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Biliary atresia (BA) is a rare disorder of unknown etiology. There is a debate as to whether maternal microchimerism plays a significant role in the development of BA or in graft tolerance after liver transplantation. Here, we performed quantitative-PCR-based assays for liver tissues of children with BA and other diseases. Maternal cells were detected in 4/13 and 1/3 of the BA and control groups, respectively. The estimated number of maternal cells ranged between 0 and 34.7 per 106 total cells. The frequency and severity of maternal microchimerism were similar between the BA and control groups, and between patients with and without acute rejection of maternal grafts. These results highlight the high frequency of maternal microchimerism in the liver. This study provides no evidence for roles of microchimerism in the etiology of BA or in graft tolerance. Thus, the biological consequences of maternal microchimerism need to be clarified in future studies.

The effectiveness of laparoscopic Kasai portoenterostomy (Laparoscopic-KPE) for biliary atresia (BA) has been reported but remains controversial. We reviewed our own cases and cases described in previous studies of liver transplantation (LT) for BA after Laparoscopic-KPE to investigate the efficacy of Laparoscopic-KPE. Subjects were children of ≤ 2 years old with LT for BA after KPE who underwent Laparoscopic-KPE (n = 10) or Open-KPE (n = 115) between 2009 and 2020. Propensity score matching was performed to reduce the effect of treatment selection bias. The clinical data regarding the preoperative characteristics and surgical results were compared. The rates of hypoplastic portal vein and retrograde portal vein flow were lower in the Laparoscopic-KPE group than in the Open-KPE group (0 vs. 40.0%, p = 0.02 and 0 vs. 35.0%, p = 0.04). There was no marked difference in the operation time or duration of hepatectomy. For portal vein reconstruction, a vein graft was not required in the Laparoscopic-KPE group (0 vs. 35.0%, p = 0.03). No patients in the Laparoscopic-KPE group developed portal vein complications or required re-laparotomy for bowel perforation or re-bleeding, in contrast to the Open-KPE group (0 vs. 15.0% and 0 vs. 10.0%, respectively). Laparoscopic-KPE may reduce postoperative complications that necessitate re-laparotomy in LT.

Surgical management of tumor thrombus extending to the major vascular system for children with hepatoblastoma is challenging and insufficiently discussed.We conducted a retrospective review of hepatoblastoma with tumor thrombus extending to the major vascular system (inferior vena cava, 3 hepatic veins, and portal vein trunk) treated at our center between May 2010 and June 2021. We describe our preoperative assessment, surgical strategies, and outcomes.We identified 9 patients (median age at the diagnosis: 3.4 years). All patients received chemotherapy before liver surgery. At the time of the diagnosis, tumor thrombus extended to the portal vein trunk (n = 6), inferior vena cava (n = 3), and 3 hepatic veins (n = 2). Among the 9 patients, 4 underwent liver resection. Liver transplantation was performed in 5 patients. The inferior vena cava wall was circumferentially resected for tumor removal in 1 patient and partially resected in 2 patients. One patient underwent liver transplantation using veno-venous bypass. Patients with tumor thrombus extending to the portal vein trunk were more likely to be managed by liver transplantation in comparison to those with tumor thrombus spreading to the inferior vena cava. The median follow-up period was 5.5 years. One patient underwent transhepatic balloon dilatation for biliary stricture after liver resection. Tumor recurrence was seen in 3 patients (33.3%; lung, n = 2; lymph node and liver, n = 1). No patients died during the follow-up period.Surgical intervention for pediatric hepatoblastoma with tumor thrombus extending into the major vascular system is safe, feasible, and achieves excellent outcomes.

Although nephrolithiasis (NL) and nephrocalcinosis (NC) are very common features of primary hyperoxaluria type 1 (PH1), the long-term prognosis of NL and NC after preemptive liver transplantation (PLT) has not been elucidated.We describe the cases of two chronic kidney disease (CKD) stage three patients with different clinical courses after PLT for PH1.The first patient underwent PLT at 7 years of age with an estimated glomerular filtration rate (eGFR) of 47.8 ml/min/1.73 mRegardless of the regression in NC seen on X-ray, long-term maintenance of the renal function in patients with PH1 with CKD stage 3 can be achieved with PLT. In patients with NL, there is a risk of serious complications due to posttransplant immunosuppressive therapy when obstructive pyelonephritis occurs after LT.

Various approaches to portal vein (PV) reconstruction have been described; however, there is still scope to improve the safety and efficiency of how PV anastomosis is performed in infants.We analyzed, retrospectively, the predictive factors for PV reanastomosis during living donor liver transplantation (LDLT) in 117 infants ( 1 year old) with biliary atresia (BA).Twenty-six infants required PV reanastomosis because of intraoperative PV thrombosis in 21 and insufficient PV flow in 5. Univariate analysis identified multiple previous laparotomies and a prolonged anhepatic phase (AHP) as significant risk factors for repeated PV anastomosis. The AHP cutoff value for identifying patients requiring PV reanastomosis was 134 min. Multivariate analysis revealed prolonged AHP 134 min (odds ratio, 15.98; 95% confidence interval, 5.05-50.6; P 0.001) as an independent risk factor for repeated PV anastomosis. The plasma D-dimer and fibrinogen degradation product concentration just after reperfusion were significantly higher in patients requiring PV reanastomosis (P 0.001). Patients requiring reanastomosis of the PV had a higher incidence of PV complications after LDLT than those who did not need PV reanastomosis (P = 0.01).Attempting to minimize the AHP is indispensable for successful LDLT in infants with BA.

Background In Japan, a recent gradual increase in deceased donor donation has expanded opportunities for pediatric patients to obtain deceased grafts. Methods Forty-three children underwent deceased donor liver transplantation (DDLT) at our institute before February 2020. Twenty-five patients received a split or reduced graft and 18 patients received a whole graft. The clinical outcomes of DDLT were retrospectively analyzed. Results The main organ resource was split/reduced grafts retrieved from adult donors; however, the number of whole grafts retrieved from pediatric donors has increased. The rates of major complications were similar in the two groups. The 5-year graft survival rate of patients who received a split/reduced graft (78.0%) was lower than that of patients who received a whole graft (88.9%; P = .40). The 3-year graft survival rates of patients who recently received a split/reduced graft and a whole graft improved to 92.3% and 91.7%, respectively. Conclusions The recent amendment of the organ allocation system, especially the introduction of pediatric prioritization, can effectively increase the chance to obtain deceased donor grafts for pediatric DDLT in Japan. The recent refinements in donor and recipient selection and in the surgical technique of split DDLT can improve the outcomes of pediatric DDLT in Japan.

Chronic myeloid leukemia (CML) is a myeloproliferative disorder caused by the BCR-ABL1 tyrosine kinase1,2. ABL1-selective tyrosine kinase inhibitors (TKIs) including nilotinib have dramatically improved the prognosis of patients with CML3–7. The ultimate goal of CML treatment is likely to be TKI-free maintenance of deep molecular response (DMR), which is defined by quantitative measurement of BCR-ABL1 transcripts on the international scale (IS)8, and durable DMR is a prerequisite to reach this goal9. Thus, an algorithm to enable the early prediction of DMR achievement on TKI therapy is quite valuable. Here, we show that our mathematical framework based on a clinical trial dataset10 can accurately predict the response to frontline nilotinib. We found that our simple dynamical model can predict nilotinib response by using two common laboratory findings (observation values): IS11,12 and white blood cell (WBC) count. Furthermore, our proposed method identified patients who failed to achieve DMR with high fidelity according to the data collected only at three initial time points during nilotinib therapy. Since our model relies on the general properties of TKI response, our framework would be applicable to CML patients who receive frontline nilotinib or other TKIs in clinical practice.Significance StatementChronic myeloid leukemia (CML) is a myeloproliferative disorder caused by the BCR-ABL1 tyrosine kinase. The goal of this treatment is the sequential achievement of deep molecular response (DMR). Tyrosine kinase inhibitors (TKIs) are effective in the reduction because they inhibit CML cell proliferation. However, because of individual differences in the TKI efficacy, some patients are unable to achieve DMR, so that early prediction of DMR reachability is necessary for personalized medicine. By combining time series analysis and mathematical modeling, we developed a mathematical method that accurately predicts patients who do not achieve DMR in the early stages of TKI administration. Our prediction method gives a basis of effective personalized treatments for CML patients.

X-linked myotubular myopathy (MTM) (OMIM 310400) is a severe neuromuscular disorder caused by mutations in the myotubularin (MTM1) gene. Liver hemorrhaging due to peliosis hepatis (PH) is a fatal complication. We herein report 2 successful cases of living-donor liver transplantation (LDLT) for MTM patients due to liver hemorrhaging caused by PH and review previous reports. A boy who was 9 years and 4 months old initially underwent left lateral segmentectomy due to massive hepatic and intraperitoneal hemorrhaging. As bleeding from the remnant liver continued after hepatectomy, this patient emergently underwent LDLT using a left lateral segment graft from his father. Another boy who was 1 year and 7 months old underwent transcatheter arterial embolization due to hepatic hemorrhaging and was referred to our hospital for LDLT using a left lateral segment graft from his father. The pathological findings in both cases showed sinusoidal dilatation with degenerative changes in reticular fiber and hematoma in the explanted liver, which were consistent with PH associated with MTM. LT should be considered as a treatment option for patients with episodes of hepatic hemorrhaging due to MTM in order to protect against fatal bleeding.

Anaplastic large cell lymphoma (ALCL) accounts for 10-15% of childhood non-Hodgkin lymphoma cases; it is generally chemo-sensitive and is one of the most curable pediatric cancers. We report here a case of pediatric ALCL complicated with acute liver failure due to the aggravation of pre-existing biliary hepatopathy by lymphoma-associated hemophagocytic lymphohistiocytosis (HLH). Although the initial treatment response against ALCL was very good, poor and irreversible liver function due to biliary cirrhosis worsening by lymphoma-associated HLH prevented the patient from receiving further consolidation chemotherapies. To make matters worse, his condition was accompanied with intrahepatic fungal pseudoaneurysm and invasive fungal infection. Thus, we decided to perform an urgent living-donor liver transplantation from his father to correct the patient's liver function and make it possible to proceed with further ALCL therapy. After the living-donor liver transplantation, the patient successfully received consolidation therapy with brentuximab vedotin. To our knowledge, this may be an early reported case of a pediatric patient undergoing liver transplantation during treatment for ALCL. In most patients with HLH-associated ALCL, liver function improves when ALCL is controlled. However, acute liver failure is occasionally observed in HLH cases with pre-existing liver dysfunction. In such cases, liver transplantation should be considered to correct liver dysfunctions if the disease control of HLH is satisfactory.

For patients who have chronic myeloid leukemia (CML), one of the primary treatment options is administration of nilotinib 300 mg twice daily (BID). In previous studies which compared outcomes associated with nilotinib or imatinib treatment, nilotinib achieved a higher rate of deep molecular response (MR). We conducted a phase II, open‐label, multicenter study to investigate an intrapatient nilotinib dose‐escalation strategy for patients with newly diagnosed chronic‐phase (CP) CML based on early MR4.5 achievement. The primary study endpoint was achievement of MR4.5 by 24 months following the initiation of nilotinib 300 mg BID. Fifty‐three patients were enrolled, 51 received nilotinib, and 37 completed the treatment. An increase in the nilotinib dose (to 400 mg BID) was allowed when patients satisfied our criteria for no optimal response at any time point. The median (range) dose intensity was 600 (207‐736) mg/day. Of 46 evaluable patients, 18 achieved an optimal response and 28 did not. Of the latter, nine patients underwent dose escalation to 400 mg BID, and none achieved MR4.5. The remaining 19 patients could not undergo dose escalation, 12 (63%) because of adverse events (AEs), and 7 (37%) for non‐AE related reasons. Four of these patients achieved MR4.5. The MR4.5 rate by 24 months was 45.7%. The progression‐free, overall and event‐free survival were each 97.6%. No new safety concerns were observed. Our findings support the use of continuous nilotinib at a dose of 300 mg BID for newly diagnosed patients with CML‐CP., The N‐Road study aimed to determine the optimal treatment strategy for nilotinib use in patients with newly diagnosed CML‐CP based on early achievement of deep molecular response. Most patients received nilotinib at a dose of 300 mg BID and were able to achieve an early deep molecular response. Together with the finding that none of the patients whose dose was escalated to 400 mg BID achieved MR4.5, we consider that continuous nilotinib at a dose of 300 mg BID may be the optimal treatment strategy for newly diagnosed patients with CML‐CP.

AIM It has been reported that the long-term outcome for children with metabolic disorders after liver transplantation (LT) is excellent. However, there are several reports citing LT patients with metabolic disorders developing liver dysfunction early after LT. We examined the pathogenesis of liver dysfunction observed after LT in recipients with metabolic disorders at the National Center for Child Health and Development. METHODS Of 106 children (aged

Hepatic epithelioid hemangioendothelioma is a rare neoplasm with a variable malignant potential and a high risk of recurrence. No general treatment guidelines have been established. Fortunately, we were able to minimize immunosuppressant after liver transplantation because of a full HLA‐matched case. There was no recurrence 1 year after treatment.

Background The native liver of patients with maple syrup urine disease (MSUD) (1st recipients) can be used as a graft for non-MSUD patients with end-stage liver disease (2nd recipients). This study aimed to demonstrate the optimal operational procedures and the long-term outcomes of 2nd recipients. Methods Six 2nd recipients of living donor domino liver transplantation (LD-DLT) (age: 42.5 [22-169] months at DLT) received a native liver as a graft from an MSUD patient at our hospital between June 2014 and April 2020. We reviewed the operational procedures and outcomes of 2nd recipients after LD-DLT. Results The 2nd recipients' original diseases included biliary atresia, congenital hepatic fibrosis, congenital protein C deficiency, familial hypercholesterolemia, hepatoblastoma, and mitochondrial hepatopathy. Five of the six recipients had a whole liver and one had a right lobe graft. The site at which the vessels of the MSUD liver were dissected prioritized the safety of the 1st recipient. At the end of follow-up, all recipients were doing well without surgical complications. The mean serum amino acid values of the 2nd recipients did not exceed the upper limit of the reference values during the long-term observation period. All patients showed normal growth while maintaining the same z-score of height and weight after LD-DLT as the preoperative level. Conclusion The liver of patients with MSUD can be used safely without concern regarding long-term complications or de novo MSUD development. LD-DLT using the MSUD liver can expand the donor pool as an alternative graft in pediatric LT.

Background LT is an elective treatment choice for children diagnosed with GSD1b that can improve their quality of life and stabilize their glucose intolerance. However, careful attention should be paid to immunosuppression after LT due to the susceptibility to infection because of neutropenia and neutrophil dysfunction in GSD1b patients. This study revealed the immunological features and complications in the early post-LT period. Methods We compared findings between 11 (1.9%) children with GSD1b and 273 children with BA. Analyses using the PSM were performed to overcome selection bias. Results Despite persistent low tacrolimus trough levels in GSD1b patients, none of these children developed TCMR within 1 month after LDLT (GSD1b: 0/11 [0%] vs. BA: 86/273 [31.5%], p = .038). This result was also confirmed in PSM. The incidence of bloodstream infections was higher in GSD1b patients than in BA patients in the early phase of the post-transplant period (GSD1b: 4/11 [36.4%] vs. BA: 33/273 [12.1%], p = .041), but not reach statistical significance in PSM. In a phenotypic analysis, the ratio of CD8+ T cells in GSD1b recipients' peripheral blood mononuclear cell samples was lower than in recipients with BA through the first month after LDLT. Conclusions We found that GSD1b recipients were more likely to develop postoperative bloodstream infection than recipients with BA but did not experience TCMR despite low tacrolimus levels in the early post-LDLT period. A tailored immunosuppression protocol should be prepared for GSD1b recipients after LDLT.

Aim Mitochondrial respiratory chain disorder (MRCD) can cause acute liver failure (ALF), which may necessitate liver transplantation (LT). However, MRCD is often difficult to diagnose before LT and the indications of LT are controversial due to the likelihood of progressive neurological disease. The present study further characterized the patient population and described the outcomes. Methods Thirteen patients who underwent LT for MRCD from November 2005 to May 2020 were enrolled in this study. Results Six of 13 MRCD patients were diagnosed with a mitochondrial inner membrane protein 17-related mitochondrial DNA depletion syndrome (MTDPS). Overall, nine survived with a median follow-up of 1.8 years (IQR, 1.3-5.1 years); four died within 2 years. In the long-term, seven survivors showed no progression of hypotonia after LT and attended a normal kindergarten or primary school. Neurological abnormalities were observed in two survivors, including vison loss related to Leber's hereditary optic neuropathy in one patient and psychomotor retardation related to Leigh syndrome in the other. Three non-survivors after LT were diagnosed with MTDPS and died of severe pulmonary hypertension, which had developed at 8, 9, and 18 months after LT (n=1 each). The remaining patient died of postoperative respiratory infection with respiratory syncytial virus. Conclusion The long-term results support the performance of LT in patients with MRCD, although a genetic diagnosis is preferable for determining the accurate indications for LT in these patients. Furthermore, care should be taken to avoid complications due to mitochondrial dysfunction during the long-term follow-up.

The differential diagnosis between Alagille syndrome (AGS) with extrahepatic bile duct obstruction (EHBDO) and biliary atresia (BA) is difficult. We report a case series of AGS with EHBDO with detailed validation of the morphological and histopathological features for the differential diagnosis of BA. Six liver transplantations (LTs) were performed for AGS with EHBDO. All patients were diagnosed with BA at the referring institution and the diagnosis of AGS was then confirmed based on a genetic analysis before LT. We verified the morphological and histopathological findings of the porta hepatis and liver at the diagnosis of BA and at LT. All patients had acholic stool in the neonatal period and were diagnosed with BA by cholangiography. The gross liver findings included a smooth and soft surface, without any cirrhosis. The gross findings of the porta hepatis included aplasia of the proximal hepatic duct, or subgroup “o”, in five patients. The histopathological examination of the EHBD also revealed obstruction/absence of the hepatic duct. There were no patients with aplasia of the common bile duct. Aplasia of the hepatic duct and the macroscopic liver findings may help in to differentiate between AGS with EHBDO and BA.

Excessive alcohol consumption has a negative impact on graft survival after liver transplantation (LT). However, it is difficult to predict alcohol relapse before LT. This study surveyed the alcohol consumption of LT recipients to identify the risk factors for harmful drinking. We surveyed the alcohol consumption of LT recipients by using the Alcohol Use Disorders Identification Test-Consumption (AUDIT-C). AUDIT-C scores ≥ 5 points in men and ≥ 4 points in women indicated a high risk for harmful and hazardous drinking. Excessive alcohol consumption was considered to be20 g per day. Ninety-nine LT recipients completely filled out the questionnaire. Alcohol consumption after LT was detected in 26 recipients (26.5%); 4 of them had alcoholic liver disease before transplantation and 22 did not have alcoholic liver disease. The amount of alcohol consumption per day significantly decreased after LT (alcohol consumption per day: 49.6 g before LT vs 8.1 g after LT, P .05). Fourteen recipients (14.1%) consumed alcohol excessively after LT. The AUDIT-C score before LT and smoking were risk factors for excessive alcohol consumption in the multivariate analysis. To properly manage post-transplant recipients, assessing the risk of excessive alcohol consumption by using the AUDIT-C is necessary.

Refractory ascites after liver transplant commonly occurs in living donor liver transplant (LDLT). Refractory ascites is associated with postoperative complications and poor prognosis. This study sought to determine the risk factors of refractory ascites and discuss their perioperative management.A retrospective study of 122 living donor liver transplant recipients between 2008 and 2017 was performed to analyze the risk factors, incidence, and characteristics of refractory ascites. Refractory ascites post LDLT was defined as the production of ascites fluid1000 mL/d on postoperative day 14 or required repeated drainage.A total of 24 patients (19.6%) developed refractory ascites. The 1-year survival rate was significantly worse in the refractory ascites group compared with the nonrefractory ascites group (P .001). In a univariate analysis, patients with refractory ascites had a higher Model for End-Stage Liver Disease (MELD) score, donor age, presence of left lobe graft, ascites at laparotomy, portal venous pressure just after surgery, cold ischemia time, and absence of hepatocellular carcinoma compared with patients without refractory ascites. Multivariate proportional regression analyses revealed that MELD score ≥20, left lobe graft, donor age 50 years or older, and ascites at laparotomy ≥350 mL were independently associated with refractory ascites. Postoperative complications, such as bleeding (P .001), sepsis (P .001), and bloodstream infection within 30 days after LDLT (P .00), were significantly higher in the refractory ascites group.Refractory ascites is associated with reduced 1-year survival and increased postoperative complications. Four factors including MELD score ≥20, donor age 50 years or older, presence of left graft, and ascites at laparotomy ≥350 mL were independent predictors for refractory ascites.

This paper presents an integrated framework to design a flexible multi-stage telecommunication satellite configuration deployment strategy considering the uncertainties in the evolution of the areas of interest over time. The constructed stochastic demand model considers multiple possible scenarios for the evolution of the areas of interest with probabilities based on the market share growth in each area. The optimization aims to find each stage's design with minimum expected lifecycle cost considering all possible scenarios. Each stage of the constellation, assumed to be Flower constellation with circular orbits, provides a regional coverage of the current area of interest as well as additional coverage for the potential future areas of interest. The proposed multi-stage satellite constellation enables the constellation designer to react flexibly and efficiently to the uncertain future expansion of the areas of interest. A case study reveals a reduction in the expected lifecycle cost for an optimized system compared with the all-in-single-stage system and global coverage constellation.

BACKGROUND Fragility fractures caused by osteoporosis are common complications seen in recipients of organ transplantation who survive long term. Although many risk factors have been identified for osteoporosis after organ transplantation, none of them have been recognized as the main cause of development of the condition. Several studies have examined vitamin D receptor (VDR) gene single-nucleotide polymorphisms (SNPs) for their influence on bone mineral density (BMD) and fracture risk, but with variable results. We aimed to elucidate the risk factors that affect incidence of osteoporosis and fragility fractures in liver transplant recipients. MATERIAL AND METHODS In this study, we monitored incidence of fragility fracture and osteoporosis in 45 patients who had been evaluated with dual-energy X-ray absorptiometry (DXA) after liver transplantation. We also analyzed the association between VDR SNPs such as BsmI, ApaI, FokI, and TaqI with osteoporosis and fracture incidence in 27 patients in our cohort in whom SNPs were evaluated and DXA performed after liver transplantation. RESULTS Osteoporosis was diagnosed in 17 of 45 patients in whom BMD was measured after liver transplantation. Of the patients with osteoporosis, 15 (88.2%) subsequently had fragility fractures. The incidence of postoperative osteoporosis was significantly higher in the recipients who had alcoholic liver cirrhosis as their primary disease. Interestingly, there were significantly more patients with a homozygous BsmI GG genotype in the group diagnosed with osteoporosis. CONCLUSIONS Our study suggests that patients who undergo liver transplantation and have alcoholic liver cirrhosis or the BsmI GG genotype may be at increased risk for osteoporosis. Further research is necessary to confirm these findings.

Background EBV-associated HLH driven by EBV-infected CD8+ T cells is a rare complication after pediatric solid organ transplantation. The etiology and disease spectrum of post-transplant EBV-HLH are poorly understood, and making a precise diagnosis and providing optimal treatment remain a challenge. Methods/case description/results We report a 2-year-old multivisceral transplant recipient who developed fever and cytopenia with a persistent high EBV-load state. Repeated tissue examinations and CT scans could not identify a localized mass, which is the key to the diagnosis of PTLD as per the WHO classification. Hence, EBV-HLH was diagnosed by clinical manifestations as well as characterization of EBV-infected cells, pathological examination on cell block of pleural effusion and clonality analysis. This EBV-HLH did not respond to intensive chemotherapy, resulted in the recipient's death, acting similarly to hematological malignancy. Conclusions Characterization of EBV-infected cells in peripheral blood should be considered when persistent high EBV loads develop with symptoms consistent with PTLD, but no evidence of localized mass, and the tissue diagnosis is unavailable after pediatric solid organ transplantation.

While sarcopenia is an important predictor of LT outcomes in adults, few studies have examined the association of sarcopenia with LT outcomes in pediatric patients. We investigated the clinical influence of sarcopenia on the post-transplant outcomes in infants with BA. To define sarcopenia in infants, the cross-sectional area of the tPMA in 93 healthy control infants was measured by computed tomography. Sarcopenia was defined as a tPMA lower than two standard deviations below the mean of healthy control infants. Eighty-nine infants with BA with a median age at LT of 7.6 months old were enrolled. The clinical characteristics and outcomes of LT were verified in the sarcopenia group (n = 21) and non-sarcopenia group (n = 68). The sarcopenia group had a significantly longer operation time and greater blood loss during LT than the non-sarcopenia group (P = .03 and 0.02). The incidence of portal vein stenosis and post-operative bloodstream infection was also significantly higher in the sarcopenia group than in the non-sarcopenia group (23.8% vs 4.4%, P = .02 and 28.6% vs 10.3%, P = .04, respectively). The total length of hospital stay did not differ significantly. The 1-year patient and graft survival rates tended to be lower in the sarcopenia group than in the non-sarcopenia group (90.5% vs 98.5%, P = .07 and 85.7% vs 97.1%, P = .05, respectively). Sarcopenia in infants with BA may be associated with the patient survival and serve as an effective marker for post-operative outcomes of LT.

Surgical intervention for HB with tumor thrombi extending into the IVC and the RA might requires careful planning of the surgical procedures, including vascular reconstruction and extracorporeal circulation. We herein report a successful case of LDLT for HB with atrial extension of a tumor thrombus by extracorporeal circulation with a transdiaphragmatic approach. The patient was a 5-year-old boy with PRETEXT IV HB with a tumor thrombus that extended into the IVC and the RA. After 4 cycles of chemotherapy and resection of bilateral lung metastases, the size of the primary HB tumor decreased. As the tumor extension from the LHV to the RA had decreased but was still present, we performed LDLT with tumor thrombectomy. The central part of the diaphragm was sagittally incised to expose the suprahepatic IVC and the RA. Venovenous bypass was achieved from the right femoral vein and IMV to the RA En bloc resection of the native liver with the tumor thrombus was then performed. HV anastomosis was made between the newly created orifice on the IVC and the graft LHV. The duration of LDLT was 10 hours and 44 minutes (extracorporeal circulation time: 78 minutes). Pediatric LT for HB with the extension of tumor thrombi into the RA under extracorporeal circulation is a feasible option and allows for the expansion of the indications for transplantation for children with unresectable liver tumors.

Sufficient PV flow is necessary to achieve successful PV reconstruction in pediatric LDLT. IOCP can be used to assess the severity of PV stenosis and to identify potential portosystemic collateral pathways. The present study reviewed the utility of IOCP and the outcomes of patients who underwent assessment with an IOCP. Consecutive primary LDLTs were performed in 488 pediatric recipients between November 2005 and October 2019. IOCP was used in patients who were unable to achieve sufficient PV flow after the ligation of collaterals. In total, 11 patients underwent IOCP to assess potential portosystemic collateral pathways. The median age and body weight was 8 months (IQR, 6-11 months) and 6.6 kg (IQR, 5.7-8.9 kg), respectively. The reasons for using the IOCP were recurrent PV thrombus in seven patients and insufficient PV flow in four patients. IOCP revealed remaining collaterals in six patients and residual hypoplastic PV in eight patients. Two patients required additional interruption of the potential collaterals under IOCP, which were unable to be recognized as a dominant portosystemic collateral pathway on preoperative imaging. All eight patients with residual hypoplastic PV required vein graft interposition for the complete removal of the hypoplastic PV. All the patients are currently doing well with a median follow-up period of 4.9 years (IQR, 2.2-5.6 years). IOCP can be an effective tool for precisely detecting occult portosystemic collateral pathways and for assessing the patency of the PV anastomosis in pediatric LDLT.

OBJECTIVES Central perivenulitis can occur in association with T-cell-mediated rejection and can sometimes require strong immunosuppressant therapy as refractory rejection. Furthermore, patients with central perivenulitis are more likely to have subsequent episodes of T-cell-mediated rejection and develop chronic rejection than those without central perivenulitis. We retrospectively analyzed clinical data of pediatric patients with episodes of T-cell-mediated rejection according to severity of central perivenulitis and monitored HLA-DR-positive CD8-positive T cells and recent thymic emigrants during treatment for T-cell-mediated rejection. MATERIALS AND METHODS We identified biopsy-proven T-cell-mediated rejection in 50 liver transplant recipients (45 with living-related donors, 5 with deceased donors) between September 2014 and August 2018. Lymphocyte subsets in peripheral blood samples were analyzed. RESULTS Of 50 pediatric patients, 30 were boys and 20 were girls (median age at transplant of 1.1 y; interquartile range, 0.6-6.2 y). Central perivenulitis was found in 46 patients (92%), which was mild in 13, moderate in 16, and severe in 17. Antithymocyte globulin was more frequently administered to patients with severe central perivenulitis than others (P < .05). Patients with antithymocyte globulin treatment were less likely to have subsequent episodes of T-cell-mediated rejection than those without this treatment (P < .05). The absolute number of CD8-positive HLA-DR-positive T cells in patients during treatment was significantly higher than in control patients (P < .05). The absolute number of recent thymic emigrants in patients with active infection was significantly lower than in patients without infection (P < .05). CONCLUSIONS Our results suggest the efficacy and safety of antithymocyte globulin for treating T-cell-mediated rejection with severe central perivenulitis in pediatric liver transplant recipients and suggest that antithymocyte globulin can prevent subsequent episodes of T-cell-mediated rejection. Analyzing lymphocyte subsets during treatment for rejection may help highlight viable therapeutic strategies for achieving a good outcome.

Background Because immunosuppression is necessary for kidney transplant recipients, malignant tumorigenesis of recipient organs is a concern; however, few studies have discussed the malignant alteration of transplanted grafts that have been functional for a long time. In addition, a urothelial carcinoma (UC) in transplanted kidney graft is a rare disease. Case report A 62-year-old man had end-stage renal failure 31 years ago and received a kidney transplant from his father. Acute renal failure due to obstruction of the transplanted ureter was diagnosed. Ultrasound, ureterogram, and non-enhanced computed tomography scans revealed no obvious evidence of any neoplastic lesion. We treated the obstruction and hydronephrosis with transplant ureter stenting. However, the regional lymph nodes enlarged, and it became necessary to change the ureteral stent frequently because of stent stenosis; therefore, he underwent lower transplant ureteral resection and reconstruction. Histopathology confirmed a UC with a flat and infiltrative growth pattern. The patient then underwent graftectomy including right external iliac vein resection and reconstruction; however, because of numerous metastatic nodules, radical surgery could not be performed. The patient subsequently died because of septic shock after the second surgery. Conclusion We report a case of an invasive UC with a flat and infiltrative growth pattern derived from a transplant kidney graft that occurred 31 years after a living-donor transplant that could not be treated immediately and was difficult to diagnose.