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In order to study the in vitro effects of intact immunoglobulin (Ig) and gamma-interferon (INF-gamma) in patients with Kawasaki disease, the production of tumour necrosis factor-alpha (TNF-alpha) and interleukin-1 beta (IL-1 beta) was measured in peripheral blood monocytes (PBM) both before and after intravenous immunoglobulin (IVIG) therapy. Spontaneous production of TNF-alpha and IL-1 beta both before and after IVIG therapy was significantly higher than in healthy controls. Intact Ig enhanced in vitro the production of TNF-alpha and IL-1 beta both before and after IVIG therapy approximately 3-4 times as compared to the spontaneous production. INF-gamma did not affect the production of the two cytokines. Ig enhanced IL-1 beta mRNA expression in PBM of KD by 3-8 times more than that of spontaneous production. [ABSTRACT FROM AUTHOR]

Primary pulmonary vein stenosis (PVS) is rare within the pediatric population and its pathophysiology remains unclear, especially as to how the histopathology relates to its refractoriness to treatment. We report the case of a 4-month-old girl with primary PVS. The lesion in this patient was characterized by fatal obstruction of intraparenchymal small pulmonary veins, associated with localized stenosis at the four pulmonary veno-atrial junctions. All four localized stenoses underwent transcatheter stent implantation. Although the procedure was technically successful, her clinical status failed to improve, and she died 2 months after stenting. Histopathological examination of lung specimens showed severe luminal obstruction by marked intimal proliferation with fibrosis in the intraparenchymal small pulmonary veins, and these findings were present in every lobe. To the best of our knowledge, the histopathological findings and clinical course in this case, including the response to treatments, are extremely rare. We suggest that the histological findings of the small pulmonary veins are important in deciding the indication and appropriate timing of intervention. < Learning objective: The outcome of primary pulmonary vein stenosis has remained poor despite aggressive treatment, while the indication and appropriate timing of intervention has not been clarified. The histological findings of small pulmonary veins are important to predict the response to treatment and outcome, and early intervention might prevent the secondary progression of this disease.>.

Recently, transcatheter device occlusion has become the first choice treatment for adult persistent ductus arteriosus (PDA). However, various complications such as atrial fibrillation requiring anticoagulation, pulmonary hypertension, and ventricular dysfunction may challenge the interventionist. We report a 61-year-old patient with a large PDA complicated by left ventricular dysfunction, atrial fibrillation, and left atrial thrombus. Computed tomography documented the PDA of Krichenko type A with the narrowest diameter of 8 mm. We successfully closed the PDA using an Amplatzer duct occluder under anticoagulation with wafarin. His post-operative course was complicated by ventricular tachycardia and deteriorating left ventricular pump function. Although endomyocardial biopsy from the left ventricle showed myocardial hypertrophy and interstitial fibrosis, possibly caused by chronic volume overload, left ventricular pump function improved dramatically with restoration of sinus rhythm during follow-up. Left ventricular dysfunction, even when associated with histological changes, may be nearly normalized by volume unloading in an adult with a large PDA. < Learning objective: Transcatheter device occlusion has become the first choice for adult persistent ductus arteriosus (PDA). It is important to note various complications associated with transcatheter occlusion of PDA in adult, such as arrhythmia, pulmonary hypertension, and ventricular dysfunction. Temporally dysfunction of left ventricular (LV) systolic performance usually occurs following PDA closure, because of reduced muscle fiber stretch by the sudden reduction in LV volume overload and increased LV afterload. Although histological remodeling of LV may be caused by volume overload due to significant left-to-right shunt in adults, volume unloading can, not only prevent further deterioration of LV function, but also may also reverse the substrate of arrhythmia.>.

Kawasaki disease (KD) was considered to be good prognosis in self-limited at first. But, sudden death followed KD has been reported. It became clear that KD produce the peculiar state of myocardial infarction in infants and young children. Now, KD took the place of the rheumatic fever and got the most important disease of acquired heart disease in childhood. Cardiovascular disorder is mainly included with the coronary arterial aneurysm. And aneurysm in an subaxial and iliac artery were complicated. Every organ flame in a whole body was produced with systematic vasculitis mainly on a medium size or small size artery of a whole body. We summarize about complications of a cardiovascular system and the other organs followed KD.

Based on the nationwide database of Japan in January 2006, a cumulative number of the patients exceeded 200,000, while 90,000 patients are now older than 20 years old. The Japan Kawasaki Disease Committee established the guideline of its management in 2002. In adult patients with coronary sequelae, there is no doubt on the necessity of life-long-follow-up, while there is still arguments on how do we care the adult patients with no or mild coronary involvements in the acute phase which regressed spontaneously. The coronary artery sequelae of Kawasaki disease may be an important cause of ischemic heart disease in young adults, particularly under 40 years of age. In this review, we discuss how we care adult patients with a history of Kawasaki disease with coronary sequelae. Finally, we recommend opening the outpatient office for adult patients with a history of Kawasaki disease, because the frequency where adult patients followed Kawasaki disease stops consulting a medical institution became high.