KDL indices may be helpful in discriminating KDL from lymphadenitis at admission. It is important to monitor the symptoms of KD in a patient with three or four KDL indices at admission.

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). ...

We present a case of a 9-year-old boy with nemaline myopathy and dilated cardiomyopathy. The combination of nemaline myopathy and cardiomyopathy is rare, and this is the first reported case of dilated cardiomyopathy associated with childhood-onset nemaline myopathy. A novel mutation, p.W358C, in ACTA1 was detected in this patient. An unusual feature of this case was that the patient' s cardiac failure developed during early childhood with no delay of gross motor milestones. The use of a b-blocker did not improve his clinical course, and the patient died 6 months after diagnosis of dilated cardiomyopathy. Congenital nonprogressive nemaline myopathy is not necessarily a benign disorder: deterioration can occur early in the course of dilated cardiomyopathy with neuromuscular disease, and careful clinical evaluation is therefore necessary.