Cardiovascular magnetic resonance findings in Danon disease: a case series of a family

BackgroundCardiac involvement constitutes the primary cause of mortality in patients with Danon disease (DD). This study aimed to explore the cardiac magnetic resonance (CMR) features and progressions of DD cardiomyopathies in a family with long-term follow-up.MethodsSeven patients (five females and two males), belonging to the same family and afflicted with DD, were enrolled in this study between 2017 and 2022. The cardiac structure, function, strain, tissue characteristics on CMR and their evolutions during follow-up were analyzed.ResultsThree young female patients (3/7, 42.86%) exhibited normal cardiac morphology. Four patients (4/7, 57.14%) displayed left ventricle hypertrophy (LVH), and mostly with septal thickening (3/4, 75%). A single male case (1/7, 14.3%) showed decreased LV ejection fraction (LVEF). Nonetheless, the global LV strain of the four adult patients decreased in different degree. The global strain of adolescent male patients was decreased compared to the age-appropriate female patients. Five patients (5/7, 71.43%) exhibited late gadolinium enhancement (LGE), with proportion ranging from 31.6% to 59.7% (median value 42.7%). The most common LGE location was the LV free wall (5/5, 100%), followed by right ventricle insertion points (4/5, 80%) and intraventricular septum (2/5, 40%). Segmental radial strain (rs = −0.586), circumferential strain (r = 0.589), and longitudinal strain (r = 0.514) were all moderately correlated with the LGE proportions of corresponding segments (P < 0.001). T2 hyperintense and perfusion defect foci were identified, overlapping with the LGE areas. During follow-up, both the young male patients exhibited notable deterioration of their cardiac symptoms and CMR. The LVEF and strain decreased, and the extent of LGE increased year by year. One patient underwent T1 mapping examination. The native T1 value was sensitively elevated even in regions without LGE.ConclusionsLeft ventricular hypertrophy, LGE with sparing or relatively less involved IVS, and LV dysfunction are prominent CMR features of Danon cardiomyopathy. Strain and T1 mapping may have advantages in detecting early-stage dysfunction and myocardial abnormalities in DD patients, respectively. Multi-parametric CMR can serve as an optimal instrument for detecting DD cardiomyopathies

Identification, characterization and in vitro neuroprotection of N-6-(4-hydroxybenzyl) adenine riboside and its metabolites

N-6-(4-hydroxybenzyl) adenine riboside (NHBA), isolated from Gastrodia elata Blume, has been demonstrated to show great pharmacological effects. The present study aimed to synthesize and identify the metabolites of NHBA, and to determine their neuroprotective potentials in vitro. After incubation with rat liver microsomes in the presence of NADPH, two metabolites were detected, which were further semisynthesized and identified as N-6-(4-hydroxylbenzyl) purine (NHBP) and N-6-(3,4-dihydroxylbenzyl) adenine riboside (ONHBA) by UPLC-QTOF-MS, H-1 NMR and C-13 NMR. Furthermore, the neuroprotective activities of NHBA and two metabolites were evaluated in SH-SY5Y cells. Our results demonstrated that NHBA substantially protected against H2O2-induced neuronal death in SH-SY5Y cells. Moreover, both ONHBA and NHBP could significantly prevent A beta oligomers-and H2O2-induced neuronal death in SH-SY5Y cells. These results suggested that NHBA and its metabolites, ONHBA and NHBP, might be suitable for the development of new drugs in the treatment of neurodegenerative diseases, including Alzheimer's disease in particular

Band Engineering of BiFeO3 Nanosheet for Boosting Hydrogen Evolution by Synergetic Piezo-photocatalysis

Hydrogen (H2) production through water splitting using sustainable energy sources is gaining increasing attention. However, limited catalytic performance based on a single driving force presents both opportunities and challenges for achieving a high-efficiency H2 output. Exploring multiple driving sources to enhance the comprehensive catalytic performance shows great promise. BiFeO3 (BFO), with features of narrow band gap and ultrahigh ferroelectric polarizations, is considered as a promising alternative candidate for green H2 production from water splitting. However, the inherent overpositive conduction band edge restricts its applications. Therefore, a strategy involving band engineering through heteroatom Co-doping (Cox-BFO) has been proposed to boost piezo-photocatalytic H2 evolution performance. Based on the synergetic effects of the polarization field induced by piezoelectric Co1.8-BFO, suppressed recombination of charge carriers, prolonged charge carrier lifetime, modulated band alignment, and lowered energy barrier for surface H2O adsorption/activation/reduction, the Co1.8-BFO nanosheets exhibit significant piezo-photocatalytic activities with an enhanced H2 generation rate (321.9 μmol·g-1·h-1). That rate is much higher than those of piezocatalytic (127 μmol·g-1·h-1) and photocatalytic (163 μmol·g-1·h-1) performances alone. This work provides an effective approach to advancing the BFO water splitting performance through band engineering

Output Characteristics of External-Cavity Mode-Hop-Free Tunable Laser Source in C+L Band

Tunable laser sources with a wide wavelength tuning range, mode-hop-free (MHF) operation, and high spectral purity are essential for applications such as high-resolution spectroscopy, coherent detection, and intelligent fiber sensing. In this paper, we present a wide-range tunable laser source that operates without mode hopping, based on external cavity feedback using a semiconductor gain chip as the laser gain medium. The wavelength, power, and spectral characteristics of the laser are experimentally measured. A wide MHF continuous wavelength tuning range from 1480 nm to 1620 nm with a side-mode suppression ratio of more than 61.65 dB is achieved. An output optical power of more than 11.14 dBm with good power stability can also be realized in the full C+L band. This proposed external-cavity tunable laser source features a narrow intrinsic linewidth and MHF tunable radiation with a maximum sweep speed of 200 nm/s, enabling practical applications such as high-resolution vector spectrum analysis