MCID: NPH012
MIFTS: 66

Nephrotic Syndrome malady

Genetic diseases, Nephrological diseases, Rare diseases, Immune diseases categories
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Summaries for Nephrotic Syndrome

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65Wikipedia, 33MalaCards
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Wikipedia:65 Nephrotic syndrome is a nonspecific kidney disorder characterised by a number of signs of disease:... more...

MalaCards: Nephrotic Syndrome is related to glomerulonephritis and glomerulosclerosis. An important gene associated with Nephrotic Syndrome is NPHS2 (nephrosis 2, idiopathic, steroid-resistant (podocin)), and among its related pathways are Selected targets of C EBPalpha and Nephrin/Neph1 signaling in the kidney podocyte. The drugs cyclophosphamide and bendroflumethiazide and the compounds kp-1019 and iopentol have been mentioned in the context of this disorder. Affiliated tissues include kidney, t cells and endothelial, and related mouse phenotypes are nervous system and endocrine/exocrine gland.

Aliases & Classifications for Nephrotic Syndrome

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8Disease Ontology, 10DISEASES, 45Novoseek, 62UMLS, 58SNOMED-CT, 35MeSH, 40NCIt, 27ICD9CM, 25ICD10
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Classifications:



Aliases & Descriptions:

nephrotic syndrome 8 10 45 62


External Ids:

Disease Ontology8 DOID:1184
MeSH35 D009404
NCIt40 C34845
ICD9CM27 581
ICD1025 N04, N04.9

Related Diseases for Nephrotic Syndrome

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17GeneCards, 18GeneDecks
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Diseases in the Nephrotic Syndrome, Type 1 family:

Familial Nephrotic Syndrome nephrotic syndrome
Nephrotic Syndrome Type 3 Hereditary Nephrotic Syndromes
Nephrotic Syndrome, Type 2 Nephrotic Syndrome, Type 4
Nephrotic Syndrome, Type 6 Nephrotic Syndrome, Type 10
Nephrotic Syndrome, Type 7 Nephrotic Syndrome, Type 8
Nephrotic Syndrome, Type 9

Diseases related to Nephrotic Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 443)
idRelated DiseaseScoreTop Affiliating Genes
1glomerulonephritis31.0PTPRO, NAGLU, ACTN4, ALB, NPHS1, NPHS2
2glomerulosclerosis30.9PTPRO, MPV17, ACTN4, ALB, NPHS1, NPHS2
3diffuse mesangial sclerosis30.8WT1, NPHS2, NPHS1
4congenital nephrotic syndrome finnish type30.8ACTN4, NPHS1, NPHS2, CD2AP, WT1
5nephritis30.8MPV17, ALB, NPHS1, NPHS2, CD79A
6focal segmental glomerulosclerosis30.8WT1, CD79A, CD2AP, NPHS2, PTPRO, NAGLU
7membranous glomerulonephritis30.8ALB, NPHS1, NPHS2, CD2AP, CD79A
8hypertension30.7MPV17, ALB, CD79A
9nephrosis30.7LAMB2, ACTN4, NPHS1, NPHS2
10denys-drash syndrome30.6WT1
11proteinuria30.5WT1, CD79A, TF, PTPRO, MPV17, CD2AP
12membranoproliferative glomerulonephritis30.5NPHS1, CD79A
13frasier syndrome30.5WT1
14nail-patella syndrome30.5NPHS2, CD2AP
15chronic pyelonephritis30.5NAGLU
16pyelonephritis30.5NAGLU, CD79A
17lipoid nephrosis30.5TF, NAGLU, IL13, ALB, NPHS1, NPHS2
18diabetes mellitus30.4TF, NAGLU, ALB, NPHS1, CD79A
19lupus nephritis30.2NAGLU, ALB, CD79A
20wilms tumor30.2PTPRO, ACTN4, NPHS1, NPHS2, CD2AP, WT1
21pneumonia30.2CD79A, IL13, NAGLU
22nephroblastoma30.2WT1, NPHS1
23diabetic nephropathy30.1NPHS2, NPHS1, ALB, NAGLU, TF
24iga glomerulonephritis30.0NAGLU, ALB, NPHS1, NPHS2, CD2AP, CD79A
25conjunctivitis30.0CD79A
26acute poststreptococcal glomerulonephritis30.0CD79A
27tetanus29.9CD79A, ALB, IL13
28chronic kidney failure29.6TF, NAGLU, ACTN4, ALB, NPHS1, NPHS2
29steroid-resistant nephrotic syndrome11.1
30amyloidosis10.6
31hepatitis10.5
32familial nephrotic syndrome10.5
33syphilis10.5
34pierson syndrome10.5
35thromboembolism10.5
36proliferative glomerulonephritis10.4
37cerebritis10.4
38microcephaly, hiatal hernia and nephrotic syndrome10.4
39nephrotic syndrome, type 110.4
40peritonitis10.4
41endotheliitis10.4
42hepatitis b10.4
43nephrotic syndrome, type 5, with or without ocular abnormalities10.4
44strongyloidiasis10.4
45secondary syphilis10.4
46hypothyroidism10.4
47interstitial nephritis10.4
48nephrotic syndrome type 310.4
49microcephaly10.3
50nephrocalcinosis10.3

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



Diseases related to nephrotic syndrome

Symptoms for Nephrotic Syndrome

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Drugs & Therapeutics for Nephrotic Syndrome

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Genetic Tests for Nephrotic Syndrome

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Anatomical Context for Nephrotic Syndrome

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33MalaCards
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MalaCards organs/tissues related to Nephrotic Syndrome:

33
Kidney, T cells, Endothelial, Testes, Thyroid, Bone, Liver, Lung, Heart, B cells, Monocytes, Prostate, Cervix, Eye, Spinal cord, Testis, Skin, Breast, Whole blood, Pancreas, Brain, Bone marrow

Animal Models for Nephrotic Syndrome or affiliated genes

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37MGI
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Publications for Nephrotic Syndrome

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52PubMed
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Articles related to Nephrotic Syndrome:

(show top 50)    (show all 1593)
idTitleAuthorsYear
1
Close association between proteinuria and regulatory T cells in patients with idiopathic nephrotic syndrome. (23263711)
2013
2
Letter to the editor regarding 'Youssef et al.'s multi-drug resistance-1 gene polymorphisms in nephrotic syndrome: Impact on susceptibility and response to steroids' (24374471)
2013
3
Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China. (23778422)
2013
4
Patterns of childhood nephrotic syndrome in Aljouf region, Saudi Arabia. (24029283)
2013
5
Profound nephrotic syndrome in a patient with ovarian teratoma. (23816730)
2013
6
Association of the ACE-II genotype with the risk of nephrotic syndrome in Pakistani children. (22033511)
2012
7
Long-term outcome of the difficult nephrotic syndrome in children. (22982908)
2012
8
Intra- and postoperative adverse events in children with nephrotic syndrome requiring surgery under general anesthesia. (22142331)
2012
9
NPHS2 mutations in Indian children with sporadic early steroid resistant nephrotic syndrome. (22080622)
2012
10
Acute inferior myocardial infarction with nephrotic syndrome. (21159478)
2012
11
Nephrotic syndrome occurring during tiopronin treatment for cystinuria. (20924604)
2011
12
Autosomal dominant polycystic kidney disease (ADPKD) associated with steroid-sensitive nephrotic syndrome in childhood. (21116827)
2011
13
Successful treatment of steroid-resistant nephrotic syndrome associated with WT1 mutations. (20191369)
2010
14
Clinical features of the nephrotic syndrome associated with ichthyosis vulgaris and analysis of related gene mutation]. (20441703)
2010
15
Successful use of single-dose rituximab for the maintenance of remission in a patient with steroid-resistant nephrotic syndrome. (19881243)
2009
16
Randomized cross-over trial comparing albumin and frusemide infusions in nephrotic syndrome. (19142668)
2009
17
Interleukin-18 expression in peripheral blood mononuclear cells in children with steroid-resistant nephrotic syndrome]. (19470251)
2009
18
Clinical features and long-term outcome of nephrotic syndrome associated with heterozygous NPHS1 and NPHS2 mutations. (19406966)
2009
19
Nephrotic syndrome in diabetic kidney disease: an evaluation and update of the definition. (19556043)
2009
20
Experience with tacrolimus in children with steroid-resistant nephrotic syndrome. (19499248)
2009
21
Congenital nephrotic syndrome. (17968594)
2009
22
Low prevalence of NPHS2 mutations in African American children with steroid-resistant nephrotic syndrome. (18543005)
2008
23
Fetuin A in children with nephrotic syndrome]. (18924506)
2008
24
Podocyte proteoglycan synthesis is involved in the development of nephrotic syndrome. (16622173)
2006
25
Angiotensin-converting enzyme gene polymorphism in children with idiopathic nephrotic syndrome. (16645262)
2006
26
Glucocorticoid receptor and vascular endothelial growth factor in nephrotic syndrome. (16825140)
2006
27
Genetic variations of the NR3C1 gene in children with sporadic nephrotic syndrome. (16890204)
2006
28
Relationship of serum interleukin-7 concentration and the coagulation state in children with nephrotic syndrome. (16091081)
2005
29
Polymorphism of the interleukin-4, interleukin-13, and signal transducer and activator of transcription 6 genes in Indonesian children with minimal change nephrotic syndrome. (15687724)
2005
30
Varicella-induced remission of steroid-resistant nephrotic syndrome in a child. (17642785)
2004
31
Secondary IgA nephropathy presenting as nephrotic syndrome with glomerular crescentic changes and acute renal failure in a patient with autoimmune hepatitis. (15151269)
2004
32
Apolipoprotein E in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. (12631135)
2003
33
Insulin resistance, dyslipidaemia, inflammation and endothelial function in nephrotic syndrome. (12454236)
2002
34
Congenital nephrotic syndrome. (11261689)
2001
35
Pregnancy outcome in nephrotic syndrome with mixed connective tissue disease. (11598404)
2001
36
Mutation spectrum in the nephrin gene (NPHS1) in congenital nephrotic syndrome. (11317351)
2001
37
The role of lung scintigraphy in the diagnosis of nephrotic syndrome with pulmonary embolism. (10698409)
2000
38
Recombinant human erythropoietin for anaemia in nephrotic syndrome with thalassemia. (9128797)
1997
39
Nephrotic syndrome and concurrent infectious mononucleosis. (9090679)
1997
40
A prospective clinical trial comparing the treatment of idiopathic membranous nephropathy and nephrotic syndrome with simvastatin and diet, versus diet alone. (8905205)
1996
41
Steroid resistant nephrotic syndrome associated with spondyloepiphyseal dysplasia, transient ischemic attacks and lymphopenia. (7736684)
1995
42
Role of renal insufficiency in altered erythropoietin metabolism in the nephrotic syndrome. (1524095)
1992
43
Lovastatin in the treatment of hypercholesterolemia in nephrotic syndrome due to diabetic nephropathy stage IV-V. (1638777)
1992
44
rT3 metabolism in patients with nephrotic syndrome and normal GFR compared with normal subjects. (2018127)
1991
45
Transgenic mouse model of kidney disease: insertional inactivation of ubiquitously expressed gene leads to nephrotic syndrome. (1696177)
1990
46
Nephrotic syndrome in two members of a family with mercury poisoning. (2136288)
1990
47
Neonatal cytomegalovirus infection with pancreatic cystadenoma and nephrotic syndrome. (3035523)
1986
48
Renal amyloidosis with nephrotic syndrome in a child suspected of having idiopathic pulmonary hemosiderosis. (617983)
1977
49
Nephrotic syndrome and secondary syphilis. (1175490)
1975
50
Nephrotic syndrome: a complication of secondary syphilis. (5124440)
1971

Variations for Nephrotic Syndrome

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Expression for genes affiliated with Nephrotic Syndrome

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Nephrotic Syndrome

Search GEO for disease gene expression data for Nephrotic Syndrome.

Pathways for genes affiliated with Nephrotic Syndrome

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50PathCards, 12EMD Millipore, 38NCBI BioSystems Database, 60Thomson Reuters, 55Reactome, 53QIAGEN
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Pathways related to Nephrotic Syndrome according to GeneCards/GeneDecks:

(show all 11)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8ALB, TF
29.6CD2AP, NPHS2, NPHS1
39.4PLCE1, ARHGDIA
49.3CD2AP, NPHS1, ACTN4
5
Show member pathways
9.3CD2AP, NPHS1, ACTN4
6
Show member pathways
9.3TF, ACTN4, ALB
7
Show member pathways
8.9PLCE1, IL13, ACTN4, PTPRO
8
Show member pathways
8.9CD79A, PLCE1, ITGA3, LAMB2
9
Show member pathways
8.8TF, ACTN4, ITGA3, ALB
10
Show member pathways
Cytoskeleton remodeling Fibronectin binding integrins in cell motility60
Cytoskeleton remodeling Integrin outside in signaling60
Cytoskeleton remodeling Regulation of actin cytoskeleton by Rho GTPases60
Cell adhesion Tight junctions60
Development MAG dependent inhibition of neurite outgrowth60
8.7ACTN4, ITGA3, ARHGDIA
11
Show member pathways
7.7LAMB2, ACTN4, ITGA3, IL13, PLCE1, ARHGDIA

Compounds for genes affiliated with Nephrotic Syndrome

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45Novoseek, 24HMDB, 11DrugBank, 51PharmGKB, 29IUPHAR, 61Tocris Bioscience
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Compounds related to Nephrotic Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 62)
idCompoundScoreTop Affiliating Genes
1kp-10194510.2TF, ALB
2iopentol4510.2ALB, NAGLU
3bismuth45 2411.2ALB, TF
4p-aminohippuric acid4510.2ALB, NAGLU
5gelfoam4510.2ALB, TF
6aluminium45 1111.1NAGLU, TF
7suprofen45 1111.1TF, ALB
8inulin4510.1NAGLU, ALB
9iohexol4510.1NAGLU, ALB
10puromycin aminonucleoside4510.0CD2AP, NPHS2, NPHS1
11fructosamine45 2411.0ALB, NAGLU
12ovomucoid459.9IL13, TF
13tetracycline45 1110.9TF, ALB, WT1
14gamma globulin459.8ALB, CD79A
15perindopril45 51 1111.8ALB, NAGLU
16diclofenac45 29 51 1112.7NAGLU, IL13, ALB
17ramipril45 1110.6CD79A, ALB
18glutaraldehyde459.6TF, ALB, CD79A
19vitamin b12459.6TF, ALB, CD79A
20alpha tocopherol459.6ALB, IL13, TF
21polyethylene glycol459.5TF, ALB, CD79A
22piroxicam45 1110.5CD79A, ALB
23praziquantel45 1110.5CD79A, IL13
24cyclophosphamide45 51 1111.4NPHS2, IL13, NAGLU, TF
25penicillin459.4TF, IL13, CD79A
26sialic acid459.4CD79A, NAGLU, TF
27vitamin a45 24 1111.3TF, NAGLU, IL13, ALB
28vitamin d459.3NAGLU, ALB, CD79A, WT1
29citrate459.2TF, NAGLU, ALB, CD79A
30methotrexate51 45 1111.2CD79A, ALB, NAGLU, TF
31carbachol45 29 1111.2TF, IL13, CD79A
32indomethacin45 29 61 1112.1CD79A, NPHS1, ALB, IL13
33inositol459.1TF, IL13, CD79A
34prednisolone45 29 1111.1CD79A, ALB, IL13, TF
35thyroxine45 2410.1CD79A, ALB, IL13, TF
36histamine45 29 2411.0CD79A, ALB, IL13, TF
37hyaluronic acid45 249.8NAGLU, IL13, ALB, CD79A, WT1
38vegf458.8TF, IL13, NPHS1, CD79A, WT1
39heparin45 29 24 1111.8CD79A, ALB, IL13, NAGLU, LAMB2
40tacrolimus45 51 1110.7CD79A, ALB, IL13, NAGLU, TF
41lactate458.7TF, NAGLU, IL13, ALB, CD79A
42aspartate458.6ALB, IL13, NAGLU, TF
43cyclosporin a45 29 6110.6TF, NAGLU, IL13, NPHS2, CD79A, WT1
44alanine458.6TF, NAGLU, IL13, ALB, CD79A
45calcium45 51 24 1111.5LAMB2, NAGLU, ACTN4, PLCE1, ALB, NPHS1
46creatinine458.5TF, NAGLU, ALB, NPHS1, NPHS2, CD79A
47retinoic acid45 249.4ITGA3, IL13, NPHS1, CD79A, WT1
48tyrosine458.3PTPRO, ACTN4, ITGA3, IL13, NPHS1, NPHS2
49cysteine458.3NAGLU, IL13, ALB, CD79A, WT1
50steroid458.0WT1, PTPRO, NAGLU, IL13, NPHS1, NPHS2

GO Terms for genes affiliated with Nephrotic Syndrome

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

idNameGO IDScoreTop Affiliating Genes
1slit diaphragmGO:0360579.9NPHS2, NPHS1
2protein complexGO:0432349.2ACTN4, ALB, NPHS1, NPHS2
3membrane raftGO:0451219.1NPHS1, NPHS2, CD79A
4perinuclear region of cytoplasmGO:0484718.7CD2AP, ITGA3, ACTN4, TF
5extracellular vesicular exosomeGO:0700626.2ARHGDIA, TF, PTPRO, LAMB2, NAGLU, ACTN4

Biological processes related to Nephrotic Syndrome according to GeneCards/GeneDecks:

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1metanephric glomerular visceral epithelial cell developmentGO:07224910.1NPHS2, LAMB2
2glomerular visceral epithelial cell differentiationGO:07211210.0WT1, PTPRO
3glomerular basement membrane developmentGO:0328369.9MPV17, NPHS1, WT1
4glomerulus developmentGO:0328359.9WT1, PTPRO, PLCE1
5retina homeostasisGO:0018959.8ALB, TF
6heart developmentGO:0075079.5WT1, PLCE1, ITGA3
7platelet degranulationGO:0025769.4TF, ACTN4, ALB
8platelet activationGO:0301689.0ALB, ACTN4, TF
9blood coagulationGO:0075968.7TF, ACTN4, ITGA3, ALB

Molecular functions related to Nephrotic Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1integrin bindingGO:0051789.2ITGA3, ACTN4, LAMB2
2protein bindingGO:0055155.5ARHGDIA, TF, PTPRO, ACTN4, ITGA3, IL13

Products for genes affiliated with Nephrotic Syndrome

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Sources for 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