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BACKGROUND: Fabry disease is characterized by deficient expression/activity of α-GalA with consequent lysosomal accumulation in various organs of its substrate Gb3. Despite enzyme replacement therapy, Fabry disease progresses with serious myocardial, cerebral and renal manifestations. Gb3 accumulation may induce oxidative stress (OxSt), production of inflammatory cytokines and reduction of nitric oxide, which may impact on Fabry diseases clinical manifestations. METHODS: OxSt status was characterized in 10 patients compared with 10 healthy subjects via protein expression of p22phox, subunit of NADH/NADPH oxidase, (Western blot), Heme oxygenase (HO)-1 levels (ELISA), antioxidant/anti-inflammatory, lipid peroxidation as malondialdehyde (MDA) production (colorimetric assay), phosphorylation state of Extracellular Signal Regulated Kinase (ERK)1/2 and Myosin Phosphatase Target Protein (MYPT)-1 (Western blot), marker of Rho kinase activation, both involved in OxSt signaling. Cardiac left ventricular (LV) mass was also evaluated (M-mode echocardiography). RESULTS: LV mass was higher in Fabrys males (123.72±2.03SEM g/m2) and females (132.09±6.72g/m2). p22phox expression was also higher in patients (1.04±0.09 d.u. vs 0.54±0.05 d.u. p

Anorexia nervosa (AN) is characterised by the restriction of energy intake in relation to energy needs and a significantly lowered body weight than normally expected, coupled with an intense fear of gaining weight. Treatment of AN is currently based on psychological and refeeding approaches, but their efficacy remains limited since 40% of patients after 10 years of medical care still present symptoms of AN. The intestine hosts a large community of microorganisms, called the "microbiota", which live in symbiosis with the human host. The gut microbiota of a healthy human is dominated by bacteria from two phyla: Firmicutes and, majorly, Bacteroidetes. However, the proportion in their representation differs on an individual basis and depends on many external factors including medical treatment, geographical location and hereditary, immunological and lifestyle factors. Drastic changes in dietary intake may profoundly impact the composition of the gut microbiota, and the resulting dysbiosis may play a part in the onset and/or maintenance of comorbidities associated with AN, such as gastrointestinal disorders, anxiety and depression, as well as appetite dysregulation. Furthermore, studies have reported the presence of atypical intestinal microbial composition in patients with AN compared with healthy normal-weight controls. This review addresses the current knowledge about the role of the gut microbiota in the pathogenesis and treatment of AN. The review also focuses on the bidirectional interaction between the gastrointestinal tract and the central nervous system (microbiota–gut–brain axis), considering the potential use of the gut microbiota manipulation in the prevention and treatment of AN. [ABSTRACT FROM AUTHOR]

Objective: We investigated fat distribution and tissue-specific insulin-stimulated glucose uptake (GU) in seven fat compartments (visceral and subcutaneous) and skeletal muscle in morbidly obese patients with (T2D) and without (ND) type 2 diabetes before and 6 months after bariatric surgery.Research Design and Methods: A total of 23 obese patients (BMI 43.0 ± 3.6 kg/m(2); 9 T2D and 14 ND) were recruited from a larger, randomized multicenter SLEEVEPASS study. MRI (for fat distribution) and [(18)F]-fluorodeoxyglucose PET (for GU) studies were performed for the obese patients before and 6 months postsurgery; 10 lean subjects served as control subjects and were studied once.Results: At baseline, visceral fat GU was 30 ± 7% of muscle GU in control subjects and 57 ± 5% in obese patients. Visceral and deep subcutaneous fat were more abundant (despite same total fat mass) and less insulin sensitive in T2D than ND; in both, GU was impaired compared with control subjects. Postsurgery, visceral fat mass decreased (∼40%) more than subcutaneous fat (7%). Tissue-specific GU was improved, but not normalized, at all sites in T2D and ND alike. The contribution of visceral fat to whole-body GU was greater in T2D than ND but decreased similarly with surgery. Subcutaneous fat made a fourfold greater contribution to whole-body GU in obese versus lean subjects (15% vs. 4%) both before and after surgery.Conclusions: Bariatric surgery leads to sustained weight loss and improves tissue-specific glucose metabolism in morbidly obese patients. We conclude that 1) enhanced visceral fat accumulation is a feature of T2D, 2) severe obesity compromises muscle insulin sensitivity more than fat insulin sensitivity, and 3) fat mass expansion is a sink for plasma glucose. [ABSTRACT FROM AUTHOR]

Sarcopenia is a progressive clinical syndrome characterized by general loss of skeletal muscle mass and impaired muscle function. Due to the general progressive aging of population, sarcopenia has gradually grown into a global health problem, with great negative impact on the quality of life of the affected patients as well as an adverse societal impact. In this review, we discuss the pathophysiology, diagnosis, and determination of severity of sarcopenia. Special attention is given to the rehabilitative treatment against sarcopenia, pointing out that a thorough evaluation and intervention of nutritional deficits and exercise capacity as well as treatment with medical agents are needed in order to personalize the treatment according to the patient's needs. By targeting goals to improve musculoskeletal and functional disorders associated with chronic conditions, clinicians could apply a therapeutic plan focusing on how to prevent/delay the development of sarcopenia and improve the quality of the patients' lives.

Significant technical advances in small animal molecular imaging techniques and in imaging probes with high specificity for various molecular targets have been produced in the last ten years. Notwithstanding, the clinical applicability of molecular imaging proceeds slowly. In animal experiments, multimodality molecular imaging techniques based on hybrid scanners are increasing, providing more insight into path physiologic phenomena associated with cardiovascular disease. In parallel, we assisted in the development of a new generation of multi-imaging probes, such as PET/MRI probes, particularly effective in hybrid scanners. More recently, in order to gain in inherently low sensitivity of MRI, hyperpolarized magnetic resonance spectroscopy using hyperpolarized (13)C was proposed. Preliminary results obtained in experimental animal studies seem to confirm the potentialities of hyperpolarized (13)C magnetic resonance to monitor myocardial energetics. In this review the preclinical cardiovascular applications and the potential for clinical translation are discussed.

This article reviews the current imaging techniques and the methodologies used to derive quantitative markers of hypertension in the cardiovascular system. Firstly, simple but effective methods to assess regional and global function of large arteries are discussed. After, the role of echocardiography and high-resolution magnetic resonance imaging to assess geometric and mechanical indices of hypertension related cardiac diseases are summarized. In particular, quantitative indices of deformation and strain are derived from quantitative analysis of doppler tissue and tagged magnetic resonance images. Finally, the importance of high field magnetic resonance imaging to assess myocardial microcirculation is described.

This article reviews the current techniques employed to assess endothelial dysfunction in different categories of smokers. Simple but effective methods to assess regional and local properties of large arteries for epidemiologic studies are firstly discussed. After, more complex but accurate image-based methods are described. In particular, the role of high resolution magnetic resonance to quantify, in a single examination, vascular function at different sites of peripheral and central arteries is summarized. Finally, the role of positron emission tomography and magnetic resonance flow mapping are described to assess myocardial microcirculation at rest and under external stressors.