Masahiko Okuno

Expressional Patterns of Cytokines in a Murine Model of Acute Myocarditis: Early Expression of Cardiotrophin-1

To determine the role of cytokines in acute myocarditis, we examined expressional patterns of cardiotrophin-1 (CT-1), TNF-α, and IL-1α in a murine model of acute myocarditis. Ten-day-old Institute of Cancer Research mice were injected with Coxsackievirus B3 and killed on Days 1, 2, 3, 4, 5, 7, 10, 14, and 28 of injection. TNF-α and IL-1α expressions were investigated on histological sections from each heart, and mRNA expression of TNF-α, IL-1α, and CT-1 in the heart was examined by reverse transcription-polymerase chain reaction and RNase protection assay. To determine myocardial regeneration, cardiomyocytic DNA synthesis was investigated using bromodeoxyuridine on Days 3, 5, 7, and 10, and the labeling index was calculated in each heart. Age-matched uninfected mice were used as controls. TNFα and IL-1α expression was first detected in the cardiomyocytes on Day 3 and reached the maximum level on Day 7, when inflammatory changes were most prominent. Although an increased expression of TNFα and IL-1α mRNA was also detected on Day 3, CT-1 mRNA expression was distinctly augmented on Day 2. The labeling indices in the hearts with myocarditis were significantly higher than in those of the controls in all of the time points examined. CT-1 expression preceded TNF-α and IL-1α expressions and active DNA synthesis in a murine model of acute myocarditis. All CVB3-infected mice with anti-glycoprotein-130 antibody treatment died within 6 days. CT-1 may exert a protective role by modulating cytokine production and by inducing cardiomyocytic proliferation in CVB3-infected murine hearts.

Bernard-Soulier syndrome associated with 22q11.2 microdeletion

We describe a Japanese girl with Bernard-Soulier syndrome and 22q11.2 microdeletion. She had viral infections and recurrent thrombocytopenia and hemorrhagic diathesis after cardiac surgery. As congenital heart defects and abnormal immunity are the most common clinical manifestations associated with 22q11.2 deletion, patients with this association may have a greater risk of developing a severe bleeding disorder.

Association of atrial septal defect with Poland-Moebius syndrome : Vascular disruption can be a common etiologic factor. -A case report-

The authors describe an association of atrial septal defect with partial symptoms of the Poland-Moebius syndrome. Both are thought to be caused by developmental disorders of the mesenchyme and ectodermal derivatives. This anomalous association can be accepted as one concept of the subclavian artery blood supply disruption sequence during embryo genesis.

Effects of Intracoronary Tissue-Type Plasminogen Activator Treatment in Kawasaki Disease and Acute Myocardial Infarction

We retrospectively studied 3 patients with Kawasaki disease (KD) and acute myocardial infarction (AMI) who were treated with intracoronary administration of tissue-type plasminogen activator (t-PA). Two-dimensional echocardiogram on the next day of the treatment revealed reduction of thrombus and improvement of the cardiac function in all 3 patients. However, a 12-month-old patient treated with 200,000 U/kg of t-PA at 48 h after the onset of AMI died of recurrent myocardial infarction. The other 2 patients treated with 400,000 and 800,000 U/kg, respectively, showed clear, though not prompt, improvement in clinical symptoms and laboratory data. The intracoronary thrombolytic therapy using high-dose t-PA appears effective in treating AMI associated with KD.

Case of Williams elfin facies syndrome with pulmonary artery sling

We report the presence of pulmonary artery sling in a 4-year-old girl with the Williams elfin facies syndrome. A history of heavy and persistent wheezing during the first few months of life, associated with the current presentation of heart murmur and pulmonary stenosis, led us to investigate the presence of pulmonary artery sling. Both pulmonary artery sling and cardiovascular anomalies in Williams elfin facies syndrome have previously been linked to developmental anomalies of the 6th aortic arch. This appears to be the second reported case of this rare combination of anomalies.

Transesophageal Atrial Pacing in Paroxysmal Supraventricular Tachycardia in Infants and Children

Fourteen patients with paroxysmal supraventricular tachycardia (PSVT) underwent serial electrophysiologic studies using transesophageal atrial pacing (EPS‐EP), to determine the predictive value of this method in the selection of antiarrhythmic therapy and to study the mechanism of the tachycardia.