A young child with pediatric multisystem inflammatory syndrome successfully treated with high-dose immunoglobulin therapy.

Pediatric multisystem inflammatory syndrome (MIS-C) is a disease that presents mainly in older children after coronavirus disease 2019 (COVID-19) and is associated with Kawasaki-like symptoms and multiple-organ failure. The number of cases of MIS-C has increased since April 2020, with reports mainly from Europe and the United States. The reason is unclear, but few cases of MIS-C have been reported in Asian countries, including Japan. No treatment has been established for MIS-C. In this study, we report the case of a young boy treated with IVIg for MIS-C by measuring the cytokine profile over time. A 4-year-old boy presented with Kawasaki disease-like symptoms 28 days after a positive result from polymerase chain reaction test for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), meeting the World Health Organization criteria for MIS-C diagnosis. Blood tests showed lower levels of C-reactive protein and ferritin, and no decrease in lymphocyte count (<1000/µL) or more increase in fibrinogen than those reported in Japan for MIS-C in school-aged children and older. Neopterin, interleukin (IL)-6, IL-18, soluble tumor necrosis factor receptor (sTNF-R)I and sTNF-RII were all high at disease onset, but neopterin, IL-6, and sTNF-RII rapidly decreased with fever resolution after the second dose of IVIg, while IL-18 and sTNF-RI decreased bimodally. As far as we can determine, this case represents the youngest identified in Japan. The key point of difference between MIS-C and Kawasaki disease is older age in MIS-C, but attention is also needed in infants.

Early repolarization pattern associated with sudden cardiac death: long-term follow-up in patients with chronic heart failure.

BACKGROUND: Identification of patients with chronic heart failure (CHF) at a risk for sudden cardiac death (SCD) is an important objective. Early repolarization pattern (ERP) is associated with ventricular fibrillation in patients without structural heart diseases. Moreover, ERP was reported to be associated with SCD in patients with old myocardial infarction in a case-control study. However, little information is available on the prognostic significance of ERP in CHF patients. Thus, we aimed to investigate whether ERP is associated with SCD in CHF patients. METHODS AND RESULTS: The study population consisted of 132 consecutive outpatients with NYHA class I, II and III congestive heart failure and radionuclide left ventricular ejection fraction less than 40%. All patients underwent the standard 12-lead electrocardiogram at enrollment, where we assessed the presence of ERP using the criteria of J-point elevation ≥ 0.1 mV in at least 2 inferior or lateral leads. The primary endpoint of this study was SCD. At enrollment, 16 patients had ERP. During the follow-up period of 6.7 ± 3.5 years, 26 patients had SCD. Kaplan-Meier analysis showed that SCD was observed significantly more frequently in patients with ERP than in those without ERP (63% [10/16] vs 14% [16/116], P < 0.0001]. A multivariate Cox analysis revealed that ERP was significantly and independently associated with SCD (hazard ratio, 3.7; 95% confidence interval, 1.6-8.6; P = 0.002). CONCLUSION: ERP in inferior leads would be associated with an increased risk of SCD in CHF patients.

Kidney enhancement on X-rays following emergency percutaneous coronary procedure predicts poor short- and long-term clinical outcomes.

BACKGROUND: We assessed radiographic kidney enhancement following an emergency coronary procedure as a predictor of contrast-induced nephropathy (CIN) and poor long-term outcome. METHODS AND RESULTS: We enrolled 126 consecutive patients who underwent an emergency coronary procedure and abdominal X-ray within 24h. We defined kidney enhancement as positive when the density of the kidneys was equal to or higher than that of the lumbar vertebrae. Of the 126 patients, 11 showed kidney enhancement and 115 did not. There were no significant differences in the baseline characteristics of patients with and without kidney enhancement. The incidence of CIN was significantly higher in patients with than in those without kidney enhancement (91% vs. 6%, P<0.01). During a mean follow-up of 21±16 months, 5 of 11 patients with kidney enhancement had poor outcomes, such as renal replacement therapy or death, whereas poor outcomes were observed in only 12 of 115 patients without kidney enhancement. Kaplan-Meier analysis revealed a significant difference in the probability of a poor outcome between patients with and those without kidney enhancement (46% vs. 10%, P<0.01). CONCLUSIONS: Radiographic kidney enhancement following a percutaneous coronary procedure predicts the occurrence of CIN and poor clinical outcome.

Prevention of contrast-induced nephropathy by bolus injection of sodium bicarbonate in patients with chronic kidney disease undergoing emergent coronary procedures.

We conducted a prospective study to determine whether a bolus injection of sodium bicarbonate before emergent coronary procedures in patients with chronic kidney disease (CKD) might prevent contrast-induced nephropathy (CIN). We enrolled 59 patients with CKD, defined by a serum creatinine concentration of >1.1 mg/dl or an estimated glomerular filtration rate of <60 ml/min, who were scheduled at admission to undergo an emergent coronary procedure. The patients were randomized to receive a bolus intravenous injection of 154 mEq/L of sodium bicarbonate (n = 30) or sodium chloride (n = 29) at the dose of 0.5 ml/kg, before contrast administration, followed by infusion of 154 mEq/L sodium bicarbonate at 1 ml/kg/hour for 6 hours in both groups. The primary end point was the occurrence of CIN, defined as an increase by > 25% or > 0.5 mg/dl of the serum creatinine level within 2 days after the procedure. In the sodium bicarbonate group, the serum creatinine concentration remained unchanged within 2 days of contrast administration (from 1.32 ± 0.46 to 1.38 ± 0.60 mg/dl, p = 0.33). In contrast, it had increased in the sodium chloride group (1.51 ± 0.59 to 1.91 ± 1.19 mg/dl, p = 0.006). The incidence of CIN was significantly lower in the sodium bicarbonate group than in the sodium chloride group (3.3% vs 27.6%, p = 0.01). In conclusion, rapid alkalization by bolus injection of sodium bicarbonate was effective for the prevention of CIN in patients with CKD undergoing emergent procedures.