Favorable Outcomes of Liver Transplantation for Hepatopulmonary Syndrome.

BACKGROUND: Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of chronic liver disease, which develops insidiously as a result of chronic liver disease. The prognosis for untreated patients with HPS is extremely poor, and liver transplantation (LT) serves as the only effective means for treating this condition. Here, we performed a retrospective analysis to evaluate the efficacy of LT on the survival and long-term prognosis of patients with HPS. METHODS: Clinical data, including survival and postoperative efficacy, from patients with HPS from records as obtained over the period from January 1 to December 31, 2022. All records were from a waiting list for LT at the Beijing Friendship Hospital Affiliated with Capital Medical University. RESULTS: Among the 274 patients on the LT waiting list, 37 were diagnosed with HPS (13.50%) and were enrolled. Survival rates of patients with HPS receiving an LT were greater, whereas a statistically significant difference was obtained between patients with LT vs non-LT with moderate to severe HPS (P = .003). The overall time until death without LT was 4-72 days after their initial HPS diagnosis. Patients with HPS receiving an LT showed a significant improvement in the state of oxygenation after surgery (P = .001). CONCLUSION: Comprehensive preoperative screening of patients on the waiting list for LT is critical to identify those patients with HPS who would maximally benefit from LT. Survival rates of patients with moderate to severe HPS are significantly increased after LT, a procedure that should be performed as soon as possible in these patients with HPS.

Long-term clinical outcomes and health-related quality of life in patients with isolated methylmalonic acidemia after liver transplantation: experience from the largest cohort study in China.

BACKGROUND: 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. METHODS: 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). RESULTS: 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%. Metabolic stability was achieved in all patients with liberalized dietary protein intake. There was a significant overall improvement in height Z scores (P = 0.0047), and some preexisting neurological complications remained stable or even improved after LT. On the Pediatric Quality of Life Inventory (PedsQL™) generic core scales, the mean total, physical health, and psychosocial health scores improved significantly posttransplant (P < 0.05). In the family impact module, higher mean scores were noted for all subscales post-LT, especially family function and daily activities (P < 0.01). However, the total scores on the generic core scales and transplant module were significantly lower (Cohen's d = 0.57-1.17) when compared with BA recipients. In particular, social and school functioning (Cohen's d = 0.86-1.76), treatment anxiety, and communication (Cohen's d = 0.99-1.81) were far behind, with a large effect size. CONCLUSIONS: This large single-center study of the mainland of China showed an overall favorable impact of LT on isolated MMA in terms of long-term survival, metabolic control, and HRQoL in children and families. The potential for persistent neurocognitive impairment and inherent metabolic fragility requires long-term special care. Video Abstract (MP4 153780 KB).

Impact of immunosuppression level on liver allograft fibrosis after pediatric liver transplantation: a retrospective cohort study.

BACKGROUND: Liver allograft fibrosis (LAF) is prevalent among children with long-term survival after liver transplantation (LT). The authors aimed to identify clinical risk factors, with a focus on the impact of immunosuppression (IS) level in the early post-transplant period on LAF. METHODS: A retrospective study was conducted on pediatric LT recipients with at least 1-year of follow-up. Cox regression models were used to analyze risk factors associated with LAF, and landmark analysis was used to evaluate the impact of IS level on LAF. Longitudinal analysis was also conducted in patients with paired biopsies. RESULTS: A total of 139 patients involving 174 liver biopsies were included. With 2.3 to 5.9 years of follow-up, LAF was detected in 91.4% of patients (7.9% were significant), up to 88.2% of whom showed normal liver function. Episodes of acute rejection, biliary complications, cytomegalovirus infection, and prolonged cold ischemia time were independent risk factors. Besides, the risk of LAF in patients with relatively low IS levels at postoperative 1-3, 3-6, 6-12, and 12-36 months was higher than the counterparts. Especially, in patients with relatively high IS levels (mean tacrolimus trough concentration ≥5.1 ng/ml) during postoperative 12-36 months, the risk of LAF was 67% lower in the short future ( P =0.006). In paired analysis, patients with increased IS levels were more likely to achieve fibrosis-reduction (HR=7.53, P =0.025). CONCLUSIONS: Mild to moderate LAF is common among pediatric LT recipients and can appear early and silently. Maintaining adequate levels of IS during 1-3 years after LT seems crucial to ensure protection against LAF.

Impact of pure laparoscopic surgery on bile duct division of living donor left lateral section procurement.

Background: Although laparoscopic living donor left lateral section liver procurements represents an established and safe procedure, there remains much discussion on this topic. In particular, the issue of whether laparoscopic living donor liver procurement increases the difficulty of the surgery and potential complications for recipients continue to confound experts in this field. Methods: In this report, data from 180 cases of living donor left lateral section liver transplantation patients were analyzed retrospectively. Of these 180 cases, 106 grafts were procured by open surgery and 74 by pure laparoscopic surgery. Results: While surgery durations and blood loss were decreased in donors from the laparoscopic surgery group, increased biliary openings of grafts and relatively high peak aspartate aminotransferase (AST) levels were present in both donors and recipients with this procedure. Conclusions: Laparoscopic living donor left lateral section liver procurement represents a safe and effective procedure for both donors and recipients. However, laparoscopic surgery can more frequently lead to multiple biliary tracts in the graft and its impact on the prognosis of recipients remains uncertain. Use of routine X-ray based intraoperative cholangiography may help to reduce this problem.

Outcomes from a Single Transplant Center of 5 Pediatric Cases of Domino Liver Transplantation from Live Donors with Maple Syrup Urine Disease.

BACKGROUND Maple syrup urine disease (MSUD) is a rare genetic deficiency of the branched-chain alpha-keto acid dehydrogenase (BCKAD) complex that breaks down amino acids, resulting in multi-organ failure. This report is of 5 pediatric cases of domino liver transplantation (DLT) from live donors with MSUD from a single transplant center in Beijing. CASE REPORT All MSUD donors were confirmed to have disease-causing mutations in BCKDHA (branched-chain keto acid dehydrogenase E1, alpha polypeptide) or BCKDHB (branched-chain keto acid dehydrogenase E1, ß polypeptide) genes by peripheral blood whole-exon sequencing. Serum leucine and valine concentrations were significantly higher than normal values. Recipients ranged in age from 0.75 to 9 years old. Three patients underwent auxiliary liver transplantation, and the other children all underwent liver or partial liver transplantation. This case report was followed up for 25 to 79 months. The prognosis, growth, and development of patients were followed up. By the end of the last follow-up, all children had survived. All patients had normal serum leucine and valine concentrations after surgery. In case 1, portal vein stenosis post-operatively. In case 2, stenosis of hepatic artery and bile duct occurred. In case 5, hepatic artery and portal vein stenosis occurred, resulting in graft loss.   CONCLUSIONS The findings from our center support the findings from other pediatric liver transplant centers that liver transplantation using MSUD donors can have successful outcomes without the development of MSUD in the recipient.

A novel approach of intraoperative cholangiography in laparoscopic left lateral sectionectomy in living donor liver transplantation.

BACKGROUND: Accurate division of bile duct during laparoscopic donor hepatectomy in living donor liver transplantation is essential. We here present a novel approach to achieve cholangiography via the bile duct stump of segment IV (B4 stump) during laparoscopic donor hepatectomy in adult-to-pediatric living donor liver transplantation. PATIENTS AND METHODS: Donors who underwent laparoscopic left lateral sectionectomy (LLLS) from January 2022 to April 2022 in our liver transplant center were retrospectively analyzed. A total of 32 donors were eventually enrolled into this study. Cholangiography via the B4 stump was performed in 11 donors (B4 group) while indocyanine green (ICG) fluorescence guiding was performed in 21 donors (ICG group). Perioperative data were collected and compared between groups. RESULTS: Cholangiography by catheterizing the B4 stump was successfully performed in all 11 donors in the B4 group. The mean time of this procedure was 12.82 ± 9.11 min. Compared to the ICG group, it was more likely to acquire single bile duct orifice on graft in the B4 group (B4: 10/11, 90.91% vs ICG: 9/21, 42.86%) and it was significantly different (p = 0.030). The donors' complications (Clavien-Dindo grade III-IV) were not significantly different. There was one donor developed intraperitoneal effusion in B4 group, while two donors (one bile leakage and one biliary stricture) developed biliary tract related complications in the ICG group. A Roux-en-Y was performed to solve the biliary stricture in the ICG group. The recipients' outcomes were not significantly different between groups. CONCLUSIONS: Cholangiography via the B4 stump catheterization is feasible and safe in identifying the bifurcation of bile duct during LLLS.

Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disease stemming from a deficiency in liver-specific alanine-glyoxylate aminotransferase, resulting in increased endogenous oxalate deposition and end-stage renal disease. Organ transplantation is the only effective treatment. However, its approach and timing remain controversial. CASE SUMMARY: We retrospectively analyzed 5 patients diagnosed with PH1 from the Liver Transplant Center of the Beijing Friendship Hospital from March 2017 to December 2020. Our cohort included 4 males and 1 female. The median age at onset was 4.0 years (range: 1.0-5.0), age at diagnosis was 12.2 years (range: 6.7-23.5), age at liver transplantation (LT) was 12.2 years (range: 7.0-25.1), and the follow-up time was 26.3 mo (range: 12.8-40.1). All patients had delayed diagnosis, and 3 patients had progressed to end-stage renal disease by the time they were diagnosed. Two patients received preemptive LT; their estimated glomerular filtration rate was maintained at > 120 mL/min/1.73 m2, indicating a better prognosis. Three patients received sequential liver and kidney transplantation. After transplantation, serum and urinary oxalate decreased, and liver function recovered. At the last follow-up, the estimated glomerular filtration rates of the latter 3 patients were 179, 52 and 21 mL/min/1.73 m2. CONCLUSION: Different transplantation strategies should be adopted for patients based on their renal function stage. Preemptive-LT offers a good therapeutic approach for PH1.

Laparoscopic Anatomic Segment III Procurement in Pediatric Living Donor Liver Transplantation Using Real-Time ICG Fluorescence In Situ Reduction by the Glissonean Approach.

BACKGROUND: Nowadays, anatomic hepatectomy has been widely accepted and acknowledged as a feasible practice during laparoscopic procedure. We herein report the first case of laparoscopic anatomic segment III (S3) procurement in pediatric living donor liver transplantation using real-time indocyanine green (ICG) fluorescence in situ reduction by Glissonean approach. METHODS: A 36-year-old father volunteered for living donation to his daughter who was diagnosed with liver cirrhosis and portal hypertension due to biliary atresia. Preoperative liver function was normal with mild fatty liver. Liver dynamic computed tomography showed a left lateral graft volume of 379.43 cm3 with a graft to recipient weight ratio (GRWR) of 4.77%. The ratio of the maximum thickness of the left lateral segment to the anteroposterior diameter of the recipient's abdominal cavity was 1.20. Hepatic veins of segment II (S2) and S3 separately flowed into the middle hepatic vein. The estimated S3 volume was 173.16 cm3 and GRWR was 2.18%. The estimated S2 volume was 118.54 cm3 and GRWR was 1.49%. Laparoscopic anatomic S3 procurement was scheduled. RESULTS: Liver parenchyma transection was divided into 2 steps. Step I: Anatomic in situ reduction of S2 by using real-time ICG fluorescence. Step II: Separating the S3 along the right side of sickle ligament. The left bile duct was identified and divided by ICG fluorescence cholangiography. The total operation time was 318 minutes without transfusion. The final graft weight was 208 g with GRWR of 2.62%. The donor was discharged uneventfully on postoperative day 4, and the graft function recovered to normal in the recipient without any graft related complication. CONCLUSION: Laparoscopic anatomic S3 procurement with in situ reduction is a feasible and safe procedure in selected donors in pediatric living donor liver transplantation.

Domino hepatocyte transplantation using explanted human livers with metabolic defects attenuates D-GalN/LPS-induced acute liver failure.

BACKGROUND: Explanted livers from patients with inherited metabolic liver diseases possess the potential to be a cell source of good-quality hepatocytes for hepatocyte transplantation (HT). This study evaluated the therapeutic effects of domino HT using hepatocytes isolated from explanted human livers for acute liver failure (ALF). METHODS: Isolated hepatocytes were evaluated for viability and function and then transplanted into D-galactosamine/lipopolysaccharide-induced ALF mice via splenic injection. The survival rate was analyzed by the Kaplan-Meier method and log-rank test. Liver function was evaluated by serum biochemical parameters, and inflammatory cytokine levels were measured by ELISA. The pathological changes in the liver tissues were assessed by hematoxylin-eosin staining. Hepatocyte apoptosis was investigated by TUNEL, and hepatocyte apoptosis-related proteins were detected by western blot. The localization of human hepatocytes in the injured mouse livers was detected by immunohistochemical analyses. RESULTS: Hepatocytes were successfully isolated from explanted livers of 10 pediatric patients with various liver-based metabolic disorders, with an average viability of 85.3% ± 13.0% and average yield of 9.2 × 106 ± 3.4 × 106 cells/g. Isolated hepatocytes had an excellent ability to secret albumin, produce urea, uptake indocyanine green, storage glycogen, and express alpha 1 antitrypsin, albumin, cytokeratin 18, and CYP3A4. Domino HT significantly reduced mortality, decreased serum levels of alanine aminotransferase and aspartate aminotransferase, and improved the pathological damage. Moreover, transplanted hepatocytes inhibited interleukin-6 and tumor necrosis factor-α levels. Domino HT also ameliorates hepatocyte apoptosis, as evidenced by decreased TUNEL positive cells. Positive staining for human albumin suggested the localization of human hepatocytes in ALF mice livers. CONCLUSION: Explanted livers from patients with inheritable metabolic disorders can serve as a viable cell source for cell-based therapies. Domino HT using hepatocytes with certain metabolic defects has the potential to be a novel therapeutic strategy for ALF.

Impact of living donor liver with steatosis and idiopathic portal inflammation on clinical outcomes in pediatric liver transplantation: Beijing experience.

Background: To evaluate the impact of steatosis and/or idiopathic portal inflammation (IPI) in living donor livers on recipients' clinical outcomes. Methods: We assessed 305 qualified donor liver samples from June 2013 to December 2018. Donors and recipients' clinical characteristics, including follow-up data were retrieved. The graft and overall survival with/without steatosis or portal inflammation were compared by Kaplan-Meier analysis. Results: For living donors, the medium age of was 31.2 (28, 35.8) years old; liver histopathology showed macrovesicular steatosis: 0-5% 264/305 (86.6%) and 5-30% 41/305 (13.4%), IPI: no 220/305 (72.1%) and mild 85/305 (27.9%). For recipients, the medium age was 1.0 (0.6, 1.5) years old; the median pediatric-end-stage-liver-disease score was 16 (5.0, 26.0) and medium follow-up time was 32.8 (24.8, 52.0) months. Biliary atresia (69.5%) was the main indication for liver transplantation (LT). Conclusions: The presence of steatosis and portal inflammation of the donor liver did not impact the clinical outcomes including transaminase or bilirubin normalization, short-/long-term complications and recipients' survival. However, recipients with high pediatric-end-stage-liver-disease score (>16) receiving donor liver with portal inflammation, but not steatosis, had trend negative effect on recipients' survival. In conclusion, donor livers with mild steatosis and portal inflammation were qualified for pediatric living donor LT. However, donor liver with mild portal inflammation would better not be allocated to recipients with high pediatric-end-stage-liver-disease score. This study provided new evidence in pediatric living donor liver allocation.

The effect of liver transplantation for argininemia-the largest experiences in a single center.

Background: Argininemia, a rare urea cycle disorder resulting from an arginase-1 deficiency, is characterized by a progressive spastic paraplegia. While advances in diagnosis and treatment have increased the management of this condition, not all symptoms are resolved in response to traditional therapies. Interestingly, there exist some rare reports on the use of liver transplantation (LT) for the treatment of argininemia. Methods: We conducted a retrospective study of eleven patients with argininemia receiving a LT as performed at our center over the period from January 2015 to November 2019. These patients were included due to their poor responses to protein restriction diets and alternative therapies of nitrogen scavengers. Detailed information on coagulation, liver function, histopathological and morphological examination of liver samples, and other clinical presentations were extracted from these patients. A grading scale was used for evaluating the neurological status, classification of physical growth and quality of life of these patients in response to the LT. Results: Prior to LT, high levels of arginine were detected in all of argininemia patients and liver enzymes were elevated in nine of those patients. Nine patients presented with coagulation dysfunction without bleeding symptoms. Spastic paraplegia, irritability, intellectual developmental disability, and growth deficits were hallmarks of these nine patients, while four patients showed repeated, generalized tonic-clonic seizures before the operation. Seven novel mutations were found in these patients. The indication for LT in this series of patients was a presentation of progressive neurological impairments. After LT, the coagulation index and plasma arginine levels returned to normal and episodes of seizure were controlled in four patients. To date, all patients have survived and their LT has resulted a restoration of arginine metabolism and liver function, along with preventing further neurological deterioration, all of which provided an opportunity for future recuperation. Overall, the neurological status, growth deficits and quality of life were all significantly improved after LT with no evidence of severe complications. Conclusions: LT can serve as an effective treatment for argininemia in patients who respond poorly to traditional therapy. An early intervention of LT should be conducted in these patients to prevent neurological damage and improve their quality of life.

Liver Graft-to-Spleen Volume Ratio as a Useful Predictive Factor of the Outcomes in Living Donor Liver Transplantation: A Retrospective Study.

Background: In 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. Methods: A 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. Results: According 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). Conclusions: Without splenectomy, low GSVR was an important predictor of portal hypertension and impaired graft function after LDLT.

Therapeutic potential of living donor liver transplantation from heterozygous carrier donors in children with propionic acidemia.

BACKGROUND: Current world experience regarding living donor liver transplantation (LDLT) in the treatment of propionic acidemia (PA) is limited, especially in terms of using obligate heterozygous carriers as donors. This study aimed to evaluate the clinical outcomes of LDLT in children with PA. METHODS: From November 2017 to January 2020, 7 of the 192 children who underwent LDLT at our institution had been diagnosed with PA (median age, 2.1 years; range, 1.1-5.8 years). The primary indication for transplantation was frequent metabolic decompensations in 6 patients and preventative treatment in 1 patient. Of the seven parental living donors, six were genetically proven obligate heterozygous carriers. RESULTS: During a median follow-up of 23.9 months (range, 13.9-40.2 months), all patients were alive with 100% allograft survival, and no severe transplant-related complications occurred. In the case of liberalized protein intake, they did not suffer metabolic decompensation or disease-related complications and made progress in neurodevelopmental delay and body growth, as well as blood and urinary metabolite levels. In one patient with pre-existing mild dilated cardiomyopathy, her echocardiogram results completely normalized 13.8 months post-transplant. All living donors recovered well after surgery, with no metabolic decompensations or procedure-related complications. Western blotting revealed that the hepatic expressions of PCCA and PCCB in one of the heterozygous donors were comparable to those of the normal healthy control at the protein level. CONCLUSIONS: LDLT using partial liver grafts from asymptomatic obligate heterozygous carrier donors is a viable therapeutic option for selected PA patients, with no negative impact on donors' and recipients' clinical courses.

Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia.

The aim is to explore the impact of the Kasai procedure (KP) and the length of native liver survival time (NLST) on outcomes of liver transplantation (LT). Patients with biliary atresia (BA), who underwent LT in Beijing Friendship Hospital from January 2017 to December 2019, were enrolled and divided into non-KP (N-KP) and post-KP (P-KP) groups. The patients in the P-KP group were further divided into early failure (KP-EF) defined by NLST <1 year, medium failure (KP-MF, NLST 1-5 years), and late failure (KP-LF, NLST >5 years) subgroups. Clinical data at baseline and during follow-up were collected. The inverse probability of treatment weighting method was used to evaluate the independent effect of KP and the length of NLST on clinical outcomes. Among 197 patients with BA, the N-KP group accounted for 43 (21.8%), KP-EF 71 (46.1%), KP-MF 59 (38.3%), and KP-LF 24 (15.6%) cases, respectively. The N-KP and KP-EF groups had significantly longer hospitalization and intensive care unit stays after LT. Graft and overall survival rates were 93.0% in the N-KP group and 97.4% in P-KP group, respectively. The mortality rate in the P-KP group were significantly lower compared with that of the N-KP group with a hazard ratio (HR) of 0.2 (P = 0.02). The risks of biliary and vascular complications and cytomegalovirus (CMV) infection after LT were significantly higher in KP-EF group than those in the KP-MF and KP-LF groups (HRs = 0.09, 0.2, and 0.3, respectively; all P < 0.001). The KP significantly improved after LT overall survival. Patients with early native liver failure after KP have significantly higher risks for biliary and vascular complications and CMV infection.

Association of Gut Microbiota and Metabolites With Disease Progression in Children With Biliary Atresia.

Background and Aims: Biliary atresia is the most common cause of liver disease and liver transplantation in children. The accumulation of bile acids in hepatocytes and the stimulation of the intestinal microbiome can aggravate the disease progression. This study investigated changes in the composition of the gut microbiota and its metabolites in biliary atresia and the possible effects of these changes on disease progression. Methods: Stool samples of biliary atresia at different disease stages and matched control individuals were collected (early stage: 16 patients, 16 controls; later stage: 16 patients, 10 controls). Metagenomic sequencing was performed to evaluate the gut microbiota structure. Untargeted metabolomics was performed to detect and analyze the metabolites and bile acid composition. Results: A disturbed gut microbiota structure occurred in the early and later stages of biliary atresia. Klebsiella, Streptococcus, Veillonella, and Enterococcus have always been dominant. The abundance of V. atypica displayed significant changes between the early and later stages of biliary atresia. Combined with clinical indicators, Spearman's analysis showed that Klebsiella and Veillonella atypica strongly correlated with liver enzymes. Enterococcus faecium had an enormously positive relationship with lithocholic acid derivatives. Metabolites involved in tryptophan metabolism were changed in the patients with biliary atresia, which had a significant association with stool V. atypica and blood total bilirubin (p < 0.05). Conclusions: The liver damage of biliary atresia was directly or indirectly exacerbated by the interaction of enriched Klebsiella (K. pneumoniae), Veillonella (V. atypica), and Enterococcus (E. faecium) with dysmetabolism of tryptophan and bile acid.

Auxiliary Liver Graft Can Be Protected From HBV Infection in HBsAg Positive Blood Circulation.

Auxiliary grafts have a high risk of Hepatitis B virus (HBV) infection in patients with chronic HBV-related diseases. Hepatitis B virus-related auxiliary partial orthotopic liver transplantation (APOLT) cases were reviewed to show the results of current methods to block native-to-graft HBV transmission. Three patients received APOLT for HBV-related liver cirrhosis and a recurrent upper gastrointestinal hemorrhage between April 2015 and January 2017 by the liver transplant team of Beijing Friendship Hospital affiliated with Capital Medical University. All three patients were positive for HBV surface antigen (HBsAg) and had a negative HBV DNA test result before transplantation. After auxiliary transplantations, HBsAg was found to be positive in two patients and negative in one patient. To avoid graft infection of HBV, entecavir-based therapy was employed and the remnant native livers of the recipients were removed 51-878 days after liver transplantation. Then, serum conversions of HBsAg were found in all three cases. For the first time, this case series shows the possibility of blocking the transmission of HBV from a native liver to a graft in auxiliary transplantation by entecavir-based therapy. Among the cases, a left lobe graft was successfully implanted as a replacement of the right lobe of the recipient, which is also discussed.

Characteristics of Gut Microbiota in Children With Biliary Atresia After Liver Transplantation.

BACKGROUND AND AIMS: Biliary atresia (BA) is an idiopathic neonatal cholestasis and is the most common indication in pediatric liver transplantation (LT). Previous studies have suggested that the gut microbiota (GM) in BA is disordered. However, the effect of LT on gut dysbiosis in patients with BA has not yet been elucidated. METHODS: Patients with BA (n = 16) and healthy controls (n = 10) were recruited. In the early life of children with BA, Kasai surgery is a typical procedure for restoring bile flow. According to whether BA patients had previously undergone Kasai surgery, we divided the post-LT patients into the with-Kasai group (n = 8) and non-Kasai group (n = 8). Fecal samples were collected in both the BA and the control group; among BA patients, samples were obtained again 6 months after LT. A total of 40 fecal samples were collected, of which 16 were pre-LT, 14 were post-LT (8 were with-Kasai, 6 were non-Kasai), and 10 were from the control group. Metagenomic sequencing was performed to evaluate the GM. RESULTS: The Kruskal-Wallis test showed a statistically significant difference in the number of genes between the pre-LT and the control group, the pre-LT and the post-LT group (P < 0.05), but no statistical difference between the post-LT and the control group. Principal coordinate analysis also showed that the microbiome structure was similar between the post-LT and control group (P > 0.05). Analysis of the GM composition showed a significant decrease in Serratia, Enterobacter, Morganella, Skunalikevirus, and Phifllikevirus while short chain fatty acid (SCFA)-producing bacteria such as Roseburia, Blautia, Clostridium, Akkermansia, and Ruminococcus were increased after LT (linear discriminant analysis > 2, P < 0.05). However, they still did not reach the normal control level. Concerning functional profiles, lipopolysaccharide metabolism, multidrug resistance, polyamine biosynthesis, GABA biosynthesis, and EHEC/EPEC pathogenicity signature were more enriched in the post-LT group compared with the control group. Prior Kasai surgery had a specific influence on the postoperative GM. CONCLUSION: LT partly improved the GM in patients with BA, which provided new insight into understanding the role of LT in BA.

Cholangiojejunostomy for multiple biliary ducts in living donor liver transplantation: A case report.

BACKGROUND: Laparoscopic living donor hepatectomy (LLDH) has been successfully carried out in several transplant centers. Biliary reconstruction is key in living donor liver transplantation (LDLT). Reliable biliary reconstruction can effectively prevent postoperative biliary stricture and leakage. Although preoperative magnetic resonance cholangiopancreatography and intraoperative indocyanine green cholangiography have been shown to be helpful in determining optimal division points, biliary variability and limitations associated with LLDH, multiple biliary tracts are often encountered during surgery, which inhibits biliary reconstruction. A reliable cholangiojejunostomy for multiple biliary ducts has been utilized in LDLT. This procedure provides a reference for multiple biliary reconstructions after LLDH. CASE SUMMARY: A 2-year-old girl diagnosed with ornithine transcarbamylase deficiency required liver transplantation. Due to the scarcity of deceased donors, she was put on the waiting list for LDLT. Her father was a suitable donor; however, after a rigorous evaluation, preoperative magnetic resonance cholangiopancreatography examination of the donor indicated the possibility of multivessel variation in the biliary tract. Therefore, a laparoscopic left lateral section was performed on the donor, which met the estimated graft-to-recipient weight ratio. Under intraoperative indocyanine green cholangiography, 4 biliary tracts were confirmed in the graft. It was difficult to reform the intrahepatic bile ducts due to their openings of more than 5 mm. A reliable cholangiojejunostomy was, therefore, utilized: Suture of the jejunum to the adjacent liver was performed around the bile duct openings with 6/0 absorbable sutures. At the last follow-up (1 year after surgery), the patient was complication-free. CONCLUSION: Intrahepatic cholangiojejunostomy is reliable for multiple biliary ducts after LLDH in LDLT.

Is simultaneous partial splenectomy during pediatric liver transplantation safe and effective for severe hypersplenism? A prospective cohort study.

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.