MCID: NPH012
MIFTS: 60

Nephrotic Syndrome malady

Genetic diseases, Nephrological diseases, Rare diseases categories

Aliases & Classifications for Nephrotic Syndrome

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Sources:
9Disease Ontology, 11DISEASES, 43Novoseek, 60UMLS, 55SNOMED-CT, 27ICD9CM, 33MeSH, 38NCIt, 25ICD10
See all sources

Nephrotic Syndrome, Aliases & Descriptions:

Name: Nephrotic Syndrome 9 11 43 60
Finnish Congenital Nephrotic Syndrome 60
 
Finnish Congenital Nephrosis 9


Classifications:

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


External Ids:

Disease Ontology9 DOID:1184
ICD9CM27 581
MeSH33 D009404
NCIt38 C34845
ICD1025 N04, N04.9

Summaries for Nephrotic Syndrome

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Wikipedia:63 Nephrotic syndrome is a nonspecific kidney disorder characterized by a number of signs of disease:... more...

MalaCards based summary: Nephrotic Syndrome, also known as finnish congenital nephrotic syndrome, is related to denys-drash syndrome and frasier syndrome. 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 bendroflumethiazide and bumetanide and the compounds kp-1019 and iopentol have been mentioned in the context of this disorder. Affiliated tissues include kidney, t cells and testes, and related mouse phenotypes are nervous system and endocrine/exocrine gland.

Related Diseases for Nephrotic Syndrome

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Diseases in the Nephrotic Syndrome family:

Nephrotic Syndrome, Type 2 Nephrotic Syndrome, Type 3
Nephrotic Syndrome, Type 4 Nephrotic Syndrome, Type 6
Nephrotic Syndrome, Type 10 Nephrotic Syndrome, Type 7
Nephrotic Syndrome, Type 8 Nephrotic Syndrome, Type 9
Nephrotic Syndrome, Type 1 Familial Nephrotic Syndrome
Hereditary Nephrotic Syndromes Hereditary Nephrotic Syndromes, Autosomal Dominant
Hereditary Nephrotic Syndromes, Autosomal Recessive Lamb2-Related Infantile-Onset Nephrotic Syndrome

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

(show top 50)    (show all 452)
idRelated DiseaseScoreTop Affiliating Genes
1denys-drash syndrome31.3WT1
2frasier syndrome31.2WT1
3diffuse mesangial sclerosis31.2WT1, NPHS2, NPHS1
4chronic pyelonephritis31.1NAGLU
5nail-patella syndrome31.0NPHS2, CD2AP
6nephrosis30.9LAMB2, ACTN4, NPHS1, NPHS2
7membranoproliferative glomerulonephritis30.9NPHS1, CD79A
8congenital nephrotic syndrome finnish type30.8ACTN4, NPHS1, NPHS2, CD2AP, WT1
9nephritis30.7MPV17, ALB, NPHS1, NPHS2, CD79A
10pyelonephritis30.7NAGLU, CD79A
11wilms tumor30.7WT1, NPHS1
12conjunctivitis30.3CD79A
13acute poststreptococcal glomerulonephritis30.3CD79A
14lupus nephritis30.3NAGLU, ALB, CD79A
15interstitial lung disease30.3CD79A
16glomerulonephritis30.2PTPRO, NAGLU, ACTN4, ALB, NPHS1, NPHS2
17glomerulosclerosis30.1PTPRO, MPV17, ACTN4, ALB, NPHS1, NPHS2
18pneumonia29.9CD79A, IL13, NAGLU
19focal segmental glomerulosclerosis29.9WT1, CD79A, CD2AP, NPHS2, PTPRO, NAGLU
20tetanus29.8CD79A, ALB, IL13
21lipoid nephrosis29.7TF, NAGLU, IL13, ALB, NPHS1, NPHS2
22membranous glomerulonephritis, antenatal29.7ALB, NPHS1, NPHS2, CD2AP, CD79A
23iga glomerulonephritis29.6NAGLU, ALB, NPHS1, NPHS2, CD2AP, CD79A
24proteinuria29.4WT1, CD79A, TF, PTPRO, MPV17, CD2AP
25chronic kidney failure28.9TF, NAGLU, ACTN4, ALB, NPHS1, NPHS2
26steroid-resistant nephrotic syndrome11.1
27amyloidosis10.7
28familial nephrotic syndrome10.6
29hepatitis10.6
30galloway-mowat syndrome10.6
31membranous glomerulonephritis10.5
32syphilis10.5
33pierson syndrome10.5
34hyperthyroxinemia10.5ALB
35nephrotic syndrome, type 110.5
36cerebritis10.5
37proliferative glomerulonephritis10.5
38hepatorenal syndrome10.4ALB
39nephrotic syndrome, idiopathic, steroid-resistant10.4
40nephrotic syndrome, type 5, with or without ocular abnormalities10.4
41nephrotic syndrome, type 310.4
42hepatitis b10.4
43secondary syphilis10.4
44peritonitis10.4
45endotheliitis10.4
46nephrotic syndrome, type 410.4
47nephrotic syndrome, type 610.4
48nephrotic syndrome, type 710.4
49interstitial lung disease, nephrotic syndrome, and epidermolysis bullosa, congenital10.4
50schimke immunoosseous dysplasia10.4

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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Genetic Tests for Nephrotic Syndrome

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Anatomical Context for Nephrotic Syndrome

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MalaCards organs/tissues related to Nephrotic Syndrome:

31
Kidney, T cells, Testes, Endothelial, Bone, Thyroid, Liver, Lung, B cells, Heart, Monocytes, Prostate, Neutrophil, Eye, Whole blood, Brain, Spinal cord, Pancreas, Breast, Skin, Testis, Cervix, Appendix, Bone marrow

Animal Models for Nephrotic Syndrome or affiliated genes

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Publications for Nephrotic Syndrome

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Articles related to Nephrotic Syndrome:

(show top 50)    (show all 1697)
idTitleAuthorsYear
1
Defects of CRB2 cause steroid-resistant nephrotic syndrome. (25557779)
2015
2
Change of Chylous Ascites During Low-Density Lipoprotein Apheresis in a Patient With Idiopathic Nephrotic Syndrome. (25256915)
2014
3
Is Two Month Initial Prednisolone Treatment for Nephrotic Syndrome Inferior to Longer Duration Therapy?: Pediatric Nephrologist's Viewpoint. (25362014)
2014
4
PD42 - Is rituximab a trigger for persistent hypogammaglobulinemia in idiopathic nephrotic syndrome? (25045499)
2014
5
Immunosuppressive treatment for idiopathic membranous nephropathy in adults with nephrotic syndrome. (25318831)
2014
6
Close association between proteinuria and regulatory T cells in patients with idiopathic nephrotic syndrome. (23263711)
2013
7
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
8
Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China. (23778422)
2013
9
Role of PCSK9 and IDOL in the pathogenesis of acquired LDL receptor deficiency and hypercholesterolemia in nephrotic syndrome. (24166456)
2013
10
Patterns of childhood nephrotic syndrome in Aljouf region, Saudi Arabia. (24029283)
2013
11
Profound nephrotic syndrome in a patient with ovarian teratoma. (23816730)
2013
12
Association of the ACE-II genotype with the risk of nephrotic syndrome in Pakistani children. (22033511)
2012
13
Long-term outcome of the difficult nephrotic syndrome in children. (22982908)
2012
14
Intra- and postoperative adverse events in children with nephrotic syndrome requiring surgery under general anesthesia. (22142331)
2012
15
NPHS2 mutations in Indian children with sporadic early steroid resistant nephrotic syndrome. (22080622)
2012
16
Acute inferior myocardial infarction with nephrotic syndrome. (21159478)
2012
17
Nephrotic syndrome occurring during tiopronin treatment for cystinuria. (20924604)
2011
18
Successful treatment of steroid-resistant nephrotic syndrome associated with WT1 mutations. (20191369)
2010
19
Clinical features of the nephrotic syndrome associated with ichthyosis vulgaris and analysis of related gene mutation]. (20441703)
2010
20
Successful use of single-dose rituximab for the maintenance of remission in a patient with steroid-resistant nephrotic syndrome. (19881243)
2009
21
Randomized cross-over trial comparing albumin and frusemide infusions in nephrotic syndrome. (19142668)
2009
22
Interleukin-18 expression in peripheral blood mononuclear cells in children with steroid-resistant nephrotic syndrome]. (19470251)
2009
23
Clinical features and long-term outcome of nephrotic syndrome associated with heterozygous NPHS1 and NPHS2 mutations. (19406966)
2009
24
Nephrotic syndrome in diabetic kidney disease: an evaluation and update of the definition. (19556043)
2009
25
Experience with tacrolimus in children with steroid-resistant nephrotic syndrome. (19499248)
2009
26
Congenital nephrotic syndrome. (17968594)
2009
27
Low prevalence of NPHS2 mutations in African American children with steroid-resistant nephrotic syndrome. (18543005)
2008
28
Fetuin A in children with nephrotic syndrome]. (18924506)
2008
29
Podocyte proteoglycan synthesis is involved in the development of nephrotic syndrome. (16622173)
2006
30
Angiotensin-converting enzyme gene polymorphism in children with idiopathic nephrotic syndrome. (16645262)
2006
31
Glucocorticoid receptor and vascular endothelial growth factor in nephrotic syndrome. (16825140)
2006
32
Genetic variations of the NR3C1 gene in children with sporadic nephrotic syndrome. (16890204)
2006
33
Relationship of serum interleukin-7 concentration and the coagulation state in children with nephrotic syndrome. (16091081)
2005
34
Varicella-induced remission of steroid-resistant nephrotic syndrome in a child. (17642785)
2004
35
Apolipoprotein E in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. (12631135)
2003
36
Insulin resistance, dyslipidaemia, inflammation and endothelial function in nephrotic syndrome. (12454236)
2002
37
Congenital nephrotic syndrome. (11261689)
2001
38
Pregnancy outcome in nephrotic syndrome with mixed connective tissue disease. (11598404)
2001
39
Mutation spectrum in the nephrin gene (NPHS1) in congenital nephrotic syndrome. (11317351)
2001
40
The role of lung scintigraphy in the diagnosis of nephrotic syndrome with pulmonary embolism. (10698409)
2000
41
Recombinant human erythropoietin for anaemia in nephrotic syndrome with thalassemia. (9128797)
1997
42
Nephrotic syndrome and concurrent infectious mononucleosis. (9090679)
1997
43
A prospective clinical trial comparing the treatment of idiopathic membranous nephropathy and nephrotic syndrome with simvastatin and diet, versus diet alone. (8905205)
1996
44
Role of renal insufficiency in altered erythropoietin metabolism in the nephrotic syndrome. (1524095)
1992
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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Search GEO for disease gene expression data for Nephrotic Syndrome.

Pathways for genes affiliated with Nephrotic Syndrome

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Pathways related to Nephrotic Syndrome according to GeneCards Suite gene sharing:

(show all 11)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.6CD2AP, NPHS2, NPHS1
29.4PLCE1, ARHGDIA
39.3CD2AP, NPHS1, ACTN4
4
Show member pathways
9.3CD2AP, NPHS1, ACTN4
5
Show member pathways
9.3TF, ACTN4, ALB
6
Show member pathways
8.9PLCE1, IL13, ACTN4, PTPRO
7
Show member pathways
8.9CD79A, PLCE1, ITGA3, LAMB2
8
Show member pathways
8.8TF, ACTN4, ITGA3, ALB
9
Show member pathways
Cytoskeleton remodeling Fibronectin binding integrins in cell motility58
Cytoskeleton remodeling Integrin outside in signaling58
Cytoskeleton remodeling Regulation of actin cytoskeleton by Rho GTPases58
Cell adhesion Tight junctions58
Development MAG dependent inhibition of neurite outgrowth58
8.7ACTN4, ITGA3, ARHGDIA
10
Show member pathways
7.7LAMB2, ACTN4, ITGA3, IL13, PLCE1, ARHGDIA

Compounds for genes affiliated with Nephrotic Syndrome

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Compounds related to Nephrotic Syndrome according to GeneCards Suite gene sharing:

(show top 50)    (show all 62)
idCompoundScoreTop Affiliating Genes
1kp-10194310.2TF, ALB
2iopentol4310.2ALB, NAGLU
3bismuth43 2411.2ALB, TF
4p-aminohippuric acid4310.2ALB, NAGLU
5gelfoam4310.2ALB, TF
6aluminium43 1211.1NAGLU, TF
7suprofen43 1211.1TF, ALB
8inulin4310.1NAGLU, ALB
9iohexol4310.1NAGLU, ALB
10puromycin aminonucleoside4310.0CD2AP, NPHS2, NPHS1
11fructosamine43 2411.0ALB, NAGLU
12ovomucoid439.9IL13, TF
13tetracycline43 1210.9TF, ALB, WT1
14gamma globulin439.8ALB, CD79A
15antipyrine43 1210.8ALB, TF
16diclofenac43 28 49 1212.7NAGLU, IL13, ALB
17ramipril43 1210.6CD79A, ALB
18glutaraldehyde439.6TF, ALB, CD79A
19vitamin b12439.6TF, ALB, CD79A
20alpha tocopherol439.6ALB, IL13, TF
21polyethylene glycol439.5TF, ALB, CD79A
22piroxicam43 1210.5CD79A, ALB
23praziquantel43 1210.5CD79A, IL13
24cyclophosphamide43 49 1211.4NPHS2, IL13, NAGLU, TF
25penicillin439.4TF, IL13, CD79A
26sialic acid439.4CD79A, NAGLU, TF
27vitamin a43 24 1211.3TF, NAGLU, IL13, ALB
28vitamin d439.3NAGLU, ALB, CD79A, WT1
29citrate439.2TF, NAGLU, ALB, CD79A
30methotrexate49 43 1211.2CD79A, ALB, NAGLU, TF
31carbachol43 28 1211.2TF, IL13, CD79A
32indomethacin43 28 59 1212.1CD79A, NPHS1, ALB, IL13
33inositol439.1TF, IL13, CD79A
34prednisolone43 28 1211.1CD79A, ALB, IL13, TF
35thyroxine43 2410.1CD79A, ALB, IL13, TF
36histamine43 28 2411.0CD79A, ALB, IL13, TF
37hyaluronic acid43 249.8NAGLU, IL13, ALB, CD79A, WT1
38vegf438.8TF, IL13, NPHS1, CD79A, WT1
39heparin43 28 24 1211.8CD79A, ALB, IL13, NAGLU, LAMB2
40tacrolimus43 49 1210.7CD79A, ALB, IL13, NAGLU, TF
41lactate438.7TF, NAGLU, IL13, ALB, CD79A
42aspartate438.6ALB, IL13, NAGLU, TF
43cyclosporin a43 28 5910.6TF, NAGLU, IL13, NPHS2, CD79A, WT1
44alanine438.6TF, NAGLU, IL13, ALB, CD79A
45calcium43 49 24 1211.5LAMB2, NAGLU, ACTN4, PLCE1, ALB, NPHS1
46creatinine438.5TF, NAGLU, ALB, NPHS1, NPHS2, CD79A
47retinoic acid43 249.4ITGA3, IL13, NPHS1, CD79A, WT1
48tyrosine438.3PTPRO, ACTN4, ITGA3, IL13, NPHS1, NPHS2
49cysteine438.3NAGLU, IL13, ALB, CD79A, WT1
50steroid438.0WT1, PTPRO, NAGLU, IL13, NPHS1, NPHS2

GO Terms for genes affiliated with Nephrotic Syndrome

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

idNameGO IDScoreTop Affiliating Genes
1slit diaphragmGO:00360579.9NPHS2, NPHS1
2protein complexGO:00432349.2ACTN4, ALB, NPHS1, NPHS2
3membrane raftGO:00451219.1NPHS1, NPHS2, CD79A
4perinuclear region of cytoplasmGO:00484718.7CD2AP, ITGA3, ACTN4, TF
5extracellular vesicular exosomeGO:00700626.2ARHGDIA, TF, PTPRO, LAMB2, NAGLU, ACTN4

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

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1metanephric glomerular visceral epithelial cell developmentGO:007224910.1NPHS2, LAMB2
2glomerular visceral epithelial cell differentiationGO:007211210.0WT1, PTPRO
3glomerular basement membrane developmentGO:00328369.9MPV17, NPHS1, WT1
4glomerulus developmentGO:00328359.9WT1, PTPRO, PLCE1
5retina homeostasisGO:00018959.8ALB, TF
6heart developmentGO:00075079.5WT1, PLCE1, ITGA3
7platelet degranulationGO:00025769.4TF, ACTN4, ALB
8platelet activationGO:00301689.0ALB, ACTN4, TF
9blood coagulationGO:00075968.7TF, ACTN4, ITGA3, ALB

Molecular functions related to Nephrotic Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1integrin bindingGO:00051789.2ITGA3, ACTN4, LAMB2
2protein bindingGO:00055155.5ARHGDIA, TF, PTPRO, ACTN4, ITGA3, IL13

Products for genes affiliated with Nephrotic Syndrome

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Sources for Nephrotic Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet