Alterations in Coagulation Factor Levels in Patients With End Stage Liver Disease
Liver CirrhosisHemophilia5 moreThe goal of this observational study is to learn about the changes in coagulation factor VIII and IX levels in patients undergoing liver transplantation to help guide future management of coagulation factor replacement in patients with hemophilia and liver disease. The question we aim to answer is: should the recommendations for factor replacement in patients with hereditary bleeding disorders be altered in the setting of end stage liver cirrhosis? Participants will be asked to provide two blood samples, one at the beginning of their liver transplant, and one after their liver transplant.
Pharmacokinetic Study of Icenticaftor in Participants With Hepatic Impairment
Hepatic FailureThe primary purpose of this study is to evaluate the effect of hepatic impairment on the systemic pharmacokinetics, safety, and tolerability of icenticaftor in participants with varying degrees of hepatic impairment.
Biliary Anastomosis Using Surgical Loupe Versus Microscope in Living Donor Liver Transplantation...
Liver FailureLiver Diseases3 moreComparison of biliary complications in right lobe living donor liver transplantation patients undergoing biliary reconstructions using microscope versus surgical loupes .
Albumin Infusion in Inpatients With Decompensated Cirrhosis
Decompensated CirrhosisAcute-On-Chronic Liver FailureAlbumin infusion in patients with hospitalized decompensated, even in short-term period use, could improve survival through the reduction of systemic inflammation, which is the main driver of acute-on-chronic liver failure in cirrhosis. The effects could be highly associated with the albumin dosage. A comprehensive evaluation of the inflammation response by robust measurement is needed to prove insights into the therapeutic implications of albumin infusion. The purpose of this study is to compare the effects of different amount of human albumin infusion per week in patients with hospitalized decompensated cirrhosis on 28-day transplant-free survival and to further compare the alleviation of inflammation, reduction of incidence of nosocomial infection, spontaneous bacterial peritonitis (SBP), acute kidney injury (AKI), acute-on-chronic liver failure (ACLF), and 90-day transplant-free survival. This will be a multicenter, national, retrospective study. There will be no randomization in this retrospective study. All patients who meet the inclusion criteria and not the exclusion criteria will be enrolled. All identified patients who meet criteria will be given an ID number comprised of a site number and patient number.
Endoscopic Ultrasound Guided Liver Biopsy and Portal Pressure Registry
Liver DiseasesLiver Neoplasms5 morePatient Registry aiming to provide regional evidence documenting the clinical merit of EUS (Endoscopic_ Ultrasound) guided liver biopsy, per local standard of practice, in patients with suspected liver disease indicated for an endoscopic intervention and a liver biopsy.
Validation of a Prognostic Score for Steroid Therapy Response in Acute Severe Autoimmune Hepatitis...
Liver FailureAcute4 moreAutoimmune hepatitis (AIH) is a chronic liver disease, which is characterized by the increase of immunoglobulin G (IgG) level, the presence of auto-antibodies and a typical histology, in the absence of other liver disease. Due to the heterogeneity of AIH manifestations, different scoring systems have been validated in order to make a reliable diagnosis. The two most recent scoring systems are: the revised International Autoimmune Hepatitis Group (IAIHG) criteria and the IAIHG simplified criteria. The second one is recommended by the European Association for the Study of the Liver (EASL) clinical practice guidelines (CPGs). The EASL clinical practice guidelines suggests that the treatment of ASAIH (Acute Severe AIH) is high doses of corticosteroids (superior to 1mg/kg/day) as early as possible and a lack of improvement within seven days should lead to listing for emergency liver transplantation (LT). However, the "lack of improvement" is not objectively defined and the grading of recommendation is III (Opinions of respected authorities). The hypothesis of the study is that the previously developed decisional score on a retrospective series will prospectively allow the differentiation between patients with ASAIH (Acute Severe AIH) who respond to corticosteroid therapy and should be maintained on treatment and patients who do not respond and should be rapidly evaluated for LT. The score will be computed at day 3 since corticosteroid introduction.
To Study the Role of Plasma Von Willebrand Factor Antigen (vWF) to A Disintegrin-like and Metalloproteinase...
Acute on Chronic Liver FailureAcute on chronic liver failure (ACLF) is a syndrome characterized by acute decompensation of chronic liver disease associated with organ failures and high short- term mortality. Development of systemic inflammation and subsequent organ failures determines is associate with poor outcome and short-term mortality. Previous studies have shown that endothelial injury leading to increase in levels of and exhaustion of its cleaving protein a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS 13) which promotes the platelet microthrombi formation and subsequent organ ischemia. We propose that the vWF : ADAMTS 13 ratio can be predict the organ failure development and subsequent mortality in ACLF patients, which is considered to be a inflammatory state.
Phase II Safety Study of 2 Dose Regimens of HepaStem in Patients With ACLF
Acute-On-Chronic Liver FailureThe study will assess the safety of different dose regimens of HepaStem in cirrhotic Patients with ACLF or with acute decompensation at risk of developing ACLF up to Day 28 of the active study period.
To Compare the Response Rate of Noradrenaline vs. Terlipressin in Hepatorenal Syndrome in Patients...
Acute on Chronic Liver FailureContinous infusion of nor adrenaline + albumin Continous infusion of terlipressin + albumin Response will assessed at every 48 hour (i) Complete response: Regression of acute kidney injury stage with reduction of S. Cr within 0.3 mg/dl of baseline (ii) Partial response: Regression of acute kidney injury stage with reduction of S. Cr to ≥0.3 mg/dl above baseline (iii) No response: No regression of acute kidney injury Treatment will be extended until reversal of HRS (decrease in creatinine below 1.5 mg/dL) or for a maximum of 7 days after rescue treatment will be followed. If intolerant to terlipressin, excluded from study and rescue treatment will be given in form of noradrenaline or octreotide and midodrine.
Efficacy of Nonselective Beta Blocker vs Placebo in Patients With Acute-on-chronic Liver Failure...
Acute on Chronic Liver FailureAcute on chronic liver failure patients with HVPG (Hepatic Venous Pressure Gradient) ≥ 12 mmHg + No/small esophageal varices who present to the Department of Hepatology at Institute of Liver and Billiary Sciences, who meet the inclusion criteria and who provide informed consent.