MCID: LVR032
MIFTS: 26

Liver Adenomatosis

Categories: Liver diseases, Rare diseases

Aliases & Classifications for Liver Adenomatosis

MalaCards integrated aliases for Liver Adenomatosis:

Name: Liver Adenomatosis 58
Hepatic Adenomatosis 58

Classifications:



External Ids:

Orphanet 58 ORPHA566841

Summaries for Liver Adenomatosis

MalaCards based summary : Liver Adenomatosis, also known as hepatic adenomatosis, is related to hepatocellular adenoma and adenoma. An important gene associated with Liver Adenomatosis is HNF1A (HNF1 Homeobox A), and among its related pathways/superpathways are Sudden Infant Death Syndrome (SIDS) Susceptibility Pathways and Integrated Breast Cancer Pathway. Affiliated tissues include liver and kidney, and related phenotypes are endocrine/exocrine gland and integument

Related Diseases for Liver Adenomatosis

Diseases related to Liver Adenomatosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 81)
# Related Disease Score Top Affiliating Genes
1 hepatocellular adenoma 29.9 HNF1A CTNNB1
2 adenoma 10.5
3 maturity-onset diabetes of the young 10.2
4 maturity-onset diabetes of the young, type 3 10.1
5 hemosiderosis 10.0
6 diabetes mellitus, noninsulin-dependent 10.0
7 glycogen storage disease 10.0
8 oncocytic breast carcinoma 10.0 PGR AR
9 apocrine sweat gland neoplasm 10.0 PGR AR
10 apocrine adenocarcinoma 10.0 PGR AR
11 gender identity disorder 10.0 PGR AR
12 sweat gland disease 9.9 PGR AR
13 sweat gland cancer 9.9 PGR AR
14 prostatic hypertrophy 9.9 PGR AR
15 sertoli cell tumor 9.9 PGR AR
16 amelogenesis imperfecta, type ig 9.9 PGR AR
17 amelogenesis imperfecta 9.9 PGR AR
18 estrogen-receptor negative breast cancer 9.9 PGR AR
19 breast fibroadenoma 9.9 PGR AR
20 teeth hard tissue disease 9.9 PGR AR
21 incontinentia pigmenti 9.9
22 aplastic anemia 9.9
23 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 9.9
24 non-alcoholic fatty liver disease 9.9
25 glucose intolerance 9.9
26 gestational diabetes 9.9
27 hyperglycemia 9.9
28 liver cirrhosis 9.9
29 myeloid leukemia 9.9
30 fatty liver disease 9.9
31 erythrokeratoderma ''en cocardes'' 9.9
32 rare hereditary hemochromatosis 9.9
33 hepatoportal sclerosis 9.9
34 fatty liver disease, nonalcoholic 1 9.9
35 deficiency anemia 9.9
36 portal hypertension 9.9
37 portal vein thrombosis 9.9
38 iron deficiency anemia 9.9
39 cholestasis 9.9
40 granulomatous hepatitis 9.9
41 iron metabolism disease 9.9
42 liver disease 9.9
43 congenital extrahepatic portosystemic shunt 9.9
44 congenital portosystemic shunt 9.9
45 gynecomastia 9.9 PGR AR
46 female breast cancer 9.9 PGR AR
47 adenosquamous carcinoma 9.9 PGR AR
48 estrogen-receptor positive breast cancer 9.9 PGR AR
49 ductal carcinoma in situ 9.8 PGR AR
50 cervical serous adenocarcinoma 9.8 PGR CTNNB1

Graphical network of the top 20 diseases related to Liver Adenomatosis:



Diseases related to Liver Adenomatosis

Symptoms & Phenotypes for Liver Adenomatosis

MGI Mouse Phenotypes related to Liver Adenomatosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.67 AR CTNNB1 HNF1A PGR
2 integument MP:0010771 9.62 AR CTNNB1 HNF1A PGR
3 muscle MP:0005369 9.56 AR CTNNB1 HNF1A PGR
4 neoplasm MP:0002006 9.33 AR CTNNB1 PGR
5 reproductive system MP:0005389 9.26 AR CTNNB1 HNF1A PGR
6 skeleton MP:0005390 8.92 AR CTNNB1 HNF1A PGR

Drugs & Therapeutics for Liver Adenomatosis

Search Clinical Trials , NIH Clinical Center for Liver Adenomatosis

Genetic Tests for Liver Adenomatosis

Anatomical Context for Liver Adenomatosis

MalaCards organs/tissues related to Liver Adenomatosis:

40
Liver, Kidney

Publications for Liver Adenomatosis

Articles related to Liver Adenomatosis:

(show top 50) (show all 100)
# Title Authors PMID Year
1
Liver adenomatosis in patients with hepatocyte nuclear factor-1 alpha maturity onset diabetes of the young (HNF1A-MODY): Clinical, radiological and pathological characteristics in a French series. 61
31166087 2020
2
Natural history of liver adenomatosis: A long-term observational study. 61
31419515 2019
3
Diffuse Adenomatosis and Hepatocellular Carcinoma Treated with Liver Transplantation in an Adolescent Female with Kabuki Syndrome with a Novel KMT2D Gene Mutation. 61
31179148 2019
4
Bile Collection Detected With BrIDA Scintigraphy in a Patient With Hepatocellular Carcinoma. 61
30222677 2018
5
Incidentally detected PET-avid liver adenomatosis: MRI and 18F-FDG PET-CT findings. 61
30260790 2018
6
A case of extensive hepatic adenomatosis in a renal transplant patient. 61
30093994 2018
7
Nonalcoholic steatohepatitis and hepatic adenomatosis: casual or causal relationship? 61
29277999 2018
8
Hepatocellular Carcinoma in Fanconi-Bickel Syndrome. 61
28382841 2018
9
Role of selective intra-arterial embolization in benign liver tumors. 61
28551065 2017
10
Exuberant liver adenomatosis presenting with iron deficiency anemia. 61
28469852 2017
11
Unusual Discovery after an Examination for Abdominal Pain: Abernethy 1b Malformation and Liver Adenomatosis. A Case Report. 61
28338118 2017
12
[Extracapsular hepatic adenoma. Case report and literature review]. 61
26920214 2017
13
Hepatic adenomatosis in liver cirrhosis. 61
28932768 2017
14
Case 235: Hepatic Adenomatosis Due to Inflammatory Adenomas. 61
27755934 2016
15
Multiple liver adenomatosis with (18)F-FDG uptake: A rare manifestation of an infrequent entity. 61
26748847 2016
16
Hepatocyte Nuclear Factor 1α Mutation-associated MODY-3 and Familial Liver Adenomatosis. 61
26646800 2016
17
Living Donor Liver Transplantation for Unresectable Liver Adenomatosis Associated with Congenital Absence of Portal Vein: A Case Report and Literature Review. 61
26386552 2015
18
Treatment of Hepatic Adenomatosis. 61
26430609 2015
19
Hepatic adenomatosis: a rare but important liver disease with severe clinical implications. 61
26011213 2015
20
Management of hepatic adenomatosis. 61
25740249 2015
21
Hepatic adenomatosis: a rare cause of liver transplant. 61
25490174 2014
22
[Hepatic adenomatosis: a disease with controversial treatment]. 61
24314609 2014
23
Liver adenomatosis: serial investigation on MRI. 61
24375021 2014
24
Hepatic adenomatosis: MR imaging features. 61
25073243 2014
25
Primary hepatocellular neoplasms in a MODY3 family with a novel HNF1A germline mutation. 61
23707370 2013
26
Maturity-onset diabetes of the young and hepatic adenomatosis - characterisation of a new mutation. 61
23616187 2013
27
Hepatic adenomatosis: spectrum of imaging findings. 61
22610057 2013
28
A new long term hepatic complication in survivors of childhood haematological malignancy. 61
22951417 2012
29
Hepatic adenomatosis may mimic metastatic lesions of liver with 18F-FDG PET/CT. 61
22691518 2012
30
Early onset of liver steatosis in a Japanese girl with maturity-onset diabetes of the young type 3 (MODY3). 61
22672869 2012
31
Hepatocellular adenoma: an excellent indication for laparoscopic liver resection. 61
22568415 2012
32
Double heterozygous germline HNF1A mutations in a patient with liver adenomatosis. 61
22517943 2012
33
Case 177: Congenital absence of the portal vein with hepatic adenomatosis. 61
22190661 2012
34
Laparoscopic liver resection for hepatocellular adenoma. 61
21860698 2011
35
Combined liver kidney transplantation: critical analysis of a single-center experience. 61
21486624 2011
36
Solid hypervascular liver lesions: accurate identification of true benign lesions on enhanced dynamic and hepatobiliary phase magnetic resonance imaging after gadobenate dimeglumine administration. 61
21102346 2011
37
Management of liver adenomatosis by radiofrequency ablation. 61
21540605 2011
38
Solid focal liver lesions: dynamic and late enhancement patterns with the dual phase contrast agent gadobenate dimeglumine. 61
20405242 2010
39
[Multiple liver adenomatosis treated with surgical resection and radiofrequency]. 61
20206344 2010
40
Hepatic adenomatosis in a 7-year-old child treated earlier with a Fontan procedure. 61
20204345 2010
41
The role of liver transplantation for hepatic adenomatosis in the pediatric population: case report and review of the literature. 61
19490491 2010
42
A missense TCF1 mutation in a patient with mody-3 and liver adenomatosis. 61
21120312 2010
43
Multidetector CT findings of spontaneous rupture of hepatic adenoma in a patient with hepatic adenomatosis. 61
19517384 2009
44
Multiple spontaneous liver cell adenomas of different types (liver adenomatosis). 61
19395881 2009
45
Liver adenomatosis: a rare indication for living donor liver transplantation. 61
19460563 2009
46
Hepatic adenomatosis. 61
19061298 2008
47
[Hepatic adenomatosis]. 61
18448003 2008
48
MRI of hepatic adenomatosis: initial observations with gadoxetic acid contrast agent in three patients. 61
18430814 2008
49
Liver adenomatosis: re-evaluation of aetiology and management. 61
18339077 2008
50
[Hepatic adenomatosis in glycogen storage disease]. 61
18367864 2008

Variations for Liver Adenomatosis

Expression for Liver Adenomatosis

Search GEO for disease gene expression data for Liver Adenomatosis.

Pathways for Liver Adenomatosis

Pathways related to Liver Adenomatosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.67 CTNNB1 AR
2 11.62 CTNNB1 AR
3
Show member pathways
11.57 CTNNB1 AR
4 11.49 HNF1A CTNNB1
5 11.28 CTNNB1 AR
6
Show member pathways
11.21 PGR AR
7 11.05 CTNNB1 AR
8 10.71 CTNNB1 AR
9 10.29 HNF1A CTNNB1

GO Terms for Liver Adenomatosis

Cellular components related to Liver Adenomatosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nuclear chromatin GO:0000790 9.13 PGR HNF1A AR
2 protein-containing complex GO:0032991 8.8 HNF1A CTNNB1 AR

Biological processes related to Liver Adenomatosis according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 regulation of transcription by RNA polymerase II GO:0006357 9.7 HNF1A CTNNB1 AR
2 positive regulation of transcription, DNA-templated GO:0045893 9.67 HNF1A CTNNB1 AR
3 positive regulation of transcription by RNA polymerase II GO:0045944 9.67 PGR HNF1A CTNNB1 AR
4 positive regulation of MAPK cascade GO:0043410 9.51 CTNNB1 AR
5 steroid hormone mediated signaling pathway GO:0043401 9.48 PGR AR
6 androgen receptor signaling pathway GO:0030521 9.46 CTNNB1 AR
7 pancreas development GO:0031016 9.43 HNF1A CTNNB1
8 bone resorption GO:0045453 9.4 HNF1A CTNNB1
9 cellular process GO:0009987 9.32 CTNNB1 AR
10 reproductive structure development GO:0048608 9.26 HNF1A AR
11 epithelial cell differentiation involved in prostate gland development GO:0060742 9.16 CTNNB1 AR
12 positive regulation of epithelial cell proliferation involved in prostate gland development GO:0060769 8.96 CTNNB1 AR
13 tertiary branching involved in mammary gland duct morphogenesis GO:0060748 8.62 PGR AR

Molecular functions related to Liver Adenomatosis according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 DNA-binding transcription factor activity, RNA polymerase II-specific GO:0000981 9.7 PGR HNF1A AR
2 sequence-specific DNA binding GO:0043565 9.69 PGR HNF1A AR
3 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.63 PGR HNF1A AR
4 chromatin binding GO:0003682 9.54 HNF1A CTNNB1 AR
5 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.5 PGR HNF1A AR
6 ATPase binding GO:0051117 9.49 PGR AR
7 RNA polymerase II transcription factor binding GO:0001085 9.48 CTNNB1 AR
8 steroid hormone receptor activity GO:0003707 9.43 PGR AR
9 enzyme binding GO:0019899 9.43 PGR CTNNB1 AR
10 nuclear receptor activity GO:0004879 9.4 PGR AR
11 transcription factor binding GO:0008134 9.33 HNF1A CTNNB1 AR
12 steroid binding GO:0005496 8.96 PGR AR
13 DNA-binding transcription factor activity GO:0003700 8.92 PGR HNF1A CTNNB1 AR

Sources for Liver Adenomatosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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