Liver Transplantation For Decompensated Cirrhosis Research Articles

Outcome of hepatitis C-related liver transplantation in direct-acting antiviral era.

Hepatitis C virus (HCV) has become an easily treatable disease after the introduction of sofosbuvir-based direct-acting antiviral (DAA) regimens. This is a large single center experience of changing severity and outcome profile of HCV-related liver disease after availability of DAAs. A retrospective analysis of prospectively collected liver transplantation (LT) database of adults (age > 18years at the time of LT) was performed from June 2010 to July 2018. A total of 410 patients (including 26 co-infection with hepatitis B) underwent LT for hepatitis C-related decompensated cirrhosis and/or hepatocellular carcinoma (HCC) out of 1754 adult transplantation in the defined period. The study group comprised of 296 males and 114 females aged 52.1 ± 7.9years. HCV-related decompensated cirrhosis and/or HCC as indication of LT was present in 289/1016 (28.4%) during 2010-2014, which was reduced to 121/738 (16.3%) during2015-2018 (p = 0.000). The LT recipients for HCV-related cirrhosis had significantly lower Child's and model for end-stage liver disease (MELD) score during 2015-2018 as compared to that during2010-2014; Child's score was 7.9 ± 2.2 vs. 8.6 ± 2.1, p = 0.003; MELD scorewas 13.9 ± 5.3 vs. 17.1 ± 5.8, p = 0.000, respectively. There was a trend towards better survival in HCV patients during 2015-2018 as compared to that during 2010-2014. Significantly more patients had HCV RNA negative status before LT during 2015-2018 (38.8% vs. 13%, p = 0.000); moreover, the proportion of LT for decompensated cirrhosis (without HCC) decreased significantly in the latter period, 64.0% vs. 42.1% (p = 0.000). In the DAA era, HCV as an indication for LT has decreased and patients have less severe disease at transplantation. There is a trend towards better patient survival.

Efficacy and safety profile of calcineurin inhibitor salvage therapy in autoimmune hepatitis

Background As data is limited on the outcomes of calcineurin inhibitors (CNI) in autoimmune hepatitis (AIH), we evaluated the efficacy and safety of CNI in AIH patients who failed prior treatment(s). Methods A retrospective study was performed of AIH patients who received cyclosporine A (CsA) and/or tacrolimus (TAC) after prior treatment(s) failure. Records were reviewed for baseline demographic and clinical characteristics, and treatment outcomes. The primary outcome was biochemical remission. Results: Thirty-three AIH patients received CNI across seven liver centers:17 received CsA, 21 TAC and 5 TAC after CsA failure/intolerance. 82% received CNI for an insufficient response to treatment(s). Overall, 48% of CNI treated patients achieved biochemical remission including 41% in prior non-responders and 83% in treatment intolerant patients. Remission rates with CNI as second-line and third-line therapy were 63% and 29% respectively. There were no baseline predictors of response to CNI on multivariate analysis. Eighteen (55%) patients developed significant side effects and 8 (24%) discontinued due to intolerance. Three patients required liver transplantation for decompensated cirrhosis and 6 patients died including one from malignancy possibly related to CNI. Conclusion CNI salvage therapy is well tolerated and moderately effective achieving remission in around 50% of AIH who failed standard therapy.

S2581 Distal (Type I) Renal Tubular Acidosis Associated With Primary Sclerosing Cholangitis

INTRODUCTION: Distal Renal Tubular Acidosis (dRTA) is associated with conditions such as Sjogren's syndrome, lupus, rheumatoid arthritis, primary biliary cholangitis, autoimmune hepatitis, Wilson disease and other conditions. We present a case of a 25-year-old man with primary sclerosing cholangitis (PSC) presenting with dRTA. CASE DESCRIPTION/METHODS: A 25-year-old man with a history of decompensated cirrhosis due to primary sclerosing cholangitis complicated by encephalopathy, esophageal varices presented to the hospital with worsened hepatic encephalopathy. His serum chemistries revealed hypokalemia and non-anion gap metabolic acidosis [serum sodium 140 mEq/L (normal: 136-145), serum potassium 3.3 mEq/L (normal: 3.5-5.1), serum chloride 116 mEq/L (normal: 98-107), serum bicarbonate 16 mEq/L (normal: 22-32), serum albumin 2.3 g/dl (normal: 3.4-5), bilirubin 37.4 mg/dl (normal < 1), alkaline phosphatase 151 U/L (normal: 45-117), aspartate aminotransferase 222 U/L (normal: 15-37), alanine aminotransferase 128 U/L (normal: 16-61)]. Work up was initiated to evaluate the cause of non-anion gap metabolic acidosis. In absence of fistula, ileal conduit, diarrhea, contributing medications, hyperparathyroidism or saline administration with urinary pH 6.0, urine osmolal gap 15, urinary sodium 84 mEq/L, normal urine uric acid and amino acid excretion, a diagnosis of dRTA in the setting of PSC was made. Acidosis was corrected by supplementation of bicarbonate. Encephalopathy responded to lactulose/rifaximin administration. Patient underwent liver transplantation for decompensated cirrhosis which was curative in resolving his dRTA. DISCUSSION: Distal type I renal tubular acidosis (dRTA) may be hereditary, associated with autoimmune diseases (Sjogren, lupus, rheumatoid arthritis and others), chemotherapy drugs or toluene intoxication. In GI realm, dRTA has traditionally been associated with primary biliary cholangitis, autoimmune hepatitis and Wilson disease. Putated mechanisms for its pathogenesis involve gene mutation in hereditary forms, antibodies against vacuolar H+-ATPase, carbonic anhydrase II enzymes in autoimmune forms, direct toxic effect of bilirubin on the renal tubules in cholestatic conditions. Correction of acidosis should be accomplished using potassium citrate – potassium bicarbonate tablets as they contain more mEq of bicarbonate per tablet than commonly used sodium bicarbonate. This abstract helps raise awareness amongst fellows and practicing gastroenterologists in recognizing PSC as a cause of dRTA.

An atypical presentation of biliary cast syndrome (BCS)

A 33-years-old man underwent liver transplantation for decompensated cirrhosis in August 2014. Eight weeks after transplantation, he presented a significant rise in cholestasis and diffuse biliary dilation. Neither biliary strictures or biliary casts (BC) were detected on non-enhanced T1-weighted sequences (Panel A) in MRI. An ERCP was subsequently performed with diagnosis of intra-ductal casts. The patient was re-transplanted in December 2014. In the explant, diffuse BCs were detected inside the biliary tree (Panel B).

Long-term survival after portal vein arterialization for portal vein thrombosis in orthotopic liver transplantation

Portal vein thrombosis is a relatively common finding during liver transplantation. The management of portal vein thrombosis during liver transplantation is technically demanding and ensures adequate portal flow to the liver graft. Eversion thromboendovenectomy and bypass using a patent splanchnic vein and cavoportal hemitransposition are the most often used procedures to treat portal vein thrombosis. There have been anecdotal reports of portal vein arterialization. We report a case of portal vein arterialization during orthotopic liver transplantation for decompensated cirrhosis. When thromboendovenectomy failed to restore sufficient portal flow and completion of arterial anastomosis between the recipient hepatic artery and the donor celiac trunk, a calibrated end-to-side anastomosis between the donor splenic artery and the donor portal vein was performed. With a 6-year follow-up, there are no symptoms related to portal hypertension, liver function is normal. However, an aneurismal dilatation of the portal branches has progressively developed. Calibrated portal vein arterialization is a possible option for portal vein thrombosis in liver transplantation, allowing long-term patient and graft survival.

The natural history of non-alcoholic fatty liver disease in children: a follow-up study for up to 20 years

Objectives:The long-term prognosis of non-alcoholic fatty liver disease (NAFLD) in children remains uncertain. We aimed at determining the long-term outcomes and survival of children with NAFLD.Design:Retrospective longitudinal hospital-based cohort study.Patients:Sixty-six...

Liver transplantation for decompensated cirrhosis after jejunoileal bypass: a strategy for management.

Although jejunoileal bypass results in end-stage liver disease in up to 100% of patients, little is known about outcome after liver transplantation. The clinical courses of six patients who underwent liver transplantation at UCLA for decompensated cirrhosis owing to a jejunoileal bypass were reviewed. Liver function, allograft pathology, renal function, and nutritional status were assessed. Of the four patients with an intact jejunoileal bypass, two of the three who were biopsied had recurrent steatotic liver disease. The two patients whose jejunoileal bypass was reversed at the time of liver transplantation had lower alkaline phosphatase, lower creatinine, higher albumin, and higher cholesterol, and were more obese than their counterparts with intact bypasses. Patients undergoing liver transplantation for jejunoileal bypass-associated liver disease should, if possible, have their bypass reversed at the time of transplantation; otherwise, they must be followed closely and be biopsied routinely. Recurrent liver disease should prompt reversal of the jejunoileal bypass.