Late‐onset carbamoyl phosphate synthetase 1 deficiency in an adult cured by liver transplantation

Timothy R. Bates, Barry D. Lewis, John R. Burnett, Kenji So, Andrew Mitchell, Luc Delriviere, Gary P. Jeffrey – 11 August 2011 – Urea cycle disorders (UCDs) are rare causes of hyperammonemic encephalopathy in adults. Most UCDs present in childhood and, if unrecognized, are rapidly fatal. Affected individuals who survive to adulthood may remain undiagnosed because of clinicians' unawareness of the condition or atypical presentations. We describe the case of a 49‐year‐old man who initially presented with a stroke and developed hyperammonemic encephalopathy over a period of 8 months.

Optimizing cost‐effectiveness in perioperative care for liver transplantation: A model for low‐ to medium‐income countries

Karina Rando, Claus U. Niemann, Pilar Taura, John Klinck – 11 August 2011 – Although liver transplantation (LT) is a highly effective treatment, it has been considered too costly for publicly funded health systems in many countries with low to medium average incomes. However, with economic growth and improving results, some governments are reconsidering this position. Cost‐effectiveness data for LT are limited, especially in perioperative care, and the techniques and costs vary widely between centers without overt differences in outcomes.

Impact of calcineurin inhibitors with or without interferon on hepatitis C virus titers in a chimeric mouse model of hepatitis C virus infection

Norman M. Kneteman, Sonal Asthana, Jamie Lewis, Chelcey Dibben, Donna Douglas, Mahra Nourbakhsh, Lorne J. Tyrrell, Garry Lund – 11 August 2011 – Cyclosporine A (CSA) has potent effects against hepatitis C virus (HCV) in vitro, but its clinical efficacy after liver transplantation (LT) is uncertain. We evaluated the impact of CSA and tacrolimus (TAC) with or without concomitant interferon (IFN) therapy on serum HCV titers in a chimeric mouse model of HCV infection. Six groups of HCV‐infected mice received only the vehicle, IFN, CSA, CSA and IFN, TAC, or TAC and IFN for 4 weeks.

Waitlist survival of patients with primary sclerosing cholangitis in the model for end‐stage liver disease era

David Goldberg, Benjamin French, Arwin Thomasson, K. Rajender Reddy, Scott D. Halpern – 11 August 2011 – The ability of the Model for End‐Stage Liver Disease (MELD) score to capture the urgency of transplantation may not be generalizable to patients with primary sclerosing cholangitis (PSC) because these patients face unique risks of death or removal from the liver transplant waitlist due to disease‐specific complications (eg, repeated bouts of bacterial cholangitis and cholangiocarcinoma).

Priority of candidates with hepatocellular carcinoma awaiting liver transplantation can be reduced after successful bridge therapy

Alessandro Cucchetti, Matteo Cescon, Eleonora Bigonzi, Fabio Piscaglia, Rita Golfieri, Giorgio Ercolani, Maria Cristina Morelli, Matteo Ravaioli, Antonio Daniele Pinna – 11 August 2011 – The allocation rules for patients with hepatocellular carcinoma (HCC) who are awaiting liver transplantation (LT) are a difficult issue and are continually evolving. To reduce tumor progression or down‐stage advanced disease, most transplant centers have adopted the practice of treating HCC candidates with resection or locoregional therapies.

Impact of liver transplantation on the survival of patients treated for hepatorenal syndrome type 1*

Thomas D. Boyer, Arun J. Sanyal, Guadalupe Garcia‐Tsao, Frederick Regenstein, Lorenzo Rossaro, Beate Appenrodt, Veit Gülberg, Samuel Sigal, Alice S. Bexon, Peter Teuber, the Terlipressin Study Group – 11 August 2011 – The development of hepatorenal syndrome type 1 (HRS1) is associated with a poor prognosis. Liver transplantation improves this prognosis, but the degree of the improvement is unclear. Most patients receive vasoconstrictors such as terlipressin before transplantation, and this may affect the posttransplant outcomes.

Reply

Mariëlle Verweij, Tessa M. van Ginhoven, James R. Mitchell, Wim Sluiter, Sandra van den Engel, Henk P. Roest, Elham Torabi, Jan N. M. IJzermans, Jan H. J. Hoeijmakers, Ron W. F. de Bruin – 11 August 2011

Subscribe to