Perform Liver Transplantation Research Articles

A Feasibility Study on 3-D Imaging of Intrahepatic Bile Ducts in Patients with Biliary Atresia Using Airy Beam Excited Two-Photon Microscopy.

The purpose of this study was to utilize a two-photon microscope excitation Airy beam to achieve three-dimensional imaging of intrahepatic bile ducts in BDL mice and patients with biliary atresia (BA). Ten male Balb/c mice aged 6-8weeks underwent extrahepatic bile duct ligation (BDL), and 10 underwent sham operation as control. After the operation, the mice resulted in symptoms such as jaundice, darkened urine, and weight loss. Taken liver tissues from BDL and control mice and trimmed to 5*5*3 mm3 after 10days. Sixteen patients with BA were included in this study. Liver transplantation was performed in 12 cases of them; liver hilar and liver margin tissues were taken during the operation. Kasai portoenterostomy (KPE) was performed in 4 cases, and liver margin tissues were taken. Intraoperative liver tissue samples were trimmed to a size of 5*5*5 mm3. The specimens were subjected to tissue fixation, antigen retrieval, antibody incubation, and subsequent tissue hyalinization following the principles of immunofluorescence staining. Subsequently, light-sheet fluorescence microscopy (LSFM) was followed, and intrahepatic bile ducts of the specimen were imaged utilizing Airy beam which was excited with high imaging depth attenuation-compensated two-photon. Deconvolution was applied to image processing to construct a three-dimensional model of intrahepatic bile ducts. Three-dimensional imaging of liver tissue was conducted in both BDL mice and BA patients, and the distribution of intrahepatic bile ducts was visualized. BDL mice exhibited notable widening of intrahepatic bile ducts, accompanied by bile duct hyperplasia. There was no obvious hyperplasia of intrahepatic bile duct in the control group. Significant small bile duct hyperplasia was seen on imaging of the intrahepatic bile ducts in patients with BA. The intrahepatic bile duct was disorganized and hyperplasia especially in patients who performed liver transplantation. The technique of Airy beam three-dimensional reconstruction can effectively image the intrahepatic bile ducts in Balb/c mice and BA patients in three dimensions. This approach contributes to a better understanding of the distribution of intrahepatic bile ducts in BA patients. Moreover, it facilitates the exploration of models that more accurately simulate BA disease by elucidating the distribution of intrahepatic bile ducts in animal models. Understanding the distribution characteristics of intrahepatic bile duct will facilitate the formulation of hilar bile duct microstructure classification, which can guide the operation and evaluate the prognosis better.

Outcomes of Liver Transplantation with Incidental Intrahepatic Cholangiocarcinoma-Own Experience and a Systematic Review.

Background: Cholangiocarcinoma, the second most common primary liver cancer, is still a contraindication for performing liver transplantation in most patients. Despite various trials being performed in large clinical centers, the results are still not satisfactory. The aim of this study was to present cases from our own cohort and perform a systematic review of the results of liver transplantation in patients with incidental intrahepatic cholangiocarcinoma. Materials and methods: We retrospectively reviewed the records of all patients who underwent liver transplantation and identified two patients with incidental intrahepatic cholangiocarcinoma via histopathological examination of the explanted liver. The results of radiological and biochemical screening performed during liver transplantation, standardized histopathological examination and follow-up data are presented. Additionally, a systematic review of PubMed and Cochrane Reviews based on the PRISMA protocol was performed, yielding 413 similar cases. Results: We present two cases of incidental intrahepatic cholangiocarcinoma found after liver transplantation. The patients were managed according to a standard protocol with no consecutive modification of immunosuppression or chemotherapy. There was no recurrence or mortality. In this systematic review, the mean reported number of lesions ranged between 1 and 2 per patient. A total of 42 recurrences were reported. The percentage of recurrences ranged between 28.6% and 80%. Conclusions: Despite not being a frequent finding, follow-up and further treatment of patients with incidental iCCA should be reported and analyzed. Extra carefulness in screening is advised in patients who are already diagnosed with oncological disease of the liver. In long-term follow-up, recurrence of the disease is rather probable.

Use of HBsAg-positive donors in liver transplantation: An ILTS-EASL-AASLD multisociety survey.

The gap between organ supply and demand in liver transplantation remains large in most parts of the world. One strategy to increase the donor pool is to use grafts infected with HCV, HBV, and/or HIV viruses. We aimed to explore the current use of HBsAg-positive liver grafts worldwide. A prospective cross-sectional web-based survey was designed, with a total of 28 queries, assessing national and local regulations, center experience, and center-specific experience related to the topic, and sent to all members of International Liver Transplantation Society, European Association for the Study of the Liver, and American Association for the Study of the Liver, and promoted on social media. A total of 135 liver transplant centers answered the survey: 38% from WHO European Regions, 39% from American regions, and 9.7% from South-East Asian regions. Most of the participating centers (67.3%) had been performing liver transplantation for over 15 years, with a mean of 66.5 liver transplants per year, and 54% also performed living-donor liver transplants. HBV-related disease was the indication for liver transplantation in an average of 15% of all liver transplantation cases. Regarding national and/or regional regulations, 40% of the centers reported that the use of HBsAg-positive donors was permitted, and an additional 20% could use them under special circumstances. Thirty-two centers (31%) had previously used HBsAg-positive donors. Among these centers, 62.5% conducted living-donor liver transplants and showed an increased inclination toward the use of HBsAg-positive grafts in centers with elevated waitlist mortality. HBsAg-positive donors are underutilized worldwide. The use of HBsAg-positive liver grafts could help to increase the donor pool, particularly in highly endemic areas.

Variation in DCD Liver Transplant Protocols Among Transplant Centers in the United States.

Variation in donation after circulatory death (DCD) organ recovery and liver transplant practices exist among transplant centers. This study aimed to evaluate these practices among centers in the United States. Scientific Registry of Transplant Recipients data were accessed to identify centers that performed liver transplantation in 2021 and 2022. Surveys were sent to transplant centers that consistently performed ≥5 DCD liver transplants per year. DCD liver transplants were performed by 95 centers (65.1%) of the 146 liver transplant centers in the United States. Survey results were recorded from 42 centers that consistently performed ≥5 DCD liver transplants per year, with a 59.5% response rate. Withdrawal-to-asystole and agonal time were used to define donor warm ischemia time (WIT) in 16% and 84% centers, respectively. Fifty-six percent of the centers did not use oxygen saturation to define donor WIT. Systolic blood pressure cutoffs used to define agonal time varied between 50 and 80 mm Hg, donor age cutoffs ranged between 55 and 75 y, and cold ischemia times varied between 4 and 10 h. Seventy-six percent of centers used normothermic machine perfusion for DCD liver transplantation. This study highlights the wide variation in use, recovery, and definition of donor WIT. Using national data to rigorously define best practices will encourage greater utilization of this important donor resource.

Hybrid Bioactive Hydrogel Promotes Liver Regeneration through the Activation of Kupffer Cells and ECM Remodeling After Partial Hepatectomy.

Partial hepatectomy is an essential surgical technique used to treat advanced liver diseases such as liver tumors, as well as for performing liver transplants from living donors. However, postoperative complications such as bleeding, abdominal adhesions, wound infections, and inadequate liver regeneration pose significant challenges and increase morbidity and mortality rates. A self-repairing mixed hydrogel (O5H2/Cu2+/SCCK), containing stem cell derived cytokine (SCCK) derived from human umbilical cord mesenchymal stem cells (HUMSCs) treated with the traditional Chinese remedy Tanshinone IIA (TSA), is developed. This SCCK, in conjunction with O5H2, demonstrates remarkable effects on Kupffer cell activation and extracellular matrix (ECM) remodeling. This leads to the secretion of critical growth factors promoting enhanced proliferation of hepatocytes and endothelial cells, thereby facilitating liver regeneration and repair after partial hepatectomy. Furthermore, the hydrogel, featuring macrophage-regulating properties, effectively mitigates inflammation and oxidative stress damage in the incision area, creating an optimal environment for postoperative liver regeneration. The injectability and strong adhesion of the hydrogel enables rapid hemostasis at the incision site, while its physical barrier function prevents postoperative abdominal adhesions. Furthermore, the hydrogel's incorporation of Cu2+ provides comprehensive antibacterial effects, protecting against a wide range of bacteria types and reducing the chances of infections after surgery.

Recipient With Influenza A Infection: Contraindication to Transplant? A Case Report

Active infections in recipients are considered an absolute contraindication to liver transplantation due to the risk of severe disease caused by the required immunosuppression. Even though viral infections, such as Influenza A, have been treated in liver transplant patients both in the immediate and late postoperative period with favorable outcomes, no cases of prediagnosed Influenza A have been described in which the decision to proceed with the indication had to be made. In certain viral infections, like Influenza A, the paucisymptomatic state and the availability of appropriate treatment could open the door to performing liver transplants with positive results.

Outcomes of Patients with Acute Hepatotoxicity Caused by Mushroom-Induced Poisoning.

This study aimed to investigate the characteristics of severe hepatitis (SH), acute liver injury (ALI), and acute liver failure (ALF) in patients with mushroom-induced hepatotoxicity. Data of patients between 2010 and 2019 were retrospectively reviewed. Twenty-four patients with mushroom-induced hepatotoxicity were included and divided into 3 groups: SH, ALI, and ALF. SH was defined as transaminase level ≥10 times ULN, international normalized ratio (INR) ≤1.5, and the absence of hepatic encephalopathy (HE). ALI was defined as INR > 1.5, presumed acute illness onset, and the absence of HE. ALF was diagnosed based on the presence of HE of any degree, with INR > 1.5, presumed acute illness onset, and the absence of cirrhosis. The mean age of the patients was 51.6 years; 13 (54.2%) were female. At admission, 18 patients (75%) had SH, 5 (21%) had ALI, and 1 (4.1%) had ALF. During follow-up, 6 of the 18 SH (33%) patients progressed to ALI and 2 of the 5 ALI (40%) patients progressed to ALF. No progression to ALI or ALF was observed in the 8 SH cases with a baseline MELD score of <15. One patient with grade 4 HE died (4.1%). None underwent liver transplantation. The survival was 100% in the ALI and SH groups. A MELD score of <15 at admission may be used as a predictor of no progression to ALI or ALF in patients with SH. However, since 40% of ALI cases may progress to ALF, these cases should be followed up in a tertiary care center that is equipped to perform liver transplantation and advanced therapies.

Perceptions of surgical difficulty in liver transplantation: A European survey and development of the Pitié-Salpêtrière classification

BackgroundSignificant variations exist regarding the definition of difficult liver transplantation. The study goals were to investigate how liver transplant surgeons evaluate the surgical difficulty of liver transplantation and to use the identified factors to classify liver transplantation difficulty. MethodsA Web-based online European survey was presented to liver transplant surgeons. The survey was divided into 3 parts: (1) participant demographics and practices; (2) various situations based on recipient, liver disease, tumor treatment, and technical factors; and (3) 8 real-life clinical vignettes with different levels of complexity. In part 3 of the survey, respondents were asked whether they would perform liver transplantation but were not aware that these patients eventually underwent liver transplantation. ResultsA total of 143 invites were sent out, and 97 (67.8%) participants completed the survey. Most participants considered previous spontaneous bacterial peritonitis, previous supra-mesocolic surgery, hypertrophy of segment I, and obesity to be recipient factors for high-difficulty liver transplantation. Most participants considered liver transplantation to be challenging in patients with Budd–Chiari syndrome, Kasai surgery, polycystic liver disease, diffuse portal vein thrombosis, and a history of open hepatectomy. The proportion of participants indicating that liver transplantation was warranted varied across the 8 cases, from 69% to 100%. Our classification of the surgical difficulty of liver transplantation employed these recipient-related, surgical history–related, and liver disease–related variables and 3 difficulty groups were identified: low, intermediate, and high difficulty groups. ConclusionThis survey provides an overview of the surgical difficulty of various situations in liver transplantation that could be useful for further benchmark and textbook outcome studies.

Poor Access to Liver Transplantation and Survival of Children With Acute Liver Failure, Acute-on-chronic Liver Failure or Chronic Liver Disease.

We describe the survival of children with acute liver failure (ALF), chronic liver disease (CLD), or acute-on-chronic liver failure (ACLF) with poor access to liver transplantation (LT). A retrospective cohort study of 42 patients <18 years of age was conducted in the Hospital Civil de Guadalajara "Dr. Juan I. Menchaca". The median age was 76 months; 57.1% were female, 40.5% presented with ALF, 35.7% with CLD, and 23.8% with ACLF. Also, 38.1% (16/42) presented liver disease of unknown etiology. Death occurred in 45.2%; 14.3% were transferred to another hospital, and none received LT. Mortality in ALF, CLD, and ACLF was 76%, 0%, and 60%, respectively. In the survival analysis, within the first 20 months after diagnosis, the mortality rate was greater than 50% with ALF. The importance of having referral programs that perform liver transplantation is highlighted by the poor prognosis of the patients, despite conservative treatment.

Bio-Artificial Liver Support System: A Prospective Future Therapy

Whether acute or chronic, liver failure is a state of liver dysfunction that can progress to multiorgan failure. Mortality in liver failure patients is approximately 80–90% and is caused by detoxification failure, which triggers other immediate complications, such as encephalopathy, coagulopathy, jaundice, cholestasis, and acute kidney failure. The ideal treatment for liver failure is liver transplantation, but the long waiting period for the right donor match causes unavoidable deaths in most patients. Therefore, new therapies, such as tissue engineering, hepatocyte transplantation, and stem cells, are now being studied to anticipate the patient’s condition while waiting for liver transplantation. This literature review investigated the effectiveness of some bio-artificial liver support systems using review methods systematically from international publication sites, including PubMed, using keywords, such as bio-artificial liver, acute and chronic liver failure, extracorporeal liver support system (ECLS), MARS, single-pass albumin dialysis (SPAD). Artificial and bioartificial liver systems can show specific detoxification abilities and pathophysiological improvements in liver failure patients but cannot reach the ideal criteria for actual liver function. The liver support system must provide the metabolic and synthetic function as in the actual liver while reducing the pathophysiological changes in liver failure. Aspects of safety, cost efficiency, and practicality are also considered. Identifying the technology to produce high-quality hepatocytes on a big scale is essential as a medium to replace failing liver cells. An increase in detoxification capacity and therapeutic effectiveness must also focus on patient survival and the ability to perform liver transplantation.

Where Are We Now with Liver Transplantation in Neuroendocrine Neoplasms? The Place of Liver Transplantation for Grades 1 and 2 Well-Differentiated Unresectable Liver Metastatic Neuroendocrine Tumours

This review outlines the role of liver transplantation in selected patients with unresectable neuroendocrine tumour liver metastases. It discusses the international consensus on eligibility criteria and outlines the efforts taking place in the UK and Ireland to develop effective national liver transplant programmes for neuroendocrine tumour patients. In the early history of liver transplantation, indications included cancer metastases to the liver as well as primaries of liver origin. Often, liver transplantation was a salvage procedure. The early results were disappointing, including in patients with neuroendocrine tumours. These data discouraged the widespread adoption of liver transplantation for neuroendocrine tumour liver metastases (NET LM). A few centres persisted in performing liver transplantation for patients with NET LM and in determining parameters predictive of good outcomes. Their work has provided evidence for benefit of liver transplantation in a selected group of patients with NET LM. Liver transplantation for NET LM is now accepted as a valid indication by many professional bodies, including the European Neuroendocrine Tumour Society (ENETS) and the United Network for Organ Sharing (UNOS). It is nevertheless rarely utilised. The UK and the Republic of Ireland are commencing a pilot programme of liver transplantation in selected patients. This programme will help develop the expertise and infrastructure to make liver transplantation for NET LM a routine procedure.

Liver transplant training in United States: A two-year survey of transplant surgery

Background: The current requirement for fellows to receive the certificate of completion is to participate and log 45 liver transplant cases performed as a “principal role”. Our primary outcome assessed the comfort level of the fellows in performing liver transplants and the postoperative complications. A secondary analysis was performed to assess predictors of lack of preparedness among 2nd-year fellows in performing liver transplants

417.5: Pediatric Liver Transplantation: Twenty-Five-Year Experience At a Single Center

Introduction: Pediatric liver transplantation (Tx) is a worldwide accepted therapy for those disorders that generate severe and irreversible acute and chronic liver diseases. We aim to report our 25-year experience, performing liver transplantation in children at a single center. Material and methods: Retrospective analysis of patients <18 years old who underwent primary Tx or re-transplant (reTx) of the liver between January 1995 and December 2021 at a single center. Demographic data, time on the waiting list, indication and type of Tx, length of hospital stay, ischemia times, complications, overall graft and patient survivals were analyzed. Survival analysis was also performed comparing 2 different groups, G1: 1995-2008 and G2: 2009-2022. Statistical analysis was performed with SPSS version 25.0. Results: A total of 243 Tx were done, 210 (86%) were primary Tx, 29 were re-Tx and 4 had a 3rd Tx, and 122 (50%) were female. The median age was 33 months (R=1-208), median weight was 13 kg (R=4-74 and z score weight for age -1.07 ± 1.5) and mean height of 100±35 cm (z score height for age -1,7 ± 1,8). Indications for Tx were: 88 (42%) biliary atresia, 33 (16%) cryptogenic acute liver failure, 24 (11%) fulminant viral hepatitis (HAV), 15 (7%) autoimmune hepatitis, 14 (7%) Alagille`s syndrome, 17% others. Re-Tx. were performed due to: chronic ductopenic rejection in 12 cases, arterial thrombosis in 7, primary graft dysfunction in 7, biliary complications in 4 and others in 3. The mean time on the waiting list for cadaveric Tx was 81 ± 191 days, mean post Tx hospitalization was 28±24 days (12±13 ICU and 15±19 general ward). One hundred fourteen (47%) Tx were performed with living donor; 129 (53%) with cadaveric grafts, of them 61(47%) with partial graft. The mean cold and warm ischemia time were 250±245 minutes and 44±42 minutes respectively: 402±127* and 45±17 minutes for cadaveric donors and 85±67* and 43±12 for living donors of cold and warm ischemia time (* p=0.001). The most frequent surgical complications were: 49 (20%) biliary (30 leaks and 19 strictures), 14(6%) hepatic artery thrombosis (11 early and 3 late), 10 (4%) portal vein (8 thrombosis and 2 stenosis), the remaining 10% were other complications (postoperative bleeding, acute ventral hernia, surgical wound infection, peritonitis); 14 (6%) cases of PTLD. Figure 1 shows patient and graft survival at 1, 5 and 10 years for those who received primary Tx and re-Tx. Figure 2 shows patient’s survival divided in primary Tx and re-Tx. Conclusions: The most frequent cause of primary Tx is biliary atresia, while for re-Tx is chronic rejection. The most frequent early complication was bile leak, and the most frequent late complication was chronic rejection. Long-term survivals exceed the results from other regional and international centers.

Liver transplantation from active COVID-19 donors: Is it ethically justifiable?

The debate on the opportunity to use organs from donors testing positive for Severe Acute Respiratory Syndrome Coronavirus 2 (SARS‐CoV‐2) in recipients with naïve resolved or active COVID‐19 is ongoing. We aim to present the ethical analyses underlying the decision to perform liver transplantation (LT) in selected patients with resolved or active COVID‐19 in Italy. We used Jonsen, Siegler, and Winslade's Four‐Boxes casuistic method, addressing the four topics considered as constitutive of the essential structure of single clinical cases for their ethical analysis (medical indications, patient preferences, quality of life, and contextual features) to enable decision‐making on a case‐by‐case basis. Based on these topics, we elucidate the meaning and balance among the principles of biomedical ethics. Clinical ethics judgment based on the relation between the risk of acquiring SARS‐CoV‐2 along with its potentially negative effects and the expected benefits of transplant lead to consider LT as clinically appropriate. Shared decision‐making allows the integration of clinical options with the patient's subjective preferences and considerations, enabling a valid informed consent specifically tailored to the patients’ individual circumstances. The inclusion of carefully selected SARS‐CoV‐2 positive donors represents an opportunity to offer lifesaving LT to patients who might otherwise have limited opportunities to receive one. COVID‐19 positive donor livers are fairly allocated among equals, and respect for fundamental rights of the individual and the broader community in a context of healthcare rationing is guaranteed.The ethical analysis of the decision to perform LT in selected patients shows that the decision is ethically justifiable.

Liver-First Strategy for Combined Lung and Liver Transplantation: 15-Year Single-Center Experience

<h3>Purpose</h3> Combined lung and liver transplantation is an uncommon procedure and poses several management challenges. At our institution, since 2005, we have been performing liver transplantation before lung transplantation (liver first strategy), because this strategy might improve the liver coagulopathy thus reducing the risk of bleeding, and spare the successively transplanted lung allograft from the liver reperfusion fluid. Aim of this study was to present our 15-year experience with the liver first strategy for combined lung-liver transplantation. <h3>Methods</h3> Records of lung-transplanted patients between 01/2005 and 10/2021 at our institution were retrospectively reviewed. Outcomes were compared between patients undergoing combined lung-liver vs. patients undergoing lung-only transplantation. Median (IQR) follow-up was 53 (22-93) months. <h3>Results</h3> During the study period, among the 1822 lung-transplanted patients, 17 (0.9%) patients required combined lung-liver transplantation (male sex, n=15; median age, 30 years; lung transplant indication: cystic fibrosis, n=15; lung fibrosis, n=1; porto-pulmonary hypertension, n=1), the remaining 1805 (99.1%) patients undergoing lung-only transplantation. While median lung cold ischemic time (min.) was longer (first lung, 610 vs. 401, p<0.01; second lung, 692 vs. 510, p<0.01) and the intraoperative need for packed red blood cells greater (10 vs. 2 units, p<0.01) in the combined group, only one patient in this group showed primary graft dysfunction (PGD) grade 3 at 72 hours after transplantation (5.9% vs. 5.4%, p=0.61). Prevalence of rethoracotomy for bleeding (18% vs. 10%, p=0.41), temporary dialysis (24% vs. 11%, p=0.12), anti-HLA donor-specific antibodies (DSA, 12% vs. 19%, p=0.35), and in-hospital mortality (12% vs. 7%, p=0.32) did not differ in the lung-liver vs. lung-only transplantation group. Median and hospital stay time (33 vs. 23 days, p<0.01) was longer in the lung-liver group. At-5 years, lung graft survival (62% vs. 66%) and freedom from chronic lung allograft dysfunction (62% vs. 63%) did not differ between the lung-liver vs. lung-only groups (p=0.74 and p=0.71, respectively). <h3>Conclusion</h3> Notwithstanding the longer ischemic times and the complexity of the procedure, the liverfirst strategy for combined lung-liver transplantation did not impair lung graft survival and function.

Acute hepatic porphyria: when to perform liver transplantation?

Acute hepatic porphyrias (AHPs) are inborn errors of hemebiosynthesis and its most common and severe type is the acute intermittent porphyria (AIP). AIP is an hereditary autosomal dominant disease caused by accumulated porphobilinogen deaminase (PBG) and delta aminolevulin acid (ALA) products. The main symptoms are severe abdominal pain, neuromuscular and psychiatric disturbances, nausea, vomiting, encephalopathy, tachycardia, seizures, tremors and hypertension, that usually are manifested by acute crises. The treatment is based on clinical management and in cases which the patient’s quality of life is affected liver transplantation (LT) may be an alternative choice. We report the case of a patient with AHP presenting recurrent crisis leading to chronic symptoms occurrence and poor quality of life with progressive unresponsiveness to hemin treatment. Patient was submitted to LT as curative therapy proposal, but patient still presents some clinical manifestations that may indicate the possibility of a secondary cause to explain persistence of her symptoms despite of biochemical normalization of ALA and PBG.

Liver Transplantation in Progressive Familial Intrahepatic Cholestasis with Normal Gamma-Glutamyl Transferase: Evaluation of Post-transplant Steatosis and Steatohepatitis

Background: Progressive familial intrahepatic cholestasis is a disease presenting with severe cholestasis and progressing to the end-stage liver disease later. Liver transplantation is a treatment modality available for progressive familial intrahepatic cholestasis, especially in patients with end-stage liver disease or those who are unsuitable for or have failed biliary diversion. Objectives: To evaluate clinical and pathological characteristics of progressive familial intrahepatic cholestasis patients who had undergone liver transplantation and to determine post-transplant steatosis and steatohepatitis. Methods: We evaluated 111 progressive familial intrahepatic cholestasis patients with normal gamma-glutamyl transferase that performed liver transplantation in Shiraz Transplant Center in Iran between March 2000 and March 2017. Results: The most common clinical manifestations were jaundice and pruritus. Growth retardation and diarrhea were detected in 76.6% and 42.5% of the patients. After transplantation, growth retardation was seen in 31.5% of the patients, and diarrhea in 36.9% of them. Besides, 29.1% of the patients died post-transplant. Post-transplant liver biopsies were taken from 50 patients, and 15 (30%) patients had steatosis or steatohepatitis, five of whom (10%) had macrovesicular steatosis alone, and 10 (20%) had steatohepatitis. Only one patient showed moderate bridging fibrosis (stage III), and none of them showed severe fibrosis. Conclusions: Liver transplantation is the final treatment option for these patients, and it can relieve most clinical manifestations. However, post-transplant mortality rate was relatively high in our center. Diarrhea, growth retardation, and steatosis are unique post-transplant complications in these patients. The rate of post-transplant steatosis and steatohepatitis in patients with liver biopsy in our study was 30%, with a significant difference from previous studies.

Macroscopic Characteristics of the Native Liver in Children With MPV17-Related Mitochondrial DNA Depletion Syndrome: An Indication for Performing Liver Transplantation?

Mitochondrial respiratory chain disorders (MRCDs) are rare conditions with various clinical manifestations that depend on the genetic type, with subsequent severity of the liver disease. Clinical presentations include acute liver failure or chronic liver failure with liver cirrhosis, which are cruelly progressive and fatal. We report six cases of LT for acute liver failure diagnosed pathogenic MPV17 variants. Three out of six cases were siblings with the MPV17-mutation (c.148C>T, p.R50W / c.149G>A; p.R50Q), also known as the childhood form of Navajo neurohepatopathy. Those three patients underwent LT for liver cirrhosis at the ages of 5, 7, and 7 years, respectively. All three recipients are currently doing well without any neurological sequelae. In contrast, the three additional patients that presented with an acute liver failure and subsequent diagnosis of mutation in MPV17 (homozygous c.451dupC: p.L151PfsX39; homozygous c.451dupC : p.L151PfsX39; c.451dupC : p.L151PfsX39 / c.71-2_79del11ins4), underwent LT at the ages of 4 months, 11 months, and 1 year, respectively. All three recipients died from pulmonary hypertension following successful LT. Interestingly, the intraoperative macroscopic findings of the native liver at the time of LT revealed distinctive, microgranular nodules and liver fibrosis with severe steatosis ("avocado-like" liver). LT might be contraindicated in MPV17 patients with c.451dupC mutation due to the extremely poor prognosis caused by fatal progressive pulmonary hypertension. The identification of the typical macroscopic finding ("avocado-like"), seen in such pediatric transplant candidates with acute liver failure, might be helpful to provide an early diagnosis of MPV17-related c.451dupC mutation.

Experience of our first-ever living donor liver transplantation in HIV-positive patient: Report from developing country with literature review

Human Immunodeficiency Virus (HIV) is one of the gravest health challenges worldwide in the present era as it has been for the past 4 decades. HIV has always been a sort of stigma and most clinicians are reluctant to perform liver transplantation for chronic liver disease patients with HIV co-infection. Patients with CD4+ cell count &gt;100/ml along with suppressed mRNA levels on PCR, stable HAART regimen, and absence of any AIDS-specific illness or opportunistic infections are the requirements for selecting HIV-positive recipients for liver transplantation.

Impact of the Share 35 Policy on Perioperative Management and Mortality in Liver Transplantation Recipients.

BackgroundThe Share 35 policy was introduced in 2013 by the Organ Procurement and Transplantation Network (OPTN) to increase opportunities of sicker patients to access liver transplantation. However, it has the disadvantage of higher MELD score associated with adverse postoperative transplant outcomes. Early data after implementation of the Share 35 policy showed significantly poorer post-transplantation survival in some UNOS regions. We aimed to analyze the impact of Share 35 on demographics of patients, perioperative management, and perioperative mortality.Material/MethodsA retrospective analysis of data was performed from an institutional liver transplantation cohort from 1 January 2008 to 31 December 2017. Adult patients who underwent liver transplantation before 2013 were defined as the pre-Share 35 group and the other group was defined as the post-Share 35 group. The MELD score of each patient was calculated at the time of transplantation. Perioperative mortality was defined as death within 30 days after the operation.ResultsA total of 1596 patients underwent liver transplantation. Of those, 895 recipients underwent OLT in the pre-Share 35 era and 737 in the post-Share 35 era. The median MELD score was significantly higher in the post-Share 35 group (30 vs 26, P<0.001) and 45.7% of the post-Share 35 group had MELD scores ≥35. In intraoperative management, patients required significantly more blood component transfusion, intraoperative vasopressor, and fluid replacement. Veno-venous bypass (VVB) usage was significantly higher in the post-Share 35 era (47.2% vs 38.1%, P<0.001). In the subgroup of patients with MELD scores ≥35, the median waiting time was significantly shorter (18.5 vs 14.5 days, P=0.045). Overall perioperative mortality was not significantly difference between groups (P=0.435).ConclusionsAfter implementation of the Share 35 policy, we performed liver transplantation in significantly higher medical acuity patients, which required more medical resources to obtain a result comparable to that of the pre-Share 35 era.