Case Report: A NR2F1 mutation and clinical characteristics of Bosch-Boonstra-Schaaf optic atrophy syndrome in a Chinese patient.

Purpose: This study aimed to report the clinical characteristics, genetic findings, and treatment outcomes of a Chinese patient with Bosch-Boonstra-Schaaf optic atrophy syndrome (BBSOAS) caused by a mutation in the NR2F1 gene.

Method: A retrospective chart review was conducted, including the patient's medical history, brain magnetic resonance imaging (MRI), electroencephalogram (EEG), and brainstem auditory evoked potential (BAEP) test results. A detailed ophthalmic examination was recorded, including gaze following, fundus photography, flash-electroretinogram (f-ERG), and flash visual evoked potential (f-VEP).

Estimating Sensitivity Maps for X-Nuclei Magnetic Resonance Spectroscopic Imaging.

The purpose of this research is to estimate sensitivity maps when imaging X-nuclei that may not have a significant presence throughout the field of view. We propose to estimate the coil's sensitivities by solving a least-squares problem where each row corresponds to an individual estimate of the sensitivity for a given voxel. Multiple estimates come from the multiple bins of the spectrum with spectroscopy, multiple times with dynamic imaging, or multiple frequencies when utilizing spectral excitation.

Breast cancer pathology image recognition based on convolutional neural network.

This study presents a convolutional neural network (CNN)-based method for the classification and recognition of breast cancer pathology images. It aims to solve the problems existing in traditional pathological tissue analysis methods, such as time-consuming and labour-intensive, and possible misdiagnosis or missed diagnosis. Using the idea of ensemble learning, the image is divided into four equal parts and sixteen equal parts for data augmentation.

Implication of heart rhythm complexity in predicting long-term outcomes in pulmonary hypertension.

Pulmonary hypertension (PH) is a serious disease, however simple tools to predict outcomes are lacking. In our previous investigation, we found that heart rate variability (HRV) and heart rhythm complexity (HRC) were associated with the detection and severity of PH, however their association with PH mortality remains unclear. The aim of this study was to investigate these metrics as a tool for determining long-term outcomes in PH patients.

A pharmacokinetic model for hyperpolarized C-pyruvate MRI when using metabolite-specific bSSFP sequences.

Purpose: Metabolite-specific balanced SSFP (MS-bSSFP) sequences are increasingly used in hyperpolarized [1-C]Pyruvate (HP C) MRI studies as they improve SNR by refocusing the magnetization each TR. Currently, pharmacokinetic models used to fit conversion rate constants, k and k, and rate constant maps do not account for differences in the signal evolution of MS-bSSFP acquisitions.

Methods: In this work, a flexible MS-bSSFP model was built that can be used to fit conversion rate constants for these experiments.

Drp1-dependent mitochondrial fragmentation mediates photoreceptor abnormalities in type 1 diabetic retina.

Recent studies have highlighted that retinal neurodegeneration precedes microvascular changes in diabetic retinopathy (DR), but the specific mechanisms remain unclear. Given the pivotal role of dysfunctional mitochondria and oxidative stress in early DR, our objective was to observe mitochondria-related alterations in the neural retina of type one diabetic mellitus mice with no evidence of DR (T1DM-NDR). We aimed to identify the key mitochondrial-related proteins contributing to mitochondrial injury.

Regional quantification of cardiac metabolism with hyperpolarized [1-C]-pyruvate CMR evaluated in an oral glucose challenge.

Background: The heart has metabolic flexibility, which is influenced by fed/fasting states, and pathologies such as myocardial ischemia and hypertrophic cardiomyopathy (HCM). Hyperpolarized (HP) C-pyruvate MRI is a promising new tool for non-invasive quantification of myocardial glycolytic and Krebs cycle flux. However, human studies of HP C-MRI have yet to demonstrate regional quantification of metabolism, which is important in regional ischemia and HCM patients with asymmetric septal/apical hypertrophy.

Heart rhythm complexity analysis in patients with inferior ST-elevation myocardial infarction.

Heart rhythm complexity (HRC), a subtype of heart rate variability (HRV), is an important tool to investigate cardiovascular disease. In this study, we aimed to analyze serial changes in HRV and HRC metrics in patients with inferior ST-elevation myocardial infarction (STEMI) within 1 year postinfarct and explore the association between HRC and postinfarct left ventricular (LV) systolic impairment. We prospectively enrolled 33 inferior STEMI patients and 74 control subjects and analyzed traditional linear HRV and HRC metrics in both groups, including detrended fluctuation analysis (DFA) and multiscale entropy (MSE).

H/F Substitution Achieved Enantiomeric Organic Inorganic Hybrid Perovskites and Trigonal Structure [DMFP](CdBr)(CdBr).

Organic-inorganic hybrid perovskites (OIHPs) have been emerging as a hot research topic due to their potential applications in energy storage, semiconductors, and electronic devices. Herein, we systematically investigated the synthesis and phase transition behaviors of the enantiomeric OIHPs, () and ()-,-dimethyl-3-fluoropyrrolidinium cadmium bromide ([DMFP][CdBr]), and the hybrid trigonal structure [DMFP] (CdBr)(CdBr). The enantiomers have a mirror-symmetric structure and enhanced solid-state phase transition points of 417 and 443 K, in contrast to the nonfluorinated parent compound, ,-dimethyl-pyrrolidinium cadmium bromide ([DMP][CdBr], 385 K).

Regional quantification of cardiac metabolism with hyperpolarized [1-C]-pyruvate MRI evaluated in an oral glucose challenge.

Background: The heart has metabolic flexibility, which is influenced by fed/fasting states, and pathologies such as myocardial ischemia and hypertrophic cardiomyopathy (HCM). Hyperpolarized (HP) C-pyruvate MRI is a promising new tool for non-invasive quantification of myocardial glycolytic and Krebs cycle flux. However, human studies of HP C-MRI have yet to demonstrate regional quantification of metabolism, which is important in regional ischemia and HCM patients with asymmetric septal/apical hypertrophy.

A metabolite specific 3D stack-of-spirals bSSFP sequence for improved bicarbonate imaging in hyperpolarized [1-C]Pyruvate MRI.

C-bicarbonate is a crucial measure of pyruvate oxidation and TCA cycle flux, but is challenging to measure due to its relatively low concentration and thus will greatly benefit from improved signal-to-noise ratio (SNR). To address this, we developed and investigated the feasibility of a 3D stack-of-spirals metabolite-specific balanced steady-state free precession (MS-bSSFP) sequence for improving the SNR and spatial resolution of dynamic C-bicarbonate imaging in hyperpolarized [1-C]pyruvate studies. The bicarbonate MS-bSSFP sequence was evaluated by simulations, phantoms studies, preclinical studies on five rats, brain studies on two healthy volunteers and renal study on one renal cell carcinoma patient.

Ferroelectric Phase Transition Driven by Switchable Covalent Bonds.

The mechanism on ferroelectric phase transitions is mainly attributed to the displacive and/or order-disorder transition of internal components since the discovery of the ferroelectricity in 1920, rather than the breaking and recombination of chemical bonds. Here, we demonstrate how to utilize the chemical bond rearrangement in a diarylethene-based crystal to realize the light-driven mm2F1-type ferroelectric phase transition. Such a photoinduced phase transition is entirely driven by switchable covalent bonds with breaking and reformation, enabling the reversible light-controllable ferroelectric polarization switching, dielectric and nonlinear optical bistability.

Treatment Response Evaluation by Computed Tomography Pulmonary Vasculature Analysis in Patients With Chronic Thromboembolic Pulmonary Hypertension.

Objective: To quantitatively assess the pulmonary vasculature using non-contrast computed tomography (CT) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) pre- and post-treatment and correlate CT-based parameters with right heart catheterization (RHC) hemodynamic and clinical parameters.

Materials And Methods: A total of 30 patients with CTEPH (mean age, 57.9 years; 53% female) who received multimodal treatment, including riociguat for ≥ 16 weeks with or without balloon pulmonary angioplasty and underwent both non-contrast CT for pulmonary vasculature analysis and RHC pre- and post-treatment were included.

High- Single-Component Organosilicon Ferroelectric Crystal Obtained by H/F Substitution.

