MCID: NPH012
MIFTS: 68

Nephrotic Syndrome malady

Genetic diseases, Nephrological diseases, Rare diseases categories

Summaries for Nephrotic Syndrome

About this section


Wikipedia:65 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 nephrosis, 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 testes, and related mouse phenotypes are nervous system and endocrine/exocrine gland.

Aliases & Classifications for Nephrotic Syndrome

About this section
Sources:
10Disease Ontology, 12DISEASES, 45Novoseek, 62UMLS, 57SNOMED-CT, 29ICD9CM, 35MeSH, 40NCIt, 27ICD10
See all sources

Nephrotic Syndrome, Aliases & Descriptions:

Name: Nephrotic Syndrome 10 12 45 62
 
Finnish Congenital Nephrosis 10


Classifications:

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


External Ids:

Disease Ontology10 DOID:1184
ICD9CM29 581
MeSH35 D009404
NCIt40 C34845
ICD1027 N04, N04.9

Related Diseases for Nephrotic Syndrome

About this section

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/GeneDecks gene sharing:

(show top 50)    (show all 452)
idRelated DiseaseScoreTop Affiliating Genes
1glomerulonephritis31.1PTPRO, NAGLU, ACTN4, ALB, NPHS1, NPHS2
2glomerulosclerosis31.1PTPRO, MPV17, ACTN4, ALB, NPHS1, NPHS2
3focal segmental glomerulosclerosis30.9WT1, CD79A, CD2AP, NPHS2, PTPRO, NAGLU
4congenital nephrotic syndrome finnish type30.9ACTN4, NPHS1, NPHS2, CD2AP, WT1
5nephritis30.8MPV17, ALB, NPHS1, NPHS2, CD79A
6diffuse mesangial sclerosis30.8WT1, NPHS2, NPHS1
7nephrosis30.7LAMB2, ACTN4, NPHS1, NPHS2
8proteinuria30.7WT1, CD79A, TF, PTPRO, MPV17, CD2AP
9lipoid nephrosis30.7TF, NAGLU, IL13, ALB, NPHS1, NPHS2
10denys-drash syndrome30.6WT1
11nail-patella syndrome30.6NPHS2, CD2AP
12frasier syndrome30.6WT1
13membranoproliferative glomerulonephritis30.6NPHS1, CD79A
14pyelonephritis30.4NAGLU, CD79A
15chronic pyelonephritis30.4NAGLU
16wilms tumor30.4WT1, NPHS1
17lupus nephritis30.2NAGLU, ALB, CD79A
18pneumonia30.1CD79A, IL13, NAGLU
19iga glomerulonephritis30.0NAGLU, ALB, NPHS1, NPHS2, CD2AP, CD79A
20conjunctivitis29.9CD79A
21acute poststreptococcal glomerulonephritis29.9CD79A
22interstitial lung disease29.9CD79A
23tetanus29.9CD79A, ALB, IL13
24membranous glomerulonephritis, antenatal29.8ALB, NPHS1, NPHS2, CD2AP, CD79A
25chronic kidney failure29.8TF, NAGLU, ACTN4, ALB, NPHS1, NPHS2
26steroid-resistant nephrotic syndrome11.1
27amyloidosis10.7
28familial nephrotic syndrome10.6
29hepatitis10.6
30galloway-mowat syndrome10.6
31membranous glomerulonephritis10.6
32syphilis10.5
33pierson syndrome10.5
34cerebritis10.5
35proliferative glomerulonephritis10.5
36nephrotic syndrome, type 110.4
37hepatitis b10.4
38secondary syphilis10.4
39peritonitis10.4
40nephrotic syndrome, idiopathic, steroid-resistant10.4
41endotheliitis10.4
42nephrotic syndrome, type 5, with or without ocular abnormalities10.4
43nephrotic syndrome, type 310.4
44nephrotic syndrome, type 410.4
45nephrotic syndrome, type 610.4
46nephrotic syndrome, type 710.4
47schimke immunoosseous dysplasia10.4
48strongyloidiasis10.4
49microcephaly10.4
50hypothyroidism10.4

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



Diseases related to nephrotic syndrome

Symptoms for Nephrotic Syndrome

About this section

Drugs & Therapeutics for Nephrotic Syndrome

About this section

Genetic Tests for Nephrotic Syndrome

About this section

Anatomical Context for Nephrotic Syndrome

About this section

MalaCards organs/tissues related to Nephrotic Syndrome:

33
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

About this section

Publications for Nephrotic Syndrome

About this section

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

About this section

Expression for genes affiliated with Nephrotic Syndrome

About this section

Search GEO for disease gene expression data for Nephrotic Syndrome.

Pathways for genes affiliated with Nephrotic Syndrome

About this section

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

About this section

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 2611.2ALB, TF
4p-aminohippuric acid4510.2ALB, NAGLU
5gelfoam4510.2ALB, TF
6aluminium45 1311.1NAGLU, TF
7suprofen45 1311.1TF, ALB
8inulin4510.1NAGLU, ALB
9iohexol4510.1NAGLU, ALB
10puromycin aminonucleoside4510.0CD2AP, NPHS2, NPHS1
11fructosamine45 2611.0ALB, NAGLU
12ovomucoid459.9IL13, TF
13tetracycline45 1310.9TF, ALB, WT1
14gamma globulin459.8ALB, CD79A
15antipyrine45 1310.8ALB, TF
16diclofenac45 30 51 1312.7NAGLU, IL13, ALB
17ramipril45 1310.6CD79A, ALB
18glutaraldehyde459.6TF, ALB, CD79A
19vitamin b12459.6TF, ALB, CD79A
20alpha tocopherol459.6ALB, IL13, TF
21polyethylene glycol459.5TF, ALB, CD79A
22piroxicam45 1310.5CD79A, ALB
23praziquantel45 1310.5CD79A, IL13
24cyclophosphamide45 51 1311.4NPHS2, IL13, NAGLU, TF
25penicillin459.4TF, IL13, CD79A
26sialic acid459.4CD79A, NAGLU, TF
27vitamin a45 26 1311.3TF, NAGLU, IL13, ALB
28vitamin d459.3NAGLU, ALB, CD79A, WT1
29citrate459.2TF, NAGLU, ALB, CD79A
30methotrexate51 45 1311.2CD79A, ALB, NAGLU, TF
31carbachol45 30 1311.2TF, IL13, CD79A
32indomethacin45 30 61 1312.1CD79A, NPHS1, ALB, IL13
33inositol459.1TF, IL13, CD79A
34prednisolone45 30 1311.1CD79A, ALB, IL13, TF
35thyroxine45 2610.1CD79A, ALB, IL13, TF
36histamine45 30 2611.0CD79A, ALB, IL13, TF
37hyaluronic acid45 269.8NAGLU, IL13, ALB, CD79A, WT1
38vegf458.8TF, IL13, NPHS1, CD79A, WT1
39heparin45 30 26 1311.8CD79A, ALB, IL13, NAGLU, LAMB2
40tacrolimus45 51 1310.7CD79A, ALB, IL13, NAGLU, TF
41lactate458.7TF, NAGLU, IL13, ALB, CD79A
42aspartate458.6ALB, IL13, NAGLU, TF
43cyclosporin a45 30 6110.6TF, NAGLU, IL13, NPHS2, CD79A, WT1
44alanine458.6TF, NAGLU, IL13, ALB, CD79A
45calcium45 51 26 1311.5LAMB2, NAGLU, ACTN4, PLCE1, ALB, NPHS1
46creatinine458.5TF, NAGLU, ALB, NPHS1, NPHS2, CD79A
47retinoic acid45 269.4ITGA3, 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

About this section

Cellular components related to Nephrotic Syndrome according to GeneCards/GeneDecks:

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:00484718.7ACTN4, ITGA3, CD2AP, TF
5extracellular vesicular exosomeGO:00700626.2TF, ARHGDIA, NPHS2, NPHS1, ALB, ITGA3

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

(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/GeneDecks:

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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Nephrotic Syndrome

About this section
4CDC
15ExPASy
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet