Genomes and Genes
Summary: Clinical association of heavy PROTEINURIA; HYPOALBUMINEMIA; and generalized EDEMA.
Publications236 found, 100 shown here
- Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 casesVincent 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. 2008Several 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...
- Rituximab in refractory nephrotic syndromeAgnieszka Prytuła
Department of Paediatric Nephrology, Great Ormond Street Hospital NHS Trust, London, UK
Pediatr Nephrol 25:461-8. 2010The aim of this study was to establish the efficacy and safety of rituximab in refractory nephrotic syndrome (NS)...
- WT1 and glomerular diseasesPatrick 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, ..
- ACE gene insertion/deletion polymorphism in childhood idiopathic nephrotic syndromeErkin 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...
- Loss of glomerular foot processes is associated with uncoupling of podocalyxin from the actin cytoskeletonT 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...
- Urinary proteome of steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome of childhoodRobert P Woroniecki
Section of Pediatric Nephrology, Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY 10467, USA
Am J Nephrol 26:258-67. 2006The 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 ..
- Ocular findings in Japanese children with nephrotic syndrome receiving prolonged corticosteroid therapyYoriko Hayasaka
Department of Ophthalmology, Toyama Medical and Pharmaceutical University, Toyama, Japan
Ophthalmologica 220:181-5. 2006To examine ocular findings in Japanese children with nephrotic syndrome who receive prolonged corticosteroid treatment.
- Change of the course of steroid-dependent nephrotic syndrome after rituximab therapyKerstin Benz
Department of Pediatrics, , Loschgestrasse 15, 91054 Erlangen, Germany
Pediatr Nephrol 19:794-7. 2004A 16-year-old patient with steroid-dependent nephrotic syndrome with more than 35 relapses developed severe relapsing idiopathic thrombocytopenic purpura (ITP)...
- Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UKP 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 ..
- ACE I/D gene polymorphism in primary FSGS and steroid-sensitive nephrotic syndromeFaruk 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)...
- Effect of angiotensin-converting enzyme gene insertion/deletion polymorphism on steroid resistance in Egyptian children with idiopathic nephrotic syndromeMaha 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.
- Clinicopathological features and prognosis of Chinese children with idiopathic nephrotic syndrome between different age groupsJei Wen Chang
Department of Pediatrics, Taipei Veterans General Hospital, Taipei 11217, Taiwan
Eur J Pediatr 168:1189-94. 2009Ethnicity and age play important roles in the epidemiology of idiopathic nephrotic syndrome (INS) in children...
- ACE gene polymorphism in Egyptian children with idiopathic nephrotic syndromeM E Fahmy
Pediatric Department, Research Institute of Ophthalmology, Cairo, Egypt
Bratisl Lek Listy 109:298-301. 2008Idiopathic 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.
- Hereditary nephrotic syndrome: a systematic approach for genetic testing and a review of associated podocyte gene mutationsGenevieve Benoit
INSERM, U983, Hopital Necker Enfants Malades, Paris, France
Pediatr Nephrol 25:1621-32. 2010Several genes have been implicated in genetic forms of nephrotic syndrome occurring in children...
- Rituximab failed to improve nephrotic syndrome in renal transplant patients with recurrent focal segmental glomerulosclerosisJ 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...
- Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosisEllen T McCarthy
Kidney Institute, University of Kansas Medical Center, Kansas City, KS 66160, USA
Clin J Am Soc Nephrol 5:2115-21. 2010Circulating permeability factors may be important in idiopathic nephrotic syndrome (INS) including focal segmental glomerulosclerosis (FSGS) and in recurrence after renal transplantation...
- Hepatic cholesterol metabolism in experimental nephrotic syndromeA Al-Shurbaji
Department of Medical Laboratory Sciences, Karolinska Institutet at Huddinge University Hospital, Sweden
Lipids 33:165-9. 1998Hypercholesterolemia is a consistent feature of the nephrotic syndrome. However, the mechanisms underlying this perturbation are unclear...
- Angiotensin converting enzyme gene insertion/deletion polymorphism in idiopathic nephrotic syndrome in Kuwaiti Arab childrenA 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.
- A nonsense mutation in the LIMP-2 gene associated with progressive myoclonic epilepsy and nephrotic syndromeAndrea 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...
