Database: Ovid MEDLINE(R) <2006 to March Week 3 2010>
Search Strategy:
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1 *nephrotic syndrome/ (812)
2 limit 1 to (english language and "all child (0 to 18 years)") (400)
3 limit 2 to "review articles" (30)
4 from 3 keep 1-30 (30)
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<1>
Unique Identifier
19410518
Status
MEDLINE
Authors
Bruneau S. Dantal J.
Authors Full Name
Bruneau, Sarah. Dantal, Jacques.
Institution
INSERM, U643, Nantes, F44093, France.
Title
New insights into the pathophysiology of idiopathic nephrotic syndrome. [Review] [80 refs]
Source
Clinical Immunology. 133(1):13-21, 2009 Oct.
Abstract
Corticoresistant idiopathic nephrotic syndrome (INS) is a glomerulopathy of unknown etiology whose original aspect is its recurrence after kidney transplantation in 30 to 50% of patients with end-stage renal disease. This suggests the involvement of circulating factors that would alter the glomerular filtration barrier, but whose nature remains elusive. Although a T cell immune origin has been suggested, the actual role of these cells in INS recurrence is still unclear. Here we present an 8-year-old patient with corticoresistant INS who developed a recurrence of her initial disease after kidney transplantation. Rituximab therapy was proposed 11 months after transplantation; although no immediate effect was induced, a slow but persistent decrease in proteinuria began a few months after Rituximab infusions despite cessation of plasma exchanges and steroid therapy. The pathophysiology of INS and the putative mechanisms of action of Rituximab are discussed. [References: 80]
Publication Type
Case Reports. Journal Article. Research Support, Non-U.S. Gov't. Review.
<2>
Unique Identifier
19169768
Status
MEDLINE
Authors
Khaira A. Upadhyay BK. Sharma A. Das P. Mahajan S. Makhariya G. Dinda AK. Agarwal SK. Tiwari SC.
Authors Full Name
Khaira, Ambar. Upadhyay, Bala Krishna. Sharma, Alok. Das, Prasenjit. Mahajan, Sandeep. Makhariya, Govind. Dinda, Amit K. Agarwal, Sanjay K. Tiwari, Suresh C.
Institution
Department of Nephrology, All India Institute of Medical Sciences, New Delhi, India.
Title
Hepatitis B virus associated focal and segmental glomerular sclerosis: report of two cases and review of literature. [Review] [17 refs]
Source
Clinical & Experimental Nephrology. 13(4):373-7, 2009 Aug.
Abstract
The hepatitis B virus (HBV) is estimated to have infected about 350 million people worldwide, making it one of the most common human pathogens. Renal involvement is among its most common extra hepatic manifestations and usually manifests in the form of immune complex mediated glomerulopathy, such as membranous glomerulonephritis (MGN), membranoproliferative glomerulonephritis (MPGN), mesangioproliferative glomerulonephritis and immunoglobulin A (IgA) nephropathy. Occurrence of focal and segmental glomerular sclerosis (FSGS) with HBV infection is rare and only five cases have been reported earlier. We report two cases of hepatitis B associated FSGS. In both the cases, HBsAg was demonstrated in the renal tissue and both the cases showed response to treatment with lamivudine, thus indicating a possible causal association between the viral infection and occurrence of nephrotic syndrome. [References: 17]
Publication Type
Case Reports. Journal Article. Review.
<3>
Unique Identifier
19615560
Status
MEDLINE
Authors
Lane JC. Kaskel FJ.
Authors Full Name
Lane, Jerome C. Kaskel, Frederick J.
Institution
Division of Kidney Diseases, Department of Pediatrics, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614, USA.
Title
Pediatric nephrotic syndrome: from the simple to the complex. [Review] [40 refs]
Source
Seminars in Nephrology. 29(4):389-98, 2009 Jul.
Abstract
Remarkable advances have been made in the past decade in understanding the pathophysiology of idiopathic nephrotic syndrome. Although the initiating events leading to the onset of proteinuria still are not well defined, it has become increasingly clear that many glomerular diseases can be classified as podocytopathies, with injury to the podocyte playing a major role in the development and progression of disease. A complex interaction of immune system mediators, slit diaphragm signal transduction, podocyte injury and conformational change, and mediators of apoptosis and fibrosis determine the extent and nature of proteinuria and progression of glomerulosclerosis. New insights into the pathogenesis of idiopathic nephrotic syndrome likely will lead to innovative therapies and new approaches to management and prevention. [References: 40]
Publication Type
Journal Article. Review.
<4>
Unique Identifier
19606070
Status
MEDLINE
Authors
Bramham K. Hunt BJ. Goldsmith D.
Authors Full Name
Bramham, Kate. Hunt, Beverley J. Goldsmith, David.
Institution
Biomedical Research Centre, Guy's and St Thomas' Foundation Hospitals, London SE1 9RT, United Kingdom.
Title
Thrombophilia of nephrotic syndrome in adults. [Review] [72 refs]
Source
Clinical Advances in Hematology & Oncology. 7(6):368-72, 2009 Jun.
Publication Type
Journal Article. Review.
<5>
Unique Identifier
19495800
Status
MEDLINE
Authors
Haffner D. Fischer DC.
Authors Full Name
Haffner, Dieter. Fischer, Dagmar-Christiane.
Title
Nephrotic syndrome and rituximab: facts and perspectives. [Review] [25 refs]
Source
Pediatric Nephrology. 24(8):1433-8, 2009 Aug.
Abstract
Idiopathic nephrotic syndrome is the most frequent glomerular disease that presents during childhood and is mainly due to minimal change nephropathy (MCNS) and focal-segmental glomerulosclerosis (FSGS). Its treatment is still challenging, with up to 50% of the patients who are initially steroid sensitive (usually MCNS) being frequent relapsers and requiring additional long-term immunosuppression. However, current immunosuppressive regimens are associated with severe toxicity. Only half of the steroid-resistant patients (usually FSGS) achieve long-term remission even with intensive immunosuppression and plasma exchange. Rituximab (RTX), a chimeric monoclonal antibody inhibiting CD20-mediated B-cell proliferation and differentiation, has recently gained attention as a potentially successful therapy for complicated idiopathic nephrotic syndrome in children. A number of case reports and one prospective non-controlled multicenter trial point to the beneficial effects of RTX as a rescue therapy in children with steroid/cyclosporine-dependent or -resistant nephrotic syndrome. However, publication bias often results in positive outcomes being more likely to be reported than negative ones and, in particular, the safety profile of this drug in this group of patients remains unclear. Therefore, controlled randomized studies are required to assess this issue, to develop treatment guidelines, to evaluate the therapeutic and economical efficacy, and to define criteria for the selection of patients. [References: 25]
Publication Type
Editorial. Review.
<6>
Unique Identifier
19052471
Status
MEDLINE
Authors
Wang DY. Mao JH. Zhang Y. Gu WZ. Zhao SA. Chen YF. Liu AM.
Authors Full Name
Wang, D Y. Mao, J H. Zhang, Y. Gu, W Z. Zhao, S A. Chen, Y F. Liu, A M.
Institution
The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China.
Title
Kimura disease: a case report and review of the Chinese literature. [Review] [26 refs]
Source
Nephron. 111(1):c55-61, 2009.
Abstract
BACKGROUND: Kimura disease, often accompanied by nephrotic syndrome, is a rare, chronic inflammatory disorder of unknown cause. In this report, the clinical and histopathological characteristics of 20 Chinese patients with Kimura disease-associated nephrotic syndrome were retrospectively evaluated. METHODS: We report a case of Kimura disease that was diagnosed recently in our ward, with steroid-responsive but recurrent minimal-change nephrotic syndrome. Meanwhile, we also used three powerful Chinese journal search engines (Cqvip.com, Wanfang.data and ScienceChina) to search the cases reported in Chinese from 1984 to 2007. RESULTS: The nephrotic syndrome of our patient occurred 20 months after the onset of Kimura disease. Renal biopsy revealed minimal-change lesions. The patient was responsive to the steroid, but proteinuria recurred. In most of the 19 other cases, the onset of nephrotic syndrome occurred after subcutaneous masses. Renal biopsy in 13 cases showed mesangial proliferative glomerulonephritis in 9, minimal change disease in 2 and membrane nephropathy in 2 cases. Serum creatinine levels were elevated in 5 patients. CONCLUSION: Normally, Kimura disease-associated nephrotic syndrome patients are sensitive to prednisone therapy but are likely to relapse. In patients with recurrent nephrotic syndrome, renal insufficiency is not uncommon. Copyright 2008 S. Karger AG, Basel. [References: 26]
Publication Type
Case Reports. Journal Article. Research Support, Non-U.S. Gov't. Review.
<7>
Unique Identifier
19158142
Status
MEDLINE
Authors
Hasan F.
Authors Full Name
Hasan, Fyeza.
Institution
Molecular Haematology and Cancer Biology Unit, Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK.
Title
Towards evidence based medicine for paediatricians. Does daily prednisolone reduce the risk of relapse secondary to viral infections in steroid-dependent nephrotic syndrome?. [Review] [4 refs]
Source
Archives of Disease in Childhood. 94(2):168-9, 2009 Feb.
Publication Type
Journal Article. Review.
<8>
Unique Identifier
18937561
Status
MEDLINE
Authors
Traum AZ.
Authors Full Name
Traum, Avram Z.
Institution
Harvard Medical School, Boston, MA, USA.
Title
Urine proteomic profiling to identify biomarkers of steroid resistance in pediatric nephrotic syndrome. [Review] [29 refs]
Source
Expert Review of Proteomics. 5(5):715-9, 2008 Oct.
Abstract
Long-term prognosis for children with nephrotic syndrome (NS) is directly related to steroid responsiveness. There are currently no diagnostic tests that accurately predict steroid responsiveness in pediatric NS. The initial prolonged course of daily, high-dose corticosteroid therapy thus serves both as a diagnostic and therapeutic maneuver. Urine proteomics is emerging as a potentially rich source of noninvasive biomarkers of drug responsiveness in NS. In this article, we discuss some of the initial studies of the urinary proteome in NS as well as ongoing and future challenges, define the normal urinary proteome and address the overwhelming abundance of urinary albumin and its impact on biomarker discovery. [References: 29]
Publication Type
Journal Article. Research Support, Non-U.S. Gov't. Review.
<9>
Unique Identifier
18462046
Status
MEDLINE
Authors
Liapis H.
Authors Full Name
Liapis, Helen.
Institution
Department of Pathology & Immunology, Washington University School of Medicine, St Louis, MO 63110, USA.
Title
Molecular pathology of nephrotic syndrome in childhood: a contemporary approach to diagnosis. [Review] [55 refs]
Source
Pediatric & Developmental Pathology. 11(4):154-63, 2008 Jul-Aug.
Abstract
Molecular and genetic studies in the last 2 decades have shed new light on the understanding of congenital and infantile nephrotic syndrome (NS). Glomerular pathology may appear as minimal change disease, focal segmental glomerulosclerosis, or diffuse mesangial sclerosis, glomerular diseases now recognized as podocyte injuries and in part caused by altered podocyte genes. Even though genetic mutations are not implicated in all infants with NS, the study of familial disease and congenital NS reveals that proteinuria is in many patients due to specific gene mutations. The most common mutations are in 4 genes, 3 of which are podocyte genes: NPHS1 (Finnish nephropathy), NPHS2 (podocin-induced focal segmental glomerulosclerosis), WT1 (diffuse mesangial sclerosis), and LAMB2 (Pierson syndrome). Furthermore, these studies have improved our understanding of steroid-resistant NS in older children, particularly girls, in whom proteinuria may be due to WT1 mutations. Availability of molecular genetic testing and antibodies to specific gene products are closing the gap between histopathology of pediatric glomerular disease and molecular genetic diagnosis. Recognition of NS variants, which may be reversible (eg, mitochondrial mutations, viral disease), is important. This review discusses the most common entities and the differential diagnosis of pediatric NS from the pathologist's point of view, with an emphasis on congenital (<3 months) and infantile (3 months to 1 year) NS in light of molecular and genetic studies. [References: 55]
Publication Type
Journal Article. Review.
<10>
Unique Identifier
18425948
Status
MEDLINE
Authors
Yuan W. Wang J. Wu T.
Authors Full Name
Yuan, W. Wang, J. Wu, T.
Title
Chinese herbal medicine Huangqi type formulations for nephrotic syndrome. [Review] [202 refs]
Source
Cochrane Database of Systematic Reviews. (2):CD006335, 2008.
Abstract
BACKGROUND: At present, there is a lack of safe and effective drugs for nephrotic syndrome (NS). Huangqi type formulations have been used to treat nephrotic syndrome for years in China, however the effects and safety of these formulations have not been systematically reviewed. OBJECTIVES: To assess the benefits and harms of Huangqi and Huangqi type formulations in treating NS in any age group, either as sole agents or in addition to other drug therapies. SEARCH STRATEGY: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, Chinese Biomedicine Database (CBM), CNKI, VIP and reference lists of articles. There was no language restriction. Date of most recent search: June 2006. SELECTION CRITERIA: All randomised controlled trials (RCTs) assessing the use of Huangqi or Huangqi type formulations in treating NS in adults and children, either as sole agents or in addition to other drug therapies. DATA COLLECTION AND ANALYSIS: Two authors independently assessed study quality and extracted data. For dichotomous outcomes (remission, side effects and Inefficacy rate), results were expressed as relative risk (RR) and 95% confidence intervals (CI). Continuous outcomes (triglycerides cholesterol, plasma albumin) results were expressed as mean difference (WMD) with 95% CI. MAIN RESULTS: Three studies were identified (n = 128), all comparing Huangqi type formulations with placebo. Huangqi injection had a positive effect on plasma albumin (WMD 6.90, 95% Cl 3.60 to 10.20) and cholesterol (WMD 2.13, 95% Cl -2.97 to -1.29). Huangqi and red Chinese date reduced some adverse reactions (Cushing's syndrome: RR 0.55, 95% Cl 0.32 to 0.94; hormone reduced syndrome: RR 0.58, 95% Cl 0.39 to 0.85, respiratory tract infection: RR 0.27, 95% Cl 0.08 to 0.88), but no benefit on reducing relapse. Huangqi and Danggui had a positive effect on cholesterol (WMD -0.85, 95% Cl -1.70 to 0.00). AUTHORS' CONCLUSIONS: Huangqi type formulations may have some positive effects in treating NS by increasing plasma albumin and reducing blood cholesterol, Cushing's syndrome, hormone reduced syndrome and respiratory tract infection. However, limited by the lack of high quality clinical studies, we are unable to recommend Huangqi type formulations for NS. Large, properly randomised, placebo-controlled, double-blind studies are required. [References: 202]
Publication Type
Journal Article. Meta-Analysis. Review.
<11>
Unique Identifier
18401158
Status
MEDLINE
Authors
Candiano G. Musante L. Petretto A. Bruschi M. Santucci L. Urbani A. Scolari F. Gusmano R. Carraro M. Zennaro C. Vincenti F. Ghiggeri GM.
Authors Full Name
Candiano, Giovanni. Musante, Luca. Petretto, Andrea. Bruschi, Maurizio. Santucci, Laura. Urbani, Andrea. Scolari, Francesco. Gusmano, Rosanna. Carraro, Michele. Zennaro, Cristina. Vincenti, Flavio. Ghiggeri, Gian Marco.
Institution
Laboratory on Pathophysiology of Uremia, G. Gaslini Children Hospital, Genoa, Italy.
Title
Proteomics of plasma and urine in primary nephrotic syndrome in children. [Review] [61 refs]
Source