Organic single-component ferroelectrics are highly desirable for their low molecular mass, light weight, low processing temperature, and excellent film-forming properties. Organosilicon materials with a strong film-forming ability, weather resistance, nontoxicity, odorlessness, and physiological inertia are very suitable for device applications related to the human body. However, the discovery of high- organic single-component ferroelectrics has been very scarce, and the organosilicon ones even less so.

Axial-Chiral BINOL Multiferroic Crystals with Coexistence of Ferroelectricity and Ferroelasticity.

Chirality exists everywhere from natural amino acids to particle physics. The introduction of point chirality has recently been shown to be an efficient strategy for the construction of molecular ferroelectrics. In contrast to point chirality, however, axial chirality is rarely used to design ferroelectrics so far.

Development of specialized magnetic resonance acquisition techniques for human hyperpolarized [ C, N ]urea + [1- C]pyruvate simultaneous perfusion and metabolic imaging.

Purpose: This study aimed to develop and demonstrate the in vivo feasibility of a 3D stack-of-spiral balanced steady-state free precession(3D-bSSFP) urea sequence, interleaved with a metabolite-specific gradient echo (GRE) sequence for pyruvate and metabolic products, for improving the SNR and spatial resolution of the first hyperpolarized C-MRI human study with injection of co-hyperpolarized [1- C]pyruvate and [ C, N ]urea.

Methods: A metabolite-specific bSSFP urea imaging sequence was designed using a urea-specific excitation pulse, optimized TR, and 3D stack-of-spiral readouts. Simulations and phantom studies were performed to validate the spectral response of the sequence.

H/F Substitution on the Spacer Cations Leads to 1D-to-2D Increment of the Pyrrolidinium-Containing Lead Iodide Hybrid Perovskites.

Hybrid organic-inorganic perovskites (HOIPs) have emerged as multifunctional materials with remarkable optical and electronic properties. In particular, 2D-layered lead iodide-based HOIPs possess great practical application potential in the photoelectric field. In this work, we report H/F substitution-induced 1D-to-2D increment of lead iodide HOIPs.

H/F Substitution induced switchable coordination bonds in a cyano-bridged hybrid double perovskite ferroelastic.

A three-dimensional cyano-bridged double perovskite ferroelastic [(CH)NCHF][KFe(CN)] was constructed by introducing unprecedented switchable C-F-K coordination bonds. H/F substitution not only preserves the basic structure of the parent [(CH)N][KFe(CN)] but also affords an 3̄F2/-type ferroelastic phase transition.

Association between Levels of Urine Di-(2-ethylhexyl)phthalate Metabolites and Heart Rate Variability in Young Adults.

Phthalate exposure is associated with cardiovascular risk. Among the various phthalates, di-(2-ethylhexyl) phthalate (DEHP) is a deleterious plasticizer in our daily lives. This study investigated the association between DEHP exposure and the alteration of heart rate variability (HRV).

The Value of Heart Rhythm Complexity in Identifying High-Risk Pulmonary Hypertension Patients.

Pulmonary hypertension (PH) is a fatal disease-even with state-of-the-art medical treatment. Non-invasive clinical tools for risk stratification are still lacking. The aim of this study was to investigate the clinical utility of heart rhythm complexity in risk stratification for PH patients.

Metabolic imaging with hyperpolarized C pyruvate magnetic resonance imaging in patients with renal tumors-Initial experience.

Background: Optimal treatment selection for localized renal tumors is challenging because of their variable biologic behavior and limitations in the preoperative assessment of tumor aggressiveness. The authors investigated the emerging hyperpolarized (HP) C magnetic resonance imaging (MRI) technique to noninvasively assess tumor lactate production, which is strongly associated with tumor aggressiveness.

Methods: Eleven patients with renal tumors underwent HP C pyruvate MRI before surgical resection.

Di-chromatic interpolation of magnetic resonance metabolic images.

Objective: Magnetic resonance imaging with hyperpolarized contrast agents can provide unprecedented in vivo measurements of metabolism, but yields images that are lower resolution than that achieved with proton anatomical imaging. In order to spatially localize the metabolic activity, the metabolic image must be interpolated to the size of the proton image. The most common methods for choosing the unknown values rely exclusively on values of the original uninterpolated image.