Exceptional manifestation of Madelung's disease after liver transplantation.

Unlike simple obesity, Madelung's disease (MD) is a rare disease characterized by symmetric accumulation of massive adipose tissue on the neck, the superior part of the trunk and limbs, leading to a pathognomonic cosmetic deformity. Here, we report an extremely rare case of MD associated with bilateral gynecomastia in a 61-year-old man, with a history of recent liver transplantation for alcoholic liver disease (ALD).

Machine Perfusion: Cold versus Warm, versus Neither. Update on Clinical Trials.

Machine perfusion (MP) preservation is potentially one of the most significant improvements in the field of liver transplantation in the last 20 years, and it has been considered a promising strategy for improved preservation and ex situ evaluation of extended criteria donor (ECD) organs. However, MP preservation adds significant cost and logistical considerations to liver transplantation. MP protocols are mainly classified according to the perfusion temperature with hypothermic machine perfusion (HMP) and normothermic machine perfusion (NMP) being the two categories most studied so far. After extensive preclinical work, MP entered the clinical setting, and there are now several studies that demonstrated feasibility and safety. However, because of the limited quality of clinical trials, there is no compelling evidence of superiority in preservation quality, and liver MP is still considered experimental in most countries. MP preservation is moving to a more mature phase, where ongoing and future studies will bring new evidence in order to confirm their superiority in terms of clinical outcomes, organ utilization, and cost-effectiveness. Here, we present an overview of all preclinical MP studies using discarded human livers and liver MP clinical trials, and discuss their results. We describe the different perfusion protocols, pitfalls in MP study design, and provide future perspectives. Recent trials in liver MP have revealed unique challenges beyond those seen in most clinical studies. Randomized trials, correct trial design, and interpretation of data are essential to generate the data necessary to prove if MP will be the new gold standard method of liver preservation.

Primum Non Nocere: Organ Donation After Electrocution and Transplantation of Electricity-Damaged Livers: Report of 2 Cases.

Liver transplantation remains the treatment of choice for patients with end-stage liver disease. However, allograft availability continues to be a problem, and extending the criteria for organ acceptance is key. Deceased donors after electrical accidents, as well as electricity-traumatized allografts, are not common but should be considered suitable. This study describes 2 cases of heart-beating organ donors with electrical injury to the liver. In 1 case, the electric shock was the cause of death; in the second case, the injury was caused by defibrillation at organ procurement. Both allografts had sustained sizeable electrical injury, and both resulted in excellent early posttransplant outcomes. These cases demonstrate that electrocution is not a contraindication to donation and that electricity-traumatized allografts may remain transplantable after careful assessment. Education of all staff in the management of such donors can optimize utility of such allografts.

Molecular Characterization of Acute Cellular Rejection Occurring During Intentional Immunosuppression Withdrawal in Liver Transplantation.

Acute cellular rejection occurs frequently during the first few weeks following liver transplantation. During this period, its molecular phenotype is confounded by peri- and postoperative proinflammatory events. To unambiguously define the molecular profile associated with rejection, we collected sequential biological specimens from 55 patients at least 3 years after liver transplantation who developed rejection during trials of intentional immunosuppression withdrawal. We analyzed liver tissue and blood samples obtained before initiation of drug withdrawal and at rejection, alongside blood samples collected during the weaning process. Gene expression profiling was conducted using whole-genome microarrays and real-time polymerase chain reaction. Rejection resulted in distinct blood and liver tissue transcriptional changes in patients who were either positive or negative for hepatitis C virus (HCV). Gene expression changes were mostly independent from pharmacological immunosuppression, and their magnitude correlated with severity of histological damage. Differential expression of a subset of genes overlapped across all conditions. These were used to define a blood predictive model that accurately identified rejection in HCV-negative, but not HCV-positive, patients. Changes were detectable 1-2 mo before rejection was diagnosed. Our results provide insight into the molecular processes underlying acute cellular rejection in liver transplantation and help clarify the potential utility and limitations of transcriptional biomarkers in this setting.

Impact of steroid-avoidance immunosuppression on long-term outcome after liver transplantation for HCV cirrhosis: the need for well documented long-term follow-up.

AIM: study impact of steroid avoidance on HCV recurrence after transplantation. METHODS AND MATERIAL: 35 HCV pats, being part of prospective, randomized, double-blind, placebo-controlled study comparing Tacrolimus (TAC)-Placebo (PLAC) (n = 14) to TAC-short-term (2 mo) low-dose steroid (STER) (n = 21), had 5 years follow-up. Primary endpoint was 1 and 5 years survival; secondary (composite) endpoint comprised HCV related cirrhosis, re-transplantation (re-LT) and death. RESULTS: 1 and 5-years survival were 93% and 75% in TAC-PLAC group; 91% and 66% in TAC-STER group (p 0.38). Two (14.3%) TAC-PLAC pats died due to HCV cirrhosis at 54 and 72 mo; 7 (33%) TAC-STER pats died due to cholestatic hepatitis at 5.8 and 9 mo, to cirrhosis at 18, 22, 34, 73 and 79 mo (p 0.20). Composite endpoint at 5 years didn't show advantage in favor of TAC-PLAC patients (5/14 [35.7%] vs. 9/21 [42.8%] pts, p.0.69). Early biopsies seemed more favorable in TAC-PLAC pats; at 5 years results were identical for both groups. Only 1 (7.1%) TAC-PLAC and 2 (9.5%) TAC-STER pats needed rejection treatment. CONCLUSION: immunosuppression using steroid avoidance or short-term use had similar outcomes. Well documented long-term follow-up, including biopsies, is necessary in order to make conclusions in relation to impact of steroid use on outcome of HCV liver recipients.