MCID: NDL013
MIFTS: 51

Nodular Regenerative Hyperplasia malady

Rare diseases, Liver diseases categories

Aliases & Classifications for Nodular Regenerative Hyperplasia

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Sources:
42NIH Rare Diseases, 48Orphanet, 61UMLS, 26ICD10 via Orphanet
See all sources

Aliases & Descriptions for Nodular Regenerative Hyperplasia:

Name: Nodular Regenerative Hyperplasia 42
Nodular Regenerative Hyperplasia of the Liver 42 48
Non-Cirrhotic Nodulation 42 48
Non-Cirrhotic Nodular Transformation 42
 
Miliary Hepatocellular Adenomatosis 42
Non-Cirrhotic Portal Hypertension 42
Nodular Hyperplasia 61


Classifications:

Orphanet: 48 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

48

External Ids:

Orphanet48 48372
ICD10 via Orphanet26 K76.8

Summaries for Nodular Regenerative Hyperplasia

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Wikipedia:64 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 cholestasis, 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.

Related Diseases for Nodular Regenerative Hyperplasia

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

(show top 50)    (show all 126)
idRelated DiseaseScoreTop Affiliating Genes
1inflammatory bowel disease31.2TPMT, ITPA, IL6
2cholestasis30.8JAG1, NOTCH2, GGT1
3protein s deficiency30.7APOH, F3
4systemic lupus erythematosus30.7IL6, F3, TPMT, APOH
5rheumatic disease30.7APOH, TPMT, IL6R
6antiphospholipid syndrome30.7F3, APOH
7autoimmune hepatitis30.6GGT1, TPMT
8pancreatitis30.5TPMT, ITPA, IL6, GGT1
9connective tissue disease30.4IL6, F3, APOH
10ulcerative colitis30.4IL6R, IL6, ITPA, TPMT
11liver disease30.3GGT1, PCNA, IL6, F3
12rheumatoid arthritis30.3APOH, TPMT, IL6, IL6R
13myeloma30.2NOTCH1, IL6R, IL6
14hepatocellular carcinoma30.0JAG1, IL6, IL6R, PCNA, NOTCH1, GGT1
15breast cancer29.6JAG1, 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
29central retinal vein occlusion10.5APOH
30thrombophlebitis10.5APOH
31retinal artery occlusion10.5APOH
32biliary atresia10.4GGT1
33factor vii deficiency10.4F3
34arthritis10.4
35leukemia10.4
36myasthenia gravis10.4
37membranous glomerulonephritis10.4
38macroglobulinemia10.4
39adenoma10.4
40vasculitis10.4
41castleman's disease10.4
42rheumatoid vasculitis10.4
43hepatoportal sclerosis10.4
44aortic valve disease 110.4NOTCH1
45dyskeratosis congenita10.3
46membranoproliferative glomerulonephritis10.3
47hypersplenism10.3
48biliary atresia extrahepatic10.3JAG1, GGT1
49cholestasis, progressive familial intrahepatic 110.3GGT1, JAG1
50intrahepatic cholestasis10.3GGT1, JAG1

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:

 48
  • 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:

31
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:

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

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 Suite gene sharing:

(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 Pathway36
Notch Signaling Pathway36
9.2NOTCH1, NOTCH2, JAG1
12
Show member pathways
Notch signaling pathway36
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 Suite gene sharing:

(show top 50)    (show all 62)
idCompoundScoreTop Affiliating Genes
1purine analogues5010.3ITPA, TPMT
26 thioguanine4410.2TPMT, ITPA
3thiopurine4410.2ITPA, TPMT
46 mercaptopurine4410.2ITPA, TPMT
5inosine triphosphate44 2411.1ITPA, TPMT
6kaolin4410.0APOH, F3
7coumarins4410.0APOH, F3
8tocilizumab44 1110.9IL6, IL6R
9ticlopidine44 1110.9APOH, F3
10ptca449.9APOH, F3
11inosine44 2410.8TPMT, ITPA
12warfarin44 50 24 1112.7TPMT, F3, APOH
13rituximab44 50 1111.5APOH, IL6R, IL6
14diethylnitrosamine449.5PCNA, GGT1
15dextran sulfate449.5IL6, F3, APOH
16leflunomide44 50 1111.4PCNA, IL6
17aspirin44 50 28 2412.4PCNA, APOH, F3
18endotoxin449.4F3, APOH, IL6
19hcec449.4IL6, GGT1
20diethyl dithiocarbamate449.3TPMT, GGT1
21lycopene44 2410.3PCNA, GGT1
22prednisolone44 28 1111.3IL6R, IL6, TPMT, APOH
23cyclophosphamide44 50 1111.2F3, PCNA, TPMT, APOH
24propylthiouracil44 1 1111.2GGT1, PCNA, APOH
25vitamin b12449.2GGT1, APOH, PCNA
26betacarotene449.2PCNA, GGT1, APOH
27fibrinogen449.1F3, IL6R, IL6, APOH
28histamine44 28 2411.1TPMT, IL6R, IL6, PCNA
29epinephrine44 24 1111.1IL6, PCNA, F3
30cyclosporin a44 28 6011.1IL6R, PCNA, IL6, TPMT
31arsenite44 2410.0PCNA, F3, GGT1
32pentoxifylline44 1110.0F3, IL6, GGT1
33tacrolimus44 50 1110.8F3, TPMT, GGT1, PCNA
34heparin44 28 24 1111.7GGT1, APOH, F3, PCNA
35azathioprine44 1 50 1111.6PCNA, GGT1, TPMT, APOH, ITPA
36cholesterol44 28 24 1111.6IL6R, APOH, IL6, GGT1
37n acetylcysteine448.6GGT1, IL6, PCNA
38arginine448.6APOH, IL6R, GGT1, PCNA
39aspartate448.5F3, PCNA, NOTCH1, GGT1
40lactate448.5IL6, PCNA, GGT1, APOH
41indomethacin44 28 60 1111.5IL6, PCNA, GGT1
42testosterone44 60 24 1111.5IL6, GGT1, IL6R, PCNA
43retinoic acid44 249.4PCNA, NOTCH1, F3, IL6, IL6R
44nitric oxide44 24 1110.4JAG1, NOTCH1, IL6, APOH
45lipid448.1GGT1, NOTCH1, IL6R, IL6, APOH
46alanine448.1GGT1, PCNA, F3, IL6, TPMT
47vegf448.1APOH, IL6, IL6R, PCNA, NOTCH1, F3
48dexamethasone44 50 28 1111.1NOTCH1, IL6R, GGT1, PCNA, IL6
49methotrexate50 44 119.6IL6R, GGT1, PCNA, APOH, TPMT, ITPA
50creatinine447.5PCNA, GGT1, APOH, TPMT, F3, IL6R

GO Terms for genes affiliated with Nodular Regenerative Hyperplasia

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Cellular components related to Nodular Regenerative Hyperplasia according to GeneCards Suite gene sharing:

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 Suite gene sharing:

(show all 33)
idNameGO IDScoreTop Affiliating Genes
1morphogenesis of an epithelial sheetGO:000201110.1JAG1, NOTCH2
2distal tubule developmentGO:007201710.0JAG1, NOTCH1
3cell fate determinationGO:000170910.0NOTCH2, JAG1
4negative regulation of stem cell differentiationGO:0200073710.0JAG1, NOTCH1
5cardiac septum morphogenesisGO:006041110.0JAG1, NOTCH1
6response to muramyl dipeptideGO:003249510.0NOTCH1, JAG1
7neuronal stem cell maintenanceGO:009715010.0JAG1, NOTCH1
8interleukin-4 secretionGO:00726029.9NOTCH2, NOTCH1
9hepatic immune responseGO:00023849.9IL6R, IL6
10negative regulation of collagen biosynthetic processGO:00329669.9IL6R, IL6
11neutrophil mediated immunityGO:00024469.9IL6R, IL6
12interleukin-6-mediated signaling pathwayGO:00701029.9IL6, IL6R
13positive regulation of leukocyte chemotaxisGO:00026909.8IL6, IL6R
14inflammatory response to antigenic stimulusGO:00024379.8NOTCH2, NOTCH1
15positive regulation of chemokine productionGO:00327229.8IL6, IL6R
16monocyte chemotaxisGO:00025489.7IL6R, IL6
17bone remodelingGO:00468499.7NOTCH2, IL6
18defense response to Gram-negative bacteriumGO:00508299.7IL6R, IL6
19positive regulation of tyrosine phosphorylation of Stat3 proteinGO:00425179.7IL6, IL6R
20negative regulation of fat cell differentiationGO:00455999.7JAG1, IL6
21defense response to Gram-positive bacteriumGO:00508309.7IL6R, IL6
22pulmonary valve morphogenesisGO:00031849.6NOTCH1, NOTCH2, JAG1
23Notch signaling involved in heart developmentGO:00613149.6JAG1, NOTCH2, NOTCH1
24Notch receptor processingGO:00072209.6NOTCH2, JAG1, NOTCH1
25positive regulation of JAK-STAT cascadeGO:00464279.6NOTCH1, IL6
26acute-phase responseGO:00069539.6IL6, IL6R
27endocrine pancreas developmentGO:00310189.6IL6, IL6R
28Notch signaling pathwayGO:00072199.6JAG1, NOTCH2, NOTCH1
29positive regulation of interleukin-6 productionGO:00327559.5IL6, IL6R
30positive regulation of smooth muscle cell proliferationGO:00486619.5IL6R, IL6
31humoral immune responseGO:00069599.3NOTCH2, IL6, NOTCH1
32determination of left/right symmetryGO:00073689.2NOTCH1, NOTCH2
33positive regulation of apoptotic processGO:00430658.8IL6, NOTCH1, NOTCH2

Molecular functions related to Nodular Regenerative Hyperplasia according to GeneCards Suite gene sharing:

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

Sources for Nodular Regenerative Hyperplasia

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2CDC
12ExPASy
13FDA
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
57SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet