Publications by authors named "Perlman E"

Background: In the fifth National Wilms Tumor Study, patients received vincristine and dactinomycin (VA) without radiation for stage I focal anaplastic Wilms tumor (FAWT) and VA plus doxorubicin (DD4A) and radiation for stage II-IV FAWT. Four-year event-free survival (EFS) and overall survival (OS) for stage I FAWT were 67.5% and 88.

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Purpose: National Wilms Tumor Study-5 (NWTS-5) and AREN0321 evaluated the outcomes of children with rhabdoid tumor of the kidney (RTK) and malignant rhabdoid tumor of soft tissues (MRT).

Patients And Methods: Eligible patients with RTK were enrolled prospectively on NWTS-5 (1995-2002) and treated with carboplatin and etoposide alternating with cyclophosphamide (Regimen RTK). Patients with RTK or MRT were enrolled on AREN0321 (2005-2012) and received vincristine, doxorubicin, and cyclophosphamide alternating with carboplatin, cyclophosphamide, and etoposide (Regimens UH-1 or dose-reduced Revised UH-1).

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Article Synopsis
  • The research focuses on the fruit fly Drosophila melanogaster as a crucial model in neuroscience, aided by extensive resources like the FlyWire whole-brain connectome and a hierarchical annotation of neuron classes and types.
  • The study reveals 8,453 annotated cell types, with 4,581 being newly identified, highlighting the complexity of the fly brain and emphasizing the difficulty in reidentifying some hemibrain cell types in FlyWire.
  • A new definition of cell type is proposed based on cell similarities across different brains, and the study illustrates findings related to neuron connectivity, structural stability, and a consensus atlas for the fly brain's neuroanatomy, supporting future comparative studies.
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  • Researchers have created a detailed neuronal wiring diagram of the whole brain of a fruit fly (Drosophila melanogaster), mapping over 5 billion chemical synapses between more than 139,000 neurons, to better understand brain function.
  • The study includes detailed annotations about various cell types, nerve pathways, and neurotransmitter identities, and the data is freely available for other researchers to use and explore.
  • By analyzing synaptic pathways and connections, the project helps illustrate how neural structures relate to sensorimotor behaviors, paving the way for similar studies in other species.
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Progress in histological methods and in microscope technology has enabled dense staining and imaging of axons over large brain volumes, but tracing axons over such volumes requires new computational tools for 3D reconstruction of data acquired from serial sections. We have developed a computational pipeline for automated tracing and volume assembly of densely stained axons imaged over serial sections, which leverages machine learning-based segmentation to enable stitching and alignment with the axon traces themselves. We validated this segmentation-driven approach to volume assembly and alignment of individual axons over centimeter-scale serial sections and show the application of the output traces for analysis of local orientation and for proofreading over aligned volumes.

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Background: On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children's Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT.

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Context.—: The distinction between well-differentiated epithelial favorable-histology Wilms tumor (EFHWT) and metanephric adenoma (MA) in children has historically been determined by the required absence of both a fibrous pseudocapsule and mitotic activity in MA. More recently these features have been allowed in adult MA.

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Expansion microscopy and light sheet imaging enable fine-scale resolution of intracellular features that comprise neural circuits. Most current techniques visualize sparsely distributed features across whole brains or densely distributed features within individual brain regions. Here, we visualize dense distributions of immunolabeled proteins across early visual cortical areas in adult macaque monkeys.

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Background: Patients with stage IV favorable histology Wilms tumor (FHWT) with extrapulmonary metastases (EPM) constitute a small subset of patients with FHWT. Because of their rarity and heterogeneity, optimal FHWT treatment is not well understood. Children's Oncology Group protocol AREN0533 assigned patients with FHWT and EPM to intensified chemotherapy, regimen M, after initial DD-4A chemotherapy.

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Introduction: The prognostic impact of positive lymph nodes (LN+) and/or singular loss of heterozygosity (LOH) of 1p or 16q were assessed in children with stage III favorable histology Wilms tumor (FHWT) enrolled on AREN0532 or AREN03B2 alone.

Patients And Methods: A total of 635 stage III FHWT vincristine/dactinomycin/doxorubicin (DD4A)-treated patients met inclusion criteria. Event-free survival (EFS) and overall survival are reported overall and by LN sampling, LN status, LOH 1p, LOH 16q, and a combination of LN status and singular LOH.

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Article Synopsis
  • - The study of Wilms tumour, which began with Alfred Knudson's 'two-hit' model, has advanced significantly over the last 50 years, leading to important discoveries in its genetics and biology.
  • - Research has focused on identifying prognostic biomarkers to improve treatment outcomes, although variability in these biomarkers within tumors creates challenges for individual patient care.
  • - Despite progress, ongoing investigations aim to deepen molecular understanding of Wilms tumour, including causes of relapse and bilateral cases, with international collaboration being key to addressing these complex issues.
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Objective: Excessive alcohol consumption and its consequences among college women continues despite prevention efforts. One common consequence, alcohol-related blackouts (ARBs), are periods of alcohol-activated anterograde amnesia. The purpose of the current project is to extend the ARB and drinking motive literature by examining their relationship over time.

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Unlabelled: Long noncoding RNAs (lncRNA) play an important role in gene regulation and contribute to tumorigenesis. While pan-cancer studies of lncRNA expression have been performed for adult malignancies, the lncRNA landscape across pediatric cancers remains largely uncharted. Here, we curated RNA sequencing data for 1,044 pediatric leukemia and extracranial solid tumors and integrated paired tumor whole genome sequencing and epigenetic data in relevant cell line models to explore lncRNA expression, regulation, and association with cancer.

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Connections between neurons can be mapped by acquiring and analyzing electron microscopic (EM) brain images. In recent years, this approach has been applied to chunks of brains to reconstruct local connectivity maps that are highly informative, yet inadequate for understanding brain function more globally. Here, we present the first neuronal wiring diagram of a whole adult brain, containing 5×10 chemical synapses between ~130,000 neurons reconstructed from a female .

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Article Synopsis
  • The fruit fly is a key model organism in neuroscience due to its complex behaviors and accessible nervous system, bolstered by collaborative genetic resources.*
  • The FlyWire project has produced the first complete brain connectome of an adult fruit fly, providing a detailed catalog of approximately 130,000 neurons, including 4,552 cell types.*
  • Analysis indicated that while some neuronal connections were stable, others showed variability across individuals, revealing complexities in brain function and suggesting some cell types from previous studies may not be reliably identified in this new dataset.*
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Purpose: The safety of reintroducing chemotherapy in the pediatric renal tumor setting after severe hepatopathy (SH), including sinusoidal obstruction syndrome (SOS), is uncertain. We describe the incidence, severity, outcomes, and impact on subsequent treatment for patients with SH from National Wilms Tumor Study (NWTS) protocols 3-5.

Patients And Methods: Archived charts for patients enrolled on NWTS 3-5 who met study inclusion criteria for SH by using established hepatopathy grading scales and clinical criteria were reviewed for demographics, tumor characteristics, radio- and chemotherapy details, SH-related dose modifications, and oncologic outcomes.

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Introduction: The pediatric emergency department (PED) provides a wealth of learning opportunities for residents. However, delivering dedicated education can be a significant challenge due to considerable variability in day-to-day schedules, volume, cases, time, and resource availability. Case-based and learner-centered teaching models are well suited for ambulatory settings like the emergency department.

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  • * Current assessments often rely on visual interpretation, but using precise quantitative SUV ratios allows for earlier detection of amyloid plaques and tracking of antiamyloid treatment effectiveness.
  • * The Quantitative Imaging Biomarkers Alliance has established guidelines to reduce variability in measurements, leading to high statistical power for study conclusions and improved precision in clinical and research settings.
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Serial-section electron microscopy (ssEM) is the method of choice for studying macroscopic biological samples at extremely high resolution in three dimensions. In the nervous system, nanometer-scale images are necessary to reconstruct dense neural wiring diagrams in the brain, so -called . The data that can comprise of up to 10 individual EM images must be assembled into a volume, requiring seamless 2D registration from physical section followed by 3D alignment of the stitched sections.

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