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.