- [Hereditary angiopathy with nephropathy, aneurysms and muscle cramps (HANAC): a new basement membrane-disease associated with mutations of the COL4A1 gene]Pierre Ronco
Néphrologie et Dialyses, Unité INSERM UMR S 702, UPMC, Hopital Tenon, Paris
Bull Acad Natl Med 192:971-84; discussion 984-6. 2008..We discuss phenotype-genotype correlations and the implications of the HANAC syndrome for the diagnosis of autosomal dominant hematuria, cystic kidney disease, intracranial aneurysms, and muscle cramps...
- Anti-glomerular basement membrane nephritis and bullous pemphigoid caused by distinct anti-alpha 3(IV)NC1 and anti-BP180 antibodies in a patient with Crohn's diseaseEmmanuelle Plaisier
INSERM U489 and Department of Nephrology, Hopital Tenon, Assistance Publique Hopitaux de Paris, Paris, France
Am J Kidney Dis 40:649-54. 2002..We report a patient with Crohn's disease who developed anti-GBM nephritis and the skin blistering disorder bullous pemphigoid, owing to distinct autoantibodies...
- Identification of two candidate collecting duct cell-specific cis-acting elements in the Hoxb-7 promoter regionEmmanuelle Plaisier
INSERM Unit 489 and University Paris VI, Paris, France
Biochim Biophys Acta 1727:106-15. 2005..CDSE-2 (-34/-13) contains sequence bears high homology with a segment of the Pax-2 promoter. CDSE-2 also conveys cell specificity but has no enhancer activity by itself...
- Autosomal-dominant familial hematuria with retinal arteriolar tortuosity and contractures: a novel syndromeEmmanuelle Plaisier
INSERM Unit 489 Departments of Neurology, and Nephrology, Tenon Hospital AP HP, Paris, France
Kidney Int 67:2354-60. 2005..Patients with thin basement membrane (BM) disease do not have extrarenal manifestations, while those with Alport syndrome often present with hearing loss, anterior lenticonus, and dot-and-fleck retinopathy...
- Glomerular permeability is altered by loss of P0, a myelin protein expressed in glomerular epithelial cellsEmmanuelle Plaisier
Department of Nephrology, INSERM Unit 702, Tenon Hospital AP HP, University Pierre et Marie Curie, Paris, France
J Am Soc Nephrol 16:3350-6. 2005..These results demonstrate that P0, the major myelin protein, is also expressed during nephrogenesis and in mature kidney, mostly in podocytes. They suggest that P0 gene mutations might be involved in renal diseases...
- Predictors of early relapse in patients with non-infectious mixed cryoglobulinemia vasculitis: results from the French nationwide CryoVas surveyBenjamin Terrier
Department of Internal Medicine, National Reference Center for Autoimmune Diseases, Hopital Cochin, Assistance Publique Hopitaux de Paris AP HP, Université Pierre Descartes, Paris 5, Paris, France
Autoimmun Rev 13:630-4. 2014..We aimed to identify predictors of early relapses occurring within the first 12months of treatment in such patients...
- Tubulointerstitial nephropathies in HIV-infected patients over the past 15 years: a clinico-pathological studyMohamad Zaidan
Assistance Publique Hopitaux de Paris, Department of Nephrology and Dialysis, Tenon Hospital, Paris, France
Clin J Am Soc Nephrol 8:930-8. 2013..This study analyzed the characteristics of HIV-infected patients and biopsy-proven tubulointerstitial nephropathies to define disease patterns and therapeutic implications...
- COL4A1 mutations and hereditary angiopathy, nephropathy, aneurysms, and muscle crampsEmmanuelle Plaisier
INSERM Unité 702, Universite Pierre et Marie Curie, Paris 6, Unités Mixtes de Recherche Scientifique 702, Assistance Publique Hopitaux de Paris, Hopital Tenon, France
N Engl J Med 357:2687-95. 2007..However, the causative genes for a number of hereditary multicystic kidney diseases, myopathies with cramps, and heritable intracranial aneurysms remain unknown...
- The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 casesBenjamin Terrier
Department of Internal Medicine, Groupe Hospitalier Pitie Salpetriere, Assistance Publique Hopitaux de Paris, Universite Pierre et Marie Curie, Paris 6, Paris, France
Medicine (Baltimore) 92:61-8. 2013..Besides alkylating agents, the use of regimens based on rituximab, thalidomide or lenalinomide, and bortezomib are interesting alternative options, although the exact role of each strategy remains to be defined...
- Novel COL4A1 mutations associated with HANAC syndrome: a role for the triple helical CB3[IV] domainEmmanuelle Plaisier
AP HP, Department of Nephrology, Tenon Hospital, INSERM U702, UMRS702, Universite Pierre et Marie Curie Paris 6, Paris, France
Am J Med Genet A 152:2550-5. 2010....
- HANAC Col4a1 Mutation in Mice Leads to Skeletal Muscle Alterations due to a Primary Vascular DefectSimon Guiraud
INSERM, UMRS1155, Paris, France Sorbonne Universités, UPMC Univ Paris 06, UMR1155, Paris, France Medical Research Council Functional Genomics Unit, Department of Physiology, Anatomy, and Genetics, University of Oxford, Oxford, United Kingdom
Am J Pathol . 2017....
- HANAC Syndrome Col4a1 Mutation Causes Neonate Glomerular Hyperpermeability and Adult Glomerulocystic Kidney DiseaseZhiyong Chen
Institut National de la Sante et de la Recherche Medicale, Unité Mixte de Recherche UMR S 1155, Paris, France
J Am Soc Nephrol 27:1042-54. 2016....
- Rituximab for Severe Membranous Nephropathy: A 6-Month Trial with Extended Follow-UpKarine Dahan
Department of Nephrology and Dialysis, Assistance Publique Hopitaux de Paris, Hopital Tenon, Paris, France
J Am Soc Nephrol . 2016..01). Positive effect of rituximab on proteinuria remission occurred after 6 months. These data suggest that PLA2R-Ab levels are early markers of rituximab effect and that addition of rituximab to NIAT does not affect safety...
- HIV-associated kidney glomerular diseases: changes with time and HAARTFrancois Xavier Lescure
Department of Infectious and Tropical Diseases, Tenon Hospital, AP HP, Paris, France
Nephrol Dial Transplant 27:2349-55. 2012..Our objective was to assess the change in distribution of the glomerular diseases in HIV patients...
- [Kidney and HIV infection]Emmanuelle Plaisier
Hopital Tenon, Service de Néphrologie et Dialyses, 75970 Paris, France
Presse Med 41:267-75. 2012..Proximal renal tubular toxicity must be further evaluated in the presence of eGFR decrease and/or hypophosphatemia under tenofovir therapy...
- Patterns of noncryoglobulinemic glomerulonephritis with monoclonal Ig deposits: correlation with IgG subclass and response to rituximabElsa Guiard
Department Nephrology, Hopital Europeen Georges Pompidou, Paris, France
Clin J Am Soc Nephrol 6:1609-16. 2011..The aim of this study was to describe the distribution of the different pathologic subtypes of IgG-associated glomerulopathy and to evaluate the IgG isotype involved in these diseases...
- Monoclonal immunoglobulin light and heavy chain deposition diseases: molecular models of common renal diseasesPierre Ronco
UPMC Univ Paris 06, Hopital Tenon, Service de Néphrologie et Dialyses, Paris, France
Contrib Nephrol 169:221-31. 2011....
- D-Penicillamine-induced ANCA-associated crescentic glomerulonephritis in Wilson diseaseFrank Bienaime
Department of Nephrology and Dialysis, Tenon Hospital, APHP, Paris, France
Am J Kidney Dis 50:821-5. 2007..The presentation and rapidly progressive and potentially severe outcome of this complication dramatically contrast with those of membranous and minimal change glomerulopathy, also induced by the sulfhydryl compound...
- Spectrum and Prognosis of Noninfectious Renal Mixed Cryoglobulinemic GNMohamad Zaidan
Departments of Nephrology Transplantation, Institut Necker Enfants Malades, Paris, France Paris Descartes University, Sorbonne Paris Cité, Paris, France
J Am Soc Nephrol 27:1213-24. 2016..Severe infections and new-onset B-cell lymphoma occurred in 29.1% and 8.9% of cases, respectively; 24% of patients died. In conclusion, noninfectious MCGN has a poor long-term outcome with severe infections as the main cause of death...
- Bilateral kidney infarction due to primary Al amyloidosis: a first case reportFabrice Mihout
From the Department of Nephrology and Dialysis FM, LJ, PR, EP Department of Pathology IB, Tenon Hospital, APHP, Paris, France Clinical Hematology Unit VL and Department of Cardiac Surgery SV, La Pitie Salpetriere Hospital, APHP, F 75013 Paris, France
Medicine (Baltimore) 94:e777. 2015..In addition to the very uncommon presentation, this case illustrates the tremendous progress that has occurred in the management of severe forms of AL amyloidosis. ..
- Acute metabolic acidosis in a GLUT2-deficient patient with Fanconi-Bickel syndrome: new pathophysiology insightsFabrice Mihout
Department of Nephrology and Dialysis, AP HP, Tenon Hospital, Paris, France
Nephrol Dial Transplant 29:iv113-6. 2014..These profound alterations of the transport systems most likely contributed to proximal tubule alterations and profound bicarbonate loss. ..
- Rituximab treatment for membranous nephropathy: a French clinical and serological retrospective study of 28 patientsPierre Antoine Michel
Service de Néphrologie et Dialyses, Hopital Tenon, Assistance Publique Hopitaux de Paris, France
Nephron Extra 1:251-61. 2011..Antibodies became negative in all 5 responsive patients with available follow-up sera. In this retrospective study, a high rate of remission was achieved 12 months after treatment...
- Ig-related renal disease in lymphoplasmacytic disorders: an updatePierre Ronco
Assistance Publique Hopitaux de Paris, Hopital Tenon, Service de Néphrologie et Dialyses, Paris, France
Semin Nephrol 30:557-69. 2010..In the past 10 years, considerable advances have occurred in chemotherapy regimens with the advent of new classes of drugs, which already result in markedly improved renal and vital survival...
- INF2 mutations in Charcot-Marie-Tooth disease with glomerulopathyOlivia Boyer
INSERM Unité 983, Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, Assistance Publique Hopitaux de Paris, Paris, France
N Engl J Med 365:2377-88. 2011..We therefore hypothesized that INF2 may be responsible for cases of Charcot-Marie-Tooth neuropathy associated with FSGS...
- Immunoglobulin light (heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapyPierre Ronco
INSERM UMR S 702, Universite Pierre et Marie Curie Paris 6, Paris, France
Clin J Am Soc Nephrol 1:1342-50. 2006....
- Renal outcomes after up to 8 years of tenofovir exposure in HIV-HBV-coinfected patientsAnders Boyd
INSERM, UMR_S1136, Institut Pierre Louis d Epidémiologie et de Santé Publique, Paris, France
Antivir Ther . 2016..Nevertheless, little is known regarding TDF-use on renal impairment during HIV-HBV coinfection. We aimed to evaluate the progression and determinants of renal impairment in coinfected patients undergoing TDF...
- Systemic vasculitis associated with vemurafenib treatment: Case report and literature reviewAdrien Mirouse
aAPHP, Service de médecine interne et immunologie clinique, Groupe Hospitalier Pitié Salpêtrière bDHU Inflammation, Immunopathologie, biothérapie, Université Pierre et Marie Curie cAPHP, Service de Dermatologie, Groupe Hospitalier Pitié Salpêtrière dAPHP, Service de néphrologie et dialyse, Hôpital Tenon eAPHP, Service d Anatomopathologie, Groupe Hospitalier Pitie Salpetriere, Paris, France
Medicine (Baltimore) 95:e4988. 2016..Inflammatory adverse effects have been increasingly reported after vemurafenib treatment...
- Acute renal failure associated with immune restoration inflammatory syndromeEric Daugas
Department of Nephrology, Tenon Hospital, Paris, France
Nat Clin Pract Nephrol 2:594-8; quiz 599. 2006..A renal biopsy showed acute interstitial nephritis. Extensive investigations failed to detect active infection. The efficacy of HAART was attested by increased CD4+ cell counts and undetectable viral replication...
- Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas surveyBenjamin Terrier
Department of Internal Medicine, Groupe Hospitalier Pitie Salpetriere, Assistance Publique des Hopitaux de Paris, Universite Pierre et Marie Curie, 47 83 boulevard de l Hopital, Paris, France
Blood 119:5996-6004. 2012..The role of each of these strategies remains to be defined in well-designed randomized controlled trials...
- Efficacy and tolerability of rituximab with or without PEGylated interferon alfa-2b plus ribavirin in severe hepatitis C virus-related vasculitis: a long-term followup study of thirty-two patientsBenjamin Terrier
Department of Internal Medicine, Groupe Hospitalier Pitie Salpetriere, Assistance Publique Hopitaux de Paris AP HP, CNRS UMR 7211, and Université Pierre et Marie Curie Paris 6, Paris, France
Arthritis Rheum 60:2531-40. 2009..To report on the long-term followup of a cohort of patients with hepatitis C virus (HCV)-related vasculitis treated with rituximab with or without PEGylated interferon alfa-2b (PEG-IFN alfa-2b) plus ribavirin...