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MCID: CRY036
MIFTS: 36

Cryptogenic Cirrhosis

Categories: Liver diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Cryptogenic Cirrhosis

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MalaCards integrated aliases for Cryptogenic Cirrhosis:

Name: Cryptogenic Cirrhosis 25
Cirrhosis, Cryptogenic 25 29 6 39 71

Classifications:

MalaCards categories:
Anatomical: Liver diseases
See all MalaCards categories (disease lists)


External Ids:

UMLS 71 C0267809
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Summaries for Cryptogenic Cirrhosis

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Genetics Home Reference : 25 Cryptogenic cirrhosis is a condition that impairs liver function. People with this condition develop irreversible liver disease caused by scarring of the liver (cirrhosis), typically in mid- to late adulthood. The liver is a part of the digestive system that helps break down food, store energy, and remove waste products, including toxins. Minor damage to the liver can be repaired by the body. However, severe or long-term damage can lead to the replacement of normal liver tissue with scar tissue. In the early stages of cryptogenic cirrhosis, people often have no symptoms because the liver has enough normal tissue to function. Signs and symptoms become apparent as more of the liver is replaced by scar tissue. Affected individuals can experience fatigue, weakness, loss of appetite, weight loss, nausea, swelling (edema), enlarged blood vessels, and yellowing of the skin and whites of the eyes (jaundice). People with cryptogenic cirrhosis may develop high blood pressure in the vein that supplies blood to the liver (portal hypertension). Cryptogenic cirrhosis can lead to type 2 diabetes, although the mechanism is unclear. Some people with cryptogenic cirrhosis develop cancer of the liver (hepatocellular cancer).

MalaCards based summary : Cryptogenic Cirrhosis, also known as cirrhosis, cryptogenic, is related to liver cirrhosis and autoimmune hepatitis. An important gene associated with Cryptogenic Cirrhosis is KRT18 (Keratin 18), and among its related pathways/superpathways are Folate Metabolism and Formation of Fibrin Clot (Clotting Cascade). The drugs Exenatide and Insulin aspart have been mentioned in the context of this disorder. Affiliated tissues include liver, skin and eye, and related phenotypes are growth/size/body region and liver/biliary system

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Related Diseases for Cryptogenic Cirrhosis

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Diseases related to Cryptogenic Cirrhosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 203)
# Related Disease Score Top Affiliating Genes
1 liver cirrhosis 32.1 KRT8 KRT18 HFE F2
2 autoimmune hepatitis 30.7 KRT8 KRT18 F2
3 non-alcoholic steatohepatitis 30.6 SERPINE1 KRT18 HFE
4 liver disease 30.2 KRT8 KRT18 HFE F2
5 hepatitis c 30.0 KRT8 KRT18 HFE
6 viral hepatitis 29.7 KRT8 KRT18 HFE F2
7 alpha-1-antitrypsin deficiency 29.6 SERPINE1 HFE F2
8 budd-chiari syndrome 29.1 MTHFR F5 F2
9 varicose veins 28.6 MTHFR HFE F5 F2
10 non-alcoholic fatty liver disease 11.8
11 fatty liver disease 10.6
12 neurenteric cyst 10.3 KRT8 KRT18
13 proliferating trichilemmal cyst 10.3 KRT8 KRT18
14 portal hypertension 10.3
15 primary biliary cirrhosis 10.3
16 intrahepatic gall duct cancer 10.3 KRT8 KRT18
17 squamous cell bile duct carcinoma 10.3 KRT8 KRT18
18 odontoma 10.3 KRT8 KRT18
19 cutaneous adenocystic carcinoma 10.3 KRT8 KRT18
20 mucinous tubular and spindle renal cell carcinoma 10.3 KRT8 KRT18
21 adhesive otitis media 10.3 KRT8 KRT18
22 lymphoepithelioma-like carcinoma 10.3 KRT8 KRT18
23 hemochromatosis, type 1 10.3
24 cholangitis 10.3
25 pleomorphic carcinoma 10.2 KRT8 KRT18
26 basaloid squamous cell carcinoma 10.2 KRT8 KRT18
27 hepatitis c virus 10.2
28 cholangitis, primary sclerosing 10.2
29 sclerosing cholangitis 10.2
30 hepatocellular carcinoma 10.2
31 hypertriglyceridemia, familial 10.2
32 mammary paget's disease 10.2 KRT8 KRT18
33 luminal breast carcinoma a 10.2 KRT8 KRT18
34 paracetamol poisoning 10.1 F5 F2
35 fatty liver disease, nonalcoholic 1 10.1
36 hepatitis b 10.1
37 hepatopulmonary syndrome 10.1
38 dic in newborn 10.1 SERPINE1 F2
39 prothrombin deficiency, congenital 10.1 F5 F2
40 sagittal sinus thrombosis 10.1 F5 F2
41 mediastinitis 10.1 F5 F2
42 sticky platelet syndrome 10.0 SERPINE1 F5
43 sneddon syndrome 10.0 F5 F2
44 factor x deficiency 10.0 F5 F2
45 hypercholesterolemia, familial, 1 10.0
46 celiac disease 1 10.0
47 body mass index quantitative trait locus 11 10.0
48 body mass index quantitative trait locus 9 10.0
49 body mass index quantitative trait locus 8 10.0
50 body mass index quantitative trait locus 4 10.0

Graphical network of the top 20 diseases related to Cryptogenic Cirrhosis:



Diseases related to Cryptogenic Cirrhosis
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Symptoms & Phenotypes for Cryptogenic Cirrhosis

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MGI Mouse Phenotypes related to Cryptogenic Cirrhosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 9.76 F2 F5 HFE KRT18 KRT8 LIPA
2 liver/biliary system MP:0005370 9.5 F5 HFE KRT18 KRT8 LIPA MTHFR
3 mortality/aging MP:0010768 9.23 F2 F5 HFE KRT18 KRT8 LIPA
Sources

Drugs & Therapeutics for Cryptogenic Cirrhosis

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Drugs for Cryptogenic Cirrhosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 22)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Exenatide Approved, Investigational Phase 4 141758-74-9 15991534
2
Insulin aspart Approved Phase 4 116094-23-6 16132418
3
Insulin detemir Approved Phase 4 169148-63-4 5311023
4 Insulin, Globin Zinc Phase 4
5 Hypoglycemic Agents Phase 4
6 insulin Phase 4
7 Liver Extracts Phase 4
8 Hormones Phase 4
9 Anti-Obesity Agents Phase 4
10 Hormone Antagonists Phase 4
11 Incretins Phase 4
12
Metformin Approved 657-24-9 14219 4091
13
Pentoxifylline Approved, Investigational 6493-05-6 4740
14
Methionine Approved, Nutraceutical 63-68-3 6137
15 Phosphodiesterase Inhibitors
16 Antioxidants
17 Vasodilator Agents
18 Platelet Aggregation Inhibitors
19 Protective Agents
20 Radiation-Protective Agents
21 Immunoglobulins
22 Antibodies

Interventional clinical trials:

(show all 14)
# Name Status NCT ID Phase Drugs
1 A New Treatment Strategy of Adding Exenatide to Insulin Therapy for Patients With Type 2 Diabetes and Non-Alcoholic Fatty Liver Disease (NAFLD) Completed NCT01006889 Phase 4 Exenatide
2 An Open Label, Multicenter, Dose Escalation Study to Evaluate the Safety, Tolerability, Efficacy, Pharmacokinetics, and Pharmacodynamics of SBC-102 (Sebelipase Alfa) in Children With Growth Failure Due to Lysosomal Acid Lipase Deficiency Completed NCT01371825 Phase 2, Phase 3 Sebelipase alfa (SBC-102)
3 An Open Label Multicenter Extension Study to Evaluate the Long-term Efficacy and Safety of SBC-102 in Children With Lysosomal Acid Lipase Deficiency Who Previously Received Treatment With SBC-102 Terminated NCT01473875 Phase 2, Phase 3 SBC-102
4 A Phase 2, Open Label, Multicenter Study to Evaluate the Safety, Tolerability, Efficacy, and Pharmacokinetics of Sebelipase Alfa in Infants With Rapidly Progressive Lysosomal Acid Lipase Deficiency Terminated NCT02193867 Phase 2 Sebelipase Alfa
5 Measurement of Fibrinogen in Patients With Systemic Inflammatory Response Syndrome, Sepsis, Chronicle Liver Disease or After Lysis on Intensive Care Units Unknown status NCT01169168
6 Assessment of the Prevalence of Lysosomal Acid Lipase Deficiency in Patients Waiting for a Liver Transplant. Unknown status NCT02852304
7 Assessement of the Prevalence of Lysosomal Acid Lipase Deficiency in Liver Post-transplant Patients Unknown status NCT02851550
8 Non Alcoholic Fatty Liver Disease (NAFLD) in Hong Kong: Natural History and Development of Liver Complications Unknown status NCT00577044
9 Prevalence and Clinical Characteristics of the Patients With Liver Cirrhosis and Different Glucose Metabolism Disorders - A Prospective Study. Unknown status NCT01396954
10 Effect of Metformin on Disease Progression in Patients With Cryptogenic Cirrhosis (NASH-related Cirrhosis) With Diabetes or Impaired Glucose Tolerance or Insulin Resistance: a Prospective Randomized, Open Labeled, Clinical Trial" Completed NCT02234440 Metformin;Insulin
11 A Randomized Controlled Trial to Study the Efficacy of S-adenosyl Methionine (SAMe) Versus Pentoxiphylline in Patients With Non-alcoholic Steatohepatitis With Fibrosis. Completed NCT02231333 S-adenosylmethionine (SAMe);pentoxiphylline (PTX)
12 The Frequency of Cholesteryl Ester Storage Disease in Children With Unexplained Transaminase Elevation and Chronic Liver Disease Completed NCT02372513
13 Undetected Bartonella Spp. Infection Puts Liver Transplant Patients at Great Risk Recruiting NCT02595710
14 PROtocol of Metabolic and Cryptogenic livEr Disease regisTry for intEgration of Omic Studies Recruiting NCT04371042

Search NIH Clinical Center for Cryptogenic Cirrhosis

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Genetic Tests for Cryptogenic Cirrhosis

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Genetic tests related to Cryptogenic Cirrhosis:

# Genetic test Affiliating Genes
1 Cirrhosis, Cryptogenic 29
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Anatomical Context for Cryptogenic Cirrhosis

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MalaCards organs/tissues related to Cryptogenic Cirrhosis:

40
Liver, Skin, Eye, Kidney, Testes, Neutrophil, Bone
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Publications for Cryptogenic Cirrhosis

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Articles related to Cryptogenic Cirrhosis:

(show top 50) (show all 746)
# Title Authors PMID Year
1
Non-amyloid liver involvement in familial Mediterranean fever: A systematic literature review. 61
32196885 2020
2
Nonalcoholic Steatohepatitis is the Most Rapidly Increasing Indication for Liver Transplantation in the United States. 61
32531342 2020
3
Pathologic findings of patients with nonalcoholic fatty liver disease and the impact of concurrent hepatitis B virus infection in Taiwan. 61
32499209 2020
4
Use of Nonvascularized Fascia in Liver Transplantation. 61
32204902 2020
5
Incidence, aetiology and related comorbidities of cirrhosis: a Swedish population-based cohort study. 61
32245414 2020
6
Non-alcoholic fatty liver disease after liver transplantation in patients with non-alcoholic steatohepatitis and cryptogenic cirrhosis: the impact of pre-transplant graft steatosis. 61
31431413 2020
7
Significance of Occult Hepatitis C Virus Infection in Liver Transplant Patients With Cryptogenic Cirrhosis. 61
30346262 2020
8
Prevalence of Occult HBV Infection in Western Countries. 61
32275083 2020
9
Etiology of cirrhosis in the young. 61
31698008 2020
10
Explant liver evaluation decodes the mystery of cryptogenic cirrhosis! 61
32055695 2020
11
Changing prevalence of aetiological factors and comorbidities among Australians hospitalised for cirrhosis. 61
32096890 2020
12
Hepatic FATP5 expression is associated with histological progression and loss of hepatic fat in NAFLD patients. 61
31602526 2020
13
Herbal Medicine in the Treatment of Non-Alcoholic Fatty Liver Diseases-Efficacy, Action Mechanism, and Clinical Application. 61
32477116 2020
14
Lysosomal acid lipase activity and liver fibrosis in the clinical continuum of non-alcoholic fatty liver disease. 61
31392821 2019
15
Acute-on-Chronic Liver Failure: Etiology of Chronic and Acute Precipitating Factors and Their Effect on Mortality. 61
31889750 2019
16
Metastatic cholangiocarcinoma in a patient with undiagnosed Wilson disease. 61
31167735 2019
17
Cryptogenic cirrhosis: Old and new perspectives in the era of molecular and genomic medicine. 61
31395291 2019
18
Effects of Share 35 Policy on Liver Transplantation Outcomes for Patients With Nonalcoholic Steatohepatitis. 61
31146627 2019
19
Reduced lysosomal acid lipase activity: A new marker of liver disease severity across the clinical continuum of non-alcoholic fatty liver disease? 61
31435171 2019
20
Cryptogenic liver cirrhosis and hepatitis E virus (HEV): Are they related? 61
31130469 2019
21
Clinical course and mortality by etiology of liver cirrhosis in Sweden: a population based, long-term follow-up study of 1317 patients. 61
30957910 2019
22
Position statement on the diagnosis and management of non-alcoholic fatty liver disease. 61
31219486 2019
23
NASH Is the Most Rapidly Growing Etiology for Acute-on-Chronic Liver Failure-Related Hospitalization and Disease Burden in the United States: A Population-Based Study. 61
30861321 2019
24
Predisposing Conditions to Pediatric Hepatocellular Carcinoma and Association With Outcomes: Single-center Experience. 61
30676520 2019
25
Outcome of Liver Transplant Patients With Intraoperative-Detected Portal Vein Thrombosis: A Retrospective Cohort Study in Shiraz, Iran. 61
30995894 2019
26
The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease. 61
30540705 2019
27
Evaluation of Underlying Liver Disease and Its Severity in Children Referred for Liver Transplant: a Single-Center Report From Nemazee Hospital of Shiraz. 61
30836904 2019
28
Fibrinolysis Shutdown Is Associated With Thrombotic and Hemorrhagic Complications and Poorer Outcomes After Liver Transplantation. 61
30548128 2019
29
Complete Heart Block due to Octreotide Infusion in Patient with Cryptogenic Cirrhosis. 61
30911266 2019
30
Senescent Hepatocytes in Decompensated Liver Show Reduced UPRMT and Its Key Player, CLPP, Attenuates Senescence In Vitro. 61
30878663 2019
31
Lysosomal Acid Lipase: Can it be a New Non-Invasive Serum Biomarker of Cryptogenic Liver Fibrosis and Cirrhosis? 61
31113613 2019
32
Technical success and outcomes in pediatric patients undergoing transjugular intrahepatic portosystemic shunt placement: a 20-year experience. 61
30291382 2019
33
Increasing Incidence of Nonalcoholic Steatohepatitis as an Indication for Liver Transplantation in Australia and New Zealand. 61
30609187 2019
34
Waiting List Mortality and Transplant Rates for NASH Cirrhosis When Compared With Cryptogenic, Alcoholic, or AIH Cirrhosis. 61
29985186 2019
35
Poor Survival After Retransplantation in NASH Cirrhosis. 61
29470354 2019
36
The conundrum of cryptogenic cirrhosis: Adverse outcomes without treatment options. 61
30144554 2018
37
A decompensated cryptogenic cirrhosis? No, a late liver histiocytosis! 61
30077466 2018
38
Nonalcoholic fatty liver disease is an increasing indication for liver transplantation in the Nordic countries. 61
29630771 2018
39
A changing etiologic scenario in liver transplantation for hepatocellular carcinoma in a multicenter cohort study from Latin America. 61
29773419 2018
40
NASH-related and cryptogenic cirrhosis similarities extend beyond cirrhosis. 61
30227919 2018
41
Reply to: "NASH-related and cryptogenic cirrhosis similarities extend beyond cirrhosis": Cryptogenic cirrhosis should not be equated with NASH cirrhosis based on UNOS data mining and Bayesian 'doctrine of chances'. 61
29983203 2018
42
Clinical characteristics and outcomes of hepatocellular carcinoma: results from prospective study, from a tertiary referral center in 
Sri Lanka 61
30415518 2018
43
Pernicious anemia associated with cryptogenic cirrhosis: Two case reports and a literature review. 61
30278550 2018
44
Non-cirrhotic portal hypertension: an imaging review. 61
29564495 2018
45
Liver Transplantation (LT) for Cryptogenic Cirrhosis (CC) and Nonalcoholic Steatohepatitis (NASH) Cirrhosis: Data from the Scientific Registry of Transplant Recipients (SRTR): 1994 to 2016. 61
30075518 2018
46
Identification of rare diseases by screening a population selected on the basis of routine pathology results-the PATHFINDER project: lysosomal acid lipase/cholesteryl ester storage disease substudy. 61
29358478 2018
47
Case report of high-dose hydroxocobalamin in the treatment of vasoplegic syndrome during liver transplantation. 61
29573551 2018
48
Role of Glutathione S Transferase M1 and T1 Gene Polymorphism in Hepatitis B Related Liver Diseases and Cryptogenic Cirrhosis. 61
29892180 2018
49
A growing hepatic mass in a cryptogenic cirrhosis patient. 61
29050462 2018
50
Raised plasma levels of H2S and nitrate predict intrapulmonary vascular dilations: A preliminary report in patients with cryptogenic cirrhosis. 61
29984390 2018
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Variations for Cryptogenic Cirrhosis

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Expression for Cryptogenic Cirrhosis

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Search GEO for disease gene expression data for Cryptogenic Cirrhosis.
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Pathways for Cryptogenic Cirrhosis

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Pathways related to Cryptogenic Cirrhosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Folate Metabolism 1
Show member pathways
 11.66 SERPINE1 MTHFR F2
2
Formation of Fibrin Clot (Clotting Cascade) 65
Show member pathways
 11.33 SERPINE1 F5 F2
3
Complement and coagulation cascades 36 1
10.96 SERPINE1 F5 F2
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GO Terms for Cryptogenic Cirrhosis

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Biological processes related to Cryptogenic Cirrhosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hemostasis GO:0007599 9.43 F5 F2
2 extrinsic apoptotic signaling pathway GO:0097191 9.4 KRT8 KRT18
3 acute-phase response GO:0006953 9.37 HFE F2
4 positive regulation of receptor-mediated endocytosis GO:0048260 9.32 SERPINE1 HFE
5 fibrinolysis GO:0042730 9.26 SERPINE1 F2
6 positive regulation of blood coagulation GO:0030194 9.16 SERPINE1 F2
7 hepatocyte apoptotic process GO:0097284 8.96 KRT8 KRT18
8 negative regulation of fibrinolysis GO:0051918 8.62 SERPINE1 F2
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Sources for Cryptogenic Cirrhosis

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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