Serum cytokine profiling differentiates underlying diseases in cytokine storm syndrome.

Objective: Cytokine storm syndrome (CSS), commonly associated with hemophagocytic lymphohistiocytosis (HLH), is a fatal hyperinflammatory syndrome. Differentiating the underlying diseases responsible for CSS is essential for timely therapeutic decisions. This study explored the clinical usefulness of serum cytokine profiling in distinguishing underlying diseases in patients with CSS.

Relationship between clinical and pathologic findings and the presence of genetic variants in patients with steroid-resistant nephrotic syndrome.

Background: Genetic analysis, crucial in determining treatment strategies for steroid-resistant nephrotic syndrome (SRNS), can be performed in limited facilities and requires a long time. Predicting the presence or absence of genetic variants by clinical and pathologic features is preferable.

Methods: In this multicenter, retrospective study, we compared the clinical or pathologic features between the patients with and without genetic variants in children with SRNS and evaluated the efficacy of immunosuppressive treatment and long-term kidney outcomes.

TCF3::ZNF384 induces steroid resistance in B-cell precursor acute lymphoblastic leukemia cells.

Background: ZNF384 rearrangements (ZNF384-r) are associated with distinct subgroups of B-cell precursor acute lymphoblastic leukemia (BCP-ALL) and the mixed phenotype of acute leukemia. Types of BCP-ALL with ZNF384-r exhibit common immunophenotypic characteristics, whereas their clinical features are not uniform and TCF3::ZNF384-positive patients show a significantly poorer steroid response and higher frequency of relapse, while EP300::ZNF384-positive patients exhibit a favorable response to conventional chemotherapy. Therefore, we aimed to investigate the differences in biological effects between these two ZNF384-r molecules.

Treatment of Intermediate-Risk Hepatoblastoma Using a Combined Cisplatin and Doxorubicin (PLADO) Regimen and Optimized Surgical Resection.

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.

A novel assessment of anthracycline-induced cardiotoxicity: topological flow data analysis in childhood cancer survivors.

Anthracycline chemotherapy improves survival rates in childhood cancer patients but poses a significant risk of late-onset cardiotoxicity, potentially leading to cardiac dysfunction and heart failure. Recently, flow dynamics parameters derived from two-dimensional fluid dynamics may be sensitive indicators of cardiac dysfunction. Topological flow data analysis (TFDA) mathematically defines vortical flow structures and offers a novel approach to cardiac function assessment.

Increased kidney length in mild urinary tract dilatation is a significant prognostic factor for non-resolution.

Background: Asymptomatic, mild urinary tract dilatation (UTD) that does not resolve can progress in severity, which suggests the need for continued observation. However, no studies have investigated the factors that contribute to the non-resolution of mild UTD.

Methods: We conducted this prospective cohort study of children who were newly diagnosed with mild UTD during the neonatal period from 2013 to 2021.

Clinical characteristics and outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulopathy in Japanese children.

Background: Membranoproliferative glomerulonephritis (MPGN) can be divided into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G), which includes dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). These conditions result from abnormalities in different complement pathways and may lead to different prognoses. However, there are limited studies describing the respective clinical courses.

Isolation and identification of from blood culture by MALDI-MS.

is a genus of budding yeast that is mainly isolated from environmental samples, and 40 species have been detected. The yeast isolated from human clinical samples usually only contain three species: , and . In this study, we isolated from a blood sample of a six-year-old female with a history of B-cell precursor lymphoblastic leukemia in Japan in 2022.

Recurrent spinal atypical teratoid/rhabdoid tumor with pulmonary metastasis.

Background: Atypical teratoid/rhabdoid tumors (ATRT) are aggressive pediatric central nervous system malignancies that predominantly affect the brain and have poor survival outcomes. However, spinal ATRT is an uncommon subset of ATRT, and its clinical course and management are poorly understood.

Case: We describe a case of spinal ATRT in a previously healthy 5-year-old girl who initially presented with rapid-onset gait disturbance.

Clinical Usefulness of T-Cell Receptor Vβ Repertoire Analysis for Differentiating Multisystem Inflammatory Syndrome in Japanese Children From Toxic Shock Syndrome and Kawasaki Disease.

The specific expansion of T-cell receptor β chain variable region (TCR-Vβ21.3 + ) CD4 + and CD8 + T cells was observed in Japanese patients with multisystem inflammatory syndrome in children. In contrast, these findings were not observed in patients with toxic shock syndrome and Kawasaki disease.

Assessment of anthracycline-induced cardiotoxicity in childhood cancer survivors during long-term follow-up using strain analysis and intraventricular pressure gradient measurements.

Background: Cardiac dysfunction due to cardiotoxicity from anthracycline chemotherapy is a leading cause of morbidity and mortality in childhood cancer survivors (CCS), and the cumulative incidence of cardiac events has continued to increase. This study identifies an adequate indicator of cardiac dysfunction during long-term follow-up.

Procedure: In total, 116 patients (median age: 15.

Long-term outcome of combination therapy with corticosteroids, mizoribine and RAS inhibitors as initial therapy for severe childhood IgA vasculitis with nephritis.

Background: Patients with severe IgA vasculitis with nephritis (IgAVN) typically receive aggressive therapy as an initial approach. We have consistently performed combination therapy including corticosteroids and immunosuppressants as initial therapy for severe IgAVN over a 20-year-plus period, with only minor changes to the treatment protocol. This study seeks to reveal the efficacy of combination therapy for severe IgAVN.

Clinical significance of serum cytokine profiles for differentiating between Kawasaki disease and its mimickers.

Objectives: To investigate the clinical significance of serum cytokine profiles for differentiating between Kawasaki disease (KD) and its mimickers.

Methods: Patients with KD, including complete KD, KD shock syndrome (KDSS), and KD with macrophage activation syndrome (KD-MAS), and its mimickers, including multisystem inflammatory syndrome in children, toxic shock syndrome, and Yersinia pseudotuberculosis infection, were enrolled. Serum levels of interleukin (IL)-6, soluble tumor necrosis factor receptor type II (sTNF-RII), IL-10, IL-18, and chemokine (C-X-C motif) ligand 9 (CXCL9) were measured using enzyme-linked immunosorbent assay and compared them with clinical manifestations.

Successful all robotic-assisted excision of highly malignant mediastinal neuroblastoma in a toddler: A case report.

An otherwise well 28-month-old girl presented with fever/left thigh pain. Computed tomography identified a 7 cm right posterior mediastinal tumor extending to the paravertebral and intercostal spaces with multiple bone and bone marrow metastases on bone scintigraphy. Thoracoscopic biopsy diagnosed MYCN non-amplified neuroblastoma.

The dynamics of laboratory markers reflecting cytokine overproduction in macrophage activation syndrome complicated with systemic juvenile idiopathic arthritis.

Objectives: To validate the correlation between laboratory markers reflecting disease activity of macrophage activation syndrome (MAS) and serum cytokine levels and identify the valuable laboratory markers that change over time for a prompt MAS diagnosis.

Methods: Serum cytokine levels were determined by enzyme-linked immunosorbent assay and compared with laboratory markers reflecting MAS disease activity.The changes in values were evaluated from the acute phase of systemic juvenile idiopathic arthritis (s-JIA) to MAS diagnosis.