We identified a human multiprotein complex (WINAC) that directly interacts with the vitamin D receptor (VDR) through the Williams syndrome transcription factor (WSTF). WINAC has ATP-dependent chromatin-remodeling activity and contains both SWI/SNF components and DNA replication-related factors. The latter might explain a WINAC requirement for normal S phase progression. WINAC mediates the recruitment of unliganded VDR to VDR target sites in promoters, while subsequent binding of coregulators requires ligand binding. This recruitment order exemplifies that an interaction of a sequence-specific regulator with a chromatin-remodeling complex can organize nucleosomal arrays at specific local sites in order to make promoters accessible for coregulators. Furthermore, overexpression of WSTF could restore the impaired recruitment of VDR to vitamin D regulated promoters in fibroblasts from Williams syndrome patients. This suggests that WINAC dysfunction contributes to Williams syndrome, which could therefore be considered, at least in part, a chromatin-remodeling factor disease.

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Intravascular or intracardiac stenoses occur in many forms of congenital heart disease or after attempted surgical repair. Although balloon dilation is one option for management, restenosis can occur due to elastic recoil immediately after the procedure. To address to such stenotic lesions, many reports support implanting endovascular stents to provide a framework for vessel expansion. Both balloon-expandable fixed tubular mesh stainless steel devices, and self-expandable stents have had an extensive clinical application. In pediatric patients, stents are used for a variety of stenoses, such as systemic venous obstruction pathways (eg, Mustard, Fontan baffle, or bidirectional cavopulmonary connections), pulmonary artery, right ventricular to pulmonary conduits, aortic coarctation, the arterial duct, aorticopulmonary collaterals, or postoperative systemic to pulmonary shunts. Because of improvements in device profile, implantation rates have increased. Complications such as stent fracture, migration, aneurysm formation, and in-stent restenosis occur but only rarely. This latter event may be because of intimal hyperplasia and/or continued vessel (and patient) growth related to the stent diameter. As such, some instances require redilation to manage the acquired lesion. Stent application has importantly altered management algorithms in congenital heart disease.

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Thirteen children (seven male) with coronary artery fistula underwent percutaneous transcatheter occlusion. The age range was 8 months to 14 years (mean, 6.3 years). The fistulas had their origins from the right coronary artery (six), from the left anterior descending coronary artery (three), and from the left circumflex coronary artery (four). Drainage was to the right ventricle (seven), the right atrium (three), and one each to the pulmonary artery, left atrium, and superior caval vein. The fistulas were closed with coils in 10 patients, a Rashkind double-umbrella device in 1 patient, and an Amplatzer Duct Occluder in 2 patients. Complete occlusion was achieved in 9 of 13 patients. Complications consisted of migration of coils in four and transient arrhythmias or changes in the resting electrocardiogram in four patients. Follow-up studies 1 to 31 months (mean, 14.6 months) after occlusion noted only four patients with trivial (clinically insignificant) residual shunts. Owing to various coronary fistula morphologies, transcatheter occlusion requires availability of different embolization techniques. Short-term follow-up supports persistent clinical efficacy and transcatheter closure techniques as the initial form of therapy.

This persistent expression of ICAM-1 suggests that myocardial cell damage may persist immunologically for a long period in myocarditis. In addition, immunostaining for these adhesion molecules may be diagnostic value in clinically silent lymphocytic myocarditis and chronic cardiomyopathy.