A5064477682- Renal Diseases and Glomerulopathies
- Genetic and Kidney Cyst Diseases
- Renal and related cancers
- Biomedical Research and Pathophysiology
- Cell Adhesion Molecules Research
- Chronic Kidney Disease and Diabetes
- Amyloidosis: Diagnosis, Treatment, Outcomes
- Dialysis and Renal Disease Management
- Electrolyte and hormonal disorders
- Renal and Vascular Pathologies
- Lysosomal Storage Disorders Research
- Glycogen Storage Diseases and Myoclonus
- Platelet Disorders and Treatments
- Complement system in diseases
- Pregnancy and preeclampsia studies
- Pediatric Urology and Nephrology Studies
- Bipolar Disorder and Treatment
- Blood Pressure and Hypertension Studies
- Vasculitis and related conditions
- Systemic Lupus Erythematosus Research
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Hemoglobinopathies and Related Disorders
- Cancer, Hypoxia, and Metabolism
- Blood Coagulation and Thrombosis Mechanisms
- Renin-Angiotensin System Studies
Inserm
1995-2023
Université Paris Cité
2008-2023
Institut des Maladies Génétiques Imagine
2023
Hôpital Necker-Enfants Malades
2008-2017
Délégation Paris 5
1998-2017
Assistance Publique – Hôpitaux de Paris
2005-2017
Sorbonne Paris Cité
2014
Délégation Paris 6
1997-2011
Service de la Santé Publique
2011
UniLaSalle Amiens (ESIEE-Amiens)
2011
Abstract. Even 10 yr after the identification of antiphospholipid syndrome (APS), renal involvement in course APS is still relatively unrecognized, and probably underestimated. The association anticardiolipin antibodies and/or lupus anticoagulant with development a vaso-occlusive process involving numerous organs now confirmed. In multicenter study, 16 cases “primary” (PAPS) were found followed for 5 or more, all biopsy. PAPS, there was vascular nephropathy characterized by small vessel...
The fate of octogenarians reaching end-stage renal disease (ESRD) is poorly defined, and implicit dialysis rationing may be practiced in this age group. main objectives study were to analyze the characteristics pre-ESRD offered or not identify factors influencing mortality while on dialysis, improve prognosis assessment decision-making. In single-center cohort, 146 consecutive referred a nephrology unit over 12-yr period (1989 2000). Main outcome measures baseline patients conservative...
Abnormal control of the complement alternative pathway (CAP) (factor H, factor I and membrane cofactor protein (MCP) deficiencies) is a well established risk for occurrence haemolytic uraemic syndrome (HUS). In some instances, HUS may be associated with an unusual glomerulonephritis isolated C3 deposits (glomerulonephritis C3). We determined whether share common genetic susceptibility factors.We identified 19 patients C3. measured levels circulating components, performed assays detection...
Abstract. It has been shown previously that proteinase 3 (PR3), a neutrophil intracellular protease is the main antigen of antineutrophil cytoplasm (ANCA) autoantibodies, present on plasma membrane subset freshly isolated neutrophils. This study shows size this PR3-positive (mPR3 + ) neutrophils stable feature given individual, most likely genetically controlled. ranges from 0 to 100% and allows us define new polymorphism in healthy population, with three discrete phenotypes corresponding...
Background. In Fabry nephropathy, alpha-galactosidase deficiency leads to accumulation of glycosphingolipids in all kidney cell types, proteinuria and progressive loss function.
The recent identification of mutations in the INF2 gene, which encodes a member formin family actin-regulating proteins, cases familial FSGS supports importance an intact actin cytoskeleton podocyte function. To determine better prevalence autosomal dominant FSGS, we screened 54 families (78 patients) and detected 17% them. All were missense variants localized to N-terminal diaphanous inhibitory domain protein, region that interacts with C-terminal autoregulatory domain, thereby competing...
SUMMARY Autoantibodies directed against polymorphonuclear neutrophils (PMN) have been observed in serum from patients with ulcerative colitis (UC), Crohn's disease (CD) and primary sclerosing cholangitis (PSC) using indirect immunofluorescence fixed granulocyte ELISA. Our study demonstrates the presence of these autoantibodies which bind to an azurophilic granule component distinct proteinase 3, elastase myeloperoxidase. These thus belong ANCA family, but their antigen specificity differs...