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We report 5 cases of acute liver failure related to herpes simplex (HSV) infection in 1 immunocompetent and 4 immunosuppressed patients. One patient was too ill for liver transplantation indication. Three patients, among the 4 listed, underwent liver transplantation. Three patients died 11 days to 1 year after transplantation and 2 patients died 2 to 3 days after admission. All presented with fever and none with skin lesions. The diagnosis of HSV-related hepatitis was made antemortem in only 2 patients on the basis of positive blood cultures and/or immunohistochemic findings. In the remaining patients, HSV diagnosis was made retrospectively on further histologic and virologic investigations. Primary HSV infection was certain or likely in all cases, including an HSV2 superinfection of an anti-HSV1-positive patient and two HSV superinfections of hepatitis B virus (HBV)-related chronic liver disease. In these latter patients, HSV diagnosis was totally unsuspected, despite fever. HSV superinfection has significantly contributed to liver dysfunction aggravation and death. In conclusion, the diagnosis of HSV hepatitis is difficult to establish in the absence of specific clinical signs. This may suggest the need for early administration of acyclovir in patients with suspected HSV hepatitis, without waiting for virologic confirmation. Diagnosis methods providing fast results (real-time polymerase chain reaction [PCR]) should be implemented.

A recent ELTR study clearly showed that liver graft preservation with HTK is an independent risk factor for graft loss after liver transplantation (1). Naturally this result is highly relevant to all the transplant teams that use this solution. However, as suggested by a recent letter to the Editor (2) it may be questioned as it comes from a registry and therefore subject to possible bias. However, not only were the results of the original analysis confirmed by multivariate analysis but additional analyses excluding German centers and living donor liver transplants, also conveyed the same message (3). This article is protected by copyright. All rights reserved.

Background. The yearly increasing survival rates testify to the success of transplantation, but questions remain relating to the quality of life (QOL) associated with long-term survival. Methods. A sample of 126 liver recipients (Liver-R), 229 kidney recipients (Kidney-R), and 113 heart recipients (Heart-R) with more than 10 years posttransplant follow-up were included in the study with a response rate of 86%. Respondents were matched with healthy subjects recruited from general population (GP). The three groups of recipients and GP subjects completed a French version of the questionnaire used by the National Institute of Diabetes and Digestive and Kidney Disease, Pittsburgh, PA, and were compared for each score, with adjustments for age and sex. Results. Personal function and measures of disease by the transplant recipients were significantly worse than in the GP (P

We assessed the impact of liver transplantation (LT) on the quality of life (QOL) of French recipients 1 year after surgery. A French version of the questionnaire used by the National Institute of Diabetes and Digestive and Kidney Disease—Pittsburg, USA (NIDDK), was validated by the back-translation method. Five QOL domains were evaluated: measures of disease, psychological distress, personal function, social function, and general health perception. Patients enrolled onto the waiting list completed the questionnaire before and 1 year after LT. Respondents were age- and gender-matched with healthy subjects recruited from the general population (GP). One year after LT, the analysis of data from 67 consecutive patients showed dramatic improvement in the five domains. Compared with baseline, patients noted fewer disease-related symptoms (P < .0001) and lower level of distress overall (P < .001). However, levels of distress caused by excess appetite (P < .01), trembling (P < .05), and headaches (P = .06) were more likely to increase than decrease. Twenty-five percent of patients prevented by their disease from going to work before LT were no longer so limited at 1-year follow-up. General health perception improved remarkably, with seven times as many recipients reporting improved health as reporting worse health. A correlation was found between the pretransplantation severity of cirrhosis and the social and role function after LT (P < .05). In summary, the French version of the NIDDK questionnaire seems to be reliable. The results of transplant recipients were generally close to those of the general population. Although it is not a true return to normal status, it approaches it. (Liver Transpl 2003;9:703-711.)

Background No model exists for liver transplantation to estimate the mortality risk in a given patient, and no standard by which to assess performance in different centres. We investigated the intrinsic mortality risk in the absence of known mortality risk factors, Methods We identified mortality risk factors and risk ratios quantified in data from the European Liver Transplant Registry (22 089 patients at 102 centres in 18 countries) registered from 1988 to 1997. To develop a model of the intrinsic risk and the risk ratios for specific factors, univariate and multivariate analyses were done separately for the overall population, for adults, and for children younger than 15 years, and the number of deaths were estimated, We validated the model by comparing mortality in patients without risk factors with the model-adjusted mortality in patients with risk factors. Findings Overall 5-year and 8-year actuarial survival was 66% (95% CI 65-66) and 61% (60-62). 65% of deaths occurred within 6 months, Retransplantation, transplantation for cancer, acute liver failure, fewer than 20 split-liver grafts per year, and a centre workload of fewer than 25 transplants per year were the main risk factors of 12 identified factors, 1-year and 5-year death rates among adults with no risk factors were similar to model estimates (15 [13-16] vs 14% [13-15], and 22 (20-24) vs 23% [21-24]). Corresponding data for paediatric transplants were 9% (7-12) compared with 11% (9-12) and 13% (10-17) compared with 14% (11-16). The reduction of mortality risk in high-volume centres was even greater in patients without risk factors (48 vs 23%, p

Keywords: clinical research/practice; liver transplantation/hepatology; liver transplantation: living donor; liver transplantation: split; liver transplantation: auxiliary; liver allograft function/dysfunction

BACKGROUND & AIMS: HBV-related chronic liver disease is one of the most common indications for liver transplantation (LT) in Europe. The ELTR database was used to evaluate outcomes and evolution over 20 years (01/1988 and 12/2010). METHODS: HBV transplanted patients were analysed according to indication for LT: decompensated cirrhosis (HBVdec) or hepatocellular carcinoma (HBV/HCC). These groups were compared with co-infected patients HBV/HDV (HBDV), HBV/HCV (HBCV), HBV/HDV/HCV (HBDCV); n = 16,664 and with HCV patients (n = 2452) according to LT indication. RESULTS: 5912 patients were transplanted for HBV (78% HBVdec, 22% HBV/HCC), with HBV/HCC patients who increased from 15.8% in 1988-1995 to 29.6% in 2006-2010 (p < 0.001). In HBVdec patients, 1, 3, 5, and 10 year patient and graft survival was 83%, 78%, 75%, 68%, and 80%, 74%, 71%, 64%, respectively, significantly better than HBV/HCC (84%, 73%, 68%, 61%, and 81%, 70%, 65%, 58% respectively; p = 0.001 and p = 0.026). In 2006-2010 patient and graft survival significantly improved compared to 1988-1995, both for HBVdec and HBV/HCC (each p < 0.001). A better patient and graft survival was seen in HBV/HCC patients with HBV-DNA(-) compared to HBV-DNA(+) at the time of LT (p < 0.001). Disease recurrence, as cause of death/graft loss, was significantly reduced in recent years compared to the past: currently

Donor criteria for liver grafts have been expanded because of organ shortage. Currently, no exact definitions for extended donor grafts have been established. The aim of this study was to analyze the impact of donor-specific risk factors, independent of recipient characteristics. In collaboration with Eurotransplant and European Liver Transplant Register, solely donor-specific parameters were correlated with 1-year survival following liver transplantation. Analyses of 4701 donors between 2000 and 2005 resulted in the development of a nomogram to estimate graft survival for available grafts. Predictions by nomogram were compared to those by Donor Risk Index (DRI). In the multivariate analysis, cold ischemic time (CIT), highest sodium, cause of donor death, γ-glutamyl transferase (γ-GT), and donor sex (female) were statistically significant factors for 3 months; CIT, γ-GT, and cause of donor death for 12-month survival. The median DRI of this study population was 1.45 (Q1: 1.17; Q3: 1.67). The agreement between the nomogram and DRI was weak (kappa = 0.23). Several donor-specific risk factors were identified for early survival after liver transplantation. The provided nomogram will support quick organ quality assessment. Nevertheless, this study showed the difficulties of determining an exact definition of extended criteria donors. © 2013 Steunstichting ESOT.

