A5047401981- Pancreatic and Hepatic Oncology Research
- Pediatric Hepatobiliary Diseases and Treatments
- Hepatocellular Carcinoma Treatment and Prognosis
- Liver Disease Diagnosis and Treatment
- Neuroendocrine Tumor Research Advances
- Liver Disease and Transplantation
- Neuroblastoma Research and Treatments
- Liver physiology and pathology
- Organ Transplantation Techniques and Outcomes
- Metabolism and Genetic Disorders
- Cholangiocarcinoma and Gallbladder Cancer Studies
- Pancreatitis Pathology and Treatment
- Liver Diseases and Immunity
- Hepatitis B Virus Studies
- Genetic and Kidney Cyst Diseases
- Gastrointestinal disorders and treatments
- Drug Transport and Resistance Mechanisms
- Pancreatic function and diabetes
- Viral-associated cancers and disorders
- RNA modifications and cancer
- Renal and related cancers
- Virus-based gene therapy research
- Cancer-related gene regulation
- Sarcoma Diagnosis and Treatment
- Eosinophilic Disorders and Syndromes
Assistance Publique – Hôpitaux de Paris
2015-2024
Hôpital Necker-Enfants Malades
2015-2024
Université Paris Cité
1999-2024
Sorbonne Université
1990-2020
Inserm
1992-2020
Centre National de la Recherche Scientifique
2006-2020
Institut de Biologie Paris-Seine
2020
Institut Gustave Roussy
1998-2018
Bordeaux Population Health
2017
Université de Bordeaux
2017
Hepatocellular carcinoma (HCC) is the major primary malignant tumor in human liver, but molecular changes leading to liver cell transformation remain largely unknown. The Wnt-β-catenin pathway activated colon cancers and some melanoma lines, has not yet been investigated HCC. We have examined status of β-catenin gene different transgenic mouse lines HCC obtained with oncogenes c- myc or H- ras . Fifty percent hepatic tumors these mice had activating somatic mutations within similar those...
Hepatocellular adenomas are benign tumors that can be difficult to diagnose. To refine their classification, we performed a comprehensive analysis of genetic, pathological, and clinical features. A multicentric series 96 liver with firm or possible diagnosis hepatocellular adenoma was reviewed by pathologists. In all cases, the genes coding for hepatocyte nuclear factor 1alpha (HNF1alpha) beta-catenin were sequenced. No mutated in both HNF1alpha enabling classified into 3 groups, according...
Progressive familial intrahepatic cholestasis (PFIC) types 1 and 2 are characterized by normal serum gamma-glutamyl transferase (GGT) activity due to mutations in ATP8B1 (encoding FIC1) ABCB11 bile salt export pump [BSEP]), respectively. Our goal was evaluate the features that may distinguish PFIC1 from PFIC2 ease their diagnosis. We retrospectively reviewed charts of 62 children with normal-GGT PFIC whom a search for and/or mutation, liver BSEP immunostaining, analysis were performed. Based...
Microbial nucleic acid recognition serves as the major stimulus to an antiviral response, implying a requirement limit misrepresentation of self acids non-self and induction autoinflammation. By systematic screening using panel interferon-stimulated genes we identify two siblings singleton variably demonstrating severe neonatal anemia, membranoproliferative glomerulonephritis, liver fibrosis, deforming arthropathy increased anti-DNA antibodies. In both families biallelic mutations in DNASE2,...
OBJECTIVE: Preoperative diagnosis of cystic lesions the pancreas remains difficult despite improvement in imaging modalities and fluid analysis. The aim our study was to assess performance endoscopic ultrasonography (EUS) EUS-guided fine needle aspiration (FNA) pancreatic lesions. METHODS: Data from a series 127 consecutive patients with were prospectively studied. EUS FNA performed all patients, material used for cytological histological analysis as well biochemical tumor markers...
ABSTRACT Background and Objective: Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was propose a clear definition the risks congenital in children according our experience review literature. Patients Methods: Twenty‐two with complicated shunt were studied institution. When necessary, included portal pressure measurement vein angiography during an occlusion test closure by surgical...
The aim of this study was to review our experience in orthotopic liver transplantation (OLT) for biliary atresia (BA) children and analyze the survival prognostic factors, long-term outcome. We reviewed 332 OLTs performed 280 between years 1986 2000. Univariate multivariate analysis were on patient graft survivals according recipients' donors' characteristics as well intraoperative data. outcome among 80 living at 10 after OLT studied growth, immunosuppressive therapy, renal functions. Liver...
Lipid peroxidation is an autocatalytic mechanism leading to oxidative destruction of cellular membranes. The deleterious consequences this are related in part the formation reactive aldehydic products that bind intra- or extracellular molecules form adducts. Specific antibodies directed against malondialdehyde (MDA) and 4-hydroxynonenal (HNE) adducts, major metabolites lipid peroxidation, allowed us investigate situ, with immunohistochemical procedure, occurrence a panel different chronic...
Myc activation has been implicated in the pathogenesis of hepatoblastoma (HB), a rare embryonal neoplasm derived from liver progenitor cells. Here, microRNA (miR) expression profiling 65 HBs evidenced differential patterns related to developmental stage and activity. Undifferentiated aggressive overexpressed miR-371–3 cluster with concomitant down-regulation miR-100/let-7a-2/miR-125b-1 cluster, evoking an ES cell profile. ChIP inhibition assays hepatoma cells demonstrated that both miR...
To evaluate the feasibility of using supersonic shear-wave elastography (SSWE) in children and normal values liver stiffness with use control patients different ages (from neonates to teenagers) diagnostic accuracy shear wave for assessing fibrosis by histologic scoring system as reference method disease, a special concern early stages fibrosis.The institutional review board approved this prospective study. Informed consent was obtained from parents older than 7 years. First, 51 healthy...
Phenotypic identification of focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCA) subtypes using immunohistochemical markers has been developed from their molecular characteristics. Our objective was to evaluate the sensitivity these in definitive diagnosis lesions by core needle biopsies. A total 239 biopsies paired with surgical resection specimen (group A) or without an associated B) were reviewed. Using a step-by-step algorithm after standard staining, appropriate...
Surgery and cisplatin‐based treatment of hepatoblastoma (HB) currently guarantee the survival 70%‐80% patients. However, some important challenges remain in diagnosing high‐risk tumors identifying relevant targetable pathways offering new therapeutic avenues. Previously, two molecular subclasses HB have been described, C1 C2, with C2 being subgroup poorest prognosis, a more advanced tumor stage, worst overall rate. An associated 16‐gene signature to discriminate tumoral subgroups was...
<b>Background and study aims:</b> A new core biopsy needle for endoscopic ultrasound (EUS)-guided sampling has recently been developed. The aim of this prospective multicenter was to compare with a standard in patients solid pancreatic masses. <b>Patients methods:</b> Consecutive masses referred 17 centers EUS-guided were included. Each patient had two passes 22G single pass performed randomized order. Samples from both needles separately processed liquid-based cytology cell-block...
Hepatoblastoma (HB) is a rare disease. Nevertheless, it the predominant pediatric liver cancer, with limited therapeutic options for patients aggressive tumors. Herein, we aimed to uncover mechanisms of HB pathobiology and identify new biomarkers targets in move towards precision medicine advanced HB.We performed comprehensive genomic, transcriptomic epigenomic characterization 159 clinically annotated samples from 113 HB, using high-throughput technologies.We discovered widespread...
Abstract Pediatric liver cancers (PLC) comprise diverse diseases affecting infants, children, and adolescents. Despite overall good prognosis, PLCs display heterogeneous response to chemotherapy. Integrated genomic analysis of 126 pediatric tumors showed a continuum driver mechanisms associated with patient age, including new targetable oncogenes. In 10% patients hepatoblastoma, all before three years old, we identified mosaic premalignant clonal expansion cells altered at the 11p15.5 locus....
Abstract Hepatoblastomas (HB) display heterogeneous cellular phenotypes that influence the clinical outcome, but underlying mechanisms are poorly understood. Here, we use a single-cell multiomic strategy to unravel molecular determinants of this plasticity. We identify continuum HB cell states between hepatocytic (scH), liver progenitor (scLP) and mesenchymal (scM) differentiation poles, with an intermediate scH/LP population bordering scLP scH areas in spatial transcriptomics. Chromatin...