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Background: Persistent low-grade fever has been observed in some patients during intravenous immunoglobulin (IVIG) therapy for Kawasaki disease (KD); however, smoldering fever (SF) has not previously been reported in patients with KD. This study aimed to clarify the clinical characteristics of SF in patients with KD., Methods: A single-center retrospective cohort study, which included a total of 621 patients who received IVIG therapy, was conducted. Patients with a fever of 37.5-38°C lasting ≥3 days after 2 days of the initial-IVIG were defined as the SF group. Patients were divided into four groups according to the fever course: SF (n = 14), biphasic fever (BF, n = 78), non-fever after initial-IVIG (NF, n = 384), and persistent fever (PF, n = 145). The clinical features of SF were described and compared between the groups., Results: The median duration of fever in the SF group was 16 days, which was longer than that in any other group. The neutrophil fraction after IVIG therapy in the SF group was higher than that in the BF and NF groups but similar to that in the PF group. Repeated IVIG administration in the SF group resulted in increased IgG levels but decreased serum albumin levels. In the SF group, 29% of the patients had coronary artery lesions at 4 weeks., Conclusions: The frequency of SF in KD was 2.3%. Patients with SF continued to have moderate inflammatory responses. Repeated administration of IVIG doses was not effective in treating SF, and acute coronary artery lesions were occasionally observed. Active therapeutic intervention was needed., (© 2023 Japan Pediatric Society.)

Background: Patients whose parents had a history of Kawasaki disease (KD) often show a poor response to intravenous immunoglobulin (IVIG). There are very few reports describing the clinical characteristics and no reports on changes in C-reactive protein (CRP) level after IVIG administration in such cases., Methods: A single-center cohort study including 950 patients hospitalized for KD and treated with IVIG was conducted. The patients were divided into two groups: patients whose parents had a history of KD (PHK) (N = 16) and patients whose parents had no history of KD (NPHK) (N = 934). The clinical characteristics and responsiveness to IVIG were compared between the two groups., Results: The PHK group had higher CRP levels before administering IVIG than the NPHK group (P = 0.0010). CRP levels after IVIG administration were higher in the PHK group than in the NPHK group (P = 0.0094). The percentage of patients who received a second administration of IVIG on day 2 after the initial IVIG therapy was higher in the PHK group than in the NPHK group (P = 0.0016). The percentage of patients who required plasma exchange therapy in the PHK group was significantly higher than that in the NPHK group (P = 0.0010)., Conclusions: Patients in the PHK group had severe KD before IVIG administration, and these patients showed poor responsiveness to IVIG therapy., (© 2021 Japan Pediatric Society.)

Background: The purpose of this study was to predict which patients would require plasma exchange therapy (PEX) using laboratory findings during intravenous immunoglobulin (IVIG) therapy in Kawasaki disease., Methods: A retrospective, single-center, cohort study was conducted. Of the 621 IVIG-treated patients, 166 patients who received a second IVIG dose on the second day after initiation of IVIG were included. The endpoint was PEX. Participants were divided into two groups: 10 patients who received PEX and 156 patients who did not. The variables were C-reactive protein (CRP), white blood cell count (WBC), and neutrophil fraction (NEUT) at three time points: pre-initial IVIG, pre-second IVIG, and post-second IVIG. The change ratio (post-IVIG value/pre-IVIG value) of each variable, with each IVIG dose, was calculated. Receiver operating characteristic analysis determined the area under the curve (AUC) and cut-off values., Results: The variables with an AUC > 0.9 were CRP (post-second IVIG), change ratio of CRP (CRP value post-second IVIG/CRP value pre-second IVIG), NEUT (pre-second IVIG), and NEUT (post-second IVIG). Among these, the variables with high sensitivity were CRP (post-second IVIG) and the change ratio of CRP (second IVIG), with cut-off values of 9.52 mg/dL and 0.99, respectively. The sensitivity and specificity of these variables were 100% and 91%, and 100% and 80%, respectively. The combined sensitivity and specificity (95% confidence intervals) of these two variables were 100% (59-100) and 94% (89-97)., Conclusion: High CRP levels and the change ratio of CRP after the second IVIG dose were associated with PEX., (© 2022 Japan Pediatric Society.)

Tolvaptan is an oral vasopressin type 2 receptor antagonist that can be used for heart failure patients with hyponatremia or symptomatic congestion. Although the effects of tolvaptan in adults have been well documented, only limited information is available in children. The case of a neonate with congestive heart failure complicated with chylothorax after palliative surgery for transposition of the great arteries treated with tolvaptan is reported. Slow up-titration to 0.1 mg/kg successfully increased urine output and improved refractory congestive heart failure without hypernatremia. Subsequently, bodyweight and chylothorax decreased gradually. Moreover, the use of tolvaptan reduced the dosage of furosemide. Tolvaptan could be an alternative drug for neonates with congestive heart failure. Further large studies are needed to confirm the efficacy and identify the appropriate dose of tolvaptan in neonates., (© 2015 Japan Pediatric Society.)