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.
SUMMARY:To determine the role of cytokines in acute myocarditis, we examined expressional patterns of cardiotrophin-1 (CT-1), TNF-␣, and IL-1␣ 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-␣ and IL-1␣ expressions were investigated on histological sections from each heart, and mRNA expression of TNF-␣, IL-1␣, 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. TNF␣ and IL-1␣ 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 TNF␣ and IL-1␣ 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-␣ and IL-1␣ 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. (Lab Invest 2000, 80:433-440).V iral myocarditis results from a viral infection that induces myocardial necrosis and triggers a series of immune responses to eliminate the viral agent. Although several studies have examined the crucial roles of cytokines and the induction of apoptosis in the myocardium, the pathogenic mechanisms of myocarditis remain unclear
The clinical course of ischemic heart disease due to Kawasaki disease was analyzed. The subjects (children aged two months to eight years) were divided into two groups. Group 1 (n = 23) consisted of children who had sustained myocardial infarction (MI) and group 2 (n = 13) of those without clinical symptoms or signs of MI, but in whom signs of an obstructive lesion had appeared on coronary arteriography during the follow-up period. Changes in the left ventricular ejection fraction (LVEF) and the appearance of coronary arterial lesions on first and second angiography were analyzed in the two groups. It was found that (a) LVEF (51.4 +/- 13.4%, mean +/- SD) at the first study, obtained after MI in group 1, was significantly lower than that (64.3 +/- 3.7%) at the second one in group 2, which revealed recently developed obstructive lesions; (b) there was no significant difference between the two groups as to the severity of stenotic lesions on coronary arteriography; and (c) comparison of LVEF at the first angiography with that at the second study showed significant improvement in group 1 (1st, 54.2 +/- 12.0%; and 2nd, 60.8 +/- 9.7%) and significant depression in group 2 (1st, 68.1 +/- 4.4%; and 2nd, 64.3 +/- 3.7%).
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.
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.
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