Evaluation of medication withdrawal in patients with non-systemic juvenile idiopathic arthritis in Japan using a web-based survey.

Objectives: Although treatments for juvenile idiopathic arthritis (JIA) have seen considerable advancements, there remains a lack of clear guidelines on withdrawing medications. This study aimed to investigate the current strategies for discontinuing non-systemic JIA treatment.

Methods: A web-based questionnaire was distributed to members of the Pediatric Rheumatology Association of Japan.

Survey of diversity awareness and environment among members of the Pediatric Rheumatology Association of Japan.

Background: Diversity management has gained traction in Japan. The Pediatric Rheumatology Association of Japan (PRAJ) has an Advisory Committee for Diversity Promotion with a broader focus on promoting diversity. The objectives of this study were to better understand the problems faced by PRAJ members regarding the work environment, childcare and nursing care, and work-life balance.

CCIVR2 facilitates comprehensive identification of both overlapping and non-overlapping antisense transcripts within specified regions.

Pairs of sense and antisense transcriptions that are adjacent at their 5' and 3' regions are called divergent and convergent transcription, respectively. However, the structural properties of divergent/convergent transcription in different species or RNA biotypes are poorly characterized. Here, we developed CCIVR2, a program that facilitates identification of both overlapping and non-overlapping antisense transcripts produced from divergent/convergent transcription whose transcription start sites (TSS) or transcript end sites (TES) are located within a specified region.

Transition of Patients with Childhood-onset Rheumatic Diseases to Adult Medical Care in Japan.

Medical advances in childhood-onset chronic diseases (CCDs) have significantly improved the prognosis of these diseases; however, they have also resulted in an increase in the number of cases requiring continued medical care in adulthood. The transition from the pediatric to adult healthcare system has recently received worldwide attention. In Japan, in the last decade, there has been a growing awareness of the transition to adult health care in each specialized chronic disease area with a childhood onset.

[Significant Prolongation of the International Normalized Ratio Associated with COVID-19 Treatment: Possible Drug Interaction with Remdesivir].

A 55-year-old man with hypertrophic cardiomyopathy and a pacemaker was admitted with coronavirus disease 2019 (COVID-19). Before admission, the patient's medications included amiodarone, diltiazem, bisoprolol, atorvastatin, etizolam, and warfarin (WF). After admission, dexamethasone (DXM) and remdesivir (RDV) were initiated for treating COVID-19.

Pre-Vaccination Anti-Severe Acute Respiratory Syndrome Coronavirus 2 Antibody Seroprevalence in Workers at Three Japanese Hospitals.

Background: Antibody testing is essential for accurately estimating the number of people infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). This study aimed to investigate the influence of background factors on seroprevalence by testing for anti-SARS-CoV-2 antibodies in blood samples obtained from the staff of three hospitals.

Methods: This cross-sectional observational study was conducted from June 8 to July 4, 2020, as part of a mandatory health examination.

Orchestration of Ion Channels and Transporters in Neocortical Development and Neurological Disorders.

Electrical activity plays crucial roles in neural circuit formation and remodeling. During neocortical development, neurons are generated in the ventricular zone, migrate to their correct position, elongate dendrites and axons, and form synapses. In this review, we summarize the functions of ion channels and transporters in neocortical development.

Simultaneous two-photon imaging of action potentials and subthreshold inputs in vivo.

To better understand the input-output computations of neuronal populations, we developed ArcLight-ST, a genetically-encoded voltage indicator, to specifically measure subthreshold membrane potentials. We combined two-photon imaging of voltage and calcium, and successfully discriminated subthreshold inputs and spikes with cellular resolution in vivo. We demonstrate the utility of the method by mapping epileptic seizures progression through cortical circuits, revealing divergent sub- and suprathreshold dynamics within compartmentalized epileptic micronetworks.

Toll signalling promotes blastema cell proliferation during cricket leg regeneration via insect macrophages.

Hemimetabolous insects, such as the two-spotted cricket Gryllus bimaculatus, can recover lost tissues, in contrast to the limited regenerative abilities of human tissues. Following cricket leg amputation, the wound surface is covered by the wound epidermis, and plasmatocytes, which are insect macrophages, accumulate in the wound region. Here, we studied the function of Toll-related molecules identified by comparative RNA sequencing during leg regeneration.

Cartilage-hair Hypoplasia Complicated with Liver Cirrhosis Due to Chronic Intrahepatic Cholestasis.

We herein report a rare case of cartilage-hair hypoplasia (CHH) complicated with liver cirrhosis. A 20-year-old Japanese man with CHH was found incidentally to have liver cirrhosis and an esophageal varix. This patient had been treated for infections due to immunodeficiency since early childhood.

Involvement of Netrins and Their Receptors in Neuronal Migration in the Cerebral Cortex.

In mammals, excitatory cortical neurons develop from the proliferative epithelium and progenitor cells in the ventricular zone and subventricular zone, and migrate radially to the cortical plate, whereas inhibitory GABAergic interneurons are born in the ganglionic eminence and migrate tangentially. The migration of newly born cortical neurons is tightly regulated by both extracellular and intracellular signaling to ensure proper positioning and projections. Non-cell-autonomous extracellular molecules, such as growth factors, axon guidance molecules, extracellular matrix, and other ligands, play a role in cortical migration, either by acting as attractants or repellents.

Comparative Evaluation of Genetically Encoded Voltage Indicators.

Imaging voltage using fluorescent-based sensors could be an ideal technique to probe neural circuits with high spatiotemporal resolution. However, due to insufficient signal-to-noise ratio (SNR), imaging membrane potential in mammalian preparations is still challenging. In recent years, many genetically encoded voltage indicators (GEVIs) have been developed.

Flexible Nanopipettes for Minimally Invasive Intracellular Electrophysiology In Vivo.

Intracellular recordings in vivo remains the best technique to link single-neuron electrical properties to network function. Yet existing methods are limited in accuracy, throughput, and duration, primarily via washout, membrane damage, and movement-induced failure. Here, we introduce flexible quartz nanopipettes (inner diameters of 10-25 nm and spring constant of ∼0.

Hematopoietic stem cell transplantation for progressive combined immunodeficiency and lymphoproliferation in patients with activated phosphatidylinositol-3-OH kinase δ syndrome type 1.

Background: Activated phosphatidylinositol-3-OH kinase δ syndrome type 1 (APDS1) is a recently described primary immunodeficiency syndrome characterized by recurrent respiratory tract infections, lymphoid hyperplasia, and Herpesviridae infections caused by germline gain-of-function mutations of PIK3CD. Hematopoietic stem cell transplantation (HSCT) can be considered to ameliorate progressive immunodeficiency and associated malignancy, but appropriate indications, methods, and outcomes of HSCT for APDS1 remain undefined.

Objective: Our objective was to analyze the clinical manifestations, laboratory findings, prognosis, and treatment of APDS1 and explore appropriate indications and methods of HSCT.

Simultaneous two-photon imaging and two-photon optogenetics of cortical circuits in three dimensions.

The simultaneous imaging and manipulating of neural activity could enable the functional dissection of neural circuits. Here we have combined two-photon optogenetics with simultaneous volumetric two-photon calcium imaging to measure and manipulate neural activity in mouse neocortex in vivo in three-dimensions (3D) with cellular resolution. Using a hybrid holographic approach, we simultaneously photostimulate more than 80 neurons over 150 μm in depth in layer 2/3 of the mouse visual cortex, while simultaneously imaging the activity of the surrounding neurons.

Encapsulated pleural effusion due to biotype II in a child with trisomy 21: A case report and literature review.

(Hi) can colonize in the upper respiratory tract and cause severe pulmonary infections, especially among immunocompromised children. Herein, we report a case of left encapsulated pleural effusion (EPE) due to Hi in a 24-month-old girl with trisomy 21. She was already vaccinated against Hi type b.

Haploinsufficiency of TNFAIP3 (A20) by germline mutation is involved in autoimmune lymphoproliferative syndrome.

Background: Autoimmune diseases in children are rare and can be difficult to diagnose. Autoimmune lymphoproliferative syndrome (ALPS) is a well-characterized pediatric autoimmune disease caused by mutations in genes associated with the FAS-dependent apoptosis pathway. In addition, various genetic alterations are associated with the ALPS-like phenotype.

Imprinting and recalling cortical ensembles.

Neuronal ensembles are coactive groups of neurons that may represent building blocks of cortical circuits. These ensembles could be formed by Hebbian plasticity, whereby synapses between coactive neurons are strengthened. Here we report that repetitive activation with two-photon optogenetics of neuronal populations from ensembles in the visual cortex of awake mice builds neuronal ensembles that recur spontaneously after being imprinted and do not disrupt preexisting ones.

Control of Spontaneous Ca2+ Transients Is Critical for Neuronal Maturation in the Developing Neocortex.

Neural activity plays roles in the later stages of development of cortical excitatory neurons, including dendritic and axonal arborization, remodeling, and synaptogenesis. However, its role in earlier stages, such as migration and dendritogenesis, is less clear. Here we investigated roles of neural activity in the maturation of cortical neurons, using calcium imaging and expression of prokaryotic voltage-gated sodium channel, NaChBac.

Universal acquired melanosis: carbon baby.

We report a 3-year-old boy born with light brown skin that progressively became much darker. The color change was insidious in onset at the age of 3 months, asymptomatic, and progressive involving the entire body surface. Hyperpigmentation may be congenital or acquired, hereditary or nonhereditary, localized or universal, of known or unknown origin.

A novel strategy for hemolytic uremic syndrome: successful treatment with thrombomodulin α.

Hemolytic uremic syndrome (HUS) is a life-threatening infectious disease in childhood for which there is no confirmed therapeutic strategy. Endothelial inflammation leading to microthrombosis formation via complement activation is the main pathology of HUS. Thrombomodulin is an endothelial membrane protein that has anticoagulation and anti-inflammatory effects, including the suppression of complement activity.

Dysfunction of KCNK potassium channels impairs neuronal migration in the developing mouse cerebral cortex.

Development of the cerebral cortex depends partly on neural activity, but the identity of the ion channels that might contribute to the activity-dependent cortical development is unknown. KCNK channels are critical determinants of neuronal excitability in the mature cerebral cortex, and a member of the KCNK family, KCNK9, is responsible for a maternally transmitted mental retardation syndrome. Here, we have investigated the roles of KCNK family potassium channels in cortical development.