Monocyte STAT1 phosphorylation and treatment response of JAK inhibitors in chronic nonbacterial osteomyelitis.

Background: Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory disease of unknown cause, predominantly affecting teens and young adults. The early diagnosis and management are challenging due to the lack of reliable diagnostic markers and the occasional intractable cases despite conventional anti-inflammatory treatments. Janus kinase (JAK) inhibitors have recently shown potential utility; however, reports on their use for pediatric patients with CNO remain limited, and no established biomarkers exist to monitor disease activity.

Parental occupational exposure to anticancer drugs and radiation: Risk of fetal loss and physical abnormalities in The Japan Environment and Children's Study.

Background: Many studies have indicated an association between maternal occupational exposure to hazardous agents, such as anticancer drugs and ionizing radiation, and an increased risk of adverse pregnancy outcomes, including stillbirths or miscarriages and physical abnormalities in offspring. However, the effects of recent advancements in protective measures to reduce these risks have not been clarified. Aim To investigate the current impact of parental occupational exposure to anticancer drugs and ionizing radiation on stillbirths or miscarriages as well as physical abnormalities under the circumstances of the developed safety protocols.

Investigation of Teicoplanin Trough Concentrations and Safety Following High-Dose Loading in a Pediatric Population.

Background: Therapeutic drug monitoring-informed teicoplanin dosage adjustments are recommended for safe and effective use. The authors' group previously reported that only half of children reached the recommended blood concentration range at the standard teicoplanin loading dose. It has been suggested that higher loading doses are necessary; however, the usefulness and safety of high-dose loading in pediatric patients in clinical practice are unknown.

Genome Analysis of Japanese Yersinia pseudotuberculosis Strains Isolated From Kawasaki Disease Patients and Other Sources and Their Phylogenetic Positions in the Global Y. pseudotuberculosis Population.

Yersinia pseudotuberculosis (Ypt) is a gram-negative bacterium that infects both humans and animals primarily through fecal‒oral transmission. While Ypt causes acute gastroenteritis in humans, an association with Kawasaki disease (KD), a disease that primarily affects infants and young children and causes multisystemic vasculitis, has also been suspected. Although KD represents a significant health concern worldwide, the highest annual incidence rate is reported in Japan.

Access Program for Unapproved and Off-Label Drug Use in Pediatric BRAF V600E-Mutated Brain Tumors in Japan.

Programs allowing access to investigational drugs and off-label drug use for serious diseases have often been applied to pediatric cancers. A clinical study conducted under the Japanese "Patient-Proposed Healthcare Services" evaluated the efficacy and safety of dabrafenib plus trametinib in children with BRAF V600 mutant glioma (jRCTs071210071). This study successfully provided unapproved and off-label medications to four enrolled patients, two with low-grade glioma and two with high-grade glioma (median age: 10.

Impact of electrical muscle stimulation-induced muscle contractions on endogenous pain modulatory system: a quantitative sensory testing evaluation.

Background: Exercise-induced hypoalgesia (EIH) is characterized by a reduction in pain perception and sensitivity across both exercising and non-exercising body parts during and after a single bout of exercise. EIH is mediated through central and peripheral mechanisms; however, the specific effect of muscle contraction alone on EIH remains unclear. Moreover, previous studies on electrical muscle stimulation (EMS) have primarily focused on local analgesic effects, often relying on subjective pain reports.

Adjunctive effects of eltrombopag on immunosuppressive therapy for childhood aplastic anemia.

Eltrombopag is used with first-line immunosuppressive therapy for adult aplastic anemia, although its practical utility in childhood remains unclear. We retrospectively analyzed the outcomes of pediatric patients who received eltrombopag in Japan. Of the 27 eligible patients, 23 (85%) were previously treated, and 15 (56%) had severe or very-severe disease.

HINODE study: haemophilia A in infancy and newborns - protocol for a prospective, multicentre, observational study evaluating the coagulation potential and safety of emicizumab prophylaxis.

