P Niaudet

Summary

Country: France

Publications

  1. ncbi request reprint Respiratory chain deficiency presenting as congenital nephrotic syndrome
    Alice Goldenberg
    Departement de Genetique Medicale, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France
    Pediatr Nephrol 20:465-9. 2005
  2. ncbi request reprint A multicenter, open-label, pharmacokinetic/pharmacodynamic safety, and tolerability study of basiliximab (Simulect) in pediatric de novo renal transplant recipients
    Gisela Offner
    Kinderklinik der Medizinische Hochschule, Hannover, Germany
    Transplantation 74:961-6. 2002
  3. ncbi request reprint [Henoch-Schönlein purpura]
    P Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149, rue de Sevres, 75015 Paris, France
    Arch Pediatr 13:599-601. 2006
  4. ncbi request reprint [Nephronophtisis]
    Patrick Niaudet
    Service de néphrologie pédiatrique et Inserm U574, Hopital Necker Enfants Malades, 149, rue de Sevres, 75743 Paris Cedex 15, France
    Nephrol Ther 2:200-6. 2006
  5. ncbi request reprint [Hemolytic and uremic syndrome in the child]
    Patrick Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 5743 Paris cedex 15, France
    Nephrol Ther 4:34-40. 2008
  6. pmc Nephronophthisis
    Remi Salomon
    Pediatric Nephrology, Centre de Référence des Maladies Rénales Héréditaires de l Enfant et de l Adulte, Hopital Necker Enfants Malades, Paris, France
    Pediatr Nephrol 24:2333-44. 2009
  7. doi request reprint Enteric-coated mycophenolate sodium in de novo pediatric renal transplant patients
    Patrick Niaudet
    Pediatric Nephrology, Necker Hospital, 161 rue de Sevres, 75743, Paris, Cedex, France
    Pediatr Nephrol 24:395-402. 2009
  8. ncbi request reprint One-year routine renal transplant biopsies in children
    J P Benamenyo
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France
    Pediatr Nephrol 16:971-7. 2001
  9. ncbi request reprint A pharmacokinetic study of Neoral in childhood steroid-dependent nephrotic syndrome
    P Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, Paris, France
    Pediatr Nephrol 16:154-5. 2001
  10. ncbi request reprint WT1 and glomerular diseases
    Patrick Niaudet
    Service de Néphrologie Pédiatrique and INSERM U574, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743, Paris, Cedex 15, France
    Pediatr Nephrol 21:1653-60. 2006

Detail Information

Publications68

  1. ncbi request reprint Respiratory chain deficiency presenting as congenital nephrotic syndrome
    Alice Goldenberg
    Departement de Genetique Medicale, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France
    Pediatr Nephrol 20:465-9. 2005
    ..Mitochondrial RC complex II+V deficiency was identified in the three patients. Based on these observations, we suggest that RC disorders should be considered in patients with congenital NS...
  2. ncbi request reprint A multicenter, open-label, pharmacokinetic/pharmacodynamic safety, and tolerability study of basiliximab (Simulect) in pediatric de novo renal transplant recipients
    Gisela Offner
    Kinderklinik der Medizinische Hochschule, Hannover, Germany
    Transplantation 74:961-6. 2002
    ..We report on the safety and preliminary efficacy of basiliximab in pediatric de novo renal transplant recipients...
  3. ncbi request reprint [Henoch-Schönlein purpura]
    P Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149, rue de Sevres, 75015 Paris, France
    Arch Pediatr 13:599-601. 2006
  4. ncbi request reprint [Nephronophtisis]
    Patrick Niaudet
    Service de néphrologie pédiatrique et Inserm U574, Hopital Necker Enfants Malades, 149, rue de Sevres, 75743 Paris Cedex 15, France
    Nephrol Ther 2:200-6. 2006
    ..Several genes which are involved in nephronophtisis, encode proteins that localize in different cell compartments, in particular to the primary apical cilia, as it is the case for many other cystic kidney diseases...
  5. ncbi request reprint [Hemolytic and uremic syndrome in the child]
    Patrick Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 5743 Paris cedex 15, France
    Nephrol Ther 4:34-40. 2008
    ..Familial forms are frequent. Recurrence of HUS following renal transplantation is exceptional in the typical form but is frequent in atypical HUS...
  6. pmc Nephronophthisis
    Remi Salomon
    Pediatric Nephrology, Centre de Référence des Maladies Rénales Héréditaires de l Enfant et de l Adulte, Hopital Necker Enfants Malades, Paris, France
    Pediatr Nephrol 24:2333-44. 2009
    ..NPH and associated disorders are considered as ciliopathies, as all NPHP gene products are expressed in the primary cilia, similarly to the polycystic kidney disease (PKD) proteins...
  7. doi request reprint Enteric-coated mycophenolate sodium in de novo pediatric renal transplant patients
    Patrick Niaudet
    Pediatric Nephrology, Necker Hospital, 161 rue de Sevres, 75743, Paris, Cedex, France
    Pediatr Nephrol 24:395-402. 2009
    ..However, a larger, controlled trial is required to confirm these results...
  8. ncbi request reprint One-year routine renal transplant biopsies in children
    J P Benamenyo
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France
    Pediatr Nephrol 16:971-7. 2001
    ..Renal function remained stable for up to 10 years in patients with normal parenchyma or non-specific lesions, while serum creatinine levels increased after the 2nd year in patients with chronic allograft nephropathy...
  9. ncbi request reprint A pharmacokinetic study of Neoral in childhood steroid-dependent nephrotic syndrome
    P Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, Paris, France
    Pediatr Nephrol 16:154-5. 2001
    ..We conclude that Neoral results in an increased bioavailability of cyclosporine (CsA) as compared to Sandimmun in patients with steroid-dependent nephrotic syndrome in remission...
  10. ncbi request reprint WT1 and glomerular diseases
    Patrick Niaudet
    Service de Néphrologie Pédiatrique and INSERM U574, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743, Paris, Cedex 15, France
    Pediatr Nephrol 21:1653-60. 2006
    ..The same mutations have been observed in genetically female patients with isolated FSGS. Transmission of the mutation is possible. Frasier mutations have also been reported in children with Denys-Drash syndrome...
  11. ncbi request reprint [Lipoid nephrosis in childhood]
    Patrick Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 75743 Paris Cedex 15
    Rev Prat 53:2027-32. 2003
    ..In case of steroid resistance, the risk is that of progression to renal failure which occurs in approximately 50% of patients after 5 years. Moreover, the nephrotic syndrome may recur after renal transplantation...
  12. ncbi request reprint [Congenital and infantile nephrotic syndrome]
    Patrick Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149, rue de Sevres, 75743 Paris Cedex 15, France
    Nephrol Ther 1:63-70. 2005
    ..Renal failure develops in early childhood. Therapy is aimed to prevent oedema, denutrition, infections and thrombosis. Proteinuria does not recur after renal transplantation. Other causes are less frequent...
  13. ncbi request reprint Pretransplant blood transfusions with cyclosporine in pediatric renal transplantation
    P Niaudet
    Hopital Necker Enfants Malades, Paris, France
    Pediatr Nephrol 14:451-6. 2000
    ..Graft survival was significantly improved in this group of patients and graft loss due to rejection was exceptional. This effect should be further evaluated in prospective studies...
  14. ncbi request reprint Genetic forms of nephrotic syndrome
    Patrick Niaudet
    Pediatr Nephrol 19:1313-8. 2004
    ..Data from large cohorts indicate that the risk of recurrence of nephrotic syndrome after renal transplantation in patients with podocin mutations is very low...
  15. ncbi request reprint Treatment of lupus nephritis in children
    P Niaudet
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149, rue de Sevres, F 75015 Paris, France
    Pediatr Nephrol 14:158-66. 2000
    ..In those patients who progress to end-stage renal disease, clinical and serological remission is common and renal transplantation can be performed, as recurrence in the transplant is very rare...
  16. ncbi request reprint Long term results of liver-kidney transplantation in children with primary hyperoxaluria
    M F Gagnadoux
    Department of Pediatric Nephrology, , , 75743 Paris Cedex 15, France
    Pediatr Nephrol 16:946-50. 2001
    ..All six children or adolescents now live a normal life. From this series, we conclude that early combined liver-kidney transplantation is the treatment of choice for children with ESRF due to primary hyperoxaluria...
  17. ncbi request reprint NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome
    N Boute
    INSERM U423, Tour Lavoisier, Universite Rene Descartes, Paris, France
    Nat Genet 24:349-54. 2000
    ..We found ten different NPHS2 mutations, comprising nonsense, frameshift and missense mutations, to segregate with the disease, demonstrating a crucial role for podocin in the function of the glomerular filtration barrier...
  18. ncbi request reprint NPHS2 mutation analysis shows genetic heterogeneity of steroid-resistant nephrotic syndrome and low post-transplant recurrence
    Stefanie Weber
    Inserm U574, Necker Enfants Malades Hospital, Paris 5 University, Paris, France
    Kidney Int 66:571-9. 2004
    ..Mutations of NPHS2 are causative in familial autosomal-recessive (AR) and sporadic steroid-resistant nephrotic syndrome (SRNS). This study aimed to determine the spectrum of NPHS2 mutations and to establish genotype-phenotype correlations...
  19. doi request reprint Renal transplantation in 4 patients with methylmalonic aciduria: a cell therapy for metabolic disease
    A Brassier
    Centre de référence des maladies héréditaires du métabolisme de l enfant et de l adulte MaMEA, Hopital Necker Enfants Malades, Universite Paris Descartes, Institut Imagine, France
    Mol Genet Metab 110:106-10. 2013
    ..A kidney transplant may partially correct the metabolic dysfunctions. Liver, kidney and combined liver-kidney transplantations have been advocated but no guidelines are available to identify the most suitable organ to transplant...
  20. ncbi request reprint Intravenous cyclosporine therapy in recurrent nephrotic syndrome after renal transplantation in children
    Remi Salomon
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 75743 Paris Cedex 15, France
    Transplantation 75:810-4. 2003
    ..We report our experience with the use of intravenous (IV) cyclosporine (CsA) in children with recurrent proteinuria after renal transplantation...
  21. pmc Genotype-phenotype correlations in non-Finnish congenital nephrotic syndrome
    Eduardo Machuca
    INSERM, U983, Hopital Necker Enfants Malades, Paris, France
    J Am Soc Nephrol 21:1209-17. 2010
    ..In summary, these data demonstrate that CNS is not a distinct clinical entity in non-Finnish populations but rather a clinically and genetically heterogeneous group of disorders...
  22. doi request reprint Long-term outcome in methylmalonic aciduria: a series of 30 French patients
    M A Cosson
    Metabolic Center, Necker Enfants Malades Hospital, Assistance Publique Hopitaux de Paris, Paris, France
    Mol Genet Metab 97:172-8. 2009
    ..To better delineate the natural history of patients with methylmalonic aciduria (MMA)...
  23. ncbi request reprint WT1 splice-site mutations are rarely associated with primary steroid-resistant focal and segmental glomerulosclerosis
    E Denamur
    INSERM U458, Hopital Robert Debre, and Laboratoire d Anatomopathologic and EA 3102, Paris, France
    Kidney Int 57:1868-72. 2000
    ..The aim of this work was to investigate the possibility that some cases of primary steroid-resistant nephrotic syndrome associated with FSGS may be caused by WT1 splice-site mutations...
  24. ncbi request reprint Cyclosporin therapy in patients with Alport syndrome
    Marina Charbit
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743, Paris Cedex 15, France
    Pediatr Nephrol 22:57-63. 2007
    ..Based on these results we conclude that while cyclosporin therapy can decrease proteinuria in most patients with AS, it may be associated with nephrotoxicity, thereby precluding its long-term use...
  25. doi request reprint Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation
    J Zuber
    Assistance Publique Hopitaux de Paris, Hôpital Necker Université Paris Descartes, Sorbonne Paris Cité Department of Renal Transplantation, Paris, France
    Am J Transplant 12:3337-54. 2012
    ..Altogether these data suggest that long-term eculizumab is highly effective for preventing and treating posttransplant aHUS recurrence. Our study also indicates that anti-C5 should be promptly started if a recurrence occurs...
  26. pmc Software and database for the analysis of mutations in the human WT1 gene
    C Jeanpierre
    INSERM U383, Hopital Necker Enfants Malades, Universite Rene Descartes, Paris V, 149 rue de Sevres, 75743 Paris Cedex 15, France
    Nucleic Acids Res 26:271-4. 1998
    ..To facilitate the genotype-phenotype correlation analyses, we have created a software package along with a computerized database of germline (70 entries) and somatic (28 entries) mutations reported in the literature...
  27. doi request reprint Anti-Factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome
    M Le Quintrec
    Universite Paris Descartes, Service de Transplantation Rénale et Soins Intensifs, Hopital Necker, APHP, Paris, France
    Am J Transplant 9:1223-9. 2009
    ..This exceptional case suggests that early, specific management based on immunosuppressive therapy and plasma exchanges monitored by anti-FH IgG titer may result in long-term graft survival...
  28. doi request reprint Growth in boys with idiopathic nephrotic syndrome on long-term cyclosporin and steroid treatment
    Valerie Leroy
    Assistance Publique Hopitaux de Paris, Hopital Robert Debre, Service de Nephrologie Pediatrique, Paris, France
    Pediatr Nephrol 24:2393-400. 2009
    ..Careful monitoring of growth is recommended, given than up to 25% of patients experienced severe growth retardation during the course of their disease...
  29. doi request reprint Renal transplantation under prophylactic eculizumab in atypical hemolytic uremic syndrome with CFH/CFHR1 hybrid protein
    S Krid
    Pediatric Nephrology, Hopital Necker Enfants Malades, Paris, France
    Am J Transplant 12:1938-44. 2012
    ..We conclude that eculizumab alone, without plasma therapy (plasma infusion and/or plasma exchange), is sufficient to prevent recurrence of aHUS and to maintain long-term graft function...
  30. doi request reprint Population pharmacokinetics and pharmacogenetics of tacrolimus in de novo pediatric kidney transplant recipients
    W Zhao
    Department of Pediatric Pharmacology and Pharmacogenetics, Hopital Robert Debre, Paris, France
    Clin Pharmacol Ther 86:609-18. 2009
    ..Individualization of therapy will enable the optimization of tacrolimus exposure, with resultant beneficial effects on kidney function in the initial post-transplantation period...
  31. doi request reprint Liver hepatoblastoma and multiple OXPHOS deficiency in the follow-up of a patient with methylmalonic aciduria
    M A Cosson
    Metabolic Unit, Reference Center, Necker Enfants Malades Hospital, University Paris Descartes, 149 rue de Sevres, 75009 Paris, France
    Mol Genet Metab 95:107-9. 2008
    ..Mitochondrial dysfunction and/or post-transplant immunosuppressive therapy should be considered as a possible cause of liver cancer in this patient...
  32. pmc Identification of constitutional WT1 mutations, in patients with isolated diffuse mesangial sclerosis, and analysis of genotype/phenotype correlations by use of a computerized mutation database
    C Jeanpierre
    Institut National de la Santé et de la Recherche Médicale INSERM U423
    Am J Hum Genet 62:824-33. 1998
    ....
  33. doi request reprint Recurrence of nephrotic syndrome after transplantation in a mixed population of children and adults: course of glomerular lesions and value of the Columbia classification of histological variants of focal and segmental glomerulosclerosis (FSGS)
    Guillaume Canaud
    Department of Kidney Transplantation, Necker Hospital, 149 rue de Sevres, 75015, Paris, USA
    Nephrol Dial Transplant 25:1321-8. 2010
    ..However, the predictive value of this classification to assess the risk of recurrence after transplantation has not been addressed...
  34. ncbi request reprint Improvement of renal function in pediatric heart transplant recipients treated with low-dose calcineurin inhibitor and mycophenolate mofetil
    Olivia Boyer
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, Paris, France
    Transplantation 79:1405-10. 2005
    ..The goal of the study was to investigate the effect of a reduction of calcineurin inhibitor dosage with the concomitant introduction of mycophenolate mofetil on both renal function and cardiac allograft function...
  35. ncbi request reprint Steroid-sensitive nephrotic syndrome: from childhood to adulthood
    Fadi Fakhouri
    Department of Nephrology, Assistance Publique Hopitaux de Paris, Hopital Necker Enfants Malades, Paris, France
    Am J Kidney Dis 41:550-7. 2003
    ..We undertook to conduct a retrospective study of the outcome in adulthood of a large cohort of patients diagnosed with an SSNS during childhood...
  36. ncbi request reprint Alterations of protein metabolism by metabolic acidosis in children with chronic renal failure
    Y Boirie
    Laboratoire de Nutrition Humaine, Universite d Auvergne, Clermont Ferrand, Paris, France
    Kidney Int 58:236-41. 2000
    ..However, the effects of metabolic acidosis on protein metabolism kinetics have never been investigated in these settings...
  37. ncbi request reprint PAX2 mutations in oligomeganephronia
    R Salomon
    Pediatric Nephrologic Department, Necker Enfants Malades Hospital, Paris, France
    Kidney Int 59:457-62. 2001
    ..Heterozygous Pax2 mutants in mice are characterized by renal hypoplasia and retinal defects, and in humans, PAX2 mutations have been described in the renal-coloboma syndrome...
  38. ncbi request reprint High serum levels of a non-(1-84) parathyroid hormone (PTH) fragment in pediatric haemodialysis patients
    R Salomon
    , , , 75743 Paris Cedex 15, France
    Pediatr Nephrol 16:1011-4. 2001
    ..Although the two assays correlate well, they may provide different clinical information in some HD children which could lead to different therapeutic decisions...
  39. ncbi request reprint Mapping a gene (SRN1) to chromosome 1q25-q31 in idiopathic nephrotic syndrome confirms a distinct entity of autosomal recessive nephrosis
    A Fuchshuber
    INSERM U423, Hopital Necker Enfants Malades, Paris, France
    Hum Mol Genet 4:2155-8. 1995
    ..Exclusion of linkage to the entire region in one family proves genetic heterogeneity...
  40. ncbi request reprint Mutations in the vasopressin V2 receptor and aquaporin-2 genes in 12 families with congenital nephrogenic diabetes insipidus
    R Vargas-Poussou
    Institut National de la Santé et de la Recherche Médicale U 423, and Department of Physiology, Universite Rene Descartes, Hopital Necker Enfants Malades, Paris, France
    J Am Soc Nephrol 8:1855-62. 1997
    ..This test also identified an unexpectedly high urinary osmolality (614 mosmol/kg) in a patient with a P322S mutation of AVPR2 gene and a mild form of CNDI...
  41. ncbi request reprint [Analgesia with oxygen-nitrous oxide mixture during percutaneous renal biopsy in children]
    C Pietrement
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149, rue de Sevres, 75743 Paris, France
    Arch Pediatr 8:145-9. 2001
    ..The efficacy of an inhaled equimolar mixture of nitrous oxide and oxygen (Entonox/MEOPA) to prevent procedural pain during renal percutaneous biopsies in children was assessed...
  42. ncbi request reprint High NPHP1 and NPHP6 mutation rate in patients with Joubert syndrome and nephronophthisis: potential epistatic effect of NPHP6 and AHI1 mutations in patients with NPHP1 mutations
    Kalman Tory
    INSERM, U574, Paris, France
    J Am Soc Nephrol 18:1566-75. 2007
    ..Epistatic effects that are provided by heterozygous NPHP6 and AHI1 mutations and variants may contribute to the appearance of extrarenal symptoms in patients with NPHP1 mutations...
  43. pmc Nephrin mutations can cause childhood-onset steroid-resistant nephrotic syndrome
    Aurélie Philippe
    Inserm U574, Hopital Necker Enfants Malades, Paris, France
    J Am Soc Nephrol 19:1871-8. 2008
    ..The presence of at least one "mild" mutation in these patients likely explains the later onset and milder course of disease. These results broaden the spectrum of renal disease related to nephrin mutations...
  44. doi request reprint Therapeutic approach to focal and segmental glomerulosclerosis recurrence in kidney transplant recipients
    Guillaume Canaud
    Universite Paris Descartes, Paris, France
    Transplant Rev (Orlando) 24:121-8. 2010
    ..Recent advances in our understanding of primary FSGS and podocyte function open the way to more targeted therapies. This review summarizes the therapeutic approach to FSGS recurrence...
  45. ncbi request reprint The ciliary gene RPGRIP1L is mutated in cerebello-oculo-renal syndrome (Joubert syndrome type B) and Meckel syndrome
    Marion Delous
    Institut National de la Santé et de la Recherche Médicale INSERM U 574, Hopital Necker Enfants Malades, 75015 Paris, France
    Nat Genet 39:875-81. 2007
    ..Our findings show that mutations in RPGRIP1L can cause the multiorgan phenotypic abnormalities found in CORS or MKS, which therefore represent a continuum of the same underlying disorder...
  46. ncbi request reprint [Cardiovascular impact of end-stage renal insufficiency in children undergoing hemodialysis]
    Y Aggoun
    , , Paris
    Arch Mal Coeur Vaiss 93:1009-13. 2000
    ..005). These patients have an impaired FMD (4 +/- 2 vs 7 +/- 1%; p = 0.02) with a normal GTNMD. This study shows that early arterial dysfunction can occur in children with ESRD...
  47. ncbi request reprint A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure
    P de Lonlay
    INSERM U393, Hopital Necker Enfants Malades, 149 rue de Sevres, 75015 Paris
    Nat Genet 29:57-60. 2001
    ..Complementation study in yeast confirmed the deleterious effect of these mutations. Mutation of BCS1L would seem to be a frequent cause of CIII deficiency, as one-third of our patients have BCS1L mutations...
  48. doi request reprint Pulse cyclophosphamide therapy and clinical remission in atypical hemolytic uremic syndrome with anti-complement factor H autoantibodies
    Olivia Boyer
    Néphrologie Pédiatrique et centre de référence MARHEA, Hopital Necker Enfants Malades, APHP, Universite Paris Descartes, Paris, France
    Am J Kidney Dis 55:923-7. 2010
    ..Cyclophosphamide pulses with PE may lead to a prolonged decrease in CFH antibody titers and a favorable outcome of atypical hemolytic uremic syndrome and kidney function...
  49. ncbi request reprint Glucocorticoid pharmacokinetics and growth retardation in children with renal transplants
    Claire Chavatte
    Service Pharmacie, Hopital Necker Enfants Malades, 149 rue de Sevres, 75015 Paris, France
    Pediatr Nephrol 19:898-904. 2004
    ..We, therefore, do not recommend using glucocorticoid pharmacokinetics to predict growth retardation in children with renal transplantation...
  50. ncbi request reprint Long-term social outcome of children after kidney transplantation
    Michel Broyer
    Department of Pediatric Nephrology, and Hôpital Necker Enfants Malades and University Paris V René Descartes, Paris, France
    Transplantation 77:1033-7. 2004
    ..The aim of this study was to collect information on this outcome in a cohort of 366 children who underwent transplantation between 1973 and 1985...
  51. doi request reprint Complement factor H deficiency and posttransplantation glomerulonephritis with isolated C3 deposits
    Olivia Boyer
    Pediatric Nephrology, Hopital Necker Enfants Malades, Universite Paris Descartes, Paris, France
    Am J Kidney Dis 51:671-7. 2008
    ..Several factors could influence the disease, such as degree of CFH haploinsufficiency and other complement alternative pathway regulator abnormalities, such as a membrane cofactor protein polymorphism...
  52. doi request reprint In vivo confocal microscopy and anterior segment optical coherence tomography analysis of the cornea in nephropathic cystinosis
    Antoine Labbe
    Department of Ophthalmology III, Quinze Vingts National Ophthalmology Hospital, 28 rue de Charenton, Paris, France
    Ophthalmology 116:870-6. 2009
    ..To analyze the corneas of patients with nephropathic cystinosis using in vivo confocal microscopy (IVCM) and anterior segment optical coherence tomography (AS-OCT)...
  53. doi request reprint Multiple OXPHOS deficiency in the liver, kidney, heart, and skeletal muscle of patients with methylmalonic aciduria and propionic aciduria
    Yves de Keyzer
    Metabolic Unit and Paediatric Department, Hopital Necker Enfants Malades, INSERM U781, University Paris Descartes, Paris, France
    Pediatr Res 66:91-5. 2009
    ..Our results bring further support for a role of secondary respiratory deficiency in the development of late multiorgan complications of these diseases...
  54. doi request reprint Effect of hydrochlorothiazide on urinary calcium excretion in dent disease: an uncontrolled trial
    Anne Blanchard
    Universite Paris 5, Faculté de Médecine Paris Descartes, Paris, France
    Am J Kidney Dis 52:1084-95. 2008
    ..However, they can cause adverse metabolic effects in the long term. In treating hypertension in children, lower thiazide doses have been shown to be as effective and well tolerated...
  55. ncbi request reprint Prognosis of autosomal dominant polycystic kidney disease diagnosed in utero or at birth
    Olivia Boyer
    Service de Nephrologie Pediatrique, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743, Paris Cedex 15, France
    Pediatr Nephrol 22:380-8. 2007
    ..The sex of the transmitting parent is not a risk factor of prenatal ADPKD. A high proportion of siblings develop early renal cysts. Abnormalities visualized by ultrasonography appear to be associated to more clinical manifestations...
  56. ncbi request reprint Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases
    Marie Agnès Dragon-Durey
    INSERM U430, Institut des Cordeliers, Paris, France
    J Am Soc Nephrol 15:787-95. 2004
    ..This report emphasizes the variability in the clinical progression of kidney diseases associated with FH deficiencies. Genetic analysis reveals the molecular abnormalities associated with FH deficiencies to be polymorphous...
  57. ncbi request reprint Congenital disorders of glycosylation type I: a rare but new cause of hyperechoic kidneys in infants and children due to early microcystic changes
    Lucie Hertz-Pannier
    Department of Paediatric Radiology, Hopital Necker Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France
    Pediatr Radiol 36:108-14. 2006
    ..Congenital disorders of glycosylation (CDGs) are a rapidly growing family of inherited disorders due to defects in the synthesis of the glycans of glycoproteins or other glycoconjugates...
  58. ncbi request reprint Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome
    Anne Laure Sellier-Leclerc
    Service de Nephrologie, Assistance Publique Hopitaux de Paris, Hopital Robert Debre, 48 Boulevard Serurier, 75 019 Paris, France
    J Am Soc Nephrol 18:2392-400. 2007
    ..New therapies are urgently needed, and further research should elucidate the unexplained HUS group...
  59. ncbi request reprint The risk of recurrence of hemolytic uremic syndrome after renal transplantation in children
    Chantal Loirat
    Service de Nephrologie, Hopital Robert Debre, 48 Boulevard Serurier, 75019 Paris, France
    Pediatr Nephrol 18:1095-101. 2003
    ....
  60. doi request reprint Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases
    Vincent Guigonis
    Department of Pediatrics, Centre de Référence des Maladies Rénales Rares du Sud Ouest, hôpital de la Mère et de l Enfant, 8 Ave Dominique Larrey, 87000 Limoges, France
    Pediatr Nephrol 23:1269-79. 2008
    ..Adverse effects were observed in 45% of cases, but most of them were mild and transient. This study suggests that RTX could be an effective treatment for severe steroid-dependent nephrotic syndrome...
  61. doi request reprint Multicenter trial of everolimus in pediatric renal transplant recipients: results at three year
    Robert Ettenger
    Department of Pediatrics, Mattel Children s Hospital, University of California Los Angeles, Los Angeles, CA 90095 1752, USA
    Pediatr Transplant 12:456-63. 2008
    ..4 mg/dL). This is the first long-term prospective study to demonstrate that a regimen of everolimus, cyclosporine, and corticosteroids provides good efficacy, tolerability, and safety in de novo pediatric renal transplant patients...
  62. ncbi request reprint [Henoch-Schönlein purpura]
    Evangeline Pillebout
    Service de néphrologie et de transplantation, Hopital Saint Louis, Paris
    Rev Prat 58:507-11. 2008
    ..Treatment of severe HSP nephritis is still debated but some studies, which need to be confirmed, have reported the beneficial effect of corticosteroids combined with immunosuppressive drugs...
  63. ncbi request reprint Podocin and nephrotic syndrome: implications for the clinician
    Patrick Niaudet
    J Am Soc Nephrol 15:832-4. 2004
  64. ncbi request reprint A rational dosing algorithm for basiliximab (Simulect) in pediatric renal transplantation based on pharmacokinetic-dynamic evaluations
    John M Kovarik
    Novartis Pharmaceuticals, Basel, Switzerland
    Transplantation 74:966-71. 2002
    ..The pharmacokinetics and immunodynamics of basiliximab were assessed in 39 pediatric de novo kidney allograft recipients to rationally chose a dose regimen for this age group...
  65. pmc Truncation of C-mip (Tc-mip), a new proximal signaling protein, induces c-maf Th2 transcription factor and cytoskeleton reorganization
    Philippe Grimbert
    Unite INSERM 99, Hopital Henri Mondor, AP HP, Universite Paris XII, 51 Avenue du Marechal de Lattre de Tassigny, 94010 Creteil, France
    J Exp Med 198:797-807. 2003
    ..Tc-mip represents therefore the first identified protein, which links proximal signaling to c-maf induction...
  66. ncbi request reprint Everolimus in pediatric de nova renal transplant patients
    Peter F Hoyer
    Department of Pediatric Nephrology, Universitätsklinik Essen, Hufelandstr 55, D 45122 Essen, Germany
    Transplantation 75:2082-5. 2003
    ..The steady-state pharmacokinetics of everolimus were longitudinally assessed in pediatric de novo kidney allograft recipients during a 6-month period...
  67. ncbi request reprint Different inactivating mutations of the mineralocorticoid receptor in fourteen families affected by type I pseudohypoaldosteronism
    Paola Sartorato
    Institut National de la Sante et de la Recherche Medicale, Unité 478, Faculte de Medecine Xavier Bichat, 75018 Paris, France
    J Clin Endocrinol Metab 88:2508-17. 2003
    ..Their absence in some families underscores the importance of an extensive investigation of the hMR gene and the role of precise diagnostic procedures to allow for identification of other genes potentially involved in the disease...
  68. ncbi request reprint Nephron number and primary hypertension
    Uwe Querfeld
    N Engl J Med 348:1717-9; author reply 1717-9. 2003