- Rituximab treatment for posttransplant lymphoproliferative disorder (PTLD) induces complete remission of recurrent nephrotic syndromeKandai 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 ..
- Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndromeM Kestila
Department of Biochemistry, University of Oulu, Finland
Mol Cell 1:575-82. 1998Congenital nephrotic syndrome of the Finnish type (NPHS1) is an autosomal-recessive disorder, characterized by massive proteinuria in utero and nephrosis at birth...
- Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter studyDjalila 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. 2009Long-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...
- Acute renal failure in children with idiopathic nephrotic syndromeNagamani Agarwal
Department of Pediatrics, Children's Kidney Care Center, St. Johns Medical College and Hospital, Sarjapur Road, Bangalore 560034, India
Pediatr Nephrol 18:1289-92. 2003Acute renal failure (ARF) is an uncommon but alarming complication of idiopathic nephrotic syndrome. The renal failure could be secondary to causes evident from the history and evaluation, such as severe intravascular volume depletion, ..
- The effects of corticosteroids on behavior in children with nephrotic syndromeA 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 ..
- WT1 gene mutations in three girls with nephrotic syndromeKhalid 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...
- Expression of nephrin, podocin, alpha-actinin, and WT1 in children with nephrotic syndromeNa Guan
Department of Pediatrics, Peking University First Hospital, Peking, China
Pediatr Nephrol 18:1122-7. 2003..at the slit diaphragm and expressed by the podocyte, were found to be causative in congenital/familial nephrotic syndrome (NS), but their role in acquired NS remains unclear...
- Successful treatment of nephrotic syndrome due to FMF amyloidosis with azathioprine: report of three Turkish casesHayriye 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.
- Decreased antioxidant activity in hypercholesterolemic children with nephrotic syndromeJacek Zachwieja
Department of Nephrology, Institute of Pediatrics, Medical University, Poznan, Poland
Med Sci Monit 9:CR235-9. 2003The aim of the present study was to estimate the total antioxidant status (TAS) and main antioxidant enzyme activity in children with nephrotic syndrome (NS).
- Predisposition to relapsing nephrotic syndrome by a nephrin mutation that interferes with assembly of functioning microdomainsAkemi 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. 2009Minimal-change disease (MCD) is the most common cause of nephrotic syndrome (NS) and is characterized only by minor morphological alterations in podocytes...
- Rituximab for refractory cases of childhood nephrotic syndromeJameela A Kari
Department of Pediatrics, King Abdulaziz University, Jeddah, Saudi Arabia
Pediatr Nephrol 26:733-7. 2011Rituximab has been used over the last decade as a rescue therapy for refractory cases of nephrotic syndrome (NS)...
- Renal artery stenosis and nephrotic syndrome: a rare combination in an infantMia 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. 2003We describe an uncommon pediatric finding of unilateral renal artery stenosis, which presented as nephrotic syndrome, hypertension, failure to thrive, and hyponatremia...
- Role of the immune system in the pathogenesis of idiopathic nephrotic syndromeJosé G Van Den Berg
Department of Pathology, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Clin Sci (Lond) 107:125-36. 2004Idiopathic NS (nephrotic syndrome) is characterized by massive proteinuria, due to a leak in the glomerular barrier to proteins...
- A case of necrotizing glomerulonephritis presenting with nephrotic syndrome associated with pulmonary cryptococcosisMasaru 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. 2005We 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...
- Independent risk factors for chronic cyclosporine induced nephropathy in children with nephrotic syndromeS Fujinaga
Division of Nephrology, Saitama Children s Medical Centre, 2100 Magome, Iwatsuki ku, Saitama shi 339 8551, Japan
Arch Dis Child 91:666-70. 2006Cyclosporine 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...
- Histopathological diagnosis and outcome of paediatric nephrotic syndromeIftikhar Ejaz
Department of Paediatrics, King Edward Medical College, Lahore
J Coll Physicians Surg Pak 14:229-33. 2004To determine the histological picture and outcome of treatment in cases of childhood nephrotic syndrome who needed renal biopsy.
- Podocin and nephrotic syndrome: implications for the clinicianPatrick Niaudet
J Am Soc Nephrol 15:832-4. 2004
- Management of edema in nephrotic syndromeAnil 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. 2004b>Nephrotic syndrome is a recurrent or chronic disorder characterized by heavy proteinuria, hypoalbuminemia and edema...
- Community-acquired pneumonia due to Bordetella holmesii in a patient with frequently relapsing nephrotic syndromeChristina 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.
- The Nephrotic Syndrome is an immunoinflammatory disorderMarcello Camici
Internal Medicine Department, Pisa University, Via Roma n 67, 56126 Pisa, Italy
Med Hypotheses 68:900-5. 2007The Nephrotic Syndrome is still a therapeutic and physiopathological challenge. The clinical response to systemic immunosuppression shows that an immunoinflammatory disorder supports the nephrotic syndrome...
- COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafnessSaskia F Heeringa
Department of Pediatrics, University of Michigan, Ann Arbor, Michigan, USA
J Clin Invest 121:2013-24. 2011Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of end-stage renal failure...
- Focal and segmental glomerulosclerosis in children: a longitudinal assessmentOlivia 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)...
- Treatment of nephrotic syndrome in children and controlled trialsGuido Filler
Department of Paediatrics, Division of Nephrology, Children s Hospital of Eastern Ontario, University of Ottawa, Canada
Nephrol Dial Transplant 18:vi75-8. 2003To determine the sequential therapy of childhood nephrotic syndrome (NS) with presumed minimal change nephropathy using the evidence from clinical trials.
- Th1 and Th2 cytokine mRNA profiles in childhood nephrotic syndrome: evidence for increased IL-13 mRNA expression in relapseH K Yap
Department of Pediatrics, National University of Singapore, Singapore
J Am Soc Nephrol 10:529-37. 1999Idiopathic 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...
- Dynamic (re)organization of the podocyte actin cytoskeleton in the nephrotic syndromeJun 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...
- Experience with tacrolimus in children with steroid-resistant nephrotic syndromeLavjay Butani
Department of Pediatrics, University of California Davis Children s Hospital, 2516 Stockton Boulevard, Sacramento, CA 95817, USA
Pediatr Nephrol 24:1517-23. 2009Children with steroid-resistant nephrotic syndrome (SRNS) are at risk of developing renal failure...
- Defective nephrin trafficking caused by missense mutations in the NPHS1 gene: insight into the mechanisms of congenital nephrotic syndromeL Liu
Division of Matrix Biology, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, S-171 77 Stockholm, Sweden
Hum Mol Genet 10:2637-44. 2001Congenital nephrotic syndrome of the Finnish type (CNF or NPHS1) is an autosomal recessive kidney disorder resulting in severe proteinurea and renal dysfunction...
- Infantile nephrotic syndrome and congenital glaucomaJ A Kari
Pediatrics Department, King Abdulaziz University Hospital, PO Box 80215, Jeddah 21589, Saudi Arabia
Pediatr Nephrol 16:894-7. 2001A case of infantile nephrotic syndrome (NS) with advanced membranoproliferative glomerulonephritis (MPGN), type I, and bilateral congenital glaucoma, is presented...
- FSGS permeability factor-associated nephrotic syndrome: remission after oral galactose therapyEric De Smet
Hôpital du Sacré Coeur and Université de Montréal, QC, Canada
Nephrol Dial Transplant 24:2938-40. 2009Some cases of nephrotic syndrome in focal and segmental glomerulosclerosis (FSGS) are associated with a circulating factor, the FSGS permeability factor (FSPF)...
- Pathogenesis of edema formation in the nephrotic syndromeJ G Vande Walle
Department of Pediatric Nephrology, University of Gent, Belgium
Pediatr Nephrol 16:283-93. 2001Based on observations in 110 children with nephrotic syndrome (NS) and data from the literature, existing concepts on the pathogenesis of edema formation in the NS have been modified...
- Pulse methylprednisolone treatment of idiopathic steroid-resistant nephrotic syndromeP 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...
- Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: a single center studyJameela A Kari
Department of Pediatrics, King Abdul Aziz University Hospital, Jeddah, Saudi Arabia
Saudi J Kidney Dis Transpl 20:854-7. 2009Steroid resistant nephrotic syndrome (SRNS) remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment...
- Management and outcome of steroid-resistant nephrotic syndrome in childrenHassan Otukesh
Department of Nephrology, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran
Iran J Kidney Dis 3:210-7. 2009Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes.
- Pharmacotherapeutic review and update of idiopathic nephrotic syndrome in childrenSilvia Manrique-Rodríguez
Pharmacy Department, Gregorio Maranon General University Hospital, Servicio de Farmacia, Madrid, Spain
Pharm World Sci 32:314-21. 2010The 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...
- Treatment of steroid resistant nephrotic syndrome in childrenJameela A Kari
Department of Pediatrics, King Abdul Aziz University Hospital, Jeddah, Saudi Arabia
Saudi J Kidney Dis Transpl 21:484-7. 2010Achieving remission in children with steroid-resistant nephrotic syndrome (SRNS) could be difficult. Many immunosuppressive drugs are used with variable success rates...
- Failure of regulation results in an amplified oxidation burst by neutrophils in children with primary nephrotic syndromeR Bertelli
Division of Nephrology and Laboratory on Pathophysiology of Uremia, Istituto Giannina Gaslini IRCCS, Genova, Italy
Clin Exp Immunol 161:151-8. 2010The 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...
- Genotype-phenotype correlations in non-Finnish congenital nephrotic syndromeEduardo Machuca
INSERM, U983, Hopital Necker Enfants Malades, Paris, France
J Am Soc Nephrol 21:1209-17. 2010Mutations 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 ..
- Effect of levamisole in steroid-dependent nephrotic syndromeAbbas Madani
Division of Pediatric Nephrology, Children Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran
Iran J Kidney Dis 4:292-6. 2010INTRODUCTION. Childhood idiopathic nephrotic syndrome is characterized by frequent relapsing courses or steroid dependency. Levamisole is a popular drug for treatment of these patients...
- Polymorphisms in interleukin-4-related genes in patients with minimal change nephrotic syndromeYuka Ikeuchi
Department of Pediatrics and Developmental Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, 371 8511, Japan
Pediatr Nephrol 24:489-95. 2009Minimal change nephrotic syndrome (MCNS) in children is frequently associated with allergy and immunoglobulin E production...
- Genetic models: clues for understanding the pathogenesis of idiopathic nephrotic syndromeCorinne Antignac
, , , , 75015 Paris, France
J Clin Invest 109:447-9. 2002
- Analysis of serum and urinary lysophospholipase D/autotaxin in nephrotic syndromeKazuhiro Nakamura
Clin Chem Lab Med 46:150-1. 2008
- Effect of glucocorticoid treatment on insulin like growth factor-I and its binding proteins in children with nephrotic syndromeFeng Dong
Department of Pediatrics, China-Japan Friendship Hospital, Beijing 100029, China
Chin Med J (Engl) 115:1383-5. 2002..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...
- Anti-oxidant vitamins and steroid responsive nephrotic syndrome in Indian childrenJ L Mathew
Departments of Paediatrics, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India
J Paediatr Child Health 38:450-37. 2002In recent years, it has been proposed that nephrotic syndrome is a consequence of an imbalance between oxidant and anti-oxidant activity...
- Nephrotic syndrome in a patient with intravascular lymphomatosisHaruko Kakumitsu
Department of Internal Medicine, Hamanomachi General Hospital, Fukuoka
Intern Med 42:98-101. 2003The 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...
- Proteinuria and fusion of podocyte foot processes in rats after infusion of cytokine from patients with idiopathic minimal lesion nephrotic syndromeEduardo H Garin
Department of Pediatrics, University of South Florida, Tampa, FL, USA
Nephron Exp Nephrol 102:e105-12. 2006We report on the isolation of a factor secreted by peripheral blood mononuclear cells from patients with idiopathic minimal lesion nephrotic syndrome (IMLNS) in relapse and its effect on proteinuria and podocyte morphology in the rat.
- Treatment of steroid-resistant nephrotic syndrome with cyclosporine: study of 17 cases and a literature reviewPaula Frassinetti Castelo Branco Camurça Fernandes
Division of Nephrology, School of Medicine, Walter Cantidio University Hospital, Federal University of Ceara, Fortaleza, Ceará Brazil
J Nephrol 18:711-20. 2005Cyclosporine (CsA) has been used in steroid-resistant idiopathic nephrotic syndrome (INS) in many previous studies.
- Vascular endothelial growth factor in children with nephrotic syndrome treated with cyclosporine AAnna Wasilewska
First Department of Paediatrics, Medical University of Bialystok, Bialystok, Poland
Acta Paediatr 95:291-6. 2006To assess the effect of cyclosporine A (CyA) on the level of vascular endothelial growth factor (VEGF) in the plasma and urine of nephrotic syndrome children.
- Adrenocortical suppression increases the risk of relapse in nephrotic syndromeAsiri S Abeyagunawardena
Department of Paediatrics, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka
Arch Dis Child 92:585-8. 2007Children with nephrotic syndrome (NS) are usually treated with long-term low dose alternate day prednisolone with or without glucocorticoid sparing therapy, such as levamisole or ciclosporin, to maintain remission...
- Is single-daily low-dose cyclosporine therapy really effective in children with idiopathic frequent-relapsing nephrotic syndrome?S Fujinaga
Division of Nephrology, Saitama Children s Medical Center, Saitama, Japan
Clin Nephrol 69:84-9. 2008..However, the efficacy of this regimen in children with idiopathic frequent-relapsing nephrotic syndrome (NS) remains controversial.
- Mycophenolate mofetil in children with frequently relapsing nephrotic syndrome: a report from the Southwest Pediatric Nephrology Study GroupRonald J Hogg
St Joseph s Hospital and Medical Center, Phoenix, AZ 85013, USA
Clin J Am Soc Nephrol 1:1173-8. 2006Children with frequently relapsing nephrotic syndrome (FRNS) often develop adverse effects from prednisone. Attempts to induce long-term remission in such patients have had varying levels of success...
- C1Q nephropathy in childrenTanja Kersnik Levart
Department of Pediatric Nephrology, University Medical Centre, Ljubljana, Slovenia
Pediatr Nephrol 20:1756-61. 2005..Eight children presented with nephrotic syndrome, while one had nephrotic proteinuria and renal insufficiency that progressed to end-stage renal failure...
- C1-C2 point monitoring of low-dose cyclosporin a given as a single daily dose in children with steroid-dependent relapsing nephrotic syndromeT Nakahata
Department of Pediatrics, Hirosaki University School of Medicine, Hirosaki, Japan
Clin Nephrol 64:258-63. 2005..drug than the conventional twice daily administration dosing used for the treatment of childhood idiopathic nephrotic syndrome (INS)...
- Recurrence of nephrotic syndrome after renal transplantation: influence of increased immunosuppressionSylvie Nathanson
Pediatric Nephrology Unit, , Paris, France
Pediatr Nephrol 20:1801-4. 2005Recurrence of nephrotic syndrome after renal transplantation leads to graft loss within 1 year in 50-80% of patients who do not receive any specific treatment...
- Tacrolimus: a new therapy for steroid-resistant nephrotic syndrome in childrenSanjeev Gulati
Department of Nephrology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
Nephrol Dial Transplant 23:910-3. 2008..The study group comprised of 22 consecutive children with steroid-resistant nephrotic syndrome (SRNS) who were studied prospectively. TAC was initiated with a dose of 0...
- High-dose mizoribine therapy for childhood-onset frequently relapsing steroid-dependent nephrotic syndrome with cyclosporin nephrotoxicityYoshiyuki Ohtomo
Department of Nephrology, Saitama Children s Medical Center, Saitama, Japan
Pediatr Nephrol 20:1744-9. 2005Cyclosporin A (CsA) is an effective treatment for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal...
- [Prognostic factors in children with lupus nephritis]Irena Wikiera-Magott
Wiad Lek 58:50-4. 2005..The correlation between the age of lupus onset, impaired renal function at the beginning of the disease, anemia, proteinuria, and poor renal outcome was not found...
- Circulating anti-actin and anti-ATP synthase antibodies identify a sub-set of patients with idiopathic nephrotic syndromeL Musante
Laboratory on Pathophysiology of Uremia, G. Gaslini Children Hospital, Genoa, Italy
Clin Exp Immunol 141:491-9. 2005Idiopathic nephrotic syndrome (iNS) with resistance or dependence to steroids is a common disease in children but in spite of an increasing clinical impact its pathogenesis is unknown...
- Oral mizoribine pulse therapy for patients with steroid-resistant and frequently relapsing steroid-dependent nephrotic syndromeYukihiko Kawasaki
Department of Pediatrics, School of Medicine, Fukushima Medical University, 1 Hikariga oka, Fukushima City, Fukushima 960 1295, Japan
Nephrol Dial Transplant 20:2243-7. 2005We investigated the efficacy of oral mizoribine pulse therapy (mizoribine-pulse) for cyclosporin (CyA)-dependent, steroid-resistant nephrotic syndrome (SRNS) and frequently relapsing, steroid-dependent nephrotic syndrome (FR-SDNS).
- Cyclosporin A therapy for severe Henoch-SchÃ¶nlein nephritis with nephrotic syndromeJae Il Shin
Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Seodaemun-ku, Seoul, Korea
Pediatr Nephrol 20:1093-7. 2005..efficacy of cyclosporin A (CyA) for treating severe Henoch-SchÃ¶nlein nephritis (HSN), seven patients with nephrotic syndrome, aged 3.9-13.8 years (mean 6.5 years), were analyzed retrospectively. Mean follow-up times were 5...
- Recurrence of proteinuria following renal transplantation in congenital nephrotic syndrome of the Finnish typeTarak Srivastava
Section of Nephrology, The Children s Mercy Hospital, 2401 Gillham Road, Kansas City, Missouri 64108, USA, and Department of Biochemistry, University of Oulu, Finland
Pediatr Nephrol 21:711-8. 2006We report a Caucasian boy of Italian descent with congenital nephrotic syndrome of the Finnish type (NPHS1, CNF, MIM 256300) who developed recurrence of proteinuria and hypoalbuminemia on the seventh post-operative day following living ..
- Initial treatment of idiopathic nephrotic syndrome in children: prednisone versus prednisone plus cyclosporine A: a prospective, randomized trialPeter F Hoyer
Department of Pediatric Nephrology, University Clinic Essen, Hufelandstrasse 55, D 45122 Essen, Germany
J Am Soc Nephrol 17:1151-7. 2006Previous studies of the Arbeitsgemeinschaft für Pädiatrische Nephrologie in children with steroid-sensitive nephrotic syndrome have shown that the length of initial prednisone therapy has an impact on the subsequent relapse rate...
- High-sensitivity C-reactive protein (hs-CRP) level in children with nephrotic syndromeAnna Wasilewska
1st Department of Pediatrics, Medical University of Białystok, Ul Waszyngtona 17, 15 274, Białystok, Poland
Pediatr Nephrol 22:403-8. 2007..study was to assess the serum concentration of high-sensitivity C-reactive protein (hs-CRP) in children with nephrotic syndrome (NS) treated with prednisone and cyclosporine A (CyA)...
- A prospective study on the use of mycophenolate mofetil in children with cyclosporine-dependent nephrotic syndromeShuichiro Fujinaga
Division of Nephrology, Saitama Children s Medical Center, 2100 Magome, Iwatsuki ku, Saitama, 339 8551, Japan
Pediatr Nephrol 22:71-6. 2007Cyclosporine A (CsA) has relieved children with steroid-dependent nephrotic syndrome (NS) from steroid toxicity...
- Follow-up of linear growth of body height in children with nephrotic syndromeYeh-Ting Hung
Department of Pediatrics, Section of Immunology and Nephrology, Taipei Veterans General Hospital, Taipei, Taiwan
J Microbiol Immunol Infect 39:422-5. 2006BACKGROUND AND PURPOSE: Steroid treatment is a mainstay in the management of nephrotic syndrome, but has the potential to impair growth and development of children...
- Association of steroid and cyclosporin resistance in focal segmental glomerulosclerosisIbrahim F Shatat
Pediatric Nephrology, Children s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY 10467, USA
Pediatr Nephrol 22:834-9. 2007Given the variable response of steroid-resistant nephrotic syndrome (SRNS) to treatment with cyclosporin (CsA), it may be inappropriate to expose all SR patients to additional immunosuppression...
- Efficacy of single dose of oral mizoribine pulse therapy two times per week for frequently relapsing nephrotic syndromeYukihiko Kawasaki
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima Japan
J Nephrol 20:52-6. 2007..single dose of oral mizoribine (MZB) pulse therapy two times per week for children with frequently relapsing nephrotic syndrome (FRNS)...
- Primary focal segmental glomerular sclerosis in children: clinical course and prognosisKyung Hoon Paik
Department of Pediatrics, Seoul National University Children s Hospital, South Korea
Pediatr Nephrol 22:389-95. 2007..4+/-42.4 months. The mean follow-up duration was 98.2+/-63.3 months. Eighty-five patients presented with nephrotic syndrome and seven presented with asymptomatic proteinuria...
- Idiopathic membranous nephropathy in childrenBeom Hee Lee
Department of Pediatrics, Seoul National University Children's Hospital, 28 Yongon-dong, Chongno-gu, Seoul, 110-744, South Korea
Pediatr Nephrol 21:1707-15. 2006Idiopathic membranous nephropathy (MN) is a rare cause of asymptomatic proteinuria (AP) or nephrotic syndrome (NS) in childhood...
- Urinary fructose-1,6-bisphosphatase activity as a marker of the damage to the renal proximal tubules in children with idiopathic nephrotic syndromeAlina Kepka
Department of Laboratory Diagnostics of the Institute Pomnik Centrum Zdrowia Dziecka, Warsaw, Poland
Clin Chem Lab Med 46:831-5. 2008..The aim of our present research was to determine FBP-1 activity in urine as an indicator of damage to renal proximal tubules in children with idiopathic nephrotic syndrome (INS).
- FK506 in the treatment of children with nephrotic syndrome of different pathological typesZ Xia
Department of Pediatrics, Jinling Hospital, Nanjing University School of Medicine, 305 East Road ZhongShan, Nanjing 210002, PR China
Clin Nephrol 66:85-8. 2006To evaluate the efficacy of FK506 in the treatment of children with nephrotic syndrome of different underlying pathology.
- Effect of oral mizoribine pulse therapy for frequently relapsing steroid-dependent nephrotic syndromeM Fujieda
Department of Pediatrics, Kochi University Medical School, Kochi, Japan
Clin Nephrol 69:179-84. 2008To evaluate the efficacy of oral mizoribine (MZB) pulse therapy given twice a week for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS).
- Response to prednisone in relation to NR3C1 intron B polymorphisms in childhood nephrotic syndromeGrzegorz Zalewski
Department of Clinical Molecular Biology, Medical University of Białystok, Białystok, Poland
Pediatr Nephrol 23:1073-8. 2008The variation in time required to obtain cessation of proteinuria in children with nephrotic syndrome (NS) represents one aspect of the variations shown by these children in response to glucocorticoid (GC) treatment...
- Comparison of pulse and oral steroid in childhood membranoproliferative glomerulonephritisElif Bahat
Department of Pediatric Nephrology, Karadeniz Technical University, Trabzon, Turkey
J Nephrol 20:234-45. 2007..This study aimed to compare these therapies and renal outcome over a long-term period for MPGN...
- Granulomatous interstitial nephritis treated with a tumour necrosis factor-alpha inhibitorLaurie Tomlinson
Brighton and Sussex Medical School, University of Sussex, Falmer, Sussex, BN 9PX, UK
Nephrol Dial Transplant 21:2311-4. 2006
- Long-term follow-up after cyclophosphamide and cyclosporine-A therapy in steroid-dependent and -resistant nephrotic syndromeViktória Sümegi
Department of Paediatrics, University of Szeged, Faculty of Medicine, Koranyi fasor 14 15, Szeged, Hungary
Pediatr Nephrol 23:1085-92. 2008A 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)...
- Angiotensin-converting enzyme gene polymorphism in children with idiopathic nephrotic syndromeI Jung Tsai
Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
Am J Nephrol 26:157-62. 2006To investigate the genetic polymorphism of angiotensin-converting enzyme (ACE) insertion/deletion (I/D) in children with idiopathic nephrotic syndrome (INS), as well as its relationship with patient's clinical response to steroid therapy.
- Remission of steroid- and CyA-resistant nephrotic syndrome using multiple drug immunosuppressionTim Ulinski
Department of Pediatric Nephrology and INSERM U515, Hopital Armand Trousseau, AP HP, University Paris VI, 26, Avenue du Docteur Netter, 75571 Paris, France
Pediatr Nephrol 22:1723-6. 2007..Three children (2 years, 3 years, and 6 years of age) with secondary steroid-resistant nephrotic syndrome (NS) were included, as they remained resistant to 30 days of treatment with prednisone (60 mg/m(2) per day), ..
- Efficacy of steroids, cyclosporin and cyclophos-phamide in steroid resistant idiopathic nephrotic syndromeFarkhanda Hafeez
Department of Paediatric Nephrology, The Children s Hospital and Institute of Child Health, Lahore
J Coll Physicians Surg Pak 15:329-32. 2005To determine the efficacy of three different treatment protocols in steroid resistant idiopathic nephrotic syndrome, (SRINS).
- [Membranous nephropathy]Elsa Martin Passos
, , CHU Rouen, 76031 Rouen Cedex
Rev Prat 53:2033-8. 2003Membranous nephropathy is the most common cause of idiopathic nephrotic syndrome in adults. The frequency of secondary forms varies from 20 to 30 per cent...
- Use of mycophenolate mofetil in steroid-dependent and -resistant nephrotic syndromeGina Marie Barletta
University of Michigan Health Sciences Center, Mott Children s Hospital, 1505 Simpson Road East, F6865 Box 0297, Ann Arbor, MI 48109 0297, USA
Pediatr Nephrol 18:833-7. 2003Cyclosporin (Cs-A) is an effective treatment for difficult cases of nephrotic syndrome (NS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal...
- Lipid peroxidation in human proteinuric diseaseM L Solin
The Haartman Institute, Division of Bacteriology and Immunology, University of Helsinki, Helsinki, Finland
Kidney Int 59:481-7. 2001..METHODS: Here we studied kidneys from patients with congenital nephrotic syndrome of the Finnish type (CNF), a human model disease of isolated proteinuria...
- Immunosuppressive agents in childhood nephrotic syndrome: a meta-analysis of randomized controlled trialsA M Durkan
Centre for Kidney Research, The Children's Hospital at Westmead, New South Wales, Australia
Kidney Int 59:1919-27. 2001BACKGROUND: Many children with steroid-sensitive nephrotic syndrome (SSNS) relapse frequently and receive immunosuppressive agents...
- Fibrotic Sequelae of Childhood Renal DiseaseAgnes Fogo; Fiscal Year: 2007..Kidney Int 58:1219-1227, 2000. Fogo A: Nephrotic syndrome: molecular and genetic basis. Nephron 85:8-13, 2000...
- Protein Misprocessing in Krabbe DiseaseChristopher 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...
- Slit Diaphragm and Actin DynamicsLawrence 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 ..
- Slit Diaphragm and Actin DynamicsLawrence 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 ..
- Slit Diaphragm and Actin DynamicsLawrence 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 ..
- BASIS FOR RENAL RESISTANCE TO ATRIAL NATRIURETIC PEPTIDEMichael Humphreys; Fiscal Year: 2004DESCRIPTION: 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 ..
- LAMININ BETA2 AND GLOMERULAR FILTRATIONJEFFREY 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 ..
- LAMININ BETA2 AND GLOMERULAR FILTRATIONJEFFREY 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 ..
- LAMININ BETA2 AND GLOMERULAR FILTRATIONJeffrey 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 ..
- Podocyte Protein Alterations Induced by GlucocorticoidsRichard Ransom; Fiscal Year: 2005b>Nephrotic syndrome (NS) is one of the most common kidney diseases seen in children...
- Membranous Nephritis Antigen Defined by Phage AntibodiesSudesh 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...
- Genetics of Renal End Organ Damage in HypertensionMichael 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...
- Genetics of Renal End Organ Damage in HypertensionMichael 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...
- Treatment of Diabetic NephropathyJulia 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 ..
- Proteolytic Regulation of Podocyte Function by Cathepsin LJochen 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...
- Proteolytic Regulation of Podocyte Function by Cathepsin LJochen 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...
- GLUCOCORTICOID-INDUCED OSTEOPENIA IN CHILDRENMary 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...
- Proteolytic Regulation of Podocyte Function by Cathepsin LJochen 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...