ABSTRACT. Objectives. To determine initial intravenous gammaglobulin (IVIG) treatment failures in Kawasaki disease (KD) and to report the outcome of retreatment and our use of pulse intravenous (IV) methylprednisolone and cyclophosphamide in patients with persistent KD.Study Design. Retrospective analysis of the treatment and response of children with KD over 3 years.Results. Fifty (77%) of 65 patients completely responded to a single treatment with IVIG (2 g/kg). Fifteen patients (23%) required retreatment; 10 patients fully responded but 5 had persistent disease (3 developed coronary aneurysms and 4 developed coronary artery thrombosis). Four of these 5 patients with persistent disease were treated with pulse IV methylprednisolone and 2 were also treated with IV cyclophosphamide. There was no progression of coronary aneurysms and no deaths. No initial patient characteristics predicted IVIG treatment failure or the development of coronary aneurysms.Conclusion. Nearly 23% of patients with KD may require retreatment and 8% may develop coronary aneurysm. Additional antiinflammatory therapy, such as IV methylprednisolone and IV cyclophosphamide, may be helpful in treating persistent KD. Pediatrics 2000;105(6). URL: http://www.pediatrics.org/cgi/content/full/105/6/ e78; corticosteroids, vasculitis, Kawasaki disease, heart disease, gammaglobulin therapy, cyclophosphamide.

The mechanisms by which T lymphocytes acquire the capacity to produce interleukin 4 (IL-4) and other lymphokines during intrathymic and extrathymic development are poorly understood. To gain insight into this process, we determined the capacity of human neonatal and adult T lineage cell populations to produce IL4 after polyclonal activation. IL-2 and interferon-y (IFN-'y) production were studied in parallel, since their production by neonatal T cells is known to be similar or diminished, respectively, compared to adult T cells. Production of IL4 by neonatal CD4' T cells and IFN-y by neonatal CD4' and CD8+T cells was markedly lower compared with analogous adult cell populations, whereas IL-2 production was similar. Transcription of IL4, as determined by nuclear run-on assays, and IL4 mRNA-containing cells, as determined by in situ hybridization, were undetectable in neonatal T cells, whereas both were detectable in adult T cells. IFN-'y transcription and IFN--y mRNAcontaining cells were reduced in neonatal T cells compared with adult T cells. Reduced lymphokine production by neonatal T cells correlated with their lack of a CD45R-(putative memory T cell) population; cells with this surface phenotype comprised 30-40% of the adult CD4' T cells and were highly enriched for IL4 and IFN--y, but not IL-2 production. IL-4, IFN-y, and IL-2 mRNA expression by neonatal CD4+CD8-thymocytes was similar to that found in circulating neonatal CD4+ T cells. Taken together, these findings suggest that the extrathymic generation of memory T cells during postnatal life may result in an increased capacity for IL4 and IFN-y gene expression. In addition, IFN-,y and IL-2 mRNA were significantly more abundant than IL4 mRNA in activated neonatal CD4+CD8-thymocytes and CD4+ T cells, as well as adult CD4' CD45R-T cells. Therefore, the capacity of T lineage cells to express the IL4 gene may be more restricted compared to other lymphokine genes beginning in intrathymic development. This restricted capacity appears to persist during postnatal extrathymic maturation of T cells. (J. Clin. Invest. 1991. 87:194-202.).

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