Despite its notoriously mild phenotype, the dystrophin-deficient mdx mouse is the most common model of Duchenne muscular dystrophy (DMD). By mimicking a human DMD-associated metabolic comorbidity, hyperlipidemia, in mdx mice by inactivating the apolipoprotein E gene (mdx-ApoE) we previously reported severe myofiber damage exacerbation via histology with large fibro-fatty infiltrates and phenotype humanization with ambulation dysfunction when fed a cholesterol- and triglyceride-rich Western diet (mdx-ApoE). Herein, we performed comparative lipidomic and metabolomic analyses of muscle, liver and serum samples from mdx and mdx-ApoE mice using solution and high-resolution-magic angle spinning (HR-MAS) H-NMR spectroscopy.
View Article and Find Full Text PDFBackground: Atriofascicular fibers (AFFs) are rare accessory pathways that have higher rates of recurrence after ablation because of either failure to identify AFF (M) potentials or mechanical termination with contact.
Objective: We aimed to evaluate whether electroanatomic mapping (EAM) using multielectrode, high-density nonlinear catheters can reliably localize AFF potentials and determine a site for ablation without causing mechanical termination.
Methods: Seven patients underwent electrophysiology studies (EPS) and EAM using high-density, multielectrode catheters for antidromic tachycardia using AFFs.
Weekly Steroids in Muscular Dystrophy (WSiMD) was a pilot study to evaluate once weekly prednisone in patients with Limb Girdle and Becker muscular dystrophy (LGMD and BMD, respectively). At study endpoint, there were trends towards increased lean mass, reduced fat mass, reduced creatine kinase and improved motor function. The investigation was motivated by studies in mouse muscular dystrophy models in which once weekly glucocorticoid exposure enhanced muscle strength and reduced fibrosis.
View Article and Find Full Text PDFObjective: The objective of this study is to evaluate the diagnostic performance of urine cytology using The Paris System (TPS 2.0) in comparison with TPS 1.0, and the Four-Tier Reporting System (FTRS) of our institute for identifying high-grade urothelial carcinoma (HGUC).
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