Sleep and quiet wakefulness signify an idling brain hub for creative insights.

Long-term potentiation of synaptic strength is a fundamental aspect of learning and memory. Memories are believed to be stored within specific populations of neurons known as engram cells, which are subsequently reactivated during sleep, facilitating the consolidation of stored information. However, sleep and offline reactivations are associated not only with past experiences but also with anticipation of future events.

Technical advances in the surgical management of Wilms tumors in children.

Surgery is one of the cornerstones of Wilms tumor treatment. In this article, we present technical advancements that are finding their way into the armamentarium of pediatric cancer surgeons. We discuss the current approaches, challenges, opportunities, and future directions of minimally invasive surgery (laparoscopic and robotics), image-guided surgery, and fluorescence-guided surgery.

Memory reactivations during sleep.

Neuronal activities that occur during awake periods are often reactivated again during sleep, to consolidate recently encoded memories, a process known as consolidation. In recent years, advanced tools, specially optical techniques and in-vivo live Ca imaging, have revealed a deeper understanding to the offline periods' neuronal activities and their correspondence to later awake behavioral outputs. Recently, there is a growing consensus that sleep is more of an active process.

Sevoflurane-induced amnesia is associated with inhibition of hippocampal cell ensemble activity after learning.

General anesthesia could induce amnesia, however the mechanism remains unclear. We hypothesized that suppression of neuronal ensemble activity in the hippocampus by anesthesia during the post-learning period causes retrograde amnesia. To test this hypothesis, two experiments were conducted with sevoflurane anesthesia (2.

The locus coeruleus as a regulator of memory linking.

A core function of our memory system is the ability to link appropriate events. In this issue of Neuron, Chowdhury et al. (2022) pinpoint a specific neuromodulatory circuit for contextual memory linking, but not formation, in the hippocampus.

Evidence-based surgical guidelines for treating children with Wilms tumor in low-resource settings.

Background: Survival of Wilms tumor (WT) is > 90% in high-resource settings but < 30% in low-resource settings. Adapting a standardized surgical approach to WT is challenging in low-resource settings, but a local control strategy is crucial to improving outcomes.

Objective: Provide resource-sensitive recommendations for the surgical management of WT.

Outcomes of Pediatric Patients With Metastatic Ewing Sarcoma Treated With Interval Compression.

Background: Interval compression (IC), a regimen of alternating vincristine/doxorubicin/cyclophosphamide and ifosfamide/etoposide every 2 weeks, improves survival for localized Ewing sarcoma (ES), with uncertain effect on metastatic disease.

Materials And Methods: We reviewed the charts of pediatric patients with metastatic ES treated with IC at our center between January 2013 and March 2020. We calculated event-free survival and overall survival (OS) and used log-rank tests for univariate comparisons.

Global Neuroblastoma Network: An international multidisciplinary neuroblastoma tumor board for resource-limited countries.

Background: Tumor boards are part of standard care of patients with complex cancers, but appropriate multidisciplinary expertise and infrastructure are often not available in low- and middle-income countries (LMIC) for pediatric cancers, such as neuroblastoma. Our goal was to review results of a Global Neuroblastoma Network (GNN) tumor board accessible to LMIC.

Methods: De-identified clinical cases presented via internet conference during a weekly GNN virtual tumor board from 2010 through 2020 were evaluated in a standardized format, including diagnostic imaging, pathology, therapy information, resource limitations, and questions for discussion.

Pcdhβ deficiency affects hippocampal CA1 ensemble activity and contextual fear discrimination.

Clustered protocadherins (Pcdhs), a large group of adhesion molecules, are important for axonal projections and dendritic spread, but little is known about how they influence neuronal activity. The Pcdhβ cluster is strongly expressed in the hippocampus, and in vivo Ca imaging in Pcdhβ-deficient mice revealed altered activity of neuronal ensembles but not of individual cells in this region in freely moving animals. Specifically, Pcdhβ deficiency increased the number of large-size neuronal ensembles and the proportion of cells shared between ensembles.

Orchestrated ensemble activities constitute a hippocampal memory engram.

The brain stores and recalls memories through a set of neurons, termed engram cells. However, it is unclear how these cells are organized to constitute a corresponding memory trace. We established a unique imaging system that combines Ca imaging and engram identification to extract the characteristics of engram activity by visualizing and discriminating between engram and non-engram cells.

Inferior vena cava involvement in children with Wilms tumor.

Objectives: Retrospective review of children with WT thrombus involving the IVC.

Methods: We reviewed the charts of 123 patients with WT diagnosed between January 2006 and December 2015. Patients with caval tumor thrombus were identified, demographic data, radiological images, extent of thrombus, chemo- and radiotherapy, surgical approach, pathology reports and outcomes were analyzed.

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy.

Background: The care of rhabdomyosarcoma (RMS) is complex due to its multimodal nature. By following standard protocols with acceptable toxicity and building local expertise, better outcome should be achievable.

Procedure: A retrospective study was conducted of records of patients (n = 45; 31 males; median age 26 months) with RMS treated at King Hussein Cancer Center in Jordan from January 2004 to December 2008.

Inflammatory myofibroblastic tumor causing unexplained anemia in a toddler: a case report.

