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Morning ReportJuly 2, 2013

Good Morning

SymptomsAcute /subacute Chronic

Localized DiffuseSingle MultipleStatic Progressive

Constant IntermittentSingle Episode Recurrent

Abrupt GradualSevere MildPainful NonpainfulBilious 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

Semantic Qualifiers

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

Differential Diagnosis**

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

Predisposing ConditionsWhich 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 = 3:2Which 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 arteriesProcess

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

injectionNo exudatePainlessLimbic sparingShortly after

fever starts

Rash**Various forms

Nonspecific, diffuse with scattered macules & erythematous papules

Occasionally scarlatiniform, erythroderma, erythema multiforme,

uriticarial, or a fine micropustular eruptionNot bullous of vesicularOften involves diaper areaWithin 5d of fever

Adenopathy**

Least common feature

Anterior cervical triangle

Usually unilateral> 1.5 cm Firm, nontenderNo 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 solesFirm, sometimes painful induration of the hands

and feetLater 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 jointsIn children, what behavioral complaint do parents

often give? Irritability***

GI complaints Diarrhea Vomiting Abdominal pain

Hepatomegaly and jaundiceWhat abnormal finding may be seen on abdominal

imaging (esp. RUQ) Acalculous distension of gallbladder…hydrops of the

gallbladder

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 LFTsMild hyperbilirubinemiaWhat 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

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

MyocarditisValvulitisPericarditis 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

Thanks

No noon conference today!