MCID: STR044
MIFTS: 31

Steroid-Resistant Nephrotic Syndrome malady

Genetic diseases (common), Nephrological diseases categories

Aliases & Classifications for Steroid-Resistant Nephrotic Syndrome

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Aliases & Descriptions for Steroid-Resistant Nephrotic Syndrome:

Name: Steroid-Resistant Nephrotic Syndrome 22 65
 
Srn1 22


Classifications:



Summaries for Steroid-Resistant Nephrotic Syndrome

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MalaCards based summary: Steroid-Resistant Nephrotic Syndrome, also known as srn1, is related to membranous nephropathy and nephrotic syndrome. An important gene associated with Steroid-Resistant Nephrotic Syndrome is NPHS2 (Nephrosis 2, Idiopathic, Steroid-Resistant (Podocin)), and among its related pathways are Nephrin/Neph1 signaling in the kidney podocyte and Cell junction organization. Affiliated tissues include testes, kidney and t cells, and related mouse phenotypes are renal/urinary system and homeostasis/metabolism.

Related Diseases for Steroid-Resistant Nephrotic Syndrome

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Diseases related to Steroid-Resistant Nephrotic Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 100)
idRelated DiseaseScoreTop Affiliating Genes
1membranous nephropathy29.6ALB, CD2AP, NPHS1, NPHS2
2nephrotic syndrome11.4
3glomerulosclerosis10.7
4focal segmental glomerulosclerosis10.6
5hypothyroidism10.5
6sporadic idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis10.5
7familial idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis10.5
8child syndrome10.5
9glomerulonephritis10.5
10urinary system disease10.5
11kid syndrome10.5
12nephrotic syndrome, idiopathic, steroid-resistant10.5
13familial steroid-resistant nephrotic syndrome with sensorineural deafness10.5
14familial idiopathic steroid-resistant nephrotic syndrome10.5
15sporadic idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial proliferation10.5
16sporadic idiopathic steroid-resistant nephrotic syndrome with minimal changes10.5
17sporadic idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial sclerosis10.5
18familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial sclerosis10.5
19familial idiopathic steroid-resistant nephrotic syndrome with minimal changes10.5
20familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial proliferation10.5
21sporadic idiopathic steroid-resistant nephrotic syndrome10.5
22sporadic idiopathic steroid-resistant nephrotic syndrome with collapsing glomerulopathy10.5
23nephrotic syndrome, type 210.4
24adult syndrome10.4
25glomerulosclerosis, focal segmental, 110.4
26chronic kidney failure10.4
27primary hyperoxaluria10.4
28kidney disease10.4
29glomerular disease10.4
30encephalopathy10.4
31systemic lupus erythematosus10.2
32nephrotic syndrome, type 410.2
33multiple myeloma10.2
34noonan syndrome 110.2
35coenzyme q10 deficiency, primary, 610.2
36mass syndrome10.2
37galloway-mowat syndrome10.2
38nephrotic syndrome, type 1010.2
39nephrotic syndrome, type 110.2
40glomerulosclerosis, focal segmental, 410.2
41glomerulosclerosis, focal segmental, 210.2
42west syndrome10.2
43end stage renal failure10.2
44sagittal sinus thrombosis10.2
45good syndrome10.2
46anuria10.2
47lipoid nephrosis10.2
48mesangial proliferative glomerulonephritis10.2
49cerebritis10.2
50proliferative glomerulonephritis10.2

Graphical network of the top 20 diseases related to Steroid-Resistant Nephrotic Syndrome:



Diseases related to steroid-resistant nephrotic syndrome

Symptoms for Steroid-Resistant Nephrotic Syndrome

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Drugs & Therapeutics for Steroid-Resistant Nephrotic Syndrome

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Drugs for Steroid-Resistant Nephrotic Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
rituximabapprovedPhase 4, Phase 2, Phase 31514174722-31-710201696
Synonyms:
AntiCD20
IDEC-102
IDEC-C2B8
 
Ig gamma-1 chain C region
MabThera
Mabthera
Rituxan
rituximab
2
Tacrolimusapproved, investigationalPhase 4992104987-11-3445643, 439492, 445647, 439492, 445647
Synonyms:
(-)-FK 506
(3S,4R,5S,8R,9E,12S,14S,15R,16S,18R,19R,26aS)-5,19-dihydroxy-3-{(1E)-1-[(1R,3R,4R)-4-hydroxy-3-methoxycyclohexyl]prop-1-en-2-yl}-14,16-dimethoxy-4,10,12,18-tetramethyl-8-(prop-2-en-1-yl)-5,6,8,11,12,13,14,15,16,17,18,19,24,25,26,26a-hexadecahydro-3H-15,19-epoxypyrido[2,1-c][1,4]oxazacyclotricosine-1,7,20,21(4H,23H)-tetrone
104987-11-3
109581-93-3 (Hydrate)
15,19-epoxy-3H-pyrido[2,1-c][1,4]oxaazacyclotricosine-1,7,20,21(23H)-tetrone,
3S-[3R*[E(1S*,3S*,4S*)],4S*,5R*,8S*,9E,12R*,14R*,15S*,16R*,18S*,19S*,26aR*]]-5,6,8,11,12,13,14,15,16,17,18,19,24,25,26,26a-hexadecahydro-5, 19-dihydroxy-3-[2-(4-hydroxy-3-methoxycyclohexyl)-1-methylethenyl]-14,16-dimethoxy-4,10,12,18-tetramethyl-8-(2-propenyl)-15,19-epoxy-3H-pyrido[2,1-c] [1,4] oxaazacyclotricosine-1,7,20,21(4H,23H)-tetrone
8-DEETHYL-8-[BUT-3-ENYL]-ASCOMYCIN
8-DEETHYL-8-[but-3-enyl]-ascomycin
AC-1182
AC1L1K7H
AC1L97GB
AC1L9IBU
AKOS005145901
Advagraf
Ambap104987-11-3
Ambap5429
BCBcMAP01_000194
BRD-K35452788-001-02-1
BSPBio-001279
BSPBio_001279
Bio-0921
Bio2_000470
Bio2_000950
C01375
C44H69NO10
CCRIS 7124
CHEBI:100924
CHEBI:61049
CHEMBL1200738
CID11158639
CID11556866
CID439492
CID445643
CID5372
CID5472317
CID6426916
CID6436007
CID6473866
CID6536850
CID6610362
CID6912836
CID9832283
CID9853905
CID9918805
CID9940643
CID9963169
CPD-10016
CPD000466356
D08556
DB00864
DivK1c_001040
FK 506
FK-506
FK5
FK506
FR 900506
FR-900506
FR900506
 
FT-0082660
Fk-506
Fujimycin
Graceptor
HMS1362O21
HMS1792O21
HMS1990O21
HMS2051C18
HMS2093M19
HMS503O21
Hecoria
IDI1_001040
IDI1_002225
K506
KBio1_001040
KBio2_000619
KBio2_003187
KBio2_005755
KBio3_001097
KBio3_001098
KBioGR_000619
KBioSS_000619
L 679934
L-679934
LCP-Tacro
LMPK04000003
LS-64247
MLS000759471
MLS001424054
Modigraf
MolPort-003-666-518
NCGC00163470-01
NCGC00163470-02
NCGC00163470-03
NCGC00163470-04
NCGC00179232-01
NChemBio.2007.16-comp1
NINDS_001040
NSC717865
Prograf
Prograf (TN)
Protopic
Protopy
S5003_Selleck
SAM001246677
SMR000466356
Tacarolimus
Tacrolimus
Tacrolimus (INN)
Tacrolimus (Prograf?)
Tacrolimus (anhydrous)
Tacrolimus Hydrate
Tacrolimus anhydrous
Tacrolimus hydrate
Tsukubaenolide
UNII-Y5L2157C4J
nchembio.2007.23-comp2
tacrolimus
tacrolimus hydrate
3
Irbesartanapproved, investigationalPhase 295138402-11-63749
Synonyms:
138402-11-6
2-Butyl-3-(p-(o-1H-tetrazol-5-ylphenyl)benzyl)-1,3-diazaspiro(4.4)non-1-en-4-one
2-Butyl-3-[2'-(1H-tetrazol-5-yl)biphenyl-4-ylmethyl]1,3-diaza-spiro[4.4]non-1-en-4-one
2-Butyl-3-{[2'-(1H-tetrazol-5-yl)biphenyl-4-yl]methyl}-1,3-diazaspiro[4.4]non-1-en-4-one
2-butyl-3-[ p -( o -1 H -tetrazol-5-ylphenyl)benzyl]-1,3-diazaspiro[4,4]non-1-en-4-one
2-butyl-3-[2'-(1h-tetrazol-5-yl)-biphenyl-4-ylmethyl]-1,3-diaza-spiro[
2-butyl-3-[2'-(1h-tetrazol-5-yl)biphenyl-4-ylmethyl]1,3-diaza-spiro[
2-butyl-3-[p-(o-1H-tetrazol-5-ylphenyl)benzyl]-1,3-diazaspiro[4.4]non-1-en-4-one
2-butyl-3-{[2'-(1H-tetrazol-5-yl)[1,1'-biphenyl]-4-yl]methyl}-1,3-diazaspiro[4.4]non-1-en-4-one
2-butyl-3-{[2'-(1H-tetrazol-5-yl)biphenyl-4-yl]methyl}-1,3-diazaspiro[4.4]non-1-en-4-one
2-n-butyl-3-((2'-(1H-tetrazol-5-yl)biphenyl-4-yl)methyl)-1,3-diazaspiro(4,4)non-1-en-4-one
8-butyl-7-[[4-[2-(2H-tetrazol-5-yl)phenyl]phenyl]methyl]-7,9-diazaspiro[4.4]non-8-en-6-one
AC-537
AC1L1GMK
Aprovel
Avalide
Avapro
Avapro (TN)
BIDD:GT0347
BMS 186295
BMS Brand of Irbesartan
BMS-186295
BMS-186295, SR-47436, Aprovel, Karvea, Irbesartan
BRD-K60038276-001-02-5
BSPBio_002687
Bio-0053
Bristol Myers Brand of Irbesartan
C07469
C081309
C25H28N6O
CHEBI:5959
CHEMBL1513
CID3749
CPD000466306
D00523
DB01029
HMS1922J05
HMS2051L08
HMS2093E16
 
I06-0690
Irbesarran
Irbesartan
Irbesartan (JAN/USAN/INN)
Irbesartan [USAN:INN]
Irbesartan [Usan:Inn]
Irbetan
Jsp002315
KBio2_002231
KBio2_004799
KBio2_007367
KBio3_001907
KBioGR_001603
KBioSS_002231
Karvea
L000319
LS-60064
Lrbesartan
MLS000759408
MLS001424099
MolPort-003-666-550
NCGC00095122-01
NCGC00095122-02
NCGC00095122-03
S1507_Selleck
SAM001246548
SMR000466306
SPBio_001889
SPECTRUM1504259
SR 47436
SR-47436
STK645362
Sanofi Winthrop Brand of Irbesartan
Spectrum2_001675
Spectrum3_000994
Spectrum4_001122
Spectrum5_001288
Spectrum_001751
TL8000875
UNII-J0E2756Z7N
irbesartan
4
Angiotensin IIPhase 298568521-88-0, 11128-99-7172198
Synonyms:
1-8-Angiotensin I
1-L-Aspasaginyl-5-L-valyl angiotensin octapeptide
Ang II
Angiotensin 2
Angiotensin II (human)
 
Angiotensin II (mouse)
Angiotonin
Asp-arg-val-TYR-ile-his-pro-phe
Human angiotensin II
Hypertensin
Ile(5)-angiotensin II
5Angiotensin II Type 1 Receptor BlockersPhase 2928

Interventional clinical trials:

idNameStatusNCT IDPhase
1Efficacy and Safety of Rituximab to That of Calcineurin Inhibitors in Children With Steroid Resistant Nephrotic SyndromeRecruitingNCT02382575Phase 4
2Therapeutic Effect of Tacrolimus on Primary Nephrotic Syndrome in ChildrenWithdrawnNCT01162005Phase 4
3Rituximab Trial for Pediatric Nephrotic SyndromeRecruitingNCT01716442Phase 2, Phase 3
4Randomized, Double-Blind, Safety and Efficacy Study of RE-021 (Sparsentan) in Focal Segmental GlomerulosclerosisRecruitingNCT01613118Phase 2
5Oral Galactose in Children With Steroid Resistant Nephrotic SyndromeCompletedNCT01113385
6Angiotensin Converting Enzyme Gene Polymorphism in Children With Idiopathic Nephrotic SyndromeCompletedNCT00172276
7Cardiomyopathy in Steroid-resistant Nephrotic Syndrome: Impact of Focal Segmental GlomerulosclerosisTerminatedNCT00883636

Search NIH Clinical Center for Steroid-Resistant Nephrotic Syndrome

Genetic Tests for Steroid-Resistant Nephrotic Syndrome

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Genetic tests related to Steroid-Resistant Nephrotic Syndrome:

id Genetic test Affiliating Genes
1 Steroid-Resistant Nephrotic Syndrome22 NPHS2

Anatomical Context for Steroid-Resistant Nephrotic Syndrome

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MalaCards organs/tissues related to Steroid-Resistant Nephrotic Syndrome:

33
Testes, Kidney, T cells, Thyroid, Heart

Animal Models for Steroid-Resistant Nephrotic Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Steroid-Resistant Nephrotic Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053677.1ACTN4, ALB, CD2AP, NPHS1, NPHS2, WT1
2MP:00053767.1ACTN4, ALB, CD2AP, NPHS1, NPHS2, WT1
3MP:00107687.0ACTN4, ALB, CD2AP, NPHS1, NPHS2, WT1

Publications for Steroid-Resistant Nephrotic Syndrome

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Articles related to Steroid-Resistant Nephrotic Syndrome:

(show top 50)    (show all 251)
idTitleAuthorsYear
1
Defects of CRB2 cause steroid-resistant nephrotic syndrome. (25557779)
2015
2
Clinical efficacy and pharmacokinetics of tacrolimus in children with steroid-resistant nephrotic syndrome. (26135137)
2015
3
From microscopy to molecules: unraveling the pathogenesis of steroid-resistant nephrotic syndrome. (25899183)
2015
4
NPHS2 R229Q polymorphism in steroid resistant nephrotic syndrome: is it responsive to immunosuppressive therapy? (24519673)
2014
5
Significance of combined cyclosporine-prednisolone therapy and cyclosporine blood concentration monitoring for idiopathic membranous nephropathy with steroid-resistant nephrotic syndrome: a randomized controlled multicenter trial. (24363128)
2013
6
R: Histological patterns of idiopathic steroid resistant nephrotic syndrome in Egyptian children: A single centre study. (24475443)
2013
7
A large intracardiac thrombus in a child with steroid-resistant nephrotic syndrome. (22813602)
2013
8
Wilms' tumor suppressor gene mutations in girls with sporadic isolated steroid-resistant nephrotic syndrome. (24338413)
2013
9
NPHS2 mutations in Indian children with sporadic early steroid resistant nephrotic syndrome. (22080622)
2012
10
Steroid-resistant nephrotic syndrome associated with steroid sulfatase deficiency-x-linked recessive ichthyosis: a case report and review of literature. (22419362)
2012
11
Histopathological spectrum of childhood idiopathic steroid-resistant nephrotic syndrome in Tunisia. (21387228)
2011
12
Novel mutations in steroid-resistant nephrotic syndrome diagnosed in Tunisian children. (21125408)
2011
13
Successful treatment of steroid-resistant nephrotic syndrome associated with WT1 mutations. (20191369)
2010
14
Plasmapheresis-induced clinical improvement in a patient with steroid-resistant nephrotic syndrome due to podocin (NPHS2) gene mutation. (21171529)
2010
15
Childhood idiopathic steroid resistant nephrotic syndrome in Southwestern Nigeria. (20814150)
2010
16
Treatment with Cyclophosphamide for steroid-resistant nephrotic syndrome in children. (21977148)
2010
17
Long-term outcome of children with steroid-resistant nephrotic syndrome treated with tacrolimus. (20217433)
2010
18
Successful use of single-dose rituximab for the maintenance of remission in a patient with steroid-resistant nephrotic syndrome. (19881243)
2009
19
Interleukin-18 expression in peripheral blood mononuclear cells in children with steroid-resistant nephrotic syndrome]. (19470251)
2009
20
Experience with tacrolimus in children with steroid-resistant nephrotic syndrome. (19499248)
2009
21
Management and outcome of steroid-resistant nephrotic syndrome in children. (19841524)
2009
22
Clinical and epidemiological assessment of steroid-resistant nephrotic syndrome associated with the NPHS2 R229Q variant. (19145239)
2009
23
Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome. (19714370)
2009
24
Low prevalence of NPHS2 mutations in African American children with steroid-resistant nephrotic syndrome. (18543005)
2008
25
Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome-a randomized controlled multicentre trial by the Arbeitsgemeinschaft fA1r PAodiatrische Nephrologie. (18481113)
2008
26
Steroid-resistant nephrotic syndrome. (18596732)
2008
27
Treatment of steroid-resistant nephrotic syndrome with cyclosporine: study of 17 cases and a literature review. (16358229)
2005
28
Mutations in NPHS2 in sporadic steroid-resistant nephrotic syndrome in Chinese children. (15769810)
2005
29
Steroid-resistant nephrotic syndrome and NPHS2 gene]. (15833180)
2005
30
Superior sagittal sinus thrombosis in steroid-resistant nephrotic syndrome. (15797188)
2005
31
Varicella-induced remission of steroid-resistant nephrotic syndrome in a child. (17642785)
2004
32
Enalapril dosage in steroid-resistant nephrotic syndrome. (14648339)
2004
33
Pulse cyclophosphamide therapy in steroid resistant nephrotic syndrome associated with mesangial proliferative glomerulonephritis. (17642763)
2004
34
NPHS2 mutation analysis shows genetic heterogeneity of steroid-resistant nephrotic syndrome and low post-transplant recurrence. (15253708)
2004
35
Three siblings with steroid-resistant nephrotic syndrome: new NPHS2 mutations in a Turkish family. (15264208)
2004
36
Cyclosporine in patients with steroid-resistant nephrotic syndrome: an open-label, nonrandomized, retrospective study. (15531003)
2004
37
Short term efficacy of intravenous dexamethasone and methylprednisolone therapy in steroid resistant nephrotic syndrome. (15523124)
2004
38
A gene locus for steroid-resistant nephrotic syndrome with deafness maps to chromosome 14q24.2. (12761252)
2003
39
NPHS2 mutations in sporadic steroid-resistant nephrotic syndrome in Japanese children. (12687458)
2003
40
Steroid resistant nephrotic syndrome. (12557961)
2002
41
Not all in the family: mutations of podocin in sporadic steroid-resistant nephrotic syndrome. (11805190)
2002
42
Steroid-resistant nephrotic syndrome at 4 months: no complications over 10 years. (10872200)
2000
43
NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome. (10742096)
2000
44
Successful management of steroid-resistant nephrotic syndrome using ibuprofen. (9021245)
1997
45
Steroid-resistant nephrotic syndrome associated with malignant thymoma. (8953125)
1996
46
Presymptomatic diagnosis of familial steroid-resistant nephrotic syndrome. (8606597)
1996
47
Neurotoxicity of Cyclosporine A in therapy of steroid resistant nephrotic syndrome in a 10-year old boy. (8711194)
1996
48
Steroid resistant nephrotic syndrome associated with spondyloepiphyseal dysplasia, transient ischemic attacks and lymphopenia. (7736684)
1995
49
Inefficacy and toxicity of pefloxacin in focal and segmental glomerulosclerosis with steroid-resistant nephrotic syndrome. (8099164)
1993
50
Mercaptopurine in the treatment of steroid-resistant nephrotic syndrome. (5831732)
1965

Variations for Steroid-Resistant Nephrotic Syndrome

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Expression for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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Search GEO for disease gene expression data for Steroid-Resistant Nephrotic Syndrome.

Pathways for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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GO Terms for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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Cellular components related to Steroid-Resistant Nephrotic Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1slit diaphragmGO:003605710.2NPHS1, NPHS2
2platelet alpha granule lumenGO:00310939.9ACTN4, ALB
3cell-cell junctionGO:00059119.1ACTN4, CD2AP, NPHS2
4protein complexGO:00432347.8ACTN4, ALB, CD2AP, NPHS2
5extracellular exosomeGO:00700627.6ACTN4, ALB, CD2AP, NPHS1, NPHS2

Biological processes related to Steroid-Resistant Nephrotic Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1glomerular basement membrane developmentGO:00328369.5NPHS1, WT1
2platelet degranulationGO:00025769.4ACTN4, ALB
3excretionGO:00075889.2NPHS1, NPHS2

Sources for Steroid-Resistant Nephrotic Syndrome

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet