MCID: NDL013
MIFTS: 56

Nodular Regenerative Hyperplasia malady

Rare diseases, Liver diseases categories

Summaries for Nodular Regenerative Hyperplasia

About this section
Sources:
66Wikipedia, 34MalaCards
See all sources

Fully expand this MalaCard

Download this MalaCard
Wikipedia:66 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 inflammatory bowel disease and systemic lupus erythematosus, and has symptoms including chronic arterial hypertensionand 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

About this section
Sources:
44NIH Rare Diseases, 50Orphanet, 27ICD10 via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Liver diseases
Orphanet: 50 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

50

Aliases & Descriptions:

nodular regenerative hyperplasia 44
nodular regenerative hyperplasia of the liver 44 50
non-cirrhotic nodulation 44 50
non-cirrhotic nodular transformation 44
miliary hepatocellular adenomatosis 44
non-cirrhotic portal hypertension 44


External Ids:

ICD10 via Orphanet27 K76.8

Related Diseases for Nodular Regenerative Hyperplasia

About this section
Sources:
18GeneCards, 19GeneDecks
See all sources

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

(show top 50)    (show all 128)
idRelated DiseaseScoreTop Affiliating Genes
1inflammatory bowel disease31.0TPMT, ITPA, IL6
2systemic lupus erythematosus30.7F3, TPMT, APOH, IL6
3hepatocellular carcinoma30.7GGT1, PCNA, IL6R, IL6, JAG1, NOTCH1
4castleman's disease30.7IL6, IL6R
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 disease30.3IL6, F3, APOH
12ulcerative colitis30.3IL6R, TPMT, ITPA, IL6
13multiple sclerosis30.3IL6R, IL6, JAG1, TPMT
14rheumatoid arthritis30.3IL6R, TPMT, APOH, IL6
15myeloma30.3NOTCH1, IL6, IL6R
16breast cancer30.2JAG1, IL6R, PCNA, NOTCH2, NOTCH1
17hypertension11.0
18portal hypertension10.9
19hepatitis10.8
20primary biliary cirrhosis10.6
21peliosis hepatis10.6
22lupus erythematosus10.6
23celiac disease10.5
24felty's syndrome10.5
25liver disease10.5
26hepatopulmonary syndrome10.5
27polyarteritis nodosa10.5
28common variable immunodeficiency10.5
29glomerulonephritis10.5
30membranous glomerulonephritis10.4
31macroglobulinemia10.4
32adenoma10.4
33arthritis10.4
34myasthenia gravis10.4
35vasculitis10.4
36rheumatoid vasculitis10.4
37hepatoportal sclerosis10.4
38dyskeratosis congenita10.3
39membranoproliferative glomerulonephritis10.3
40hypersplenism10.3
41graft versus host disease10.2
42crohn's disease10.2
43sickle cell anemia10.2
44hepatocellular adenoma10.2
45cystinosis10.2
46portal vein thrombosis10.2
47idiopathic pulmonary fibrosis10.2
48sjogren's syndrome10.2
49visceral leishmaniasis10.2
50poems syndrome10.2

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



Diseases related to nodular regenerative hyperplasia

Symptoms for Nodular Regenerative Hyperplasia

About this section
Sources:
50Orphanet
See all sources


Symptoms:

50
  • chronic arterial hypertension
  • hepatocellular liver disease/hepatic failure

Drugs & Therapeutics for Nodular Regenerative Hyperplasia

About this section
Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
See all sources

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

About this section

Anatomical Context for Nodular Regenerative Hyperplasia

About this section
Sources:
34MalaCards
See all sources

MalaCards organs/tissues related to Nodular Regenerative Hyperplasia:

34
Liver, Bone marrow, Bone, Fetal liver, Endothelial, B cells, T cells, Myeloid, Testes, Skin, Breast

Animal Models for Nodular Regenerative Hyperplasia or affiliated genes

About this section
Sources:
38MGI
See all sources

MGI Mouse Phenotypes related to Nodular Regenerative Hyperplasia:

38 (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

About this section
Sources:
53PubMed
See all sources

Articles related to Nodular Regenerative Hyperplasia:

(show top 50)    (show all 213)
idTitleAuthorsYear
1
Familial occurrence of nodular regenerative hyperplasia of the liver. (23287953)
2013
2
Lengthy follow-up after liver transplantation for nodular regenerative hyperplasia in human immunodeficiency virus-infected patients: does the disease recur? (24296606)
2013
3
Hepatic hemodynamics in 24 patients with nodular regenerative hyperplasia and symptomatic portal hypertension. (22554152)
2012
4
The role of nucleoside and nucleotide analogues in nodular regenerative hyperplasia in HIV-infected patients: a case control study. (21056493)
2011
5
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
6
Absence of nodular regenerative hyperplasia after low-dose 6-thioguanine maintenance therapy in inflammatory bowel disease patients. (18083079)
2008
7
Nodular regenerative hyperplasia of liver. (18417867)
2008
8
Histopathology of liver biopsies from a thiopurine-naA^ve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia. (18415755)
2008
9
Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine. (17504943)
2007
10
Thioguanin-induced nodular regenerative hyperplasia of the liver-ROC analysis of different MR techniques. (17221208)
2007
11
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
12
Nodular regenerative hyperplasia: not all nodules are created equal. (16799965)
2006
13
Celiac disease associated with nodular regenerative hyperplasia, pulmonary abnormalities, and IgA anticardiolipin antibodies. (16394874)
2006
14
Concomitant hepatocellular carcinoma and non-Hodgkin's lymphoma in a patient with nodular regenerative hyperplasia. (16669877)
2006
15
Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. (17063088)
2006
16
Nodular regenerative hyperplasia: a reversible entity associated with azathioprine therapy. (16607155)
2006
17
Nodular regenerative hyperplasia of the liver and Hodgkin's disease: a case report. (15727082)
2004
18
Nodular regenerative hyperplasia of the liver: an under-recognized cause of portal hypertension in hematological disorders. (15054815)
2004
19
Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody. (15194657)
2004
20
Nodular regenerative hyperplasia of the liver: a case report. (14593541)
2003
21
Nodular regenerative hyperplasia of the liver. A review of 14 cases. (11677979)
2001
22
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
23
Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature. (10663513)
2000
24
Results of liver transplantation for nodular regenerative hyperplasia. (11090022)
2000
25
Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature. (10192512)
1999
26
Nodular regenerative hyperplasia of the liver: description of two cases. (8544802)
1996
27
Childhood nodular regenerative hyperplasia of the liver complicated by severe hypoxemia. (7608831)
1995
28
Nodular regenerative hyperplasia of the liver following orthotopic liver transplantation. (7652907)
1995
29
A peculiar distribution of peroxisomes in a patient with nodular regenerative hyperplasia of the liver. (8014452)
1994
30
Nodular regenerative hyperplasia of the liver and primary SjAPgren's syndrome. (8151575)
1994
31
Nodular regenerative hyperplasia of the liver: ultrasonographic appearance and echo-guided bioptic diagnosis. (7812029)
1994
32
Nodular regenerative hyperplasia: a controversial indication for orthotopic liver transplantation. (7916934)
1994
33
Hepatic infarction and nodular regenerative hyperplasia of the liver with associated anticardiolipin antibodies in a young woman. (8071516)
1994
34
Diffuse nodular regenerative hyperplasia of the liver associated with human immunodeficiency virus and visceral leishmaniasis. (8438854)
1993
35
Azathioprine induced liver disease: nodular regenerative hyperplasia of the liver and perivenous fibrosis in a patient treated for multiple sclerosis. (2060883)
1991
36
Nodular regenerative hyperplasia: a cause of ascites and hepatomegaly after chemotherapy for leukemia. (1986559)
1991
37
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
38
Nodular regenerative hyperplasia mimicking cirrhosis of the liver. (2379880)
1990
39
Nodular regenerative hyperplasia of the liver, peliosis hepatis, and perisinusoidal fibrosis. Association with angioimmunoblastic lymphadenopathy and severe hypoxemia. (2344932)
1990
40
Peliosis hepatis, nodular regenerative hyperplasia of the liver, and light-chain deposition in a patient with WaldenstrAPm's macroglobulinemia. (3134270)
1988
41
Nodular regenerative hyperplasia of the liver with and without portal hypertension: a comparison. (3963734)
1986
42
Systemic lupus erythematosus and nodular regenerative hyperplasia of the liver. (3947146)
1986
43
Nodular regenerative hyperplasia and focal nodular hyperplasia of the liver associated with severe cholestasis. Report of a case with autopsy findings. (3713453)
1986
44
Nodular regenerative hyperplasia of the liver in a patient with multiple myeloma and systemic amyloidosis. (2416186)
1985
45
Nodular regenerative hyperplasia of the liver. Report of a case and review of the literature. (4047265)
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

About this section

Expression for genes affiliated with Nodular Regenerative Hyperplasia

About this section
Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
See all sources
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

About this section
Sources:
51PathCards, 31KEGG, 58SinoBiological, 56Reactome, 39NCBI BioSystems Database, 61Thomson Reuters, 5Cell Signaling Technology, 55R&D Systems, 54QIAGEN
See all sources

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 Pathway39
Notch Signaling Pathway39
9.2NOTCH1, NOTCH2, JAG1
12
Show member pathways
Notch signaling pathway39
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

About this section
Sources:
52PharmGKB, 46Novoseek, 25HMDB, 12DrugBank, 30IUPHAR, 3BitterDB, 62Tocris Bioscience
See all sources

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

(show top 50)    (show all 62)
idCompoundScoreTop Affiliating Genes
1purine analogues5210.3ITPA, TPMT
26 thioguanine4610.2TPMT, ITPA
3thiopurine4610.2ITPA, TPMT
46 mercaptopurine4610.2ITPA, TPMT
5inosine triphosphate46 2511.1ITPA, TPMT
6kaolin4610.0APOH, F3
7coumarins4610.0APOH, F3
8tocilizumab46 1210.9IL6, IL6R
9ticlopidine46 1210.9APOH, F3
10ptca469.9APOH, F3
11inosine46 2510.8TPMT, ITPA
12warfarin46 52 25 1212.7TPMT, F3, APOH
13rituximab46 52 1211.5APOH, IL6R, IL6
14diethylnitrosamine469.5PCNA, GGT1
15dextran sulfate469.5IL6, F3, APOH
16leflunomide46 52 1211.4PCNA, IL6
17aspirin46 52 30 2512.4PCNA, APOH, F3
18endotoxin469.4F3, APOH, IL6
19hcec469.4IL6, GGT1
20diethyl dithiocarbamate469.3TPMT, GGT1
21lycopene46 2510.3PCNA, GGT1
22prednisolone46 30 1211.3IL6R, IL6, TPMT, APOH
23cyclophosphamide46 52 1211.2F3, PCNA, TPMT, APOH
24propylthiouracil46 3 1211.2GGT1, PCNA, APOH
25vitamin b12469.2GGT1, APOH, PCNA
26betacarotene469.2PCNA, GGT1, APOH
27fibrinogen469.1F3, IL6R, IL6, APOH
28histamine46 30 2511.1TPMT, IL6R, IL6, PCNA
29epinephrine46 25 1211.1IL6, PCNA, F3
30cyclosporin a46 30 6211.1IL6R, PCNA, IL6, TPMT
31arsenite46 2510.0PCNA, F3, GGT1
32pentoxifylline46 1210.0F3, IL6, GGT1
33tacrolimus46 52 1210.8F3, TPMT, GGT1, PCNA
34heparin46 30 25 1211.7GGT1, APOH, F3, PCNA
35azathioprine46 3 52 1211.6PCNA, GGT1, TPMT, APOH, ITPA
36cholesterol46 30 25 1211.6IL6R, APOH, IL6, GGT1
37n acetylcysteine468.6GGT1, IL6, PCNA
38arginine468.6APOH, IL6R, GGT1, PCNA
39aspartate468.5F3, PCNA, NOTCH1, GGT1
40lactate468.5IL6, PCNA, GGT1, APOH
41indomethacin46 30 62 1211.5IL6, PCNA, GGT1
42testosterone46 62 25 1211.5IL6, GGT1, IL6R, PCNA
43retinoic acid46 259.4PCNA, NOTCH1, F3, IL6, IL6R
44nitric oxide46 25 1210.4JAG1, NOTCH1, IL6, APOH
45lipid468.1GGT1, NOTCH1, IL6R, IL6, APOH
46alanine468.1GGT1, PCNA, F3, IL6, TPMT
47vegf468.1APOH, IL6, IL6R, PCNA, NOTCH1, F3
48dexamethasone46 52 30 1211.1NOTCH1, IL6R, GGT1, PCNA, IL6
49methotrexate52 46 129.6IL6R, GGT1, PCNA, APOH, TPMT, ITPA
50creatinine467.5PCNA, GGT1, APOH, TPMT, F3, IL6R

GO Terms for genes affiliated with Nodular Regenerative Hyperplasia

About this section
Sources:
17Gene 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:0099868.3NOTCH1, NOTCH2, IL6R, F3, APOH
3extracellular regionGO:0055768.0NOTCH1, 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:00201110.1JAG1, NOTCH2
2distal tubule developmentGO:07201710.0NOTCH1, JAG1
3negative regulation of stem cell differentiationGO:200073710.0JAG1, NOTCH1
4cell fate determinationGO:00170910.0JAG1, NOTCH2
5cardiac septum morphogenesisGO:06041110.0NOTCH1, JAG1
6response to muramyl dipeptideGO:03249510.0NOTCH1, JAG1
7neuronal stem cell maintenanceGO:09715010.0NOTCH1, JAG1
8interleukin-4 secretionGO:0726029.9NOTCH2, NOTCH1
9hepatic immune responseGO:0023849.9IL6, IL6R
10negative regulation of collagen biosynthetic processGO:0329669.9IL6R, IL6
11neutrophil mediated immunityGO:0024469.9IL6R, IL6
12interleukin-6-mediated signaling pathwayGO:0701029.9IL6R, IL6
13positive regulation of leukocyte chemotaxisGO:0026909.8IL6R, IL6
14inflammatory response to antigenic stimulusGO:0024379.8NOTCH2, NOTCH1
15positive regulation of chemokine productionGO:0327229.8IL6, IL6R
16monocyte chemotaxisGO:0025489.7IL6, IL6R
17bone remodelingGO:0468499.7IL6, NOTCH2
18defense response to Gram-negative bacteriumGO:0508299.7IL6R, IL6
19positive regulation of tyrosine phosphorylation of Stat3 proteinGO:0425179.7IL6R, IL6
20negative regulation of fat cell differentiationGO:0455999.7IL6, JAG1
21defense response to Gram-positive bacteriumGO:0508309.7IL6, IL6R
22pulmonary valve morphogenesisGO:0031849.6NOTCH1, NOTCH2, JAG1
23Notch signaling involved in heart developmentGO:0613149.6JAG1, NOTCH2, NOTCH1
24Notch receptor processingGO:0072209.6NOTCH1, NOTCH2, JAG1
25positive regulation of JAK-STAT cascadeGO:0464279.6NOTCH1, IL6
26acute-phase responseGO:0069539.6IL6R, IL6
27positive regulation of interleukin-6 productionGO:0327559.6IL6, IL6R
28Notch signaling pathwayGO:0072199.6JAG1, NOTCH2, NOTCH1
29endocrine pancreas developmentGO:0310189.5IL6, IL6R
30positive regulation of smooth muscle cell proliferationGO:0486619.5IL6R, IL6
31humoral immune responseGO:0069599.3IL6, NOTCH2, NOTCH1
32determination of left/right symmetryGO:0073689.2NOTCH1, NOTCH2
33positive regulation of apoptotic processGO:0430658.8NOTCH1, NOTCH2, IL6

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

idNameGO IDScoreTop Affiliating Genes
1growth factor activityGO:0080839.3IL6, JAG1, GFER
2interleukin-6 receptor bindingGO:0051389.2IL6R, IL6
3protein bindingGO:0055155.7IL6, F3, APOH, IL6R, PCNA, NOTCH2

Products for genes affiliated with Nodular Regenerative Hyperplasia

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Nodular Regenerative Hyperplasia

About this section
4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet