A5011042532- Neuroblastoma Research and Treatments
- Congenital gastrointestinal and neural anomalies
- Renal and related cancers
- Clinical Nutrition and Gastroenterology
- Congenital Diaphragmatic Hernia Studies
- Intestinal Malrotation and Obstruction Disorders
- Testicular diseases and treatments
- Congenital Anomalies and Fetal Surgery
- Adrenal and Paraganglionic Tumors
- Pancreatic and Hepatic Oncology Research
- Organ Transplantation Techniques and Outcomes
- Pediatric Hepatobiliary Diseases and Treatments
- Neuroendocrine Tumor Research Advances
- Renal cell carcinoma treatment
- Neurofibromatosis and Schwannoma Cases
- Ovarian cancer diagnosis and treatment
- Esophageal and GI Pathology
- Reproductive Biology and Fertility
- Childhood Cancer Survivors' Quality of Life
- Gastrointestinal disorders and treatments
- Tracheal and airway disorders
- T-cell and B-cell Immunology
- Sarcoma Diagnosis and Treatment
- Glioma Diagnosis and Treatment
- Urological Disorders and Treatments
Hôpital Necker-Enfants Malades
2016-2025
Assistance Publique – Hôpitaux de Paris
2016-2025
Institut des Maladies Génétiques Imagine
2017-2025
Université Paris Cité
2016-2025
Sorbonne Paris Cité
2013-2025
Centre de Recherche des Cordeliers
2024
Inserm
1995-2023
Collaborative Group (United States)
2023
University of Zurich
2023
Sydney Children's Hospital
2023
The cellular immune response to tissue damage and infection requires the recruitment of blood leukocytes. This process is mediated through a classical multistep mechanism, which involves transient rolling on endothelium recognition inflammation followed by extravasation. We have shown, direct examination monocyte functions in vivo, that subset monocytes patrols healthy tissues long-range crawling resting endothelium. patrolling behavior depended integrin LFA-1 chemokine receptor CX(3)CR1 was...
Neuroblastoma is an enigmatic disease entity; some tumors disappear spontaneously without any therapy, while others progress with a fatal outcome despite the implementation of maximal modern therapy. However, strong prognostic factors can accurately predict whether children have "good" or "bad" at diagnosis, and clinical stage currently most significant clinically relevant factor. Therefore, for individual patient, proper staging paramount importance risk assessment selection optimal...
Abstract Purpose: This single-institutional feasibility study prospectively characterized genomic alterations in recurrent or refractory solid tumors of pediatric patients to select a targeted therapy. Experimental Design: Following treatment failure, with signed consent and ages above 6 months, underwent tumor biopsy surgical resection primary metastatic site. These newly acquired samples were analyzed by comparative hybridization array, next-generation sequencing for 75 target genes,...
Mouse splenic marginal zone precursors (MZPs) differentiate into B (MZB) cells under a signaling pathway involving Notch2 and its ligand, delta-like 1 ligand (Dll1). We report the identification of an MZP subset in spleen young children. These MZPs MZ-like vitro presence OP9 expressing human DLL1, as demonstrated by up-regulation classical MZB cell markers. A set diagnostic genes discriminating IgM+IgD+CD27+ blood from switched was identified (up-regulation SOX7, down-regulation TOX, COCH,...
Mouse splenic marginal zone precursors (MZPs) differentiate into B (MZB) cells under a signaling pathway involving Notch2 and its ligand, delta-like 1 ligand (Dll1). We report the identification of an MZP subset in spleen young children. These MZPs MZ-like vitro presence OP9 expressing human DLL1, as demonstrated by up-regulation classical MZB cell markers. A set diagnostic genes discriminating IgM(+)IgD(+)CD27(+) blood from switched was identified (up-regulation SOX7, down-regulation TOX,...
Abstract Purpose To evaluate the impact of image‐defined risk factor (IDRF) modification after chemotherapy on surgical outcomes, event‐free survival (EFS), and overall (OS) among patients enrolled in European Unresectable Neuroblastoma (EUNB) study. Methods IDRFs were assigned according to corresponding factors list reported database. Surgical EFS, OS related IDRF with chemotherapy. The predictive value preoperative for outcomes was analyzed. Cox proportional hazards models EFS OS,...
The preservation of fertility is an integral part care children requiring gonadotoxic treatments for cancer or non-malignant diseases. In France, the cryopreservation ovarian tissue has been considered and offered as a clinical treatment since its inception. aim this study to review 20 years activity in by (OTC) feasibility oocyte isolation from at single center. Retrospective including patients aged 15 younger who underwent OTC, combined some with isolated oocytes, before highly malignant...
