We searched the PubMed on-line database for all Englishlanguage papers on nephrotic syndrome published between 1996 and 2002. Our key search terms were “nephrotic syndrome”, limited to “all children”. We chose papers relevant to the paediatric population and pertaining to the topics of epidemiology, pathophysiology, diagnosis, and management as the basis of further review for this seminar.
SeminarNephrotic syndrome in childhood
Section snippets
Epidemiology and classification
Idiopathic nephrotic syndrome has a reported incidence of two to seven cases per 100 000 children and a prevalence of nearly 16 cases per 100 000. There are three distinct histological variants of primary idiopathic nephrotic syndrome: minimal-change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (figure 1). MCNS and FSGS may represent opposite ends of one pathophysiological process or distinct disease entities. By contrast, membranous
Primary glomerular defect
The glomerular capillary wall consists of three structural elements that constitute the permselectivity barrier: endothelial cells separated by fenestrae, the glomerular basement membrane made up of a network of matrix proteins, and specialised epithelial cells (podocytes) connected to each other via an interdigitating network of slit diaphragms. Normally, proteins the size of albumin (69 kd) and larger are excluded from filtration, a restriction that depends substantially on the integrity of
Natural history and prognosis
The most important prognostic indicator in nephrotic syndrome is steroid responsiveness. Overall, 60–80% of steroid-responsive nephrotic children will relapse and about 60% of those will have five or more relapses. Age older than 4 years at presentation and remission within 7–9 days of the start of steroid treatment in the absence of microhaematuria are predictive of fewer relapses.137, 138, 139 In a natural-history study of 398 children, the proportion that became non-relapsers rose from 44%
Search Strategy
References (140)
The nephrotic syndrome
Pediatr Clin North Am
(1971)- et al.
Changing trend of histopathology in childhood nephrotic syndrome
Am J Kidney Dis
(1999) - et al.
Changing patterns in the histopathology of idiopathic nephrotic syndrome in children
Kidney Int
(1999) - et al.
Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome
Cell
(1991) - et al.
WT1 splice-site mutations are rarely associated with primary steroid-resistant focal and segmental glomerulosclerosis
Kidney Int
(2000) - et al.
A glomerular permeability factor produced by human T cell hybridomas
Kidney Int
(1991) - et al.
In vitro decrease of glomerular heparan sulfate by lymphocytes from idiopathic nephrotic syndrome patients
Kidney Int
(2001) - et al.
Dominant T cells in idiopathic nephrotic syndrome of childhood
Kidney Int
(2000) - et al.
T-lymphocyte populations and cytokines in childhood nephrotic syndrome
Am J Kidney Dis
(2002) - et al.
Parvovirus B19 DNA in kidney tissue of patients with focal segmental glomerulosclerosis.
Am J Kidney Dis
(2000)
Mechanisms and management of nephrotic edema
Kidney Int
Blood volume, colloid osmotic pressure and F-cell ratio in children with the nephrotic syndrome
Kidney Int
Atrial natriuretic peptide and the renal response to hypervolemia in nephrotic humans.
Kidney Int
A critique of the overfill hypothesis of sodium and water retention in the nephrotic syndrome
Kidney Int
Up-regulation of acyl-coenzyme A: cholesterol acyltransferase (ACAT) in nephrotic syndrome
Kidney Int
Proteinuria and plasma compositional changes contribute to defective lipoprotein catabolism in the nephrotic syndrome by separate mechanisms
Am J Kidney Dis
Hypoalbuminemia and proteinuria contribute separately to reduced lipoprotein catabolism in the nephrotic syndrome.
Kidney Int
Childhood steroid-sensitive nephrotic syndrome: does the histology matter?
Am J Kidney Dis
Idiopathic membranous glomerulonephritis
Kidney Int
Immunosuppressive agents in childhood nephrotic syndrome: a meta-analysis of randomized controlled trials.
Kidney Int
Management of minimal lesion glomerulonephritis: evidence-based recommendations
Kidney Int Suppl
Cyclosporin in frequently relapsing minimal change nephrotic syndrome
Lancet
Risk factors for cyclosporine-induced tubulointerstitial lesions in children with minimal change nephrotic syndrome
Kidney Int
A multicenter trial of mizoribine compared with placebo in children with frequently relapsing nephrotic syndrome
Kidney Int
Mycophenolate mofetil treatment for primary glomerular diseases
Kidney Int
A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome
Kidney Int
Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone. French Society of Pediatric Nephrology
J Pediatr
A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome Study Group
Kidney Int
Management of focal segmental glomerulosclerosis: evidence-based recommendations
Kidney Int Suppl
High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood
Pediatr Nephrol
a report of the International Study of Kidney Disease in Children
Kidney Int
Changing trends of histopathology in childhood nephrotic syndrome in western Saudi Arabia
Saudi Med J
Racial differences in the incidence and renal outcome of idiopathic focal segmental glomerulosclerosis in children
Pediatr Nephrol
The paucity of minimal change disease in adolescents with primary nephrotic syndrome
Pediatr Nephrol
Discovery of the congenital nephrotic syndrome gene discloses the structure of the mysterious molecular sieve of the kidney
Int J Dev Biol
NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome
Nat Genet
Mutations in ACTN4, encoding alpha-actinin-4, cause familial focal segmental glomerulosclerosis
Nat Genet
Not all in the family: mutations of podocin in sporadic steroid-resistant nephrotic syndrome
J Am Soc Nephrol
WT1 is a key regulator of podocyte function: reduced expression levels cause crescentic glomerulonephritis and mesangial sclerosis
Hum Mol Genet
Donor splice-site mutations in WT1 are responsible for Frasier syndrome
Nat Genet
Insight into podocyte differentiation from the study of human genetic disease: nail-patella syndrome and transcriptional regulation in podocytes
Pediatr Res
Mutant chromatin remodeling protein SMARCAL1 causes Schimke immuno-osseous dysplasia
Nat Genet
Congenital nephrotic syndrome in mice lacking CD2-associated protein
Science
Genetic polymorphisms in CD2AP are common in patients with glomerular disease
J Am Soc Nephrol
Prevalence, genetics, and clinical features of patients carrying podocin mutations in steroid-resistant nonfamilial focal segmental glomerulosclerosis
J Am Soc Nephrol
Novel mutations in NPHS2 detected in both familial and sporadic steroid-resistant nephrotic syndrome
J Am Soc Nephrol
Mutations in NPHS2 encoding podocin are a prevalent cause of steroid-resistant nephrotic syndrome among Israeli-Arab children
J Am Soc Nephrol
Nephrotic syndrome and end-stage renal disease with WT1 mutation detected at 3 years
Pediatr Nephrol
Clinical and genetic evaluation of familial steroid-responsive nephrotic syndrome in childhood
J Am Soc Nephrol
Circulating mediators of proteinuria in idiopathic minimal lesion nephrotic syndrome
Pediatr Nephrol
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