BACKGROUND:The use of laparoscopic procedures for liver transplantation (LT) remains controversial. This study aimed to present our preliminary experience with a minimally invasive pediatric living donor liver transplantation (LDLT). METHODS:The medical records of pediatric patients who underwent laparoscopy-assisted LDLT, namely laparoscopic mobilization of the diseased liver followed by open explant hepatectomy and graft implantation using an upper midline incision at Beijing Friendship Hospital between November 2019 and March 2023, were retrospectively reviewed, focusing on demographics and pre-, intra-, and postoperative outcomes. RESULTS:Laparoscopy-assisted LDLT was successfully performed in all 13 pediatric patients. The mean total operative time was 478 min (range, 315-615 min), the median portal vein clamping time was 47 min (range, 33-107 min), the average time required to remove the liver was 229 min (range, 115-357 min), and the median cold ischemia time was 85 min (range, 29-214 min). The intraoperative course for all the recipients was uneventful. All patients recovered well without any significant acute postoperative problems, with only one patient presenting with hepatic artery thrombosis on postoperative day 1 who made a good recovery after immediate thrombectomy and re-anastomosis. During a median follow-up of 41.8 (range, 24.3-64.3 months), all recipients survived with 100% graft survival. CONCLUSION:This is the world's largest single-center cohort study of pediatric laparoscopy-assisted LDLT using an upper midline incision. Our results provide preliminary evidence for the safety and feasibility of minimally invasive LDLT in selected pediatric patients, which may be a reasonably compromised strategy before attempting pure laparoscopic LT. TRIAL REGISTRATION:Chinese Clinical Trial Registry Identifier: ChiCTR2400086319.

Background: Portal vein (PV) thrombosis (PVT) adds to the surgical complexity and complications of liver transplant (LT). We aimed to identify predictors for unsatisfactory thrombectomy (UST) in pre-LT occlusive PVT, and to demonstrate our experience in non-anatomical PV reconstruction. Methods: A registry-based cohort of adult LT recipients was established. PVT was diagnosed with pre-LT ultrasound and/or iodine-based contrast-enhanced computed tomography imaging, and backup plan of nonanatomical PV reconstruction was preemptively designed accordingly for all patients. Thrombectomy was considered unsatisfactory when recipient PV failed to spurt at unclamping, and the reconstruction should achieve a peak velocity >20 cm/s by Doppler ultrasound in right PV. Results: Among 570 candidates from 2016 to 2024, 45 with pre-LT occlusive PVT were enrolled. Satisfactory thrombectomy and anatomical PV reconstruction were accomplished in 33 (73%) subjects, and 11 of them had major portosystemic shunts ligated to optimize PV blood flow. The rest 12 (27%) subjects were unsatisfactorily thrombectomized, who received porto-variceal anastomosis (5/12), portocaval hemitransposition (5/12), or portocaval transposition (2/12) for non-anatomical PV reconstruction. Compared with the others, unsatisfactorily thrombectomized patients featured more PVT calcification, higher grades of thrombosis in superior mesenteric (SMV) and splenic vein, more extra graft vessels, but comparable anhepatic period. PVT calcification, SMV thrombosis, and upper gastrointestinal bleeding (UGIB) history were identified as independent predictors for UST by logistic regression. Despite preventive anticoagulation in 33 (73%) subjects, 9 (20%) subjects developed PV restenosis/rethrombosis events in the first postoperative year, and all but one cases were managed with interventional radiology. The overall survival (1-year rate 86.3%) and PV patency (1-year rate 76.9%) were comparable between satisfactorily and unsatisfactorily thrombectomized patients, and no significant prognostic differences were observed across patients of different pre-LT PVT gradings (P>0.05). Conclusions: UST for pre-LT occlusive PVT could be forecast with PVT calcification, SMV thrombosis, and UGIB history, and the 1st year post-LT should be closely monitored for restenosis/rethrombosis. With comprehensive preoperative design and careful intraoperative management, non-anatomical PV reconstruction could achieve a favorable outcome comparable to that of anatomical PV reconstruction.

BACKGROUND:Neonatal brain injury due to bilirubin toxicity presents critical need for effective healing treatments. Docosahexaenoic acid (DHA), having neuroprotective properties, offers potential therapeutic benefits in promoting brain repair and recovery. OBJECTIVES:This study focused on evaluating the healing capabilities of DHA in neonatal brains damaged by bilirubin-induced injury, with particular attention to its role in enhancing brain tissue repair mechanisms. METHODS:Employing the bilirubin encephalopathy model in neonatal Sprague-Dawley rats and neuronal cell cultures, we investigated the therapeutic impact of DHA. RESULTS:The study measured improvements in brain tissue integrity, assessed bilirubin levels, analyzed gene and protein expressions pertinent to the brain's recovery process. DHA administration resulted in significant repair in neonatal brains, evidenced by reduction in bilirubin levels and restoration of normal brain tissue architecture. CONCLUSION:Molecular analysis indicated the distinct modulation of the CTBP1/miR-155-5p/KDM5A pathway, critical for cellular repair processes and marked decrease in markers of cellular damage and stress.

BACKGROUND:Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) syndrome is a rare urea cycle disorder caused by mutations in the SLC25A15 gene, leading to metabolic and neurological impairments. Liver transplantation (LT) may restore urea cycle function and prevent disease progression. METHODS:This retrospective study analyzed six patients with HHH syndrome who underwent LT between 2016 and 2022. Pre- and post-transplant evaluations included biochemical tests, genetic analysis, neurological assessments, and quality-of-life measures. RESULTS:LT successfully normalized metabolic parameters (ammonia and amino acid levels) and allowed patients to resume normal diets. Early transplantation resulted in neurological improvement in 5 of 6 patients (83.3%), including reduced lower limb spasticity and improved walking ability. Two patients (33.3%) achieved nearly normal gait, and one patient (16.7%) recovered to normal motor function within three months after LT. Quality-of-life scores improved in 2 patients (33.3%). The overall survival rate was 83.3%, with one patient dying from unrelated causes 5 years post-transplant. No significant long-term complications were observed in the surviving patients. CONCLUSIONS:Liver transplantation is an effective treatment for HHH syndrome, halting neurological decline and improving quality of life. Early LT before irreversible damage provides the best outcomes, making it a viable option for patients with progressive symptoms unresponsive to conventional therapies. CLINICAL TRIAL NUMBER:Not applicable.

Abstract Background Homozygous familial hypercholesterolaemia (HoFH) increases risk of premature cardiovascular events and cardiac death. In severe cases of HoFH, clinical signs and symptoms cannot be controlled well by non-surgical treatments, liver transplantation (LT) currently represents the viable option. Method To assess the clinical efficacy, prognosis, and optimal timing of LT for HoFH, a retrospective analysis was conducted on the preoperative, surgical conditions, and postoperative follow-up of children who received an LT for HoFH at the Beijing Friendship Hospital over the period from December 2014 to August 2022. Results Xanthoma and decreased activity tolerance were the primary clinical manifestations in the 7 HoFH children initially assessed (one child died suddenly prior to surgery due to cardiac arrest). Accompanying these symptoms were increased blood total cholesterol (TC) and low density lipoprotein (LDL) levels, along with severe cardiovascular diseases. HoFH was confirmed in all cases by genetic and biochemical assays. Initial treatments administered to these patients consisted of low-fat diets and lipid-lowering drugs with poor outcomes. Accordingly, all 6 patients received orthotopic liver transplantations (OLT), with the result that significant postoperative reductions were observed in levels of TC and LDL. The median follow-up of these six cases was 37.41 months (range: 19.40–94.10 months). Regular postoperative follow-ups revealed that all survived and showed significant improvements in their clinical symptoms. Conclusion So far, LT is the only way to heal HoFH. LT before the appearance of obvious cardiovascular atherosclerotic lesions can significantly improve the quality of life and prognosis of patients. At the same time, the blood cholesterol level of patients should be continuously monitored after LT to further control the progression of vascular complications.

Liver transplantation (LT) has been proposed as a viable treatment option for selected methylmalonic acidemia (MMA) patients. However, there are still controversies regarding the therapeutic value of LT for MMA. The systematic assessment of health-related quality of life (HRQoL)-targeted MMA children before and after LT is also undetermined. This study aimed to comprehensively assess the long-term impact of LT on MMA, including multiorgan sequelae and HRQoL in children and families. We retrospectively evaluated 15 isolated MMA patients undergoing LT at our institution between June 2013 and March 2022. Pre- and post-transplant data were compared, including metabolic profiles, neurologic consequences, growth parameters, and HRQoL. To further assess the characteristics of the HRQoL outcomes in MMA, we compared the results with those of children with biliary atresia (BA). All patients had early onset MMA, and underwent LT at a mean age of 4.3 years. During 1.3–8.2 years of follow-up, the patient and graft survival rates were 100

Introduction: Propionic acidemia (PA) is a rare mitochondrial metabolic disorder involving multiple organs throughout the body. Since adherence to recommended conservative metabolic management cannot reliably prevent disease progression, liver transplantation (LT) is emerging as a viable alternative therapeutic option in selected PA patients. However, therapeutic outcomes of LT for PA associated cardiomyopathy are rarely reported. Method: A thorough review of the current published literature was performed to evaluate outcomes of LT for patients with PA-related cardiomyopathy. Clinical, biochemical, and neurophysiological outcomes were compared with the 2-year-old female child managed at our institution. Results: The pretransplant echocardiogram showed left ventricular dilatation and systolic dysfunction, and thus dilated cardiomyopathy was considered. A living donor liver transplant was performed using the mother’s left lateral lobe. On the 35.8 months postoperatively, the child was on a liberated protein diet, but still required levocarnitine supplementation. The hepatic and cardiac function were both normal, but growth retardation was still present. During the follow-up period, there were no further propionic acidemia-related complications, such as metabolic decompensation, or any transplant-related complications. Published literature revealed that the cardioprotective potential of LT for individuals with PA has been proved by the fact that reversal of cardiomyopathy was achieved in all previously reported 11 pediatric patients with pre-existing PA-associated cardiomyopathy. In line with previous results, our patient with mild dilated cardiomyopathy also displayed a complete recovery of cardiac function after LT. Conclusion: This case report and systematic review demonstrates that LT can successfully treat patients with PA-related cardiomyopathy, which relieves strict protein restriction, provides systemic metabolic stability, improves quality of life, and reverses cardiomyopathy. Nevertheless, recipients with PA remain at risk of developing PA-related complications including cardiomyopathy.

BackgroundIn living donor liver transplantation (LDLT), graft-to-recipient weight ratio (GRWR) <0. 8% is an important index for predicted portal hypertension, which may induce the graft small-for-size syndrome (SFSS). Recently, the value of graft-to-spleen volume ratio (GSVR) on predicted portal hypertension had been reported, whether without splenectomy prevent portal hypertension in transplantation remains disputed, we aimed to identify GSVR contributing to portal venous pressure (PVP) and outcomes without simultaneous splenectomy in LDLT.MethodsA retrospective study had been designed. Excluded patients with splenectomy, 246 recipients with LDLT between 2016 and 2020 were categorized into a low GSVR group and a normal GSVR group. Preoperative, intraoperative, and postoperative data were collected, then we explored different GSVR values contributing to portal hypertension after reperfusion.ResultsAccording to the first quartile of the distributed data, two groups were divided: low GSVR (<1.03 g/mL) and normal GSVR (>1.03 g/mL). For the donors, there were significant differences in donor age, graft type, liver size, GRWR, and GSVR (P < 0.05). Following the surgical factors, there were significant differences in blood loss and CRBC transfusion (P < 0.05). The low GSVR has demonstrated had a significant relationship with ascites drainage and portal venous flow after LDLT (P < 0.05). Meanwhile, low GSVR heralds worse results which covered platelet count, international normalized ratio (INR), and portal venous velocity. Kaplan–Meier analysis showed that there was a significant difference between the two groups, while the low GSVR group demonstrated worse recipients survival compared with the normal GSVR group (P < 0.05).ConclusionsWithout splenectomy, low GSVR was an important predictor of portal hypertension and impaired graft function after LDLT.

BACKGROUND:Simultaneous splenectomy during liver transplantation is indicated for patients with cirrhosis complicated by severe hypersplenism, but disastrous procedure-related complications remain a special concern. Simultaneous partial splenectomy was adopted in pediatric liver transplant recipients with severe hypersplenism-related pancytopenia at our institution.METHODS:A prospective, single-center analysis of 21 pediatric patients diagnosed with cirrhosis and severe hypersplenism, who underwent liver transplantation between January 2015 to December 2019, was conducted. Patient characteristics, intraoperative parameters, and postoperative outcomes were compared between patients with simultaneous partial splenectomy (n = 13) and those without (n = 8).RESULTS:Simultaneous partial splenectomy significantly increased platelet and leukocyte counts in the early postoperative period, without increasing operative time, intraoperative blood loss and postoperative hospital stay (P = 0.64, P = 0.44, P = 0.26, respectively). No significant differences were observed between the two groups regarding the incidence of postoperative hemorrhage (P = 0.38), pneumonia (P = 0.33), cholangitis (P = 0.38), thrombotic complications (P = 1.00), cytomegalovirus infection (P = 0.53), Epstein-Barr virus infection (P = 0.20) and acute rejection (P = 0.26).CONCLUSION:Simultaneous partial splenectomy during liver transplantation could serve as a feasible alternative to splenectomy in selected patients with severe hypersplenism, which can achieve a satisfactory long-term hematological response, but avoid untoward complications of splenectomy.

Background Argininemia is a rare disease caused by inborn errors of metabolism. Advances in diagnosis and treatment have increased the number of patients receiving effective management. Argininemia is characterized by progressive spastic paraplegia, mental retardation, failure to thrive and irritability, and patients with coagulopathy are paid more attention. We studied the changes in coagulation dysfunctions and neurodevelopment in patients with argininemia before and after liver transplantation(LT). Methods The data in this study were obtained from the Liver Transplantation Center of Beijing Friendship Hospital, Capital Medical University between January 2015 and November 2019. We identified data related to argininemia in all patients diagnosed with urea cycle disorder, and extracted the details on coagulation, liver function, neurodevelopmental outcomes, histopathological and morphological examination, and other clinical presentations. The patients were followed up by telephone and/or in the clinic. Results Nine patients with argininemia diagnosed by tandem mass spectrometry and symptoms, and confirmed by gene sequencing. The symptoms deteriorated after dietary restriction in all patients with argininemia. Coagulopathy manifested before surgery, and no significant correlation was detected with plasma ammonia concentration. The coagulation dysfunction was completely resolved and progressive neurological impairment was prevented in seven patients with LT. Conclusions Coagulation dysfunctions are common manifestations of patients with

Background Nowadays, laparoscopic left lateral sectionectomy has been acknowledged as a standard practice in pediatric living donor liver transplantation (PLDLT).(1, 2) We here report the first case of laparoscopic left lateral monosegmentectomy (L-LLM) in PLDLT using real-time ICG fluorescence in situ reduction in China. Method A 35-year-old father volunteered for living donation to his daughter who diagnosed with liver cirrhosis and portal hypertension after Kasai operation due to biliary atresia. Preoperative liver function was normal. Liver dynamic CT showed a left lateral graft volume of 387.5cm(3) with a graft to recipient weight ratio (GRWR) of 4.45%. Ratio of the maximum thickness of the left lateral segment to the anteroposterior diameter of the recipient's abdominal cavity was 1.09. The estimated segment II volume was 245.3cm(3) and GRWR was 2.82%. L-LLM was scheduled.(3) No anatomic variation was seen. Results The transection was divided into two stages. Stage I: Separating the left lateral section along the right side of sickle ligament. Stage II: Anatomic in situ reduction of segment III by using real-time ICG fluorescence. The left bile duct was transected above the bifurcation by ICG fluorescence cholangiography. The total operation time was 200 min without transfusion. The final graft weight was 225.2 g with GRWR of 2.59%. The donor was discharged uneventfully on postoperative day 4, while the graft function recovered to normal in recipient without any graft-related complication. Conclusion L-LLM with in situ reduction is feasible in PLDLT by using real-time ICG fluorescence in experienced transplant center.

To The Editor: Bariatric surgery, as an effective treatment for performed, and thick veins were directly ligated. The start patients with morbid obesity and its related metabolic point of the gastric resection was 6 cm away from the diseases, is gaining increasing popularity.Non-alcoholic fatty liver disease (NAFLD) is a common comorbidity associated withmorbidobesity.Growing evidence suggests thatpatients with non-alcoholic steatohepatitis are at high-risk of adverse outcomes such as cirrhosis and liver-related mortality. Liver function failure caused byNAFLD is predicted to become the most common reason for liver transplantation (LT) in the United States by 2025. The estimated prevalence of obesity is 20% to 30% in LT recipients in the United States. LT is the only radical treatment for end-stage liver disease. If patients with end-stage liver disease and morbid obesity cannot effectively control their body weight after LT, the donor liver will be at high-risk of NAFLD again. Simultaneous LT and sleeve gastrectomy (SG) may be performed for effectively controlling post-operative body weight and metabolic disorders in these patients. We present a case of simultaneousLTandSG for a patientwith end-stage liver disease and morbid obesity.