MCID: STR044
MIFTS: 37

Steroid-Resistant Nephrotic Syndrome malady

Genetic diseases (common), Nephrological diseases categories
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Summaries for Steroid-Resistant Nephrotic Syndrome

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MalaCards: Steroid-Resistant Nephrotic Syndrome, also known as nephrotic syndrome, steroid-resistant, autosomal recessive, is related to nephrotic syndrome and glomerulosclerosis. An important gene associated with Steroid-Resistant Nephrotic Syndrome is NPHS2 (nephrosis 2, idiopathic, steroid-resistant (podocin)), and among its related pathways are Cell junction organization and Nephrin interactions. The compounds puromycin aminonucleoside and lipid have been mentioned in the context of this disorder. Affiliated tissues include testes, kidney and heart, and related mouse phenotypes are growth/size/body and renal/urinary system.

Aliases & Classifications for Steroid-Resistant Nephrotic Syndrome

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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases (common)
Anatomical: Nephrological diseases


Aliases & Descriptions:

steroid-resistant nephrotic syndrome 20 62
nephrotic syndrome, steroid-resistant, autosomal recessive 62


Related Diseases for Steroid-Resistant Nephrotic Syndrome

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17GeneCards, 18GeneDecks
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Diseases related to Steroid-Resistant Nephrotic Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 52)
idRelated DiseaseScoreTop Affiliating Genes
1nephrotic syndrome32.0NPHS1, NPHS2, CD2AP, WT1
2glomerulosclerosis30.8NPHS1, NPHS2, CD2AP, WT1
3wilms tumor30.6NPHS1, NPHS2, CD2AP, WT1
4proteinuria30.6NPHS1, NPHS2, CD2AP, WT1
5focal segmental glomerulosclerosis30.5NPHS1, NPHS2, CD2AP, WT1
6nephrosis30.4NPHS2, NPHS1
7glomerulonephritis30.3CD2AP, NPHS2, NPHS1
8hypothyroidism10.5
9nephrotic syndrome, idiopathic, steroid-resistant10.5
10sporadic idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis10.5
11familial idiopathic steroid-resistant nephrotic syndrome with focal segmental hyalinosis10.5
12sporadic idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial proliferation10.4
13sporadic idiopathic steroid-resistant nephrotic syndrome with minimal changes10.4
14sporadic idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial sclerosis10.4
15familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial sclerosis10.4
16familial idiopathic steroid-resistant nephrotic syndrome with minimal changes10.4
17familial idiopathic steroid-resistant nephrotic syndrome with diffuse mesangial proliferation10.4
18sporadic idiopathic steroid-resistant nephrotic syndrome10.4
19sporadic idiopathic steroid-resistant nephrotic syndrome with collapsing glomerulopathy10.4
20anuria10.2
21cerebritis10.2
22systemic lupus erythematosus10.2
23sagittal sinus thrombosis10.2
24proliferative glomerulonephritis10.2
25mesangial proliferative glomerulonephritis10.2
26food allergy10.2
27lupus erythematosus10.2
28lymphopenia10.2
29multiple myeloma10.2
30myeloma10.2
31peritonitis10.2
32thymoma10.2
33dense deposit disease10.2
34nephrotic syndrome, type 210.2
35nephrotic syndrome, type 410.2
36coenzyme q10 deficiency, primary, 610.2
37nephrotic syndrome, type 110.2
38glomerulosclerosis, focal segmental, 410.2
39denys-drash syndrome10.1WT1
40frasier syndrome10.1WT1
41nail-patella syndrome10.0NPHS2, CD2AP
42nephroblastoma10.0WT1, NPHS1
43nephritis10.0NPHS2, NPHS1
44pre-eclampsia10.0NPHS2, NPHS1
45primary hyperoxaluria10.0NPHS2, NPHS1
46diffuse mesangial sclerosis10.0WT1, NPHS2, NPHS1
47lipoid nephrosis10.0CD2AP, NPHS2, NPHS1
48membranous glomerulonephritis10.0CD2AP, NPHS2, NPHS1
49iga glomerulonephritis10.0NPHS1, NPHS2, CD2AP
50diabetic nephropathy10.0NPHS2, NPHS1

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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42NIH Clinical Center, 6ClinicalTrials
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Drug clinical trials:

Search ClinicalTrials for Steroid-Resistant Nephrotic Syndrome

Search NIH Clinical Center for Steroid-Resistant Nephrotic Syndrome

Genetic Tests for Steroid-Resistant Nephrotic Syndrome

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20GeneTests
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Genetic tests related to Steroid-Resistant Nephrotic Syndrome:

id Genetic test Affiliating Genes
1 Steroid-Resistant Nephrotic Syndrome20 NPHS2

Anatomical Context for Steroid-Resistant Nephrotic Syndrome

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33MalaCards
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MalaCards organs/tissues related to Steroid-Resistant Nephrotic Syndrome:

33
Testes, Kidney, Heart, T cells

Animal Models for Steroid-Resistant Nephrotic Syndrome or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Steroid-Resistant Nephrotic Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053788.3WT1, CD2AP, NPHS2, NEIL1
2MP:00053677.5WT1, CD2AP, NPHS2, NPHS1, NEIL1
3MP:00053767.3NEIL1, NPHS1, NPHS2, CD2AP, WT1

Publications for Steroid-Resistant Nephrotic Syndrome

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52PubMed
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Articles related to Steroid-Resistant Nephrotic Syndrome:

(show top 50)    (show all 213)
idTitleAuthorsYear
1
NPHS2 Mutations in Steroid-Resistant Nephrotic Syndrome: A Mutation Update and the Associated Phenotypic Spectrum. (24227627)
2014
2
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
3
A large intracardiac thrombus in a child with steroid-resistant nephrotic syndrome. (22813602)
2013
4
Wilms' tumor suppressor gene mutations in girls with sporadic isolated steroid-resistant nephrotic syndrome. (24338413)
2013
5
NPHS2 mutations in Indian children with sporadic early steroid resistant nephrotic syndrome. (22080622)
2012
6
Steroid-resistant nephrotic syndrome associated with steroid sulfatase deficiency-x-linked recessive ichthyosis: a case report and review of literature. (22419362)
2012
7
Combined renin angiotensin blockade in childhood steroid-resistant nephrotic syndrome. (22621380)
2012
8
Histopathological spectrum of childhood idiopathic steroid-resistant nephrotic syndrome in Tunisia. (21387228)
2011
9
Novel mutations in steroid-resistant nephrotic syndrome diagnosed in Tunisian children. (21125408)
2011
10
Successful treatment of steroid-resistant nephrotic syndrome associated with WT1 mutations. (20191369)
2010
11
Plasmapheresis-induced clinical improvement in a patient with steroid-resistant nephrotic syndrome due to podocin (NPHS2) gene mutation. (21171529)
2010
12
Childhood idiopathic steroid resistant nephrotic syndrome in Southwestern Nigeria. (20814150)
2010
13
Treatment with Cyclophosphamide for steroid-resistant nephrotic syndrome in children. (21977148)
2010
14
Long-term outcome of children with steroid-resistant nephrotic syndrome treated with tacrolimus. (20217433)
2010
15
Successful use of single-dose rituximab for the maintenance of remission in a patient with steroid-resistant nephrotic syndrome. (19881243)
2009
16
Interleukin-18 expression in peripheral blood mononuclear cells in children with steroid-resistant nephrotic syndrome]. (19470251)
2009
17
Experience with tacrolimus in children with steroid-resistant nephrotic syndrome. (19499248)
2009
18
Management and outcome of steroid-resistant nephrotic syndrome in children. (19841524)
2009
19
Clinical and epidemiological assessment of steroid-resistant nephrotic syndrome associated with the NPHS2 R229Q variant. (19145239)
2009
20
Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome. (19714370)
2009
21
Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: a single center study. (19736491)
2009
22
Genetic variations of NPHS2 and WT1 genes in 15 Chinese children with sporadic steroid-resistant nephrotic syndrome]. (19573440)
2009
23
Low prevalence of NPHS2 mutations in African American children with steroid-resistant nephrotic syndrome. (18543005)
2008
24
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
25
Steroid-resistant nephrotic syndrome. (18596732)
2008
26
Treatment of steroid-resistant nephrotic syndrome with cyclosporine: study of 17 cases and a literature review. (16358229)
2005
27
Mutations in NPHS2 in sporadic steroid-resistant nephrotic syndrome in Chinese children. (15769810)
2005
28
Steroid-resistant nephrotic syndrome and NPHS2 gene]. (15833180)
2005
29
Superior sagittal sinus thrombosis in steroid-resistant nephrotic syndrome. (15797188)
2005
30
Varicella-induced remission of steroid-resistant nephrotic syndrome in a child. (17642785)
2004
31
Enalapril dosage in steroid-resistant nephrotic syndrome. (14648339)
2004
32
Pulse cyclophosphamide therapy in steroid resistant nephrotic syndrome associated with mesangial proliferative glomerulonephritis. (17642763)
2004
33
NPHS2 mutation analysis shows genetic heterogeneity of steroid-resistant nephrotic syndrome and low post-transplant recurrence. (15253708)
2004
34
Three siblings with steroid-resistant nephrotic syndrome: new NPHS2 mutations in a Turkish family. (15264208)
2004
35
Cyclosporine in patients with steroid-resistant nephrotic syndrome: an open-label, nonrandomized, retrospective study. (15531003)
2004
36
Short term efficacy of intravenous dexamethasone and methylprednisolone therapy in steroid resistant nephrotic syndrome. (15523124)
2004
37
Efficacy of methylprednisolone pulse therapy in steroid-resistant nephrotic syndrome. (15322892)
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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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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Pathways for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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50PathCards, 55Reactome, 38NCBI BioSystems Database
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Compounds for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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45Novoseek, 24HMDB
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Compounds related to Steroid-Resistant Nephrotic Syndrome according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1puromycin aminonucleoside459.0CD2AP, NPHS2, NPHS1
2lipid458.9CD2AP, NPHS2, NPHS1
3cytosine45 249.9WT1, NEIL1
4creatinine458.9NPHS1, NPHS2, WT1
5steroid458.5WT1, CD2AP, NPHS2, NPHS1

GO Terms for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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16Gene Ontology
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Cellular components related to Steroid-Resistant Nephrotic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1slit diaphragmGO:0360579.4NPHS2, NPHS1
2protein complexGO:0432349.4NPHS2, NPHS1
3cell projectionGO:0429959.2NPHS1, COQ6
4membrane raftGO:0451219.1NPHS2, NPHS1

Biological processes related to Steroid-Resistant Nephrotic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1excretionGO:0075889.4NPHS2, NPHS1
2glomerular basement membrane developmentGO:0328369.1WT1, NPHS1

Molecular functions related to Steroid-Resistant Nephrotic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein C-terminus bindingGO:0080229.1CD2AP, NEIL1

Products for genes affiliated with Steroid-Resistant Nephrotic Syndrome

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Sources for Steroid-Resistant Nephrotic Syndrome

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet