Background: Dexmedetomidine (DEX) is a highly selective alpha 2 receptor agonist that has the advantage of causing less respiratory depression than other sedative agents. We evaluated the add-on effects of DEX on sedation among pediatric patients who received midazolam and pentazocine during cardiac catheterization.
Methods: 120 cardiac catheterization procedures in 110 patients under deep sedation at Department of Pediatrics, Kanazawa University Hospital from January 2013 to August 2018: 63 procedures without DEX (i.
Background: Children born with very low birth weight (VLBW) are at higher risk for cognitive impairment, including language deficits and sensorimotor difficulties. Voice-evoked response (P1m), which has been suggested as a language development biomarker in young children, remains unexplored for its efficacy in VLBW children. Furthermore, the relation between P1m and sensory difficulties in VLBW children remains unclear.
View Article and Find Full Text PDFChronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by molecular defects in nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. p67-CGD is an autosomal recessive CGD, which is caused by a defect in the cytosolic components of NADPH oxidase, p67, encoded by NCF2. We previously established a flow cytometric analysis for p67 expression, which allows accurate assessment of residual protein expression in p67-CGD.
View Article and Find Full Text PDFThis phase 3, open-label, multidose study (NCT04346108) evaluated the pharmacokinetics, safety, tolerability, and efficacy of immunoglobulin subcutaneous (human) 20% solution (Ig20Gly) administered weekly and every 2 weeks in Japanese patients with primary immunodeficiency diseases (PIDs). The study was conducted at eight study sites in Japan and enrolled patients aged ≥2 years with PIDs treated using a stable intravenous immunoglobulin dose for ≥3 months prior to the study. Patients received intravenous immunoglobulin every 3 or 4 weeks at pre-study dose (200-600 mg/kg) for 13 weeks (Epoch 1), subcutaneous Ig20Gly (50-200 mg/kg) once weekly for 24 weeks (Epoch 2), and Ig20Gly (100-400 mg/kg) every 2 weeks for 12 weeks (Epoch 3).
View Article and Find Full Text PDFAim: This study examined the associations between geriatric factors and decreased opportunities for conversation among older adults amid a period of self-restraint during the COVID-19 pandemic.
Methods: A cross-sectional questionnaire-based survey was carried out in October 2020. The participants were 204 residents aged ≥65 years staying at a private care home in Kyoto city, Japan.
Aim: To examine the actual conditions of older patients receiving home medical care after hospitalization over a period of 2 years in Japan.
Methods: The study population included 102 participants, aged ≥65 years, receiving home medical care, who consented to participate in the Osaka Home Care Registry (OHCARE) study in Japan over a period of 2 years. We investigated the actual conditions for returning home after hospitalization.
Background Despite several rapid influenza diagnostic tests (RIDTs), they are predicting whether a patient has influenza before rapid testing is important. Here, we assessed factors predictive of a positive flu test via RIDTs by combining interviews and physical examination. Methods We analyzed the relationship between interviews and physical findings and results of RIDTs using multivariable logistic regression.
View Article and Find Full Text PDFOligomeganephronia is a congenital anomaly of the kidney and urinary tract. It is often categorized as one of the hypoplastic kidney conditions. The pathological diagnosis of oligomeganephronia is challenged by the absence of clear diagnostic criteria, which often leads to subjective interpretations by pathologists.
View Article and Find Full Text PDFJuvenile xanthogranuloma (JXG) is usually identified by Touton giant cells, so their absence can complicate diagnosis. We encountered a case of non-typical neonatal JXG lacking Touton giant cells, which was difficult to differentiate from aleukemic leukemia cutis because of overlapping histopathological characteristics. A 1 month-old girl presented with a blueberry muffin rash and multiple 1-2 cm nodules within the subcutaneous and deeper soft tissues.
View Article and Find Full Text PDFChronic active Epstein-Barr virus disease (CAEBV), formerly named chronic active Epstein-Barr virus infection, is characterized by systemic inflammation and clonal proliferation of Epstein-Barr virus (EBV)-infected T or NK cells. As CAEBV is a potentially life-threatening illness, appropriate diagnosis and therapeutic interventions are necessary for favorable clinical outcomes. Substantial evidence regarding the pathogenesis and treatment of CAEBV has been accumulated since previous guidelines for the diagnosis of CAEBV were proposed.
View Article and Find Full Text PDFNeurodegenerative Langerhans cell histiocytosis (ND-LCH) manifests several years after onset of LCH, with progressive neurological symptoms and characteristic brain imaging features. Although ND-LCH has a dismal neurological prognosis, distinct treatment strategies are not available owing to the unknown pathophysiology. We describe the case of a 6-year-old boy who developed left convergent strabismus four years after onset of multisystem LCH (MS-LCH).
View Article and Find Full Text PDFJ Allergy Clin Immunol
October 2023
Background: IL-18 and IL-1β play a central role in the pathogenesis of systemic juvenile idiopathic arthritis and its life-threatening complication, macrophage activation syndrome (MAS).
Objectives: This study aimed to clarify the role of IL-18 and IL-1β in the pathogenesis of MAS.
Methods: We developed a mouse model to evaluate the role of each cytokine with Toll-like receptor 9 stimulation after continuous infusion with IL-18, IL-1β, and a combination of both for 7 days.
Background: Evaluation of type I interferons (IFNs) in inflammatory or autoimmune diseases is challenging because of their rapid clearance in peripheral blood. The IFN gene expression signature has recently been used to evaluate the IFN status; however, this is often a labor-intensive and time-consuming procedure. Therefore, we assessed the feasibility of measuring expression of an IFN-inducible protein, CD169 (Siglec-1), on monocytes and circulating levels of soluble CD169 as alternative markers for type I IFN status in various pediatric inflammatory diseases.
View Article and Find Full Text PDFAcute myeloid leukemia (AML) with chromosome 7 abnormalities has a dismal prognosis due to a poor complete remission (CR) rate after induction chemotherapy. Although various salvage therapies for refractory AML have been developed for adults, few salvage therapies are available for children. Here, we report the cases of three patients with refractory AML with chromosome 7 abnormalities (Patient 1, with inv(3)(q21;3q26.
View Article and Find Full Text PDFAim: Although the relationship between impaired glucose tolerance (IGT) and mortality has been investigated in diverse populations, few studies have focused on older populations. This study aimed to investigate the relationship between glucose tolerance and overall mortality among populations aged ≥75 years.
Methods: Data were obtained from the Tosa Longitudinal Aging Study, a community-based cohort survey conducted in Kochi, Japan.
Immunoglobulin A vasculitis (IgAV) is the most common vasculitis of childhood. However, its etiology remains unknown. In the Mediterranean region, 10% of patients with IgAV harbor homozygous and compound heterozygous mutations in the Mediterranean fever () gene.
View Article and Find Full Text PDFThe development of hematopoietic stem cell (HSCs) gene therapy for DNA repair disorders, such as Fanconi anemia and Bloom syndrome, is challenging because of the induction of HSCs apoptosis by cytokine stimulation. Although the Baboon envelope pseudotyped lentiviral vector (BaEV-Rless-LV) has been reported as a non-stimulatory gene transfer tool, the virus titer of BaEV-Rless-LV is too low for use in clinical applications. Transfected 293 T cells with helper plasmids, including the BaEV-Rless plasmid, showed morphological changes, such as syncytium formation and detachment.
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