Association of the immunogenicity of intramuscular SARS-CoV-2 mRNA vaccination with computed tomography muscle images in patients with muscular disorders.

Backgrounds: Intramuscular mRNA vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have a low intensity and latency of antibody response in patients with muscular disorders (MDs). However, the mechanisms involved in this phenomenon remain unknown. This study aimed to clarify the mechanism of the low immunogenicity of intramuscular SARS-CoV-2 mRNA vaccination in patients with MDs.

Urinary titin reflects the severity of walking ability, muscle strength, and muscle and cardiac damage in patients with Becker muscular dystrophy.

Background: Becker muscular dystrophy (BMD) is a dystrophinopathy caused by a pathological variant of the DMD gene. Urinary titin, a degradation product of the giant protein titin present in muscle sarcomeres, has been used as a biomarker to reflect muscle degradation in Duchenne muscular dystrophy, a more severe dystrophinopathy. However, the clinical significance of urinary titin levels in BMD remains unclear.

Safety and efficacy of viltolarsen treatment in patients with Duchenne muscular dystrophy: A retrospective study with 3-year follow-up.

Background: Duchenne muscular dystrophy (DMD) is a hereditary neuromuscular disorder characterized by severe, progressive muscle wasting. Viltolarsen, a formulation consisting of exon 53-skipping antisense oligonucleotides of the dystrophin gene, has been indicated for some patients with DMD. However, reports describing the efficacy and safety of viltolarsen treatment in patients with DMD, particularly those comparing patients receiving viltolarsen with age- and time-period-matched controls, are limited.

High-efficiency green light emission from InGaN/GaN using localized surface plasmon resonance tuned by combination of Ag nanoparticles and dielectric thin film.

We achieved significant enhancements in green light emission (550 nm) from InGaN/GaN quantum wells (QWs) by tuning the localized surface plasmon resonance (LSPR) of self-assembled Ag nanoparticles (NPs) through the application of a SiO thin film. The LSPR wavelength of Ag NPs was shifted towards shorter wavelengths by 80 nm using a 5 nm SiO layer to separate Ag NPs from GaN surface, thereby aligning it effectively with the green region. This strategic placement of Ag NPs and a 5 nm SiO film resulted in significant enhancements of photoluminescence (PL) by 15- and 8.

Comparison of Analgesic Efficacy and Safety of Low-Dose Transdermal Fentanyl and Oral Oxycodone in Opioid-Naïve Patients with Cancer Pain.

In Japan, a low-dose transdermal fentanyl (TDF; 0.5 mg) has been approved to address pain in opioid-naïve patients with cancer; however, efficacy and safety data are lacking. To determine the efficacy and safety of TDF, patients with opioid-naïve cancer pain prescribed TDF (0.

Later efficacy of nusinersen treatment in adult patients with spinal muscular atrophy: A retrospective case study with a median 4-year follow-up.

Background: Spinal muscular atrophy (SMA) is a hereditary neuromuscular disorder characterized by skeletal muscle atrophy and weakness. New treatments for SMA have been developed namely, the drugs nusinersen, onasemnogene abeparvovec, and risdiplam. However, there are limited reports on their effects on adult patients with SMA, particularly over long periods.

Flexible topographical design of light-emitting diodes realizing electrically controllable multi-wavelength spectra.

Multi-wavelength visible light emitters play a crucial role in current solid-state lighting. Although they can be realized by combining semiconductor light-emitting diodes (LEDs) and phosphors or by assembling multiple LED chips with different wavelengths, these design approaches suffer from phosphor-related issues or complex assembly processes. These challenges are significant drawbacks for emerging applications such as visible light communication and micro-LED displays.

Immunogenicity of SARS-CoV-2 mRNA intramuscular vaccination in patients with muscular disorders.

Backgrounds: Little clinical data is available on severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in patients with muscular disorders (MDs). The immunogenicity of SARS-CoV-2 vaccines against MDs, in particular, remains unknown. Thus, this study aimed to confirm the immunogenicity and safety of the SARS-CoV-2 vaccine against MDs.

Efficacy of interferential current transcutaneous electrical sensory stimulation through the neck skin for treating dysphagia in children with disabilities: A case series.

Finding a suitable treatment for dysphagia has been challenging and the efficacy of neuromuscular electrical stimulation has been recognized. Moreover, the beneficial effect of interferential current transcutaneous electrical sensory stimulation has recently been described. However, the efficacy of interferential current transcutaneous electrical sensory stimulation in children with disabilities is unknown.

Intellectual disability and abnormal cortical neuron phenotypes in patients with Bloom syndrome.

Bloom syndrome (BS) is a rare autosomal recessive disorder characterized by genomic instability that leads to various complications, including cancer. Given the low prevalence of BS in Japan, we conducted a nationwide survey. We recruited eight patients with BS, three of whom exhibited intellectual disability.

RNA-seq analysis, targeted long-read sequencing and in silico prediction to unravel pathogenic intronic events and complicated splicing abnormalities in dystrophinopathy.

Dystrophinopathy is caused by alterations in DMD. Approximately 1% of patients remain genetically undiagnosed, because intronic variations are not detected by standard methods. Here, we combined laboratory and in silico analyses to identify disease-causing genomic variants in genetically undiagnosed patients and determine the regulatory mechanisms underlying abnormal DMD transcript generation.

Pediatric Advance Care Planning for a Patient with a Severe Motor and Intellectual Disorder through Cooperation between an Acute Care Hospital and a Residential Facility.

Background: With the rapid progress of medical technology, the number of children with medical complexities who require advanced medical care, including mechanical ventilators, has been increasing steadily in Japan. Accordingly, the issue of how to provide holistic care and support for the entire life of the children with severe motor and intellectual disabilities (SMID) who live at home has become a new challenge.

Case Presentation: We present the case of a three-year-old boy with SMID due to HHV-6B-induced hemorrhagic shock encephalopathy who was cared for at home by the home visit medical team of Osaka Developmental Rehabilitation Center (ODRC; residential facilities with the department of home medical treatment and care).

Association between Happiness and Economic Status among Older Adults in Two Myanmar Regions.

Few studies have examined whether objective or subjective economic status (ES) has a greater association with the happiness of older adults, despite concerns regarding the growing economic cost of morbidity and their functional dependence in developing countries with aging populations. Thus, this study examined whether objective/subjective ES was associated with happiness in older adults in two Myanmar regions. A multistage random sampling procedure and face-to-face interviews were conducted in the urban and rural areas of Myanmar.

Microscopic origin of thermal droop in blue-emitting InGaN/GaN quantum wells studied by temperature-dependent microphotoluminescence spectroscopy.

To elucidate the microscopic origin of the thermal droop, a blue-emitting indium gallium nitride (InGaN) quantum well grown on epitaxially laterally overgrown gallium nitride was investigated using temperature-dependent microphotoluminescence spectroscopy. Below 300 K, the sample exhibited a well-known dislocation-tolerant luminescence behavior. However, as temperature increases from 300 K to 500 K, the near band-edge emission at the wing region (with lower threading dislocation densities) was stronger than that at the seed region (with higher threading dislocation densities), indicating that threading dislocations are the microscopic origin of the thermal droop.

Access to HPV vaccination in Japan: Increasing social trust to regain vaccine confidence.

Vaccine hesitancy is a growing concern in global public health, and illustrates serious problems arising from loss of social trust. Japan is experiencing a human papillomavirus (HPV) vaccine crisis that started with a rapid decline in the vaccination rate in 2013 from approximately 70% to less than 1% and lasting for 7 years. We analyze Japan's case of vaccine hesitancy for HPV vaccine, using a framework for examining barriers to access and use of health technologies according to four categories: architecture, availability, affordability, and adoption.

A web-based questionnaire survey on the influence of coronavirus disease-19 on the care of patients with muscular dystrophy.

To clarify the influence of coronavirus disease-19 (COVID-19) on the care of muscular dystrophy patients, we performed a questionnaire survey that was posted on the internet on May 11, 2020. By the end of July 2020, 542 responses had been collected. Approximately 30% of patients postponed regular consultations, and one-quarter of patients who received consultation more than once a month used telephone consultations.

Factors associated with high care burden of primary caregivers of children with medical complexity after completing a discharge-support program in a recovery center.

Introduction: Recently, many seriously ill children requiring medical equipment are being recommended to transition from hospital to home care in Japan. Since 2011, our recovery center has provided a support program for the transfer process from hospital to home for ill children and their families. The purpose of this study was to evaluate the factors related to high care burden after completing the discharge-support program.