Publications by authors named "Osamu Miyazaki"

Background: Liver transplantation (LT) has been recommended for hepatoblastoma (HB) involving all four liver sectors at diagnosis (PRETEXT IV). However, recent studies question the universal indication for primary LT in PRETEXT IV HB.

Methods: We reviewed 32 PRETEXT IV HB patients treated at our center from February 2007 to July 2024.

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The present study aimed to investigate the impact of weight loss and abstinence and exercise on liver fibrosis in metabolic dysfunction-associated steatotic liver disease (MASLD) and metabolic dysfunction-associated steatotic liver disease and alcoholic-related liver disease (MetALD). A total of 67 patients with MASLD and 33 patients with MetALD were retrospectively evaluated after six months of follow-up for weight loss, abstinence and exercise. A decrease in liver fibrosis (decrease in dLSM2B) was defined as a reduction in liver stiffness (LS) by FibroScan and macrophage galactose-specific lectin-2 binding protein glycosylation isomer (M2BPGi) over the observation period.

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Background: Although focused assessment with sonography for trauma (FAST) is considered to have high specificity, there are some case reports of false positives. This study aimed to investigate the characteristics of pediatric patients with blunt abdominal trauma and without intra-abdominal injuries (IAI) among FAST-positive patients.

Methods: This retrospective study was conducted on children less than 17 years old who visited the emergency department because of trauma and underwent FAST between April 2010 and March 2014.

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Background: Complete resection of the primary tumor is critical for the survival of children with hepatoblastomas (HBs). This prospective clinical study aimed to clarify the outcome of a chemotherapy regimen comprising cisplatin and doxorubicin (PLADO) followed by definitive surgery conducted at the appropriate time in patients with intermediate-risk HB.

Methods: Intermediate-risk HB was defined as patients without evidence of metastatic disease who met any of the following criteria: age ≥3 years; PRETEXT IV disease; presence of more than one PRETEXT annotation factor.

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Aim: Osteoporosis (Op) and sarcopenia are common complications of chronic liver disease. We sought to identify clinical screening indicators for Op, and evaluated their relationship with bone and muscle.

Method: We retrospectively evaluated 495 patients (264 women) with chronic liver disease who underwent a bone mineral density evaluation at their first outpatient visit.

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Background: This study assesses the safety and efficacy of hepatocyte-like cell (HLC) infusion therapy derived from human embryonic stem cells as bridging therapy for neonatal-onset urea cycle disorders (UCD). The research includes both preclinical and clinical evaluations to determine the feasibility of HLC infusion as a therapeutic option for safer pediatric liver transplantation.

Methods: Preclinical studies were conducted to validate the safety, biodistribution, and ammonia metabolism capabilities of HLCs using SCID mice models of UCD and extensive animal studies.

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The BCG vaccine is effective in preventing severe tuberculosis infections in infants and young children, and it is commonly administered in Japan to those aged between 5 to 8 months. Although the incidence of adverse reactions is low, disseminated lymphadenitis and osteomyelitis have been reported as potential side effects. We encountered a case of BCG osteomyelitis with typical imaging findings.

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Background: Chronic obstructive pulmonary disease (COPD) is a pulmonary and systemic inflammatory disease, and the management of systemic comorbidities is important. We previously reported that a lower mean corpuscular hemoglobin concentration (MCHC) at admission was an independent prognostic factor for death in patients with COPD exacerbation. This study aimed to determine the association between MCHC levels and prognosis in patients with stable COPD.

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Purpose: To assess the impact of the relationship between renal pedicles and tumors on surgical outcomes in patients with non-high-risk abdominal neuroblastoma.

Methods: We retrospectively analyzed cases of neuroblastoma without metastasis treated at our hospital between March 2002 and December 2023. Cases in which surgical resection was performed were divided into three groups according to imaging findings at the time of diagnosis and before surgery: Group E (tumor encasing renal pedicles), Group C (tumor in contact with renal pedicles), and Group S (tumor separated from renal pedicles).

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Introduction: Type 1 congenital pulmonary airway malformation (CPAM) is characterized by large, dilated cysts that rapidly expand due to aeration immediately after birth, often necessitating surgical resection. In atypical cases of CPAM type 1, fetal T2-weighted magnetic resonance imaging (MRI) reveals a low-intensity solid lung mass containing multiple irregular T2 high-intensity areas.

Methods: Data were retrospectively collected for infants with atypical CPAM type 1 born at our hospital between March 2002 and December 2022.

