Enhancement of cascaded frequency upconversion by ultrafast temporal correlation of twin beams.

We experimentally and numerically study the effect of ultrafast temporal correlations in two-stage frequency upconversion pumping by using intense twin beams. Enhancement in the upconversion efficiency of each stage due to ultrafast temporal correlation is evaluated by varying the time delay between pumping beams. It is found that the temporal correlation of the twin beams is transferred to the first upconverted beam, thereby also enhancing the efficiency of the second sum-frequency generation (SFG).

Manual compression hemostasis using a hemostatic pad for the distal radial artery approach.

Background: The method of hemostasis for the distal radial approach has not been standardized, although this approach has become increasingly popular due to its advantages. In this study, we investigated the feasibility of manual compression hemostasis using a calcium alginate pad after coronary angiography via the distal radial approach.

Methods: We retrospectively collected 150 consecutive patients (mean age, 74.

T Intratracheal Therapy Alleviates Pulmonary Pathology in an Elastase-Induced Emphysema-Dominant COPD Mouse Model.

Chronic obstructive pulmonary disease (COPD) is a complex pulmonary condition characterized by bronchitis, emphysema, and mucus stasis. Due to the variability in symptoms among patients, traditional approaches to treating COPD as a singular disease are limited. This led us to focus on phenotype/endotype classifications.

Clinical features of two Japanese siblings of neuronal ceroid lipofuscinosis type 1 (CLN1) complicated with TypeⅡ diabetes mellitus.

Neuronal ceroid lipofuscinosis type1(CLN1), is a one form of the group of neuronal ceroid lipofuscinoses (NCLs), which is a neurodegenerative disorder characterized by progressive psychomotor deterioration, ataxia, epilepsy, and visual impairment. Neurological manifestations occur at a wide range of ages, from infancy to adulthood, but are most common in infancy. The prevalence of CLN1 is unclear; however, it is very rare in Japan and Europe.

A scoping review of remote facilitation during simulation-based healthcare education.

Background: Remote facilitation is a synchronous distance education method where instructors facilitate a lesson, in real-time, in physically separate conditions. In this scoping review, we aimed to describe types of remote facilitation used in a healthcare simulation, the influences on learner outcomes, and related factors.

Methods: We accessed PubMed, EMBASE, CINAHL, ERIC, and Web of Science using our search strategies.

Safety and tolerability of agalsidase beta infusions shorter than 90 min in patients with Fabry disease: post-hoc analysis of a Japanese post-marketing study.

Background: Agalsidase beta, an enzyme replacement therapy for Fabry disease, is dosed biweekly at 1 mg/kg body weight, with increasing infusion rates based on tolerability. The US label specifies ≥ 90-min infusions for all patients; the US and EU labels require ≤ 15 mg/hr infusions in patients < 30 kg. The Japanese label allows infusions up to 30 mg/hr, allowing < 90-min dosing for some patients weighing < 45 kg.

Positive Rate and Utility of Blood Culture among Nursing and Healthcare-associated Pneumonia Inpatients: A Cross-sectional Study.

Objective Although blood cultures to identify the presence of bacteremia are recommended for nursing- and healthcare-associated pneumonia (NHCAP), the incidence of true bacteremia and the relationship between true bacteremia and the outcome remain unclear. Physicians can therefore sometimes be confused regarding whether or not blood cultures should be obtained for NHCAP patients. This study assessed the incidence of true bacteremia and the relationship between true bacteremia and the outcome of NHCAP in a Japanese hospital setting.

A case of toxic epidermal necrolysis comorbid with severe burns.

Unlabelled: Toxic epidermal necrolysis (TEN) and severe burns both have high mortality rates, but coexistence is extremely rare. The specificity of developing TEN in burn patients is not well understood and its treatment strategy is not established.

Case Presentation: A 68-year-old man was carried to our hospital with severe burns covering 35% of his body surface area.

Improved waveguide-based ultraviolet light generation and pulsed squeezing at 795 nm.

We report on the waveguide-based generation of pulsed squeezed light at 795 nm, suitable for quantum enhanced measurements with rubidium atoms. Pulsed ultraviolet second harmonic light with a power of more than 400 mW is produced using a periodically poled LiNbO (PPLN) waveguide and is injected into another PPLN waveguide to generate quadrature squeezing. We find that the phase of the second harmonic pulse is shifted within a pulse, and we attribute the shift to heating due to blue-light induced infrared absorption (BLIIRA) from a comparison between the experiment and a numerical simulation.

Characterization of cellular phenotypes in neurons derived from induced pluripotent stem cells of male patients with Fabry disease.

Fabry disease (FD) is an X-linked inherited lysosomal metabolism disorder in which globotriaosylceramide (Gb3) accumulates in various organs resulting from a deficiency in alpha-galactosidase A. The clinical features of FD include progressive impairments of the renal, cardiac, and peripheral nervous systems. In addition, patients with FD often develop neuropsychiatric symptoms, such as depression and dementia, which are believed to be induced by the cellular injury of cerebrovascular and partially neuronal cells due to Gb3 accumulation.

Development of a Highly Sensitive and Rapid Liquid Chromatography-Tandem Mass Spectrometric Method Using a Basic Mobile Phase Additive to Determine the Characteristics of the Urinary Metabolites for Niemann-Pick Disease Type C.

As Niemann-Pick disease type C (NPC) is difficult to diagnose owing to its various clinical symptoms; biomarker tests have been developed. Previously, we revealed urinary sulfated cholesterol metabolites as noninvasive biomarkers for NPC. However, LC/tandem mass spectrometry (LC/MS/MS) requires long separation time and large urine volumes.

