MCID: NDL013
MIFTS: 49

Nodular Regenerative Hyperplasia malady

Liver category

Summaries for Nodular Regenerative Hyperplasia

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64Wikipedia, 33MalaCards
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Wikipedia:64 Nodular regenerative hyperplasia is a form of liver hyperplasia associated with portal hypertension. more...

MalaCards: Nodular Regenerative Hyperplasia, also known as nodular regenerative hyperplasia of the liver, is related to systemic lupus erythematosus and vasculitis, and has symptoms including hepatocellular liver disease/hepatic failureand chronic arterial hypertension. An important gene associated with Nodular Regenerative Hyperplasia is GFER (growth factor, augmenter of liver regeneration), and among its related pathways are Pre-NOTCH Processing in Golgi and Post-transcriptional Silencing By Small RNAs. The compounds propylthiouracil and aspartate have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, liver and breast, and related mouse phenotypes are vision/eye and endocrine/exocrine gland.

Aliases & Classifications for Nodular Regenerative Hyperplasia

Sources:
43NIH Rare Diseases, 49Orphanet, 26ICD10 via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Liver


Characteristics (Orphanet epidemiological data):

49

Aliases & Descriptions:

nodular regenerative hyperplasia 43
nodular regenerative hyperplasia of the liver 43 49
non-cirrhotic nodulation 43 49
non-cirrhotic nodular transformation 43
miliary hepatocellular adenomatosis 43
non-cirrhotic portal hypertension 43


External Ids:

ICD10 via Orphanet26 K76.8

Related Diseases for Nodular Regenerative Hyperplasia

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Nodular Regenerative Hyperplasia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 147)
idRelated DiseaseScoreTop Affiliating Genes
1systemic lupus erythematosus30.8TPMT, F3, IL6, APOH
2vasculitis30.5TPMT, APOH
3arthritis30.5IL6R, IL6
4adenoma30.5PCNA, IL6R, GGT1
5sjogren's syndrome30.3APOH
6autoimmune hepatitis30.3TPMT
7hepatitis b30.3GGT1, IL6R
8rheumatic disease30.3TPMT, IL6R, APOH
9hepatitis c30.3ITPA, IL6R, GGT1
10ulcerative colitis30.3ITPA, IL6, IL6R, TPMT
11vascular disease30.3APOH, PCNA, IL6, F3
12portal hypertension10.9
13n syndrome10.9
14primary biliary cirrhosis10.6
15peliosis hepatis10.6
16felty's syndrome10.5
17celiac disease10.5
18hepatopulmonary syndrome10.5
19common variable immunodeficiency10.4
20polyarteritis nodosa10.4
21primary pulmonary hypertension10.4
22membranous glomerulonephritis10.4
23macroglobulinemia10.4
24rheumatoid vasculitis10.4
25hepatoportal sclerosis10.4
26membranoproliferative glomerulonephritis10.3
27dyskeratosis congenita10.3
28hypersplenism10.3
29graft versus host disease10.2
30antiphospholipid syndrome10.2
31idiopathic pulmonary fibrosis10.2
32schnitzler syndrome10.2
33visceral leishmaniasis10.2
34sickle cell anemia10.2
35liver lymphoma10.2
36cystinosis10.2
37portal vein thrombosis10.2
38hepatitis d10.2
39focal glomerulosclerosis10.2
40poems syndrome10.2
41protein c deficiency10.2
42hepatitis a10.2
43amyloidosis10.2
44aplastic anemia10.2
45biliary tract disease10.2
46testicular cancer10.2
47pulmonary fibrosis, familial10.2
48wilms tumor10.2
49amyopathic dermatomyositis10.2
50chronic graft versus host disease10.2

Graphical network of the top 20 diseases related to Nodular Regenerative Hyperplasia:



Diseases related to nodular regenerative hyperplasia

Clinical Features for Nodular Regenerative Hyperplasia

Sources:
49Orphanet
See all sources


Symptoms:

49
  • hepatocellular liver disease/hepatic failure
  • chronic arterial hypertension

Drugs & Therapeutics for Nodular Regenerative Hyperplasia

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Nodular Regenerative Hyperplasia

Drug clinical trials:

Search ClinicalTrials for Nodular Regenerative Hyperplasia

Search NIH Clinical Center for Nodular Regenerative Hyperplasia

Search CenterWatch for Nodular Regenerative Hyperplasia

Genetic Tests for Nodular Regenerative Hyperplasia

Anatomical Context for Nodular Regenerative Hyperplasia

Sources:
33MalaCards
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MalaCards organs/tissues related to Nodular Regenerative Hyperplasia:

33
Bone marrow, Liver, Breast, Skin, Myeloid, T cells, B cells, Endothelial, Fetal liver

Animal Models for Nodular Regenerative Hyperplasia or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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Publications for Nodular Regenerative Hyperplasia

Sources:
51PubMed
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Articles related to Nodular Regenerative Hyperplasia:

(show top 50)    (show all 219)
idTitleAuthorsYear
1
Familial occurrence of nodular regenerative hyperplasia of the liver. (23287953)
2013
2
Portal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer. (23984149)
2013
3
Review article: the association between nodular regenerative hyperplasia, inflammatory bowel disease and thiopurine therapy. (24099468)
2013
4
Diffuse Muscular Pain, Skin Tightening, and Nodular Regenerative Hyperplasia Revealing Paraneoplastic Amyopathic Dermatomyositis due to Testicular Cancer. (23316407)
2012
5
Nodular regenerative hyperplasia and portal hypertension in a patient with coeliac disease. (22606433)
2011
6
Nodular regenerative hyperplasia of the liver associated with didanosine persists for years even after its interruption. (22696691)
2011
7
Successful use of ursodeoxycholic acid in nodular regenerative hyperplasia of the liver. (21450925)
2011
8
Hepatic encephalopathy as an unusual late complication of transjugular intrahepatic portosystemic shunt insertion for non-cirrhotic portal hypertension caused by nodular regenerative hyperplasia in an HIV-positive patient on highly active antiretroviral therapy. (20029067)
2010
9
Anticoagulant therapy for nodular regenerative hyperplasia in a HIV-infected patient. (20082713)
2010
10
Thioguanin-induced nodular regenerative hyperplasia of the liver-ROC analysis of different MR techniques. (17221208)
2007
11
6-thioguanine associated nodular regenerative hyperplasia in patients with inflammatory bowel disease may induce portal hypertension. (17894846)
2007
12
Nodular regenerative hyperplasia is a new cause of chronic liver disease in HIV-infected patients. (17197809)
2007
13
Nodular regenerative hyperplasia: a new serious antiretroviral drugs side effect? (17589205)
2007
14
Unique hepatic imaging features in a patient with nodular regenerative hyperplasia of the liver associating with systemic lupus erythematosus. (17432107)
2007
15
Concomitant hepatocellular carcinoma and non-Hodgkin's lymphoma in a patient with nodular regenerative hyperplasia. (16669877)
2006
16
Portal hypertension and nodular regenerative hyperplasia in a patient with celiac disease. (16688657)
2006
17
Coexistence of nodular regenerative hyperplasia of the liver and pulmonary arterial hypertension in patients with connective tissue diseases: report of three cases and review of the literature. (17165002)
2006
18
Nodular regenerative hyperplasia and severe portal hypertension in cystinosis. (16527704)
2006
19
Nodular regenerative hyperplasia, portal vein thrombosis, and avascular hip necrosis due to hyperhomocysteinaemia. (15951553)
2005
20
Azathioprine induced nodular regenerative hyperplasia in IBD patients. (15980758)
2005
21
Nodular regenerative hyperplasia of the liver: an under-recognized cause of portal hypertension in hematological disorders. (15054815)
2004
22
Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody. (15194657)
2004
23
Nodular regenerative hyperplasia of the liver and focal global glomerulosclerosis associated with sickle cell anemia. (11855565)
2002
24
The liver in collagen diseases: pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver. (11092254)
2000
25
Nodular regenerative hyperplasia of the liver: SPECT evaluation with Tc-99m stannous colloid and Tc-99m neoglycoalbumin (10512115)
1999
26
Familial occurrence of nodular regenerative hyperplasia of the liver: a report on three families. (10403744)
1999
27
Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. (9233888)
1997
28
Pseudotumoral presentation of nodular regenerative hyperplasia of the liver: imaging in five patients including MR imaging. (9166561)
1997
29
Childhood nodular regenerative hyperplasia of the liver complicated by severe hypoxemia. (7608831)
1995
30
Nodular regenerative hyperplasia of the liver following orthotopic liver transplantation. (7652907)
1995
31
Hepatic lesions of vascular origin in multicentric Castleman's disease, plasma cell type: report of one case with peliosis hepatis and another with perisinusoidal fibrosis and nodular regenerative hyperplasia. (8822119)
1995
32
A peculiar distribution of peroxisomes in a patient with nodular regenerative hyperplasia of the liver. (8014452)
1994
33
Diffuse nodular regenerative hyperplasia of the liver associated with human immunodeficiency virus and visceral leishmaniasis. (8438854)
1993
34
Nodular regenerative hyperplasia in partial hepatectomy specimens. (8214260)
1993
35
Diagnostic problems in nodular regenerative hyperplasia (nodular transformation) of the liver. Review of the literature and report of two cases. (1590015)
1992
36
Nodular regenerative hyperplasia of the liver in children. (2035739)
1991
37
Nodular regenerative hyperplasia of the liver in idiopathic hypereosinophilic syndrome. (1918853)
1991
38
Alpha-1-antitrypsin distribution in nodular regenerative hyperplasia of liver. (2295462)
1990
39
Ultrasonography, angiography, computed tomography and magnetic resonance in nodular regenerative hyperplasia of the liver: report of a pseudo-tumoral case. (3059397)
1988
40
Nodular regenerative hyperplasia of the liver associated with primary pulmonary hypertension. (3378792)
1988
41
Three cases of nodular regenerative hyperplasia of the liver following renal transplantation. (3140108)
1988
42
Nodular regenerative hyperplasia of the liver in renal transplantation. (3313894)
1987
43
Nodular regenerative hyperplasia with portal hypertension in a patient with myasthenia gravis. (3496785)
1987
44
Myeloid metaplasia, perisinusoidal fibrosis, and nodular regenerative hyperplasia of the liver. (4079668)
1985
45
Nodular regenerative hyperplasia of the liver in a patient with rheumatoid vasculitis: a morphometric study suggesting a role for hepatic arteritis in the pathogenesis. (6520840)
1984
46
POEMS syndrome with neuroarthropathy and nodular regenerative hyperplasia of the liver. (6089843)
1984
47
Nodular regenerative hyperplasia of the liver. (6315554)
1983
48
Nodular regenerative hyperplasia of the liver in a patient with diabetes mellitus. (7059913)
1982
49
Nodular regenerative hyperplasia of the liver associated with macroglobulinemia. A clue to the pathogenesis. (6786096)
1981
50
Nodular regenerative hyperplasia of the liver in hematologic disorders: a possible response to obliterative portal venopathy. A morphometric study of nine cases with an hypothesis on the pathogenesis. (7432153)
1980

Genetic Variations for Nodular Regenerative Hyperplasia

Expression for genes affiliated with Nodular Regenerative Hyperplasia

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Nodular Regenerative Hyperplasia

Search GEO for disease gene expression data for Nodular Regenerative Hyperplasia.

Pathways for genes affiliated with Nodular Regenerative Hyperplasia

Sources:
54Reactome, 30KEGG, 52QIAGEN, 38NCBI BioSystems Database, 4Cell Signaling Technology, 53R&D Systems
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Pathways related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show all 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
9.7NOTCH2, NOTCH1
2
Hide members
9.7NOTCH1, NOTCH2
39.7NOTCH1, NOTCH2
4
Immune response Oncostatin M signaling via JAK-Stat in human cells
Hide members
9.5IL6R, IL6
5
Hide members
9.4NOTCH2, NOTCH1, JAG1
6
Hide members
9.4NOTCH1, JAG1, NOTCH2
79.4NOTCH1, JAG1, NOTCH2
89.4NOTCH1, NOTCH2, JAG1
99.4NOTCH1, JAG1, NOTCH2
10
Hide members
9.4NOTCH1, JAG1, NOTCH2
11
Hide members
9.4NOTCH1, NOTCH2, JAG1
129.4NOTCH1, JAG1, NOTCH2
13
Hide members
9.0NOTCH1, GFER, IL6R, NOTCH2
14
Hide members
8.4NOTCH2, NOTCH1, GFER, IL6R, IL6

Compounds for genes affiliated with Nodular Regenerative Hyperplasia

Sources:
45Novoseek, 2BitterDB, 11DrugBank, 29IUPHAR, 24HMDB, 50PharmGKB, 60Tocris Bioscience
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Compounds related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show all 37)
idCompoundScoreTop Affiliating Genes
1propylthiouracil45 2 1112.5APOH
2aspartate4510.3NOTCH1
3heparin45 29 11 2413.3APOH
46 thioguanine4510.1TPMT, ITPA
5thiopurine4510.0TPMT, ITPA
66 mercaptopurine4510.0TPMT, ITPA
7kaolin459.9APOH, F3
8tocilizumab45 1110.9IL6, IL6R
9coumarins459.9F3, APOH
10inosine triphosphate45 2410.8TPMT, ITPA
11ptca459.8APOH
12warfarin45 50 11 2412.6APOH, F3, TPMT
13ticlopidine45 1110.5APOH, F3
14rituximab45 50 1111.4IL6R, IL6, APOH
15diethylnitrosamine459.4GGT1, PCNA
16dextran sulfate459.3F3, IL6, APOH
17hcec459.3GGT1, IL6
18arsenite45 2410.2GGT1, PCNA
19prednisolone45 29 1111.2APOH, IL6, IL6R, TPMT
20lycopene45 2410.1GGT1, PCNA
21endotoxin459.1APOH, IL6, F3
22cyclophosphamide45 50 1111.1APOH, PCNA, F3, TPMT
23vitamin b12459.1GGT1, PCNA, APOH
24betacarotene459.1APOH, PCNA, GGT1
25histamine45 29 2411.0TPMT, IL6R, IL6, PCNA
26cyclosporin a45 29 6010.9PCNA, IL6, IL6R, TPMT
27fibrinogen458.9APOH, IL6, IL6R, F3
28pentoxifylline45 119.9GGT1, F3, IL6
29tacrolimus45 50 1110.7TPMT, GGT1, F3, PCNA
30azathioprine45 50 2 1111.5APOH, ITPA, PCNA, GGT1, TPMT
31creatinine458.4APOH, IL6R, F3, GGT1, TPMT
32lactate458.4APOH, PCNA, IL6, GGT1
33retinoic acid45 249.2NOTCH1, F3, IL6R, IL6, PCNA
34dexamethasone45 50 29 1111.0GGT1, NOTCH1, IL6R, IL6, PCNA
35alanine458.0PCNA, IL6, F3, GGT1, TPMT
36vegf458.0APOH, PCNA, IL6, IL6R, F3, NOTCH1
37methotrexate45 50 119.5TPMT, GGT1, IL6R, IL6, PCNA, ITPA

GO Terms for genes affiliated with Nodular Regenerative Hyperplasia

Sources:
16Gene Ontology
See all sources

Cellular components related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1interleukin-6 receptor complexGO:0058969.2IL6, IL6R
2cell surfaceGO:0099868.3APOH, IL6R, F3, NOTCH1, NOTCH2
3extracellular regionGO:0055768.1JAG1, IL6, IL6R, GFER, NOTCH1, NOTCH2

Biological processes related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show all 21)
idNameGO IDScoreTop Affiliating Genes
1distal tubule developmentGO:07201710.0JAG1, NOTCH1
2negative regulation of stem cell differentiationGO:200073710.0JAG1, NOTCH1
3cardiac septum morphogenesisGO:06041110.0JAG1, NOTCH1
4cell fate determinationGO:00170910.0NOTCH2, JAG1
5response to muramyl dipeptideGO:0324959.9JAG1, NOTCH1
6neuronal stem cell maintenanceGO:0971509.9JAG1, NOTCH1
7hepatic immune responseGO:0023849.8IL6, IL6R
8negative regulation of collagen biosynthetic processGO:0329669.8IL6R, IL6
9neutrophil mediated immunityGO:0024469.7IL6R, IL6
10interleukin-6-mediated signaling pathwayGO:0701029.7IL6, IL6R
11positive regulation of leukocyte chemotaxisGO:0026909.7IL6R, IL6
12pulmonary valve morphogenesisGO:0031849.7NOTCH2, JAG1, NOTCH1
13Notch signaling involved in heart developmentGO:0613149.7JAG1, NOTCH1, NOTCH2
14Notch receptor processingGO:0072209.7NOTCH1, JAG1, NOTCH2
15positive regulation of JAK-STAT cascadeGO:0464279.6IL6, NOTCH1
16bone remodelingGO:0468499.6IL6, NOTCH2
17Notch signaling pathwayGO:0072199.5JAG1, NOTCH1, NOTCH2
18monocyte chemotaxisGO:0025489.5IL6R, IL6
19positive regulation of tyrosine phosphorylation of Stat3 proteinGO:0425179.5IL6, IL6R
20positive regulation of chemokine productionGO:0327229.4IL6R, IL6
21defense response to Gram-negative bacteriumGO:0508299.2IL6, IL6R

Molecular functions related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1growth factor activityGO:0080839.3GFER, IL6, JAG1
2interleukin-6 receptor bindingGO:0051389.2IL6R, IL6

Products for genes affiliated with Nodular Regenerative Hyperplasia

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Nodular Regenerative Hyperplasia

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet