The efficacy and safety, including arrhythmia and sudden death, 1 2 of intravenous methylprednisolone pulse (IVMP) therapy in patients with Kawasaki disease (KD) are uncertain. We conducted a control study in KD patients with persistent or recurrent fever (>37.5˚C) 48 hours after a single infusion of initial intravenous immunoglobulin (IVIG) 2 g/kg. At enrolment (day 1), the subjects were randomised to receive IVMP (30 mg/kg/day of methylprednisolone for three days), or additional IVIG (2 g/kg). Heparin was also continuously infused (15-20 units/kg/h) in the IVMP group. The study was halted prematurely because of adverse effects of IVMP when 22 patients were recruited; they accounted for 13% of KD patients treated with initial IVIG. The antipyretic effect of IVMP was superior to that of additional IVIG on day 2 (p = 0.02, repeated measures analysis), but not on day 3 and later (fig 1). The fraction of febrile patients was significantly lower in the IVMP group until day 3 (1/11 v 8/11, p , 0.001, Fisher exact test), but not on day 4 and later (6/11 v 6/11). Coronary artery dimensions and the prevalence of coronary artery lesions (2/11 v 3/11) were similar in the two groups. Regarding adverse effects, sinus bradycardia and hyperglycaemia occurred more often in the IVMP group (table 1). Hypertension occurred in 91% of the IVMP group, but the fraction did not differ significantly, probably due to the small sample size. All of the adverse effects were transient. There were no convulsions, gastrointestinal symptoms, infection, malignant arrhythmia, or sudden death in any subjects. KD patients refractory to initial IVIG should be treated with additional IVIG, 3 4 because IVMP induced faster but temporary resolution of fever and more adverse effects. Further investigations with steroid therapy are necessary to determine the indication and the appropriate dose in KD.

This study aimed to determine the effects of intravenous methylprednisolone pulse (IVMP) therapy on cytokine levels in patients with acute Kawasaki disease (KD) unresponsive to initial intravenous immunoglobulin (IVIG) therapy. Fifteen KD patients unresponsive to initial IVIG, 2 g/kg/day, were randomized to receive IVMP (n = 7), 30 mg/kg/day for 3 days or additional IVIG (n = 8), 2 g/kg/day, and plasma cytokine levels were compared. The fraction of febrile patients was significantly lower in the IVMP group than in the additional IVIG group on day 2 (0/7 vs. 3/8, p = 0.03), but not on day 4 and later (3/7 vs. 4/8, p = 1.00) because of recurrent fever. The prevalence of coronary lesions was similar between the two groups (2/7 vs. 2/8, p = 1.00). The ratios of plasma levels of tumor necrosis factor-alpha and monocyte chemoattractant protein-1 to those at enrollment (defined as day 1) were significantly lower in the IVMP group on day 4 (0.50 +/- 0.27 vs. 1.01 +/- 0.46, 0.53 +/- 0.39 vs. 0.93 +/- 0.44, p = 0.02 and 0.045, respectively), but not on day 7 (0.54 +/- 0.34 vs. 0.88 +/- 0.39, 0.76 +/- 0.39 vs. 0.61 +/- 0.17, p = 0.07 and 0.83, respectively). The ratios of interleukin-2 receptor, interleukin-6, and vascular endothelial cell growth factor to those at enrollment did not differ significantly between the two groups. In conclusion, for KD patients unresponsive to initial IVIG, IVMP suppresses cytokine levels faster, but subsequently similarly, compared with additional IVIG.

Chromosome 22q11.2 deletion causes DiGeorge syndrome, velocardiofacial syndrome, conotruncal anomaly face syndrome with tetralogy of Fallot (TOF), and sporadic or familial TOF. To determine the prevalence and clinical importance of the 22q11.2 deletion in TOF, a series of 212 Japanese TOF patients was studied. The type of pulmonary blood supply, which may lead to various clinical outcomes, and other additional anomalies were evaluated clinically. The 22q11.2 deletion was diagnosed by fluorescence in situ hybridization with N25 and TUPLE1 probes. Of the 212 patients examined, 28 (13%) had a 22q11.2 deletion, the frequency being higher than that in TOF patients with trisomy 21. The prevalence of the deletion in TOF patients with pulmonary atresia (PA) plus major aortico-pulmonary collateral arteries (MAPCA) was significantly higher than the value in patients with PA plus patent ductus arteriosus (PDA) (P = 0.04) or with pulmonary stenosis (PS) (P < 0.0001). All 28 patients with 22q11.2 deletion had one or more extracardiac abnormalities. Four of 9 patients with the 22q11.2 deletion and TOF-PA-MAPCA suffered from bronchomalacia, while none of 19 patients with TOF-PA-PDA or TOF-PS manifested bronchomalacia (P = 0.006). These results indicate that 22q11.2 deletion is the most frequent cause of syndromic TOF, especially for TOF-PA-MAPCA, and bronchomalacia is the clinically most important associated anomaly in TOF-PA-MAPCA patients.

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