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Happy Monday!. Morning Report July 16, 2012. Semantic Qualifiers. Illness Script. Predisposing Conditions Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc) Pathophysiological Insult
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Happy Monday! Morning Report July 16, 2012
Illness Script • Predisposing Conditions • 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
Predisposing Conditions • Highest prevalence in Japan (10x that of US) • In the US • Most common in Asians and Pacific Islanders • Least common in caucasians • Age • Median = 2yo • 76% of cases in <5yo • Male:Female = 3:2 • Seasonal peaks in winter and spring • Genetic predisposition
Pathophysiology • Complete etiology is unknown, but features suggest an infectious source. • Generalized vasculitis • Affects all blood vessels throughout the body • 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** “C R A S H”
Conjunctivitis** • Bilateral bulbar injection • No exudate • Painless • Limbic sparing • Shortly after fever starts
Rash** • Various forms • Nonspecific, diffuse with scattered macules & erythematouspapules • Occasionally scarlatiniform, erythroderma, erythemamultiforme, • uriticarial, or a fine micropustular eruption • Not bullous of vesicular • Within 5d of fever • Often involves diaper area
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 • Strawberry tongue • 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)
Other** • Arthritis/arthralgias that involve multiple joints • Irritability*** • GI complaints • Diarrhea • Vomiting • Abdominal pain • Hepatomegaly and jaundice • Acalculous distension of gallbladder
Labs** • 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 • Sterile pyuria • 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
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** • Obtain on all patients with suspected Kawasaki • At diagnosis • Follow-up…usually at 2 weeks and 6 weeks after diagnosis
Thanks • EVERY Content Spec!! • “Kawasaki Disease.” Pediatrics in Review. 2008, v29 (9), p308. • Noon conference = Feedback and Evaluations (Residents ONLY)