in a patient with suspected Kawasaki Disease?

Predisposing Conditions Which country has the highest prevalence of Kawasaki Disease? Japan (10x that of US) In the US, which ethnicity is most commonly affected? Least commonly? Most common in Asians and Pacific Islanders Least common in caucasians Age Median = 2yo 76% of cases in <5yo Male:Female = 3:2 Which seasons are you more likely to see patients present with KD? Seasonal peaks in winter and spring

Pathophysiology Complete etiology is unknown, but features suggest an infectious source. Generalized vasculitis Affects all blood vessels throughout the body Which specific vessels are affected in KD? Preferentially involves the coronary arteries Process Initial neutrophil influx  Large mononuclear cells w/lymphocytes and plasma cells  Active inflammation  Progressive fibrosis and scar formation

Clinical Manifestations You are seeing a patient with multiple days of fever as well as a rash and some other non-focal symptoms. How many days of fever must be present before diagnosing a patient with KD? What are the other 5 criteria used to diagnose KD?

Conjunctivitis** Bilateral bulbar injection No exudate Painless Limbic sparing Shortly after fever starts

Rash** Various forms Not bullous of vesicular Nonspecific, diffuse with scattered macules & erythematous papules Occasionally scarlatiniform, erythroderma, erythema multiforme, uriticarial, or a fine micropustular eruption Not bullous of vesicular Often involves diaper area Within 5d of fever

Adenopathy** Least common feature Anterior cervical triangle Usually unilateral > 1.5 cm Firm, nontender No overlying erythema

Strawberry Tongue** Changes of the lips and oral cavity Cracked, red, swollen, bleeding lips Diffuse erythema of oral mucosa Oral ulcers and exudates are not seen

Hands and Feet** Erythema of palms and soles Firm, sometimes painful induration of the hands and feet Later desquamation that usually begins in periungal region (2-3 weeks after fever onset)

Clinical Manifestations** “C R A S H”

Other** Arthritis/arthralgias that involve multiple joints In children, what behavioral complaint do parents often give? Irritability*** GI complaints Diarrhea Vomiting Abdominal pain Hepatomegaly and jaundice What abnormal finding may be seen on abdominal imaging (esp. RUQ) Acalculous distension of gallbladder…hydrops of the gallbladder Chlamydia: Tachypnea, interstitial infiltrates, afebrile, not ill-appearing

Labs** What would your CBC look like? Leukocytosis Majority with WBC > 15,000 Predominance of immature and mature granulocytes Anemia Thrombocytosis…with platelet counts 500-1000 x 103 Elevated ESR (>40 mm/hr) and CRP (>3mg/dL) Mild to moderate elevation of LFTs Mild hyperbilirubinemia What abnormality could you see on the UA and urine culture? Sterile pyuria…+WBC Negative cultures Aseptic meningitis (if CSF obtained)

Treatment** High-dose aspirin (80-100mg/kg/day divided QID) during acute phase of illness  3-5mg/kg/day until no evidence of coronary changes by 6-8 weeks Continued aspirin therapy if coronary changes present IVIG 2g/kg/dose (up to 2-3 doses depending on fever) Children treated with IVIG and ASA had faster resolution of fever and fewer coronary abnormalities than those treated with ASA alone Refractory KD…treatment is controversial Abscess: thick-walled cavity with air-fluid level, often follows aspiration, mouth organisms (strep and anaerobes +/- Staph and GNR), TB should be considered; needle aspiration for cx specimen is recommended; CT scan can provide more detail Necrotizing PNA: rare complication of bacterial PNA in which liquefaction and necrosis of lung tissue is caused by toxins of highly virulent organisms; children appear VERY ill; tx consis of long course (4wks) of IV abx…typially vanc or clinda (for Strep, GAS, and staph)

Cardiac Complications** Coronary artery aneurysm (identified on echo within 1-2mo of diagnosis) 20-25% of untreated patients; 5% of treated patients Resolution within 1-2 years in approximately 50% Myocardial infarction Principal cause of death Most occur within 1 year of disease onset but can occur years later Myocarditis Valvulitis Pericarditis with effusion

Echocardiogram** When should you obtain an echo on patients with suspected Kawasaki disease? Obtain on all patients with suspected Kawasaki At diagnosis Follow-up…usually at 2 weeks and 6 weeks after diagnosis

Follow-Up

Atypical Kawasaki

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