Patient-Reported Outcomes in RLBP1 Retinal Dystrophy: Longitudinal Assessment in a Prospective Natural History Study.

Purpose: To evaluate the performance of two non-disease-specific patient-reported outcome (PRO) instruments, the National Eye Institute Visual Function Questionnaire-25 (VFQ-25) and the Low Luminance Questionnaire (LLQ), in patients with retinaldehyde-binding protein 1 retinal dystrophy (RLBP1 RD).

Methods: PROs were assessed using the VFQ-25 and LLQ. Rasch analysis was conducted to estimate person and item measures of the VFQ-25 and LLQ questionnaires to determine the association between the two PROs.

Utilizing artificial intelligence to determine bone mineral density using spectral CT.

Background: Dual-energy computed tomography (DECT) has demonstrated the feasibility of using HAP-water to respond to BMD changes without requiring dedicated software or calibration. Artificial intelligence (AI) has been utilized for diagnosising osteoporosis in routine CT scans but has rarely been used in DECT. This study investigated the diagnostic performance of an AI system for osteoporosis screening using DECT images with reference quantitative CT (QCT).

Validation of high-sensitivity assays to quantitate cerebrospinal fluid and serum β-galactosidase activity in patients with GM1-gangliosidosis.

GM1-gangliosidosis (GM1) is a lysosomal storage disorder caused by mutations in the galactosidase beta 1 gene () that leads to reduced β-galactosidase (β-gal) activity. This enzyme deficiency results in neuronal degeneration, developmental delay, and early death. A sensitive assay for the measurement of β-gal enzyme activity is required for the development of disease-modifying therapies.

Retinal Dystrophy Associated With RLBP1 Retinitis Pigmentosa: A Five-Year Prospective Natural History Study.

Purpose: To assess the progression in functional and structural measures over a five-year period in patients with retinal dystrophy caused by RLBP1 gene mutation.

Methods: This prospective, noninterventional study included patients with biallelic RLBP1 mutations from two clinical sites in Sweden and Canada. Key assessments included ocular examinations, visual functional measures (best-corrected visual acuity [BCVA], contrast sensitivity [CS], dark-adaptation [DA] kinetics up to six hours for two wavelengths [450 and 632 nm], Humphrey visual fields [HVF], full-field flicker electroretinograms), and structural ocular assessments.

Observation of the B_{c}^{+} Meson in Pb-Pb and pp Collisions at sqrt[s_{NN}]=5.02 TeV and Measurement of its Nuclear Modification Factor.

The B_{c}^{+} meson is observed for the first time in heavy ion collisions. Data from the CMS detector are used to study the production of the B_{c}^{+} meson in lead-lead (Pb-Pb) and proton-proton (pp) collisions at a center-of-mass energy per nucleon pair of sqrt[s_{NN}]=5.02  TeV, via the B_{c}^{+}→(J/ψ→μ^{+}μ^{-})μ^{+}ν_{μ} decay.

Effect of Increased IL-1β on Expression of HK in Alzheimer's Disease.

Alzheimer's disease (AD) is a neurodegenerative disease characterized by decreased glucose metabolism and increased neuroinflammation. Hexokinase (HK) is the key enzyme of glucose metabolism and is associated with mitochondria to exert its function. Recent studies have demonstrated that the dissociation of HK from mitochondria is enough to activate the NOD-like receptor protein 3 (NLRP3) inflammasome and leads to the release of interleukin-1β (IL-1β).

Constraints on the χ_{c1} versus χ_{c2} Polarizations in Proton-Proton Collisions at sqrt[s]=8 TeV.

The polarizations of promptly produced χ_{c1} and χ_{c2} mesons are studied using data collected by the CMS experiment at the LHC, in proton-proton collisions at sqrt[s]=8  TeV. The χ_{c} states are reconstructed via their radiative decays χ_{c}→J/ψγ, with the photons being measured through conversions to e^{+}e^{-}, which allows the two states to be well resolved. The polarizations are measured in the helicity frame, through the analysis of the χ_{c2} to χ_{c1} yield ratio as a function of the polar or azimuthal angle of the positive muon emitted in the J/ψ→μ^{+}μ^{-} decay, in three bins of J/ψ transverse momentum.

Adventitial Cell Atlas of wt (Wild Type) and ApoE (Apolipoprotein E)-Deficient Mice Defined by Single-Cell RNA Sequencing.

Objective- Vascular adventitia encompasses progenitors and is getting recognized as the major site of inflammation in early stage of atherosclerosis. However, the cellular atlas of the heterogeneous adventitial cells, the intercellular communication, the cellular response of adventitia to hyperlipidemia, and its contribution to atherosclerosis have been elusive. Approach and Results- Single-cell RNA sequencing was applied to wt (wild type) and ApoE (apolipoprotein E)-deficient aortic adventitia from 12-week-old C57BL/6J mice fed on normal laboratory diet with early stage of atherosclerosis.

Parity and Components of the Metabolic Syndrome Among US Hispanic/Latina Women: Results From the Hispanic Community Health Study/Study of Latinos.

Background: Physiological adaptations occurring across successive pregnancies may increase the risk of adverse cardiovascular health outcomes in later life.

Methods And Results: The association between parity and metabolic syndrome was examined among 7467 Hispanic/Latina women of diverse backgrounds, aged 18 to 74 years, who participated in the Hispanic Community Health Study/Study of Latinos (HCHS/SOL) from 2008 to 2011. Metabolic syndrome components were defined according to American Heart Association/National Heart, Lung, and Blood Institute criteria and included abdominal obesity, elevated triglycerides, low high-density lipoprotein cholesterol, high blood pressure, and elevated fasting glucose.

Antitumour activities of sesquiterpene lactones from Inula helenium and Inula japonica.

Eight sesquiterpene lactones were isolated from the roots of Inula helenium and flowers of I. japonica. Among them, isoalantolactone (3) and santamarine (6) exhibited significant growth inhibitory activities against gynecologic cancer cell lines, while others weakly inhibited the growth of the cell lines (IC50 < or = 100 microM).

Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL).

The activity and safety of the histone deacetylase inhibitor vorinostat (suberoylanilide hydroxamic acid, SAHA) were evaluated in patients with refractory cutaneous T-cell lymphoma (CTCL). Group 1 received vorinostat 400 mg daily, group 2 received vorinostat 300 mg twice daily for 3 days with 4 days rest, and group 3 received vorinostat 300 mg twice daily for 14 days with 7 days rest followed by 200 mg twice daily. Treatment continued until disease progression or intolerable toxicity.