solated noncompaction of the ventricular myocardium (INVM) is a rare disorder characterized by excessively prominent trabeculations and deep intertrabecular recesses, mainly in the left ventricle without other congenital cardiac malformations. 1 Most patients with INVM show various abnormalities on the electrocardiogram (ECG), including Wolff-Parkinson-White (WPW) syndrome, 2,3 which is currently classified into several types based on the localization of the bypass tract. It has not previously been reported which type of WPW syndrome occurs in patients with INVM. 3,4 We report 3 patients with INVM and WPW syndrome type B on ECG, which has not been previoulsy reported. 3,4 Case Reports Patient 1A 9-month-old Japanese girl was admitted to hospital because of increasing dyspnea. She was the first child of healthy nonconsanguineous parents, and the pregnancy and delivery had been uneventful.On admission, she was thin and malnourished: weight 6 kg and height 56 cm, both below the 10th percentile. She was pale and had cyanosis of the lips and nail bed. On auscultation, there was tachypnea and moderate retraction of the chest wall with moist rales over the entire lung field. A grade 3 pansystolic murmur was noted at the apex. Her abdomen was moderately distended, with the liver palpable 3 cm below the right costal margin. Chest X-ray showed marked cardiac enlargement, and pulmonary venous congestion. She died suddenly at 11 months of age. Patient 2The 6-month-old younger sister of Patient 1 was referred for examination of her cardiac function. The pregnancy and delivery had been uneventful and she was currently alert and healthy. Her heart sounds were clear and the rhythm was regular without murmur.Chest X-ray showed mild cardiac enlargement with a cardiothoracic ratio of 0.61. One year later, she still did not have symptoms, but her cardiac performance had deteriorated slightly, with a left ventricular fractional shortening (LVFS) of 24% and an ejection fraction (LVEF) of 57%. She was treated with oral angiotensin-converting enzyme inhibitor. Patient 3A 9-year-old girl was referred for an outpatient examination of an arrhythmia diagnosed previously during a school physical examination. She was alert and healthy with clear heart sounds and no murmur. No cardiac enlargement was noted on the chest roentgenogram. Electrocardiography, Echocardiography and Magnetic Resonance ImagingThe ECG of Patient 1 revealed marked tachycardia of 150 beats/min and WPW syndrome type B, with localization of the accessory pathway in the right anteroseptal area (Fig 1A). Cardiac performance was markedly depressed. A 2-dimensional (D) echocardiogram (Fig 1B) and a T1-weighted magnetic resonance imaging (MRI) scan (Fig 1C) revealed numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses, but there was no evidence of endocardial thrombus over the left ventricular wall.The ECG from Patient 2 revealed a normal sinus rhythm and WPW syndrome type B, with localization of the accessory pathway in the right anteros...

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C3 activator in sera of patients with agammaglobulinemia shows higher levels compared with age-matched healthy controls. This fact may be due to a compensatory function of the alternative pathway of the complement system to make up for the defect of production of antibody in these patients.

(Objective) Intravenous gamma globulin (IVGG) treatment is the most important therapy in the Kawasaki disease (KD), however there are many uncertainties about active mechanism of this treatment. This time, we examined active mechanisms of IVGG treatment affecting on apoptosis of neutrophils. (Patients and controls) Fifteen subjects (4 months ϳ 4 years old) were selected from the KD patients who had been admitted to Dokkyo University Hospital between June, 2000 and May, 2001. Healthy adults were used as controls.(Materials and methods) (1) Neutrophils were separated from heparinized blood by the specific gravity centrifugation. (2) Venoglobulin-I was used as human IgG for this experiment. (3) Apoptosis was determined with microscopic examination and flow cytometry. DNA quantity of the cells stained with PI alone and PI and anexin V was measured in flow cytometry.(Results) (1) Spontaneous apoptosis on KD patient neutrophils was more delayed than that on controls, as the picking illness day was earlier. (2) When healthy adult neutrophils was cultured in the presence of IgG (1mg/ml), apoptosis was promoted, but this effect was not observed under less than 0.1mg/ml IgG. Therefore, IgG was used at the concentration of 1mg/ml in further experiments. (3) The morphological changes unlike control neutrophils were observed on KD patient neutrophils after the culture in the presence of IgG. (4) Based on apoptosis pattern of neutrophils in the presence of IgG, samples were divided into two groups, promoted example and not. The promotion of apoptosis by the IgG addition in vitro was observed in about half of samples from the patients, in which IVGG gave more remarkable effects clinically. (Conclusions) We considered the possibility in which one part of the clinical effects of IVGG treatment had appeared by promoting the apoptotic effects to neutrophils.