nephrotic syndrome

Summary

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

Top Publications

  1. 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
  2. ncbi 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. ncbi Exome sequencing identified MYO1E and NEIL1 as candidate genes for human autosomal recessive steroid-resistant nephrotic syndrome
    Simone Sanna-Cherchi
    Division of Nephrology, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    Kidney Int 80:389-96. 2011
  9. 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
  10. 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

Detail Information

Publications314 found, 100 shown here

  1. 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)...
  2. ncbi 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...
  3. 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...
  4. 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 ..
  5. 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...
  6. 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...
  7. 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...
  8. ncbi Exome sequencing identified MYO1E and NEIL1 as candidate genes for human autosomal recessive steroid-resistant nephrotic syndrome
    Simone Sanna-Cherchi
    Division of Nephrology, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    Kidney Int 80:389-96. 2011
    To identify gene loci associated with steroid-resistant nephrotic syndrome (SRNS), we utilized homozygosity mapping and exome sequencing in a consanguineous pedigree with three affected siblings...
  9. 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...
  10. 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.
  11. ncbi 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...
  12. ncbi 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...
  13. 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...
  14. ncbi Neurodevelopmental deficits in Pierson (microcoria-congenital nephrosis) syndrome
    Elke Wühl
    Department of Pediatric Nephrology, University Children s Hospital, Heidelberg, Germany
    Am J Med Genet A 143:311-9. 2007
    Pierson syndrome is an autosomal recessive disorder comprising congenital nephrotic syndrome with diffuse mesangial sclerosis and distinct eye abnormalities with microcoria reported as the most prominent clinical feature...
  15. 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...
  16. 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...
  17. pmc Nephrin redistribution on podocytes is a potential mechanism for proteinuria in patients with primary acquired nephrotic syndrome
    S Doublier
    Dipartimento di Medicina Interna, , Torino, Italy
    Am J Pathol 158:1723-31. 2001
    ..investigated the distribution of nephrin by immunofluorescence microscopy in renal biopsies of patients with nephrotic syndrome: 13 with membranous glomerulonephritis (GN), 10 with minimal change GN, and seven with focal segmental ..
  18. pmc ARHGDIA: a novel gene implicated in nephrotic syndrome
    Indra Rani Gupta
    Department of Pediatrics, Division of Nephrology, Montreal Children s Hospital and McGill University, 2300, rue Tupper E222, Montreal, Quebec, Canada H3H 1P3
    J Med Genet 50:330-8. 2013
    Congenital nephrotic syndrome arises from a defect in the glomerular filtration barrier that permits the unrestricted passage of protein across the barrier, resulting in proteinuria, hypoalbuminaemia, and severe oedema...
  19. 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...
  20. pmc The natural history of the non-nephrotic membranous nephropathy patient
    Michelle A Hladunewich
    University Health Network, University of Toronto, Toronto, Ontario, Canada
    Clin J Am Soc Nephrol 4:1417-22. 2009
    ..Although early studies suggest that patients with idiopathic membranous nephropathy (MGN) and subnephrotic range proteinuria overall do well, these studies were small and follow-up was short or difficult to discern...
  21. ncbi Increased serum and urinary microRNAs in children with idiopathic nephrotic syndrome
    Yang Luo
    Department of Clinical Laboratory, Jinling Hospital, School of Life Sciences, Nanjing University, Nanjing, China
    Clin Chem 59:658-66. 2013
    MicroRNAs (miRNAs) are present in body fluids and may have the potential to serve as disease biomarkers. This study explored the clinical value of miRNAs in serum and urine as biomarkers for idiopathic childhood nephrotic syndrome (NS).
  22. 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 ..
  23. ncbi Mutational analysis of the PLCE1 gene in steroid resistant nephrotic syndrome
    Olivia Boyer
    INSERM U983, Hopital Necker Enfants Malades, 149 rue de Sevres, Paris, France
    J Med Genet 47:445-52. 2010
    ..encoding phospholipase C epsilon 1 (PLCepsilon1) have been recently described in patients with early onset nephrotic syndrome (NS) and diffuse mesangial sclerosis (DMS)...
  24. 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)...
  25. ncbi 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...
  26. 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, ..
  27. ncbi 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)...
  28. 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...
  29. 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...
  30. 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 ..
  31. 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 ..
  32. 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...
  33. 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.
  34. 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
  35. pmc Daily corticosteroids reduce infection-associated relapses in frequently relapsing nephrotic syndrome: a randomized controlled trial
    Ashima Gulati
    Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
    Clin J Am Soc Nephrol 6:63-9. 2011
    Relapses of nephrotic syndrome often follow minor infections, commonly of the upper respiratory tract...
  36. ncbi 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...
  37. ncbi 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...
  38. ncbi Immune mediators in idiopathic nephrotic syndrome: evidence for a relation between interleukin 8 and proteinuria
    Marcelo F O Souto
    Departamento de Pediatria, Universidade Federal de Minas Gerais, Belo Horizonte, MG, 30130 100, Brazil
    Pediatr Res 64:637-42. 2008
    The pathogenesis of idiopathic nephrotic syndrome (INS) remains unknown. Several findings suggest a role for the immune system...
  39. ncbi Up-regulation of hepatic Acyl CoA: Diacylglycerol acyltransferase-1 (DGAT-1) expression in nephrotic syndrome
    Nosratola D Vaziri
    Division of Nephrology and Hypertension, University of California, Irvine, California, USA
    Kidney Int 66:262-7. 2004
    b>Nephrotic syndrome is associated with hypercholesterolemia, hypertriglyceridemia, and marked elevations of plasma low-density lipoprotein (LDL) and very low-density lipoprotein (VLDL)...
  40. ncbi Plasma membrane targeting of podocin through the classical exocytic pathway: effect of NPHS2 mutations
    Séverine Roselli
    Inserm U574, Necker Hospital, University Paris 5, Paris, France
    Traffic 5:37-44. 2004
    ..in NPHS2, the gene encoding podocin, are associated with inherited and sporadic cases of steroid-resistant nephrotic syndrome. Here, we show that brefeldin A induces accumulation of newly synthesized podocin in the endoplasmic ..
  41. ncbi High-dose oral cyclosporin therapy for recurrent focal segmental glomerulosclerosis in children
    Reem H Raafat
    Division of Pediatric Nephrology, University of Texas Health Science Center San Antonio, San Antonio, TX, USA
    Am J Kidney Dis 44:50-6. 2004
    ..Focal segmental glomerular sclerosis (FSGS) has a high propensity for recurrence after renal transplantation, with a 50% risk for graft failure from recurrent disease...
  42. ncbi 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.
  43. 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...
  44. ncbi Increasing the dose of prednisolone during viral infections reduces the risk of relapse in nephrotic syndrome: a randomised controlled trial
    A S Abeyagunawardena
    Department of Paediatrics, University of Peradeniya, Peradeniya, Sri Lanka
    Arch Dis Child 93:226-8. 2008
    Relapses of nephrotic syndrome are often triggered by viral upper respiratory tract infections (URTIs), possibly mediated by cytokine release.
  45. ncbi Specific podocin mutations determine age of onset of nephrotic syndrome all the way into adult life
    Friedhelm Hildebrandt
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA
    Kidney Int 75:669-71. 2009
    In steroid-resistant nephrotic syndrome (SRNS) Machuca et al. report that mutations of the recessive podocin gene cause adult-onset SRNS if the R229Q genetic variant occurs in a compound heterozygous state with another podocin mutation...
  46. ncbi Increased expression and apical targeting of renal ENaC subunits in puromycin aminonucleoside-induced nephrotic syndrome in rats
    Soo Wan Kim
    The Water and Salt Research Center, University of Aarhus, DK 8000 Aarhus C, Denmark
    Am J Physiol Renal Physiol 286:F922-35. 2004
    b>Nephrotic syndrome is often accompanied by sodium retention and generalized edema. However, the molecular basis for the decreased renal sodium excretion remains undefined...
  47. ncbi Intravenous methylprednisolone in idiopathic childhood nephrotic syndrome
    Mohan Shenoy
    Department of Paediatric Nephrology, Royal Manchester Children s Hospital, Manchester, UK
    Pediatr Nephrol 25:899-903. 2010
    The aim of our study was to determine the clinical course of children with idiopathic childhood nephrotic syndrome (ICNS) who received intravenous methylprednisolone (ivMP) following failure to achieve remission with standard oral ..
  48. 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...
  49. ncbi Discovery and initial validation of α 1-B glycoprotein fragmentation as a differential urinary biomarker in pediatric steroid-resistant nephrotic syndrome
    Nuntawan Piyaphanee
    Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, Cincinnati, OH, USA
    Proteomics Clin Appl 5:334-42. 2011
    In this cross-sectional pilot study we set out to discover a non-invasive biomarker that could distinguish steroid-resistant nephrotic syndrome (SRNS) from steroid-sensitive nephrotic syndrome (SSNS).
  50. ncbi Regulation of the proximal tubular sodium/proton exchanger NHE3 in rats with puromycin aminonucleoside (PAN)-induced nephrotic syndrome
    Valérie Besse-Eschmann
    Department of Anatomy, University of Zurich, Irchel, Switzerland
    J Am Soc Nephrol 13:2199-206. 2002
    Excessive proteinuria due to loss of glomerular permselectivity in nephrotic syndrome can cause disturbances in renal salt and water handling with edema formation...
  51. ncbi Nephrotic syndrome and unrecognized Plasmodium malariae infection in a US Navy sailor 14 years after departing Nigeria
    Richard Hedelius
    Infectious Diseases Division, Internal Medicine Department, Naval Medical Center Portsmouth, 620 John Paul Jones Circle, Portsmouth, VA 23708, USA
    J Travel Med 18:288-91. 2011
    A 34-year-old Nigerian man presented with nephrotic syndrome. Renal biopsy revealed chronic membranous glomerulopathy with focal segmental sclerosis. Blood Giemsa smear contained rare Plasmodium sp...
  52. pmc Concurrent nephrotic syndrome and acute renal failure caused by chronic lymphocytic leukemia (CLL): a case report and literature review
    Xianrui Dou
    Department of Nephrology, Affiliated Shunde First People s Hospital of Southern Medical University, Penglai Road, Daliang District, Foshan 528300, China
    Diagn Pathol 6:99. 2011
    ..Glomerular involvement presenting as proteinuria or even nephrotic syndrome is exceptionally rare...
  53. ncbi Association of apoE gene expression and its gene polymorphism with nephrotic syndrome susceptibility: a meta-analysis of experimental and human studies
    Tian Biao Zhou
    Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, China
    Mol Biol Rep 39:9347-54. 2012
    ..The disorder of lipids metabolism is an important feature for nephrotic syndrome (NS)...
  54. pmc Gain of glycosylation in integrin α3 causes lung disease and nephrotic syndrome
    Nayia Nicolaou
    Department of Medical Genetics, University Medical Center Utrecht, Utrecht, The Netherlands
    J Clin Invest 122:4375-87. 2012
    ..a novel missense mutation in the human ITGA3 gene, causing fatal interstitial lung disease and congenital nephrotic syndrome. The mutation caused an alanine-to-serine substitution in the integrin α3 subunit, thereby introducing an N-..
  55. 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...
  56. pmc Simultaneous sequencing of 24 genes associated with steroid-resistant nephrotic syndrome
    Hugh J McCarthy
    Academic and Children s Renal Unit, University of Bristol, UK
    Clin J Am Soc Nephrol 8:637-48. 2013
    Up to 95% of children presenting with steroid-resistant nephrotic syndrome in early life will have a pathogenic single-gene mutation in 1 of 24 genes currently associated with this disease. Others may be affected by polymorphic variants...
  57. ncbi Successful management of factor IX inhibitor-associated nephrotic syndrome in a hemophilia B patient
    Priya Verghese
    University of Minnesota Pediatric Nephrology, 2450 Riverside Avenue, Minneapolis, MN 55437, USA
    Pediatr Nephrol 28:823-6. 2013
    b>Nephrotic syndrome (NS) is a recognized complication of immune tolerance induction (ITI) therapy, a treatment strategy used to treat inhibitors in patients with hemophilia B receiving factor IX concentrate.
  58. ncbi Renal interstitial fibrosis in children treated with FK506 for nephrotic syndrome
    Catherine Morgan
    Division of Pediatric Nephrology, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
    Nephrol Dial Transplant 26:2860-5. 2011
    Steroid-dependent, steroid-resistant or frequently relapsing nephrotic syndrome carries a poor prognosis, including progression to renal failure...
  59. pmc Digital pathology evaluation in the multicenter Nephrotic Syndrome Study Network (NEPTUNE)
    Laura Barisoni
    Department of Pathology, University of Miami, Miller School of Medicine, 1400 NW 12th Avenue, Miami, FL 33136, USA
    Clin J Am Soc Nephrol 8:1449-59. 2013
    ..To improve this, the Nephrotic Syndrome Study Network (NEPTUNE) is systematically applying digital pathology review in a multicenter study using ..
  60. pmc ARHGDIA mutations cause nephrotic syndrome via defective RHO GTPase signaling
    Heon Yung Gee
    Division of Nephrology, Department of Medicine, Boston Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Clin Invest 123:3243-53. 2013
    b>Nephrotic syndrome (NS) is divided into steroid-sensitive (SSNS) and -resistant (SRNS) variants. SRNS causes end-stage kidney disease, which cannot be cured...
  61. ncbi Characterisation of renal immune cell infiltrates in children with nephrotic syndrome
    Kerstin Benz
    Klinik fur Kinder und Jugendliche, Friedrich Alexander Universitat Erlangen Nurnberg, Loschgestrasse 15, 91054 Erlangen, Germany
    Pediatr Nephrol 25:1291-8. 2010
    ..evidence that not only T cells but also B cells may play an important role in the pathogenesis of idiopathic nephrotic syndrome (NS)...
  62. ncbi T-lymphocyte populations and cytokines in childhood nephrotic syndrome
    Giuliana Lama
    Department of Paediatrics, Second University of Naples, Italy
    Am J Kidney Dis 39:958-65. 2002
    ..factor-alpha [TNF-alpha]) in peripheral-blood mononuclear cells of 18 children with steroid-sensitive (SS) nephrotic syndrome (NS) and 10 children with steroid-resistant (SR) NS. Mean age was 10.9 +/- 5...
  63. ncbi Proteinuria elevates asymmetric dimethylarginine levels via protein arginine methyltransferase-1 overexpression in a rat model of nephrotic syndrome
    Yusuke Kaida
    Division of Nephrology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan
    Life Sci 91:301-5. 2012
    ..Thus, ADMA can partially account for the increased risk of CVD in CKD patients presenting proteinuria. However, a causal relationship between proteinuria and ADMA remains to be demonstrated...
  64. ncbi Increased fasting plasma acylation-stimulating protein concentrations in nephrotic syndrome
    Metin Ozata
    Department of Endocrinology, Gulhane School of Medicine, Etlik Ankara, TR 06018 Sogutozu Ankara, Turkey
    J Clin Endocrinol Metab 87:853-8. 2002
    ..However, little is known about the alterations in fasting plasma ASP and leptin concentrations in the nephrotic syndrome. As hyperlipidemia is one of the most striking manifestations of the nephrotic syndrome, we have ..
  65. ncbi 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 ..
  66. ncbi New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome
    Michael van Husen
    Pediatric Nephrology, University Medical Center Hamburg Eppendorf, Martinistr 52, 20246, Hamburg, Germany
    Pediatr Nephrol 26:881-92. 2011
    Although many children with idiopathic nephrotic syndrome (INS) respond initially to steroid therapy, repeated courses for patients with relapses often cause significant steroid toxicity...
  67. 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.
  68. 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...
  69. 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...
  70. ncbi 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...
  71. ncbi 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 ..
  72. 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...
  73. ncbi 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.
  74. ncbi 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)...
  75. ncbi 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 ..
  76. ncbi 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.
  77. 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...
  78. ncbi 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...
  79. 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).
  80. ncbi 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...
  81. 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 ..
  82. 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...
  83. ncbi 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...
  84. 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...
  85. 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...
  86. 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...
  87. 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)...
  88. 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 ..
  89. 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)...
  90. 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 ..
  91. 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.
  92. 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...
  93. 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...
  94. 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.
  95. 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...
  96. ncbi 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.
  97. 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.
  98. ncbi 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...
  99. ncbi Clinical and epidemiological assessment of steroid-resistant nephrotic syndrome associated with the NPHS2 R229Q variant
    Eduardo Machuca
    INSERM, U574, Hopital Necker, Paris, France
    Kidney Int 75:727-35. 2009
    Mutations of NPHS2, encoding podocin, are the main cause of autosomal recessive steroid-resistant nephrotic syndrome (NS) presenting in childhood...
  100. ncbi Repetitive fragmentation products of albumin and alpha1-antitrypsin in glomerular diseases associated with nephrotic syndrome
    Giovanni Candiano
    Laboratory on Pathophysiology of Uremia, G Gaslini Children Hospital, Genova, Italy
    J Am Soc Nephrol 17:3139-48. 2006
    Even if nephrotic syndrome is characterized by massive urinary loss of major plasma proteins, a clear structural characterization based on proteomics has never been reported...
  101. 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...

Research Grants66

  1. Cardiotrophin-Like Cytokine 1, a Candidate Molecule for the FSGS Factor
    Virginia J Savin; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Steroid resistant nephrotic syndrome and focal segmental glomerulosclerosis (FSGS) pose especially difficult clinical problems because the disease recurs in about 30% of such patients after renal ..
  2. Role of Endocytosis and Vesicular Trafficking in Podocyte Health and Filter Integ
    Puneet Garg; Fiscal Year: 2012
    ..Podocyte vesicles have often been observed in TEM studies on kidney biopsies from patients with nephrotic syndrome. These studies support the idea that podocytes play an active role in removing proteins from the GBM and ..
  3. Exon capture and large-scale sequencing for disease-cause identification, early d
    Friedhelm Hildebrandt; Fiscal Year: 2010
    ..capture and large-scale sequencing for disease-cause identification, early detection and drug discovery in nephrotic syndrome. Background: Chronic kidney diseases (CKD) take one of the highest tolls on human health...
  4. Mesonephric Model of Podocyte Injury and Regeneration
    Weibin Zhou; Fiscal Year: 2012
    ..is to utilize a new animal model system that I have developed for the study of molecular mechanisms of nephrotic syndrome and podocyte regeneration...
  5. FEASIBILITY OF REPAIRING GBM DEFECTS IN VIVO
    Jeffrey H Miner; Fiscal Year: 2012
    ..glomerulonephritis), and those that affect laminin beta2 (LAMB2) cause Pierson syndrome/congenital nephrotic syndrome. The well-defined nature of the glomerular defects and the existence of well- characterized mouse models ..
  6. Investigation of Nephrotic Syndrome
    LIONEL CLAUDIUS CLEMENT; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Nephrotic syndrome is a major cause of kidney failure, and patients with this condition develop large amounts of proteinuria and elevated levels of plasma lipids (triglycerides and cholesterol)...
  7. Vitamin D Deficiency in Glomerular Disease
    Michelle Denburg; Fiscal Year: 2013
    ..The proposed project comprises: (1) an ancillary cross-sectional study of 450 participants in the NIH-funded Nephrotic Syndrome Study Network (NEPTUNE) and (2) a pilot study of cholecalciferol supplementation in 35 children and young ..
  8. Use of small molecules that stabilize dynamin rings in podocytopathies
    Sanja Sever; Fiscal Year: 2013
    ..Most forms of proteinuria and nephrotic syndrome are characterized by transformation of podocyte FPs into a band of cytoplasm due to de- regulation of the ..
  9. Mesonephric Model of Podocyte Injury and Regeneration
    Weibin Zhou; Fiscal Year: 2013
    ..is to utilize a new animal model system that I have developed for the study of molecular mechanisms of nephrotic syndrome and podocyte regeneration...
  10. Molecular Mechanisms of Albumin Trafficking in Podocytes
    Judith T Blaine; Fiscal Year: 2013
    ..and advanced imaging techniques in cultured human podocytes and will extend our findings to a mouse model of nephrotic syndrome. Our proposal is novel in that it proposes a mechanistic link between impaired albumin handling and podocyte ..
  11. Role of Par1 Polarity Proteins in Podocyte Development and Glomerular Disease
    KIMBERLY JEAN REIDY; Fiscal Year: 2013
    ..Glomerular Par1a/b expression was altered in rodent models of diabetic nephropathy, nephrotic syndrome and glomerulosclerosis...
  12. Modulation of the Epithelial Sodium Channel by Proteases and Peptide Inhibitors
    CHRISTOPHER JOHN PASSERO; Fiscal Year: 2010
    ..The inactive pool may be a target of proteases in pathophysiologic states, like hypertension and nephrotic syndrome. Other serine proteases besides furin and prostasin can activate these channels...
  13. An Integrative Systems Genetics Approach to Nephrotic Syndrome
    MATTHEW GORDON SAMPSON; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Nephrotic syndrome (NS) is a clinical syndrome of massive proteinuria caused by abnormal glomerular permeability...
  14. Fat1 and Conventional Kinesin in Lateral Polarity
    DUNCAN BRUCE JOHNSTONE; Fiscal Year: 2012
    ..in abnormal development of the slit diaphragm, massive proteinuria, and death shortly after birth with nephrotic syndrome, a phenotype similar to mice deleted for nphs1 (Nephrin)...
  15. In Vivo Assessment of Calcineurin Inhibitor Toxicity in Children
    Joshua M Thurman; Fiscal Year: 2013
    ..negative effects of CNIs on kidney cell and vascular endothelial cell metabolism in pediatric patients with nephrotic syndrome and after kidney transplantation are reflected by changes in plasma and urine metabolite patterns, and - ..
  16. LAMININS AND GLOMERULAR FILTRATION
    Jeffrey H Miner; Fiscal Year: 2013
    ..and protects podocytes from the injurious effects of plasma proteins, as mice lacking laminin beta2 develop nephrotic syndrome and renal failure...
  17. Discover and functionally characterize full-penetrance causes of nephrosis/FSGS
    Friedhelm Hildebrandt; Fiscal Year: 2013
    ..b>Nephrotic syndrome (NS) is defined by significant proteinuria, resulting in hypoalbuminemia and edema...
  18. Glucocorticoid Receptor Function in Steroid Resistant Childhood Nephrotic Syndrom
    JORGE A INIGUEZ LLUHI; Fiscal Year: 2010
    b>Nephrotic Syndrome, characterized by massive proteinuria, hypoalbuminemia and edema, is one of the most frequently seen kidney diseases in children...
  19. H3Africa Kidney Disease Research Network
    Akinlolu O Ojo; Fiscal Year: 2013
    ..In addition, nearly 20 genetic variants have been linked to childhood onset nephrotic syndrome. Most of these genetic advances in elucidating the etiology of kidney disease have occurred outside sub-..
  20. Redefining membranous nephropathy by autoantibody-specific subclassification
    Laurence H Beck; Fiscal Year: 2013
    ..Idiopathic membranous nephropathy (IMN) is an autoimmune disease of the kidney and a common cause of the nephrotic syndrome in adults...
  21. Mechanisms of proteinuria induced by RhoA GTPases
    Robert Spurney; Fiscal Year: 2013
    ..Several familial forms of nephrotic syndrome are caused by mutations in proteins expressed exclusively by glomerular podocytes...
  22. Megalin function in the normal and diseased kidney
    DANIEL R BIEMESDERFER; Fiscal Year: 2010
    ..the endocytic capacity of the proximal tubule becomes saturated and results in progressive renal damage (nephrotic syndrome)...
  23. DEVELOPMENTAL NEPHROLOGY
    Frederick Kaskel; Fiscal Year: 2013
    ..Research projects, related to clinical issues, involve studies into the molecular characterization of nephrotic syndrome of childhood, immunosuppression and allograft rejection in pediatric transplantation, mechanisms of bone ..
  24. Cyclic AMP Signaling Affects Urine Concentration through UT-A1
    Mitsi A Blount; Fiscal Year: 2012
    ..water homeostasis that occurs in common clinical disorders, such as congestive heart failure, cirrhosis, and nephrotic syndrome. We hope to clarify the mechanisms of urea movement to potentially provide insight into the treatment of ..
  25. FASEB SRC on Neural Mechanisms in Cardiovascular Regulation
    Glenn M Toney; Fiscal Year: 2013
    ..diseases such as arterial hypertension/preeclampsia, heart failure, obesity/diabetes, sleep apnea, stroke, nephrotic syndrome, fever/sepsis, etc...
  26. Sidekick-1 Upregulation in Podocytes Induces FSGS by Disrupting MAGI-1 Function
    Lewis Kaufman; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Focal and segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome and end-stage renal disease worldwide...
  27. Function of Hsp27 and its Binding Proteins in the Glomerular Podocyte
    William E Smoyer; Fiscal Year: 2010
    b>Nephrotic syndrome (NS) is a common kidney disease, but the molecular mechanisms underlying the disease remain unclear for the vast majority of cases...
  28. Structure and Function of Epithelial Sodium Channels
    ISABELLE RHYSSA JOE EDURIA BACONGUIS; Fiscal Year: 2013
    ..in the activation of ENaCs, aberrant proteolysis of ENaCs contributes to NaCl retention that characterizes nephrotic syndrome. Despite such clinical importance, detail into the mechanism of ENaC function at atomic resolution has ..
  29. Glomerular Disease Mechanisms mediated by Podocyte TRPC6
    Jochen Reiser; Fiscal Year: 2013
    ..is to advance our understanding of podocyte biology and the molecular mechanisms leading to proteinuria and nephrotic syndrome that originate from podocyte injury...
  30. Mechanisms of Glucocorticoid Action in Podocytes
    RICHARD FREDERICK RANSOM; Fiscal Year: 2011
    b>Nephrotic syndrome (NS) is one of the most common kidney diseases seen in children...
  31. Nephrotic Syndrome, Cardiovascular Disease Risk and the Effect of Statin Therapy
    Laura H Mariani; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Nephrotic syndrome is a common presentation of multiple disease entities, including rare primary glomerular disease such as minimal change disease, focal segmental glomeruloscerosis and membranous ..
  32. Genetics of Renal End Organ Damage in Hypertension
    Michael R Garrett; Fiscal Year: 2013
    ..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...
  33. Mutant Laminin Chains and Nephrotic Syndrome
    YING MAGGIE CHEN; Fiscal Year: 2013
    ..Null mutations in LAMB2 cause Pierson syndrome, characterized by congenital nephrotic syndrome with severe ocular and neurological defects...
  34. Expression Genetics in Drug Therapy
    Wolfgang Sadee; Fiscal Year: 2013
    ..been initiated on estrogen and glucocorticoid receptors, the latter to be tested in glucocorticoid-resistant nephrotic syndrome in children...
  35. Small Proteins and Renal Urea Transport Regulation
    Guangping Chen; Fiscal Year: 2013
    ..the diseases with total body fluid overload such as hypertension, congestive heart failure, cirrhosis, and nephrotic syndrome. PUBLIC HEALTH RELEVANCE: Sodium and urea are the two major solutes that contribute to the medullary ..
  36. A New Locus for Hereditary FSGS on Chromosome 2p
    RASHEED ADEBAYO GBADEGESIN; Fiscal Year: 2013
    ..Recent results from positional cloning of novel genes mutated in nephrotic syndrome and FSGS have provided important insights into the pathogenesis of the disease, including a strong link ..
  37. Integrative Proteomics &Metabolomics for Pediatric Glomerula Disease Biomarkers
    William E Smoyer; Fiscal Year: 2013
    Primary nephrotic syndrome is the dominant cause of acquired kidney disease in children, with significant morbidity and mortality from the disease and its treatment, and a substantial risk of kidney failure...
  38. Multicenter Trial of Focal Glomerulosclerosis
    Debbie Gipson; Fiscal Year: 2009
    ..of a collaborative multicenter trial that will evaluate response rates of children and young adults with the nephrotic syndrome due to FSGS treated with cyclosporin A as compared to corticosteroids plus angiotensin receptor blocker ..
  39. PROMIS Pediatrics: Longitudinal Validation and Linking Pediatric and Adult Item B
    Darren A DeWalt; Fiscal Year: 2013
    ..We propose longitudinal studies in children with asthma, cancer, nephrotic syndrome, and sickle cell disease...
  40. The Genetic Basis of FSGS
    Martin R Pollak; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Identification of the first several nephrotic syndrome and focal segmental glomerulosclerosis (FSGS) genes has had a significant impact on the understanding of glomerular function and disease...
  41. OXIDANT MECHANISMS IN MEMBRANOUS NEPHROPATHY
    Sudhir Shah; Fiscal Year: 1993
    ..GSH levels and the effect of increasing glomerular non-protein sulfhydryl content on anti-Fx1A-induced nephrotic syndrome. F. To examine the role of iron in PHN...
  42. TRPC-mediated calcium signaling in podocytes
    Anna Greka; Fiscal Year: 2012
    ..in the pathogenesis of proteinuria, and thus its potential as a new therapeutic target for diseases such as nephrotic syndrome, FSGS, diabetic and hypertensive nephropathy...
  43. Podocyte Secreted Proteins
    SUMANT SINGH CHUGH; Fiscal Year: 2013
    ..expression of Angiopoietin-like 4 (Angptl4) in podocytes in human and experimental glucocorticoid sensitive nephrotic syndrome. Secretion of this protein into the glomerular capillary loop leads to binding to the glomerular basement ..
  44. Nephrotic Syndrome Rare Disease Clinical Research Network
    Matthias Kretzler; Fiscal Year: 2013
    ..For these reasons, we propose the establishment of a Nephrotic Syndrome RDCRN, a multidisciplinary research and education platform that brings together clinical and translational ..
  45. MOLECULAR MECHANISMS IN NEPHROTIC SYNDROME
    William Smoyer; Fiscal Year: 2001
    b>Nephrotic syndrome (NS), a common kidney disease in children, is characterized by retraction (effacement) of the distal "foot" processes of glomerular epithelial cells (GEC) that surround glomerular capillaries...
  46. 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...
  47. CD80 Expression on Podocytes and the Pathogenesis of Minimal Change Disease
    RICHARD JOSEPH JOHNSON; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Minimal change disease (MCD) is the most common nephrotic syndrome in children, and can be associated with marked morbidity...
  48. TRPC channels in proteinuric kidney disease
    Anna Greka; Fiscal Year: 2013
    ..kidney disease will help identify targets for the development of medications and therapies to treat and prevent diseases such as nephrotic syndrome and diabetic nephropathy, which currently affect many kidney disease patients worldwide.
  49. Nuclear Receptor and MAP Kinase Signaling in Podocyte Injury
    William E Smoyer; Fiscal Year: 2013
    ..1 billion annually. Nephrotic syndrome (NS), characterized by podocyte injury, is one of the most common forms of glomerular disease...
  50. 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...
  51. 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...
  52. 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 ..
  53. PATHOPHYSIOLOGY OF AIDS GLOMERULOPATHY
    Bryan Myers; Fiscal Year: 1992
    ..Because the idiopathic nephrotic syndrome (INS) is associated with a similar alteration of glomerular epithelial cells with or without focal and ..
  54. AQUAPORIN-2 EXCRETION IN DISORDERS OF WATER BALANCE
    Melissa Cadnapaphornchai; Fiscal Year: 2004
    ..patients during pregnancy and the menstrual cycle, and in patients with congestive heart failure, cirrhosis, nephrotic syndrome, and acquired nephrogenic diabetes insipidus due to lithium therapy or autosomal dominant polycystic kidney ..
  55. 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...
  56. Effects of FSGS factor on arachidonic acid metabolism
    Ellen McCarthy; Fiscal Year: 2003
    ..The incidence of FSGS has increased markedly over the past 20 years, and is the most common cause of primary nephrotic syndrome in adults in several series...
  57. 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 ..
  58. EICOSANOIDS IN FOCAL SEGMENTAL GLOMERULOSCLEROSIS
    Ellen McCarthy; Fiscal Year: 2002
    ..It is the now the most common progressive glomerular disease in children and account for 20_25% of nephrotic syndrome in adults...
  59. FSGS PERMEABILITY FACTOR IN CHILDHOOD NEPHROTIC SYNDROME
    Laurence Greenbaum; Fiscal Year: 1999
    ..The specter of FSGS is present when nephrotic syndrome develops in children, though most have a good prognosis...
  60. VISUALIZING DENDRITIC CELL--T CELL INTERACTIONS IN VIVO
    Elizabeth Ingulli; Fiscal Year: 2000
    ..The candidate's interest in research began in medical school and focused on childhood nephrotic syndrome and renal transplantation...
  61. NEURAL CONTROL OF RENAL FUNCTION
    GERALD DIBONA; Fiscal Year: 2000
    ..observed in rat models of congestive heart (CHF), cirrhosis due to common bile duct ligation (CBDL) and nephrotic syndrome (NS) by affecting specific central nervous system (CNS) regions involved in arterial/cardiac baroreflex ..
  62. MOLECULAR PHYSIOLOGY AND STRUCTURE OF AQUAPORINS
    Alfred Van Hoek; Fiscal Year: 2002
    ..which could lead to alternative treatments of hypertension, congestive heart failure, cirrhosis, the nephrotic syndrome, and other edema forming states...
  63. Urinary Biomarkers for Steroid Resistant Nephrotic Syndr
    Robert Mak; Fiscal Year: 2005
    The current initial approach to idiopathic nephrotic syndrome in children is a therapeutic trial of steroids and was based on data from 25 years ago, which showed that 80% of these patients have minimal-change disease and are steroid-..
  64. 6th International Podocyte Conference
    Stuart Shankland; Fiscal Year: 2006
    ..Studies have shown that nephrotic syndrome is primarily a disease of the podocyte...
  65. PATHOGENESIS OF INTERSTITIAL FIBROSIS IN PROTEINURIA
    Deborah Kees Folts; Fiscal Year: 1999
    The long-term goal of the applicant is to explore a paradigm of disease, hypothesizing that in the nephrotic syndrome, both proteinuria and hypercholesterolemia have a causative role in the development of interstitial inflammation and ..
  66. Podocalyxin in the renal filtration apparatus
    David Kershaw; Fiscal Year: 2006
    The nephrotic syndrome affects both children and adults and may lead to renal failure and death. Abnormalities of podocyte structure and the glomerular polyanion associated with nephrotic syndrome are wellrecognized...