Assessing clinical benefits of live-attenuated vaccination in post-liver transplant patients: Analysis of breakthrough infections and natural boosters.

Recently, live-attenuated measles, rubella, varicella, and mumps vaccines have been administered to carefully selected post-liver transplant patients. While attention has been on post-vaccination antibody titers and adverse events, the real-life clinical benefits remain unclear. A comprehensive analysis of breakthrough infections and natural boosters (asymptomatic cases with significant elevation in virus antibody titers) following immunization post-liver transplantation was conducted from 2002-2023, exploring the timing, frequency, correlation with domestic outbreaks, and degree of antibody elevation. During the median 10-year observation period among 68 post-liver transplant patients, breakthrough infections occurred only in chickenpox, with 7 mild cases (1 episode/64 person-years). A total of 59 natural booster episodes (1, 5, 20, and 33 for measles, rubella, chickenpox, and mumps, respectively) were observed, with incidence rates of 1 per 569, 110, 22, and 17 person-years, respectively. The timing of natural boosters closely correlated with domestic outbreaks (p<0.05, in chickenpox and mumps), influenced by local vaccine coverage. The degree of antibody elevation was significantly higher in individuals with breakthrough infections than in those with natural boosters (p<0.05). These findings suggest that immunization with live-attenuated vaccines for post-liver transplant patients has demonstrated clinical benefits. Furthermore, mass vaccination has a positive impact on post-transplant patient outcomes.

Second-Generation Antipsychotic Long-Acting Injection Reduced the Time of Restrictive Interventions in Patients With Schizophrenia: Retrospective, A 4-Year Mirror-Image Study.

BACKGROUND: Whether second-generation antipsychotic long-acting injection (SGA-LAI) reduces psychotic symptoms at relapse compared with oral antipsychotics remains unclear. The present study investigated the effects of SGA-LAI on the time (in hours) of restrictive interventions in hospitalization by conducting a retrospective observational 4-year mirror-image study at a single medical center in Japan. METHOD: We performed a retrospective observational mirror-image study conducted between November 2013 and January 2018. Data were initially retrieved from 101 patients. The 38 patients with schizophrenia who met the inclusion criteria were enrolled in the analysis. The primary outcome was the time of restrictive interventions and the secondary outcomes included the number of hospitalizations (total, voluntary, and involuntary) and bed days compared 2 years before and after initiating SGA-LAI. The restrictive interventions were defined as seclusion and physical restraints. RESULTS: The mean time of restrictive interventions significantly decreased from 43.7 to 3.03 ( P = 0.021). The number of admissions and the total number of bed days in post-SGA-LAI fell from 1.03 to 0.61 ( P = 0.011) and 130 to 39.3 ( P = 0.003), respectively, compared with pre-SGA-LAI. In particular, the number of involuntary admissions was significantly reduced (0.50-0.26, P = 0.039). CONCLUSIONS: The findings indicate that SGA-LAI reduced the time of restrictive interventions and the number of involuntary admissions. Moreover, SGA-LAI may contribute to mild psychiatric symptoms during relapse.

Feasibility of dose-dense cisplatin-based chemotherapy in Japanese children with high-risk hepatoblastoma: Analysis of the JPLT3-H pilot study.

BACKGROUND: The SIOPEL-4 study has demonstrated that dose-dense cisplatin-based chemotherapy dramatically improves outcome in children with high-risk hepatoblastoma in western countries. However, the feasibility and safety of this regimen have not been clarified in Japanese patients. METHODS: A pilot study, JPLT3-H, was designed to evaluate the safety profile of the SIOPEL-4 regimen in Japanese children with newly diagnosed hepatoblastoma with either metastatic disease or low alpha-fetoprotein. RESULTS: A total of 15 patients (three female) were enrolled. Median age was 2 years (range, 0-14). Three patients were PRETEXT II (where PRETEXT is PRETreatment EXTent of disease), six PRETEXT III, and six PRETEXT IV. All patients had lung metastasis, none had low alpha-fetoprotein. Eight patients completed the prescribed treatment, and seven patients discontinued therapy prematurely, four due to progressive disease and three due to causes other than severe toxicity. Grade 4 neutropenia was documented in most patients in preoperative cycles A1-3 (11/15 in A1, 9/11 in A2, and 7/11 in A3) and in all considering all cycles. Grade 3-4 thrombocytopenia and grade 3 anemia were also frequently observed. Patients experienced several episodes of grade 3 febrile neutropenia, but none had grade 4 febrile neutropenia or severe infections. One patient had grade 3 heart failure only in the first cycle. Other grade 3 or 4 toxicities were hypomagnesemia, anorexia, nausea, mucositis, liver enzyme elevation, fever, infection, and fatigue. There were no unexpected severe toxicities. CONCLUSION: The toxicity profile of JPLT3-H was comparable to that of SIOPEL-4. Dose-dense cisplatin-based chemotherapy may be feasible among Japanese patients with high-risk hepatoblastoma.

Methicillin-resistant Staphylococcus aureus carriers are vulnerable to bloodstream infection after living donor liver transplantation.

BACKGROUND: Bloodstream infection (BSI) is a life-threatening complication after living donor liver transplantation (LDLT). We aimed to explore the incidence and predisposing factors of BSI at our institution. METHODS: We conducted a retrospective cohort analysis on all consecutive adults with BSI within 6 months after LDLT performed between 2005 and 2016. For antimicrobial prophylaxis, ampicillin/sulbactam, cefotaxime, and micafungin were administered. From 2011, methicillin-resistant Staphylococcus aureus (MRSA) carriers were decolonized using mupirocin ointment and chlorhexidine gluconate soap. Risk factors for BSI were identified by uni- and multivariate logistic regression. RESULTS: Of a total of 106 LDLTs, 42 recipients (40%) suffered BSI. The BSI group demonstrated significantly higher in-hospital mortality rates compared with the non-BSI group (24% vs. 7%, P = .01). We identified MRSA carrier (odds ratio [OR], 19.1; P < .001), ABO incompatibility (OR, 2.9; P = .03), and estimated glomerular filtration rate <30 mL/min/1.73m2 (OR, 15.8; P = .02) as independent risk factors for BSI. Decolonization treatment for MRSA carriers did not reduce the incidence of all-cause BSI but reduced the frequency of BSI caused by MRSA. CONCLUSION: To our knowledge, for the first time, MRSA carriers were revealed to be highly vulnerable to BSI after LDLT.

Myelopathy due to human T-cell leukemia virus type-1 from the donor after ABO-incompatible liver transplantation.

We report the case of a 53-year-old-man who developed human T-cell leukemia virus type-1-associated myelopathy (HAM) after ABO-incompatible liver transplantation for alcoholic liver cirrhosis. The living donor was seropositive for human T-cell leukemia virus type-1 (HTLV-1) and the recipient was seronegative for HTLV-1 before transplantation. After transplantation, the recipient developed steroid-resistant acute cellular rejection, which was successfully treated using anti-thymocyte globulin, and he was eventually discharged. He underwent spinal surgery twice after the transplantation for the treatment of cervical spondylosis that had been present for a period of 9 months before the transplantation. The surgery improved his gait impairment temporarily. However, his gait impairment progressed, and magnetic resonance imaging revealed multiple sites of myelopathy. He was diagnosed with HAM 16 months after the transplantation. Pulse steroid therapy (1000mg) was administered over a period of 3 days, and his limb paresis improved. Presently, steroid therapy is being continued, with a plan to eventually taper the dose, and he is being carefully followed up at our institution. Our case suggests that liver transplantation involving an HTLV-1-positive living donor carries the risk of virus transmission and short-term development of HAM after transplantation.

Network elasticity of a model hydrogel as a function of swelling ratio: from shrinking to extreme swelling states.

In this work, we intended to investigate the relationship between the swelling ratio Q and Young's modulus E of hydrogels from their contracted state to extreme swelling state and elucidate the underlining molecular mechanism. For this purpose, we used tetra-poly(ethylene glycol) (tetra-PEG) gel, whose network parameters are well known, as the polymer backbone, and we succeeded in tuning the swelling of the gel by a factor of 1500 times while maintaining the topological structure of the network unchanged, using an approach combining a molecular stent method and a PEG dehydration method. A master curve of Q-E, independent of the method of obtaining Q, was obtained. Using the worm-like chain model, the experimentally determined master curve can be well reproduced. We also observed that the uniaxial stress-strain curve of the hydrogel can be well predicted by the worm-like chain model using the structure parameters determined from the fitting of the Q-E experimental curve.

Successful living donor liver retransplantation for graft failure within 7 days due to acute de novo donor-specific anti-human leukocyte antigen antibody-mediated rejection.

Growing evidence suggests a relationship between antibody-mediated rejection (AMR) and early graft failure due to a previously unknown etiology in liver transplantation (LTx). We herein report a 3-year-old boy who developed rapid graft failure due to de novo donor-specific antibody (DSA)-driven AMR a week after living donor LTx, requiring a second transplant on the 10th day after the first LTx. The pathology of the first graft showed massive necrosis in zone 3 along with positive C4d and inflammatory cell infiltrates in portal areas. The mean fluorescence intensity against human leukocyte antigen (HLA)-DR15, which was possessed by both the first and the second donor, peaked at 12 945 on the day before the second LTx. Antithymocyte globulin, plasma exchange along with i.v. immunoglobulin, rituximab, and the local infusion of prostaglandin E1, steroids, and Mesilate gabexate through a portal catheter were provided to save the second graft. To our knowledge, this is the first report to show a clear association between de novo DSA and acute AMR within 7 days of a LTx. Furthermore, we successfully rescued the recipient with a second graft despite possessing the same targeted HLA. The rapid decision to carry out retransplantation and specific strategies overcoming AMR were crucial to achieving success in this case of immunologically high-risk LTx.

Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology-from peri-transplant through total cavopulmonary connection: A case report.

Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8-month-old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology. The cardiac anomalies included total anomalous pulmonary venous return type IIb, intermediate atrioventricular septal defect, tricuspid regurgitation grade III, coarctation of aorta, interrupted inferior vena cava, bilateral superior vena cava, and polysplenia syndrome. Following LDLTx, the patient sequentially underwent total cavopulmonary shunt + Damus-Kaye-Stansel at 3 years of age and extracardiac total cavopulmonary connection (EC-TCPC) completion at 5 years of age; 7 years have now passed since LDLTx (2 years post-EC-TCPC). We describe the details of the management of LTx in the presence of cardiac anomalies and report the long-term cardiac and liver function, from peri-LDLTx through EC-TCPC completion.

The optimal immunosuppressive protocol for the portal vein infusion of PGE1 and methylprednisolone in pediatric liver transplantation for fulminant hepatic failure of unknown etiology.

The outcome of LTx in pediatric patients with FHF of unknown etiology remains inferior to that of LTx in pediatric patients with cholestatic diseases. A higher incidence of steroid-resistant severe rejection has been increasingly recognized among the responsible factors. We assessed the efficacy of the administration of steroids and PGE1 via PVI in the management of LTx for FHF in pediatric patients. In our early cohort (1995-2007), seven patients who underwent LTx for FHF of unknown etiology were treated with conventional immunosuppressive therapy (calcineurin inhibitor and a steroid). Seven of eight grafts (one patient underwent re-LTx) sustained CV and/or CPV associated with ACR, and four patients died of a graft failure or infectious complications that were associated with the treatment for rejection. Of note, the pathological incidence of CV/CPV was significantly higher in recipients with FHF of unknown etiology than in recipients with biliary cholestatic disease during the same study period (87.5% vs. 13.7%, p < 0.00001). From 2008, three patients underwent LTx for cryptogenic FHF with PVI and conventional IS. PVI was well tolerated, and no relevant severe complications were observed. More strikingly, the patients who received PVI overcame biopsy-proven immunological events and are all currently doing well with excellent graft function after more than five yr. We conclude that PVI is technically safe and effective for preventing severe rejection in pediatric patients who undergo LTx for FHF of unknown etiology and that it does not increase the risk of fatal infectious complications.

Diagnosing native liver fibrosis and esophageal varices using liver and spleen stiffness measurements in biliary atresia: a pilot study.

BACKGROUND: Biliary atresia commonly leads to liver fibrosis and cirrhotic complications, including esophageal varices. OBJECTIVE: To evaluate liver and spleen stiffness measurements using acoustic radiation force impulse (ARFI) imaging for diagnosing grade of liver fibrosis and predicting the presence of esophageal varices in patients treated for biliary atresia. MATERIALS AND METHODS: ARFI imaging of the spleen and native liver was performed in 28 patients with biliary atresia. We studied the relation between ARFI imaging values and liver histology findings (n=22), upper gastrointestinal endoscopy findings (n=16) and several noninvasive test results. Diagnostic accuracy was assessed using receiver operating characteristic curve analyses. RESULTS: Liver stiffness measurements exhibited a significant difference among the different grades of liver fibrosis (P=0.009), and showed higher values in patients with high-risk esophageal varices than in the other patients (P=0.04). The areas under the receiver operating characteristic curves of liver stiffness measurements for liver fibrosis grades ≥ F2, ≥F3 and = F4 were 0.83, 0.93 and 0.94, respectively. Patients with high-risk esophageal varices were preferentially diagnosed by the combined liver and spleen stiffness measurements (area under the curve, 0.92). CONCLUSION: Liver and spleen stiffness measurements using ARFI imaging are potential noninvasive markers for liver fibrosis and esophageal varices in patients treated for biliary atresia.

Steroid minimization immunosuppression protocol using basiliximab in adult living donor liver transplantation for hepatitis C virus-related cirrhosis.

AIM: Recent randomized trials have failed to prove the benefit of steroid-free immunosuppression in liver transplantation for hepatitis C virus (HCV)-related cirrhosis. Furthermore, there is a lack of data on the use of basiliximab in living donor liver transplantation (LDLT). This pilot study evaluated the safety and efficacy of a steroid minimization protocol using basiliximab compared with standard immunosuppression. METHODS: A single center, prospective cohort analysis was conducted to compare two immunosuppression regimens in adult recipients who underwent LDLT for HCV since 2004: calcineurin inhibitor/mizoribine/basiliximab (the St- group) and calcineurin inhibitor/mizoribine/steroid (the St+ group). Study end-points were rejection rates, recurrent HCV, patient survival and other adverse events up to 2 years after transplantation. RESULTS: A total of 27 consecutive patients were enrolled. Transplantation characteristics were similar between the two groups (14 St- and 13 St+) except ABO incompatible cases being more common in the St+ group. Rejection rates, recurrent HCV, patient survival, fibrosis stage and new-onset diabetes mellitus at 2 years were comparable between the two groups. ABO incompatibility did not affect short- and long-term outcomes. Nine St- and seven St+ recipients underwent interferon and ribavirin therapy for recurrent HCV, with a sustained virological response rate of 33% and 29%, respectively. CONCLUSION: A steroid minimization protocol with basiliximab in adult LDLT for HCV is safe and affords equivalent rejection rates compared with standard immunosuppression. However, no significant differences are observed with respect to recurrent HCV, patient survival and metabolic complications.

Spleen stiffness measurements by acoustic radiation force impulse imaging after living donor liver transplantation in children: a potential quantitative index for venous complications.