Background & Aims Liver transplantation for acute liver failure (ALF) still has a high early mortality. We evaluated changes during 20years, and identified risk factors for poor outcome. Methods Donor, graft, and recipient variables from the European Liver Transplant Registry database (January 1988–June 2009), were analysed. Aetiologies and time periods were compared. Three and 12-month survival models were generated from separate training data sets, which were validated. A sub-analysis was performed for recipient older than 50years. Results Four thousand nine hundred and three patients were evaluated. One, 5- and 10-year patient, and graft survival rates were 74%, 68%, 63%, and 63%, 57%, 50%, respectively. Survival was better in 2004–2009 compared to previous quinquennia ( p 0.001), despite donors >60years increased from 1.8% to 21%. A higher incidence of suicide or non-adherence occurred in paracetamol-related ALF ( p 0.001). Death or graft loss were independently associated with male recipients (adjusted OR 1.25), recipient >50years (1.26), incompatible ABO matching (1.93), donors >60years (1.21), and reduced size graft (1.54). For both 3- and 12-month models, incompatible ABO matching, non-viral aetiology, reduced size graft, and non-UW preservation fluid were associated with increased mortality/graft loss, whereas male recipients and age >50years were associated only at 12months. Both models had reasonable discriminative ability with good calibration at 3months. Recipients >50years, combined with donors >60years resulted in 57% mortality/graft loss within the first year. Conclusions Survival after liver transplantation has improved despite increases in donor/recipient age. Recipients >50years paired with donors >60years had a very high mortality/graft loss within the first year.

Domino transplantation using the liver (DLT) from patient with familial amyloid polyneuropathy (FAP) has been developed to alleviate the graft shortage. We herein studied 91 patients who ...

Background & Aims Most liver transplant centres have discontinued the practice of protocol liver biopsies (LB), mainly because of the perceived lack of therapeutic benefit. This study aimed to examine the usefulness of 20-year LBs. Methods Ten, 15, and 20-year protocol LBs from 147 patients surviving for >20years were reviewed. Twenty-year biopsy findings were correlated with clinical data. Results Twenty-year-biopsy patients (N=91) and 20-year-non-biopsy patients (N=56) were similar in terms of transplant data, adverse events, and liver function tests (LFTs). Twenty-year LBs revealed a 90% prevalence of abnormalities, among which viral chronic hepatitis (VCH) was the most common (46%). Between 15 and 20years, hepatic structural abnormalities were the only disorder to increase ( p =0.008). An individual progression of abnormalities occurred in 56% of patients. At 20years, the negative and positive predictive values (PV) of LFTs with respect to histological abnormalities were 95% and 18%, respectively; in VCH, Fibrotest and transient elastography displayed poor discriminative ability for fibrosis (80% and 81% discordance, respectively), but were satisfactory regarding significant fibrosis (negative PV of 77.7% and 80%, respectively). A decrease in immunosuppression was less frequent (14/91 vs . 20/56, p =0.008) while an increase was more common (15/91 vs . 2/56, p =0.017) in 20-year-biopsy patients than in non-biopsy patients. Antiviral therapy was administered in seven of the 20-year biopsy patients, but in none of the non-biopsy patients ( p =0.04). Conclusions Twenty-year LBs provided important histological information on graft function that was available to a limited degree from LFTs and non-invasive markers. They exerted an impact on immunosuppressive and antiviral therapies.

After liver transplantation (LT), hepatic veno-occlusive disease (VOD), which is also known as sinusoidal obstruction syndrome (SOS), has been reported initially in relation to azathioprine use and subsequently in relation to acute rejection (AR). Isolated veno-occlusive disease (iVOD)/SOS raises some questions about its significance and especially its treatment. From the post-LT biopsy samples of 1364 patients (2000-2008), 31 patients with index biopsy samples showing VOD/SOS (2.3%) were identified. After a review of the index biopsy samples and previous biopsy samples, those patients not exposed to azathioprine therapy were subdivided into 2 groups according to the absence or presence of AR. Fifteen of the 31 patients had no previous evidence of AR, whereas 16 experienced episodes of AR (before or concurrently with VOD). The 2 groups were similar in terms of demographic and clinical data and the range of histological centrilobular changes. AR episodes were characterized by an endothelial predilection. iVOD/SOS occurred later than acute rejection-related veno-occlusive disease (AR-VOD)/SOS (mean times of 65 and 4.4 months, respectively, P = 0.0098). There was a tendency for iVOD/SOS to progress less frequently to chronic rejection in comparison with AR-VOD/SOS (3/15 versus 9/15, P = 0.06). The histological resolution of iVOD/SOS was significantly more frequent in patients who benefited from increased immunosuppression in comparison with those who did not (5/7 versus 2/8, P = 0.05). When the groups were considered together, the same result was obtained (14/18 versus 4/12, P = 0.024). In conclusion, despite a constant overall prevalence of VOD/SOS, the proportion of iVOD/SOS has increased. The histological resolution of iVOD/SOS after increase in immunosuppression suggests an immune-mediated origin. Better optimization of immunosuppression may be a curative treatment.

Alcohol-related liver disease (ALD) is one of the most common indications for liver transplantation (LT). Long-term outcome after LT for ALD versus other etiologies is still under debate. The aim of this study was to compare outcome after LT of patients with ALD, viral (VIR), and cryptogenic cirrhosis. Donor, graft and recipient ELTR variables were analysed in transplants for alcoholic and nonalcoholic cirrhosis (1988-2005) and were correlated with patient survival. Causes of death and/or graft failure were compared between groups. Nine thousand eight hundred eighty ALD, 10,943 VIR, 1478 ALD+VIR and 2410 cryptogenic (CRYP) liver transplants were evaluated. One, 3, 5 and 10 years graft survival rates after LT in ALD patients were 84%, 78%, 73%, 58%, significantly higher than in VIR and CRYP (p=0.04, p=0.05). By multivariate analysis, ALD+VIR (RR 1.14) and viral alone (RR 1.06) were significant risk factors for mortality. De novo tumors, cardiovascular and social causes were causes of death/graft failure in higher percentage in ALD groups versus other etiologies. LT for ALD cirrhosis has a favorable outcome, however, hepatitis C virus co-infection seems to eliminate this advantage. Screening for de novo tumors and prevention of cardiovascular complications are essential to provide better long-term results.

WOS: 000276570300009, PubMed ID: 20373456, The principal aim of this study was to compare the probability of and potential risk factors for death and graft loss after primary adult and pediatric liver transplantation in patients undergoing transplantation for autoimmune hepatitis (AIH) to those in patients undergoing transplantation for primary biliary cirrhosis (PBC; used as the reference group) or alcoholic cirrhosis (used as an example of a nonautoimmune liver disease). The 5-year survival of patients undergoing transplantation for AIH (n = 827) was 0.73 [95% confidence interval (CI) = 0.67-0.77]. This was similar to that of patients undergoing transplantation for alcoholic cirrhosis (0.74, 95% CI = 0.72-0.76, n = 6424) but significantly worse than that of patients undergoing transplantation for PBC (0.83, 95% CI = 0.80-0.85, n = 1588). Fatal infectious complications occurred at an increased rate in patients with AIH (hazard ratio = 1.8, P = 0.002 with PBC as the reference). The outcome of pediatric AIH patients was similar to that of adult patients undergoing transplantation up to the age of 50 years. However, the survival of AIH patients undergoing transplantation beyond the age of 50 years (0.61 at 5 years, 95% CI = 0.51-0.70) was significantly reduced in comparison with the survival of young adult AIH patients (0.78 at 18-34 years, 95% CI = 0.70-0.86) and in comparison with the survival of patients of the same age group with PBC or alcoholic cirrhosis. In conclusion, age significantly affects patient survival after liver transplantation for AN. The increased risk of dying of infectious complications in the early postoperative period, especially above the age of 50 years, should be acknowledged in the management of AIH patients with advanced-stage liver disease who are listed for liver transplantation. It should be noted that not all risk factors relevant to patient and graft survival could be analyzed with the European Liver Transplant Registry database. Liver Transpl 16:461-469, 2010. (C) 2010 AASLD.

s / Digestive and Liver Disease 48S (2016) e1–e17 e7 36 Fondazione IRCCS Ospedale Maggiore Policlinico, Milano, Italy 37 Policlinico Giaccone, Palermo, Italy 38 Universita di Modena, Modena, Italy 39 AO San Gerardo, Monza, Italy Theaimof this studywas toevaluate theprevalenceand theclinical profile of mixed crioglobulinemia (MC) in consecutive patients enrolled in PITER. A specific questionnaire regarding the presence of crioglobulinemia and related symptoms was applied. Of 7496 patients enrolled, the MC was never tested in 6019 (80%). The remaining 1477 HCV (+) patients were classified as follows: in 22% as symptomatic MC (MCS-HCV: mean age 64±11 years), in 29% as asymptomaticMC (MC-HCV:meanage61±12years), and in49%as HCV infectedpatientswithoutMC(meanage60±13years). Among the 328 patients, declared to be “symptomatic”, detailed data that confirmed MCS were available for 252 (33% of patients with MC). MC symptoms included purpura, weakness, and arthralgia (MC triad), as well as peripheral neuropathy, siccasyndrome, myalgias, cutaneous ulcers and renal involvement. Nocturnal sweet, fever and pruritis was also rarely reported. In patientswithMCS the liver fibrosis stage, was F0-F1 in 40%; F2 in 12%; F3 in 13% and F4 in 35% of patients, with no significant differences among the different groups. Previous IFN-based therapy was reported in 54% of MCSHCV, in 45% of MC-HCV and in 50% of HCV patients. 162 (65%) of MCS-HCV patients have been treated or are on DAA treatment. Preliminary results are available showing high rate of virological and clinical response with rare (1%: 3 patients) treatment interruption due to adverse events/intolerance. In Conclusion: This nation-wide, in progress study showed that, in spite of the clinical and therapeutic importance of HCV related MC, the presence of this condition is not considered in the large majority of cases. Among patients with MC, a symptomatic form was observed in more than 30% of cases. Treated patients will be prospectively followed up to better characterize the long term outcome of this important consequence of chronic HCV infection. http://dx.doi.org/10.1016/j.dld.2015.12.027

AIM To evaluate the relationship between the state of transplanted liver graft and the recipient quality of life (QOL) of histologically proven lesions in a 10-year post liver transplantation (LT) cohort of patients. METHODS Seventy-two recipients with a functional first graft at 10 years post-LT underwent liver biopsy and completed a QOL questionnaire. Logistic regression analysis was used to explore associations between histological, clinical and QOL criteria. RESULTS Ten years after LT, fibrosis was detected in 53% of patients, and affected the general health perception, while ductopenia, present in 36%, affected the well-being (P = 0.05). Hepatic steatosis (HS) was present in 33% of patients and was associated with the worst QOL score on multiple domains. When compared to patients without HS, patients with HS had significantly higher incidence of fibrosis (P = 0.03), hepatitis C virus (HCV) infection (P = 0.007), and more patients had retired from their job (P = 0.03). Recurrent or de novo HCV-associated fibrosis and patient retirement as objective variables, and abdominal pain or discomfort and joint aches or pains as subjective variables, emerged as independent determinants of HS. CONCLUSION Long-term liver graft lesions, mainly HS presumably as a surrogate marker of HCV infection, may have a substantial impact on QOL 10 years after LT.

This paper describes the design of a low power 1.8GHz fully integrated phase locked loop based frequency synthesizer. The synthesizer includes a conventional tri-state phase frequency detector, a fully differential charge pump, a 2nd-order on-chip loop filter, a voltage-controlled ring oscillator and an integer-N multi-modulus divider. The synthesizer is implemented in a 0.13μm CMOS process and consumes less than 3.5mW of power at a supply voltage of 1.2V. The entire synthesizer is designed using current mode logic and occupies an active area of 0.34mm2.

A histologic pattern comprising centrilobular cholestasis and portal changes including edema, predominantly neutrophil polymorph infiltration, cholangiolar proliferation, and cholangiolitis is well known to correspond to biliary obstruction. This pattern, referred as biliary tract pathology (BTP) in this text, remains unclear in terms of its clinical significance. We aimed to assess the incidence, timing, and diagnostic accuracy of BTP after liver transplantation. All 248 liver biopsies and clinical records, from 94 patients, including 30 living donor, 17 split, 15 domino, and 32 cadaveric full-size primary liver transplantation, were reviewed. BTP was diagnosed in 21% of biopsies from 31% of patients at a median of 28 days after transplantation (range, 5-763 days). When radiologic imaging of the biliary tree was taken as the gold standard, biopsy was found to have a sensitivity of 87% (95% confidence interval, 73%-100%) and a specificity of 87% (95% confidence interval, 80%-95%) for the diagnosis of biliary complications. An underlying clinical condition was found in 86% of cases, which included biliary complications (69%), arterial thrombosis (3%), sepsis (10%), and recurrent disease (3%). In 14% of cases, BTP remained unexplained. In conclusion, BTP after liver transplantation has clinical significance in most cases, with a particular emphasis for true biliary complications. This pattern must incite radiographic verification of the biliary tract.

Background and aim: Autoimmune hepatitis (AIH) has been reported to recur after orthotopic liver transplantation (OLT) in 10–35% of patients in small series with a short follow up. The aim of the present study was to examine the clinical and histological outcome more than 10 years after OLT for AIH. Patients and methods: Seventeen women with a mean age of 30 (12) years at the time of OLT, selected from among 44 patients transplanted for AIH, were followed for more than 10 years. The criteria for definite AIH, as established by the International Autoimmune Hepatitis Group, were met in every case. Liver biopsies were performed 1, 2, 5, and 10 years after OLT, and when indicated by abnormal liver function tests. Specimens were examined for evidence of recurrent AIH, namely interface hepatitis, lobular activity, portal lymphoplasmocytic infiltration, and fibrosis. Other signs of recurrence included hypertransaminasaemia, serum autoantibodies, and the response to steroid reintroduction or significant steroid dose increments. Results: AIH recurred in 7 (41%) of 17 patients. In four patients histological abnormalities were detected by means of protocol biopsies 1–5 years before the onset of biochemical abnormalities. Two patients developed severe recurrences after 10 and 15 years, respectively, and required treatment with steroids and tacrolimus. In the other three patients histological recurrence was detected 0.6–3 years post-OLT, concomitantly with biochemical abnormalities. Conclusions: AIH recurred in 41% of patients followed for more than 10 years after OLT. As histological signs preceded biochemical abnormalities in four patients (23.5%), regular liver biopsy is warranted after OLT. Detection of isolated histological signs may call for closer follow up and/or a change in immunosuppressive therapy.

Background. The number of registries is increasing, but few of them perform reliability audits by comparing the data contained in the database with data contained in hospital charts. Methods. The European Liver Transplant Registry (ELTR) cocoordinating committee appointed an independent team to check the reliability of data contained in ELTR. Centers were selected at random. Ten percent of each center's files were selected at random, and 25 items per file were checked during the site visits. The rates of completeness and inconsistencies and the agreement between ELTR and charts were established. We also assessed the correlation between the quality of data and the visited centers' activity. Results. Seven hundred thirty-four files from 21 centers have been audited between June 1998 and June 2001. The rate of ELTR completeness was 95%, and the rate of consistency between charts and ELTR was 98%. The agreement between the ELTR and charts review was very good for all conditions (kappa value less than or equal to0.81). However, comparisons of rates between items indicated that specific items, mostly cause of death or graft failure and patient outcome, should be targeted for improvement. No significant correlation was found between the quality of data and the experience of visited centers. The mean (min-max) and median cost per audited file were EUR 60 (8-150) and EUR 44, respectively. Conclusion. The results of audit visits indicate that ELTR data are reliable, and the scientific results of ELTR can be considered credible and representative of liver transplantation in Europe. The method could serve as a model for auditing a registry.

Introduction Les neuropathies amyloides Familiales (NAF) sont des neuropathies de transmission autosomique dominante survenant chez l’adulte et liees a une mutation amyloidogene du gene de la transthyretine (TTR). Il s’agit de polyneuropathies sensitivomotrices progressives invalidantes avec dysautonomie letales en 10 ans en moyenne. La Transplantation Hepatique (TH), qui permet de supprimer la principale source de TTR mutee est actuellement le principal traitement propose.Objectifs : Rapporter l’evolution a long terme de la neuropathie et la survie apres TH et en determiner les facteurs pronostiques independants. Methodes Etude prospective monocentrique avec evaluation periodique clinique (dont scores PND de marche et vegetatif) et electrophysiologique. Entre 1993 et 2010, 200 patients avec une NAF- TTR symptomatique ont eu une TH, 138 (69 %) ayant un variant Met30TTR, 62 un variant non Met30 (15 varietes). L’âge moyen etait de 45,5 ans avec un debut tardif (≥ 50ans) chez 37,5 % des patients. Nous avons utilise la methode de Kaplan Meier avec le test de Log- rank et le modele de Cox multivarie pour etudier la survie et ses facteurs pronostiques. Resultats La majorite des patients (63 %) a conserve la meme autonomie de marche mais 21,3 % ont eu recours a une aide a la marche (PND3) apres TH dont un quart sont devenus grabataires. Les facteurs de risque d’atteinte du score PND 3 etaient un amaigrissement > 20 %, l’âge tardif de debut, une duree de maladie avant TH ≥ 3 ans. 76/200 patients sont decedes dont 37 % la premiere annee, le plus souvent de causes cardiaques (39 %), d’infection (24 %), ou de progression de la neuropathie (7 %). La mediane de survie post TH est de 11,4 ans ; les facteurs de risque de deces sont une hypotension orthostatique ; une incontinence urinaire, l’âge tardif de debut (≥ 50 ans), un variant non Met30. La mediane de survie globale depuis le debut de maladie est de 14,7 ans, egale a 21,3 ans pour les Met30TTR jeunes ( vs 8,9 ans pour les non Met30TTR âgees, ≥ 50 ans). Pour 20 % des patients, l’implantation de Pace Maker s’est revelee necessaire au decours de la TH. Conclusions L’evolution apres TH semble meilleure dans les formes precoces de NAF- TTR Met30 jeunes en terme de survie et d’arret de progression de la neuropathie ; elle est moins favorable dans les formes tardives majoritaires actuellement ; ceci souligne l’interet des nouvelles biotherapies telles que SiRNA, Oligonucleotides AntiSens capables de supprimer le variant TTR ainsi que le sauvage, egalement fortement pathogene.

To identify the outcomes and risks of split-liver transplantation (SLT) for two adult recipients to determine the feasibility of more widespread use of this procedure to increase the graft pool for adults.The shortage of cadaver liver grafts for adults is increasing. Using livers from donors defined as optimal, the authors have been developing techniques for SLT for two adult recipients at their center.From July 1993 to December 1999, 34 adults have undergone SLT with grafts from optimal donors prepared by ex situ split (n = 30) or in situ split (n = 4), and 88 adults received optimal whole-liver grafts that were not split. Four split-grafts were transplanted at other centers. The outcomes of transplantation with right and left split-liver grafts were compared with those of whole-liver transplants. The main end points were patient and graft survival at 1 and 2 years and the incidence and types of complications.For whole-liver, right and left split-liver grafts, respectively, patient survival rates were 88%, 74%, and 88% at 1 year and 85%, 74%, and 64% at 2 years. Graft survival rates were 88%, 74%, and 75% at 1 year and 85%, 74%, and 43% at 2 years. Patient survival was adversely affected by graft steatosis and recipients inpatient status before transplantation. Graft survival was adversely affected by steatosis and a graft-to-recipient body weight ratio of less than 1%. Primary nonfunction occurred in three left split-liver grafts. The rates of arterial (6%) and biliary (22%) complications were similar to published data from conventional transplantation for an adult and a child. SLT for two adults increased the number of recipients by 62% compared with whole-liver transplantation and was logistically possible in 16 of the 104 (15%) optimal cadaver donors.Split-liver transplantation for two adults is technically feasible. Outcomes and complication rates can be improved by rigid selection criteria for donors and recipients, particularly for the smaller left graft, and possibly also by in situ splitting in cadaver donors. Wider use will require changes in the procedures for graft allocation and coordination between centers experienced in the techniques.

3529 Background: Preoperative chemotherapy for CRLM can produce histologic changes in non tumoral liver. Methods: A retrospective analysis was performed in data from 92 patients (pts) who had liver...

During the past 3 decades, more than 2,250 liver transplants were performed at Paul Brousse Hospital. Overall patient survival was 82% at one year, 71% at 5 years and 64% at 10 years. Our group has developed a variety of approaches to liver transplantation, including: 1. Anti HBs immunoglobulin prophylaxis for the prevention of HBV recurrence. Combination prophylaxis with lamivudine and anti HBs immunoglobulins reduced the rate of HBV re-infection to 20%. 2. Transplantation of HIV-HCV and HIV-HBV infected patients. These transplants are feasible and we achieved 2- year survival rates of 70% and 90%, respectively. The main problem was HCV recurrence which was more severe in HIV co-infected patients. 3. Transplantation for hepatocellular carcinoma on a cirrhotic liver with a single tumor5 cm or3 tumors3 cm. 4. Transplantation for familial amyloidotic polyneuropathy (FAP). The 5- and 10-year survival rates were 76% and 72%, respectively. More than 100 livers obtained after hepatectomy from FAP patients were transplanted as "domino" living donor livers to patients with unresectable liver cancers with a 5-year survival rate of 64%. In some domino recipients, symptoms of FAP disease occurred more rapidly than expected and this could be an indication for a second transplantation of a non FAP-liver. 5. Split-liver transplantation for pediatric patients. This has increased the number of transplantable livers for children by 28%. 6. Split-liver transplantation for 2 adults. The grafts were prepared by ex-vivo or in-situ splitting and the overall 5-year survival rate was 56%. 7. Adult -to-adult living-related liver transplantation. There has been no mortality nor late complications in donors and the overall 5-year survival rate among recipients was 73%. 8. Liver retransplantation with good results in the elective situation. Retransplantation should be used with discretion in the emergency setting.