Long-term Results Of Liver Transplantation Research Articles

Primary sclerosing cholangitis-Diagnosis and treatment 2024

The etiology of primary sclerosing cholangitis (PSC) remains unclear, which explains in part the lack of acausal treatment. The differential diagnostic distinction from the even rarer immunoglobulin 4 (IgG4)-associated cholangitis (IAC) is becoming increasingly more successful. Advances in the understanding of different clinical courses, improvements in noninvasive diagnostics through modern magnetic resonance imaging (MRI) and the introduction of liver elastography have led to the development of improved prognostic models. The evidence for recommendations on medicinal (e.g., ursodeoxycholic acid) or endoscopic treatment (e.g., balloon dilatation and/or stent insertion) for PSC is still low. In contrast, the long-term results of liver transplantation in PSC patients are constantly improving. Due to the lack of highly sensitive and specific screening methods the early recognition of cholangiocellular carcinoma (CCC) as the most important complication is rarely successful. The continuous improvement of endoscopic retrograde cholangiopancreatography (ERCP) and direct cholangioscopy in combination with molecular biological and fluorescence in situ hybridization (FISH) analyses of bile duct tissue samples are promising for refined diagnostics. Due to the significantly increased risk of colorectal cancer, an annual colonoscopy is recommended in the presence of inflammatory bowel disease. Improvement of the early diagnostics of PSC and successful testing of new treatment strategies raise hope for acontinuous improvement in the medical support of these complex patients.

Risk for Recurrence in Long-Term Follow-Up of Children after Liver Transplantation for Hepatoblastoma or Hepatocellular Carcinoma.

The aim of this study was to assess the long-term results of liver transplantation (LT) in pediatric patients with unresectable hepatoblastoma (HB) or hepatocellular carcinoma (HCC) with special reference to the risk of tumor recurrence. We retrospectively analyzed data from 46 HB and 26 HCC patients who underwent LT between 1990 and 2022. In HCC patients, we compared outcomes depending on donor type. We evaluated the impact of a number of risk factors on recurrence-free survival after LT. Estimated patient survival after 5, 10, and 15 years was 82%, 73%, and 73% in the HB group and 79%, 75%, and 75% in the HCC group, respectively (p = 0.76). In the HCC group, living donor LT (LDLT) and deceased donor LT (DDLT) provided similar patient survival (p = 0.09). Estimated recurrence-free survival in patients who had three or fewer risk factors was significantly better than in patients with more than three risk factors (p = 0.0001). Adequate patient selection is necessary when considering LT for primary liver tumors in children. The presence of more than three risk factors is associated with a very high risk of recurrence and indicates poor prognosis, whereas extrahepatic disease may be considered a contraindication for transplantation.

Liver transplantation for metastases from neuroendocrine tumors: a literature review and clinical observation

Neuroendocrine tumors are a rare and heterogeneous group of neoplasms with different malignant potential. They often metastasize to the liver and require active combined therapy. For unresectable neuroendocrine tumors with liver metastases, transplantation has become a potential definitive treatment due to the slow growth and the clinical course of the metastatic process. We conducted a review of relevant literature, the criteria for including and excluding patients considered for liver transplantation as well as alternative methods of treatment of neuroendocrine tumors. The paper presents a clinical observation of a staged combined therapy of a patient with a neuroendocrine tumor of the small intestine and bilobar unresectable liver metastases. The study demonstrates satisfactory long-term results of liver transplantation.

Long-term results of liver transplantation for hepatocellular cancer

Aim. To study the long-term results of liver transplantation for hepatocellular carcinoma in accordance with various criteria for selecting patients for surgery. Materials and methods . The results of 71 liver transplantations for hepatocellular carcinoma have been studied. Long- term survival and disease-free survival have been studied in accordance with various criteria for selecting patients for liver transplantation. Results . The five-year survival rate was 84.3%, the median survival rate was 47.4 months. Higher overall survival and disease-free survival rates were found in patients after living donor liver transplantation compared to patients after cadaveric liver transplantation. The five-year survival rate for patients within the Milan criteria compared to those beyond the Milan criteria were 92.4% vs 74.1%. The five-year survival rate for patients within the UCSF criteria compared to patients beyond the UCSF criteria were 93.3% vs 68.3%. The five-year survival rate for patients within the Up-to-seven criteria compared to patients beyond the Up-to-seven criteria were 88.4% vs 71.0%. The five-year survival rate for patients within the Hangzhou criteria compared to patients beyond the Hangzhou criteria were 84.4% vs 72.9%. Hepatocellular carcinomas recurrent were in 8.4% of patients in the long-term period of liver transplantation. The five-year disease-free survival rate among all patients was 87.7%. The median disease-free survival rate was 43.2 months. The five-year disease-free survival rate for patients within criteria were from 85.4% (Hangzhou University) to 93.3% (UCSF). The five-year disease-free survival rate for patients beyond criteria were from 68.3% (UCSF) to 74.1% (Milan criteria). A statistically significant relapse rate was observed in patients with a Guerrini coefficient >2.3, regardless of the selection criteria in all study groups of patients. Conclusion . The optimal prognostic criteria for liver transplantation for patients with hepatocellular carcinoma are the Milan criteria. Some expansion of the Milan criteria show comparable results. In this regard, it can be assumed that the optimal criteria have not been found at present, which requires new ways to search for expansion of the Milan criteria, taking into account the level of AFP, tumor differentiation, etc.

Post-hoc analysis of a randomized controlled trial on the impact of pre-transplant use of probiotics on outcomes after liver transplantation

Perioperative use of probiotics serves as efficient prophylaxis against postoperative infections after liver transplantation, yet data on long-term effects of pre-transplant probiotic intake is lacking.The aim of this study was to assess the effects of pre-transplant probiotic administration on long-term results of liver transplantation. This was secondary analysis of a randomized trial. Patients were randomized to receive either 4-strain probiotic or placebo before liver transplantation. Five year graft survival was set as the primary end-point. Secondary end-points comprised serum bilirubin and C-reactive protein (CRP) concentration, international normalized ratio (INR), serum transaminases and gamma-glutamyl transferase (GGT) activity. Study group comprised 44 patients, of whom 21 received probiotics and 23 received placebo with 5-year graft survival of 81.0% and 87.0%, respectively (p = 0.591). Patients in the probiotic arm exhibited lower INR (p = 0.001) and CRP (p = 0.030) over the first 6 post-transplant months. In the absence of hepatitis B or C virus infection, pre-transplant administration of probiotics also reduced aspartate transaminase activity (p = 0.032). In the intervention arm, patients receiving probiotics for under and over 30 days had 5-year graft survival rates of 100% and 66.7%, respectively (p = 0.061). Duration of probiotic intake > 30 days was additionally associated with increased INR (p = 0.031), GGT (p = 0.032) and a tendency towards increased bilirubin (p = 0.074) over first 6 post-transplant months. Pre-transplant administration of probiotics has mild positive influence on 6-month allograft function, yet should not exceed 30 days due to potential negative effects on long-term outcomes. (ClinicalTrials.gov Identifier: NCT01735591).

Outcomes of liver transplantation in hepatocellular carcinoma: a single-center experience

Aim. To evaluate the long-term results of liver transplantation for hepatocellular carcinoma. To perform an analysis of risk factors for tumor recurrence.Materials and methods. A retrospective analysis of 63 deceased donor liver transplantations, which were performed from January 2010 to February 2020. Patients were divided into three groups according to the Milan and California criteria – within the Milan criteria, outside the Milan criteria, but within the California criteria, outside the California criteria.Results. There were 33 recipients within the Milan criteria, 5 within the California criteria, and 25 outside the California criteria. The 5-years recurrence rate was 9.1%, 40% and 52%, respectively (p = 0.002), five-year survival rate of – 93.2%, 66.7% and 54.7% (p = 0.041). Factors associated with an increased risk of recurrence: five and more tumor nodes (OR = 3.675; 95% CI (1.35–9.97); p = 0.011), macroscopic vascular invasion (OR = 5.97; 95% CI (2.06–17.31); p = 0.001) and inconsistency with California criteria (OR = 4.00; 95% CI (1.49–10.74); p = 0.006). The factors associated with a lower recurrence rate: one or two tumor nodes (p = 0.014 and 0.042, respectively), a high grade of histological differentiation (RR = 0.24; 95% CI (0.06–0.93); p = 0.039), compliance with Milanese criteria (RR = 0.18; 95% CI (0.06–0.58); p = 0.004).Conclusion. Liver transplantation can be performed in a number of patients beyond the Milan and California criteria. The morphological and biological characteristics of the tumor and a multidisciplinary differentiated approach to treatment have an important role in the success of treatment.

Prognostic Factors for Liver Transplantation in Unresectable Hepatoblastoma.

Hepatoblastoma is the most frequent hepatic tumor in children, and its initial presentation will affect treatment and prognosis. The aim of this study is to evaluate long-term results of liver transplantation in children with unresectable hepatoblastoma. This is a retrospective review of patients with hepatoblastoma who underwent liver transplantation, analyzing risk factors, tumor presentation, treatment, and long-term survival to identify prognostic factors. Thirty-one patients underwent liver transplantation in the context of unresectable hepatoblastoma, mainly males (67%) and with risk factors such as prematurity (12.9%), maternal smoking (6.5%), and familial adenomatous polyposis (3.2%). Most frequent presentation was multifocal PRETEXT III (51.6%) and PRETEXT IV (45.2%), with metastasis at diagnosis in 12.9% and vascular involvement in 54.8%.Twenty-one patients received a living-donor (67.7%) and 10 a cadaveric graft (32.2%), at 31.7 months of age (5-125). Most transplants were primary, and only two were performed as rescue therapy after an attempt of surgical resection of the tumor.Overall survival 1 and 5 years after transplantation were 93.3% ± 4.6% and 86.4% ± 6.3%, respectively. We could not find any statistically significant differences between risk factors, tumor presentation, type of graft, or type of transplant. Liver transplantation has increased hepatoblastoma survival in unresectable tumors. Probably due to these good results, we have not been able to find significant prognostic factors in this cohort.

Long-term results of liver transplantation for over 60 years old patients with hepatitis B virus-related end-stage liver disease

Hepatitis B virus (HBV)-related end-stage liver disease is the leading indication for liver transplantation in China, but long-term results of liver transplantation in patients aged over 60 years are not clear. The present study was to reveal the natural history of liver recipients with hepatitis B older than 60 years. The recipients who had received liver transplantation between December 2003 and December 2005 were divided into two groups: those equal or older than 60 years (older group, n=60) and those younger than 60 years (younger group, n=305). Risk factors for poor long-term outcome in patients aged over 60 years were also analyzed. Except for age and preexisting chronic disease (P<0.05), no significant differences were observed in perioperative characteristics between the two groups. There was also no significant difference in HBV and hepatocellular carcinoma recurrence (P>0.05). The actuarial 1-, 3-, 5- and 8-year survival rates were 81.6%, 71.6%, 66.7% and 63.3% respectively for the older group vs 84.9%, 77.7%, 70.8% and 65.6% for the younger group (P>0.05). Multivariate analyses showed that pre-liver transplant renal insufficiency was a risk factor for poor outcome in the older group (odds ratio=3.615, P=0.014). Liver transplantation is safe and feasible for patients with HBV-related end-stage liver disease aged over 60 years. Older patients with renal insufficiency should undergo transplantation earlier than younger patients.

Natural history, treatment and prevention of hepatitis C recurrence after liver transplantation: past, present and future.

Hepatitis C virus (HCV)-related liver disease, including cirrhosis and hepatocellular carcinoma is the main indication for liver transplantation (LT) worldwide. Post-transplant HCV re-infection is almost universal and results in accelerated progression from acute hepatitis to chronic hepatitis, and liver cirrhosis. Comprehension and treatment of recurrent HCV infection after LT have been major issues for all transplant hepatologists and transplant surgeons for the last decades. The aim of this paper is to review the evolution of our knowledge on the natural history of HCV recurrence after LT, including risk factors for disease progression, and antiviral therapy. We will focus our attention on possible ways (present and future) to improve the final long-term results of LT for HCV-related liver disease.

Long-Term Results of Liver Transplantation

Liver transplantation (LT) is an established therapy associated with a dramatic improvement in patients life expectancy. With improved early-term management, current 10-year patient survival rates in many indications exceed 70%. Life-long immunosuppressive therapy may, however, be accompanied by considerable longterm toxicity: most importantly, renal dysfunction, cardiovascular disease, and cancer, which, in addition to recurrence of the primary liver disease, emerge as key contributors to late mortality. Chronic kidney disease cumulatively affects up to 28% of patients by ten years after LT. Various factors can contribute to renal impairment, but perioperative acute kidney injury, calcineurin inhibitor toxicity, hypertension, and diabetes are considered most important. LT patients demonstrate 3-fold risk for cardiovascular events, which seems to result mostly from an excess of traditional risk factors, mainly hypertension and diabetes. The cumulative cancer incidence reaches 16-42% by 20 years after LT, and cancer rates are 2- to 4-fold higher among LT patients than among matched controls. Highest rates are for nonmelanoma skin cancer (3- to 70-fold) and lymphoma (8- to 29-fold). The liver graft usually displays uncomplicated function in the long term. Most common causes for chronic graft dysfunction include disease recurrence and biliary problems. LT generally restores patients quality of life to a level comparable with that of the general population, with only minor deficits in some areas. Thus, long-term survival after LT is impressive, and despite these long-term complications, patients quality of life remains comparable with that of the general population.

Liver Transplantation Is Feasible in Super-Obese Patients: A Case Report

Short- and long-term results of liver transplantation in morbidly obese patients may be impaired compared with the general transplant population. As a consequence, severe obesity has been considered to be a relative contraindication to liver transplantation in many centers. Surgically, liver transplantation in severe obesity may be challenging. Moreover, obesity may lead to an increased rate of early and late medical complications. Herein we have reported successful liver transplantation in a super-obese patient (body mass index, 55.1 kg/m 2) who had developed terminal acute-on-chronic liver disease. In the first 6 months of follow-up, the patient underwent a severe diet that led to a significant weight loss reduction to a body mass index of 39 kg/m 2. This report of successful liver transplantation in a super-obese patient suggests that severe obesity should not be considered to be an absolute contraindication to liver transplantation.

Long-term results of liver transplantation for Wilson's disease

Wilson's disease is a hereditary defect in hepatic copper metabolism, causing hepatic, neurological and/or psychiatric manifestations. For patients with severe disease, liver transplantation is the treatment of choice. The aim of this study was to report the long-term outcome of patients who underwent liver transplantation for Wilson's disease. Thirteen patients with Wilson's disease, transplanted in Lyon France between January 1987 and May 2006, were including in this study: eight women and five men, aged eight to 53 years (median 20 years, seven children and six adults). The diagnosis of Wilson's disease was established before liver transplantation. The indication for liver transplantation was chronic (69%) or fulminant liver failure (31%). The median follow-up after liver transplantation was 10 years with 100% patient survival. Copper metabolism returned to normal in all patients. None of the patients with exclusive liver disease required chelation treatment after liver transplantation and none developed neurological symptoms of Wilson's disease. Liver transplantation totally reverses the abnormalities of copper metabolism and subsequent hepatic failure, but the course of neurological symptoms remains unpredictable. Long-term patient survival can be excellent without occurrence of neurological complications.

Eighteen years of liver transplantation experience in patients with advanced Budd-Chiari syndrome

The long-term results of liver transplantation for Budd-Chiari syndrome (BCS) and timely indication for the procedure are still under debate. Innovations in interventional therapy and better understanding of underlying diseases have improved therapy strategies. The aim of this study was the analysis of patient and disease characteristics, outcome, and specific complications. Between September 1988 and December 2006 we performed 42 orthotopic liver transplantations (OLTs) in 39 patients with BCS. A total of 29 (74%) women and 10 men (26%) had a median age of 35 years; the median follow-up period was 96 months. Etiologically, 27 patients had a preoperative diagnosis of hematologic disease, including myeloproliferative disorders (MPD), followed by factor V Leiden mutation and antiphospholipid syndrome. The actuarial 5-year and 10-year survival rates were 89.4% and 83.5%, respectively, compared to 80.7% and 71.4%, respectively, for other indications (n = 1742). Retransplantation was necessary in 3 patients (7.1%) with portal vein thrombosis or recurrent BCS. Although the number of bleeding events was similar, incidence of vascular complications was significantly higher in patients with BCS. Thrombosis of the portal vein was observed in 4.8% versus 0.8% of the patients, whereas liver veins were affected in 7.1% versus 0.2%. Our data shows that severe acute or chronic forms of BCS with liver failure can be successfully treated by OLT. Despite higher rates of vascular complications, patient and graft survival are similar or even better compared to other indication groups. In conclusion, patients with reversible hepatic damage should be treated by combined strategies, including medical therapy and surgical or interventional shunting.

Our experience in liver transplantation in patients over 65 yr of age

The aim of this study was to analyze short- and long-term results of liver transplantation (LT) in patients over 65 yr. Between 1996 and 2004, 386 patients underwent 415 LT at our center. The main indication for LT was post-necrotic cirrhosis in 59%, followed by hepatocellular carcinoma (HCC) over cirrhosis in 33%. Half of the patients (53%) were hepatitis C virus (HCV) +. Overall, 72 patients were >65 yr of age. Actuarial survival, causes of mortality and postoperative complications were compared between groups: patients under and over 65 yr. Risk factors for poor outcome in patients over 65 yr were also analyzed. The older group had more patients at Child A stage, more HCC as an indication for LT and more HCV (+) patients, p < 0.05. No differences were observed in donor and surgery characteristics, except for lower multi-transfusion and higher incidence of grafts with steatosis in the older group (p < 0.05). Actuarial survival at one, three, five and 10 yr was 82%, 75%, 72%, and 70% for the <65 yr group vs. 77%, 66%, 55%, and 55% for the >65 yr group (p = 0.03). Main causes of mortality in patients >65 yr were recurrence of underlying disease and medical causes. In the older age group, fewer infections (p = ns) and rejections (p = 0.017) occurred in the postoperative period. Risk factor for poor outcome in the group of patients over 65 yr in multivariate analyses was pre-LT renal insufficiency (odds ratio 3.5, p = 0.002, 95% confidence interval 1.58-7.82). Results in patients >65 yr are comparable to those <65 yr if older LT candidates are carefully selected. Overimmunosuppression should be avoided in older candidates, as its effects could worsen the pre-existing diseases common in elderly patients.

Long-Term Results of Liver Transplantation for Hepatocellular Carcinoma: An Update of the University of Padova Experience

Background Hepatocellular carcinoma (HCC) competes with benign liver disease as indication for liver transplantation (OLT). The aim of this study was to determine long-term results of OLT for HCC. Methods We retrospectively analyzed the prognostic role of HCC diagnosis at pathological exam in adult OLT. In the HCC group, we evaluated the prognostic role of the time of diagnosis (incidental versus nonincidental) and of pathological tumor TNM staging. The primary endpoint was 1-, 3-, and 10-year patient survivals. Results From 1991 to 2006, among 550 adults who underwent first OLT, HCC was found in 120 patients at pathological exam. In 26 cases (22%), the diagnosis of HCC was incidental. There were 59 cases (49%) of pathological T1 to T2 tumor (one nodule < 5 cm, or two to three nodules < 3 cm, without metastases and/or vascular invasion), and 61 cases (51%) of pathologic T3-T4a tumor. HCC diagnosis did not show a significant prognostic impact by Cox survival analysis. After a median follow-up of 31 months, 1-, 5-, and 10-year survivals were 91%, 81%, and 73% in the HCC group, and 84%, 76%, and 67% in the non-HCC group. Time of HCC diagnosis (incidental versus nonincidental) and pathological TNM staging (T1 to T2 vs T3 to T4a) did not result significant survival predictors upon Cox analysis. Conclusion In our experience, the long-term results of OLT for HCC overlapped those of OLT for benign disease, although 51% of tumors were T3 to T4a at pathological exam.

