Long-Term Survey of Japanese Children with Recurrent Nephroblastoma: A Report from Japan Children's Cancer Group.

In prospective Japanese studies of pediatric renal tumors, 5-year event-free survival and overall survival (OS) for patients with nephroblastoma ranges from 75-90% and 89-97%, respectively. However, treatments strategies for recurrent nephroblastoma in Japanese patients remain unclear. This retrospective study aimed to inform the development of treatment strategies by analyzing the long-term results and side effects of salvage therapies for recurrent nephroblastoma in Japan.

Case Report: COVID-19 Pandemic Exacerbates Eating Disorder by Social and Intrafamilial Isolation.

The coronavirus disease-2019 (COVID-19) pandemic has increased the stress levels of children and their parents and diagnoses of eating disorders (EDs), irritable bowel syndrome, migraines, tension headaches, orthostatic dysregulation, and/or school refusal has increased among children. We present a case of a nine-year old girl, which rapidly worsened due to stress and isolation related to the COVID-19 pandemic. The patient's father noted her rapid weight loss due to poor oral intake.

A Case of Type 2 Diabetes Mellitus With t(6;7)(q24;q31.2) Balanced Translocation.

Most balanced translocations do not involve any gain or loss of genetic material, and individuals harboring these translocations remain clinically asymptomatic. Nevertheless, balanced translocations have reportedly been associated with several diseases. Here, we present the case of a 12-year-old boy with type 2 diabetes mellitus that could not be explained only by obesity; the patient harbored a balanced translocation (46,XY t(6;7)(q24;q31.

Diverse outcomes in extra-cranial rhabdoid tumors: A single institute experience.

Rhabdoid tumors (RTs) are a rare and aggressive pediatric cancer that commonly presents with alterations in the tumor suppressor gene . However, RT prognosis is still poor, with no standard treatment available. Moreover, no predictive biomarkers have been identified for determining its aggressiveness or chemo- and radio-sensitivities.

Secondary Pseudohypoaldosteronism Associated With Mild Hydronephrosis in a Newborn.

Neonatal hyponatremia with hyperkalemia is a rare but potentially life-threatening occurrence. Aldosterone deficiency secondary to congenital adrenal hyperplasia (CAH) is often suspected in these cases, although it is not easy to accurately diagnose it initially. We report the case of a 12-day-old female infant presenting with poor sucking, hyperkalemia, and hyponatremia.

The Novel Histone Deacetylase Inhibitor, OBP-801, Induces Apoptosis in Rhabdoid Tumors by Releasing the Silencing of .

Rhabdoid tumor is an aggressive, early childhood tumor. Biallelic inactivation of the SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 ()/integrase interactor 1 () gene is the only common genetic feature in rhabdoid tumors. Loss of function results in downregulation of several tumor suppressor genes including , and The novel histone deacetylase inhibitor, OBP-801, induces and has shown efficacy against various cancers.

Saffold Cardiovirus Infection in a 2-Year-Old Boy with Acute Pancreatitis.

Saffold cardiovirus (SAFV), first identified in a stool sample in 2007, is thought to be associated with respiratory disease and gastroenteritis. On the other hand, animal experiments suggested that the major viral load, following intraperitoneal inoculation of SAFV in mice, may be detected in the pancreas. However, until now, no cases of SAFV in patients with pancreatitis have been reported.

A NOXA/MCL-1 Imbalance Underlies Chemoresistance of Malignant Rhabdoid Tumor Cells.

Malignant rhabdoid tumor (MRT) is a rare aggressive pediatric cancer characterized by inactivation of SNF5, a core subunit of SWI/SNF complexes. Previously, we showed that SNF5 contributes to transcriptional activation of NOXA, a pro-apoptotic protein that binds and inhibits the anti-apoptotic protein MCL-1. In this study, we found that NOXA expression was downregulated in MRT cell lines as well as in clinical MRT samples and that ectopically expressed NOXA bound MCL-1 and increased the sensitivity of MRT cell lines to doxorubicin (DOX) by promoting apoptosis.

Effect of a single inhalation of laninamivir octanoate in children with influenza.

Objective: The purpose of this study was to compare the efficiency and safety of a new neuraminidase inhibitor, laninamivir octanoate (LO), with zanamivir (ZN) in pediatric patients with influenza.

Methods: One hundred twelve pediatric patients ≤ 15 years, diagnosed with a rapid diagnostic test as having influenza from January to May 2011, were randomly assigned to the LO group or the ZN group, and their parents were asked to complete a questionnaire during the recovery at home. The LO group was instructed to inhale LO once (20 or 40 mg depending on age), and the ZN group was instructed to inhale ZN (20 mg) twice daily for 5 days.

Sensitivity of malignant rhabdoid tumor cell lines to PD 0332991 is inversely correlated with p16 expression.

Malignant rhabdoid tumor (MRT) is a rare and highly aggressive neoplasm of young children. MRT is characterized by inactivation of integrase interactor 1 (INI1). Cyclin-dependent kinase 4 (CDK4), which acts downstream of INI1, is required for the proliferation of MRT cells.

The incidence and direct medical cost of hospitalization due to rotavirus gastroenteritis in Kyoto, Japan, as estimated from a retrospective hospital study.

Anticipating imminent licensure of rotavirus vaccine for use in Japan, we estimated the incidence of rotavirus hospitalization and calculated the direct medical cost associated with rotavirus hospitalization in a hospital that provided virtually exclusive pediatric beds to the local community adjacent to the northern outskirts of metropolitan Kyoto, Japan. For a 2 year period between September, 2008 and August, 2010, there were 103 hospitalizations due to acute gastroenteritis among children less than 5 years of age. Stool specimens from 77 (75%) of the 103 hospitalized patients were tested for rotavirus antigen, and 46 (60%) were positive.

Preoperative analysis of 11q loss using circulating tumor-released DNA in serum: a novel diagnostic tool for therapy stratification of neuroblastoma.

Allelic deletion of the long arm of chromosome 11 (11q loss) is closely associated with the prognosis of neuroblastoma (NB). Here we examined 11q loss using tumor-released DNA fragments in the sera of 24 cases. The allelic intensity score of a panel of polymorphic markers in 11q23 in serum DNA was significantly different between the 11q loss-positive group and the11q loss-negative group.

Restoration of p53 pathway by nutlin-3 induces cell cycle arrest and apoptosis in human rhabdomyosarcoma cells.

Purpose: Seventy to eighty percent of rhabdomyosarcoma (RMS) tumors retain wild-type p53. The tumor suppressor p53 plays a central role in inducing cell cycle arrest or apoptosis in response to various stresses. p53 protein levels are regulated by MDM2 through ubiquitin-dependent degradation.

Circulating methylated-DCR2 gene in serum as an indicator of prognosis and therapeutic efficacy in patients with MYCN nonamplified neuroblastoma.

Background: MYCN amplification (MNA) in neuroblastoma is a strong indicator of poor prognosis. However, some MYCN nonamplified (non-MNA) cases show poor outcomes, and examining the status of the gene requires an operation, which may have surgical complications. Therefore, a new marker is needed to identify cases of non-MNA neuroblastomas with poor prognoses using less risky procedures.

Trastuzumab activates allogeneic or autologous antibody-dependent cellular cytotoxicity against malignant rhabdoid tumor cells and interleukin-2 augments the cytotoxicity.

Purpose: Malignant rhabdoid tumor (MRT) is an early childhood cancer with poor prognosis. Trastuzumab, a humanized monoclonal antibody against human epidermal growth factor receptor-2 (HER-2), has been shown to be effective against breast cancer and other cancers. We investigated the effect of trastuzumab on MRT cell lines.

Effects of PAX3-FKHR on malignant phenotypes in alveolar rhabdomyosarcoma.

The malignancy of alveolar rhabdomyosarcoma (ARMS) has been linked to expression of the PAX3-FKHR chimeric gene. To understand the effect of this gene, we used RNAi to knock down its expression (without affecting the expressions of either PAX3 or FKHR) in human ARMS cell lines. Down-regulating PAX3-FKHR caused (a) tumor cells to accumulate in the G1 phase, inhibiting the rate of cellular proliferation, (b) a reduction in the levels of the MET, reducing cell motility stimulated by HGF, and (c) induction of the myogenic differentiation gene, myogenin, and muscle differentiation (morphologic change and the expression of muscle specific proteins, desmin, and myosin heavy chain).