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An analysis of 64 patients with congenital stationary night blindness showed that all had essentially normal fundi. Electroretinography (ERG) showed a normal a wave with extremely reduced b wave (negative type) when recorded with a single bright white stimulus in the dark. We classified these patients into two groups based on the evaluation of rod ERG(More)
The on- and off-responses of photopic electroretinogram (ERG) were recorded by rectangular light stimuli in 13 patients with complete type and 9 patients with incomplete type of congenital stationary night blindness. The positive on-response (b-wave) was significantly smaller in both types than in normal subjects (P less than 0.001). The rapid off-response(More)
BACKGROUND RNA interference (RNAi) has the potential to be a novel therapeutic strategy in diverse areas of medicine. Here, we report on targeted RNAi for the treatment of heart failure, an important disorder in humans that results from multiple causes. Successful treatment of heart failure is demonstrated in a rat model of transaortic banding by RNAi(More)
BACKGROUND Heart failure (HF) remains a major cause of morbidity and mortality in North America. With an aging population and an unmet clinical need by current pharmacologic and device-related therapeutic strategies, novel treatment options for HF are being explored. One such promising strategy is gene therapy to target underlying molecular anomalies in the(More)
We found an unusual form of macular dystrophy in three patients from two generations of the same family. The fundi of these patients appeared normal by ophthalmoscopy and fluorescein angiography, even in an older patient and in patients with poor visual acuity. Results of full-field electroretinograms were also normal in both cone and rod components. Focal(More)
AIMS There are few data comparing the fate of multipotent progenitor cells (MPCs) used in cardiac cell therapy after myocardial infarction (MI). To document in vivo distribution of MPCs delivered by intracoronary (IC) injection. METHODS AND RESULTS Using an anterior MI swine model, near-infrared (NIR) fluorescence was used for in vivo tracking of labelled(More)
Among the various cardiac contractility parameters, left ventricular (LV) ejection fraction (EF) and maximum dP/dt (dP/dt(max)) are the simplest and most used. However, these parameters are often reported together, and it is not clear if they are complementary or redundant. We sought to compare the discriminative value of EF and dP/dt(max) in assessing(More)
The experience of using a novel application of intravascular ultrasound (IVUS)-guided percutaneous coronary interventions for chronic total occlusions is reported in 2 cases. In the first case, an IVUS catheter was advanced into a side branch to identify the entry point of the major branch. In the second case, IVUS-guided penetration of the guidewire from(More)
BACKGROUND Pulmonary arterial hypertension (PAH) is characterized by dysregulated proliferation of pulmonary artery smooth muscle cells leading to (mal)adaptive vascular remodeling. In the systemic circulation, vascular injury is associated with downregulation of sarcoplasmic reticulum Ca(2+)-ATPase 2a (SERCA2a) and alterations in Ca(2+) homeostasis in(More)
OBJECTIVES The aim of this study was to examine the effects of sarcoplasmic reticulum Ca(2+) ATPase (SERCA2a) gene transfer in a swine heart failure (HF) model. BACKGROUND Reduced expression and activity of SERCA2a have been documented in HF. Prior studies have reported the beneficial effects of short-term SERCA2a overexpression in rodent models. However,(More)