150] CADAVERIC WHOLE LIVER TRANSPLANTATION FOR NON-ACETAMINOPHEN FULMINANT HEPATIC FAILURE: A 20-YEAR EXPERIENCE

AIM: To investigate the long-term results of liver transplantation (LT) for non-acetaminophen fulminant hepatic failure (FHF). METHODS: Over a 20-year period, 29 FHF patients underwent cadaveric whole LT. Most frequent causes of FHF were hepatitis B virus and drug-related (not acetaminophen) liver failure. All surviving patients were regularly controlled at the out-patient clinic and none was lost to follow-up. Mean follow-up was 101 mo. RESULTS: One month, one-, fi ve- and ten-year patient survival was 79%, 72%, 68% and 68%, respectively. One month, one-, fi ve- and ten-year graft survival was 69%, 65%, 51% and 38%, respectively. Six patients needed early (< 2 mo) retransplantation, four for primary non-function, one for early acute refractory rejection because of ABO blood group incompatibility, and one for a malignant tumor found in the donor. Two patients with hepatitis B FHF developed cerebral lesions peri-transplantion: one developed irreversible and extensive brain damage leading to death, and one suffered from deep defi cits leading to continuous medical care in a specialized institution.

Cadaveric liver transplantation for non-acetaminophen fulminant hepatic failure: A 20-year experience

To investigate the long-term results of liver transplantation (LT) for non-acetaminophen fulminant hepatic failure (FHF). Over a 20-year period, 29 FHF patients underwent cadaveric whole LT. Most frequent causes of FHF were hepatitis B virus and drug-related (not acetaminophen) liver failure. All surviving patients were regularly controlled at the out-patient clinic and none was lost to follow-up. Mean follow-up was 101 mo. One month, one-, five- and ten-year patient survival was 79%, 72%, 68% and 68%, respectively. One month, one-, five- and ten-year graft survival was 69%, 65%, 51% and 38%, respectively. Six patients needed early (< 2 mo) retransplantation, four for primary non-function, one for early acute refractory rejection because of ABO blood group incompatibility, and one for a malignant tumor found in the donor. Two patients with hepatitis B FHF developed cerebral lesions peri-transplantion: One developed irreversible and extensive brain damage leading to death, and one suffered from deep deficits leading to continuous medical care in a specialized institution. Long-term outcome of patients transplanted for non-acetaminophen FHF may be excellent. As the quality of life of these patients is also particularly good, LT for FHF is clearly justified, despite lower graft survival compared with LT for other liver diseases.

Comparison of Clinical and Pathological Staging and Long-Term Results of Liver Transplantation for Hepatocellular Carcinoma in a Single Transplant Center

Liver transplantation (OLT) is a treatment for hepatocellular carcinoma (HCC) superimposed on cirrhosis provided that the disease meets defined criteria. The aim of the study was to evaluate our experience with respect to clinical and pathological staging and long-term results. From 1996 to 2005, 50 patients underwent OLT for HCC including 43 men (86%) and seven women (14%) of median age 57 years (range 37 to 67). All patients fulfilled the Milan criteria. The HCC diagnosis was based on preoperative imaging and alpha-fetoprotein levels; no tumor biopsy was performed. Upon histological examination of the resected specimens, we discovered 6 (12%) incidentalomas and 8 (16%) cases of no HCC. Finally we had 42 “true” HCC. Twenty-six patients (52%) have been downstaged and 10 (20%) upstaged by preoperative imaging; 15% were pT1, 45% were pT2, 27% pT3, and 13% pT4a. Twenty-six percent of cases exceeded the Milan criteria. One patient (pT4a) with microvascular invasion died of pulmonary metastases at 14 months after transplantation. No HCC recurrences within the liver have been encountered at a median follow-up of 20 months (range 0 to 80 months). Overall the estimated 1-, 3-, and 5-year survival rates were 83%, 77%, and 72%, respectively. One-, 3-, and 5-year estimated survival rates were 87%, 75%, and 75% for pT1, and pT2, and 75%, 67%, and 67% for pT3 and pT4a, respectively ( P = .99). Based on our experience OLT for HCC has long-term results comparable to those without HCC despite the presence of a significant number of cases exceeding the Milan criteria upon pathological staging.

Liver Transplantation for alpha-One-Antitrypsin Deficiency

Purpose: We report the outcomes of liver transplantation (LT) for alpha-1-antitrypsin deficiency from a single transplant program. Methods: A retrospective review of the Organ Transplant Database at the University of Nebraska Medical Center was performed to identify all patients (pts) transplanted for AAT deficiency between 1990–2005. Pts were diagnosed with AAT deficiency if 3 of the following 4 criteria were present: depressed serum levels of AA; ZZ phenotype; liver biopsy and/or explant histopathology confirming the presence of diastase resistant AAT globules. The following outcomes were recorded: age; sex; prevalence of hepatoma; hepatic artery thrombosis (HAT); biliary complications; episodes of rejection and infection; post transplant lymphoproliferative disease (PTLD); graft and patient survival. Results: 35 pts were transplanted for AAT deficiency with a median age of 8 mths. The majority of AAT pts were male (77%) under the age of 18 yrs (76%) and received a cadaver liver (74%). Incidental hepatoma was present in 2 explants and recurred in 1 pt. HAT developed in 4 pts (8.3%) and biliary complications (leaks, strictures and /or necrosis) occurred in 9 pts (26%). Acute rejection occurred in 21 pts (60%) and chronic rejection occurred in 7 pts (20%). PTLD occurred in 7 pts (20%). Eight pts died, with sepsis accounting for cause of death in 6 pts (75%). Infection was seen in 77% of pts. Mean follow up was 7.5 years. Estimates of overall survival were based on the Kaplan Meier Method. Cumulative incidence methods were used to estimate the incidence of graft failure (GF). Outcome information was available for 32 of 35 patients. 1 year Overall Survival (OAS) was 77% (95% CI 57–88%). 3 year OAS is 72% (95% CI 52–85%). There was no difference in OAS when the patients were categorized by the number of complications (p = 0.7). Pts < 18 years tend to have improved estimates of OAS compared to pts > than 18 yrs (p = 0.087). Estimated cumulative incidence of GF at 1 year is 6% (95% CI 0–17%) and at 7 years 13% (95% CI 0- 34%). Conclusions: In this predominantly male, pediatric cohort of LT pts with AAT, long term survival is approximately 72%. Pediatric pts tend to have a better OAS than adult pts. The number of post transplant complications does not appear to be a predictor of outcome. Sepsis was the most common cause of death. Long term results of LT for AAT deficiency are otherwise encouraging with outcomes comparable to pts undergoing LT for other indications

Short- and long-term results of liver transplantation in infants aged less than 6 months.

Despite major surgical and medical advances, it is still a challenge to perform transplantation in small infants. This study, focusing on short- and long-term outcomes, summarizes our 10-year experience with liver transplantation (LTx) in infants aged less than 6 months. We analyzed 43 patients aged 6 months or less (range: 12-184 days, median: 136 days) whose median weight at the time of LTx was 5.8 kg (range: 2.8-8.0 kg). The reasons for LTx were biliary atresia (n=27; 62.8%), neonatal hepatitis (n=6; 14%), neonatal cholestasis (n=4; 9.3%), and miscellaneous (n=6; 14%). The patients were followed up for a median time of 3 years and evaluated with respect to graft function, physical, and neurodevelopmental outcome. The patient survival was 90.7% after 1 year and 87.2% after 2 years. The graft survival was 86% after 1 year and 82.1% after 2 years. Twelve patients (27.9%) experienced 15 surgical complications requiring intervention, two of whom demonstrated vascular thrombosis (4.7%). Acute early rejection occurred in 15 patients (34.9%), and chronic rejection occurred in 3 patients (7%); 83.3% of the patients had normal liver function test results at the time of evaluation. Complications such as posttransplant lymphoproliferative disease (4.7%) and persistent arterial hypertension (4.7%) were rarely seen. The physical and neurodevelopmental outcomes were good. LTx in infants aged less than 6 months provides excellent short- and long-term results. Low weight or young age of infants awaiting LTx should not be exclusion criteria for LTx.