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Good Morning . Morning Report July 2, 2013. 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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Good morning

Good Morning 

Morning Report

July 2, 2013


Semantic qualifiers

Semantic Qualifiers


Illness script

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

Differential Diagnosis**

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


Predisposing conditions

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

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

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?


C onjunctivitis

Conjunctivitis**

  • Bilateral bulbar injection

  • No exudate

  • Painless

  • Limbic sparing

  • Shortly after fever starts


R ash

Rash**

  • Various forms

    • Nonspecific, diffuse with scattered macules & erythematous papules

    • Occasionally scarlatiniform, erythroderma, erythemamultiforme,

    • uriticarial, or a fine micropustular eruption

  • Not bullous of vesicular

  • Often involves diaper area

  • Within 5d of fever


A denopathy

Adenopathy**

  • Least common feature

  • Anterior cervical triangle

  • Usually unilateral

  • > 1.5 cm

  • Firm, nontender

  • No overlying erythema


S trawberry tongue

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


H ands and feet

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 manifestations1

Clinical Manifestations**

“C R A S H”


Other

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


Good morning

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

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

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

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

Follow-Up


Atypical kawasaki

Atypical Kawasaki


Thanks

Thanks 

  • No noon conference today!


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