BACKGROUND: Living donor liver transplantation in children often results in venous complications, leading to portal hypertension. Spleen stiffness measurements have been recently proposed as a new, noninvasive parameter for portal hypertension in cirrhotic patients. OBJECTIVE: To evaluate the diagnostic value of spleen stiffness measurements by acoustic radiation force impulse (ARFI) imaging in diagnosing venous complications after pediatric living donor liver transplantation. MATERIALS AND METHODS: We prospectively enrolled 69 patients after pediatric living donor liver transplantation using a left-side liver allograft. Around the time of the protocol liver biopsy examination, spleen stiffness measurements by ARFI imaging were performed via the left intercostal space at the center of the spleen parenchyma and repeated five times. Imaging examinations around the time of the spleen stiffness measurements were retrospectively reviewed. Regarding venous complications, significant portal and hepatic venous stenosis was defined as >50% stenosis on multiphasic computed tomography. RESULTS: After post hoc exclusion, 62 patients were studied. Portal and hepatic venous stenosis was identified in three and two patients, respectively. The median spleen stiffness values were 2.70 and 4.00 m/s in patients without and with venous complications, respectively (P < 0.001). Spleen stiffness measurements showed good diagnostic power for venous complications, and the cutoff value was determined as 2.93 m/s, with 100% sensitivity and 78.9% specificity. Spleen stiffness measurements decreased with the relief of venous stenosis resulting from an interventional radiology procedure. CONCLUSION: Spleen stiffness measurements by ARFI imaging might provide a useful quantitative index for venous complications after pediatric living donor liver transplantation.

Long-term native liver fibrosis in biliary atresia: development of a novel scoring system using histology and standard liver tests.

BACKGROUND & AIMS: Although liver fibrosis is an important predictor of outcomes for biliary atresia (BA), postsurgical native liver histology has not been well reported. Here, we retrospectively evaluated postsurgical native liver histology, and developed and assessed a novel scoring system - the BA liver fibrosis (BALF) score for non-invasively predicting liver fibrosis grades. METHODS: We identified 259 native liver specimens from 91 BA patients. Of these, 180 specimens, obtained from 62 patients aged ≥1 year at examination, were used to develop the BALF scoring system. The BALF score equation was determined according to the prediction of histological fibrosis grades by multivariate ordered logistic regression analysis. The diagnostic powers of the BALF score and several non-invasive markers were assessed by area under the receiver operating characteristic curve (AUROC) analyses. RESULTS: Natural logarithms of the serum total bilirubin, γ-glutamyltransferase, and albumin levels, and age were selected as significantly independent variables for the BALF score equation. The BALF score had a good diagnostic power (AUROCs=0.86-0.94, p<0.001) and good diagnostic accuracy (79.4-93.3%) for each fibrosis grade. The BALF score revealed a strong correlation with fibrosis grade (r=0.77, p<0.001), and was the preferable non-invasive marker for diagnosing fibrosis grades ⩾F2. In a serial liver histology subgroup analysis, 7/15 patients exhibited liver fibrosis improvement with BALF scores being equivalent to histological fibrosis grades of F0-1. CONCLUSIONS: In postsurgical BA patients aged ⩾1year, the BALF score is a potential non-invasive marker of native liver fibrosis.

Critical hepatic hemangioma in infants: recent nationwide survey in Japan.

The International Society for the Study of Vascular Anomalies (ISSVA) classification divides vascular lesions into two major entities: neoplasms originating from the vascular endothelium and vascular malformations. Although this concept has been widely accepted, little has been established regarding vascular lesions in deep organs, such as infantile hepatic hemangioma (IHH). The current nationwide survey identified 19 critical infantile hemangiomas during the most recent 5 years. On histopathology all the lesions examined were neoplastic, but portovenos shunt was found histologically or clinically in some cases. High-output cardiac failure, consumption coagulopathy, and respiratory distress were the major symptoms, and treatment-resistant coagulopathy seemed to be the most reliable predictor of fatal outcome. Although steroid has been the gold standard treatment for these lesions, 25% of the patients were totally insensitive to steroids, whereas propranolol had a prompt effect in one case. For critical IHH with steroid-insensitive thrombocytopenia and prothrombin time prolongation, novel therapeutic options including beta-blocker therapy, surgery, and liver transplantation should be urgently considered as alterative treatment. The present review summarizes the results of the survey.

Application of nuclear medicine to achieve less invasive surgery for malignant solid tumors in children.

BACKGROUND: The use of nuclear medicine for the management of malignant tumor, such as radioguided surgery and sentinel lymph node biopsy (SLNB), has been widely accepted in the adult practice. However, there are very few studies to apply those techniques for pediatric diseases. The aim of this study was to investigate the feasibility of application of nuclear medicine in surgery for neuroblastoma (NB) or rhabdomyosarcoma (RMS) in children. METHODS: Radioguided surgery using (123) I-metaiodobenzylguanidine was performed on six children with NB. SLNB using technetium-labeled tin or sulfur colloid was performed on two children with perineal RMS. Histological evaluation of resected specimens was performed to determine the accuracy of intraoperative detection and SLNB. All patients were evaluated for overall survival and complications. RESULTS: Intraoperative tumor localization using hand-held gamma probe was helpful in 85.7% of NB patients. Sensitivity and specificity of this technique were 81.8% and 93.3%, respectively. There were no postoperative complications, and four out of five patients with high-risk NB experienced disease-free survival (median follow up, 57 months). Sentinel lymph nodes were easily detected in patients with perineal RMS, and histological assessment revealed complete consistency with regional lymph node status. CONCLUSIONS: Nuclear medicine may have a potential application in the use of less invasive surgery for advanced NB or perineal RMS, the two most challenging pediatric malignancies.

Acoustic radiation force impulse imaging for assessing graft fibrosis after pediatric living donor liver transplantation: a pilot study.

Graft fibrosis is a common finding during protocol biopsy examinations after pediatric liver transplantation. We evaluated the clinical utility of liver stiffness measurements by acoustic radiation force impulse (ARFI) imaging, a novel ultrasound-based elastography method, for assessing graft fibrosis after pediatric living donor liver transplantation (LDLT). We performed 73 liver stiffness measurements by ARFI imaging in 65 pediatric LDLT recipients through the upper midline of the abdomen (midline value) and the right intercostal space (intercostal value) around the time of protocol biopsy examinations. Fifty-nine of these liver stiffness measurements could be compared with histopathological findings. Graft fibrosis was assessed according to the degrees of portal and pericellular fibrosis. Significant fibrosis, which was defined as F2 or worse portal fibrosis and/or moderate or worse pericellular fibrosis, was observed in 14 examinations, which had significantly higher midline (P = 0.005) and intercostal values (P < 0.001) than the others. Liver stiffness measurements by ARFI imaging significantly increased with increases in the portal and pericellular fibrosis grades. For the diagnosis of significant fibrosis, the areas under the receiver operating characteristic curve (AUROCs) were 0.760 (P = 0.005) and 0.849 (P < 0.001) for the midline and intercostal values, respectively. The optimal cutoff values were 1.30 and 1.39 m/second for midline and intercostal values, respectively. Slight but significant elevations were noted in the results of biochemical liver tests: serum levels of γ-glutamyltransferase showed the highest AUROC (0.809, P = 0.001) with an optimal cutoff value of 20 IU/L. In conclusion, liver stiffness measurements by ARFI imaging had good accuracy for diagnosing graft fibrosis after pediatric LDLT. The pericellular pattern of fibrosis was frequently observed after pediatric LDLT, and moderate pericellular fibrosis was detectable by ARFI imaging.

Report from the Committee for Improving the Work Environment of Japanese Surgeons: survey on effects of the fee revision for medical services provided by surgeons.

PURPOSE: The aim of this study was to achieve improvements in the work environment of Japanese surgeons and shortage of surgeons. METHODS: Questionnaires were distributed to selected Japanese surgical Society (JSS) members. Retrospective analysis was conducted comparing the current 2011 survey with previous 2007 survey. To examine the influence of 2010 revision of the fee for medical services performed by surgeons, we distributed a second questionnaire to directors of hospitals and administrators of clerks belonging to official institutes in JSS. Collective data were analyzed retrospectively. RESULTS: The main potential causes for the shortage of surgeons in Japan were long hours (72.8 %), excessive emergency surgeries (69.4 %), and high risk of lawsuit (67.7 %). Mean weekly working hours of surgeons in national or public university hospitals and private university hospitals were 96.2 and 85.6, respectively. Approximately 70 % of surgeons were forced to do hardworking tasks, possibly leading to death from overwork. Of note, approximately 25 % of surgeons had over time of more than 100 h a week, coinciding to the number of hours that might lead to death from fatigue, described in the Japanese labor law. Although the total medical service fee in hospitals, especially in large-scale hospitals with more than 500 beds, increased markedly after 2010 revision of the fee for medical services performed by surgeons, few hospitals gave perquisites and/or incentives to surgeons. CONCLUSION: To prevent and avoid collapse of the surgical specialty in Japan, an improvement in the work environment of surgeons by initiation of the JSS would be required as soon as possible.

Impact of pediatric intestinal transplantation on intestinal failure in Japan: findings based on the Japanese intestinal transplant registry.

INTRODUCTION: We assessed the impact of intestinal transplantation on Japanese pediatric patients with intestinal failure with data from the Japanese intestinal transplant registry. METHODS: Standardized forms were sent to all known intestinal transplantation programs, requesting information on transplants performed between 1996 and June 30, 2012. Patients younger than 18 years were analyzed. Patient and graft survival estimates were obtained using the Kaplan-Meier method. RESULTS: Of the 14 intestinal transplants, 4 were deceased and 10 were living donor transplants. The primary indications were: short gut syndrome (n = 7), intestinal functional disorder (n = 6), and re-transplantation (n = 1). The overall 1- and 5-year patient survival rates were 77 and 57%, respectively. In transplants performed after 2006 (n = 6), the one-year patient survival rate was 83%, and the 5-year survival rate was 83%. Graft one- and 5-year survival rates were 83 and 83%, respectively. The living-related transplant survival rate was 80% at 1 year and 68% at 2 years, compared to 67 and 67% for cadaveric transplant recipients. There were no statistically significant differences in patient (p = 0.88) and graft (p = 0.76) survival rates between living donor and cadaveric transplant recipients. All current survivors discontinued PN. CONCLUSION: Intestinal transplantation has become an effective therapy for patients with intestinal failure who cannot tolerate PN.

Feasibility of Real-Time Central Surgical Review for Patients with Advanced-Stage Hepatoblastoma in the JPLT3 Trial.

In the JPLT3 study, a real-time central surgical reviewing (CSR) system was employed aimed at facilitating early referral of candidates for liver transplantation (LTx) to centers with pediatric LTx services. The expected consequence was surgery, including LTx, conducted at the appropriate time in all cases. This study aimed to review the effect of CSR on institutional surgical decisions in cases enrolled in the JPLT3 study. Real-time CSR was performed in cases in which complex surgeries were expected, using images obtained after two courses of preoperative chemotherapy. Using the cloud-based remote image viewing system, an expert panel consisting of pediatric and transplant surgeons reviewed the images and commented on the expected surgical strategy or the necessity of transferring the patient to a transplant unit. The results were summarized and reported to the treating institutions. A total of 41 reviews were conducted for 35 patients, and 16 cases were evaluated as possible candidates for LTx, with the treating institutions being advised to consult a transplant center. Most of the reviewed cases promptly underwent definitive liver surgeries, including LTx per protocol.

A long-term survival case of tracheal agenesis: management for tracheoesophageal fistula and esophageal reconstruction.

Tracheal agenesis is a very rare disorder which leads to severe respiratory disorders immediately after birth. Reports are very limited on long-term survival cases. We report here a long-term survival case with Floyd's type I tracheal agenesis. During the neonatal stage, the patient underwent abdominal esophageal banding to substitute esophagus for trachea and transection at the cervical esophagus with esophagostomy. Subsequently, airway management was difficult due to a fragile tracheoesophageal fistula, but the fistula was conservatively treated and stabilized with the patient's growth. This patient is a very rare case in whom oral feeding was achieved after esophageal reconstruction using a gastric tube. For this case, we describe mainly (1) the management method of the tracheoesophageal fistula and (2) esophageal reconstruction without thoracotomy.