nephrotic syndrome

Summary

Summary: Clinical association of heavy PROTEINURIA; HYPOALBUMINEMIA; and generalized EDEMA.

Top Publications

  1. doi 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
  2. ncbi Podocyte migration during nephrotic syndrome requires a coordinated interplay between cathepsin L and alpha3 integrin
    Jochen Reiser
    Department of Medicine, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA
    J Biol Chem 279:34827-32. 2004
  3. ncbi Nephrotic syndrome in childhood
    Allison A Eddy
    Department of Pediatrics, University of Washington, Children s Hospital and Regional Medical Center, Seattle, WA 98105, USA
    Lancet 362:629-39. 2003
  4. doi Immunohistochemical evaluation of podocalyxin expression in glomerulopathies associated with nephrotic syndrome
    Evangelia Kavoura
    First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens 11527, Greece
    Hum Pathol 42:227-35. 2011
  5. doi Slit diaphragm dysfunction in proteinuric states: identification of novel therapeutic targets for nephrotic syndrome
    Hiroshi Kawachi
    Department of Cell Biology, Institute of Nephrology, Niigata University Graduate School of Medical and Dental Sciences, 1 757 Asahimachi dori, Niigata 951 8510, Japan
    Clin Exp Nephrol 13:275-80. 2009
  6. ncbi Role of p38 mitogen-activated protein kinase activation in podocyte injury and proteinuria in experimental nephrotic syndrome
    Masao Koshikawa
    Department of Medicine and Clinical Science, Kyoto University Graduate School of Medicine, 54 Shogoin Kawahara cho, Sakyo ku, Kyoto 606 8507, Japan
    J Am Soc Nephrol 16:2690-701. 2005
  7. ncbi A randomized, controlled trial of steroids and cyclophosphamide in adults with nephrotic syndrome caused by idiopathic membranous nephropathy
    Vivekanand Jha
    Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012 India
    J Am Soc Nephrol 18:1899-904. 2007
  8. ncbi Renal macrophage activation and Th2 polarization precedes the development of nephrotic syndrome in Buffalo/Mna rats
    Ludmilla Le Berre
    Institut National de la Santé Et de la Recherche Médicale INSERM Unité 643 Immunointervention dans les Allo et Xenotransplantations, Nantes, France
    Kidney Int 68:2079-90. 2005
  9. ncbi Induction and long-term treatment with cyclosporine in membranous nephropathy with the nephrotic syndrome
    Efstathios Alexopoulos
    Department of Nephrology, Hippokration General Hospital, 49, Konstantinoupoleos str, 54642 Thessaloniki, Greece
    Nephrol Dial Transplant 21:3127-32. 2006
  10. pmc A missense LAMB2 mutation causes congenital nephrotic syndrome by impairing laminin secretion
    YING MAGGIE CHEN
    Renal Division, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Am Soc Nephrol 22:849-58. 2011

Research Grants

  1. Fibrotic Sequelae of Childhood Renal Disease
    Agnes Fogo; Fiscal Year: 2007
  2. Protein Misprocessing in Krabbe Disease
    Christopher Eckman; Fiscal Year: 2007
  3. Slit Diaphragm and Actin Dynamics
    Lawrence Holzman; Fiscal Year: 2009
  4. Slit Diaphragm and Actin Dynamics
    Lawrence B Holzman; Fiscal Year: 2010
  5. Slit Diaphragm and Actin Dynamics
    Lawrence B Holzman; Fiscal Year: 2009
  6. BASIS FOR RENAL RESISTANCE TO ATRIAL NATRIURETIC PEPTIDE
    Michael Humphreys; Fiscal Year: 2004
  7. LAMININ BETA2 AND GLOMERULAR FILTRATION
    JEFFREY MINER; Fiscal Year: 2009
  8. LAMININ BETA2 AND GLOMERULAR FILTRATION
    JEFFREY MINER; Fiscal Year: 2009
  9. LAMININ BETA2 AND GLOMERULAR FILTRATION
    Jeffrey H Miner; Fiscal Year: 2010
  10. Podocyte Protein Alterations Induced by Glucocorticoids
    Richard Ransom; Fiscal Year: 2005

Detail Information

Publications286 found, 100 shown here

  1. doi 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
    Several case reports suggest that rituximab (RTX) could be effective in steroid-dependent nephrotic syndrome, but RTX efficacy has not yet been studied in a series of patients...
  2. ncbi Podocyte migration during nephrotic syndrome requires a coordinated interplay between cathepsin L and alpha3 integrin
    Jochen Reiser
    Department of Medicine, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA
    J Biol Chem 279:34827-32. 2004
    ..In summary, these data establish that podocyte foot process effacement is a migratory event involving a novel interplay between cathepsin L and alpha(3) integrin...
  3. ncbi Nephrotic syndrome in childhood
    Allison A Eddy
    Department of Pediatrics, University of Washington, Children s Hospital and Regional Medical Center, Seattle, WA 98105, USA
    Lancet 362:629-39. 2003
    Childhood nephrotic syndromes are most commonly caused by one of two idiopathic diseases: minimal-change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS)...
  4. doi Immunohistochemical evaluation of podocalyxin expression in glomerulopathies associated with nephrotic syndrome
    Evangelia Kavoura
    First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens 11527, Greece
    Hum Pathol 42:227-35. 2011
    ..podocalyxin, a major podocyte protein, in the pathogenesis of glomerulopathies primarily associated with the nephrotic syndrome. Immunohistochemical expression of podocalyxin has been evaluated in 51 renal samples, including healthy ..
  5. doi Slit diaphragm dysfunction in proteinuric states: identification of novel therapeutic targets for nephrotic syndrome
    Hiroshi Kawachi
    Department of Cell Biology, Institute of Nephrology, Niigata University Graduate School of Medical and Dental Sciences, 1 757 Asahimachi dori, Niigata 951 8510, Japan
    Clin Exp Nephrol 13:275-80. 2009
    ..Nephrin identified as a gene product mutated in congenital nephrotic syndrome located at the outer leaflet of plasma membranes of the slit diaphragm...
  6. ncbi Role of p38 mitogen-activated protein kinase activation in podocyte injury and proteinuria in experimental nephrotic syndrome
    Masao Koshikawa
    Department of Medicine and Clinical Science, Kyoto University Graduate School of Medicine, 54 Shogoin Kawahara cho, Sakyo ku, Kyoto 606 8507, Japan
    J Am Soc Nephrol 16:2690-701. 2005
    ..These findings indicate that the activation of MAPK is necessary for podocyte injury, suggesting that p38 MAPK and, possibly, ERK should become a potential target for therapeutic intervention in proteinuric glomerulopathies...
  7. ncbi A randomized, controlled trial of steroids and cyclophosphamide in adults with nephrotic syndrome caused by idiopathic membranous nephropathy
    Vivekanand Jha
    Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh 160 012 India
    J Am Soc Nephrol 18:1899-904. 2007
    Idiopathic membranous nephropathy (IMN) is the most common cause of nephrotic syndrome in adults...
  8. ncbi Renal macrophage activation and Th2 polarization precedes the development of nephrotic syndrome in Buffalo/Mna rats
    Ludmilla Le Berre
    Institut National de la Santé Et de la Recherche Médicale INSERM Unité 643 Immunointervention dans les Allo et Xenotransplantations, Nantes, France
    Kidney Int 68:2079-90. 2005
    At 8 weeks, Buffalo/Mna rats spontaneously develop a nephrotic syndrome associated with focal segmental glomerulosclerosis (FSGS)...
  9. ncbi Induction and long-term treatment with cyclosporine in membranous nephropathy with the nephrotic syndrome
    Efstathios Alexopoulos
    Department of Nephrology, Hippokration General Hospital, 49, Konstantinoupoleos str, 54642 Thessaloniki, Greece
    Nephrol Dial Transplant 21:3127-32. 2006
    ..However, the optimal dose and the duration of treatment remain controversial issues. We evaluated the efficacy of low-dose CyA alone or combined with corticosteroids as induction and long-term treatment for nephrotic patients with MN...
  10. pmc A missense LAMB2 mutation causes congenital nephrotic syndrome by impairing laminin secretion
    YING MAGGIE CHEN
    Renal Division, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Am Soc Nephrol 22:849-58. 2011
    ..Null mutations in laminin β2 (LAMB2) cause Pierson syndrome, a severe congenital nephrotic syndrome with ocular and neurologic defects...
  11. doi Management of childhood onset nephrotic syndrome
    Debbie S Gipson
    Division of Nephrology and Hypertension, Department ofMedicine and Pediatrics, University of North Carolina, ChapelHill, North Carolina 27599 7155, USA
    Pediatrics 124:747-57. 2009
    The therapeutic approach to childhood nephrotic syndrome is based on a series of studies that began with an international collaborative effort sponsored by the International Study of Kidney Disease in Children in 1967...
  12. doi Rituximab in refractory nephrotic syndrome
    Agnieszka Prytuła
    Department of Paediatric Nephrology, Great Ormond Street Hospital NHS Trust, London, UK
    Pediatr Nephrol 25:461-8. 2010
    The aim of this study was to establish the efficacy and safety of rituximab in refractory nephrotic syndrome (NS)...
  13. ncbi ACE gene insertion/deletion polymorphism in childhood idiopathic nephrotic syndrome
    Erkin Serdaroglu
    Department of Pediatric Nephrology, Ege University Medical School, Izmir, Turkey
    Pediatr Nephrol 20:1738-43. 2005
    ..findings, treatment responses and progression to end-stage renal disease in childhood idiopathic nephrotic syndrome (NS). 227 children diagnosed with idiopathic NS were included in the study...
  14. ncbi 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
    ..Patients with Denys-Drash syndrome present with early nephrotic syndrome with diffuse mesangial sclerosis progressing rapidly to end-stage renal failure, male pseudohermaphroditism, ..
  15. doi Genetics of nephrotic syndrome: connecting molecular genetics to podocyte physiology
    Eduardo Machuca
    INSERM, U574, Hopital Necker Enfants Malades, Paris, France
    Hum Mol Genet 18:R185-94. 2009
    Urinary losses of macromolecules in nephrotic syndrome (NS) reflect a dysfunction of the highly permselective glomerular filtration barrier...
  16. pmc Induction of B7-1 in podocytes is associated with nephrotic syndrome
    Jochen Reiser
    Department of Medicine, Albert Einstein College of Medicine, New York, New York 10461, USA
    J Clin Invest 113:1390-7. 2004
    ..This explains why podocyte injury is typically associated with nephrotic syndrome. The present study uncovered an unanticipated novel role for costimulatory molecule B7-1 in podocytes as an ..
  17. ncbi Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome
    M Kestila
    Department of Biochemistry, University of Oulu, Finland
    Mol Cell 1:575-82. 1998
    Congenital nephrotic syndrome of the Finnish type (NPHS1) is an autosomal-recessive disorder, characterized by massive proteinuria in utero and nephrosis at birth...
  18. ncbi Pathologic classification of focal segmental glomerulosclerosis
    VIVETTE D'AGATI
    Department of Pathology, Columbia University, College of Physicians and Surgeons, New York, USA
    Semin Nephrol 23:117-34. 2003
    ..The defining features of these morphologic variants and of the major subcategories of FSGS are discussed with emphasis on distinguishing light microscopic patterns and clinical-pathologic correlations...
  19. ncbi Health-related quality of life and psychosocial adjustment in steroid-sensitive nephrotic syndrome
    Eva Maria Rüth
    Department of Pediatric Nephrology and Department of Psychiatry and Psychosomatics, University Children s Hospital Zurich, Zurich, Switzerland
    J Pediatr 145:778-83. 2004
    To evaluate quality of life (QOL) and psychosocial adjustment by standardized tests in well-characterized patients with steroid-sensitive nephrotic syndrome.
  20. ncbi Thromboembolic complications in the nephrotic syndrome: pathophysiology and clinical management
    Rajni Singhal
    Department of Medicine, McMaster University, 25 Charlton Avenue East, Suite 708, Hamilton, Ontario, Canada L8N lY2
    Thromb Res 118:397-407. 2006
    Patients with the nephrotic syndrome are at increased risk of developing venous and arterial thromboembolism, the most common of which is renal vein thrombosis...
  21. ncbi CCR1 blockade reduces interstitial inflammation and fibrosis in mice with glomerulosclerosis and nephrotic syndrome
    Volker Vielhauer
    Nephrologisches Zentrum, Medizinische Poliklinik Innenstadt, Ludwig Maximilians University, Munich, Germany
    Kidney Int 66:2264-78. 2004
    ..We therefore studied the effect of the small-molecule CCR1 antagonist BX471 in a murine model of adriamycin-induced focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome and progressive interstitial inflammation and fibrosis.
  22. ncbi Inherited podocytopathies: FSGS and nephrotic syndrome from a genetic viewpoint
    Martin R Pollak
    Renal Division, Brigham and Women s Hospital, 77 Avenue Louis Pasteur, Boston, MA 02115, USA
    J Am Soc Nephrol 13:3016-23. 2002
  23. pmc Podocyte-secreted angiopoietin-like-4 mediates proteinuria in glucocorticoid-sensitive nephrotic syndrome
    Lionel C Clement
    Glomerular Disease Therapeutics Laboratory, and Nephrology Research and Training Center, University of Alabama at Birmingham, Birmingham, Alabama, USA
    Nat Med 17:117-22. 2011
    The main manifestations of nephrotic syndrome include proteinuria, hypoalbuminemia, edema, hyperlipidemia and lipiduria...
  24. doi Successful use of tocilizumab in a patient with nephrotic syndrome due to a rapidly progressing AA amyloidosis secondary to latent tuberculosis
    César Magro-Checa
    Department of Rheumatology, Hospital Universitario San Cecilio, Granada, Spain
    Amyloid 18:235-9. 2011
    ..report herein a case of a latent TB infection complicated by a life-threatening AA amyloidosis presented as nephrotic syndrome. After an adequate antituberculostatic treatment, AA amyloidosis remained active and Tocilizumab (TCZ) was ..
  25. ncbi High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: results from a large retrospective cohort study
    Bakhtawar K Mahmoodi
    Division of Hemostasis, Thrombosis, and Rheology, Department of Hematology, University Medical Center Groningen, Groningen, The Netherlands
    Circulation 117:224-30. 2008
    ..the absolute risk of either venous thromboembolism (VTE) or arterial thromboembolism (ATE) in patients with nephrotic syndrome. Reported risks are based on multiple case reports and small studies with mostly short-term follow-up...
  26. ncbi Treatment of nephrotic syndrome with adrenocorticotropic hormone (ACTH)
    Andrew S Bomback
    Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Discov Med 12:91-6. 2011
    ..last two decades, adrenocorticotropic hormone (ACTH) has re-emerged as a potentially effective therapy for nephrotic syndrome, particularly for patients who have failed more conventional immunosuppressive therapies...
  27. pmc Clinical features and outcomes of IgA nephropathy with nephrotic syndrome
    Jwa Kyung Kim
    Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
    Clin J Am Soc Nephrol 7:427-36. 2012
    b>Nephrotic syndrome (NS) is a rare manifestation of IgA nephropathy (IgAN). Clinical characteristics and long-term outcomes of this condition have not yet been explored.
  28. doi Clinicopathologic characteristics and steroid response of IgM nephropathy in children presenting with idiopathic nephrotic syndrome
    Muhammed Mubarak
    Histopathology and Nephrology Departments, Sindh Institute of Urology and Transplantation, Karachi, Pakistan
    APMIS 119:180-6. 2011
    ..on clinical and immunopathologic features of immunoglobulin M nephropathy (IgMN) in children with idiopathic nephrotic syndrome (INS) in Pakistan...
  29. pmc Spontaneous remission of nephrotic syndrome in idiopathic membranous nephropathy
    Natalia Polanco
    Hospital 12 de Octubre, Madrid, Spain
    J Am Soc Nephrol 21:697-704. 2010
    ..We conducted a retrospective, multicenter cohort study of 328 patients with nephrotic syndrome resulting from idiopathic membranous nephropathy that initially received conservative therapy...
  30. doi The prevalence of Th17 cells and FOXP3 regulate T cells (Treg) in children with primary nephrotic syndrome
    Xiao Shan Shao
    Department of Nephroimmunology, Children s Hospital of Chongqing Medical University, Chongqing, Peoples Republic of China
    Pediatr Nephrol 24:1683-90. 2009
    ..of interleukin (IL)-17-producing CD4+ T cells (Th17) and regulatory T (Treg) cells in children with primary nephrotic syndrome. The study cohort consisted of 62 children who were randomly divided into control, primary nephrotic ..
  31. doi Urinary proteins from patients with nephrotic syndrome alters the signalling proteins regulating epithelial-mesenchymal transition
    Qiong Wen
    Department of Nephrology, The First Affiliated Hospital, Sun Yat Sen University, Guangzhou, Guangdong, China
    Nephrology (Carlton) 15:63-74. 2010
    ..Further studies to explore the molecular mechanisms behind the distinct clinical features of different types of nephrotic syndrome are warranted.
  32. pmc Epidemiology and risk factors for thromboembolic complications of childhood nephrotic syndrome: a Midwest Pediatric Nephrology Consortium (MWPNC) study
    Bryce A Kerlin
    Department of Pediatrics, College of Medicine, Ohio State University, Columbus, OH, USA
    J Pediatr 155:105-10, 110.e1. 2009
    To identify clinical variables predictive of the risk of thromboembolism (TE), and to confirm the incidence of TE in primary and secondary childhood nephrotic syndrome (NS).
  33. pmc Loss of podocyte aPKClambda/iota causes polarity defects and nephrotic syndrome
    Tobias B Huber
    Renal Division, University Hospital Freiburg, Breisacher Strasse 66, D 79106 Freiburg, Germany
    J Am Soc Nephrol 20:798-806. 2009
    ..Here, we report that podocyte-specific deletion of aPKClambda/iota in mice results in severe proteinuria, nephrotic syndrome, and death at 4 to 5 wk after birth...
  34. ncbi Prognosis and risk factors for idiopathic membranous nephropathy with nephrotic syndrome in Japan
    Hideo Shiiki
    First Department of Internal Medicine, Nara Medical University, Nara, Japan
    Kidney Int 65:1400-7. 2004
    Idiopathic membranous nephropathy (IMN) is a representative form of refractory nephrotic syndrome in Japan...
  35. ncbi Prophylactic anticoagulation in nephrotic syndrome: a clinical conundrum
    Richard J Glassock
    University of California Los Angeles, USA
    J Am Soc Nephrol 18:2221-5. 2007
    It has long been recognized that nephrotic syndrome is associated with an increased risk for thromboembolic complications, including deep venous thrombosis, renal vein thrombosis, and pulmonary embolism...
  36. doi Comparison of three methods for isolation of urinary microvesicles to identify biomarkers of nephrotic syndrome
    Ilse M Rood
    Department of Nephrology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
    Kidney Int 78:810-6. 2010
    ..Thus, UC-SEC following ultracentrifugation to further enrich and purify microparticles facilitates the search for prognostic biomarkers that might be used to predict the clinical course of nephrotic syndrome.
  37. pmc Clinical utility of genetic testing in children and adults with steroid-resistant nephrotic syndrome
    Sheila Santin
    Molecular Biology Laboratory, Fundacio Puigvert, Universitat Autonoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Barcelona, Spain
    Clin J Am Soc Nephrol 6:1139-48. 2011
    The increasing number of podocyte-expressed genes implicated in steroid-resistant nephrotic syndrome (SRNS), the phenotypic variability, and the uncharacterized relative frequency of mutations in these genes in pediatric and adult ..
  38. doi Common variation in GPC5 is associated with acquired nephrotic syndrome
    Koji Okamoto
    Department of Nephrology and Endocrinology, University Hospital, The University of Tokyo, Tokyo, Japan
    Nat Genet 43:459-63. 2011
    Severe proteinuria is a defining factor of nephrotic syndrome irrespective of the etiology...
  39. doi Urinary exosomal WT1 in childhood nephrotic syndrome
    Hyunkyung Lee
    Department of Pediatrics, Seoul National University Children s Hospital, 101 Daehak Ro, Jongno gu, Seoul, 110 744, Korea
    Pediatr Nephrol 27:317-20. 2012
    ..biomarker for predicting steroid responsiveness or renal pathological conditions in patients with idiopathic nephrotic syndrome (NS)...
  40. pmc Clinical value of NPHS2 analysis in early- and adult-onset steroid-resistant nephrotic syndrome
    Sheila Santin
    Fundacio Puigvert, Cartagena 340 350, 08025 Barcelona, Spain
    Clin J Am Soc Nephrol 6:344-54. 2011
    ..To date, very few cases with adult-onset focal segmental glomerulosclerosis (FSGS) carrying NPHS2 variants have been described, all of them being compound heterozygous for the p.R229Q variant and one pathogenic mutation...
  41. pmc Immunosuppression and renal outcome in congenital and pediatric steroid-resistant nephrotic syndrome
    Anja K Büscher
    Pediatric Nephrology, Pediatrics II, University of Duisburg Essen, Essen, Germany
    Clin J Am Soc Nephrol 5:2075-84. 2010
    Mutations in podocyte genes are associated with steroid-resistant nephrotic syndrome (SRNS), mostly affecting younger age groups...
  42. ncbi IgM nephropathy: clinical picture and pathological findings in 36 patients
    Ghadeer A Mokhtar
    Pathology Department, King Abdul Aziz University, Jeddah, Saudi Arabia
    Saudi J Kidney Dis Transpl 22:969-75. 2011
    ..The initial manifestations of the disease were the nephrotic syndrome in 32 patients, proteinuria in two, hematuria associated with proteinuria in 16 and isolated recurrent gross ..
  43. ncbi Guillain-Barré syndrome coexisting with pericarditis or nephrotic syndrome after influenza vaccination
    Chuen Der Kao
    Department of Neurology, National Yang Ming University School of Medicine, Taipei, Taiwan, ROC
    Clin Neurol Neurosurg 106:136-8. 2004
    ..was diagnosed in the first patient who also had precordial chest pain with referral to trapezius ridge, and nephrotic syndrome, was observed in the second patient who had leg edema and proteinuria...
  44. doi Steroid-sensitive nephrotic syndrome in two families
    Osamu Motoyama
    Department of Pediatrics, Toho University Medical Center, Sakura Hospital, 564 1 Shimoshizu, Sakura shi, Chiba, 285 8741, Japan
    Clin Exp Nephrol 13:170-3. 2009
    Few reports of familial cases of steroid-sensitive nephrotic syndrome (SSNS) are available. Of 123 children with SSNS, four cases in two families are presented. Two sisters and a father and his daughter developed SSNS...
  45. doi Sunitinib-induced nephrotic syndrome and irreversible renal dysfunction
    Daiei Takahashi
    Department of Nephrology, Tokyo Medical and Dental University, 1 5 45 Yushima, Bunkyo ku, Tokyo, 113 8510, Japan
    Clin Exp Nephrol 16:310-5. 2012
    ..We present here the case of a 72-year-old man who presented with nephrotic syndrome and renal dysfunction 6 months after administration of sunitinib...
  46. ncbi The effects of corticosteroids on behavior in children with nephrotic syndrome
    A S Hall
    Department of Pediatrics, Ward 27A, Leicester Royal Infirmary, LE1 5WW, Leicester, UK
    Pediatr Nephrol 18:1220-3. 2003
    ..the frequency and severity of the behavioral effects of high-dose oral steroid therapy in children with nephrotic syndrome. We conducted a prospective assessment of the behavior of 12 children using a standardized psychological ..
  47. ncbi Expression of intermediate filaments of podocytes within nephrotic syndrome glomerulopathies in children
    Danuta Ostalska-Nowicka
    Department of Pediatric Cardiology, Poznan University of Medical Sciences, ul Szpitalna 27 33, 60 572 Poznan, Poland
    Histochem Cell Biol 121:109-13. 2004
    The study attempted to define characteristics of renal podocytes in nephrotic syndrome glomerulopathies in children with and without glomerular immaturity based on the histochemical expression of cytokeratin 18 (CK 18) and vimentin...
  48. ncbi Th1 and Th2 cytokine mRNA profiles in childhood nephrotic syndrome: evidence for increased IL-13 mRNA expression in relapse
    H K Yap
    Department of Pediatrics, National University of Singapore, Singapore
    J Am Soc Nephrol 10:529-37. 1999
    Idiopathic nephrotic syndrome of childhood is thought to be associated with T lymphocyte dysfunction often triggered by viral infections, with the production of circulating factor(s) resulting in proteinuria...
  49. ncbi Dynamic (re)organization of the podocyte actin cytoskeleton in the nephrotic syndrome
    Jun Oh
    Division of Nephrology, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA
    Pediatr Nephrol 19:130-7. 2004
    ..This review provides an update of podocyte functions with respect to novel podocyte-specific proteins and also focuses on the dynamic interaction between the actin cytoskeleton of podocytes, their cell surface receptors and the GBM...
  50. ncbi Rituximab failed to improve nephrotic syndrome in renal transplant patients with recurrent focal segmental glomerulosclerosis
    J M Yabu
    Department of Medicine, University of California, San Francisco, CA, USA
    Am J Transplant 8:222-7. 2008
    ..None of the patients treated with rituximab achieved remission in proteinuria, and one patient experienced early graft loss. In these four adult renal transplant patients with recurrent FSGS, rituximab failed to diminish proteinuria...
  51. ncbi ACE gene polymorphism in Egyptian children with idiopathic nephrotic syndrome
    M E Fahmy
    Pediatric Department, Research Institute of Ophthalmology, Cairo, Egypt
    Bratisl Lek Listy 109:298-301. 2008
    Idiopathic nephrotic syndrome is a common renal disease in children. ACE gene insertion/deletion (I/D) polymorphism has been studied as a predictor of clinical response to steroid therapy.
  52. ncbi Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK
    P A McKinney
    Paediatric Epidemiology Group, Unit of Epidemiology and Health Services Research, University of Leeds, 30 Hyde Terrace, Leeds LS2 9LN, UK
    Pediatr Nephrol 16:1040-4. 2001
    ..childhood conditions, this study aimed to identify recent time trends and ethnic patterns of childhood nephrotic syndrome. A population-based cohort of children (0-15 years) diagnosed according to strict criteria with nephrotic ..
  53. ncbi Hepatic cholesterol metabolism in experimental nephrotic syndrome
    A Al-Shurbaji
    Department of Medical Laboratory Sciences, Karolinska Institutet at Huddinge University Hospital, Sweden
    Lipids 33:165-9. 1998
    Hypercholesterolemia is a consistent feature of the nephrotic syndrome. However, the mechanisms underlying this perturbation are unclear...
  54. ncbi ACE I/D gene polymorphism in primary FSGS and steroid-sensitive nephrotic syndrome
    Faruk Oktem
    Department of Pediatric Nephrology, School of Medicine, Suleyman Demirel University, Isparta, Turkey
    Pediatr Nephrol 19:384-9. 2004
    ..genotypes of Turkish children with primary focal segmental glomerulosclerosis (FSGS) and steroid-sensitive nephrotic syndrome (SSNS)...
  55. ncbi Change of the course of steroid-dependent nephrotic syndrome after rituximab therapy
    Kerstin Benz
    Department of Pediatrics, University of Erlangen Nurnberg, Loschgestrasse 15, 91054 Erlangen, Germany
    Pediatr Nephrol 19:794-7. 2004
    A 16-year-old patient with steroid-dependent nephrotic syndrome with more than 35 relapses developed severe relapsing idiopathic thrombocytopenic purpura (ITP)...
  56. doi Clinicopathological features and prognosis of Chinese children with idiopathic nephrotic syndrome between different age groups
    Jei Wen Chang
    Department of Pediatrics, Taipei Veterans General Hospital, Taipei 11217, Taiwan
    Eur J Pediatr 168:1189-94. 2009
    Ethnicity and age play important roles in the epidemiology of idiopathic nephrotic syndrome (INS) in children...
  57. doi Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis
    Ellen T McCarthy
    Kidney Institute, University of Kansas Medical Center, Kansas City, KS 66160, USA
    Clin J Am Soc Nephrol 5:2115-21. 2010
    Circulating permeability factors may be important in idiopathic nephrotic syndrome (INS) including focal segmental glomerulosclerosis (FSGS) and in recurrence after renal transplantation...
  58. ncbi Angiotensin converting enzyme gene insertion/deletion polymorphism in idiopathic nephrotic syndrome in Kuwaiti Arab children
    A Al-Eisa
    Paediatrics Department, Faculty of Medicine, Kuwait University, Kuwait
    Scand J Urol Nephrol 35:239-42. 2001
    ..We have investigated the association of ACE gene I/D polymorphism with the clinical presentation of idiopathic nephrotic syndrome (INS) in Kuwaiti children.
  59. ncbi Urinary proteome of steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome of childhood
    Robert P Woroniecki
    Section of Pediatric Nephrology, Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY 10467, USA
    Am J Nephrol 26:258-67. 2006
    The response to steroid therapy is used to characterize the idiopathic nephrotic syndrome (INS) of childhood as either steroid-sensitive (SSNS) or steroid-resistant (SRNS), a classification with a better prognostic capability than renal ..
  60. doi Effect of angiotensin-converting enzyme gene insertion/deletion polymorphism on steroid resistance in Egyptian children with idiopathic nephrotic syndrome
    Maha Saber-Ayad
    Department of Pharmacology, Faculty of Medicine, Cairo University, Giza, Egypt
    J Renin Angiotensin Aldosterone Syst 11:111-8. 2010
    ..D) allele in the angiotensin-converting enzyme (ACE) gene has been reported as a probable genetic risk factor for idiopathic nephrotic syndrome (INS), but its role in determining resistance to steroid therapy remains to be evaluated.
  61. pmc Hereditary nephrotic syndrome: a systematic approach for genetic testing and a review of associated podocyte gene mutations
    Genevieve Benoit
    INSERM, U983, Hopital Necker Enfants Malades, Paris, France
    Pediatr Nephrol 25:1621-32. 2010
    Several genes have been implicated in genetic forms of nephrotic syndrome occurring in children...
  62. ncbi Ocular findings in Japanese children with nephrotic syndrome receiving prolonged corticosteroid therapy
    Yoriko Hayasaka
    Department of Ophthalmology, Toyama Medical and Pharmaceutical University, Toyama, Japan
    Ophthalmologica 220:181-5. 2006
    To examine ocular findings in Japanese children with nephrotic syndrome who receive prolonged corticosteroid treatment.
  63. ncbi Changing trends of histopathology in childhood nephrotic syndrome
    S Gulati
    Department of Nephrology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
    Am J Kidney Dis 34:646-50. 1999
    This study was conducted to analyze the trend of histopathologic subtypes in idiopathic nephrotic syndrome (INS) in a homogenous racial group in India population. A prospective analysis of 400 consecutive children with INS was performed...
  64. pmc Loss of glomerular foot processes is associated with uncoupling of podocalyxin from the actin cytoskeleton
    T Takeda
    Department of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, California, USA
    J Clin Invest 108:289-301. 2001
    ..or sialidase-treated rats, which show a dramatic loss of foot processes, comparable to that seen in the nephrotic syndrome. Thus NHERF2 appears to function as a scaffold protein linking PC to ezrin and the actin cytoskeleton...
  65. doi Nephrin mutations cause childhood- and adult-onset focal segmental glomerulosclerosis
    Sheila Santin
    Molecular Biology Laboratory, Fundacio Puigvert, Universitat Autonoma de Barcelona, REDinREN, Instituto de Investigación Carlos III, Barcelona, Spain
    Kidney Int 76:1268-76. 2009
    Mutations in the NPHS1 gene cause congenital nephrotic syndrome of the Finnish type presenting before the first 3 months of life...
  66. doi A nonsense mutation in the LIMP-2 gene associated with progressive myoclonic epilepsy and nephrotic syndrome
    Andrea Balreira
    Unidade de Biologia do Lisossoma e do Peroxissoma, Instituto de Biologia Molecular e Celular, Universidade do Porto, Porto, Portugal
    Hum Mol Genet 17:2238-43. 2008
    ..from a consanguineous marriage were progressive myoclonic epilepsy without intellectual impairment and a nephrotic syndrome with a strong accumulation of C1q in capillary loops and mesangium of kidney...
  67. ncbi Rituximab treatment for posttransplant lymphoproliferative disorder (PTLD) induces complete remission of recurrent nephrotic syndrome
    Kandai Nozu
    Department of Pediatrics, Kobe University Graduate School of Medicine, 650 0017 Kobe, Japan
    Pediatr Nephrol 20:1660-3. 2005
    ..Because his original disease was nephrotic syndrome (focal segmental glomerulosclerosis, or FSGS) and electron microscopic examination of the renal biopsy ..
  68. ncbi Identification of the first gene locus (SSNS1) for steroid-sensitive nephrotic syndrome on chromosome 2p
    Rainer G Ruf
    Department of Pediatrics, University of Michigan Health System, 1150 W Medical Center Drive, Ann Arbor, MI 48109 0646, USA
    J Am Soc Nephrol 14:1897-900. 2003
    Disease mechanisms of steroid-sensitive nephrotic syndrome (SSNS) remain unknown...
  69. doi Histopathological spectrum of childhood nephrotic syndrome in Pakistan
    Muhammed Mubarak
    Department of Histopathology, Sindh Institute of Urology and Transplantation, Civil Hospital, Karachi, Pakistan
    Clin Exp Nephrol 13:589-93. 2009
    ..is no information in international literature on the pattern of glomerulopathies in children with idiopathic nephrotic syndrome (INS) in Pakistan...
  70. doi TRPC6 mutations in children with steroid-resistant nephrotic syndrome and atypical phenotype
    Maddalena Gigante
    Department of Biomedical Sciences, University of Foggia, Viale Pinto, 1, 71100 Foggia, Italy
    Clin J Am Soc Nephrol 6:1626-34. 2011
    ..To extend the screening, we analyzed TRPC6 in 33 Italian children with sporadic early-onset SRNS and three Italian families with adult-onset FSGS...
  71. doi Long-term follow-up after cyclophosphamide and cyclosporine-A therapy in steroid-dependent and -resistant nephrotic syndrome
    Viktória Sümegi
    Department of Paediatrics, University of Szeged, Faculty of Medicine, Koranyi fasor 14 15, Szeged, Hungary
    Pediatr Nephrol 23:1085-92. 2008
    A retrospective study was made on 37 children with idiopathic nephrotic syndrome (INS). At the beginning, all patients were steroid sensitive but received more than one steroid course (median 4)...
  72. pmc Idiopathic nephrotic syndrome and atopy: is there a common link?
    Maher Abdel-Hafez
    Division of Pediatric Nephrology, University of Florida, Gainesville, FL 32610, USA
    Am J Kidney Dis 54:945-53. 2009
    Numerous reports during the last 60 years have reported a strong association between idiopathic nephrotic syndrome and atopic disorders...
  73. ncbi Steroid resistant nephrotic syndrome: role of histopathology
    Sanjeev Gulati
    Department of Nephrology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
    Indian Pediatr 43:55-60. 2006
    ..study was conducted to (1) see the histopathological distribution of different subtypes in steroid resistant nephrotic syndrome (SRNS) and (2) compare the clinical, biochemical parameters and outcome between Minimal Change Disease (MCD) ..
  74. ncbi Atopy, serum IgE, and interleukin-13 in steroid-responsive nephrotic syndrome
    Wai Cheung
    Department of Pediatrics, National University of Singapore, 5 Lower Kent Ridge Crescent, 119074 Singapore
    Pediatr Nephrol 19:627-32. 2004
    Earlier studies have demonstrated a strong association of steroid-responsive nephrotic syndrome (SRNS), atopy, and elevated serum IgE levels. Interleukin (IL-13) gene expression is significantly increased in children with SRNS in relapse...
  75. ncbi Community-acquired pneumonia due to Bordetella holmesii in a patient with frequently relapsing nephrotic syndrome
    Christina Dörbecker
    Institute for Medical Microbiology, Immunology and Hygiene, University of Cologne, Goldenfelsstr 19 21, 50935 Cologne, Germany
    J Infect 54:e203-5. 2007
    ..Herein, we describe a patient with severe relapse of nephrotic syndrome associated with bacteremic B. holmesii pneumonia.
  76. doi Pharmacotherapeutic review and update of idiopathic nephrotic syndrome in children
    Silvia Manrique-Rodríguez
    Pharmacy Department, Gregorio Maranon General University Hospital, Servicio de Farmacia, Madrid, Spain
    Pharm World Sci 32:314-21. 2010
    The therapeutic management of pediatric idiopathic nephrotic syndrome is still a challenge due to the large number of potentially effective pharmacological alternatives and the insufficient scientific evidence available...
  77. ncbi Clinical and genetic evaluation of familial steroid-responsive nephrotic syndrome in childhood
    A Fuchshuber
    University Children s Hospital, Mathildenstrasse 1, 79106 Freiburg, Germany
    J Am Soc Nephrol 12:374-8. 2001
    Steroid-responsive idiopathic nephrotic syndrome (SSINS) is the most common form of nephrotic syndrome in childhood. This article reports a cohort of familial SSINS with disease onset in childhood...
  78. doi Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome
    Yuko Hamasaki
    Department of Pediatric Nephrology, Tokyo Metropolitan Kiyose Children s Hospital, Kiyose City, Japan
    Pediatr Nephrol 24:2177-85. 2009
    ..to evaluate the efficacy and safety of a 12-month course of cyclosporine in children with steroid-resistant nephrotic syndrome (SRNS)...
  79. ncbi Congenital nephrotic syndrome in mice lacking CD2-associated protein
    N Y Shih
    Center for Immunology and Department of Pathology, Washington University, Saint Louis, MO 63110, USA
    Science 286:312-5. 1999
    ..Supporting a role for CD2AP in the specialized cell junction known as the slit diaphragm, CD2AP associated with nephrin, the primary component of the slit diaphragm...
  80. pmc Risk factors for cyclosporin A nephrotoxicity in children with steroid-dependant nephrotic syndrome
    Severin Kengne-Wafo
    Division of Nephrology, Bambino Gesu Children s Hospital and Research Institute, 00165 Rome, Italy
    Clin J Am Soc Nephrol 4:1409-16. 2009
    Cyclosporin A (CsA) is a well-established treatment for steroid-dependent nephrotic syndrome (SDNS) that may, however, cause chronic ischemic renal lesions...
  81. pmc COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness
    Saskia F Heeringa
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA
    J Clin Invest 121:2013-24. 2011
    Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of end-stage renal failure...
  82. doi Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study
    Djalila Mekahli
    Service de Pediatrie, Centre de Référence des Maladies Rénales Rares, Hôpital Femme Mère Enfant and Université de Lyon, Lyon, France
    Pediatr Nephrol 24:1525-32. 2009
    Long-term outcome of idiopathic steroid-resistant nephrotic syndrome was retrospectively studied in 78 children in eight centers for the past 20 years. Median age at onset was 4.4 years (1.1-15.0 years) and the gender ratio was 1.4...
  83. ncbi WT1 gene mutations in three girls with nephrotic syndrome
    Khalid Ismaili
    Department of Pediatric Nephrology, Hopital Universitaire des Enfants Reine Fabiola, Universite Libre de Bruxelles, Brussels, Belgium
    Eur J Pediatr 167:579-81. 2008
    ..This study examines three girls with steroid-resistant nephrotic syndrome related to mutations in the WT1 gene, but with normal 46, XX karyotype and normal female phenotype...
  84. ncbi Management of edema in nephrotic syndrome
    Anil Vasudevan
    Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029, India
    Indian Pediatr 41:787-95. 2004
    b>Nephrotic syndrome is a recurrent or chronic disorder characterized by heavy proteinuria, hypoalbuminemia and edema...
  85. ncbi Analysis of serum and urinary lysophospholipase D/autotaxin in nephrotic syndrome
    Kazuhiro Nakamura
    Clin Chem Lab Med 46:150-1. 2008
  86. ncbi Focal and segmental glomerulosclerosis in children: a longitudinal assessment
    Olivia Boyer
    Division of Nephrology, Children s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Pediatr Nephrol 22:1159-66. 2007
    ..6 vs 5.7 vs 8.5 years; P=0.01), also observed in the non-FSGS children (2.2 vs 3.9 vs 4.9 years; P=0.02). In contradistinction to non-FSGS children, there was a marked increase in steroid resistance with FSGS (43% vs 62% vs 86%; P=0.03)...
  87. ncbi A case of necrotizing glomerulonephritis presenting with nephrotic syndrome associated with pulmonary cryptococcosis
    Masaru Nakayama
    Division of Nephrology and Clinical Research Institute, Department of Internal Medicine, National Kyushu Medical Center Hospital, 1 8 1 Jigyohama, Fukuoka 810 8563, Japan
    Clin Exp Nephrol 9:74-8. 2005
    We describe a 68-year-old man with necrotizing glomerulonephritis who presented with nephrotic syndrome accompanied by pulmonary cryptococcosis. He developed rheumatoid arthritis in July 1999 and was treated with low-dose prednisolone...
  88. ncbi Successful treatment of nephrotic syndrome due to FMF amyloidosis with azathioprine: report of three Turkish cases
    Hayriye Sayarlioglu
    Department of Internal Medicine, Division of Nephrology, Sutcu Imam University, Medical Faculty, Iç Hastaliklari A D, Nefroloji B D, Kahramanmaras, 46050, Turkey
    Rheumatol Int 27:197-9. 2006
    ..3, 3, and 3.5 g/dl; and urinary protein excretion 3, 8, and 1.5 g/day, respectively. Treatment with azathioprine in addition to colchicine could ameliorate the nephrotic syndrome and control the attacks very effectively in these cases.
  89. ncbi The Nephrotic Syndrome is an immunoinflammatory disorder
    Marcello Camici
    Internal Medicine Department, Pisa University, Via Roma n 67, 56126 Pisa, Italy
    Med Hypotheses 68:900-5. 2007
    The Nephrotic Syndrome is still a therapeutic and physiopathological challenge. The clinical response to systemic immunosuppression shows that an immunoinflammatory disorder supports the nephrotic syndrome...
  90. pmc Independent risk factors for chronic cyclosporine induced nephropathy in children with nephrotic syndrome
    S Fujinaga
    Division of Nephrology, Saitama Children s Medical Centre, 2100 Magome, Iwatsuki ku, Saitama shi 339 8551, Japan
    Arch Dis Child 91:666-70. 2006
    Cyclosporine A (CsA) has been widely used in children with steroid dependent and steroid resistant nephrotic syndrome (NS) because of its efficacy in relieving these patients from systemic side effects of steroids...
  91. ncbi Histopathological diagnosis and outcome of paediatric nephrotic syndrome
    Iftikhar Ejaz
    Department of Paediatrics, King Edward Medical College, Lahore
    J Coll Physicians Surg Pak 14:229-33. 2004
    To determine the histological picture and outcome of treatment in cases of childhood nephrotic syndrome who needed renal biopsy.
  92. ncbi Pulse methylprednisolone treatment of idiopathic steroid-resistant nephrotic syndrome
    P D Yorgin
    Department of Pediatrics, Lucile Salter Packard Children s Hospital, Stanford University, CA 94305, USA
    Pediatr Nephrol 16:245-50. 2001
    ..would effectively induce a more rapid remission in young children with idiopathic steroid-resistant nephrotic syndrome (SRNS). A retrospective analysis was conducted of 11 consecutive SRNS patients (mean age 3.6 +/- 1...
  93. ncbi Management and outcome of steroid-resistant nephrotic syndrome in children
    Hassan Otukesh
    Department of Nephrology, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran
    Iran J Kidney Dis 3:210-7. 2009
    Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes.
  94. ncbi Renal artery stenosis and nephrotic syndrome: a rare combination in an infant
    Mia E Lang
    Division of Pediatric Nephrology, Department of Pediatrics, University of Alberta Hospitals, 2C3 WMHSC, Edmonton, AB T6G 2R7, Canada
    Pediatr Nephrol 18:276-9. 2003
    We describe an uncommon pediatric finding of unilateral renal artery stenosis, which presented as nephrotic syndrome, hypertension, failure to thrive, and hyponatremia...
  95. ncbi Nephrotic syndrome in a patient with intravascular lymphomatosis
    Haruko Kakumitsu
    Department of Internal Medicine, Hamanomachi General Hospital, Fukuoka
    Intern Med 42:98-101. 2003
    The association of malignancy with nephrotic syndrome and renal histopathologic abnormalities is well documented. We report a case of intravascular lymphomatosis (IVL) presenting as nephrotic syndrome which was diagnosed by renal biopsy...
  96. ncbi Effect of glucocorticoid treatment on insulin like growth factor-I and its binding proteins in children with nephrotic syndrome
    Feng Dong
    Department of Pediatrics, China Japan Friendship Hospital, Beijing 100029, China
    Chin Med J (Engl) 115:1383-5. 2002
    To identify the changes in serum insulin like growth factor-I (IGF-I) and IGF binding proteins (IGFBPs) in children with nephrotic syndrome (NS) and the effect of glucocorticoid on serum IGF-I and IGFBPs.
  97. ncbi Anti-oxidant vitamins and steroid responsive nephrotic syndrome in Indian children
    J L Mathew
    Departments of Paediatrics, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India
    J Paediatr Child Health 38:450-37. 2002
    In recent years, it has been proposed that nephrotic syndrome is a consequence of an imbalance between oxidant and anti-oxidant activity...
  98. ncbi Treatment of steroid resistant nephrotic syndrome in children
    Jameela A Kari
    Department of Pediatrics, King Abdul Aziz University Hospital, Jeddah, Saudi Arabia
    Saudi J Kidney Dis Transpl 21:484-7. 2010
    Achieving remission in children with steroid-resistant nephrotic syndrome (SRNS) could be difficult. Many immunosuppressive drugs are used with variable success rates...
  99. doi Predisposition to relapsing nephrotic syndrome by a nephrin mutation that interferes with assembly of functioning microdomains
    Akemi Shono
    Department of Clinical Biology and Medicine, The University of Tokushima Graduate School of Medicine, Tokushima 770 0042, Japan
    Hum Mol Genet 18:2943-56. 2009
    Minimal-change disease (MCD) is the most common cause of nephrotic syndrome (NS) and is characterized only by minor morphological alterations in podocytes...
  100. pmc Failure of regulation results in an amplified oxidation burst by neutrophils in children with primary nephrotic syndrome
    R Bertelli
    Division of Nephrology and Laboratory on Pathophysiology of Uremia, Istituto Giannina Gaslini IRCCS, Genova, Italy
    Clin Exp Immunol 161:151-8. 2010
    The mechanism responsible for proteinuria in non-genetic idiopathic nephrotic syndrome (iNS) is unknown. Animal models suggest an effect of free radicals on podocytes, and indirect evidence in humans confirm this implication...
  101. 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
    Mutations in NPHS1, which encodes nephrin, are the main causes of congenital nephrotic syndrome (CNS) in Finnish patients, whereas mutations in NPHS2, which encodes podocin, are typically responsible for childhood-onset steroid-resistant ..

Research Grants77

  1. Fibrotic Sequelae of Childhood Renal Disease
    Agnes Fogo; Fiscal Year: 2007
    ..Kidney Int 58:1219-1227, 2000. Fogo A: Nephrotic syndrome: molecular and genetic basis. Nephron 85:8-13, 2000...
  2. Protein Misprocessing in Krabbe Disease
    Christopher Eckman; Fiscal Year: 2007
    ..misfolded protein disorders such as nephrogenic diabetes insipidus, primary hyperoxaluria type 1, congenital nephrotic syndrome, and other lysosomal storage diseases such as Gaucher disease and Fabry disease...
  3. Slit Diaphragm and Actin Dynamics
    Lawrence Holzman; Fiscal Year: 2009
    ..Project Narrative: Relevance. Diseases resulting in the nephrotic syndrome such as diabetes mellitus and other diseases of the glomerulus frequently cause progressive kidney damage ..
  4. Slit Diaphragm and Actin Dynamics
    Lawrence B Holzman; Fiscal Year: 2010
    ..Project Narrative: Relevance. Diseases resulting in the nephrotic syndrome such as diabetes mellitus and other diseases of the glomerulus frequently cause progressive kidney damage ..
  5. Slit Diaphragm and Actin Dynamics
    Lawrence B Holzman; Fiscal Year: 2009
    ..Project Narrative: Relevance. Diseases resulting in the nephrotic syndrome such as diabetes mellitus and other diseases of the glomerulus frequently cause progressive kidney damage ..
  6. BASIS FOR RENAL RESISTANCE TO ATRIAL NATRIURETIC PEPTIDE
    Michael Humphreys; Fiscal Year: 2004
    DESCRIPTION: States of pathological sodium retention and edema formation such as congestive heart failure, nephrotic syndrome, and cirrhosis of the liver are characterized by renal resistance to the natriuretic action of atrial ..
  7. LAMININ BETA2 AND GLOMERULAR FILTRATION
    JEFFREY MINER; Fiscal Year: 2009
    ..Null mutations cause Pierson syndrome (congenital nephrotic syndrome with ocular and nervous system abnormalities), whereas some missense mutations cause isolated congenital ..
  8. LAMININ BETA2 AND GLOMERULAR FILTRATION
    JEFFREY MINER; Fiscal Year: 2009
    ..Null mutations cause Pierson syndrome (congenital nephrotic syndrome with ocular and nervous system abnormalities), whereas some missense mutations cause isolated congenital ..
  9. LAMININ BETA2 AND GLOMERULAR FILTRATION
    Jeffrey H Miner; Fiscal Year: 2010
    ..Null mutations cause Pierson syndrome (congenital nephrotic syndrome with ocular and nervous system abnormalities), whereas some missense mutations cause isolated congenital ..
  10. Podocyte Protein Alterations Induced by Glucocorticoids
    Richard Ransom; Fiscal Year: 2005
    b>Nephrotic syndrome (NS) is one of the most common kidney diseases seen in children...
  11. Membranous Nephritis Antigen Defined by Phage Antibodies
    Sudesh Makker; Fiscal Year: 2007
    ..the kidney and subsequently abate to low or undetectable levels when patients first present with full-blown nephrotic syndrome is supported by similar observation in Heymann nephritis, a rat model of MGN...
  12. Genetics of Renal End Organ Damage in Hypertension
    Michael R Garrett; Fiscal Year: 2010
    ..Significant progress has been made to identify the genetic causes of familial forms of renal disease (nephrotic syndrome). However, no genetic variants have been found to be causally linked to common forms of renal disease...
  13. Genetics of Renal End Organ Damage in Hypertension
    Michael Garrett; Fiscal Year: 2009
    ..Significant progress has been made to identify the genetic causes of familial forms of renal disease (nephrotic syndrome). However, no genetic variants have been found to be causally linked to common forms of renal disease...
  14. Treatment of Diabetic Nephropathy
    Julia Lewis; Fiscal Year: 2004
    ..COX2 inhibitors in the kidney have been demonstrated to decrease proteinuria in subjects with refractory nephrotic syndrome. In this study, we propose to extend the animal and human data suggesting a potential beneficial effect of ..
  15. Proteolytic Regulation of Podocyte Function by Cathepsin L
    Jochen Reiser; Fiscal Year: 2007
    ..We have made the novel finding that cathepsin L enzyme is present within the cytoplasm of podocytes during nephrotic syndrome and cleaves CD2AP, an important SD protein...
  16. Proteolytic Regulation of Podocyte Function by Cathepsin L
    Jochen Reiser; Fiscal Year: 2010
    ..We have made the novel finding that cathepsin L enzyme is present within the cytoplasm of podocytes during nephrotic syndrome and cleaves CD2AP, an important SD protein...
  17. GLUCOCORTICOID-INDUCED OSTEOPENIA IN CHILDREN
    Mary Leonard; Fiscal Year: 2005
    ..Childhood nephrotic syndrome (NS) usually responds to GC and remains in remission as long as high-dose GC therapy is continued...
  18. Proteolytic Regulation of Podocyte Function by Cathepsin L
    Jochen Reiser; Fiscal Year: 2009
    ..We have made the novel finding that cathepsin L enzyme is present within the cytoplasm of podocytes during nephrotic syndrome and cleaves CD2AP, an important SD protein...