FVH
MCID: HPT085
MIFTS: 33

Hepatitis, Fulminant Viral (FVH)

Categories: Genetic diseases, Infectious diseases, Liver diseases, Rare diseases

Aliases & Classifications for Hepatitis, Fulminant Viral

MalaCards integrated aliases for Hepatitis, Fulminant Viral:

Name: Hepatitis, Fulminant Viral 57 73
Hepatitis, Fulminant Viral, Susceptibility to 57 6
Fvh 57 73
Fulminant Viral Hepatitis 58
Liver Failure, Acute 44

Characteristics:

OMIM®:

57 (Updated 05-Mar-2021)
Inheritance:
autosomal recessive

Miscellaneous:
one patient has been reported (last curated august 2019)
acute response to hepatitis a infection


HPO:

31
hepatitis, fulminant viral:
Inheritance autosomal recessive inheritance


Classifications:

Orphanet: 58  
Rare hepatic diseases


Summaries for Hepatitis, Fulminant Viral

UniProtKB/Swiss-Prot : 73 Hepatitis, fulminant viral: An autosomal recessive form of fulminant viral hepatitis, a disease that strikes otherwise healthy individuals during primary infection with common liver-tropic viruses. FVH is characterized by severe liver destruction in the absence of a preexisting liver disorder, leading to encephalopathy within 8 weeks of the onset of the first symptoms.

MalaCards based summary : Hepatitis, Fulminant Viral, also known as hepatitis, fulminant viral, susceptibility to, is related to viral hepatitis and hepatitis a. An important gene associated with Hepatitis, Fulminant Viral is IL18BP (Interleukin 18 Binding Protein). The drugs Prednisolone and Prednisolone acetate have been mentioned in the context of this disorder. Affiliated tissues include liver, kidney and brain, and related phenotypes are hepatomegaly and fatigue

More information from OMIM: 618549

Related Diseases for Hepatitis, Fulminant Viral

Graphical network of the top 20 diseases related to Hepatitis, Fulminant Viral:



Diseases related to Hepatitis, Fulminant Viral

Symptoms & Phenotypes for Hepatitis, Fulminant Viral

Human phenotypes related to Hepatitis, Fulminant Viral:

31 (show all 11)
# Description HPO Frequency HPO Source Accession
1 hepatomegaly 31 very rare (1%) HP:0002240
2 fatigue 31 very rare (1%) HP:0012378
3 type i diabetes mellitus 31 very rare (1%) HP:0100651
4 hashimoto thyroiditis 31 very rare (1%) HP:0000872
5 elevated hepatic transaminase 31 very rare (1%) HP:0002910
6 jaundice 31 very rare (1%) HP:0000952
7 gingival bleeding 31 very rare (1%) HP:0000225
8 coma 31 very rare (1%) HP:0001259
9 poor appetite 31 very rare (1%) HP:0004396
10 nausea 31 very rare (1%) HP:0002018
11 fulminant hepatitis 31 very rare (1%) HP:0004787

Symptoms via clinical synopsis from OMIM®:

57 (Updated 05-Mar-2021)
Abdomen Liver:
hepatomegaly
liver failure
hepatitis, fulminant, acute

Laboratory Abnormalities:
abnormal liver enzymes

Skin Nails Hair Skin:
icterus
jaundice, acute

Immunology:
il-18bp deficiency
enhanced il18-mediated cytotoxicity

Clinical features from OMIM®:

618549 (Updated 05-Mar-2021)

Drugs & Therapeutics for Hepatitis, Fulminant Viral

Drugs for Hepatitis, Fulminant Viral (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 64)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone Approved, Vet_approved Phase 4 50-24-8 5755
2 Prednisolone acetate Approved, Vet_approved Phase 4 52-21-1
3
Methylprednisolone Approved, Vet_approved Phase 4 83-43-2 6741
4
Prednisolone phosphate Approved, Vet_approved Phase 4 302-25-0
5
Methylprednisolone hemisuccinate Approved Phase 4 2921-57-5
6
Acetylcysteine Approved, Investigational Phase 4 616-91-1 12035
7
Prednisolone hemisuccinate Experimental Phase 4 2920-86-7
8 Methylprednisolone Acetate Phase 4
9 glucocorticoids Phase 4
10 Gastrointestinal Agents Phase 4
11 Anti-Inflammatory Agents Phase 4
12 Hormone Antagonists Phase 4
13 Hormones Phase 4
14 Neuroprotective Agents Phase 4
15 Antineoplastic Agents, Hormonal Phase 4
16 Antiemetics Phase 4
17 Pharmaceutical Solutions Phase 4
18 Protective Agents Phase 4
19 Respiratory System Agents Phase 4
20 Antioxidants Phase 4
21 Anti-Infective Agents Phase 4
22 Antiviral Agents Phase 4
23 Expectorants Phase 4
24 N-monoacetylcystine Phase 4
25 Antidotes Phase 4
26
Acetaminophen Approved Phase 2, Phase 3 103-90-2 1983
27
Formaldehyde Approved, Vet_approved Phase 2, Phase 3 50-00-0 712
28
Norepinephrine Approved Phase 2 51-41-2 439260
29
Dobutamine Approved Phase 2 34368-04-2 36811
30
Phenylacetic acid Approved Phase 2 103-82-2 999
31
Aspartic acid Approved, Nutraceutical Phase 2 56-84-8 5960
32
Ornithine Approved, Nutraceutical Phase 2 70-26-8, 3184-13-2 6262
33 Mitogens Phase 1, Phase 2
34 Neurotransmitter Agents Phase 2
35 N-Methylaspartate Phase 2
36
Bilirubin Phase 2 635-65-4 5280352
37 Vasoconstrictor Agents Phase 2
38 Ophthalmic Solutions Phase 2
39 Antimetabolites Phase 2
40
Lenograstim Approved, Investigational 135968-09-1
41
Sargramostim Approved, Investigational 123774-72-1, 83869-56-1
42
Mannitol Approved, Investigational 69-65-8 453 6251
43
Lamivudine Approved, Investigational 134678-17-4 60825
44
Adefovir dipivoxil Approved, Investigational 142340-99-6 60871
45
Clopidogrel Approved 120202-66-6, 113665-84-2 60606
46
Ticagrelor Approved 274693-27-5 9871419
47
Saxagliptin Approved 361442-04-8 11243969
48
Menthol Approved 2216-51-5 16666
49
Cysteine Approved, Nutraceutical 52-90-4 5862
50 Molgramostim Investigational 99283-10-0

Interventional clinical trials:

(show top 50) (show all 64)
# Name Status NCT ID Phase Drugs
1 Influence of Methylprednisolone Pulse Therapy on Liver Function in Patients With Graves' Orbitopathy Completed NCT03667157 Phase 4 every week IVMP therapy;very high doses IVMP therapy
2 Safety and Efficacy of Steroids in the Management of Fulminant Hepatic Failure Due to Hepatitis A Virus in the Pediatric Age Group Completed NCT02375867 Phase 4 prednisolone;methylprednisolone
3 A Multi-Center Trial to Study Acute Liver Failure: N-Acetylcysteine (NAC) Open Label Use Study Terminated NCT00896025 Phase 4 N-acetylcysteine
4 13C-Methacetin Breath Test for the Prediction of Outcome in in Acute Liver Injury or Acute Liver Failure Completed NCT02786836 Phase 2, Phase 3 13C-Methacetin
5 Randomized Study of Acetylcysteine in Patients With Acute Liver Failure Not Caused by Acetaminophen Completed NCT00004467 Phase 3 N-acetylcysteine (NAC);Placebo
6 A Multi-center Study of the Safety and Efficacy of N-acetylcysteine in the Treatment of Acute Liver Failure in Pediatric Patients Not Caused by Acetaminophen. Completed NCT00248625 Phase 3 N-acetylcysteine;Placebo
7 Registry Protocol for Tracking the Incidence of Transplant, the Incidence and Type of Cancer, and Survival Rate of Subjects Participating in Protocol VTI-206 (NCT00973817) Withdrawn NCT01452295 Phase 2, Phase 3 ELAD (Extracorporeal Liver Assist System)
8 Phase I/II Study of Recombinant Human Hepatocyte Growth Factor in Fulminant and Late-Onset Hepatic Failure Unknown status NCT00225901 Phase 1, Phase 2 Recombinant human hepatocyte growth factor
9 A Multicentre, Double-blind, Randomized, Placebo-controlled Study to Evaluate the Efficacy and the Safety of ALF-5755 in Patients With Nonacetaminophen Severe Acute Hepatitis and Early Stage Acute Liver Failure Unknown status NCT01318525 Phase 2 ALF-5755;Saline solution (0.9% NaCl)
10 Therapeutic Efficacy of L-Ornithine L-Aspartate Infusion in Patients With Acute Liver Failure: A Double- Blind, Randomized, Placebo- Controlled Study Unknown status NCT00470314 Phase 2 L-Ornithine L-Aspartate
11 Molecular Adsorbent Recirculating System (MARS®) for the Treatment of Patients With Hypoxic Hepatitis - a Prospective Randomized Controlled Clinical Study Unknown status NCT01690845 Phase 2
12 Infusione Sistemica e Loco-regionale di N-Acetilcisteina Nel Prelievo di Fegato: Uno Studio Prospettico Randomizzato Controllato. Fase II. Completed NCT01394497 Phase 2 N-acetylcystein
13 Phase 2 Evaluation of the Vitagen Extracorporeal Liver Assist Device (ELAD) System in the Management of the Patients With Fulminant Hepatic Failure Completed NCT00030225 Phase 2
14 A Phase 2a Study to Evaluate the Safety and Tolerability of OCR-002 (Ornithine Phenylacetate) in the Treatment of Patients With Acute Liver Failure/Severe Acute Liver Injury Completed NCT01548690 Phase 2 Ornithine Phenylacetate
15 Emergency Use of OCR-002 (Ornithine Phenylacetate) in the Treatment of Patients With Acute Liver Failure Completed NCT01634230 Phase 2 OCR-002
16 A Phase 2b Study to Evaluate Safety and Efficacy of LifeLiver (Bio Artificial Liver) in Acute or Acute-on-Chronic Liver Failure Patients Waiting Emergent Liver Transplantation Recruiting NCT03882346 Phase 2
17 Mesenchymal Stem Cell Transplantation for Acute-on-Chronic Liver Failure Not yet recruiting NCT03863002 Phase 1, Phase 2
18 An Open-Label, Multicenter, Historically-Controlled Study to Assess Safety and Efficacy of ELAD in Subjects With Acute Liver Failure (ALF) Terminated NCT01875874 Phase 2
19 A Placebo-Controlled, Multicenter, Double-Blind, Randomized, Pharmacokinetic and Pharmacodynamic Trial of IDN-6556 in Subjects With Acute-on-Chronic Liver Failure Terminated NCT01937130 Phase 2 IDN-6556
20 Safety and Efficacy of the Extracorporeal Liver Assist Device (ELAD®) In Patients With Fulminant Hepatic Failure (FHF) Withdrawn NCT00832728 Phase 2
21 The Safety Study of Stemchymal® (Allogeneic Adipose-Derived Mesenchymal Stem Cells) Treating on Acute Liver Failure - An Open-Label, Single-Center Phase I Trial Unknown status NCT03629015 Phase 1
22 Hepatocyte Transplantation for Acute Decompensated Liver Failure Withdrawn NCT01345565 Phase 1 human hepatocytes
23 G-CSF in Acute Liver Failure and Alcoholic Hepatitis - A Pilot Study Unknown status NCT01341951 Granulocyte colony stimulating factor
24 A Prospective Study of Evaluation of Minor Bleeding and Utility of Bleeding Criteria in Acute-on-chronic Liver Failure Patients in China Unknown status NCT03281252
25 Safety and Efficacy of hiHep Bioartificial Liver Support System to Treat Acute Liver Failure Unknown status NCT03084198
26 Relationship of Serological Growth Factors and Hepatic Progenitor Cells in Acute Liver Failure Unknown status NCT01312220
27 Right and Extended Right Hepatectomy in Patients Beyond 70 Years of Age - Liver Function and Outcome Unknown status NCT01471262
28 Better Diagnostic Tools for Children and Adolescents With Acute Liver Failure and Chronic Liver Insufficiency Unknown status NCT03509194
29 Breath Test for Patients With Acute Liver Disease for Early Detection of the Need for Transplant or Recovery Unknown status NCT01435421
30 Clinical and Basic Study for Pediatric Liver Transplantation Unknown status NCT02503384
31 HEpatic Regeneration With COupled Plasma Filtration and Adsorption for Liver Extracorporeal Detoxification Completed NCT03312036
32 Frontal Electroencephalogram Variables Are Associated With Outcome and Stage of Hepatic Encephalopathy in Acute Liver Failure Completed NCT01878305
33 A Prospective Multi-center Validating Cohort for ACLF Diagnosis and Prognosis From Ch-CANONIC Study Completed NCT03641872
34 Chinese Chronic Liver Failure Consortium Acute-on-Chronic Liver Disease and Failure Study --a Prospective Multi-center Study in China, the Largest Hepatitis B Virus High-endemic Region. Completed NCT02457637
35 Comparison of Two Extracorporeal Liver Support System (With or Without Recirculation) : MARS (Molecular Adsorbents Recirculating System) Versus SPAD (Single Pass Albumin Dialysis) in Severe Liver Failure. Completed NCT02310542
36 A Retrospective Multicenter Study of HBV-related Pre-acute-on-chronic Liver Failure in China Completed NCT03281265
37 Evaluation of Lactate Levels in as a Risk Factor for Posthepatectomy Liver Failure Completed NCT04512014
38 High Volume Plasma Exchange in Children With Acute Liver Failure and Acute on Chronic Liver Failure-A Prospective Pilot Study. Completed NCT02719210 Hypertonic 3% saline;Mannitol
39 High-Volume Plasma Exchange Versus Standard Medical Treatment in Patients With Acute Liver Failure-A Prospective Randomized Pilot Trial Completed NCT02718079 Prophylactic Antibiotics;N-acetyl-L-cysteine
40 Unknown Cause of Acute Liver Failure: Determination of Early Mortality Predictive Factors Completed NCT02041637
41 Prevention of Recurrent Hepatitis B After Liver Transplantation Completed NCT00059267 HBIG, Epivir, Hepsera
42 A Pharmacoepidemiological Study to Examine Patient Characteristics, Drug Utilization Pattern and Crude Incidence Rates of Selected Outcomes in New Users of Ticagrelor, Clopidogrel and Prasugrel in National Swedish Registries Completed NCT01276275 Risk of selected safety events
43 One Way to Diagnose Hepatic Insufficiency the First Postoperative Day After Resection: Prospective Cohort Study Completed NCT04570800
44 Spectrum, Profile and Outcome of Infections in Patients With Cirrhosis and/ or Acute on Chronic Liver Failure Admitted in Liver Specialty ICU (Intensive Care Unit)- An Observational Study Completed NCT01872767
45 A Prospective, Multisite Study to Evaluate the Impact of Measles, Mumps, Rubella and Varicella ProQuad® Vaccination in Pediatric Patients 6-24 Months of Age Who Are Being Considered and/or Evaluated for Any Solid Organ Transplant Completed NCT02687763 Early Phase 1
46 Role of 13C Methacetin Breath Test in Predicting Prognosis Among Patients With Acute or Acute on Chronic Liver Failure (ACLF). Completed NCT01946360
47 Evaluation of the Indocyangreen Elimination as a Prognostic Marker in Decompensated Cirrhosis and Acute Liver Failure Completed NCT00245310
48 Comparison of Risk of Hospitalization With Acute Liver Failure Between Patients With Type 2 Diabetes Initiating Saxagliptin and Those Initiating Other Oral Antidiabetic Treatments Completed NCT01086293
49 Pilot Trial on the Effect of Mild Hypothermia on Intracranial Pressure in Patients With Hyperacute Liver Failure Completed NCT00670124
50 The Effect of Prometheus (R) Liver Support Dialysis on Intracranial Pressure, Cerebral Oxidative Metabolism and Hemodynamics in Patients With Acute Liver Failure Completed NCT00655304

Search NIH Clinical Center for Hepatitis, Fulminant Viral

Cochrane evidence based reviews: liver failure, acute

Genetic Tests for Hepatitis, Fulminant Viral

Anatomical Context for Hepatitis, Fulminant Viral

MalaCards organs/tissues related to Hepatitis, Fulminant Viral:

40
Liver, Kidney, Brain, Thyroid, Bone Marrow, Bone, Heart

Publications for Hepatitis, Fulminant Viral

Articles related to Hepatitis, Fulminant Viral:

(show top 50) (show all 68)
# Title Authors PMID Year
1
Inherited IL-18BP deficiency in human fulminant viral hepatitis. 6 57
31213488 2019
2
Characteristics of Liver Function in Patients With SARS-CoV-2 and Chronic HBV Coinfection. 61
32553907 2021
3
COVID-19 and multiorgan failure: A narrative review on potential mechanisms. 61
33011887 2020
4
Acquired hemophagocytic lymphohistiocytosis as initial manifestation of multiple myeloma: A case report and literature review. 61
32991435 2020
5
Acute kidney injury after associating liver partition and portal vein ligation for staged hepatectomy for hepatocellular carcinoma: two case reports and a literature review. 61
32042811 2019
6
Mutation in the Neuroblastoma Amplified Sequence Gene as a Cause of Recurrent Acute Liver Failure, Acute Kidney Injury, and Status Epilepticus. 61
31889758 2019
7
Nosocomial Infections Are Frequent and Negatively Impact Outcomes in Hospitalized Patients With Cirrhosis. 61
31180922 2019
8
Predictors of Severity in Scrub Typhus. 61
31299836 2019
9
Exchange Transfusion and Cytarabine for Transient Abnormal Myelopoiesis in Hydrops Fetalis. 61
31015754 2019
10
Acute kidney injury in children with chronic liver disease. 61
29497824 2019
11
Dynamic Prognostication in Critically Ill Cirrhotic Patients With Multiorgan Failure in ICUs in Europe and North America: A Multicenter Analysis. 61
30106759 2018
12
Dengue shock syndrome complicated with acute liver failure and kidney injury, infective endocarditis, and deep vein thrombosis: a case report. 61
30373645 2018
13
Evaluation of postoperative ascites after somatostatin infusion following hepatectomy for hepatocellular carcinoma by laparotomy: a multicenter randomized double-blind controlled trial (SOMAPROTECT). 61
30139340 2018
14
Pyruvate dehydrogenase complex and lactate dehydrogenase are targets for therapy of acute liver failure. 61
29580866 2018
15
Hybrid Extracorporeal Therapies as a Bridge to Pediatric Liver Transplantation. 61
29652753 2018
16
A Case of Live Donor Liver Transplantation in Acute-on-Chronic Liver Failure with Budd-Chiari Syndrome: Donor and Recipient with Antiphospholipid Antibody Syndrome. 61
29959308 2018
17
Epidemiology of invasive pulmonary aspergillosis in patients with liver failure: Clinical presentation, risk factors, and outcomes. 61
29239266 2018
18
Outcome of 200 Pediatric Living Donor Liver Transplantations in India. 61
28849768 2017
19
Natural History and Treatment Outcomes of Severe Autoimmune Hepatitis. 61
28272079 2017
20
[Acute Liver Failure, Acute-On-Chronic Liver Failure, Hepatorenal Syndrome, Hepatopulmonary Syndrome and Portopulmonary Hypertension, Artificial Liver Support on the ICU]. 61
28641354 2017
21
[Thyroid storm associated with multiorganic dysfunction]. 61
28825582 2017
22
Extracorporeal support for patients with acute and acute on chronic liver failure. 61
26894968 2016
23
Abacavir-induced fulminant hepatic failure in a HIV/HCV co-infected patient. 61
26670894 2015
24
Management of alcohol recurrence before and after liver transplantation. 61
26193869 2015
25
Three decades of hepatitis B control with vaccination. 61
26328023 2015
26
Simple organ failure count versus CANONIC grading system for predicting mortality in acute-on-chronic liver failure. 61
25251968 2015
27
Magnetic resonance of the brain in chronic and acute liver failure. 61
24254992 2014
28
Pharmacologic cholinesterase inhibition improves survival in acetaminophen-induced acute liver failure in the mouse. 61
25139304 2014
29
Lung injury and its prognostic significance in acute liver failure. 61
24152589 2014
30
Hypoxic hepatitis and acute liver failure in a patient with newly onset atrial fibrillation and diltiazem infusion. 61
24042208 2013
31
Chronic hepatitis B in children and adolescents: epidemiology and management. 61
23529864 2013
32
Accumulation of xenotransplanted canine bone marrow cells in NOD/SCID/γc(null) mice with acute hepatitis induced by CCl4. 61
23411484 2013
33
[Nutrition in intensive care medicine: Part 2: special nutritional problems]. 61
23483230 2013
34
Reversal of dabigatran-induced bleeding with a prothrombin complex concentrate and fresh frozen plasma. 61
22997117 2012
35
Artificial liver support devices as treatment option for liver failure. 61
22482522 2012
36
Cell therapies for liver diseases. 61
22140063 2012
37
Incident user cohort study of risk for gastrointestinal bleed and stroke in individuals with major depressive disorder treated with antidepressants. 61
22466034 2012
38
Use of the molecular adsorbent recirculating system (MARS™) for the management of acute poisoning with or without liver failure. 61
22077243 2011
39
Multiple organ failure after an overdose of less than 0.4 mg/kg of colchicine: role of coingestants and drugs during intensive care management. 61
20969505 2010
40
Lack of therapeutic effects of gabexate mesilate on the hepatic encephalopathy in rats with acute and chronic hepatic failure. 61
20594263 2010
41
[Gelsolin - variety of structure and functions]. 61
20558869 2010
42
Serum peptide profiles during progression of chronic hepatitis B virus infection to liver failure. 61
20586930 2010
43
Thyroid storm with multiorgan failure. 61
20146655 2010
44
Moderate hypothermia with intracranial pressure monitoring as a therapeutic paradigm for the management of acute liver failure: a systematic review. 61
19847396 2010
45
Relative adrenal insufficiency in a patient with liver disease. 61
20611008 2009
46
Relative adrenal insufficiency in a patient with liver disease. 61
19382344 2009
47
Management of chronic hepatitis B in children. 61
19322053 2009
48
Clinical relevance of non-cardiac determinants of natriuretic peptide levels. 61
19012517 2008
49
NMDA receptors in hyperammonemia and hepatic encephalopathy. 61
17701332 2007
50
Artificial liver support system in China: a review over the last 30 years. 61
16684218 2006

Variations for Hepatitis, Fulminant Viral

ClinVar genetic disease variations for Hepatitis, Fulminant Viral:

6
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 IL18BP NM_001039660.2(IL18BP):c.508-19_528del Deletion risk factor 689420 rs1590835834 11:71712806-71712845 11:72001760-72001799

Expression for Hepatitis, Fulminant Viral

Search GEO for disease gene expression data for Hepatitis, Fulminant Viral.

Pathways for Hepatitis, Fulminant Viral

GO Terms for Hepatitis, Fulminant Viral

Sources for Hepatitis, Fulminant Viral

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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