steroid-resistant idiopathic Nephrotic syndrome

An autosomal recessive disorder characterized by childhood onset of PROTEINURIA; HYPOALBUMINEMIA; HYPERLIPIDEMIA; and EDEMA. Kidney biopsies show nonspecific histologic changes such as minimal change, FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS), and diffuse mesangial proliferation. The disorder is resistant to steroid treatment and progresses to END-STAGE RENAL FAILURE in the first or second decades. Germline mutations in the PDCN (NPHS2) gene have been identified. OMIM: 600995

Also Known As:

Networked: 27 relevant articles (4 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Proteinuria
2.	Nephrotic Syndrome (Syndrome, Nephrotic)
3.	Membranoproliferative Glomerulonephritis (Membranoproliferative Glomerulonephritis, Type II)
4.	Sclerosis
5.	Lipoid Nephrosis (Glomerulonephritis, Minimal Change)

Experts

1.	Cao, Qi: 2 articles (09/2008 - 12/2007)
2.	Huang, Wen-yan: 2 articles (09/2008 - 12/2007)
3.	Xu, Hong: 2 articles (09/2008 - 12/2007)
4.	Zhou, Li-jun: 2 articles (09/2008 - 12/2007)
5.	Jouve, Thomas: 1 article (01/2019)
6.	Malvezzi, Paolo: 1 article (01/2019)
7.	Naciri Bennani, Hamza: 1 article (01/2019)
8.	Noble, Johan: 1 article (01/2019)
9.	Rostaing, Lionel: 1 article (01/2019)
10.	Tetaz, Rachel: 1 article (01/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to steroid-resistant idiopathic Nephrotic syndrome:

1.	Cyclosporine (Ciclosporin)FDA LinkGeneric 11/01/2005 - "Cyclosporine (CsA) has been used in steroid-resistant idiopathic nephrotic syndrome (INS) in many previous studies. " 06/01/1993 - "A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome." 09/01/2008 - "[Long-term effects of cyclosporine A in children with steroid-resistant idiopathic nephrotic syndrome and outcomes of the patients]." 01/01/1995 - "Cyclosporin in steroid-resistant idiopathic nephrotic syndrome." 01/01/1991 - "Steroid-resistant idiopathic nephrotic syndrome and ciclosporin. "
2.	SteroidsIBA 09/01/2012 - "Management of frequently relapsing steroid-responsive or steroid-resistant idiopathic nephrotic syndrome (NS) in children has been a clinical challenge for pediatric nephrologists. " 06/01/2011 - "New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome." 09/01/2003 - "Two types of newly found haplotypes were associated with steroid-resistant idiopathic nephrotic syndrome of children, which might be responsible for steroid-resistance in partial idiopathic nephrotic syndrome of children." 02/01/2003 - "These biopsies were compared with 17 kidney biopsies of a group of 15 patients (control group) with other glomerulonephritis, including systemic lupus erythematosus, poststreptococcal acute glomerulonephritis, membranoproliferative glomerulonephritis, steroid-sensitive minimal change nephrotic syndrome, steroid-resistant idiopathic nephrotic syndrome with focal and segmental glomerulosclerosis, and persistent and isolated proteinuria with minimal change on kidney biopsy. " 01/01/2006 - "Urinary proteome of steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome of childhood."
3.	EnalaprilFDA LinkGeneric 02/01/1999 - "Efficacy of enalapril in the treatment of steroid resistant idiopathic nephrotic syndrome."
4.	Adrenal Cortex Hormones (Corticosteroids)IBA 03/01/1997 - "Several reports suggest that cyclosporine is effective in a proportion of patients with steroid-resistant idiopathic nephrotic syndrome, particularly when used in combination with corticosteroids. "
5.	sodium tetrametaphosphateIBA 06/01/2005 - "Efficacy of steroids, cyclosporin and cyclophos-phamide in steroid resistant idiopathic nephrotic syndrome."
6.	Ramipril (Altace)FDA LinkGeneric 06/01/2004 - "Twenty one (21) cases of steroid-resistant idiopathic nephrotic syndrome were randomized to receive ramipril (11) and verapamil (10) and were followed up for 12 months; monthly for the 1st 3 months and then every 3 months for the remaining study period. " 06/01/2004 - "The aim of this study was to evaluate and compare the anti-proteinuric effect of ramipril and verapamil in patients with steroid-resistant idiopathic nephrotic syndrome. " 06/01/2004 - "Both ramipril and verapamil can reduce proteinuria in patients suffering from steroid-resistant idiopathic nephrotic syndrome. "
7.	Verapamil (Calan)FDA LinkGeneric 06/01/2004 - "Twenty one (21) cases of steroid-resistant idiopathic nephrotic syndrome were randomized to receive ramipril (11) and verapamil (10) and were followed up for 12 months; monthly for the 1st 3 months and then every 3 months for the remaining study period. " 06/01/2004 - "The aim of this study was to evaluate and compare the anti-proteinuric effect of ramipril and verapamil in patients with steroid-resistant idiopathic nephrotic syndrome. " 06/01/2004 - "Both ramipril and verapamil can reduce proteinuria in patients suffering from steroid-resistant idiopathic nephrotic syndrome. "
8.	ApolipoproteinsIBA 08/01/2001 - "To study apolipoprotein E gene polymorphism in children with steroid- resistant idiopathic nephrotic syndrome (SRINS). " 08/01/2001 - "[The apolipoprotein E gene polymorphism in children with steroid-resistant idiopathic nephrotic syndrome]."
9.	Proteins (Proteins, Gene)FDA Link 07/01/2006 - "We aimed to test if fosinopril reduces urinary protein excretion and alleviates renal tubular damage in normotensive children with steroid-resistant idiopathic nephrotic syndrome (SRINS). " 02/01/2016 - "It is currently postulated that steroid-sensitive idiopathic nephrotic syndrome (SSNS) and steroid-resistant idiopathic nephrotic syndrome (SRNS), which are not related to the mutation of a gene coding for podocyte structures or for glomerular basement membrane proteins, result from a circulating factor affecting podocyte shape and function. "
10.	ProteomeIBA 01/01/2006 - "Urinary proteome of steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome of childhood."

Therapies and Procedures

1.	Hemofiltration 07/01/2015 - "Intermittent high-volume hemofiltration promotes remission in steroid-resistant idiopathic nephrotic syndrome."
2.	Therapeutics 01/01/2019 - "Apheresis Therapy for Steroid-Resistant Idiopathic Nephrotic Syndrome: Report on a Case Series." 06/01/2011 - "New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome." 09/01/2008 - "To observe the effects of long-term cyclosporine A (CsA) treatment in 20 children with steroid-resistant idiopathic nephrotic syndrome (SRNS) and analyse the relevant influencing factors of CsA therapy. " 06/01/2002 - "Clinicopathologic correlates predict the outcome in children with steroid-resistant idiopathic nephrotic syndrome treated with pulse methylprednisolone therapy." 03/01/1997 - "Cyclosporine in the therapy of steroid-resistant idiopathic nephrotic syndrome."
3.	Remission Induction 06/01/1993 - "To compare the efficacy (induction of remission) and safety of cyclosporine (CsA) with those of supportive therapy in patients with steroid-resistant idiopathic nephrotic syndrome (INS), we organized an open, prospective, randomized, multicentric, controlled study for parallel groups, stratified for adults and children. "
4.	Kidney Transplantation 03/27/2003 - "Between March 1991 and August 2001, 36 renal transplantations were performed in 35 patients with steroid-resistant idiopathic nephrotic syndrome in our institution. " 03/27/2003 - "Early recurrence of massive proteinuria after renal transplantation occurs in 20% to 30% of patients with steroid-resistant idiopathic nephrotic syndrome and is responsible for graft failure in approximately half of cases. " 04/01/1993 - "Reports on recurrence of nephrotic syndrome in patients with steroid-resistant idiopathic nephrotic syndrome following renal transplantation suggest that a humoral factor, possibly produced by T lymphocytes, may enhance glomerular permeability. "
5.	Transplantation 01/01/2018 - "Ofatumumab in post-transplantation recurrence of a pediatric steroid-resistant idiopathic nephrotic syndrome."