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Pediatrics, Kawasaki Disease: Treatment & Medication
Updated: Oct 28, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Prehospital Care
No specific prehospital care exists for Kawasaki disease. Paramedics should assess the need for intravenous access and rhythm monitoring.
Emergency Department Care
- Any young child who presents to the emergency department (ED) with symptoms of early or acute-stage Kawasaki disease should be evaluated to rule out sepsis or meningitis.
- Although the diagnosis may seem obvious, other life-threatening diseases must be ruled out.
- The ED physician must consider the possibility that a child has an incomplete case and proceed with the evaluation listed above.
- Intravenous access and cardiac monitoring should be established.
- Depending on the institution, anti-inflammatory therapy may need to begin in the ED.
- Arrangements for admission must take into consideration the potential for multiple problems. This is not a routine pediatric illness. Accordingly, transferring the patient to a pediatric referral center may be prudent.
Consultations
- Pediatrics
- Consultation with a pediatric cardiologist may be required if coronary artery aneurysms are identified or if other cardiac complications develop.
- Pediatric cardiothoracic surgery assistance may be needed in the pediatric ICU for those patients who need bypass surgery.
Medication
The medical management of Kawasaki disease primarily involves the use of gamma globulin. Although some have suggested that aspirin is no longer needed, most use high-dose aspirin for a variable period of time, followed by lower-dose aspirin for its antiplatelet effects. Of note, a 2008 Cochrane Database of Systematic Reviews article concluded that "there is insufficient evidence to indicate whether children with Kawasaki disease should continue to receive salicylate as part of their treatment regimen."19
Some controversy exists about the ideal timing to begin gamma globulin, but this is not an issue that concerns emergency physicians. It is given most often from days 5-7.
Although data are limited, authors of several case reports have suggested a possible role for thrombolysis in those with acute MI as a consequence of thrombus formation in aneurysms.20 At this time, it seems unlikely that the emergency physician will administer this therapy.
Some have suggested that there is, or may be, a role for corticosteroids. Most have pointed out that not only is there no good data to support a benefit in terms of outcome but also that current therapy with IVIG and aspirin is safe and effective.21,22,23 In a meta-analysis of 4 studies and 447 patients, Athappan et al concluded that the addition of steroids to standard therapy (IVIG + aspirin) decreased the rate of re-treatment but did not decrease the incidence of coronary aneurysms or adverse events.23
Ibuprofen antagonizes aspirin's antiplatelet activity and should be avoided.
Because these children will take aspirin for a variable period of time, vaccination against influenza and varicella must be ensured.
Studies that involved plasma exchange or cyclophosphamide have shown variable results. Both are used in cases of refractory disease.1
Gamma globulins
These agents can be used to assist in the treatment of inflammation resulting from autoimmune disorders. Much of the pathophysiology in KD involves inflammation. Early and aggressive intervention improves outcome.
Immune globulin, intravenous (Carimune, Gammagard S/D, Gammar-P, Gamunex, Polygam S/D)
Generally recommended as the first drug to be used, but it is not usually the sole therapy. Neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; may increase CSF IgG (10%).
Adult
Not established
Pediatric
A single dose of 2 g/kg IV infused over 12 h is the most common regimen; alternatively, 1 g/kg/d for 2 d
Less often, a regimen calls for 400 mg/kg/d IV qd for 4 d
Repeat course of therapy may be indicated in those who do not have an adequate response to initial treatment
Globulin preparation may interfere with immune response to live-virus vaccine (MMR) and reduce efficacy (do not administer within 3 mo of vaccine)
Documented hypersensitivity; IgA deficiency; anti-IgE/IgG antibodies; severe thrombocytopenia or coagulation disorders
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Flushing of the face, chills, nausea, dyspnea, and tachycardia are the most common adverse effects; less common adverse effects include chest tightness, dizziness, fever, headache, and diaphoresis
Check serum IgA before IVIG (use an IgA-depleted product, eg, Gammagard S/D); infusions may increase serum viscosity and thromboembolic events; infusions may increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-30 d postinfusion)
Increases risk of renal tubular necrosis in elderly patients and in patients with diabetes mellitus, volume depletion, and preexisting kidney disease; laboratory findings associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold increase in ESR for 2-3 wk, and apparent hyponatremia
Anti-inflammatory agents
These agents systemically interfere with events leading to inflammation. Aspirin is indicated for antiplatelet effect.
Aspirin (Anacin, Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin)
Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2. Adequate anti-inflammatory therapy requires that aspirin be combined with gamma globulin.
Children with coronary artery aneurysms receive aspirin for prolonged periods. First-line therapy with intravenous immunoglobulin. PO absorption of aspirin may decrease in Kawasaki disease to <50% (compared to typical bioavailability of 85-90%). This altered bioavailability may explain why higher doses required to achieve a salicylate serum concentration >20 mg/dL.
Adult
Not established
Pediatric
80-100 mg/kg/d PO divided qid for 2 wk initial; 3-5 mg/kg PO qd for 6-8 wk maintenance; may use high-dose regimen for 2 days, then switch to the low dose for the remainder of the treatment period; currently no good literature support one regimen over the other
Coronary artery abnormalities: 3-5 mg/kg PO qd long term (with or without dipyridamole)
Coadministration with ibuprofen may decrease antiplatelet effect; efficacy may also decrease when coadministered with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs
Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma; use in children (<16 y) with influenza because of association of aspirin with Reye syndrome
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, with history of blood coagulation defects, or taking anticoagulants; caution in asthma; dose is on the borderline of that causing salicylate toxicity, therefore, monitor for toxicity (ie, vomiting, hyperpnea, lethargy, liver dysfunction); monitor salicylate level and maintain at 18-28 mg/dL
More on Pediatrics, Kawasaki Disease |
| Overview: Pediatrics, Kawasaki Disease |
| Differential Diagnoses & Workup: Pediatrics, Kawasaki Disease |
 Treatment & Medication: Pediatrics, Kawasaki Disease |
| Follow-up: Pediatrics, Kawasaki Disease |
| Multimedia: Pediatrics, Kawasaki Disease |
| References |
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References
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Further Reading
Keywords
Kawasaki disease, Kawasaki disease symptoms, Kawasaki disease treatment, Kawasaki's disease, Kawasaki disease in children, incomplete Kawasaki disease, Kawasaki syndrome, myocardial infarction, myocarditis, acute vasculitic syndrome, coronary artery aneurysms, sudden death
