Purpose: Biliary atresia (BA) is the leading cause of end-stage liver disease necessitating liver transplantation in children. The aim of this study was to share our experience with liver transplantation in biliary atresia patients. Materials and Methods: The medical records of BA patients who underwent liver transplantation between December 1, 2006 and June 30, 2013 were evaluated retrospectively. Patients with a 6 month follow up period were analyzed in two groups based on history of Kasai portoenterostomy. Results: Of 184 liver transplants performed in 176 pediatric patients at our center, 40 BA patients (Group 1, with Kasai, n=28; Group 2, without Kasai, n=12) were included in the study. The patient population consisted of 21 females and 19 males with a median age of 9 months (range 5-32 months). Median body weight was 7.45 kg (range 5.3-36 kg). The most common complications in the early postoperative period were portal vein thrombosis (n=6), hepatic artery thrombosis (n=1), intraabdominal collection (n=6), gastrointestinal bleeding (n=5), biliary leakage (n=4), and intestinal perforation (n=2). Causes of mortality included septicemia (n=5), gastrointestinal system bleeding (n=1), pulmonary system bleeding (n=1), and primary nonfunction (n=1). One- and three-year patient survival were 85.7% and 85.7% for Group 1 and 75% and 75% for Group 2, respectively. There were no statistical differences between groups, but survival rates were worse in Group 2, most likely due to low body weight. Conclusion: Liver transplantation is a life saving procedure with a high survival rate for patients with BA. Timing of transplantation is important for patients with and without Kasai portoenterostomy.

, The purpose of this study is to examine the psychosocial profiles of patients with severe alcoholic hepatitis (AH) being considered for early liver transplantation (LT) at a liver transplant center in the United States. From 1/2012 to 6/2013, consecutive patients with severe AH at our LT center were evaluated for early LT on a case-by-case basis according to center-established guidelines modeled after Mathurin et al. Severe AH was defined as a discriminant function ≥ 32 with an appropriate clinical history. Candidates must have either failed medical ther-apies for AH or deemed ineligible. They underwent an expe-dited, comprehensive psychosocial evaluation by a transplant social worker and psychiatrist. Unanimous agreement regarding LT candidacy and clinical appropriateness amongst all providers was required prior to listing. Forty-three patients with severe AH were evaluated. Thirty-two patients with a median MELD 29 were declined after psychosocial evaluation, while 11 out of 43 (26%) patients were provisionally accepted as candidates for early LT with a median MELD 34. Five declined patients with incomplete psychosocial evaluations were excluded from the final analysis. In the declined cohort, 11 patients improved, 14 died and the outcomes of HA on current management guidelines. Methods: A retrospective of com-pleted. Demographic information OCP use, BMI), clinical (symptoms, interventions, follow-up), imaging, and pathology (number, size, hemorrhage, malignant change) Results: 28 patients with HA were identified, 2 males and 26 The median age was 39 (range: median BMI and the 6 inter-vention, HCC In living donor liver transplantation (LDLT), venous thromboem-bolism (VTE) has appeared as a significant source of morbidity and mortality in donors. Factor V Leiden (FVL) and prothrombin G20210A (FII) mutations are the most common inherited risk factors, which contribute to the occurrence of VTE. The aim of this retrospective cohort study is to assess the safety of utilizing living liver donors with positive thrombophilia screening. Between June 2004 and July 2012, 410 LDLTs were performed in our institution without donor mortality. A retrospective analysis of the first 214 cases revealed that, the two donors (2/214, 0.9%) who developed VTE (1 pulmonary embolism, 1 portal vein thrombosis) after donor hepatectomy had homozygous (HO) FII mutation. In April 2010, we started routine thrombophilia screening during the initial phase of evaluation in all potential donors. In a total of 665 potential donors who underwent screening, the rate of heterozygous (HE) and HO mutations for Factor V Leiden (FVL) and FII were 11.5% and 0.7%, and 4.5% and 0.6%, respectively. All potential donors with HO FVL or HO FII mutations (n=9), and those with double HE FVL-FII mutation (n=4) were eliminated. A total of 23 donors with HE-FVL mutation and 7 donors with HE-FII mutation underwent donor hepatectomy. These 30 were given low molecular weight heparin (LMWH) for VTE prophylaxis until they were discharged from the hospital. In a median follow-up of 15.0 (12.0-25.5) months, none of the donors with either HE-FVL or HE-FII mutations had VTE. In the second cohort, four donors (4/196, 2.0%) developed VTE (3 PE and 1 deep vein thrombosis) and were treated with short-term LMWH. Further hematologic work-up of these donors did not reveal any pro-thrombotic disorder. Carriers of HO FVL/FII mutations have significantly increased risk of VTE. Acquired risk factors such as hypercoagulability after partial hepatectomy may further increase the risk. We recommend routine thrombophilia screening during the evaluation of potential living liver donors, and elimination of those with HO FVL/FII mutations. Our results support the utilization of donors with a single HE-FVL or HE-FII mutation, provided that they are given LMWH for VTE prophylaxis. MELD score MELD and MELD 26 to 40). compared between LDLT with MELD scores. to stratified donor –recipient pairs and facilitate counseling patients and their potential donors in regards to clinical outcome when live donor liver is an option for transplantation. choledochojejunostomy patients undergoing LT comparing Roux-en-Y choledochojejunostomy versus duct-to-duct anastomosis during LT for PSC were identified based on systematic searches of nine electronic data-bases and multiple sources of gray literature. RESULTS: The search identified 496 citations, including 7 retrospective series, and 692 patients met eligibility criteria. The use of duct-to-duct anastomosis was not associated with a significant difference in clinical outcomes, including 1-year recipient survival rates (OR 1.02; 95% CI 0.65-1.60; p=0.95), 1-year graft survival rates (OR 1.11; 95% CI 0.72-1.71; p=0.64), risk of biliary leaks (OR of 1.23; 95% confidence interval [CI] 0.59-2.59; p=0.33), risk of biliary strictures (OR 1.99; 95% CI 0.98-4.06; p=0.06), or rate of recurrence of PSC (OR 0.94; 95% CI 0.19-4.78; p=0.94). CONCLUSION: The current evidence presented herein does not support the universal preference of Roux-en-Y choledochojejunostomy for all patients undergoing OLT for PSC, as there is no significance difference in clinical outcomes between well-selected patients who receive duct-to-duct anastomosis versus Roux-en-Y loops. Selection will con-tinue to be made by the surgeon at time of LT with or without pre-LT cholangiography, based on donor and recipient characteristics, but barring other factors such as a diseased common bile duct, our results suggest duct-to-duct anastomosis should be [Background] Glucose storage diseases (GSD) show growth retardation, but there are a few reports about the growth pattern and the effect of portocaval shunt (PCS) and liver transplantation (LT) for GSD patients. This study aims to analyze the change of physical growth and 2nd sexuality after PCS or/and LT in GSD type I. [Patients and Methods] We reviewed retrospectively 56 patients (M : F=38 : 18) with GSD type I between 1975 and 2013. Among them, 13 underwent LT (at median 14 year-old, range 9-21, LT group) and 17 with PCS (10, 4-12, PCS group). Their data were compared with the normal data of CDC & WHO and the height standard deviation scores (Z-scores) and its annual differences (delta Z-score) were calculated and presented. And a modified delta Z-score (m-delta Z-score) was defined an annual difference between Z-score of operation group and the cross-sectional median Z-score of non-operation group. [Results] Regardless of height at birth, Z score for their height was sharply decreased to less than zero within 4 years in all patients. After operations, there was a spurt of height in the postoperative period. The median Z-score was -3.1 in LT group and -2.7 in PCS group at the time of operations. They caught up growth up to Z=-0.25 at postoperative 4 years in LT group and to Z=-0.6 at postoperative 6 years in PCS group. Delta Z-score were +0.4 Background: Posthepatectomy liver failure complication after hepatectomy. As Study of Liver Surgery PHLF as increased international normalized ratio and hyperbilirubinemia on or after postoperative day 5, and graded its severity based on required clinical management. We evaluated the impact of the ISGLS definition of PHLF on hepatocellular carcinoma (HCC) patients. Methods: ISGLS definition of PHLF was retrospectively assessed with 210 consecutive HCC patients who underwent curative hepatectomy at our facility from January 2005 to December 2010. The median follow-up period after hepatectomy was 35.2 months. Results: Thirty-nine (18.6%) patients fulfilled the ISGLS definition of PHLF. Mortality, hospital stay, and morbidity excluding PHLF increased with higher grades of PHLF (P < .001). Overall survival (OS) rates at 1, 3, and 5 years in patients with/without PHLF were 69.1/93.5, 45.1/72.5, 45.1/57.8%, respectively (P = .002). Recurrence-free survival (RFS) rates at 1, 3, and 5 years in patients with/without PHLF were 40.9/65.9, 15.7/38.3, 15.7/20.3%, respectively (P = .003). Multivariate analysis revealed that PHLF was significantly associated with both OS (P = .047) and RFS (P = .019). Extent of resection (P < .001), intraoperative blood loss (P = .002), and fibrosis stage (P = .040) were identified as independent risk factors for devel-oping PHLF. Conclusion: The ISGLS definition of PHLF was associated with OS and RFS in HCC patients, and long-term survival will be improved by reducing the incidence of PHLF. Background: We previously proposed expanded selection criteria for liver transplantation (LT) for hepatocellular carcinoma (HCC), the Kyoto criteria, involving a combination of tumor number ≤ 10, maximal diameter of each tumor ≤ 5 cm, and serum des-gamma-carboxy prothrombin levels ≤ 400 mAU/mL, and we have used these criteria since January 2007. In the present study, the usefulness of the criteria was prospectively as well as retrospectively validated. Methods: Two hundred patients with HCC who underwent living donor LT (LDLT) at our institute between February 1999 and February 2012 were enrolled in this study. Overall survival and the recurrence rate were investigated in patients classified according to the Kyoto criteria and the Milan criteria. Tumor biological aggressive-ness, including microvascular invasion and histological differ-entiation, according to the criteria was also examined. Results: The median follow-up period was 98 (range 12 – 168) months. The 5-year overall survival rate for patients within the Kyoto criteria (82%) was significantly higher Background: The accurate evaluation of preoperative liver function is essential to prevent postoperative liver failure, especially in patients with cirrhotic liver. In addition to conventional exam-ination of liver function such as Child-Pugh score and indocya-nine green (ICG) test, 99mTc-diethylenetriamine pentaacetic acid galactosyl human serum albumin (99mTc-GSA) scintigraphy has been expected to be more quantitative modality. However, it still rema

Selecting a kidney for living donor nephrectomy is driven by the tenet that donors are left with the higher functioning kidney. Traditionally, the left kidney is used because it has a longer renal vein, which aids anastamosis, and has an easier surgical approach. Anomalous left renal vasculature is not considered a contraindication to living donor nephrectomy. In the case of duplicated inferior vena cava, no specific considerations have been reported. We present a 42-year-old patient with infrarenal duplication of the vena cava who underwent laparoscopic living donor nephrectomy. His postoperative course was complicated by painful scrotal swelling necessitating multiple emergency room visits. Ultrasonography revealed bilateral hydroceles 5 weeks after surgery, which resolved with the use of a scrotal sling. Intraoperative ligation of a visibly dilated left gonadal vein was the likely etiology. Careful consideration should be taken in living donor nephrectomy in patients with duplication of inferior vena cava.

Objective. We performed single-center outcome comparison of pediatric recipients who underwent liver transplantation for either genetic or metabolic disease including the clinical impact of using heterozygote parents as living donors.Materials and Methods. Pediatric liver transplant recipients from September 2007 to December 2010 were included. Patients were separated into 2 categories by etiology of liver disease: (1) genetic or metabolic liver disease (G/M) and (2) nongenetic or metabolic liver disease (non-G/M), which included all other remaining etiologies combined. Patient demographics, recipient and donor characteristics, graft type, operative data, recipient complications, allograft and patient survival were analyzed.Results. Forty liver transplants were performed on 40 patients; 18 were transplanted for G/M; mean waiting time was 101 days for G/M group and 57 days for non-G/M group; 9 patients were listed as status 1; 5 were granted PELD/MELD exceptions; the overall mean PELD/MELD score was 21. Four G/M patients had hepatocellular carcinoma in the explant without microvascular invasion. Overall complications requiring either surgery or interventional radiology occurred in 14 patients G/M (n = 5); CMV viremia was seen in 11 patients (G/M, n = 1); detectable EBV DNA was detectable in 8 patients (G/M, n = 4), acute cellular rejection was seen in 10 (G/M, n = 5), postransplant lymphoproliferative disease occurred in 2 G/M patients; and 1 G/M patient showed significantly improved posttransplant neurologic motor function. Children with G/M who received a living donor liver transplant from heterozygote parents did well without any signs of expressing underlying metabolic disease. Posttransplant graft and patient overall survival at 12 months for G/M and non-G/M was 100%, and at 36 months, 83% and 100%, respectively.Conclusion. The majority of children transplanted for either genetic or metabolic disease were status 1 or awarded UNOS exception points. Cadaveric split livers and live donors including obligate heterozygotes resulted in excellent allograft and patient survival outcomes. In metabolic and genetic liver diseases, close follow-up and timely transplantation can preclude malignant spread and prevent disease progression and consequences, as well as reverse neurologic sequelae.

Combined liver kidney transplantation (LKT) can be successfully performed on patients with liver and renal failure; however, outcomes are inferior to liver transplantation alone (OLT). Our aim was to determine the indications for and outcome of LKT and whether patients with longer wait times required more frequent LKT versus OLT alone. We included 18/93 adults who underwent LKT from August 2007 to August 2010 for hepatitis C virus (HCV, n = 7), alcohol (n = 5), nonalcoholic steatohepatitis (n = 2), primary biliary sclerosis, polycystic kidney disease with liver involvement, hepatic adenomatosis, and ischemic hepatitis. Eleven were originally listed for LKT and 7 required listing for-kidney transplantation while awaiting OLT. Eight were on dialysis when first listed and 10 had a low glomerular filtration rate or known kidney disease. The mean calculated Model for End-Stage Liver Disease (MELD) score for LKT was 31.2 ± 3.54. Seven had hepatocellular carcinoma in explants. Two patients had acute cellular kidney rejection that responded to treatment. Recurrence of HCV was documented in 5 patients within 6 months of LKT; 2/5 received HCV therapy (interferon and ribavirin) without renal allograft rejection. One-year liver graft/patient survival was 94% after LKT. One patient died at 6 months post LKT due to severe HCV recurrence. Last mean serum creatinine level was 1.35 ± 0.28 mg/dL for LKT patients. LKT is a safe procedure with favorable outcomes even in patients with a high MELD score. Transplantation of patients with a high MELD score due to regional variations in organ allocation results in additional use of kidneys by OLT patients. Improved organ allocation algorithms in OLT would help to reduce combined transplants, sparing more kidneys.