Does lymphadenectomy improve survival in patients with intermediate risk endometrial cancer? A multicentric study from the FRANCOGYN Research Group.

Objective: The role of lymphadenectomy in intermediate risk endometrial cancer remains uncertain. We evaluated the impact of lymphadenectomy on overall survival and relapse-free survival for patients with intermediate risk endometrial cancer.

Methods: We retrospectively reviewed patients from the FRANCOGYN database with intermediate risk endometrial cancer, based on pre-operative and post-operative criteria (type 1, grade 1-2 tumors with deep (> 50%) myometrial invasion and no lymphovascular space invasion), who received primary surgical treatment between November 2002 and August 2013.

Patterns of recurrence and prognosis in locally advanced FIGO stage IB2 to IIB cervical cancer: Retrospective multicentre study from the FRANCOGYN group.

Introduction: Evidence-based data describing patterns of recurrence and prognosis in women with FIGO stage IB2 to IIB locally advanced cervical cancer (LACC) are scarce. The purpose of this study was to analyse patterns of recurrence in LACC and their correlation with prognosis, depending on FIGO stage, lymph node (LN) status and treatment modalities. The endpoints of this study were the type of recurrence (locoregional or distant, and time to recurrence), the recurrence free survival, the overall survival and the cumulative incidence for both locoregional and distant recurrence.

Delayed Treatment (≥5 Days) by Flow Diversion of Ruptured Blister-Like Cerebral Aneurysms : Case Series of 8 Consecutive Patients.

Background And Purpose: Ruptured blister-like aneurysms (BLAs) are challenging lesions to treat, without any consensus on their management. Few studies have evaluated the safety and effectiveness of flow diverter stents (FDS) for this indication, with promising results. The goal was to evaluate the safety and effectiveness of a delayed (≥5 days) flow diversion strategy for the treatment of ruptured intracranial BLAs.

Complement Gene Variants and Shiga Toxin-Producing -Associated Hemolytic Uremic Syndrome: Retrospective Genetic and Clinical Study.

Background And Objectives: Inherited complement hyperactivation is critical for the pathogenesis of atypical hemolytic uremic syndrome (HUS) but undetermined in postdiarrheal HUS. Our aim was to investigate complement activation and variants of complement genes, and their association with disease severity in children with Shiga toxin-associated HUS.

Design, Setting, Participants, & Measurements: Determination of complement biomarkers levels and next-generation sequencing for the six susceptibility genes for atypical HUS were performed in 108 children with a clinical diagnosis of post-diarrheal HUS (75 Shiga toxin-positive, and 33 Shiga toxin-negative) and 80 French controls.

CT-1 (Cardiotrophin-1)-Gal-3 (Galectin-3) Axis in Cardiac Fibrosis and Inflammation.

Myocardial fibrosis is a main contributor to the development of heart failure (HF). CT-1 (cardiotrophin-1) and Gal-3 (galectin-3) are increased in HF and associated with myocardial fibrosis. The aim of this study is to analyze whether CT-1 regulates Gal-3.

AAV Gene Transfer with Tandem Promoter Design Prevents Anti-transgene Immunity and Provides Persistent Efficacy in Neonate Pompe Mice.

Hepatocyte-restricted, AAV-mediated gene transfer is being used to provide sustained, tolerogenic transgene expression in gene therapy. However, given the episomal status of the AAV genome, this approach cannot be applied to pediatric disorders when hepatocyte proliferation may result in significant loss of therapeutic efficacy over time. In addition, many multi-systemic diseases require widespread expression of the therapeutic transgene that, when provided with ubiquitous or tissue-specific non-hepatic promoters, often results in anti-transgene immunity.

Idiopathic nephrotic syndrome and rituximab: may we predict circulating B lymphocytes recovery?

Background: Rituximab (RTX) has been shown to be an efficient treatment for steroid-dependent nephrotic syndrome (SDNS). A long B cell depletion period seems to improve the duration of remission. This study reports the duration of B cell depletion after each RTX infusion in patients with nephrotic syndrome.

Multimodal magnetic resonance imaging investigation of basal forebrain damage and cognitive deficits in Parkinson's disease.

Background: Cognitive deficits in Parkinson's disease (PD) may result from damage in the cortex as well as in the dopaminergic, noradrenergic, and cholinergic inputs to the cortex. Cholinergic inputs to the cortex mainly originate from the basal forebrain and are clustered in several regions, called Ch1 to Ch4, that project to the hippocampus (Ch1-2), the olfactory bulb (Ch3), and the cortex and amygdala (Ch4).

Objective: We investigated changes in basal forebrain and their role in cognitive deficits in PD.

Therapeutic value of surgical paraaortic staging in locally advanced cervical cancer: a multicenter cohort analysis from the FRANCOGYN study group.

Background: The prognostic impact of surgical paraaortic staging remains unclear in patients with locally advanced cervical cancer (LACC). The objective of our study was to evaluate the survival impact of surgical staging in patients with LACC and no evidence of paraaortic lymph node (PALN) metastasis on pre-operative imaging work-up.

Methods: Data of 1447 patients with cervical cancer treated between 1996 and 2016 were extracted from maintained databases of 10 French University hospitals.

Slower Is Higher: Threshold Modulation of Cortical Activity in Voluntary Control of Breathing Initiation.

Speech or programmed sentences must often be interrupted in order to listen to and interact with interlocutors. Among many processes that produce such complex acts, the brain must precisely adjust breathing to produce adequate phonation. The mechanism of these adjustments is multifactorial and still poorly understood.

Agitated saline bubble-enhanced ultrasound for the positioning of cuffed, tunneled dialysis catheters in patients with end-stage renal disease.

Background: In patients with end-stage renal disease, the use of cuffed, tunneled dialysis catheters for hemodialysis has become integral to treatment plans. Fluoroscopy is a widely accepted method for the insertion and positioning of cuffed dialysis catheters, because it is easy to use, accurate and reliable, and has a relatively low incidence of complications. The purpose of our study was to evaluate the feasibility of tunneled hemodialysis catheter placement without the use of fluoroscopy but with a dynamic ultrasound-imaging-based guided technique.

Does mechanical morcellation of large glands compromise incidental prostate cancer detection on specimen analysis? A pathological comparison with open simple prostatectomy.

Objective: This study sought to compare the incidental prostate cancer (iPCa) detection rate between pathological specimens from green laser enucleation of the prostate (GreenLEP) and open simple prostatectomy (OSP).

Materials And Methods: In two institutions, the charts of all consecutive patients who underwent OSP between January 2005 and December 2010 were retrospectively reviewed, and the data of all consecutive patients who underwent GreenLEP with tissue morcellation between July 2013 and January 2018 were also collected. Preoperative demographics and pathological findings were recorded.

Author Correction: Dystonia.

The affiliation for Enza Maria Valente and Antonio Pisani at IRCCS Santa Lucia Foundation, Rome, Italy, has been amended to remove the laboratory designation. Additionally, in Figure 3a, the subthalamic nucleus was incorrectly included in the anatomy of the brain shown and this has been amended.

Integrated Multi-omic Analysis of Esthesioneuroblastomas Identifies Two Subgroups Linked to Cell Ontogeny.

Esthesioneuroblastoma (ENB) is a rare cancer of the olfactory mucosa, with no established molecular stratification to date. We report similarities of ENB with tumors arising in the neural crest and perform integrative analysis of these tumors. We propose a molecular-based subtype classification of ENB as basal or neural, both of which have distinct pathological, transcriptomic, proteomic, and immune features.

Sensing of red blood cells with decreased membrane deformability by the human spleen.

The current paradigm in the pathogenesis of several hemolytic red blood cell disorders is that reduced cellular deformability is a key determinant of splenic sequestration of affected red cells. Three distinct features regulate cellular deformability: membrane deformability, surface area-to-volume ratio (cell sphericity), and cytoplasmic viscosity. By perfusing normal human spleens ex vivo, we had previously showed that red cells with increased sphericity are rapidly sequestered by the spleen.

Dystonia.

Dystonia is a neurological condition characterized by abnormal involuntary movements or postures owing to sustained or intermittent muscle contractions. Dystonia can be the manifesting neurological sign of many disorders, either in isolation (isolated dystonia) or with additional signs (combined dystonia). The main focus of this Primer is forms of isolated dystonia of idiopathic or genetic aetiology.

Coupling between death spikes and birth troughs. Part 1: Evidence.

In the wake of the influenza pandemic of 1889-1890 Jacques Bertillon, a pioneer of medical statistics, noticed that after the massive death spike there was a dip in birth numbers around 9 months later which was significantly larger than that which could be explained by the population change as a result of excess deaths. In addition it can be noticed that this dip was followed by a birth rebound a few months later. However having made this observation, Bertillon did not explore it further.

The role of KIBRA in reconstructive episodic memory.

Background: In order to retrieve episodic past events, the missing information needs to be reconstructed using information stored in semantic memory. Failures in these reconstructive processes are expressed as false memories. KIBRA single nucleotide polymorphism (rs17070145) has been linked to episodic memory performance as well as an increased risk of Alzheimer's disease and post-traumatic stress disorder (PTSD).

Identification of Positively and Negatively Selected Driver Gene Mutations Associated With Colorectal Cancer With Microsatellite Instability.

Background & Aims: Recent studies have shown that cancers arise as a result of the positive selection of driver somatic events in tumor DNA, with negative selection playing only a minor role, if any. However, these investigations were concerned with alterations at nonrepetitive sequences and did not take into account mutations in repetitive sequences that have very high pathophysiological relevance in the tumors showing microsatellite instability (MSI) resulting from mismatch repair deficiency investigated in the present study.

Methods: We performed whole-exome sequencing of 47 MSI colorectal cancers (CRCs) and confirmed results in an independent cohort of 53 MSI CRCs.

Valproic acid is protective in cellular and worm models of oculopharyngeal muscular dystrophy.

Objective: To explore valproic acid (VPA) as a potentially beneficial drug in cellular and worm models of oculopharyngeal muscular dystrophy (OPMD).

Methods: Using a combination of live cell imaging and biochemical measures, we evaluated the potential protective effect of VPA in a stable C2C12 muscle cell model of OPMD, in lymphoblastoid cell lines derived from patients with OPMD and in a transgenic OPMD model expressing human mutant PABPN1.

Results: We demonstrated that VPA protects against the toxicity of mutant PABPN1.

A Proposal for a Classification for Recurrent Endometrial Cancer: Analysis of a French Multicenter Database From the FRANCOGYN Study Group.

Objective: Endometrial cancer (EC) recurrences are relatively common with no standardized way of describing them. We propose a new classification for them called locoregional, nodal, metastasis, carcinomatosis recurrences (rLMNC).

Patients And Methods: The data of 1230 women with EC who were initially treated by primary surgery were included in this French multicenter retrospective study.

Intermediate filaments control collective migration by restricting traction forces and sustaining cell-cell contacts.

Mesenchymal cell migration relies on the coordinated regulation of the actin and microtubule networks that participate in polarized cell protrusion, adhesion, and contraction. During collective migration, most of the traction forces are generated by the acto-myosin network linked to focal adhesions at the front of leader cells, which transmit these pulling forces to the followers. Here, using an in vitro wound healing assay to induce polarization and collective directed migration of primary astrocytes, we show that the intermediate filament (IF) network composed of vimentin, glial fibrillary acidic protein, and nestin contributes to directed collective movement by controlling the distribution of forces in the migrating cell monolayer.

Intermittent Catheterization Adherence Scale (ICAS): A new tool for the evaluation of patient adherence with clean intermittent self-catheterization.

Aim: Patients who have successfully acquired the intermittent self-catheterization (ISC) technique can be expected to use it on a long-term basis. The aim of the present study is to present a validated evaluation scale, referred to as the ICAS (Intermittent Catheterization Adherence Scale), which can be used to assess long-term patient adherence to prescribed ISC treatment.

Methods: The scale was validated in neurologic and non neurologic patients in an experienced French neurourology center, between September 2014 and February 2016.

Autosomal dominant cerebellar ataxias: Imaging biomarkers with high effect sizes.

Objective: As gene-based therapies may soon arise for patients with spinocerebellar ataxia (SCA), there is a critical need to identify biomarkers of disease progression with effect sizes greater than clinical scores, enabling trials with smaller sample sizes.

Methods: We enrolled a unique cohort of patients with SCA1 ( = 15), SCA2 ( = 12), SCA3 ( = 20) and SCA7 ( = 10) and 24 healthy controls of similar age, sex and body mass index. We collected longitudinal clinical and imaging data at baseline and follow-up (mean interval of 24 months).