MCID: NPH018
MIFTS: 44

Nephrogenic Systemic Fibrosis malady

Categories: Rare diseases, Skin diseases, Nephrological diseases

Aliases & Classifications for Nephrogenic Systemic Fibrosis

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Sources:
45NIH Rare Diseases, 51Orphanet, 65UMLS, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Nephrogenic Systemic Fibrosis:

Name: Nephrogenic Systemic Fibrosis 45 51 65
Nephrogenic Fibrosing Dermopathy 45 51 65
 
Nfd 45
Nsf 45

Classifications:

Orphanet: 51 
Rare skin diseases


External Ids:

Orphanet51 137617
UMLS via Orphanet66 C1619692
UMLS65 C1619692, C3888044

Summaries for Nephrogenic Systemic Fibrosis

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NIH Rare Diseases:45 Nephrogenic systemic fibrosis is a condition that affects different parts of the body, particularly the skin. symptoms of the condition may include progressive swelling and tightening of the skin, sometimes resulting in contractures, and pruritis (itching).  the skin findings are similar to those seen in patients with scleroderma.  nephrogenic systemic fibrosis was first described in 1997. being exposed to gadolinium-containing contrast agents during mri testing has been identified as a significant risk factor for development of this disease. last updated: 4/24/2013

MalaCards based summary: Nephrogenic Systemic Fibrosis, also known as nephrogenic fibrosing dermopathy, is related to heart disease and liver disease. An important gene associated with Nephrogenic Systemic Fibrosis is ALB (Albumin), and among its related pathways are Cytokines and Inflammatory Response and EGFR Transactivation by Gastrin. Affiliated tissues include skin, kidney and testes, and related mouse phenotypes are tumorigenesis and renal/urinary system.

Wikipedia:68 Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD) is a rare and serious... more...

Related Diseases for Nephrogenic Systemic Fibrosis

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Diseases related to Nephrogenic Systemic Fibrosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 304)
idRelated DiseaseScoreTop Affiliating Genes
1heart disease30.4ALB, EDN1, EPO
2liver disease29.8ALB, EDN1, TGFB1, TIMP1
3tetanus11.5
4n syndrome11.3
5foodborne botulism11.3
6neuronal intranuclear inclusion disease11.3
7episodic ataxia10.4ALB, TGFB1
8pancreatitis10.4
9somatoform disorder10.4ALB, EPO
10grn-related frontotemporal dementia10.4
11crohn's disease10.4
12breast cancer10.4
13endotheliitis10.4
14congenital adrenal hyperplasia due to apparent combined p450c17 and p450c21 deficiency10.4EDNRA, TGFB1
15kosztolanyi syndrome10.4ALB, EDNRA
16schizophrenia10.4
17lymphoma10.4
18angioid streaks10.3ALB, EPO
19diffuse meningeal melanocytosis10.3ALB, EPO
20blood group incompatibility10.3ALB, EPO
21hepatitis10.3
22tuberculosis10.3
23thyroiditis10.3
24retinitis pigmentosa 1710.3ALB, TGFB1
25discitis10.3ALB, EPO
26leukomelanoderma mental redardation hypotrichosis10.3EPO, SPP1
27elastoderma10.3EDN1, TGFB1
28tenosynovitis of foot and ankle10.3ALB, PIK3CA, TGFB1
29epidermolysis bullosa dystrophica10.3ALB, EPO, TGFB1
30cell type cancer10.3ALB, PIK3CA, TGFB1
31ossifying fibroma10.2ALB, EPO, TGFB1
32lung cancer10.2
33glomerulonephritis10.2
34leukemia10.2
35ischemia10.2
36cerebritis10.2
37myopathy10.2
38neuronitis10.2
39cardiomyopathy10.2
40hypoxia10.2
41marasmus10.2EDN1, TGFB1
42thymus mucoepidermoid carcinoma10.2ALB, EPO, SPP1
43lethal congenital contractural syndrome 210.2EDN1, EPO
44malignant acrospiroma10.2ALB, EPO, TGFB1
45hyperekplexia 2, autosomal recessive10.2EDN1, EDNRA
46hereditary ataxia10.2ALB, PIK3CA, TGFB1
47hiv-110.2
48asthma10.2
49renal cell carcinoma10.2
50myelodysplastic syndrome10.2

Graphical network of the top 20 diseases related to Nephrogenic Systemic Fibrosis:



Diseases related to nephrogenic systemic fibrosis

Symptoms for Nephrogenic Systemic Fibrosis

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Drugs & Therapeutics for Nephrogenic Systemic Fibrosis

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Drugs for Nephrogenic Systemic Fibrosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 10)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1Ferrosoferric OxidePhase 462
2Pharmaceutical SolutionsPhase 47004
3Parenteral Nutrition SolutionsPhase 471
4HematinicsPhase 41121
5Liver ExtractsPhase 33572
6Imatinib MesylatePhase 3, Phase 2577123596
7Protein Kinase InhibitorsPhase 3, Phase 23162
8Plasma-lyte 148Phase 1, Phase 248
9
Iodine4947553-56-2807
Synonyms:
I2
Iode
Iodine-molecule
 
Iodio
Iodum
Jod
Jood
Tincture iodine
10cadexomer iodine456

Interventional clinical trials:

(show all 23)
idNameStatusNCT IDPhase
1Primovist / Eovist in Renally Impaired PatientsCompletedNCT00908596Phase 4
2Risk of Nephrogenic Systemic Fibrosis (NSF) in Patients With Moderate Renal Insufficiency After the Administration of MagnevistCompletedNCT00744939Phase 4
3Safety of Gadovist in Renally Impaired PatientsCompletedNCT00828737Phase 4
4Post-marketing Safety Study in Patients With Moderate Renal Insufficiency Who Receive Omniscan for Contrast-enhanced Magnetic Resonance Imaging (MRI)CompletedNCT00908310Phase 4
5Feraheme As An MRI Contrast Agent For Pediatric Congenital Heart DiseaseRecruitingNCT02752191Phase 4
6Plasmapheresis for Nephrogenic Fibrosing Dermopathy (NFD)/Nephrogenic Systemic Fibrosis (NSF)TerminatedNCT01078987Phase 4
7Treatment of Patients With Nephrogenic Systemic Fibrosis With GlivecCompletedNCT00981942Phase 3
8Efficacy and Safety of Primovist in Chinese PatientsCompletedNCT00526188Phase 3
9Nephrogenic Systemic Fibrosis With GadollinumEnrolling by invitationNCT01359345Phase 2, Phase 3
10The Effect of Crystalloids and Colloids on Visceral Blood FlowCompletedNCT01087853Phase 1, Phase 2
11Pilot Study of Imatinib Mesylate to Treat Nephrogenic Systemic FibrosisActive, not recruitingNCT00677092Phase 2
12Nephrogenic Systemic Fibrosis (NSF): Analysis of Tissue Gadolinium LevelsCompletedNCT01014754
13Validation of a Questionnaire to Identify Signs and Symptoms of Nephrogenic Systemic FibrosisCompletedNCT00869479
14Prospective Evaluation of the Incidence of NSF in Patients With Kidney Disease Undergoing MRCompletedNCT01135316
15Evaluation of the Risk of Nephrogenic Systemic Fibrosis (NSF) in Patients With Chronic Kidney Disease Without Exposure to Gadolinium Based Contrast Agents (GBCA)CompletedNCT00773409
16Evaluation of the Risk of NSF Following ProHance Injection in Patients With Chronic Kidney DiseaseCompletedNCT00600834
17Evaluation of the Risk of NSF Following MultiHance Injection in Patients With Chronic Kidney DiseaseCompletedNCT00600951
18Magnevist Post-marketing Surveillance in JapanCompletedNCT01376739
19Observational Post-marketing Study on the Safety and Efficacy of Dotarem® (SECURE Study)CompletedNCT01523873
20Adverse Reactions to MR and CT-examinations (Enhanced and Unenhanced)CompletedNCT01132339
21Pilot Study of the Effect of Laser on Reversing Chronic Radiation InjuryRecruitingNCT01910818
22Observational Study on the Incidence of NSF in Renal Impaired Patients Following DOTAREM AdministrationActive, not recruitingNCT01467271
23Evaluation of Nephrogenic Systemic Fibrosis (NSF) in Patients With Chronic Kidney Disease Following OptiMARK InjectionSuspendedNCT00811863

Search NIH Clinical Center for Nephrogenic Systemic Fibrosis

Genetic Tests for Nephrogenic Systemic Fibrosis

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Anatomical Context for Nephrogenic Systemic Fibrosis

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MalaCards organs/tissues related to Nephrogenic Systemic Fibrosis:

33
Skin, Kidney, Testes, Eye, Heart, Breast, Bone

Animal Models for Nephrogenic Systemic Fibrosis or affiliated genes

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MGI Mouse Phenotypes related to Nephrogenic Systemic Fibrosis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.3ALB, DCN, PIK3CA, SPP1, TGFB1, TGM2
2MP:00053678.0ALB, DCN, EDN1, EDNRA, SPP1, TGFB1
3MP:00053697.8ALB, DCN, EDN1, EDNRA, PIK3CA, SPP1
4MP:00053797.7ALB, DCN, EDN1, EDNRA, PIK3CA, TGFB1
5MP:00053907.6DCN, EDN1, EDNRA, PIK3CA, SPP1, TGFB1
6MP:00053847.5ALB, DCN, EDNRA, EPO, MAPKAP1, PIK3CA
7MP:00053766.8ALB, DCN, EDN1, EDNRA, EPO, PIK3CA
8MP:00053856.4CXCL2, EDN1, EDNRA, EPO, MAPKAP1, PIK3CA
9MP:00107686.2ALB, DCN, EDN1, EDNRA, EPO, MAPKAP1

Publications for Nephrogenic Systemic Fibrosis

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Articles related to Nephrogenic Systemic Fibrosis:

(show top 50)    (show all 409)
idTitleAuthorsYear
1
Acute chorioamnionitis and funisitis: definition, pathologic features, and clinical significance. (26428501)
2015
2
CYP1B1-mediated Pathobiology of Primary Congenital Glaucoma. (26997841)
2015
3
Summaries of nursing care-related systematic reviews from the cochrane library: pharmacotherapy for mild hypertension. (24309431)
2014
4
Zinc, copper and C-reactive protein in children with severe Plasmodium falciparum malaria in an area of unstable malaria transmission in eastern Sudan. (23104565)
2013
5
Heavy smoking before coronary surgical procedures affects the native matrix metalloproteinase-2 and matrix metalloproteinase-9 gene expression in saphenous vein conduits. (23146278)
2013
6
Moving beyond the 'pancreatic rest' in severe and critical acute pancreatitis. (23837725)
2013
7
Bone mass and vitamin D levels in women with a diagnosis of fibromyalgia. (24008400)
2013
8
Primary repair of broad bilateral complete cleft palate with hard palate rotation flap. (23714885)
2013
9
Molecular phylogeny of broken-back shrimps (genus Lysmata and allies): a test of the 'Tomlinson-Ghiselin' hypothesis explaining the evolution of hermaphroditism. (23727055)
2013
10
Is the Cerebellum the Optimal Reference Region for Intensity Normalization of Perfusion MR Studies in Early Alzheimer's Disease? (24386081)
2013
11
The adverse prognostic hallmarks in identical twins with Langerhans cell histiocytosis: a clinical report and literature review. (23924960)
2013
12
The influence of hyperglycemia on the therapeutic effect of exercise on glycemic control in patients with type 2 diabetes mellitus. (23817567)
2013
13
Relationship between vitreous and serum vascular endothelial growth factor levels, control of diabetes and microalbuminuria in proliferative diabetic retinopathy. (22331976)
2012
14
Suspected serotonin syndrome in a patient being treated with methylene blue for ifosfamide encephalopathy. (22235061)
2012
15
How much of reinforcement learning is working memory, not reinforcement learning? A behavioral, computational, and neurogenetic analysis. (22487033)
2012
16
Stepwise regional and distant progression of recurrent pleomorphic adenoma. (21303593)
2011
17
Granulomatous mastitis: presentation, treatment and outcome in 43 patients. (20569938)
2010
18
Single nucleotide polymorphisms in the FADS gene cluster are associated with delta-5 and delta-6 desaturase activities estimated by serum fatty acid ratios. (20427696)
2010
19
Surface plasmon resonance spectroscopy in determination of the interactions between amyloid beta proteins (Abeta) and lipid membranes. (20217625)
2010
20
PRKCSH genetic mutation was not found in Taiwanese patients with polycystic liver disease. (19308730)
2010
21
Tanespimycin: the opportunities and challenges of targeting heat shock protein 90. (19466875)
2009
22
Does antineoplasm treatment decrease the glomerular filtration rate in children? (19521109)
2009
23
Orbital involvement in langerhans cell histiocytosis. (19935242)
2009
24
Familial Mediterranean fever and IgA nephropathy: case report and review of the literature. (18793551)
2008
25
Alanine scanning of a putative receptor binding surface of insulin-like growth factor-I. (18502759)
2008
26
Hypertrophic osteoarthropathy as the cause of a super scan of the bone in a patient with prostate cancer: a case report. (18394199)
2008
27
Characterization of the prefusion and transition states of severe acute respiratory syndrome coronavirus S2-HR2. (18540634)
2008
28
Multivariate geostatistics and GIS-based approach to study the spatial distribution and sources of heavy metals in agricultural soil in the Pearl River Delta, China]. (19256391)
2008
29
The effect of PPAR ligands to modulate glucose metabolism alters the incorporation of metabolic precursors into proteoglycans synthesized by human vascular smooth muscle cells. (18629682)
2008
30
KAP1-mediated epigenetic repression in the forebrain modulates behavioral vulnerability to stress. (19081377)
2008
31
Coronary heart disease and cardiovascular autonomic neuropathy in the elderly diabetic. (17702096)
2007
32
The association between vascular endothelial growth factor-C, its corresponding receptor, VEGFR-3, and prognosis in primary breast cancer: a study with 193 cases. (16465426)
2006
33
First report of prevalence of non-syndromic hereditary prosopagnosia (HPA). (16817175)
2006
34
Duox2 exhibits potent heme peroxidase activity in human respiratory tract epithelium. (16970942)
2006
35
Chemically modified thrombin and anhydrothrombin that differentiate macromolecular substrates of thrombin. (16246253)
2005
36
Functional implications of a novel EA2 mutation in the P/Q-type calcium channel. (15293273)
2004
37
Molecular characterization and mapping of the canine KRAB zinc finger gene ZNF331. (15147412)
2004
38
Current trend in ivermectin usage for scabies. (12884462)
2003
39
Role of superoxide in poly(ADP-ribose) polymerase upregulation after transient cerebral ischemia. (12750003)
2003
40
Lhx9: a novel LIM-homeodomain gene expressed in the developing forebrain. (9880598)
1999
41
Posterior chamber collamer phakic intraocular lens for myopia and hyperopia. (10445712)
1999
42
Optic nerve head neovascularization in a patient with inactive cytomegalovirus retinitis and immune recovery. (9727534)
1998
43
Self-"treatment" for laryngeal papillomatosis. (9018927)
1997
44
Positron emission tomography with oxygen-15 of stunned myocardium caused by coronary artery vasospasm after recovery. (7888266)
1995
45
Reaction of lysyl oxidase with trans-2-phenylcyclopropylamine. (8099355)
1993
46
Human insulin-like growth factor binding protein-6 is O-glycosylated. (7683533)
1993
47
Malignant fibrous histiocytomas in dogs and cats: an immunohistochemical study. (1332152)
1992
48
The role of endoscopic papillotomy in ascending cholangitis. (2053744)
1991
49
Use of emergency medical services by patients with decompensated obstructive lung disease. (2719362)
1989
50
Pseudohypoparathyroidism associated with hypercalcitoninaemia. (7355665)
1980

Variations for Nephrogenic Systemic Fibrosis

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Expression for genes affiliated with Nephrogenic Systemic Fibrosis

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Search GEO for disease gene expression data for Nephrogenic Systemic Fibrosis.

Pathways for genes affiliated with Nephrogenic Systemic Fibrosis

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Pathways related to Nephrogenic Systemic Fibrosis according to GeneCards Suite gene sharing:

(show all 16)
idSuper pathwaysScoreTop Affiliating Genes
19.7CXCL2, TGFB1
2
Show member pathways
9.5EDN1, EDNRA
39.5EDN1, EDNRA
49.4EDN1, PIK3CA, TGFB1
59.4EDN1, PIK3CA, TGFB1
6
Show member pathways
9.4EPO, PIK3CA, TIMP1
7
Show member pathways
9.4EPO, PIK3CA, TIMP1
89.4EPO, PIK3CA, TIMP1
99.3DCN, SPP1, TGFB1
109.2EDN1, EPO, TIMP1
119.2EDN1, EPO, TIMP1
129.1CXCL2, EDN1, PIK3CA
139.1EPO, PIK3CA, SPP1, TIMP1
148.8EDN1, EPO, PIK3CA, TIMP1
15
Show member pathways
8.8DCN, SPP1, TGFB1, TIMP1
16
Show member pathways
7.5ALB, DCN, EDNRA, MAPKAP1, PIK3CA, TGFB1

GO Terms for genes affiliated with Nephrogenic Systemic Fibrosis

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Biological processes related to Nephrogenic Systemic Fibrosis according to GeneCards Suite gene sharing:

(show all 20)
idNameGO IDScoreTop Affiliating Genes
1histamine secretionGO:000182110.2EDN1, EDNRA
2cell activationGO:000177510.2TGFB1, TIMP1
3positive regulation of odontogenesisGO:004248210.1EDN1, EDNRA
4artery smooth muscle contractionGO:001482410.1EDN1, EDNRA
5neural crest cell developmentGO:001403210.1EDN1, EDNRA
6maternal process involved in parturitionGO:006013710.1EDN1, EDNRA
7vasoconstrictionGO:004231010.1EDN1, EDNRA
8phosphatidylinositol 3-kinase signalingGO:001406510.1EDN1, PIK3CA
9respiratory gaseous exchangeGO:000758510.1EDN1, EDNRA
10response to testosteroneGO:003357410.0EDN1, EPO
11regulation of blood pressureGO:000821710.0EDN1, EDNRA
12platelet activationGO:003016810.0ALB, PIK3CA, TGFB1
13positive regulation of neutrophil chemotaxisGO:00900239.9CXCL2, EDNRA
14response to vitamin DGO:00332809.9SPP1, TGFB1
15protein kinase C-activating G-protein coupled receptor signaling pathwayGO:00072059.8EDN1, EDNRA
16negative regulation of cAMP biosynthetic processGO:00308189.8EDN1, EDNRA
17response to organic substanceGO:00100339.7ALB, SPP1, TGFB1
18agingGO:00075689.5DCN, EDNRA, TGFB1
19extracellular matrix disassemblyGO:00226179.3DCN, SPP1, TIMP1
20response to lipopolysaccharideGO:00324968.2CXCL2, DCN, EDN1, EPO

Sources for Nephrogenic Systemic Fibrosis

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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