MCID: NDL013
MIFTS: 58

Nodular Regenerative Hyperplasia malady

Rare diseases, Liver diseases categories

Summaries for Nodular Regenerative Hyperplasia

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Wikipedia:65 Nodular regenerative hyperplasia is a form of liver hyperplasia associated with portal hypertension. more...

MalaCards based summary: Nodular Regenerative Hyperplasia, also known as nodular regenerative hyperplasia of the liver, is related to inflammatory bowel disease and hepatocellular carcinoma, and has symptoms including hypertension, hepatic failure and hepatocellular liver disease/hepatic failure. An important gene associated with Nodular Regenerative Hyperplasia is GFER (growth factor, augmenter of liver regeneration), and among its related pathways are Notch signaling pathway and Pre-NOTCH Expression and Processing. The compounds purine analogues and 6 thioguanine have been mentioned in the context of this disorder. Affiliated tissues include liver, bone marrow and bone, and related mouse phenotypes are liver/biliary system and tumorigenesis.

Aliases & Classifications for Nodular Regenerative Hyperplasia

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Sources:
43NIH Rare Diseases, 49Orphanet, 62UMLS, 28ICD10 via Orphanet
See all sources

Nodular Regenerative Hyperplasia, Aliases & Descriptions:

Name: Nodular Regenerative Hyperplasia 43 62
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


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Liver diseases
Orphanet: 49 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

49

External Ids:

Orphanet49 48372
ICD10 via Orphanet28 K76.8

Related Diseases for Nodular Regenerative Hyperplasia

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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 126)
idRelated DiseaseScoreTop Affiliating Genes
1inflammatory bowel disease31.0TPMT, ITPA, IL6
2hepatocellular carcinoma30.9GGT1, PCNA, IL6R, IL6, JAG1, NOTCH1
3systemic lupus erythematosus30.9F3, TPMT, APOH, IL6
4liver disease30.7PCNA, IL6, F3, GGT1
5cholestasis30.6GGT1, NOTCH2, JAG1
6pancreatitis30.6TPMT, ITPA, IL6, GGT1
7protein s deficiency30.4APOH, F3
8rheumatic disease30.4IL6R, TPMT, APOH
9antiphospholipid syndrome30.4F3, APOH
10autoimmune hepatitis30.4TPMT, GGT1
11connective tissue disease30.4IL6, F3, APOH
12ulcerative colitis30.4IL6R, TPMT, ITPA, IL6
13rheumatoid arthritis30.3IL6R, TPMT, APOH, IL6
14myeloma30.3NOTCH1, IL6, IL6R
15breast cancer30.3JAG1, IL6R, PCNA, NOTCH2, NOTCH1
16portal hypertension10.9
17hepatitis10.9
18primary biliary cirrhosis10.7
19lupus erythematosus10.6
20pulmonary hypertension10.6
21peliosis hepatis10.6
22celiac disease10.5
23felty's syndrome10.5
24crest syndrome10.5
25budd-chiari syndrome10.5
26common variable immunodeficiency10.5
27glomerulonephritis10.5
28polyarteritis nodosa10.5
29arthritis10.4
30leukemia10.4
31myasthenia gravis10.4
32membranous glomerulonephritis10.4
33macroglobulinemia10.4
34adenoma10.4
35vasculitis10.4
36castleman's disease10.4
37rheumatoid vasculitis10.4
38hepatoportal sclerosis10.4
39dyskeratosis congenita10.3
40membranoproliferative glomerulonephritis10.3
41hypersplenism10.3
42sickle cell anemia10.2
43aplastic anemia10.2
44wilms tumor10.2
45hodgkin lymphoma10.2
46multiple myeloma10.2
47b-cell lymphomas10.2
48crohn's disease10.2
49myelofibrosis10.2
50pulmonary fibrosis10.2

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



Diseases related to nodular regenerative hyperplasia

Symptoms for Nodular Regenerative Hyperplasia

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Symptoms:

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

HPO human phenotypes related to Nodular Regenerative Hyperplasia:

id Description Frequency HPO Source Accession
1 hypertension hallmark (90%) HP:0000822
2 hepatic failure hallmark (90%) HP:0001399

Drugs & Therapeutics for Nodular Regenerative Hyperplasia

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Drug clinical trials:

Search ClinicalTrials for Nodular Regenerative Hyperplasia

Search NIH Clinical Center for Nodular Regenerative Hyperplasia

Genetic Tests for Nodular Regenerative Hyperplasia

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Anatomical Context for Nodular Regenerative Hyperplasia

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MalaCards organs/tissues related to Nodular Regenerative Hyperplasia:

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

Animal Models for Nodular Regenerative Hyperplasia or affiliated genes

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MGI Mouse Phenotypes related to Nodular Regenerative Hyperplasia:

37 (show all 15)
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053708.3JAG1, IL6, IL6R, NOTCH2, NOTCH1
2MP:00020068.3F3, IL6, IL6R, NOTCH2, NOTCH1
3MP:00028738.1JAG1, F3, IL6R, NOTCH2, NOTCH1
4MP:00053898.1GGT1, NOTCH2, PCNA, ITPA, TPMT
5MP:00053698.1JAG1, F3, ITPA, IL6, NOTCH2, NOTCH1
6MP:00053857.8JAG1, F3, ITPA, IL6, NOTCH2, NOTCH1
7MP:00053847.6JAG1, F3, ITPA, IL6, IL6R, NOTCH2
8MP:00053917.5JAG1, F3, IL6, NOTCH2, NOTCH1, GGT1
9MP:00107717.3JAG1, F3, ITPA, IL6, NOTCH2, NOTCH1
10MP:00053797.2JAG1, IL6, IL6R, PCNA, NOTCH2, NOTCH1
11MP:00053876.9F3, IL6, IL6R, PCNA, NOTCH2, NOTCH1
12MP:00053766.6APOH, TPMT, JAG1, F3, IL6, IL6R
13MP:00053976.2GGT1, TPMT, JAG1, F3, ITPA, IL6
14MP:00053786.1ITPA, F3, JAG1, TPMT, IL6, IL6R
15MP:00107685.8APOH, TPMT, JAG1, F3, ITPA, IL6

Publications for Nodular Regenerative Hyperplasia

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Articles related to Nodular Regenerative Hyperplasia:

(show top 50)    (show all 229)
idTitleAuthorsYear
1
A case of nodular regenerative hyperplasia of the liver combined with toxic hepatitis. (25603855)
2015
2
Familial occurrence of nodular regenerative hyperplasia of the liver. (23287953)
2013
3
Lengthy follow-up after liver transplantation for nodular regenerative hyperplasia in human immunodeficiency virus-infected patients: does the disease recur? (24296606)
2013
4
Hepatic hemodynamics in 24 patients with nodular regenerative hyperplasia and symptomatic portal hypertension. (22554152)
2012
5
The role of nucleoside and nucleotide analogues in nodular regenerative hyperplasia in HIV-infected patients: a case control study. (21056493)
2011
6
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
7
Absence of nodular regenerative hyperplasia after low-dose 6-thioguanine maintenance therapy in inflammatory bowel disease patients. (18083079)
2008
8
Nodular regenerative hyperplasia of liver. (18417867)
2008
9
Histopathology of liver biopsies from a thiopurine-naA^ve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia. (18415755)
2008
10
Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine. (17504943)
2007
11
Thioguanin-induced nodular regenerative hyperplasia of the liver-ROC analysis of different MR techniques. (17221208)
2007
12
Nodular regenerative hyperplasia and focal nodular hyperplasia of the liver mimicking hepatic metastasis in children with solid tumors and a review of literature. (17613871)
2007
13
Nodular regenerative hyperplasia: not all nodules are created equal. (16799965)
2006
14
Celiac disease associated with nodular regenerative hyperplasia, pulmonary abnormalities, and IgA anticardiolipin antibodies. (16394874)
2006
15
Concomitant hepatocellular carcinoma and non-Hodgkin's lymphoma in a patient with nodular regenerative hyperplasia. (16669877)
2006
16
Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. (17063088)
2006
17
Nodular regenerative hyperplasia: a reversible entity associated with azathioprine therapy. (16607155)
2006
18
Nodular regenerative hyperplasia of the liver and Hodgkin's disease: a case report. (15727082)
2004
19
Nodular regenerative hyperplasia of the liver: an under-recognized cause of portal hypertension in hematological disorders. (15054815)
2004
20
Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody. (15194657)
2004
21
Nodular regenerative hyperplasia of the liver: a case report. (14593541)
2003
22
Nodular regenerative hyperplasia of the liver. A review of 14 cases. (11677979)
2001
23
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
24
Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature. (10663513)
2000
25
Results of liver transplantation for nodular regenerative hyperplasia. (11090022)
2000
26
Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature. (10192512)
1999
27
Nodular regenerative hyperplasia of the liver: description of two cases. (8544802)
1996
28
Childhood nodular regenerative hyperplasia of the liver complicated by severe hypoxemia. (7608831)
1995
29
Nodular regenerative hyperplasia of the liver following orthotopic liver transplantation. (7652907)
1995
30
A peculiar distribution of peroxisomes in a patient with nodular regenerative hyperplasia of the liver. (8014452)
1994
31
Nodular regenerative hyperplasia of the liver and primary SjAPgren's syndrome. (8151575)
1994
32
Nodular regenerative hyperplasia of the liver: ultrasonographic appearance and echo-guided bioptic diagnosis. (7812029)
1994
33
Nodular regenerative hyperplasia: a controversial indication for orthotopic liver transplantation. (7916934)
1994
34
Hepatic infarction and nodular regenerative hyperplasia of the liver with associated anticardiolipin antibodies in a young woman. (8071516)
1994
35
Diffuse nodular regenerative hyperplasia of the liver associated with human immunodeficiency virus and visceral leishmaniasis. (8438854)
1993
36
Nodular regenerative hyperplasia in the rat induced by a selenium-enriched diet: study of a model. (1639352)
1992
37
Azathioprine induced liver disease: nodular regenerative hyperplasia of the liver and perivenous fibrosis in a patient treated for multiple sclerosis. (2060883)
1991
38
Nodular regenerative hyperplasia: a cause of ascites and hepatomegaly after chemotherapy for leukemia. (1986559)
1991
39
Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. (2189821)
1990
40
Nodular regenerative hyperplasia mimicking cirrhosis of the liver. (2379880)
1990
41
Nodular regenerative hyperplasia of the liver, peliosis hepatis, and perisinusoidal fibrosis. Association with angioimmunoblastic lymphadenopathy and severe hypoxemia. (2344932)
1990
42
Peliosis hepatis, nodular regenerative hyperplasia of the liver, and light-chain deposition in a patient with WaldenstrAPm's macroglobulinemia. (3134270)
1988
43
Nodular regenerative hyperplasia of the liver with and without portal hypertension: a comparison. (3963734)
1986
44
Systemic lupus erythematosus and nodular regenerative hyperplasia of the liver. (3947146)
1986
45
Nodular regenerative hyperplasia of the liver in a patient with multiple myeloma and systemic amyloidosis. (2416186)
1985
46
Nodular regenerative hyperplasia of the liver associated with progressive systemic sclerosis: a case report with ultrastructural observation. (6644697)
1983
47
Nodular regenerative hyperplasia of the liver with membranous glomerulonephritis. (7301692)
1981
48
Nodular regenerative hyperplasia of the liver following renal transplantation. (7016482)
1981
49
Nodular regenerative hyperplasia of the liver. (6945840)
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

Variations for Nodular Regenerative Hyperplasia

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Expression for genes affiliated with Nodular Regenerative Hyperplasia

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

Pathways for genes affiliated with Nodular Regenerative Hyperplasia

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Pathways related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show all 16)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.7NOTCH1, JAG1
2
Show member pathways
9.5NOTCH1, NOTCH2
39.5NOTCH2, NOTCH1
49.5IL6R, IL6
59.5IL6R, IL6
69.3IL6, NOTCH1
79.2NOTCH1, NOTCH2, JAG1
8
Show member pathways
9.2JAG1, NOTCH2, NOTCH1
99.2NOTCH1, NOTCH2, JAG1
10
Show member pathways
9.2NOTCH1, NOTCH2, JAG1
11
Show member pathways
Delta-Notch Signaling Pathway38
Notch Signaling Pathway38
9.2NOTCH1, NOTCH2, JAG1
12
Show member pathways
Notch signaling pathway38
9.2NOTCH1, NOTCH2, JAG1
139.2JAG1, NOTCH2, NOTCH1
149.0IL6, IL6R, PCNA
15
Show member pathways
8.3GFER, IL6, IL6R, NOTCH2, NOTCH1
16
Show member pathways
8.3GFER, IL6, IL6R, NOTCH2, NOTCH1

Compounds for genes affiliated with Nodular Regenerative Hyperplasia

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Compounds related to Nodular Regenerative Hyperplasia according to GeneCards/GeneDecks:

(show top 50)    (show all 62)
idCompoundScoreTop Affiliating Genes
1purine analogues5110.3ITPA, TPMT
26 thioguanine4510.2ITPA, TPMT
3thiopurine4510.2TPMT, ITPA
46 mercaptopurine4510.2ITPA, TPMT
5inosine triphosphate45 2611.1TPMT, ITPA
6kaolin4510.0F3, APOH
7coumarins4510.0F3, APOH
8tocilizumab45 1310.9IL6R, IL6
9ticlopidine45 1310.9APOH, F3
10ptca459.9F3, APOH
11inosine45 2610.8ITPA, TPMT
12warfarin45 51 26 1312.7F3, TPMT, APOH
13rituximab45 51 1311.5IL6R, IL6, APOH
14diethylnitrosamine459.5GGT1, PCNA
15dextran sulfate459.5APOH, F3, IL6
16leflunomide45 51 1311.4PCNA, IL6
17aspirin45 51 30 2612.4PCNA, F3, APOH
18endotoxin459.4IL6, F3, APOH
19hcec459.4GGT1, IL6
20diethyl dithiocarbamate459.3GGT1, TPMT
21lycopene45 2610.3GGT1, PCNA
22prednisolone45 30 1311.3IL6R, IL6, TPMT, APOH
23cyclophosphamide45 51 1311.2APOH, TPMT, F3, PCNA
24propylthiouracil45 3 1311.2APOH, PCNA, GGT1
25vitamin b12459.2GGT1, PCNA, APOH
26betacarotene459.2APOH, PCNA, GGT1
27fibrinogen459.1APOH, F3, IL6, IL6R
28histamine45 30 2611.1TPMT, IL6, IL6R, PCNA
29epinephrine45 26 1311.1F3, IL6, PCNA
30cyclosporin a45 30 6111.1TPMT, IL6, IL6R, PCNA
31arsenite45 2610.0F3, PCNA, GGT1
32pentoxifylline45 1310.0F3, IL6, GGT1
33tacrolimus45 51 1310.8GGT1, PCNA, F3, TPMT
34heparin45 30 26 1311.7APOH, F3, PCNA, GGT1
35azathioprine45 3 51 1311.6GGT1, PCNA, ITPA, TPMT, APOH
36cholesterol45 30 26 1311.6GGT1, IL6R, IL6, APOH
37n acetylcysteine458.6GGT1, PCNA, IL6
38arginine458.6APOH, IL6R, PCNA, GGT1
39aspartate458.5F3, PCNA, NOTCH1, GGT1
40lactate458.5APOH, IL6, PCNA, GGT1
41indomethacin45 30 61 1311.5IL6, PCNA, GGT1
42testosterone45 61 26 1311.5IL6, IL6R, PCNA, GGT1
43retinoic acid45 269.4NOTCH1, PCNA, IL6R, IL6, F3
44nitric oxide45 26 1310.4NOTCH1, IL6, JAG1, APOH
45lipid458.1APOH, IL6, IL6R, NOTCH1, GGT1
46alanine458.1TPMT, F3, IL6, PCNA, GGT1
47vegf458.1APOH, F3, IL6, IL6R, PCNA, NOTCH1
48dexamethasone45 51 30 1311.1IL6, IL6R, PCNA, NOTCH1, GGT1
49methotrexate51 45 139.6GGT1, APOH, TPMT, ITPA, IL6, IL6R
50creatinine457.5APOH, TPMT, F3, IL6, IL6R, PCNA

GO Terms for genes affiliated with Nodular Regenerative Hyperplasia

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

idNameGO IDScoreTop Affiliating Genes
1interleukin-6 receptor complexGO:00058969.2IL6, IL6R
2cell surfaceGO:00099868.3NOTCH1, NOTCH2, IL6R, F3, APOH
3extracellular regionGO:00055768.0NOTCH1, NOTCH2, IL6R, IL6, JAG1, GFER

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

(show all 33)
idNameGO IDScoreTop Affiliating Genes
1morphogenesis of an epithelial sheetGO:000201110.1JAG1, NOTCH2
2distal tubule developmentGO:007201710.0NOTCH1, JAG1
3negative regulation of stem cell differentiationGO:0200073710.0JAG1, NOTCH1
4cell fate determinationGO:000170910.0JAG1, NOTCH2
5cardiac septum morphogenesisGO:006041110.0NOTCH1, JAG1
6response to muramyl dipeptideGO:003249510.0NOTCH1, JAG1
7neuronal stem cell maintenanceGO:009715010.0NOTCH1, JAG1
8interleukin-4 secretionGO:00726029.9NOTCH2, NOTCH1
9hepatic immune responseGO:00023849.9IL6, IL6R
10negative regulation of collagen biosynthetic processGO:00329669.9IL6R, IL6
11neutrophil mediated immunityGO:00024469.9IL6R, IL6
12interleukin-6-mediated signaling pathwayGO:00701029.9IL6R, IL6
13positive regulation of leukocyte chemotaxisGO:00026909.8IL6R, IL6
14inflammatory response to antigenic stimulusGO:00024379.8NOTCH2, NOTCH1
15positive regulation of chemokine productionGO:00327229.8IL6, IL6R
16monocyte chemotaxisGO:00025489.7IL6, IL6R
17bone remodelingGO:00468499.7IL6, NOTCH2
18defense response to Gram-negative bacteriumGO:00508299.7IL6R, IL6
19positive regulation of tyrosine phosphorylation of Stat3 proteinGO:00425179.7IL6R, IL6
20negative regulation of fat cell differentiationGO:00455999.7IL6, JAG1
21defense response to Gram-positive bacteriumGO:00508309.7IL6, IL6R
22pulmonary valve morphogenesisGO:00031849.6NOTCH1, NOTCH2, JAG1
23Notch signaling involved in heart developmentGO:00613149.6JAG1, NOTCH2, NOTCH1
24Notch receptor processingGO:00072209.6NOTCH1, NOTCH2, JAG1
25positive regulation of JAK-STAT cascadeGO:00464279.6NOTCH1, IL6
26acute-phase responseGO:00069539.6IL6R, IL6
27positive regulation of interleukin-6 productionGO:00327559.6IL6, IL6R
28Notch signaling pathwayGO:00072199.6JAG1, NOTCH2, NOTCH1
29endocrine pancreas developmentGO:00310189.5IL6, IL6R
30positive regulation of smooth muscle cell proliferationGO:00486619.5IL6R, IL6
31humoral immune responseGO:00069599.3IL6, NOTCH2, NOTCH1
32determination of left/right symmetryGO:00073689.2NOTCH1, NOTCH2
33positive regulation of apoptotic processGO:00430658.8NOTCH1, NOTCH2, IL6

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

idNameGO IDScoreTop Affiliating Genes
1growth factor activityGO:00080839.3IL6, JAG1, GFER
2interleukin-6 receptor bindingGO:00051389.2IL6R, IL6
3protein bindingGO:00055155.7GGT1, GFER, APOH, JAG1, F3, IL6

Products for genes affiliated with Nodular Regenerative Hyperplasia

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Sources for Nodular Regenerative Hyperplasia

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4CDC
15ExPASy
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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