MCID: NDL013
MIFTS: 57

Nodular Regenerative Hyperplasia malady

Rare diseases, Liver diseases categories
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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 castleman's disease, 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 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:
42NIH Rare Diseases, 62UMLS, 48Orphanet, 26ICD10 via Orphanet
See all sources

Nodular Regenerative Hyperplasia, Aliases & Descriptions:

Name: Nodular Regenerative Hyperplasia 42 62
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


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Liver diseases
Orphanet: 48 
Rare hepatic diseases


Characteristics (Orphanet epidemiological data):

48

External Ids:

ICD10 via Orphanet26 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 132)
idRelated DiseaseScoreTop Affiliating Genes
1inflammatory bowel disease31.2TPMT, ITPA, IL6
2castleman's disease31.0IL6, IL6R
3cholestasis30.8GGT1, NOTCH2, JAG1
4protein s deficiency30.7APOH, F3
5systemic lupus erythematosus30.7F3, TPMT, APOH, IL6
6rheumatic disease30.7IL6R, TPMT, APOH
7antiphospholipid syndrome30.7F3, APOH
8autoimmune hepatitis30.6TPMT, GGT1
9pancreatitis30.6TPMT, ITPA, IL6, GGT1
10connective tissue disease30.4IL6, F3, APOH
11ulcerative colitis30.4IL6R, TPMT, ITPA, IL6
12multiple sclerosis30.4IL6R, IL6, JAG1, TPMT
13rheumatoid arthritis30.3IL6R, TPMT, APOH, IL6
14myeloma30.2NOTCH1, IL6, IL6R
15breast cancer29.6JAG1, IL6R, PCNA, NOTCH2, NOTCH1
16hypertension11.0
17portal hypertension10.9
18hepatitis10.8
19hepatocellular carcinoma10.7
20primary biliary cirrhosis10.7
21peliosis hepatis10.6
22lupus erythematosus10.6
23celiac disease10.5
24liver disease10.5
25pulmonary hypertension10.5
26felty's syndrome10.5
27crest syndrome10.5
28central retinal vein occlusion10.5APOH
29thrombophlebitis10.5APOH
30common variable immunodeficiency10.5
31glomerulonephritis10.5
32polyarteritis nodosa10.5
33retinal artery occlusion10.5APOH
34biliary atresia10.4GGT1
35factor vii deficiency10.4F3
36arthritis10.4
37myasthenia gravis10.4
38macroglobulinemia10.4
39membranous glomerulonephritis10.4
40adenoma10.4
41vasculitis10.4
42rheumatoid vasculitis10.4
43hepatoportal sclerosis10.4
44aortic valve disease10.4NOTCH1
45dyskeratosis congenita10.3
46membranoproliferative glomerulonephritis10.3
47hypersplenism10.3
48biliary atresia extrahepatic10.3JAG1, GGT1
49cholestasis, progressive familial intrahepatic 110.3GGT1, JAG1
50intrahepatic cholestasis10.3JAG1, GGT1

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:

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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:

32
Liver, Bone marrow, Bone, T cells, 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:

36 (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 221)
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
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
5
Absence of nodular regenerative hyperplasia after low-dose 6-thioguanine maintenance therapy in inflammatory bowel disease patients. (18083079)
2008
6
Nodular regenerative hyperplasia of liver. (18417867)
2008
7
Histopathology of liver biopsies from a thiopurine-naA^ve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia. (18415755)
2008
8
Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine. (17504943)
2007
9
Thioguanin-induced nodular regenerative hyperplasia of the liver-ROC analysis of different MR techniques. (17221208)
2007
10
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
11
Nodular regenerative hyperplasia: not all nodules are created equal. (16799965)
2006
12
Celiac disease associated with nodular regenerative hyperplasia, pulmonary abnormalities, and IgA anticardiolipin antibodies. (16394874)
2006
13
Concomitant hepatocellular carcinoma and non-Hodgkin's lymphoma in a patient with nodular regenerative hyperplasia. (16669877)
2006
14
Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. (17063088)
2006
15
Nodular regenerative hyperplasia: a reversible entity associated with azathioprine therapy. (16607155)
2006
16
Nodular regenerative hyperplasia of the liver and Hodgkin's disease: a case report. (15727082)
2004
17
Nodular regenerative hyperplasia of the liver: an under-recognized cause of portal hypertension in hematological disorders. (15054815)
2004
18
Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody. (15194657)
2004
19
Nodular regenerative hyperplasia of the liver: a case report. (14593541)
2003
20
Nodular regenerative hyperplasia of the liver. A review of 14 cases. (11677979)
2001
21
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
22
Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature. (10663513)
2000
23
Results of liver transplantation for nodular regenerative hyperplasia. (11090022)
2000
24
Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature. (10192512)
1999
25
Nodular regenerative hyperplasia of the liver: description of two cases. (8544802)
1996
26
Childhood nodular regenerative hyperplasia of the liver complicated by severe hypoxemia. (7608831)
1995
27
Nodular regenerative hyperplasia of the liver following orthotopic liver transplantation. (7652907)
1995
28
A peculiar distribution of peroxisomes in a patient with nodular regenerative hyperplasia of the liver. (8014452)
1994
29
Nodular regenerative hyperplasia of the liver and primary SjAPgren's syndrome. (8151575)
1994
30
Nodular regenerative hyperplasia of the liver: ultrasonographic appearance and echo-guided bioptic diagnosis. (7812029)
1994
31
Nodular regenerative hyperplasia: a controversial indication for orthotopic liver transplantation. (7916934)
1994
32
Hepatic infarction and nodular regenerative hyperplasia of the liver with associated anticardiolipin antibodies in a young woman. (8071516)
1994
33
Diffuse nodular regenerative hyperplasia of the liver associated with human immunodeficiency virus and visceral leishmaniasis. (8438854)
1993
34
Nodular regenerative hyperplasia in the rat induced by a selenium-enriched diet: study of a model. (1639352)
1992
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

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Expression for genes affiliated with Nodular Regenerative Hyperplasia

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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

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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 Pathway37
Notch Signaling Pathway37
9.2NOTCH1, NOTCH2, JAG1
12
Show member pathways
Notch signaling pathway37
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 analogues5010.3ITPA, TPMT
26 thioguanine4410.2ITPA, TPMT
3thiopurine4410.2TPMT, ITPA
46 mercaptopurine4410.2ITPA, TPMT
5inosine triphosphate44 2411.1TPMT, ITPA
6kaolin4410.0F3, APOH
7coumarins4410.0F3, APOH
8tocilizumab44 1110.9IL6R, IL6
9ticlopidine44 1110.9APOH, F3
10ptca449.9F3, APOH
11inosine44 2410.8ITPA, TPMT
12warfarin44 50 24 1112.7F3, TPMT, APOH
13rituximab44 50 1111.5IL6R, IL6, APOH
14diethylnitrosamine449.5GGT1, PCNA
15dextran sulfate449.5APOH, F3, IL6
16leflunomide44 50 1111.4PCNA, IL6
17aspirin44 50 28 2412.4PCNA, F3, APOH
18endotoxin449.4IL6, F3, APOH
19hcec449.4GGT1, IL6
20diethyl dithiocarbamate449.3GGT1, TPMT
21lycopene44 2410.3GGT1, PCNA
22prednisolone44 28 1111.3IL6R, IL6, TPMT, APOH
23cyclophosphamide44 50 1111.2APOH, TPMT, F3, PCNA
24propylthiouracil44 2 1111.2APOH, PCNA, GGT1
25vitamin b12449.2GGT1, PCNA, APOH
26betacarotene449.2APOH, PCNA, GGT1
27fibrinogen449.1APOH, F3, IL6, IL6R
28histamine44 28 2411.1TPMT, IL6, IL6R, PCNA
29epinephrine44 24 1111.1F3, IL6, PCNA
30cyclosporin a44 28 6111.1TPMT, IL6, IL6R, PCNA
31arsenite44 2410.0F3, PCNA, GGT1
32pentoxifylline44 1110.0F3, IL6, GGT1
33tacrolimus44 50 1110.8GGT1, PCNA, F3, TPMT
34heparin44 28 24 1111.7APOH, F3, PCNA, GGT1
35azathioprine44 2 50 1111.6GGT1, PCNA, ITPA, TPMT, APOH
36cholesterol44 28 24 1111.6GGT1, IL6R, IL6, APOH
37n acetylcysteine448.6GGT1, PCNA, IL6
38arginine448.6APOH, IL6R, PCNA, GGT1
39aspartate448.5F3, PCNA, NOTCH1, GGT1
40lactate448.5APOH, IL6, PCNA, GGT1
41indomethacin44 28 61 1111.5IL6, PCNA, GGT1
42testosterone44 61 24 1111.5IL6, IL6R, PCNA, GGT1
43retinoic acid44 249.4NOTCH1, PCNA, IL6R, IL6, F3
44nitric oxide44 24 1110.4NOTCH1, IL6, JAG1, APOH
45lipid448.1APOH, IL6, IL6R, NOTCH1, GGT1
46alanine448.1TPMT, F3, IL6, PCNA, GGT1
47vegf448.1APOH, F3, IL6, IL6R, PCNA, NOTCH1
48dexamethasone44 50 28 1111.1IL6, IL6R, PCNA, NOTCH1, GGT1
49methotrexate50 44 119.6GGT1, APOH, TPMT, ITPA, IL6, IL6R
50creatinine447.5APOH, 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: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:0055155.7GGT1, 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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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet