Stringent variant interpretation guidelines can lead to high rates of variants of uncertain significance (VUS) for genetically heterogeneous disease like long QT syndrome (LQTS) and Brugada syndrome (BrS). Quantitative and disease-specific customization of

Intervention to prevent obesity should be focused on late infancy in both genders and male adolescents in Japan.

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yocarditis frequently occurs in the acute phase of Kawasaki syndrome (KS) and may be transient, 1,2 but there are a few cases of severe myocarditis. [3][4][5][6][7][8] We present 4 cases of myocarditis in KS that required additional catecholamine treatment for severe left ventricular dysfunction (LVD).Case Reports (Table 1, Fig 1) Patient 1 (Fig 1A) A 7-year-old-girl (height 115 cm, body weight 20 kg) was admitted to a general hospital because of 4 days of persistent fever and right cervical adenopathy (white blood cell count (WBC) 22,600 / l, Creactive protein (CRP) 10 mg/dl). Rash and edema of the hands and feet developed on the 5th day of illness. The echocardiography revealed a normal value of ejection fraction (EF) (69%). On the 8th day, the fever was persisting and she was referred to us. Physical examination revealed a cardiac gallop rhythm, nasal alar breathing, eyelid edema, bilateral conjunctival congestion, strawberry tongue, right cervical adenopathy and edema of the hands and feet. The liver was palpable at 7 cm below the right costal margin. The results of laboratory tests were: WBC 18,700 / l, hemoglobin (Hb) concentration 9.4 g/dl, hematocrit (Hct) 32.4%, platelets 18-10 4 / l, CRP 24 mg/dl, mild liver dysfunction (aspartate aminotransferase (AST) 51 IU/L, alanine aminotransferase (ALT) 81 IU/L), hyponatremia (129 mmol/L), and hypoproteinemia (total protein 4.6 g/dl, albumin 2.0 g/dl). Hypotension became obvious with pulmonary edema and a decreased EF (44%), which were noted on chest X-ray and echocardiography at admission. With a diagnosis of KS with myocarditis, we started treatment with aspirin (30 mg·kg -1 ·day -1 ), intravenous immunoglobulin (IVIG: 2 g/kg for 24 h) and dopamine (3 g· kg -1 ·min -1 ). Despite the medication, the hypotension did not improve and sufficient urinary output was not obtained. Dobutamine (3 g·kg -1 ·min -1 ) and diuretics were added and her hypotension and oliguria improved. The fever subsided on the 10th day, but re-emerged the next day. Additional IVIG (2 g/kg for 24 h) was administered and the fever subsided on the 12th day. Giant coronary aneurysms were observed during convalescence.Patient 2 (Fig 1B) A 6-year-old-girl (height 117 cm, body weight 21 kg) was admitted to Kagoshima Medical Association Hospital because of 3 days of persistent fever and right cervical adenopathy (WBC 30,700 / l, CRP 23 mg/dl). Altered consciousness was observed on the 4th day of illness. Bilateral conjunctival congestion, strawberry tongue and rash developed on the 5th day (WBC 15,800 / l, CRP 23 mg/dl). Echocardiography revealed normal coronary arteries and normal EF (64%). With the diagnosis of KS, IVIG (2 g/kg for 24 h) was started. Because of liver dysfunction, flurbiprofen (4 mg · kg -1 · day -1 ) instead of aspirin was also started. Although the fever subsided on the 6th day, the serum level of CRP increased (26 mg/dl) and EF decreased (48%). Hypotension and oliguria were also observed together with increases in blood urea nitrogen (BUN) (55 mg/dl) and creatinine (1.3 mg/dl). ...

Application of the 3 criteria resulted in different AKI incidences, but each criterion could be useful for detecting risk factors for AKI. Notably, using m-KDIGO criteria provides more important subsequent postoperative outcomes. The m-KDIGO AKI criteria describe clinically relevant AKI in infants after cardiac surgery.

Disruption of endothelial cell homeostasis may be associated with the pathogenesis of coronary artery abnormalities (CAA) in Kawasaki disease (KD). We sought to clarify the poorly understood pathogenic role of endothelial cell survival and death in KD vasculitis. Human umbilical vein endothelial cells (HUVECs) stimulated with sera from KD patients, compared with sera from patients with bacterial infections, exhibited significant increases in cytotoxicity, high mobility group box protein 1 (HMGB-1), and caspase-3/7 and a decrease in phosphorylated Akt/Akt (pAkt/Akt) ratios. HUVECs stimulated with sera from KD patients treated with immunoglobulin (IG) showed significantly decreased cytotoxicity, HMGB-1, and caspase-3/7 levels and increased pAkt/Akt ratios, as compared with results for untreated HUVECs (P < 0.001, P = 0.008, P = 0.040, and P < 0.001, respectively). In HUVECs stimulated with sera from KD patients, the increased cytotoxicity levels and the suppression of increased pAkt/Akt ratios after subsequent IG treatment were closely related to the development of CAA (P = 0.002 and P = 0.035). Our data reveal that shifting the balance toward cell death rather than survival appears to perturb endothelial cell homeostasis and is closely related to the development of CAA. The cytoprotective effects of IG treatment appear to ameliorate endothelial cell homeostasis.

We measured serum procalcitonin concentrations in 160 patients suffering from Kawasaki disease. Serum procalcitonin was significantly higher in nonresponders to an initial intravenous immunoglobulin treatment than in responders. A cutoff value of procalcitonin (0.5 ng/mL) for nonresponders showed that the sensitivity was 85% and the accuracy was 64%. Multivariate logistic regression analysis revealed that procalcitonin-positive cases showed the highest risk for nonresponders.

Background:The corrected QT interval (QTc) according to Bazett's formula (QTc = QT/RR 1/2 ) has been used in clinical practice. Bazett's formula, however, overcorrects the QT interval at fast heart rates and undercorrects it at low heart rates. Guidelines and some investigators have recommended using Fridericia's formula (QTc = QT/RR 1/3 ) in these cases, especially in tachycardic subjects. The aim of the present study was to determine cutoffs for QTc suitable for screening pediatric subjects with prolonged QT intervals, based on manually measured values corrected by Fridericia's formula in a large number of subjects. Methods and Results:Three consecutive QT and RR intervals were measured in 4,655, 4,655, and 5,273 1st, 7th, and 10th graders, aged 6, 12, and 15 years, respectively. Each QT interval was corrected by Fridericia's formula, and mean values were calculated. Determination of the cut-offs for screening was based on the prevalence of abnormal electrocardiographic phenotypes of 1:1,164 and on the upper 0.025 percentile in the QTc distribution derived from previous studies. The tentative cut-offs suitable for screening subjects with prolonged QT intervals were 430 ms for 1st graders, 445 ms for 7th graders, and 440 and 455 ms for 10th grade boys and girls, respectively. Conclusions:These tentative cut-offs can be used to screen subjects with prolonged QT intervals in the clinical setting. Further studies are needed to confirm their validity. (Circ J 2010; 74: 1663 - 1669