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Article Synopsis
  • * A 13-year-old girl with carbamoyl phosphate synthetase I (CPS1) deficiency underwent liver transplantation from her mother, facing significant challenges due to her severe scoliosis which led to portal vein complications necessitating multiple surgical interventions.
  • * The case highlights the importance of meticulous preoperative assessments and tailored surgical strategies for patients with structural abnormalities to prevent complications and ensure successful outcomes.
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  • * It highlights the complexities of performing allogeneic transplants amid active infections, as they typically have poor outcomes.
  • * A specific case is presented about a 5-year-old boy with disseminated infection who received granulocyte transfusions from his mother and underwent successful haploidentical stem cell transplantation from his father after immunosuppressive treatment for SAA.
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  • * The research included data from 147 patients (77 children and 70 adults) who were mostly treated with phosphate and active vitamin D, revealing significant challenges related to height, pain severity, and quality of life (QOL) in both age groups.
  • * Findings showed that while children reported low pain levels, adults experienced mild-to-moderate pain, with both groups experiencing low QOL, which highlights the ongoing need for better understanding and management strategies for XL
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  • The study aimed to evaluate the impact of pemafibrate (PEM) on patients with metabolic dysfunction-associated steatotic liver disease (MASLD) and hyperlipidemia.
  • A retrospective analysis of 43 patients was conducted, assessing clinical factors and liver stiffness using FibroScan before and after 0.5 years of PEM treatment.
  • Results showed that while liver stiffness (LS) and other key liver function metrics did not decrease overall, improvements were noted in patients with more significant liver stiffness at baseline, suggesting PEM may be a potential treatment option for MASLD.*
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  • The study explored the connection between immunoglobulin A (IgA) levels and chronic liver disease in 478 patients at Nagasaki Harbor Medical Center, highlighting that higher IgA levels were found in patients with more severe conditions, such as Child-Pugh classification B and C and alcoholic liver disease.
  • Analysis revealed that factors like high IgG levels, diabetes, and certain liver disease types contribute to elevated IgA levels, suggesting that elevated IgA might indicate increased liver disease severity.
  • Results also showed a significant association between IgA levels and visceral fat, particularly in women, reinforcing the role of IgA as a potential marker for liver disease severity amidst rising cases of steatotic liver disease.
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  • The JPLT3-S study examined the effectiveness of cisplatin (CDDP) monotherapy for young children with standard-risk hepatoblastoma, aiming to reduce treatment toxicity compared to previous studies.
  • A total of 38 patients under 3 years old participated, achieving a remarkable 3-year progression-free survival rate of 93.9% and a 100% overall survival rate.
  • Despite the positive outcomes, nearly half of the patients experienced late-onset ototoxicity, highlighting a potential side effect of CDDP treatment.
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  • The study focuses on improving treatment for Langerhans cell histiocytosis (LCH), which, despite a generally good prognosis, has high recurrence rates and serious long-term effects like diabetes insipidus and neurodegenerative issues.
  • Researchers are evaluating the effectiveness of dexamethasone and intrathecal chemotherapy for multisystem disease, along with zoledronate for multifocal bone disease in previously untreated patients under 40.
  • The trial is officially registered and approved by the Central Review Board in Japan, with written consent required from participants or their guardians.
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  • * A 3-year-old girl with OI experienced a complete tibial fracture and, after recovery, developed spontaneous pain at the fracture site where new imaging revealed OO.
  • * The case highlights the rare coexistence of OO and OI, suggesting that the combination of OI's bone fragility and a prior fracture may have contributed to a refracture in the same area.
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Cigarette smoking is known to be the leading cause of chronic obstructive pulmonary disease (COPD). However, the detailed mechanisms have not been elucidated. PAF (platelet-activating factor), a potent inflammatory mediator, is involved in the pathogenesis of various respiratory diseases such as bronchial asthma and COPD.

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Introduction: Allergic bronchopulmonary mycosis (ABPM) is a chronic airway disease characterized by the presence of fungi that trigger allergic reactions and airway obstruction. Here, we present a unique case of ABPM in which a patient experienced sudden respiratory failure due to mucus plug-induced airway obstruction. The patient's life was saved by venovenous extracorporeal membrane oxygenation (VV-ECMO) and bronchoscopic removal of the plug.

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  • The study investigates the mediastinal shift angle as a new MRI index linked to survival rates in fetuses with congenital diaphragmatic hernia, but its relevance in congenital pulmonary airway malformation (CPAM) had not been explored.
  • Researchers measured the mediastinal shift angles in 124 control fetuses and 32 fetuses diagnosed with CPAM, finding significant differences and a positive correlation with the CPAM volume ratio.
  • Results suggest that the mediastinal shift angle might effectively assess CPAM severity in fetal MRI, showing promising accuracy for clinical use.
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  • PIVKA-II is a marker used to diagnose hepatoma (liver cancer) but can also appear in patients without liver issues.
  • A study analyzed the relationship between serum PIVKA-II levels and various clinical measurements of bone and muscle health in 441 patients with liver disease.
  • Results indicated that high PIVKA-II levels were linked to serious liver disease, abnormal bone health in women, and muscle weakness in men, suggesting that monitoring PIVKA-II might help manage bone and muscle health in these patients.
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