To evaluate the impact of surgeon-assessed extent primary tumor resection on local progression and survival in patients International Society Pediatric Oncology Europe Neuroblastoma Group High-Risk 1 trial.Patients recruited between 2002 2015 with stage 4 disease > year or 4/4S MYCN amplification < who had completed induction without progression, achieved response criteria for high-dose therapy (HDT), no before were included. Data collected excision, severe operative complications, outcome.A...
PIK3CA-related overgrowth spectrum (PROS) includes rare genetic conditions due to gain-of-function mutations in the PIK3CA gene. There is no approved medical therapy for patients with PROS, and alpelisib, an inhibitor oncology, showed promising results preclinical models patients. Here, we report first time outcome of two infants PROS having life-threatening treated alpelisib (25 mg) monitored pharmacokinetics. Patient 1 was 8-mo-old girl voluminous vascular malformation. 2 a 9-mo-old boy...
ABSTRACT The intestinal stage of listeriosis was studied in a rat ligated ileal loop system. Listeria monocytogenes translocated to deep organs with similar efficiencies after inoculation loops or without Peyer’s patches. Bacterial seeding demonstrated as early 15 min inoculation. It dose dependent and nonspecific, the ΔinlAB , Δhly ΔactA L. mutants nonpathogenic species, innocua similarly wild-type strains. levels uptake listeriae by patches villous intestine were low, 50 250 CFU per cm 2...
Oesophageal atresia (OA) and mandibulofacial dysostosis (MFD) are two congenital malformations for which the molecular bases of syndromic forms being identified at a rapid rate. In particular, EFTUD2 gene encoding protein spliceosome complex has been found mutated in patients with MFD microcephaly (MIM610536). Until now, no syndrome featuring both OA clearly delineated.We report on 10 cases presenting MFD, eight whom had OA, either due to de novo 17q21.31 deletions encompassing neighbouring...
Ovarian granulosa cell tumors are the most common sex-cord stromal and have juvenile (JGCTs) adult forms. In a previous study we reported occurrence of activating somatic mutations Gαs, which transduces mitogenic signals, in 30% analyzed JGCTs.We searched for alterations other proteins involved ovarian signaling. We focused on PI3K-AKT axis. As found AKT1, subcellular localization mutated performed functional explorations using Western-blot luciferase assays.We detected in-frame duplications...
Abstract Background Wilms Tumor (WT) can occur in association with tumor predisposition syndromes and/or clinical malformations. These associations have not been fully characterized at a and molecular genetic level. This study aims to describe malformations, abnormalities, patients WT propose guidelines regarding indications for explorations. Procedure retrospective analyzed abnormalities among 295 treated between 1986 2009 single pediatric oncological center. Results Clinically identified...
Patients with neuroblastoma are now stratified at diagnosis according to the presence and number of image-defined risk factors (IDRFs). We examined added value IDRF assessment after neoadjuvant chemotherapy for predicting surgical resection.From 2009-2012, 39 out 91 patients operated on in our institution neuroblastic tumors received based ongoing SIOPEN protocols or treatment guidelines. IDRFs were assessed both preoperatively CT and/or MRI.Median age was 30 months [range 2-191]. The tumor...
Abstract BACKGROUND The growing use of abdominal ultrasonography during pregnancy and in the postnatal period is leading to discovery an increasing number suprarenal masses. optimal diagnosis treatment these masses has not yet been determined. METHODS authors reviewed files patients with detected prenatally or first 3 months life, between 1986 1999, pediatric surgery oncology departments Paris hospitals. RESULTS Thirty were 23 postnatally. In latter group patients, was based on ultrasound 8...
The recently established International Consortium on Anorectal Malformations aims to identify genetic and environmental risk factors in the etiology of syndromic nonsyndromic anorectal malformations (ARM) by promoting collaboration through data sharing combined research activities. consortium attempts recruit at least 1,000 ARM cases. DNA samples are collected from case–parent triads involved ARM. Several techniques will be applied, including SNP arrays, gene whole exome sequencing, a...
Minimally invasive surgery (MIS) is still not a well-accepted surgical approach to remove neuroblastic tumors. We aimed assess the indications and limits of MIS in this childhood tumor according location image-defined risk factors (IDRFs).Between 2006 2012, 39 patients underwent for tumors, using thoracoscopic (n = 20), retroperitoneoscopic 1) or laparoscopic approaches 18). The locations were paravertebral 18; thoracic n 15, lumbar 3), perivascular 5; abdominal 2; adrenal 13), pleural 2)...