A survey on the patient journey in Fabry disease in Japan.

Background: Fabry disease (FD) is a rare X-linked lysosomal storage disease caused by a deficiency in α-galactosidase that is frequently diagnosed late after disease onset. While previous studies have focused on the multisystem manifestations that can lead to delayed or incorrect diagnosis and management, none have investigated the entire patient journey, and few have examined the patient's disease experience.

Objective: To investigate the path to diagnosis from disease onset, and the impact of the disease on daily life, among individuals with FD in Japan.

Generation and characterization of motor neuron progenitors and motor neurons using metachromatic leukodystrophy-induced pluripotent stem cells.

The pathological consequences leading to primary storage, autophagy impairment, impaired mitochondrial dynamics, and endoplasmic reticulum (ER) stress on neural cell dysfunction and apoptosis in metachromatic leukodystrophy (MLD) have been poorly elucidated. In the present study, we generated 2 cell lines of patient-specific-induced pluripotent stem cells (iPSCs) and modeled the progression of pathological events during the differentiation of iPSCs to motor neuron progenitors (MNPs) and mature motor neurons (MNs). The iPS cells were generated from two late-infantile MLD patient-derived skin fibroblasts using electroporation or the Sendai virus.

The role of native T1 values on the evaluation of cardiac manifestation in Japanese Fabry disease patients.

Aims: T1 mapping in cardiac magnetic resonance imaging enables us to distinguish various myocardial diseases showing left ventricular hypertrophy. Fabry disease is a lysosomal storage disorder causing the accumulation of glycosphingolipids into various organs, including the heart, which can be detected by native T1 values in T1 mapping. However, there is no report for the systematic evaluation of native T1 values in Fabry disease in Japan.

Metformin suppresses epithelial sodium channel hyperactivation and its associated phenotypes in a mouse model of obstructive lung diseases.

Chronic obstructive pulmonary disease (COPD) is one of the leading causes of death in the world, and has no radical treatment. Inhibition of amiloride-sensitive epithelial sodium ion channel (ENaC) has now been considered as a potential therapeutic target against COPD. One possible modulator of ENaC is AMP-activated protein kinase (AMPK), a key molecule that controls a wide variety of cellular signals; however, little is known about whether metformin, a clinically available AMPK activator, has a protective role against ENaC-associated chronic pulmonary phenotypes, such as emphysema and pulmonary dysfunction.

Varicella-zoster virus-associated meningitis, encephalitis, and myelitis with sporadic skin blisters: A case report.

Background: Varicella-zoster virus (VZV) generally causes chickenpox at first infection in childhood and then establishes latent infection in the dorsal root ganglia of the spinal cord or other nerves. Virus reactivation owing to an impaired immune system causes inflammation along spinal nerves from the affected spinal segment, leading to skin manifestations (herpes zoster). Viremia and subsequent hematogenous transmission and nerve axonal transport of the virus may lead to meningitis, encephalitis, and myelitis.

Expression of Chimeric HPV-HIV Protein L1P18 in ; Purification and Characterization of the Virus-like Particles.

Currently, three human papillomavirus (HPV) vaccines are already licensed and all of them are based on virus-like particles (VLPs) of HPV L1 capsid protein but not worldwide accessible. While about 38.0 million people were living with HIV in 2019, only 68% of HIV-infected individuals were accessing antiretroviral therapy as of the end of June 2020 and there is no HIV vaccine yet.

Enzyme Replacement Therapy with Pabinafusp Alfa for Neuronopathic Mucopolysaccharidosis II: An Integrated Analysis of Preclinical and Clinical Data.

Enzyme replacement therapy (ERT) improves somatic manifestations in mucopolysaccharidoses (MPS). However, because intravenously administered enzymes cannot cross the blood-brain barrier (BBB), ERT is ineffective against the progressive neurodegeneration and resultant severe central nervous system (CNS) symptoms observed in patients with neuronopathic MPS. Attempts to surmount this problem have been made with intrathecal and intracerebroventricular ERT in order to achieve CNS effects, but the burdens on patients are inimical to long-term administrations.

Attenuated infection by a Pteropine orthoreovirus isolated from an Egyptian fruit bat in Zambia.

Background: Pteropine orthoreovirus (PRV) is an emerging bat-borne zoonotic virus that causes severe respiratory illness in humans. Although PRVs have been identified in fruit bats and humans in Australia and Asia, little is known about the prevalence of PRV infection in Africa. Therefore, this study performed an PRV surveillance in fruit bats in Zambia.

A neuropathological cell model derived from Niemann-Pick disease type C patient-specific iPSCs shows disruption of the p62/SQSTM1-KEAP1-NRF2 Axis and impaired formation of neuronal networks.

Niemann-Pick disease type C (NPC) is a rare neurodegenerative disorder caused by a recessive mutation in the or gene, in which patients exhibit lysosomal accumulation of unesterified cholesterol and glycolipids. Most of the research on NPC has been done in patient-derived skin fibroblasts or mouse models. Therefore, we developed NPC patient neurons derived from induced pluripotent stem cells (iPSCs) to investigate the neuropathological cause of the disease.

Risk assessment and management of PM-bound heavy metals in the urban area of Kitakyushu, Japan.

The sampling campaign of PM was carried out in Kitakyushu City on the western edge of Japan from 2013 to 2019, and 29 heavy metals loaded in PM were measured in this study. During the whole sampling period, the PM mass concentration ranged from 6.3 μg·m to 57.