From the Guidelines
Gammadex is not a relevant treatment for Kawasaki disease, and IVIG 2 g/kg as a single infusion is the recommended treatment to reduce the prevalence of coronary artery abnormalities. The efficacy of IVIG administered in the acute phase of Kawasaki disease is well established to reduce the prevalence of coronary artery abnormalities 1. The mechanism of action of IVIG in treatment of Kawasaki disease is unknown, but it appears to have a generalized anti-inflammatory effect. Possible mechanisms of action include modulation of cytokine production, neutralization of toxins or other pathogenic agents, augmentation of regulatory T-cell activity, suppression of antibody synthesis, and provision of anti-idiotypic antibodies 1.
Key Points
- IVIG is a biological product made from pooled donor plasma, and potentially important product manufacturing differences exist 1.
- Adverse effects appear to vary considerably among products, and clinical studies comparing the efficacy of different immune globulin products have been conflicting 1.
- Measles, mumps, and varicella immunizations should be deferred for 11 months after receiving high-dose IVIG 1.
- Even when treated with high-dose IVIG regimens within the first 10 days of illness, 20% of children will develop transient coronary artery dilation, 5% will develop coronary artery aneurysms, and 1% will develop giant aneurysms 1.
Treatment Recommendations
- Patients should be treated with IVIG 2 g/kg as a single infusion, usually given over 10 to 12 hours, together with ASA 1.
- This therapy should be instituted within the first 10 days of illness and, if possible, within 7 days of illness 1.
- IVIG also should be administered to children presenting after the 10th day of illness if they have either persistent fever without other explanation or aneurysms and ongoing systemic inflammation 1.
From the Research
Gammadex Overview
- Gammadex is not explicitly mentioned in the provided studies, however, the studies discuss immunoglobulin replacement therapy, which may be related to Gammadex.
- Immunoglobulin replacement therapy is used to treat primary antibody deficiency syndromes, such as common variable immunodeficiency (CVID) and X-linked agammaglobulinaemia (XLA) 2.
- The therapy involves administering immunoglobulins, which can be given intravenously (IVIG) or subcutaneously (SCIG) 3, 4.
Administration and Dosage
- The recommended starting dose for IVIG is 300-400 mg/kg/month, while for SCIG it is 100 mg/week 2.
- The dose and trough level should be chosen based on the clinical response, with a target IgG trough level of >5 g/L for patients with agammaglobulinaemia and 3 g/L greater than the initial IgG level for patients with CVID 2.
- High-dose IVIG therapy (400 mg/kg/month) has been shown to be effective in increasing serum IgG levels and reducing the frequency and severity of infections 5, 6.
Comparison of IVIG and SCIG
- Both IVIG and SCIG appear to be safe and effective, with comparable efficacy 2, 3.
- IVIG requires fewer infusion sites and less frequent infusions, but may be associated with more systemic adverse reactions 3, 4.
- SCIG, on the other hand, does not require venous access and can be self-administered at home, but may be associated with more local reactions 3, 4.