Publications by authors named "Iori Sato"

Article Synopsis
  • Biliary atresia (BA) is a serious liver condition caused by abnormal development of bile ducts, leading to liver damage and possible cirrhosis; type I cystic BA is a rare but important form of this condition.
  • Researchers have created a set of human induced pluripotent stem cells (hiPSCs) from five Japanese patients with type I cystic BA, which show potential for self-renewal and differentiation.
  • Whole genome analysis revealed mutations linked to hepatobiliary issues in these patients, and studying these hiPSCs may help improve understanding of the disease and lead to new treatments.
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Polyploidy or whole-genome duplication (WGD) is a major event that drastically reshapes genome architecture and is often assumed to be causally associated with organismal innovations and radiations. The 2R hypothesis suggests that two WGD events (1R and 2R) occurred during early vertebrate evolution. However, the timing of the 2R event relative to the divergence of gnathostomes (jawed vertebrates) and cyclostomes (jawless hagfishes and lampreys) is unresolved and whether these WGD events underlie vertebrate phenotypic diversification remains elusive.

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Article Synopsis
  • - This study aimed to evaluate the natural progression of venous malformation (VM) and Klippel-Trenaunay Syndrome (KTS) over a 6-month period by observing changes in lesion volume and other health factors in 34 patients.
  • - The results showed no significant overall change in lesion volume or other health metrics such as pain, quality of life, and coagulation markers, but some patients had notable changes linked to local infections.
  • - Findings suggest that conducting a Phase 2 study to test new treatments without a placebo is justified, using this data as a standard for comparison.
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Although recent advances in genome editing technology with homology-directed repair have enabled the insertion of various reporter genes into the genome of mammalian cells, the efficiency is still low due to the random insertion of donor vectors into the host genome. To efficiently select knocked-in cells without random insertion, we developed the "double-tk donor vector system," in which the expression units of the thymidine kinase of herpes simplex virus (HSV-tk) are placed on both outer sides of homology arms. This system is superior in enriching knocked-in human induced pluripotent stem cells (hiPSCs) than conventional donor vector systems with a single or no HSV-tk cassette.

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Background: The number of adult patients with childhood-onset chronic diseases is increasing. However, the process of transitioning these patients from child- to adult-centered medical services faces many difficulties. Despite the key role that doctors in the pediatric field are considered to play in transition, few fact-finding surveys about transition have been conducted among these doctors.

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Cardiomyocytes derived from human induced pluripotent stem cells (hiPSCs) are a promising cell source for regenerative medicine and drug discovery. However, the use of animal models for studying human cardiomyocytes derived from hiPSCs in vivo is limited and challenging. Given the shared properties between humans and zebrafish, their ethical advantages over mammalian models, and their immature immune system that is rejection-free against xenografted human cells, zebrafish provide a suitable alternative model for xenograft studies.

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Background: The empowerment of families raising children with disabilities (CWD) is crucial in maintaining their health. We developed an evidence-based, family empowerment intervention program focusing on social resource utilization and reducing care burden.

Objective: This study aimed to determine the program's effectiveness in promoting family empowerment.

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Background: Families raising children with disabilities assume risks to their health and lives. Therefore, it is necessary to support these families to improve family empowerment, which is the ability of these families to control their own lives and to promote the collaborative raising of children with disabilities. This is the first online intervention program focusing on the empowerment of families raising children with disabilities who live at home in Japan.

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Nitric oxide (NO) is an ancestral key signalling molecule essential for life and has enormous versatility in biological systems, including cardiovascular homeostasis, neurotransmission and immunity. Although our knowledge of NO synthases (Nos), the enzymes that synthesize NO , is substantial, the origin of a large and diversified repertoire of gene orthologues in fishes with respect to tetrapods remains a puzzle. The recent identification of in the ray-finned fish spotted gar, which was considered lost in this lineage, changed this perspective.

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Dilated cardiomyopathy (DCM) is a refractory heart disease characterized by dilation of the left ventricle and systolic dysfunction. LMNA, the gene encoding lamin A/C (a nuclear envelope protein), is the second leading causative gene associated with familial DCM. LMNA-related DCM is likely to develop severe heart failure, various types of arrhythmias, and poor prognosis.

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It is recommended that patients with childhood-onset chronic diseases (CCD) be transferred from pediatric to adult healthcare systems when they reach adulthood. Transitional support helps adolescents with CCD transition smoothly. Transition readiness is one of the key concepts to assess the efficacy of transitional support programs.

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Background: The feasibility of tyrosine kinase inhibitor (TKI) discontinuation in pediatric chronic myeloid leukemia (CML) remains to be fully elucidated.

Procedures: TKI was prospectively discontinued in patients who were diagnosed with CML at <20 years of age, treated with TKI for ≥3 years, and sustained molecular response 4.0 (MR4.

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Background: The endostyle is an epithelial exocrine gland found in non-vertebrate chordates (amphioxi and tunicates) and the larvae of modern lampreys. It is generally considered to be an evolutionary precursor of the thyroid gland of vertebrates. Transformation of the endostyle into the thyroid gland during the metamorphosis of lampreys is thus deemed to be a recapitulation of a past event in vertebrate evolution.

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Background: PedsQL Infant Scales (PedsQL-I) are used to assess parent-reported health-related quality of life for children younger than 2 years. We determined the feasibility, reliability, and validity of the Japanese version of the PedsQL-I.

Methods: A total of 183 participants (parents) with infants aged 1-30 months were recruited from 8 day care centers and one pediatric clinic.

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Article Synopsis
  • * Treatment mainly involves chemotherapy and radiotherapy, with surgery being limited to certain cases; however, standardizing treatment is difficult due to the rarity of these tumors across institutions.
  • * In response to a higher incidence of GCTs in East Asia, the Japan Society for Neuro-Oncology developed comprehensive guidelines to aid healthcare professionals in clinical management, addressing diagnostics, treatment strategies, and long-term care for patients.
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A nationwide cross-sectional survey was conducted in long-term survivors of allogeneic hematopoietic stem cell transplantation (HSCT) in childhood to investigate the effect of chronic graft-versus-host disease (cGVHD) on quality of life (QOL) and differences in QOL assessments between raters. QOL was evaluated by a visual analogue scale (VAS). Assessments were compared between the survivor, guardian, and attending pediatrician for those aged 15 years or younger, and between the survivor and attending pediatrician for those aged 16 years or older.

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Aim: This study aimed to develop a Japanese version of the Caregiving Interface Work Scale (J-CIWS) for use with employed Japanese family caregivers.

Methods: Permission was obtained from the developer of the original CIWS. The CIWS contains 20 items: 10 measuring care interface work (CIW) and 10 measuring work interface care (WIC).

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Background: This study aims at determining the health-related quality of life (HRQOL) of children with acute lymphoblastic leukemia (ALL) after the induction therapy, assessing the agreement between child self-reports and family proxy-reports HRQOL, and determining the factors related to this agreement, especially child age, family attendance, and children's social relationships beyond the family.

Methods: We analyzed questionnaire data (2012-2017) from the Japanese Pediatric Leukemia/Lymphoma Study Group's clinical study (ALL-B12). Participants were children with B-cell precursor ALL aged 5-18 and their family members, who mostly took care of the child during hospitalization.

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Background: The evaluation of transition readiness is indispensable for long-term follow-ups of adolescent patients with childhood-onset chronic diseases (CCD). We developed a Japanese version of the TRANSITION-Q (TRANSITION-Q-J) and used it to assess Japanese patients with CCD.

Methods: The TRANSITION-Q-J was developed through forward and backward translations followed by cognitive interviews with five adolescent patients.

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Background: Patient-reported outcomes (PROs) refer to any report of the status of a patient's health condition, health behavior, or experience with healthcare directly from the patient, without interpretation of the patient's response by a clinician or any other external party. While many PROs, such as the Pediatric Quality of Life Inventory (PedsQL), were originally administered in paper-and-pencil format, these are now available as electronic versions (ePROs). Although ePROs might well have used the same structure as their paper versions, we developed an alternate ePedsQL incorporating three software functions: 1) a non-forcing non-response alert, 2) a conditional question branch of the School Functioning Scale that only displays for (pre) school children, and 3) a vertical item-by-item display for small-screen devices.

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Background: Reducing non-completion of quality-of-life assessment in clinical trials is an important challenge in obtaining accurate data and unbiased interpretation of patients' quality-of-life for each regimen. We evaluated the effect of changing our questionnaire distribution procedure in a multicenter phase II/III trial on the response rate to a quality-of-life questionnaire.

Methods: In the trial, we distributed 1767 questionnaires and 1045 were returned.

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Background: Many childhood cancer survivors (CCSs) experience physical late effects related to their cancer types and treatment modalities. Physical late effects are an important factor in various occupational outcomes among CCSs. However, the relationship between physical late effects and presenteeism has remained unclear.

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Objective: The MD Anderson Symptom Inventory for Brain Tumor (MDASI-BT) module is a widely used instrument for measuring symptom burden and interference of daily activities in brain tumor patients. This study aims to develop and validate its Japanese version (MDASI-BT-Japanese).

Methods: Following forward and backward translation of the original MDASI-BT into Japanese, understandability and feasibility were assessed by cognitive debriefing.

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Aim: Nurses are expected to have a role in the transition of care from pediatric to adult medical practices for adolescents and young adults with childhood-onset chronic diseases. This study compares the experience, knowledge, and perceptions regarding the ideal care among adult unit and pediatric nurses regarding the transition to adult care for those with childhood-onset chronic diseases.

Methods: A cross-sectional study using self-report questionnaires was conducted with nurses in a tertiary hospital in Tokyo.

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Background: Patients with childhood cancer and their families frequently experience psychosocial distress associated with cancer and its treatment. We thus examined the reliability and validity of a Japanese version of the Psychosocial Assessment Tool, which was designed to screen for psychosocial risk factors among families of children with cancer.

Methods: Forward-backward translation was used to develop the Japanese version of the Psychosocial Assessment Tool.

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