MCID: NDL013
MIFTS: 47

Nodular Regenerative Hyperplasia

Categories: Rare diseases, Liver diseases

Aliases & Classifications for Nodular Regenerative Hyperplasia

MalaCards integrated aliases for Nodular Regenerative Hyperplasia:

Name: Nodular Regenerative Hyperplasia 50 69
Nodular Regenerative Hyperplasia of the Liver 50 56
Non-Cirrhotic Nodulation 50 56
Non-Cirrhotic Nodular Transformation 50
Miliary Hepatocellular Adenomatosis 50
Non-Cirrhotic Portal Hypertension 50
Nodular Hyperplasia 69

Characteristics:

Orphanet epidemiological data:

56
nodular regenerative hyperplasia of the liver
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 56  
Rare hepatic diseases


External Ids:

Orphanet 56 ORPHA48372
ICD10 via Orphanet 34 K76.8
SNOMED-CT via HPO 65 34742003

Summaries for Nodular Regenerative Hyperplasia

NIH Rare Diseases : 50 the following summary is from orphanet, a european reference portal for information on rare diseases and orphan drugs.orpha number: 48372disease definitionnodular regenerative hyperplasia of the liver is a rare parenchymatous liver disease characterized by diffuse benign transformation of the hepatic parenchyma into multiple small nodules (composed of regenerating hepatocytes) and that is usually asymptomatic but can lead to the development of non-cirrhotic portal hypertension and its complications, including esophageal variceal bleeding, hypersplenism and ascites. it is often associated with rheumatologic, autoimmune, hematologic, and myeloproliferative disorders as well as various immune deficiency states and exposure certain drugs and toxins.visit the orphanet disease page for more resources. last updated: 4/14/2015

MalaCards based summary : Nodular Regenerative Hyperplasia, also known as nodular regenerative hyperplasia of the liver, is related to portal hypertension and hepatitis, and has symptoms including portal hypertension and abnormality of the hepatic vasculature. An important gene associated with Nodular Regenerative Hyperplasia is NOTCH1 (Notch 1), and among its related pathways/superpathways are Notch signaling pathway (KEGG) and Th17 cell differentiation. The drugs Caffeine and Analgesics have been mentioned in the context of this disorder. Affiliated tissues include liver, bone marrow and bone, and related phenotypes are growth/size/body region and cellular

Wikipedia : 72 Nodular regenerative hyperplasia is a form of liver hyperplasia associated with portal hypertension... more...

Related Diseases for Nodular Regenerative Hyperplasia

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

(show top 50) (show all 90)
id Related Disease Score Top Affiliating Genes
1 portal hypertension 10.5
2 hepatitis 10.4
3 hepatocellular carcinoma 10.2
4 inflammatory bowel disease 10.2
5 myofibromatosis, infantile 2 10.2 JAG1 NOTCH1
6 primary biliary cirrhosis 10.2
7 secondary lacrimal atrophy 10.2 JAG1 NOTCH1
8 anterior horn cell disease 10.2 NOTCH1 NOTCH2
9 opiate dependence 10.2 JAG1 NOTCH1
10 lupus erythematosus 10.2
11 cardiomyopathy, infantile hypertrophic 10.1 ITPA TPMT
12 raine syndrome 10.1 NOTCH1 NOTCH2
13 systemic lupus erythematosus 10.1
14 pulmonary hypertension 10.1
15 liver disease 10.1
16 peliosis hepatis 10.1
17 deafness, autosomal recessive 104 10.1 ITPA TPMT
18 trophoblastic neoplasm 10.1 JAG1 NOTCH1
19 celiac disease 10.1
20 common variable immunodeficiency 10.1
21 lymphoma 10.1
22 crest syndrome 10.1
23 anuria 10.0 NOTCH1 NOTCH2
24 clear cell ependymoma 10.0 JAG1 NOTCH1
25 budd-chiari syndrome 10.0
26 glomerulonephritis 10.0
27 polyarteritis nodosa 10.0
28 arthritis 9.9
29 leukemia 9.9
30 myasthenia gravis 9.9
31 thrombosis 9.9
32 cholestasis 9.9
33 pancreatitis 9.9
34 connective tissue disease 9.9
35 adenoma 9.9
36 vasculitis 9.9
37 macroglobulinemia 9.9
38 rheumatoid vasculitis 9.9
39 hepatoportal sclerosis 9.9
40 ptosis 9.9 NOTCH1 NOTCH2
41 rheumatoid arthritis 9.8
42 breast cancer 9.8
43 hodgkin lymphoma 9.8
44 aplastic anemia 9.8
45 sickle cell anemia 9.8
46 multiple myeloma 9.8
47 ataxia-telangiectasia 9.8
48 autoimmune hepatitis 9.8
49 b-cell lymphomas 9.8
50 crohn's disease 9.8

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



Diseases related to Nodular Regenerative Hyperplasia

Symptoms & Phenotypes for Nodular Regenerative Hyperplasia

Human phenotypes related to Nodular Regenerative Hyperplasia:

56 32
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 portal hypertension 56 32 occasional (7.5%) Occasional (29-5%) HP:0001409
2 abnormality of the hepatic vasculature 56 Very frequent (99-80%)

MGI Mouse Phenotypes related to Nodular Regenerative Hyperplasia:

44 (show all 11)
id Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.09 F3 NOTCH1 GFER ITPA NOTCH2 EFNB2
2 cellular MP:0005384 10.02 JAG1 F3 NOTCH1 GFER ITPA EFNB2
3 hematopoietic system MP:0005397 10.01 JAG1 F3 NOTCH1 ITPA EFNB2 NOTCH2
4 cardiovascular system MP:0005385 9.97 EFNB2 JAG1 F3 NOTCH1 ITPA NOTCH2
5 homeostasis/metabolism MP:0005376 9.91 EFNB2 JAG1 F3 GFER NOTCH1 NOTCH2
6 mortality/aging MP:0010768 9.91 JAG1 F3 NOTCH1 GFER ITPA EFNB2
7 embryo MP:0005380 9.85 EFNB2 JAG1 F3 NOTCH1 NOTCH2
8 integument MP:0010771 9.8 JAG1 F3 NOTCH1 ITPA EFNB2 NOTCH2
9 muscle MP:0005369 9.63 JAG1 F3 NOTCH1 ITPA EFNB2 NOTCH2
10 respiratory system MP:0005388 9.35 EFNB2 F3 NOTCH1 ITPA NOTCH2
11 vision/eye MP:0005391 9.02 EFNB2 JAG1 F3 NOTCH1 NOTCH2

Drugs & Therapeutics for Nodular Regenerative Hyperplasia

Drugs for Nodular Regenerative Hyperplasia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 44)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Caffeine Approved, Nutraceutical Phase 4 58-08-2 2519
2 Analgesics Phase 4
3 Analgesics, Non-Narcotic Phase 4
4 Anti-Inflammatory Agents Phase 4
5 Anti-Inflammatory Agents, Non-Steroidal Phase 4
6 Antirheumatic Agents Phase 4
7 Calcium, Dietary Phase 4
8 Mesalamine Phase 4 89-57-6
9 Peripheral Nervous System Agents Phase 4
10 Calcium Supplement Nutraceutical Phase 4
11
Propranolol Approved, Investigational Phase 3 525-66-6 4946
12 Adrenergic Agents Phase 3
13 Adrenergic Antagonists Phase 3
14 Adrenergic beta-Antagonists Phase 3
15 Anti-Arrhythmia Agents Phase 3
16 Antihypertensive Agents Phase 3
17 Neurotransmitter Agents Phase 3
18 Vasodilator Agents Phase 3
19
Epinephrine Approved, Vet_approved 51-43-4 5816
20 Racepinephrine Approved
21
Etomidate Approved 33125-97-2 36339 667484
22
Formaldehyde Approved, Vet_approved 50-00-0 712
23 Liver Extracts
24 Epinephryl borate
25 glucocorticoids
26 Hormone Antagonists
27 Hormones
28 Hormones, Hormone Substitutes, and Hormone Antagonists
29 Hematinics
30 Adrenergic Agonists
31 Adrenergic alpha-Agonists
32 Adrenergic beta-Agonists
33 Anesthetics
34 Anesthetics, General
35 Anesthetics, Intravenous
36 Anti-Asthmatic Agents
37 Autonomic Agents
38 Bronchodilator Agents
39 Central Nervous System Depressants
40 Hypnotics and Sedatives
41 Mydriatics
42 Respiratory System Agents
43 Vasoconstrictor Agents
44 alanine Nutraceutical

Interventional clinical trials:

(show all 17)

id Name Status NCT ID Phase Drugs
1 Clinical Management of Childhood Intestinal Lymphoid Nodular Hyperplasia Unknown status NCT01789294 Phase 4 Mesalamine
2 Comparison of Endoscopic Variceal Ligation (EVL) With Propranolol in Non Cirrhotic Portal Hypertension (NCPH) Completed NCT01000779 Phase 3 Propranolol
3 Contrast Enhanced Ultrasound For The Evaluation Of Focal Liver Lesions Unknown status NCT01329458
4 Contrast Enhanced (CE) Ultrasound and CE Computed Tomography or CE Magnetic Resonance Imaging in Liver Masses Unknown status NCT00828607
5 Risk Factors for Variceal Bleeding in Egyptian Patients With Non-Cirrhotic Portal Hypertension Completed NCT02635815
6 Quantitative Real-time Ultrasound Elastography for Characterisation of Liver Tumors Completed NCT02156700
7 Configuration of a New Prostate Disease Nomogram Predicting Prostate Biopsy Outcome Completed NCT01826617
8 Superb Microvascular Imaging in Focal Nodular Hyperplasia Recruiting NCT02737865 Sonazoid
9 Natural History of Noncirrhotic Portal Hypertension Recruiting NCT02417740
10 Mutations of Glucocorticoid Receptor in Bilateral Adrenal Hyperplasia Recruiting NCT02810496
11 Efficacy and Tolerability of Hemopatch After Hepatic Resection Recruiting NCT03323359
12 Comparison of Hydro-dissection Versus Ultrasonic Aspirator in Division of Liver Parenchyma in Laparoscopic Resection Recruiting NCT03208192
13 Evaluation of Patients With Liver Disease Recruiting NCT00001971
14 A Nation-wide Hospital-based Hepatitis B Registry:China Registry of Hepatitis B Recruiting NCT03108794
15 11C-Metomidate PET Versus Adrenal Vein Sampling in Primary Aldosteronism Active, not recruiting NCT01567111
16 Evaluation of Gastroesophageal Varices by Transnasal Endoscopy. Enrolling by invitation NCT02767206
17 Expression of Protein Convertases in Thyroid Nodules Enrolling by invitation NCT03160482

Search NIH Clinical Center for Nodular Regenerative Hyperplasia

Genetic Tests for Nodular Regenerative Hyperplasia

Anatomical Context for Nodular Regenerative Hyperplasia

MalaCards organs/tissues related to Nodular Regenerative Hyperplasia:

39
Liver, Bone Marrow, Bone, Kidney, Prostate, Thyroid, T Cells

Publications for Nodular Regenerative Hyperplasia

Articles related to Nodular Regenerative Hyperplasia:

(show top 50) (show all 241)
id Title Authors Year
1
Nodular regenerative hyperplasia rarely leads to liver transplantation: A 20-year cohort study in all Dutch liver transplant units. ( 28815029 )
2017
2
A Rare Cause of Portal Hypertension: Behcet's Disease and Nodular Regenerative Hyperplasia of the Liver. ( 28465872 )
2017
3
Nodular Regenerative Hyperplasia of the Liver in Patients with IBD Treated with Allopurinol-Thiopurine Combination Therapy. ( 28151736 )
2017
4
Nodular Regenerative Hyperplasia: A Case of Rare Prognosis. ( 28491877 )
2017
5
Epigastric Pain in a Patient With Nodular Regenerative Hyperplasia. ( 26827806 )
2016
6
Ataxia telangiectasia associated with nodular regenerative hyperplasia. ( 27671921 )
2016
7
The Prevalence of Nodular Regenerative Hyperplasia in Inflammatory Bowel Disease Patients Treated with Thioguanine Is Not Associated with Clinically Significant Liver Disease. ( 27482972 )
2016
8
A case of idiopathic portal hypertension associated with nodular regenerative hyperplasia-like nodule of the liver and mixed connective tissue disease. ( 27151480 )
2016
9
Indolent cytotoxic T cell lymphoproliferation associated with nodular regenerative hyperplasia: a common liver lesion in the context of common variable immunodeficiency disorder. ( 26493984 )
2015
10
P437. Short term prevalence of nodular regenerative hyperplasia of the liver in IBD patients treated with allopurinol-thiopurine combination therapy. ( 25717925 )
2015
11
Diagnosing Nodular Regenerative Hyperplasia of the Liver Is Thwarted by Low Interobserver Agreement. ( 26054009 )
2015
12
Prevalence and clinical significance of nodular regenerative hyperplasia in liver biopsies. ( 26415006 )
2015
13
A case of nodular regenerative hyperplasia of the liver combined with toxic hepatitis. ( 25603855 )
2015
14
Development of nodular regenerative hyperplasia (NRH) with portal hypertension following the administration of oxaliplatin for the recurrence of colon cancer. ( 25748953 )
2015
15
Hepatic Lesions Caused by Large Granular Lymphocyte Leukemia in Fischer 344 Rats: Similar Morphologic Features and Morphogenesis to Those of Nodular Regenerative Hyperplasia (NRH) in the Human Liver. ( 25903270 )
2015
16
Nodular Regenerative Hyperplasia in Patients Undergoing Liver Resection for Colorectal Metastases After Chemotherapy: Risk Factors, Preoperative Assessment and Clinical Impact. ( 25845431 )
2015
17
Nodular Regenerative Hyperplasia after Liver Transplantation Complicated with Inferior Vena Cava Stenosis: a Clue for Etiopathogenesis? ( 26405712 )
2015
18
Hepatopulmonary syndrome associated with nodular regenerative hyperplasia after liver transplantation in a child. ( 24820314 )
2014
19
Nodular regenerative hyperplasia of the liver and cutaneous T-cell lymphoma: an unreported association. ( 25075662 )
2014
20
Nodular Regenerative Hyperplasia After Treatment With Trastuzumab Emtansine. ( 24778392 )
2014
21
Concurrent liver hodgkin lymphoma and nodular regenerative hyperplasia on an explanted liver with clinical diagnosis of alcoholic cirrhosis at university hospital fundaciA^n santa fe de bogotA!. ( 24511402 )
2014
22
Nodular Regenerative Hyperplasia Causing Portal Hypertension in a Patient with Chronic Graft versus Host Disease: Response to Sirolimus. ( 24434665 )
2014
23
Hepatocellular carcinoma and nodular regenerative hyperplasia after chemotherapy for metastatic colorectal carcinoma. ( 24604623 )
2014
24
Drug-induced nodular regenerative hyperplasia. ( 24879987 )
2014
25
Splenic artery ligation for severe oxaliplatin induced portal hypertension: a way to improve postoperative course and allow adjuvant chemotherapy for colorectal liver metastases.: Letter to editor: comment about "Nodular regenerative hyperplasia (NRH) complicating oxaliplatin chemotherapy in patients undergoing resection of colorectal liver metastases". ( 24613742 )
2014
26
Human immunodeficiency virus and nodular regenerative hyperplasia of liver: A systematic review. ( 24653794 )
2014
27
Hepatoportal sclerosis (obliterative portal venopathy) and nodular regenerative hyperplasia in a patient with myasthenia gravis: A case report and review of the published work. ( 23675894 )
2013
28
Familial occurrence of nodular regenerative hyperplasia of the liver. ( 23287953 )
2013
29
Patients with nodular regenerative hyperplasia should be considered for hepatocellular carcinoma screening. ( 23607291 )
2013
30
Nodular regenerative hyperplasia in a patient with generalized essential telangiectasia: Endotheliopathy as causal factor. ( 24273032 )
2013
31
Downregulation of the Endothelial Genes Notch1 and EphrinB2 in Patients with Nodular Regenerative Hyperplasia. ( 23870033 )
2013
32
Nodular regenerative hyperplasia related portal hypertension in a patient with hypogammaglobulinaemia. ( 23801845 )
2013
33
Portal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer. ( 23984149 )
2013
34
Review article: the association between nodular regenerative hyperplasia, inflammatory bowel disease and thiopurine therapy. ( 24099468 )
2013
35
Nodular regenerative hyperplasia in common variable immunodeficiency. ( 23420139 )
2013
36
Nodular regenerative hyperplasia of the liver as a complication of long-standing systemic-onset juvenile idiopathic arthritis. ( 24315465 )
2013
37
Lengthy follow-up after liver transplantation for nodular regenerative hyperplasia in human immunodeficiency virus-infected patients: does the disease recur? ( 24296606 )
2013
38
Unusual Nodular Regenerative Hyperplasia Mimicking Liver Metastases in CEUS. ( 24327467 )
2013
39
Nodular regenerative hyperplasia (NRH) complicating oxaliplatin chemotherapy in patients undergoing resection of colorectal liver metastases. ( 24370284 )
2013
40
Interstitial pneumonia and nodular regenerative hyperplasia of the liver as initial manifestations of polyarteritis nodosa. ( 22449674 )
2012
41
Portal hypertension in nodular regenerative hyperplasia: a mixed bag! ( 22823913 )
2012
42
Noncirrhotic Portal Hypertension due to Nodular Regenerative Hyperplasia Treated with Surgical Portacaval Shunt. ( 22956964 )
2012
43
Nodular regenerative hyperplasia of the liver and portal thrombosis. ( 22880521 )
2012
44
Hepatic hemodynamics in 24 patients with nodular regenerative hyperplasia and symptomatic portal hypertension. ( 22554152 )
2012
45
Diffuse Muscular Pain, Skin Tightening, and Nodular Regenerative Hyperplasia Revealing Paraneoplastic Amyopathic Dermatomyositis due to Testicular Cancer. ( 23316407 )
2012
46
Clinical analysis of 15 cases of liver nodular regenerative hyperplasia. ( 22707296 )
2012
47
Successful use of ursodeoxycholic acid in nodular regenerative hyperplasia of the liver. ( 21450925 )
2011
48
Nodular regenerative hyperplasia of the liver: coral atoll-like lesions on ultrasound are characteristic in predisposed patients. ( 21697407 )
2011
49
The role of nucleoside and nucleotide analogues in nodular regenerative hyperplasia in HIV-infected patients: a case control study. ( 21056493 )
2011
50
A patient with Crohn's disease and portal hypertension. Nodular regenerative hyperplasia. ( 21276765 )
2011

Variations for Nodular Regenerative Hyperplasia

Expression for Nodular Regenerative Hyperplasia

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

Pathways for Nodular Regenerative Hyperplasia

Pathways related to Nodular Regenerative Hyperplasia according to GeneCards Suite gene sharing:

(show all 13)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.24 JAG1 NOTCH1 NOTCH2
2
Show member pathways
12.22 JAG1 NOTCH1 NOTCH2
3
Show member pathways
12.15 JAG1 NOTCH1 NOTCH2 PCNA
4 11.84 JAG1 NOTCH1 NOTCH2
5
Show member pathways
11.59 JAG1 NOTCH1 NOTCH2
6 11.2 JAG1 NOTCH1 PCNA
7 11.15 JAG1 NOTCH1
8 11 NOTCH1 NOTCH2
9 10.96 EFNB2 F3 JAG1 NOTCH1 NOTCH2
10 10.86 ITPA TPMT
11 10.62 JAG1 NOTCH1
12 10.56 JAG1 NOTCH1 NOTCH2
13 10.55 JAG1 NOTCH1

GO Terms for Nodular Regenerative Hyperplasia

Biological processes related to Nodular Regenerative Hyperplasia according to GeneCards Suite gene sharing:

(show all 16)
id Name GO ID Score Top Affiliating Genes
1 animal organ morphogenesis GO:0009887 9.65 EFNB2 JAG1 NOTCH2
2 Notch signaling pathway GO:0007219 9.63 JAG1 NOTCH1 NOTCH2
3 negative regulation of cell differentiation GO:0045596 9.56 JAG1 NOTCH1
4 negative regulation of neuron differentiation GO:0045665 9.55 JAG1 NOTCH1
5 positive regulation of Notch signaling pathway GO:0045747 9.54 JAG1 NOTCH1
6 neuronal stem cell population maintenance GO:0097150 9.52 JAG1 NOTCH1
7 cell fate determination GO:0001709 9.51 JAG1 NOTCH2
8 negative regulation of stem cell differentiation GO:2000737 9.49 JAG1 NOTCH1
9 response to muramyl dipeptide GO:0032495 9.43 JAG1 NOTCH1
10 cardiac septum morphogenesis GO:0060411 9.4 JAG1 NOTCH1
11 venous blood vessel morphogenesis GO:0048845 9.37 EFNB2 NOTCH1
12 regulation of developmental process GO:0050793 9.32 NOTCH1 NOTCH2
13 distal tubule development GO:0072017 9.26 JAG1 NOTCH1
14 positive regulation of aorta morphogenesis GO:1903849 9.16 EFNB2 NOTCH1
15 pulmonary valve morphogenesis GO:0003184 9.13 JAG1 NOTCH1 NOTCH2
16 Notch signaling involved in heart development GO:0061314 8.8 JAG1 NOTCH1 NOTCH2

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

id Name GO ID Score Top Affiliating Genes
1 Notch binding GO:0005112 8.62 JAG1 NOTCH1

Sources for Nodular Regenerative Hyperplasia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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