Database: Ovid MEDLINE(R) <2006 to March Week 3 2010>
Search Strategy:
-------------------------------------------------------------------------------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)
***************************
<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. j-lane@northwestern.edu
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 diseaseassociated 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. fyezahasan@hotmail.com
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. atraum@partners.org
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. liapis@path.wustl.edu
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
Contributions to Nephrology. 160:17-28, 2008.
Abstract
Primary nephrotic syndrome in children, especially the variant with segmental
glomerulosclerosis, remains an unsolved clinical problem. In spite of some progress, its
pathogenesis is still unknown and the therapy options are confined to gross immune modulation.
Indirect evidence based on posttransplant recurrence of the disease suggested an implication of
plasma factors, whose characterization remains in course. Besides historical candidates, research
is now considering glyco- and lipoderivatives. Structural analysis of plasma and urinary proteins
based on proteomics has recently shown an increased proteolysis of major components such as
albumin and the implication of alpha 1-antitrypsin that represents the first-line defense against
exogenous and endogenous substances with proteolytic activity. Albumin has also emerged as a
major plasma antioxidant, and recent studies have demonstrated that in patients with active
focal segmental glomerulosclerosis albumin undergoes massive and stable oxidation with
sulfonation of Cys34, formation of an adduct with +48 Da molecular weight, changes of the net
charge due to additional negative residues, and loss of free thiol group (SH) titration. Altogether,
these data suggest that oxidative stress determines selective protein damages in focal segmental
glomerulosclerosis patients with formation of new adducts and fragmentation of plasma proteins.
Research should now address whether oxidation of podocyte proteins is important for the
maintenance of renal selectivity and is involved in proteinuria. [References: 61]
Publication Type
Journal Article. Research Support, Non-U.S. Gov't. Review.
<12>
Unique Identifier
18332710
Status
MEDLINE
Authors
Del Rio M. Kaskel F.
Authors Full Name
Del Rio, Marcela. Kaskel, Frederick.
Institution
Division of Pediatric Nephrology, Children's Hospital at Montefiore, Bronx, New York 10467,
USA. mdelrio@montefiore.org
Title
Evaluation and management of steroid-unresponsive nephrotic syndrome. [Review] [59 refs]
Source
Current Opinion in Pediatrics. 20(2):151-6, 2008 Apr.
Abstract
PURPOSE OF REVIEW: Idiopathic nephrotic syndrome in children is commonly associated
with minimal change disease and response to steroid therapy. Steroid-unresponsive nephrotic
syndrome is often characterized by persistent proteinuria and progression to chronic kidney
disease. Focal segmental glomerulosclerosis is the leading cause of steroid-unresponsive nephrotic
syndrome in childhood. There is no uniformed consensus as to the treatment of steroidunresponsive nephrotic syndrome. Advances in the pathogenesis, genetics and biomarkers or
surrogate markers may be useful for the diagnosis and identification of patients with steroidunresponsive nephrotic syndrome, severity of disease, progression and response to therapy.
RECENT FINDINGS: This review is intended to describe some of the recent changes in the
epidemiology of steroid-unresponsive nephrotic syndrome, in particular focal segmental
glomerulosclerosis, its pathogenesis and alternative therapies. SUMMARY: Recent studies in
both children and adults have shown an increase in the incidence of focal segmental sclerosis as a
cause of steroid-unresponsive nephrotic syndrome. Advances in the pathogenesis and
noninvasive methods of diagnosis may allow for the identification of steroid-responsive patients.
[References: 59]
Publication Type
Journal Article. Research Support, N.I.H., Extramural. Review.
<13>
Unique Identifier
18332709
Status
MEDLINE
Authors
Hodson EM. Alexander SI.
Authors Full Name
Hodson, Elisabeth M. Alexander, Stephen I.
Institution
Centre for Kidney Research, The Children's Hospital at Westmead, Westmead, NSW,
Australia. Elisah@chw.edu.au
Title
Evaluation and management of steroid-sensitive nephrotic syndrome. [Review] [56 refs]
Source
Current Opinion in Pediatrics. 20(2):145-50, 2008 Apr.
Abstract
PURPOSE OF REVIEW: This review examines new literature published in 2006 and 2007 on
steroid-sensitive nephrotic syndrome. RECENT FINDINGS: Steroid-sensitive nephrotic
syndrome has long been thought to be due to lymphocyte-derived circulating factors leading to
podocyte injury with subsequent proteinuria. New studies support this mechanism and implicate
the T helper 2 cytokine IL-13. In addition a genetic mutation in familial nephrotic syndrome has
been reported in a child, who responded to corticosteroid therapy. There are new clinical trial
data supporting the efficacy of levamisole in steroid-sensitive nephrotic syndrome and
preliminary trial data on mycophenolate mofetil supporting its efficacy as a steroid-sparing
agent. Case reports support the use of the B cell-depleting antibody rituximab in steroid-sensitive
nephrotic syndrome. Finally there is a meta-analysis of six studies suggesting an increase in the
incidence of focal and segmental glomerulosclerosis in steroid-sensitive nephrotic syndrome over
the last 20 years. SUMMARY: Progress has been made towards elucidating the cause of steroidsensitive nephrotic syndrome. Data from adequately powered randomized controlled trials are
still required to evaluate therapies for frequently relapsing and steroid-dependent steroidsensitive nephrotic syndrome. [References: 56]
Publication Type
Journal Article. Review.
<14>
Unique Identifier
18304155
Status
MEDLINE
Authors
Shah KN. Yan AC.
Authors Full Name
Shah, Kara N. Yan, Albert C.
Institution
Pediatric Dermatology, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Title
Acquired zinc deficiency acrodermatitis associated with nephrotic syndrome. [Review] [25 refs]
Source
Pediatric Dermatology. 25(1):56-9, 2008 Jan-Feb.
Abstract
We present a child with new-onset nephrotic syndrome, acrodermatitis, low serum zinc levels
and decreased serum alkaline phosphatase. A diagnosis of acquired zinc deficiency
acrodermatitis was made. Oral zinc supplementation led to rapid clinical resolution. The etiology
of zinc deficiency in nephrotic syndrome remains unknown. [References: 25]
Publication Type
Case Reports. Journal Article. Review.
<15>
Unique Identifier
18254005
Status
MEDLINE
Authors
Hodson EM. Willis NS. Craig JC.
Authors Full Name
Hodson, E M. Willis, N S. Craig, J C.
Institution
Children's Hospital at Westmead, Centre for Kidney Research, Locked Bag 4001, Westmead,
NSW, Australia, 2145. Elisah@chw.edu.au
Title
Non-corticosteroid treatment for nephrotic syndrome in children. [Review] [96 refs][Update of
Cochrane Database Syst Rev. 2005;(2):CD002290; PMID: 15846634]
Source
Cochrane Database of Systematic Reviews. (1):CD002290, 2008.
Abstract
BACKGROUND: Eighty to 90% of children with steroid-sensitive nephrotic syndrome (SSNS)
have relapses. About half relapse frequently and are at risk of the adverse effects of
corticosteroids. Non-corticosteroid immunosuppressive agents are used to prolong periods of
remission in these children, however these agents have significant potential adverse effects.
Currently there is no consensus as to the most appropriate second line agent in children who are
steroid sensitive, but who continue to relapse. OBJECTIVES: To evaluate the benefits and harms
of non-corticosteroid immunosuppressive agents in relapsing SSNS in children. SEARCH
STRATEGY: We searched the Cochrane Central Register of Controlled Trials (CENTRAL),
MEDLINE, EMBASE, reference lists, conference abstracts and contact with known
investigators. Search date: September 2007 SELECTION CRITERIA: Randomised controlled
trials (RCTs) or quasi-RCTs were included if they compared non-corticosteroid agents with
placebo, prednisone or no treatment, different doses and/or durations of the same noncorticosteroid agent, different non-corticosteroid agents. DATA COLLECTION AND
ANALYSIS: Two authors independently assessed study quality and extracted data. Statistical
analyses were performed using a random effects model and results expressed as relative risk
(RR) with 95% confidence intervals (CI). MAIN RESULTS: We identified 26 studies (1173
children). Cyclophosphamide (RR 0.44, 95% CI 0.26 to 0.73) and chlorambucil (RR 0.15, 95% CI
0.02 to 0.95) significantly reduced the relapse risk at six to twelve months compared with
prednisone alone. There was no difference in relapse risk at two years between chlorambucil and
cyclophosphamide (RR 1.31, 95% CI 0.80 to 2.13). There was no difference at one year between
intravenous and oral cyclophosphamide (RR 0.99, 95% CI 0.76 to 1.29). Cyclosporin was as
effective as cyclophosphamide (RR 1.07, 95% CI 0.48 to 2.35) and chlorambucil (RR 0.82, 95%
CI 0.44 to 1.53) and levamisole (RR 0.43, 95% CI 0.27 to 0.68) was more effective than steroids
alone but the effects were not sustained once treatment was stopped. There was no difference in
the risk for relapse between mycophenolate mofetil and cyclosporin (RR 5.00, 95% CI 0.68 to
36.66) but CI were large. Mizoribine and azathioprine were no more effective than placebo or
prednisone alone in maintaining remission. AUTHORS' CONCLUSIONS: Eight week courses of
cyclophosphamide or chlorambucil and prolonged courses of cyclosporin and levamisole reduce
the risk of relapse in children with relapsing SSNS compared with corticosteroids alone.
Clinically important differences in efficacy are possible and further comparative studies are still
needed. [References: 96]
Publication Type
Journal Article. Meta-Analysis. Review.
<16>
Unique Identifier
18245896
Status
MEDLINE
Authors
Chiu MC.
Authors Full Name
Chiu, Man-chun.
Institution
Paediatric Nephrology Center, Department of Paediatrics and Adolescent Medicine, Princess
Margaret Hospital, Hong Kong. chiumc@ha.org.hk
Title
Management strategy for idiopathic nehprotic syndrome in children. [Review] [20 refs]
Source
Zhong Nan da Xue Xue Bao. Yi Xue Ban = Journal of Central South University. Medical
Sciences. 33(1):1-7, 2008 Jan.
Abstract
Corticosteroid, alkylating agents, like cyclophosphamide and chlorambucil, have been used to
treat idiopathic nephrotic syndrome for more than fifty years, changing the outcome of these
children. However, with long-term use of steroid, especially high dosages, they have unbearable
side effects. Newer agents like cyclosporine A, levamisole, tacrolimus, mycophenolate mofetil,
have been used to spare those unwanted side effects. In the choice of drugs, the benefits obtained
will have to be evaluated against possible side effects, with drug cost also taken into
consideration. Though most steroid sensitive nephrotic children may run a relapsing course, have
a good prognosis with many becoming non-relapsers or infrequent relapsers in adulthood, the
treatment approach should aim at using the minimal amount of drug required to keep patient in
remission to tie them over childhood. As for steroid resistant nephrotic syndrome children,
especially for focal segmental glomerulosclerosis (FSGS), because of possible grave prognosis of
going into end-stage renal failure, more aggressive approach should be adopted, including the use
of strong immunosuppressants, such as, cyclosporine, tacrolimus, or mycophenolate mofetil if
necessary. The long-term goals of treatment, other than those of physical and medical conditions,
should also consider the growth, education, and psychological impact of the disease and side
effects of drugs on the child, especially during an adolescent period, so as to allow them having
normal development into adulthood. [References: 20]
Publication Type
Journal Article. Review.
<17>
Unique Identifier
18162004
Status
MEDLINE
Authors
Ehrich JH. Pape L. Schiffer M.
Authors Full Name
Ehrich, Jochen H H. Pape, Lars. Schiffer, Mario.
Institution
Department of Pediatric Nephrology, Hannover Medical School, Hannover, Germany.
ehrich.jochen@mh-hannover.de
Title
Corticosteroid-resistant nephrotic syndrome with focal and segmental glomerulosclerosis : an
update of treatment options for children. [Review] [142 refs]
Source
Paediatric Drugs. 10(1):9-22, 2008.
Abstract
Corticosteroid-resistant nephrotic syndrome (CRNS) with focal and segmental
glomerulosclerosis (FSGS) is a heterogeneous disorder and the most severe and frequent type of
all glomerulopathies in children leading to end-stage renal failure. The podocyte is at the center
of development and progress of FSGS; this unique cell type plays a major role in the integrity of
glomerular structure and permeability. The rate of complete remission of CRNS after induction
therapy using different immunosuppressant agents is reported to range between 30% and 84%,
depending on the treatment schedule and on the underlying defects of FSGS. Children with
genetic types of FSGS barely respond to immunosuppressant therapies and over-treatment prior
to transplantation should be avoided. The response of children with an idiopathic type of FSGS
to immunosuppressants is superior to those with genetic FSGS. However, many children with
idiopathic FSGS do not enter complete remission if they are under-treated, for example, with
short-term immunosuppressant monotherapies. If immunosuppressant treatment fails, these
patients will have to undergo renal transplantation. However, as unknown pathogenetic
mechanisms may persist, more than one-third of these patients with idiopathic FSGS develop a
rapid recurrence of CRNS that responds poorly to further long-term therapeutic attempts. In
contrast with previously published data, this review takes into account recently identified genetic
etiologies of CRNS, and superior results with long-term combination therapy in idiopathic forms
to avoid over- and under-treatment. [References: 142]
Publication Type
Journal Article. Review.
<18>
Unique Identifier
18060371
Status
MEDLINE
Authors
Ciszak L. Pawlak E. Kosmaczewska A. Potoczek S. Frydecka I.
Authors Full Name
Ciszak, Lidia. Pawlak, Edyta. Kosmaczewska, Agata. Potoczek, Stanislaw. Frydecka, Irena.
Institution
Laboratory of Immunopathology, Department of Experimental Therapy, Institute of
Immunology and Experimental Therapy, Polish Academy of Sciences, R. Weigla 12, 53-114,
Wroclaw, Poland. ciszak@iitd.pan.wroc.pl
Title
Alterations in the expression of signal-transducing CD3 zeta chain in T cells from patients with
chronic inflammatory/autoimmune diseases. [Review] [114 refs]
Source
Archivum Immunologiae et Therapiae Experimentalis. 55(6):373-86, 2007 Nov-Dec.
Abstract
The CD3 zeta chain, a component of the T cell receptor (TCR)/CD3 complex, is considered to be
a limiting factor in the assembly and transport of the TCR/CD3 complex to the cell surface and is
crucial to receptor signaling function. Recent studies have demonstrated altered expression and
function of this signal transduction molecule in T and natural killer cells in patients with chronic
inflammatory/autoimmune diseases. In this review, current knowledge concerning the expression
of CD3 zeta chain as well as the mechanisms responsible for abnormal expression of this
molecule in systemic lupus erythematosus, rheumatoid arthritis, and childhood idiopathic
nephrotic syndrome are summarized. [References: 114]
Publication Type
Journal Article. Research Support, Non-U.S. Gov't. Review.
<19>
Unique Identifier
17943754
Status
MEDLINE
Authors
Hodson EM. Willis NS. Craig JC.
Authors Full Name
Hodson, E M. Willis, N S. Craig, J C.
Institution
Children's Hospital at Westmead, Centre for Kidney Research, Locked Bag 4001, Westmead,
NSW, Australia, 2145. Elisah@chw.edu.au
Title
Corticosteroid therapy for nephrotic syndrome in children. [Review] [71 refs][Update of
Cochrane Database Syst Rev. 2005;(1):CD001533; PMID: 15674881]
Source
Cochrane Database of Systematic Reviews. (4):CD001533, 2007.
Abstract
BACKGROUND: In nephrotic syndrome (NS) protein leaks from the blood to the urine
through the glomeruli resulting in hypoproteinaemia and generalised oedema. While the
majority of children with NS respond to corticosteroids, 70% experience a relapsing course.
Corticosteroids have reduced the mortality rate to around 3%. However corticosteroids have
well recognised potentially serious adverse effects such as obesity, poor growth, hypertension,
diabetes mellitus and osteoporosis. OBJECTIVES: To determine the benefits and harms of
corticosteroid regimens in preventing relapse in children with steroid sensitive NS (SSNS).
SEARCH STRATEGY: We searched CENTRAL, Cochrane Renal Group Specialised Register,
MEDLINE and EMBASE without language restriction, reference lists of articles and contact
with known investigators. Date of last search: December 2006 SELECTION CRITERIA:
Randomised controlled trials performed in children (three months to 18 years) in their initial or
subsequent episode of SSNS, comparing different durations, total doses or other dose strategies
using any corticosteroid agent, with outcome data at six months or more. DATA COLLECTION
AND ANALYSIS: Two authors independently assessed trial quality and extracted data. Results
were expressed as relative risk (RR) with 95% confidence intervals (CI) or mean difference
(WMD). Meta-regression was used to explore potential between-study differences due to baseline
risk of relapse, study quality and interventions. MAIN RESULTS: Twenty four trials were
identified. Six trials comparing two months of prednisone or prednisolone with three months or
more in the first episode showed longer duration significantly reduced the risk of relapse at 12 to
24 months (RR 0.70, 95% CI 0.58 to 0.84). There was an inverse linear relationship between
treatment duration and risk of relapse (RR = 1.26 - 0.112 duration; P = 0.03). Four trials showed
that six months of prednisone was more effective than three months in reducing the risk for
relapse (RR 0.57; 95% CI 0.45 to 0.71). Deflazacort was significantly more effective in
maintaining remission than prednisone in children who frequently relapsed in a single study (RR
0.44, 95% CI 0.25 to 0.78). There were no increases in adverse events. AUTHORS'
CONCLUSIONS: Children in their first episode of SSNS should be treated for at least three
months with an increase in benefit for up to seven months of treatment. For a baseline risk for
relapse following the first episode of 60% with two months of therapy, daily prednisone or
prednisolone given for four weeks followed by alternate-day therapy for six months would reduce
the number of children relapsing by 33%. [References: 71]
Publication Type
Journal Article. Meta-Analysis. Review.
<20>
Unique Identifier
17551756
Status
MEDLINE
Authors
Borges FF. Shiraichi L. da Silva MP. Nishimoto EI. Nogueira PC.
Authors Full Name
Borges, Fabio Fernandes. Shiraichi, Luciana. da Silva, Marcos Paulo Hippolito. Nishimoto,
Eduardo Isaac. Nogueira, Paulo Cesar Koch.
Institution
Faculdade de Ciencias Medicas de Santos, UNILUS, Sao Paulo, Brazil.
Title
Is focal segmental glomerulosclerosis increasing in patients with nephrotic syndrome?. [Review]
[18 refs]
Source
Pediatric Nephrology. 22(9):1309-13, 2007 Sep.
Abstract
Idiopathic nephrotic syndrome in children has conventionally been associated with minimal
change disease. However, recent reports have conflictingly suggested that the frequency of focal
segmental glomerulosclerosis (FSGS) in children might be on the increase, as has occurred in
adults. The aim of the present work was to review the medical literature to ascertain whether an
increase in the frequency of FSGS is occurring and, if so, to quantify such increase. We reviewed
the studies comparing the frequency of FSGS in two consecutive periods over the past three
decades (period 1 versus period 2). We pooled the data of the studies and then estimated FSGS
frequency in two ways: (a) including in the denominator all patients with nephrotic syndrome
and (b) including only patients who had undergone kidney biopsy. Both analyses were aimed to
determine the odds ratio of FSGS occurrence in the second period. Six studies fulfilled the
inclusion criteria, involving 1,149 patients with nephrotic syndrome. Four studies were used to
calculate FSGS frequency, including in the denominator all nephrotic patients (n = 885), yielding
an odds ratio of 2.22 (95% CI = 1.18-4.18). The analysis combining five studies with the number
of biopsies in the denominator (n = 603) produced an odds ratio of 1.98 (95% CI = 1.12-3.50).
These results suggest that a shift in the pathological pattern of nephrotic syndrome in children
might be occurring, resulting in an increase in FSGS frequency. This hypothesis has major
clinical significance due to the poorer prognosis associated with FSGS. [References: 18]
Publication Type
Journal Article. Review.
<21>
Unique Identifier
17594193
Status
MEDLINE
Authors
Westhoff TH. van der Giet M.
Authors Full Name
Westhoff, Timm H. van der Giet, Markus.
Institution
Charite--Campus Benjamin Franklin, Centrum 10--Nephrology, Hindenburgdamm 30, Berlin,
Germany. timm.westhoff@charite.de
Title
Tacrolimus in the treatment of idiopathic nephrotic syndrome. [Review] [100 refs]
Source
Expert Opinion on Investigational Drugs. 16(7):1099-110, 2007 Jul.
Abstract
The immunosuppressant tacrolimus (FK-506) is a calcineurin inhibitor with a widespread use
for the prevention of graft rejection in transplantation medicine. Tacrolimus inhibits the
activation of an essential transcription factor for the transcription of cytokine genes in T cells
leading to a decreased production of cytokines such as IL-2 and IFN-gamma. As T-cell activation
plays a crucial role in the pathogenesis of inflammatory glomerular diseases, there is an
increasing number of reports on the use of tacrolimus in nephrotic syndrome. In idiopathic
nephrotic syndrome, corticosteroid treatment constitutes the first-line therapy to achieve
remission. In the case of steroid resistance or steroid dependence, alternative immunosuppressive
strategies are needed. Cyclophosphamide and ciclosporin are well-established drugs in this
condition. The present article reviews the pharmacodynamics, pharmacokinetics, safety and
clinical efficacy of tacrolimus in steroid-resistant and steroid-dependent nephrotic syndrome.
[References: 100]
Publication Type
Journal Article. Review.
<22>
Unique Identifier
17537341
Status
MEDLINE
Authors
Colquitt JL. Kirby J. Green C. Cooper K. Trompeter RS.
Authors Full Name
Colquitt, J L. Kirby, J. Green, C. Cooper, K. Trompeter, R S.
Institution
Southampton Health Technology Assessments Centre, University of Southampton, UK.
Title
The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic
steroid-resistant nephrotic syndrome: a systematic review. [Review] [70 refs]
Source
Health Technology Assessment (Winchester, England). 11(21):iii-iv, ix-xi, 1-93, 2007.
Abstract
OBJECTIVES: To assess the clinical effectiveness and cost-effectiveness of treatments for
children with idiopathic steroid-resistant nephrotic syndrome (SRNS). DATA SOURCES:
Electronic databases from inception to February 2006, bibliographies of studies, and experts in
the field. REVIEW METHODS: Studies were selected, quality assessed and data were extracted
using recognised methods agreed a priori. Meta-analysis was undertaken where appropriate
using the random effects model. Where data allowed, subgroup analysis was undertaken
according to renal histopathology. RESULTS: Two systematic reviews and 11 trials were
included in the clinical effectiveness review; however, the quality of reporting and methodology
of the included studies was generally poor. No economic evaluations were identified. No
statistically significant difference in remission rates was found between cyclophosphamide plus
prednisone and prednisone alone for all children or those with focal segmental glomerulosclerosis
(FSGS), also the time to response was statistically significantly less with cyclophosphamide (38.4
days versus 95.5 days). Remission rates were not statistically significantly different between
intravenous and oral cyclophosphamide. Vomiting was common with intravenous
cyclophosphamide, while pneumonia and alopecia occurred in the oral group. Ciclosporin
statistically significantly increased the number of children with complete remission compared
with placebo or supportive treatment, but not for the FSGS subgroup, adverse effects including
infection and hypertension differed little between groups. No differences were found between
azathioprine and placebo, with about 13% of each group having remission. Complete or partial
remission occurred in six out of seven patients on the 18-month methylprednisolone regimen and
three out of five patients on the 6-month regimen, for both groups renal function improved and
adverse events such as hypertension and frequent infections occurred. Intravenous
dexamethasone and methylprednisolone produced similar complete remission rates, partial
remission rates, median time to response (about 10 days) and total number of adverse events,
with hypertension as the most common. Six-hour urinary albumin and urinary albumin to
creatinine ratio decreased statistically significantly with high-dose but not low-dose enalapril.
Tuna fish oil was not associated with any statistically significant improvements in proteinuria,
creatinine clearance, serum creatinine or lipid profiles compared with placebo. A very limited
literature was found on costs associated with SRNS in children. The pharmaceutical cost of
treatment varied considerably: an 8-week course of cyclophosphamide cost less than 6 pounds,
while a course of ciclosporin cost almost 900 pounds per year. Treatment with tacrolimus, an
alternative to ciclosporin, was estimated to cost in excess of 3400 pounds per year. Healthcare
medical management costs were estimated; varying by treatment strategy, they ranged from 250
pounds to 930 pounds per year in patients not experiencing complications. Other longer term
costs may also be incurred. Lack of data meant that cost-effectiveness modelling was not feasible.
CONCLUSIONS: The clinical effectiveness literature on treatments for idiopathic SRNS in
children is very limited. The available evidence suggests a beneficial effect of ciclosporin on
remission rates and of cyclophosphamide on time to remission; however, the strength of the
conclusions drawn is limited by the poor quality of the included studies. The other treatments
included in this review were each evaluated by only one study, and none found a statistically
significant effect. There is insufficient evidence to determine whether or not there is a clinically
significant difference. The available data on costs and outcomes are sparse and do not permit the
reliable modelling of the cost-effectiveness of treatments for SRNS at present. A modelling
framework is suggested, should more relevant data become available. A well-designed adequately
powered randomised controlled trial comparing ciclosporin with other treatments in children
with SRNS without genetic mutation is required. [References: 70]
Publication Type
Journal Article. Review.
<23>
Unique Identifier
17364996
Status
MEDLINE
Authors
Zaffanello M. Franchini M.
Authors Full Name
Zaffanello, Marco. Franchini, Massimo.
Institution
Department of Pediatrics, University of Verona, Verona, Italy. marco.zaffanello@univr.it
Title
Thromboembolism in childhood nephrotic syndrome: a rare but serious complication. [Review]
[54 refs]
Source
Hematology. 12(1):69-73, 2007 Feb.
Abstract
The main clinical features of nephrotic syndrome (NS) are heavy proteinuria,
hypoalbuminemia, hyperlipidemia and edema. In addition, multiple abnormalities in the
coagulation pathway may be a consequence of the NS. Both arterial and venous thromboembolic
complications (TEC) are relatively common and serious consequences of NS. In addition, arterial
and venous thrombosis might be unexpected events during an exacerbation of NS. Embolic
episodes may manifest in different regions of the body such as the brain or the lung. Hence,
predisposing factors, personal and family history of TEC, thrombosis location and evolution
should be always investigated in children with NS. [References: 54]
Publication Type
Journal Article. Review.
<24>
Unique Identifier
17186280
Status
MEDLINE
Authors
Fine RN.
Authors Full Name
Fine, Richard N.
Institution
School of Medicine, Stony Brook University, Stony Brook, NY 11794-8111, USA.
richard.fine@stonybrook.edu
Title
Recurrence of nephrotic syndrome/focal segmental glomerulosclerosis following renal
transplantation in children. [Review] [40 refs]
Source
Pediatric Nephrology. 22(4):496-502, 2007 Apr.
Other ID
Source: NLM. PMC1805045
Abstract
The incidence of recurrence of nephrotic syndrome/focal segmental glomerulosclerosis
(NS/FSGS) is variable (~30%). The incidence of recurrence is less in African-Americans than in
whites and Hispanics. Graft survival rates are decreased in recipients with FSGS, especially if
remission of the NS is not achieved in those with recurrence. Although controversial, the use of
living donor (LD) transplants are not contraindicated; however, obligatory heterozygote parental
grafts with a podocin mutation should be used with caution. Optimal treatment to induce a
remission post-transplant has not been delineated. Pre-transplant and/or prophylactic posttransplant pre-operative plasmapheresis (PP) for high-risk patients--especially those with
recurrence in a previous graft--may be promising. An international multicenter controlled study
is required to delineate the optimal approach to prevent and/or treat the recurrence of NS/FSGS.
[References: 40]
Publication Type
Journal Article. Review.
<25>
Unique Identifier
17285887
Status
MEDLINE
Authors
Jackson LW.
Authors Full Name
Jackson, Lori Williams.
Institution
University of Wisconsin Children's Hospital, USA.
Title
Congenital nephrotic syndrome. [Review] [36 refs]
Source
Neonatal Network - Journal of Neonatal Nursing. 26(1):47-55, 2007 Jan-Feb.
Abstract
When presented with an edematous infant who may be experiencing a severe infection,
particularly an unusual one, it is important to include nephrotic syndrome in the differential
diagnosis. Because drastic measures may be required to manage this illness, it is important to be
able to recognize symptoms, compile needed diagnostic data, and commence appropriate
treatment. A referred pediatric nephrologist can aid in diagnosis, direct management, and
educate and support parents. The nephrologist is also instrumental in guiding ongoing care and
preparing the infant for transplantation when it becomes necessary. [References: 36]
Publication Type
Journal Article. Review.
<26>
Unique Identifier
17225845
Status
MEDLINE
Authors
Anochie I. Eke F. Okpere A.
Authors Full Name
Anochie, Ifeoma. Eke, Felicia. Okpere, Augustina.
Institution
Department of Poediatrics, University of Port Harcourt Teaching Hospital, Port Harcourt,
Rivers State, Nigeria. anochieify@hotmail.com
Title
Childhood nephrotic syndrome: change in pattern and response to steroids. [Review] [44 refs]
Source
Journal of the National Medical Association. 98(12):1977-81, 2006 Dec.
Other ID
Source: NLM. PMC2569667
Abstract
BACKGROUND: In our center, childhood nephrotic syndrome (NS) had been reported for over
a decade to be steroid sensitive contrary to reports in other parts of Nigeria. The purpose of this
study was to determine if there are changes in presentation and response to steroids, with reviews
of the literature on NS. METHODS: Analysis of 28 patients seen at the University of Port
Harcourt Teaching Hospital, Nigeria, from 1999-2004 with the diagnosis of NS was performed.
RESULTS: There were 14 girls and 14 boys with NS. The peak age was 1-4 years. Twenty
(71.4%) children had idiopathic nephrotic syndrome (INS). Four had chronic renal failure, one
had sickle cell disease (HbSS), two were positive to human immunodeficiency virus (HIV) 1 and
2, and one had pulmonary tuberculosis. Anemia was found in 13 patients, while 17 had
Plasmodium falciparum. Plasmodium malariae and hepatitis-B surface antigen were not isolated.
Renal biopsy was performed in four patients and revealed minimal-change disease in one child,
focal segmental glomerulosclerosis in two and no conclusive result in one patient. Oral
prednisolone was used in INS. After one month of therapy, 16 of 20 responded, of which 12
(75%) were <5 years. The NS relapsed in 15 of 16 steroid-sensitive patients. Cyclophosphamide
and levamisole were used in four and one patients with FRNS, respectively. Four (14.3%)
patients died; all were secondary NS. CONCLUSION: INS remains common in our center, and
the majority respond to steroid therapy [References: 44]
Publication Type
Journal Article. Review.
<27>
Unique Identifier
17044482
Status
MEDLINE
Authors
Kanjanabuch T. Lewsuwan S. Kitiyakara C. Cheunsuchon B. Eiam-Ong S.
Authors Full Name
Kanjanabuch, Talerngsak. Lewsuwan, Songkiat. Kitiyakara, Chagriya. Cheunsuchon,
Boonyarit. Eiam-Ong, Somchai.
Institution
Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn
University Hospital, Bangkok, Thailand. golfnephro@hotmail.com
Title
Update in pathophysiology and histopathology of focal segmental glomerulosclerosis. [Review]
[56 refs]
Source
Journal of the Medical Association of Thailand. 89 Suppl 2:S262-79, 2006 Aug.
Abstract
Focal segmental glomerulosclerosis (FSGS) is the leading cause of nephrotic syndrome in an
adult worldwide. The prevalence of FSGS is estimated as being 20-30% in adults over the age of
15 years and slightly higher (30-35%) in the elderly (age > 60 years). The diagnosis solely relies
on pathologic findings, which sclerosis involves some, but not all glomeruli (focal), and sclerosis
affects a portion, but not the entire, glomerular tuft (segmental). The pathogenesis remains
inconclusive but podocyte injury has been postulated. Even though steroid is the mainstay
treatment, only 20-40% of patients are complete respond. [References: 56]
Publication Type
Journal Article. Review.
<28>
Unique Identifier
16691407
Status
MEDLINE
Authors
Fluss J. Geary D. deVeber G.
Authors Full Name
Fluss, Joel. Geary, Denis. deVeber, Gabrielle.
Institution
Division of Pediatric Neurology, The Hospital for Sick Children, Toronto, ON, Canada.
Title
Cerebral sinovenous thrombosis and idiopathic nephrotic syndrome in childhood: report of
four new cases and review of the literature. [Review] [52 refs]
Comments
Comment in: Eur J Pediatr. 2007 Jul;166(7):757-8; author reply 759; PMID: 17447084]
Source
European Journal of Pediatrics. 165(10):709-16, 2006 Oct.
Abstract
INTRODUCTION: Nephrotic children are prone to develop thromboembolic complications
secondary to an acquired hypercoagulable state. Cerebral sinovenous thrombosis (CSVT) is
increasingly recognised in this population, but clinical characteristics and outcome are not well
documented. PATIENTS AND METHODS: The database of the Canadian Pediatric Ischemic
Stroke Registry (Toronto Site) containing prospectively enrolled children from 1992-2004 with
CSVT identified four children with NS. A pooled literature analysis retrieved 17 additional cases
reports. RESULTS: CSVT presented in the majority of cases during the first flare or within 6
months after the onset of NS and was found to occur more often in SSNS/SDNS (n=13) than in
SRNS (n=4). Clinical manifestations were non-specific and consisted primarily of seizures (n=8)
and signs of raised intracranial pressure (n=16). Imaging studies revealed a predilection for
superior sagittal sinus involvement (n=21) and rare parenchymal lesions (n=4). The most
consistent biological risk factors were a severe hypoalbuminaemia (n=14) and, to a lesser extent,
decreased antithrombin (AT) levels (n=9/16). Deficiency of other coagulation inhibitors (protein
S, protein C) was not identified. Inherited thrombophilia was documented in a single case,
suggesting that acquired, more than genetic, coagulation factors are involved. Anticoagulation
was safe, and the outcome was good in most patients, and no recurrence of thrombotic event was
reported. DISCUSSION: In conclusion, CSVT is now a well-described complication of NS with
potential morbidity. A high index of suspicion is required, especially in young children with NS
presenting neurological symptoms. Reliable biological predictors of CSVT are lacking.
[References: 52]
Publication Type
Case Reports. Journal Article. Review.
<29>
Unique Identifier
16898477
Status
MEDLINE
Authors
Feber J. Filler G.
Authors Full Name
Feber, Janusz. Filler, Guido.
Institution
Division of Nephrology, Department of Pediatrics, Children's Hospital of Eastern Ontario,
Ottawa, Canada.
Title
Nephrotic syndrome in children: should we always use steroids for the initial therapy?.
[Review] [30 refs]
Source
Przeglad Lekarski. 63 Suppl 3:12-4, 2006.
Publication Type
Journal Article. Review.
<30>
Unique Identifier
16625586
Status
MEDLINE
Authors
Hodson EM. Habashy D. Craig JC.
Authors Full Name
Hodson, E M. Habashy, D. Craig, J C.
Institution
Children's Hospital at Westmead, Centre for Kidney Research, Locked Bag 4001, Westmead,
NSW, Australia, 2145. Elisah@chw.edu.au
Title
Interventions for idiopathic steroid-resistant nephrotic syndrome in children. [Review] [38
refs][Update of Cochrane Database Syst Rev. 2004;(2):CD003594; PMID: 15106208]
Source
Cochrane Database of Systematic Reviews. (2):CD003594, 2006.
Abstract
BACKGROUND: The majority of children who present with their first episode of nephrotic
syndrome, achieve remission with corticosteroid therapy. Children who fail to respond may be
treated with immunosuppressive agents such as cyclophosphamide, chlorambucil or cyclosporin,
or with non-immunosuppressive agents such as ACE inhibitors. Optimal combinations of these
agents with the least toxicity remain to be determined. OBJECTIVES: To evaluate the benefits
and harms of interventions used to treat idiopathic steroid resistant nephrotic syndrome (SRNS)
in children. SEARCH STRATEGY: Randomised controlled trials (RCTs) were identified from
the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE,
reference lists of articles and abstracts from conference proceedings. Date of most recent search:
June 2005 SELECTION CRITERIA: RCTs and quasi-RCTs were included if they compared
different immunosuppressive agents or non-immunosuppressive agents with placebo, prednisone
or other agent given orally or parenterally in children aged three months to 18 years with SRNS.
DATA COLLECTION AND ANALYSIS: Two reviewers independently searched the literature,
determined trial eligibility, assessed quality, extracted data and entered it in RevMan. For
dichotomous outcomes, results were expressed as relative risk (RR) and 95% confidence intervals
(CI). Data were pooled using the random effects model. MAIN RESULTS: Eleven RCTs (312
children) were included. Cyclosporin when compared with placebo or no treatment significantly
increased the number of children who achieved complete remission (three trials, 49 children: RR
for persistent nephrotic syndrome 0.64, 95% CI, 0.47 to 0.88). There was no significant difference
in the number of children who achieved complete remission between oral cyclophosphamide with
prednisone and prednisone alone (two trials, 91 children: RR 1.01, 95% CI 0.74 to 1.36), between
intravenous cyclophosphamide and oral cyclophosphamide (one study, 11 children: RR 0.09,
95% CI 0.01 to 1.39) and between azathioprine with prednisone and prednisone alone (one trial
31 children: RR 1.01, 95% CI 0.77 to 1.32). ACE inhibitors significantly reduced proteinuria
(two trials, 70 children). After 12 weeks of treatment fosinopril reduced proteinuria by 0.95 g/24
h (95% CI -1.21 to -0.69). No RCTs were identified comparing combination regimens comprising
high dose steroids, alkylating agents or cyclosporin with single agents, placebo or no treatment.
AUTHORS' CONCLUSIONS: Further adequately powered and well designed RCTs are needed
to confirm the efficacy of cyclosporin and to evaluate other regimens for idiopathic SRNS
including high dose steroids with alkylating agents or cyclosporin. [References: 38]
Publication Type
Journal Article. Meta-Analysis. Review.