大西 康晴

BACKGROUND: The usefulness of subcutaneous immunoglobulin (SCIG) for the treatment of hypogammaglobulinemia has been reported, but there are no reports in the field of pediatric liver transplantation (LT). We herein report the therapeutic efficacy of SCIG in the postoperative management after pediatric living donor LT (LDLT). METHODS: Subjects were 112 pediatric recipients who underwent LDLT between March 2012 and December 2021. SCIG administration was started in February 2017 and performed in 43 patients with hypogammaglobulinemia (< 870 mg/dL). Intravenous immunoglobulin (IVIG) administration was performed in 69 patients before January 2017. SCIG was administered subcutaneously at 130 (82-238) mg/kg/dose every week from postoperative day (POD) 2 until discharge. RESULTS: The preoperative serum IgG level in the SCIG group was 906 (249-1987) mg/dL, and the IgG level at the end of LDLT was 491 (246-823) mg/dL, showing a significant difference (p < 0.001). The median IgG levels in the SCIG group after LT were 697, 607, 579, 691, 665, and 795 mg/dL at 1, 2, 3, 4, and 5 weeks after surgery and after discharge, respectively. The incidence of bacteremia was significantly lower in the SCIG group than in the IVIG group (p = 0.025). The recipient's survival rate was not significantly different between the SCIG and IVIG groups (p = 0.080), but the recipient's survival rate in the SCIG group was 100%. The multivariate analysis revealed that the IVIG group and CMV viremia were a significant risk factors for bacteremia (p = 0.023 and 0.001, respectively). CONCLUSIONS: Postoperative SCIG administration effectively maintained serum IgG levels and was useful for preventing bacteremia.

While endogenous cortisol secretion rises in the early morning, the number of lymphocytes in the blood is higher at night, thus exhibiting an antiphase pattern to cortisol secretion. Therefore, compared with the daytime, the infiltration of lymphocytes into immune-reactive tissues is enhanced at night. This study aimed to determine whether the administration of methylprednisolone (mPSL) in the evening is more effective against T cell-mediated rejection (TCMR) after liver transplantation compared with morning administration. This study used a randomized, open-label, parallel-group comparison design. Pediatric patients scheduled to undergo living-donor liver transplantation were randomly divided into morning (8:00 a.m.) and evening (8:00 p.m.) mPSL administration groups. The primary endpoint was the occurrence of TCMR within 14 days of surgery. Sixty-two patients were enrolled between 2014 and 2023, and six patients were excluded from the analysis as their dose of mPSL deviated from the protocol within 14 days after surgery. Of the 56 subjects analyzed, TCMR was detected in 10 of the morning group (n = 29) and three of the evening group (n = 27) within 14 days after surgery. Stratified analysis of patients who did not receive preoperative rituximab treatment showed that none of the evening group and 36.4% of the morning group developed TCMR within 14 days after surgery (P < 0.01, 95% confidence interval; 2.00-infinity). Safety evaluation results were comparable between the two groups. This study shows that the evening administration of mPSL is an effective approach for suppressing TCMR. This study is hypothesis generating, and replication in further studies is needed.

BACKGROUND: Cytomegalovirus (CMV) is a major infectious complication in solid-organ transplant recipients, particularly in the context of pediatric liver transplantation. CMV serostatus is a well-established risk factor for postoperative CMV infection, with CMV seronegative recipients who receive organs from seropositive donors (D+/R-) being at the highest risk. Our previous research indicated a higher incidence of CMV infection in recipients with inherited metabolic diseases (IMDs) compared with those with biliary atresia (BA). This study aimed to determine whether IMDs constitute an independent risk factor for postoperative CMV infection. METHODS: We retrospectively analyzed data from 45 IMD and 230 BA recipients. We collected information on the occurrence and timing of episodes of CMV infections, methylprednisolone (mPSL) pulse therapy, patient characteristics, and peri- and postoperative data. RESULTS: Multivariable analysis identified mPSL pulse therapy (Odds Ratio (OR): 4.43), CMV serostatus (D+/R-) (OR: 6.03), and underlying IMDs (OR: 3.28) as independent risk factors for CMV infection. Further stratified analysis, which considered the timing of CMV infection diagnosis relative to mPSL pulse therapy, confirmed that CMV serostatus with (D+/R-) (OR: 5.61) and underlying IMDs (OR: 2.83) remained independent predictors of CMV infection, even when excluding the influence of mPSL pulse therapy. CONCLUSIONS: This study demonstrates that IMDs are a potent independent risk factor for CMV infection following pediatric liver transplantation. CLINICAL TRIAL NUMBER: Not applicable.

INTRODUCTION: When a thrombus extends to the suprahepatic inferior vena cava (IVC) in patients with Budd-Chiari syndrome (BCS) requiring liver transplantation (LT), there is a risk of thrombus migration during hepatectomy that can potentially lead to pulmonary embolism. Intraoperative pulmonary embolism can be life-threatening and may necessitate urgent thrombectomy. However, preventive strategies for pulmonary embolism during LT in BCS cases with IVC thrombosis have seldom been discussed in the literature. We report a case involving a 51-year-old woman with BCS complicated by thrombi extending into the suprahepatic IVC who underwent deceased donor LT (DDLT) for acute liver failure (ALF). CASE PRESENTATION: A previously healthy 51-year-old woman with ALF secondary to BCS was admitted to our hospital. 19 days back, BCS was diagnosed at another hospital, where computed tomography revealed thrombi in the hepatic veins and IVC. She subsequently developed grade II hepatic encephalopathy and severe liver dysfunction. Conservative treatment was ineffective, and 4 days before the current admission, she experienced grade III hepatic encephalopathy and showed hepatofugal portal flow on ultrasonography. DDLT was performed on day 13 after admission. Median sternotomy was performed to clamp the suprahepatic IVC near the right atrium, mitigating the risk of thrombus migration during hepatectomy and allowing for urgent thrombectomy in case of pulmonary embolism. Additionally, because a large-for-size graft was being used, the median sternotomy enhanced visibility and provided adequate space, facilitating suprahepatic IVC anastomosis. Postoperatively, the patient experienced no complications related to the sternotomy and was discharged 58 days after surgery. CONCLUSIONS: This case report highlights the potential utility of median sternotomy during LT for BCS, particularly for cases with concerns regarding thrombus migration from the suprahepatic IVC, the need for rapid thrombectomy in the event of pulmonary embolism, and anticipated challenges in suprahepatic IVC anastomosis due to large-for-size grafts.

Portal cavernoma cholangiopathy (PCC) is a complex condition associated with portal hypertension, particularly in patients with extrahepatic portal vein obstruction (EHPVO). Herein, we present a case of liver failure with PCC in a 55-year-old male successfully treated with living-donor liver transplantation (LDLT). The patient had a history of gastrointestinal bleeding and recurrence of cholangitis. Imaging studies confirmed cavernous transformation and pericholedochal varices. Preoperative angiography verified hepatopetal flow in the pericholedochal varix, which facilitated successful anastomosis with the donor's portal vein during LDLT. Histological examination of the explanted liver confirmed vanishing bile duct syndrome (VBDS) and secondary bile stasis was considered to have caused liver failure. No postoperative complications were observed within 13 months of LDLT. We report the first case of VBDS in the PCC resulting from EHPVO that was successfully managed with LDLT. Careful management of similar cases should involve angiography and long-term postoperative monitoring of portal vein complications.