Clinical Characteristics of Recurrent Kawasaki Disease / 소아과

PURPOSE: The purpose of this study is to investigate the clinical manifestations and the risk factors of recurrent Kawasaki disease(KD). METHODS: From March 1995 to June 2003, 14 children with recurrent KD in Ewha Womans University Hospital were etrospectively evaluated by reviewing their admission reports. The clinical characteristics, laboratory findings, treatment and complications of the recurrent KD group were compared to that of a control group. The control group was admitted once for KD. Also, for the patients in the recurrent KD group, the clinical characteristics, laboratory findings, treatment and com plications were compared between an initial and a second episode. RESULTS: Among the 561 children with KD, 14 cases(2.4%) were included in the recurrent KD. At the initial episode of recurrent KD group, the sex ratio was 1.3 : 1(male : female) and the mean age was 23+/-13 months. There were no differences in demographic characteristics, clinical characteristics, laboratory results, treatment and complications between the recurrent KD group and control group. The interval between two episodes in the recurrent KD group was 17.9+/-16.2 months(1-60 months). The total febrile period showed no differences between the two episodes in the recurrent KD group but the febrile period before admission was shorter in the second episode(P=0.02). The clinical characteristics, laboratory findings, treatment and coronary artery complications were not different in the two episodes of the recurrent KD group. CONCLUSION: There were no predictive clinical characteristics or laboratory findings for recurrent KD. Treatment and coronary artery complications were not different between an initial and a second episode of the recurrent KD.

Matrix Metalloproteinases, Tissue Inhibitors and Cytokines in Patients with Kawasaki Disease / 소아과

PURPOSE: Kawasaki disease(KD) is a multisystemic inflammatory vasculitis of unknown etiology, but immunological abnormalities have been documented and implicated in the pathogenesis of KD. Matrix metalloproteinases(MMPs) have proteolytic activity against connective tissue proteins, and increased activity of MMPs and a quantitative imbalance between MMP and tissue inhibitor of MMP (TIMP) can result in several pathologic conditions. MMP and TIMP may also be involved in the formation of coronary arterial lesions in KD. METHODS: Serum levels of MMP1, MMP2, MMP9, TIMP1, TIMP2, interleukin(IL)-6 and tumor necrosis factor(TNF)-alpha were measured in 27 KD patients(group I, 10 patients with normal coronary artery; group II, 17 patients with coronary arterial lesions) and 15 healthy children(group III). Blood samples from each study group were drawn before and after intravenous immunoglobulin(IVIG) therapy and in the convalescent stage. RESULTS: The MMP9 levels and MMP9/TIMP2 ratios before and after IVIG therapy were significantly higher in group II. The MMP9 levels were significantly higher before IVIG therapy, and decreased through the convalescent stage. The IL-6 and TNF-alpha levels were also significantly higher in group II than in the other groups. The serum MMP9 levels showed significantly positive correlation with the circulating leukocyte counts and IL-6 levels. CONCLUSION: The increased levels of MMP and the imbalance between MMP and TIMP increase the susceptibility to the coronary arterial lesions in KD. The cytokines including IL-6 and TNF-alpha are also important in the activation of MMP and formation of coronary arterial lesions in KD.

Aseptic Meningitis Secondary to High-Dose Intravenous Immunoglobulin Therapy in Kawasaki Disease / 소아과

PURPOSE: Kawasaki disease is a multisystemic inflammatory vasculitis and associated with neurologic features such as aseptic meningitis. High-dose intravenous immunoglobulin(IVIG) is used to treat Kawasaki disease and generally considered to be safe, but rare cases of aseptic meningitis with unknown etiology have been reported. The aseptic meningitis associated with Kawasaki disease was compared with meningitis as the adverse reaction of high-dose IVIG. METHODS: Sixteen Kawasaki disease patients with typical meningeal irritation symptoms were reviewed from January 1997 to July 2002. Group I(n=11) had meningitis associated with Kawasaki disease and Group II(n=5) had meningitis as the adverse reaction of high-dose IVIG therapy. The clinical features and hematologic and cerebrospinal fluid(CSF) findings were compared between the two groups. RESULTS: The duration of fever was significantly longer in group I than group II. Meningeal irritation symptoms were milder in group II than group I. Hemoglobin levels were lower in group II than group I. Monocyte counts were higher in group I than group II. In the CSF analysis, neutrophil counts were significantly higher in group II than group I. Otherwise, there were no significant differences in CSF protein and sugar levels between the two groups. CONCLUSION: Aseptic meningitis occurred in patients with Kawasaki disease after administration of high-dose IVIG. High-dose IVIG was considered to be safe in general. However, clinicians should be aware of the potential for neurologic complications such as acute aseptic meningitis in patients receiving high-dose IVIG for Kawasaki disease.