Introduction: Emicizumab prophylaxis is approved for people of all ages with haemophilia A (HA) including infants and children. Although previous studies have demonstrated the efficacy and tolerability of emicizumab in infants with HA, real-world data on emicizumab use in infants are limited. The Haemophilia A in Infancy and NewbOrns: multi-instituional prospective observational study to assess the efficacy anD safety of Emicizumab (HINODE) study aims to evaluate the coagulation potential and safety of emicizumab prophylaxis in infants with congenital HA from birth to <12 months of age.

Comprehensive method for producing high-affinity mouse monoclonal antibodies of various isotypes against (4-hydroxy-3-nitrophenyl)acetyl (NP) hapten.

Monoclonal antibody (mAb) technology has significantly contributed to basic research and clinical settings for various purposes, including protective and therapeutic drugs. However, a rapid and convenient method to generate high-affinity antigen-specific mAbs has not yet been reported. Here, we developed a rapid, easy, and low-cost protocol for antigen-specific mAb production from single memory B cells.

Successful Kidney Transplantation for Bilateral Renal Hypoplasia With Ebstein Disease.

Background: Patients with renal hypoplasia are often associated with congenital heart disease (CHD). During the perioperative period of kidney transplantation (KTx), sufficient circulation volume is required to maintain renal blood flow in the donor kidney. However, little is known about the indication and management of KTx in patients with CHD who require precise hemodynamic assessment during transplantation.

Dural arteriovenous fistulae in a 6-year-old girl with trisomy 21 and congenital heart disease.

Dural arteriovenous fistula (DAVF) represents a pathological group of intracranial shunts arising from the dural artery to venous sinus and veins. Childhood-onset DAVF is generally considered to be poor in prognosis, whereas only limited information is available for the onset and long-term outcomes. We herein report a Japanese girl with trisomy 21, large ventricular septal defects, and pulmonary vein stenosis, for which a transcatheter stent had been placed after birth.

Infants' early recovery from sleep disturbance is associated with a lower risk of developmental delay in the Japan Environment and Children's Study.

To examine whether patterns, such as the timings of onset or recovery from sleep disturbance, are associated with later developmental problems, including autism spectrum disorder (ASD). Mothers participating in the Japan Environment and Children's Study with a child aged 3 years were included in the analyses. Children were assessed for short sleep and frequent awakenings at 1 month, 6 months, and 1 year of age.

Safety and efficacy of ripasudil eye drops in preterm infants with retinopathy of prematurity: phase 1/2, open label, single-arm trial.

Purpose: To assess the safety and efficacy of ripasudil for retinopathy of prematurity (ROP).

Study Design: Phase 1/2, multicenter, open-label, single-arm, 12-week clinical trial.

Methods: Infants born with gestational age (GA) of ≤ 32 weeks or weight of ≤ 1500 g with zone I or II, ≥ stage 1, ROP in both eyes were enrolled.

The immunoreactive signature of monocyte-derived dendritic cells from patients with Down syndrome.

The clinical spectrum of Down syndrome (DS) ranges from congenital malformations to premature aging and early-onset senescence. Excessive immunoreactivity and oxidative stress are thought to accelerate the pace of aging in DS patients; however, the immunological profile remains elusive. We investigated whether peripheral blood monocyte-derived dendritic cells (MoDCs) in DS patients respond to lipopolysaccharide (LPS) distinctly from non-DS control MoDCs.

Potential role of stem cells from human exfoliated deciduous teeth in inducing liver regeneration.

Background And Aim: Even with advancement of medical technologies, liver transplantation still faces several major challenges. Hence, other treatment modalities are urgently needed for patients with end-stage liver disease. Stem cells from human exfoliated deciduous teeth (SHED) was discovered to have highly proliferative and pluripotent properties; including differentiation into hepatocyte-like cells.

Effects of motor imagery using virtual reality on pain sensitivity and affect in healthy individuals: a prospective randomized crossover study.

Objective: Exercise induces a hypoalgesic response and improves affect. However, some individuals are unable to exercise for various reasons. Motor imagery, involving kinesthetic and visual imagery without physical movement, activates brain regions associated with these benefits and could be an alternative for those unable to exercise.