Introduction: Inflammatory myofibroblastic tumor is a very rare benign tumor in children that mimics malignant tumors in its aggressiveness locally and by the possibility of recurrence after surgical resection, and causing anemia of chronic disease, which is a decrease in hemoglobin 1 to 2 g/dL below normal level in a patient with chronic illness.

Case Presentation: A 32-month-old boy from Libya presented with microcytic hypochromic anemia. He had been treated in three countries and five centers without response to medical therapy.

Distinct features of teratoid Wilms tumor.

Teratoid Wilms tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. Typically, histopathologic examination shows a mixture of mature squamous and mucus-producing columnar epithelium and a prominent component of mature adipose tissue. The treatment of this tumor has not been established because of its rarity with some reports doubting the value of chemotherapy.

INI1 (BAF 47) immunohistochemistry is an essential diagnostic tool for children with hepatic tumors and low alpha fetoprotein.

Malignant rhabdoid tumor (MRT) of the liver is a rare malignancy with grave prognosis. This entity should be considered in the differential diagnosis of any aggressive liver tumor with low levels of alpha fetoprotein. We report 2 cases of hepatic MRT presenting in infancy.

Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors.

Background: Malignant rhabdoid tumors (MRTs) are aggressive and often fatal; the Surveillance, Epidemiology, and End Results (SEER) database offers an opportunity to study this rare malignancy.

Methods: From the SEER database, we extracted records of patients with a reported diagnosis of MRT and analyzed them for clinical features and survival rates by univariate and multivariate analyses.

Results: For the 229 patients included in our data, who were diagnosed from 1986 to 2005, primary tumors were located in the central nervous system (CNS) (35%), kidneys (20%), and extra-renal non-cranial sites (ERNC-MRTs) (45%).

Local control of the primary tumour in metastatic neuroblastoma.

The previous studies have stressed on the importance of loco-regional control in the management of high-risk neuroblastoma. We searched the Surveillance, Epidemiology and End-Results (SEER) database for patients older than 2years with metastatic neuroblastoma who were diagnosed from 1998 to 2005. We identified 291 patients (mean age, 4.

From upfront nephrectomy to preoperative chemotherapy and back: a single institution experience in the treatment of Wilms tumor.

Background: Over the past decades, 2 different approaches for the treatment of Wilms tumor have emerged: upfront nephrectomy (UN) and preoperative chemotherapy (PC), with adjuvant treatment adjusted to stage, histology, and chemotherapy response.

Methods: In July 2005, we switched our strategy from UN to PC. This study is a retrospective review of patients treated at our institution between January 2003 and October 2007.

Stoichiometric alteration of PMP22 protein determines the phenotype of hereditary neuropathy with liability to pressure palsies.

Background: Hereditary neuropathy with liability to pressure palsies (HNPP) is caused by a 1.4-megabase deletion at chromosome 17p11.2, which bears the PMP22 gene and other genes.

Effect of an R69C mutation in the myelin protein zero gene on myelination and ion channel subtypes.

Background: Most mutations in the myelin protein zero gene (MPZ) typically cause a severe demyelinating/dysmyelinating neuropathy that begins in infancy or an adult-onset axonal neuropathy. Axonal degeneration in the late-onset H10P mutation may be caused by the disruption of axoglial interaction.

Objective: To evaluate sural nerve biopsy samples from a patient with early-onset Charcot-Marie-Tooth disease type 1B caused by an arg69-to-cys (R69C) mutation.

Skin biopsies demonstrate MPZ splicing abnormalities in Charcot-Marie-Tooth neuropathy 1B.

Objective: To demonstrate that intronic mutations in the myelin protein zero (MPZ) cause Charcot-Marie-Tooth neuropathy 1B (CMT1B) by disrupting MPZ splicing.

Methods: We report a family with a T>G transversion at the invariant + 2 position in intron 4 of MPZ (c.614 + 2T>G) that abolishes 5' donor site recognition and is predicted to alter MPZ splicing.

Management of unexpected difficult airway at a teaching institution over a 7-year period.

Study Objective: To review an anesthesiology department's experience with managing unexpected difficult airways over a 7-year time span.

Design: Retrospective review of unexpected difficult airway reporting forms.

Setting: A tertiary care teaching hospital.

Skin biopsies in myelin-related neuropathies: bringing molecular pathology to the bedside.

Skin biopsy is a minimally invasive procedure and has been used in the evaluation of non-myelinated, but not myelinated nerve fibres, in sensory neuropathies. We therefore evaluated myelinated nerves in skin biopsies from normal controls and patients with Charcot-Marie-Tooth (CMT) disease caused by mutations in myelin proteins. Light microscopy, electron microscopy and immunohistochemistry routinely identified myelinated dermal nerves in glabrous skin that appeared similar to myelinated fibres in sural and sciatic nerve.

The acute scrotum in Arab children with familial Mediterranean fever.

Over a period of 7 years, among 175 boys under the age of 16 years with familial Mediterranean fever (FMF), 16 (9%) developed 28 episodes of scrotal swelling that was unilateral in 26 (93%) and bilateral in 2 (7%). Fever and pain were present in 15 (94%) children; fever was characterized by a gradual onset and pain was moderate in intensity. The episodes were self-limiting and lasted from 8 h to 5 days.