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 a...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 3patients 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.展开更多
AIM:To investigate the diagnostic value of glypican-3(GPC3) and its relationship with hepatocellular carcinoma(HCC) recurrence after liver transplantation.METHODS:HCC tissue samples(n = 31) obtained from patients who ...AIM:To investigate the diagnostic value of glypican-3(GPC3) and its relationship with hepatocellular carcinoma(HCC) recurrence after liver transplantation.METHODS:HCC tissue samples(n = 31) obtained from patients who had undergone liver transplantation were analyzed.GPC3 mRNA and protein expression were analyzed by TaqMan real-time reverse transcription-polymerase chain reaction and immunohistochemistry.Correlation between the GPC3 expression and clinicopathological features was analyzed.The potential prognostic value of GPC3 was investigated by comparing recurrence-free survival between HCC patients with and without GPC3 expression.RESULTS:Using a cutoff value of 3.5 × 10-2,20 of 31 cancerous tissues had expression values of > 3.5 × 10-2,whereas 3 of 31 adjacent non-neoplastic parenchyma and 0 of 20 control liver tissues had expression values of > 3.5 × 10-2(P < 0.001).GPC3 protein was immunoexpressed in 68% of cancerous tissues,but not in adjacent non-neoplastic parenchyma and control liver tissues.Vascular invasion was significantly related to GPC3 expression(P < 0.05).Recurrence-free survival was significantly longer for patients without GPC3 mRNA overexpression(> 3.5 × 10-2) and those without vascular invasion(P < 0.05 for both).CONCLUSION:GPC3 expression may serve as a valuable diagnostic marker for HCC.GPC3 mRNA overexpression may be an adverse indicator for HCC patients after liver transplantation.展开更多
We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy(FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino ...We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy(FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino graft was implanted during each stage. During the firststage, an auxiliary domino liver transplantation was conducted using a domino graft from a 4-year-old female child with Wilson's disease. After removing the right lobe of the FAP patient's liver, the graft was rotated 90 degrees counterclockwise and placed along the right side of the inferior vena cava(IVC). The orifices of the left, middle, and right hepatic veins were reconstructed using an iliac vein patch and then anastomosed to the right side of the IVC. Thirty days later, a second domino liver graft was implanted. The second domino graft was from a 3-yearold female child with an ornithine carbamyl enzyme defect, and it replaced the residual native liver(left lobe). To balance the function and blood flow between the two grafts, a percutaneous transcatheter selective portal vein embolization was performed, and "the left portal vein" of the first graft was blocked 9 mo after the second transplantation. The liver function indices, blood ammonia, and 24-h urinary copper levels were normal at the end of a 3-year follow-up. These two domino donor grafts from donors with different metabolic disorders restored normal liver function. Our experience demonstrated a new approach for resolving metabolic disorders with domino grafts and utilizing explanted livers from children.展开更多
BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death...BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.展开更多
AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction(HVOO) following pediatric liver transplantation.METHODS A total of 246 pediatric patients underwent l...AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction(HVOO) following pediatric liver transplantation.METHODS A total of 246 pediatric patients underwent liver transplantation at our hospital between June 2013 and September 2016. Among these patients, five were ultimately diagnosed with HVOO. Seven procedures(two patients underwent two balloon dilatation procedures) were included in this analysis. The demographic data,types of donor and liver transplant, interventional examination and therapeutic outcomes of these five children were analyzed. The median interval time between pediatric liver transplantation and balloon dilatation procedures was 9.8 mo(range: 1-32).RESULTS Five children with HVOO were successfully treated by balloon angioplasty without stent placement, with seven procedures performed for six stenotic lesions. All children underwent successful percutaneous intervention. Among these five patients, four were treated by single balloon angioplasty, and these patients did not develop recurrent stenosis. In seven episodes of balloon angioplasty across the stenosis, the pressure gradient was 12.0 ± 8.8 mm Hg before balloon dilatation and 1.1 ± 1.5 mm Hg after the procedures, which revealed a statistically significant reduction(P < 0.05). The overall technical success rate among these seven procedures was 100%(7/7), and clinical success was achieved in all five patients(100%). The patients were followed for 4-33 mo(median: 15 mo). No significant procedural complications or procedurerelated deaths occurred.CONCLUSION Balloon dilatation is an effective and safe therapeutic option for HVOO in children undergoing pediatric liver transplantation. Venous angioplasty is also recommended in cases with recurrent HVOO.展开更多
BACKGROUND Portopulmonary hypertension(PoPH)is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for LT....BACKGROUND Portopulmonary hypertension(PoPH)is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for LT.Many patients with liver disease require but cannot receive LT due to severe PoPH and eventually died.We report a patient with severe PoPH who underwent successful LT and had near normal pulmonary pressure without drug treatment.CASE SUMMARY A 39-year-old woman was hospitalized with the chief complaint of jaundice and exertional dyspnea and fatigue.Caroli disease and liver cirrhosis was diagnosed 6 years previously.Her liver disease met the criteria for LT.However,right heart catheterization showed that her mean pulmonary artery pressure was increased at 50 mmHg,pulmonary vascular resistance was 460 dyn?s/cm5 and pulmonary artery wedge pressure was 20 mmHg,which may have been the reasons for her chief complaint.The patient was diagnosed with severe PoPH and was not listed for LT immediately.After 5 mo of pharmacotherapy,her severe PoPH was moderate,and she underwent successful LT.Pulmonary artery pressure steadily decreased according to post-operative echocardiographic monitoring and drugs CONCLUSION The safety of LT can be greatly improved by reducing mean pulmonary artery pressure to a low level,and LT may cure PoPH.展开更多
BACKGROUND Cytomegalovirus(CMV)infection is a common infection in liver transplant recipients,which is related to chronic rejection and biliary complications.It is often diagnosed based on serum CMV-DNA or CMV pp65.To...BACKGROUND Cytomegalovirus(CMV)infection is a common infection in liver transplant recipients,which is related to chronic rejection and biliary complications.It is often diagnosed based on serum CMV-DNA or CMV pp65.To our knowledge,this is the first report of the successful treatment of occult CMV cholangitis in a pediatric liver transplantation(LT)recipient.CASE SUMMARY A 7-mo-old baby girl received LT due to biliary atresia and cholestasis cirrhosis.At 1 mo following LT,the patient suffered from aggravated jaundice with no apparent cause.As imaging results showed intrahepatic and extrahepatic bile duct dilatation,the patient was diagnosed with biliary complications and percutaneous cholangiography and biliary drainage was performed.However,there was little biliary drainage and her liver function deteriorated.CMV-DNA was isolated from the bile with the surprising outcome that 3×106 copies/mL were present,whereas the CMV-DNA in serum was negative.Following antiviral therapy with ganciclovir,she gradually recovered and bilirubin decreased to normal levels.During the 4-year follow-up period,her liver function remained normal.CONCLUSION Bile CMV sampling can be used for the diagnosis of occult CMV infection,especially in patients with negative serum CMV-DNA and CMV pp65.Testing for CMV in the biliary tract may serve as a novel approach for the diagnosis of cholestasis post-LT.Timely diagnosis and treatment will decrease the risk of graft loss.展开更多
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 reconstr...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.展开更多
BACKGROUND Single-port laparoscopy has been used in a variety of abdominal operations.We report the first case of single-port laparoscopic left lateral sectionectomy in pediatric laparoscopic living donor liver transp...BACKGROUND Single-port laparoscopy has been used in a variety of abdominal operations.We report the first case of single-port laparoscopic left lateral sectionectomy in pediatric laparoscopic living donor liver transplantation.CASE SUMMARY A 28-year-old man volunteered for living liver donation to his daughter who was diagnosed with liver cirrhosis and portal hypertension after the Kasai procedure for biliary atresia.His body mass index was 20.5 kg/m2.Liver dynamic computed tomography showed:(1)Left lateral graft volume of 232.76 cm3 with a graft-torecipient weight ratio of 2.59%;and(2)Right hepatic artery derived from the superior mesenteric artery.A single-port access system was placed through a transumbilical incision,including four trocars:two 12-mm ports for a camera and endoscopic stapler and two 5-mm working ports.Liver parenchyma was dissected by a Harmonic and Cavitron Ultrasonic Surgical Aspirator,while bipolar was used for coagulation.The bile duct was transected above the bifurcation by indocyanine green fluorescence cholangiography.The specimen was retrieved from the umbilical incision.The total operation time was 4 h without blood transfusion.The final graft weight was 233.6 g with graft-torecipient weight ratio of 2.60%.The donor was discharged uneventfully on postoperative day 4.CONCLUSION Single-port laparoscopic left lateral sectionectomy is feasible in pediatric laparoscopic living donor liver transplantation in an experienced transplant center.展开更多
BACKGROUND Ethylmalonic encephalopathy(EE) is a rare autosomal recessive metabolic disorder caused by impaired mitochondrial sulfur dioxygenase. Due to poor therapeutic effect of current conventional treatments, progr...BACKGROUND Ethylmalonic encephalopathy(EE) is a rare autosomal recessive metabolic disorder caused by impaired mitochondrial sulfur dioxygenase. Due to poor therapeutic effect of current conventional treatments, progressive psychomotor regression and neurological impairment usually contribute to early death in the first decade of life. Liver transplantation(LT) is emerging as a novel therapeutic option for EE;however, worldwide experience is still limited.CASE SUMMARY An 18-mo-old patient with the diagnosis of EE received a living donor liver transplant in our institution, which, to our knowledge, is the first case in AsianPacific countries. During 20 mo of follow-up, the longitudinal metabolic evaluations revealed a wild fluctuation in urinary EMA levels, still far beyond the normal range. Urinary 2-methylsuccinic acid levels gradually restored to normal, whereas the concentrations of urinary isobutyrylglycine and plasma C4-and C5-acylcarnitines fluctuated markedly and still remained above the reference limits. Only mild amelioration of petechiae and ecchymosis was observed, and no dramatic reversion of chronic mucoid diarrhea and orthostatic acrocyanosis occurred. Although brain magnetic resonance imaging suggested a certain improvement in basal ganglia lesions, the patient still presented developmental delay and neurologic disability.CONCLUSION LT may bring about a partial but not complete cure to EE. Given its definite role in defending against the devastating natural progression of EE, LT should still be considered for patients with EE in the absence of other superior therapeutic options. Early establishment of diagnosis and initiation of conventional treatment pre-transplant, timely LT, and continuous administration of metabolismcorrecting medications post-transplant may be helpful in minimizing the neurologic impairment and maximizing the therapeutic value of LT in EE.展开更多
BACKGROUND Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients.However,the simultaneous occurrence of these two diseases in the same lymph ...BACKGROUND Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients.However,the simultaneous occurrence of these two diseases in the same lymph nodes is very rare.CASE SUMMARY We report the case of a 19-mo-old boy,who presented with intermittent fever and enlarged cervical lymph nodes after liver transplantation.Six cervical lymph nodes were biopsied,and the histopathological examinations revealed multifocal hyperplasia of spindle cells around small blood vessels,extravasated erythrocytes,and heavy infiltration of plasma cells in the cortex and medulla of the lymph nodes.The immunohistochemical analyses of spindle cells revealed positive expression of CD34,CD31,erythroblast transformation-specific-related gene,friend leukemia integration 1,and human herpesvirus-8.The lymphoproliferative lesions expressed CD38,CD138,and multiple myeloma 1.Epstein-Barr encoded RNA in situ hybridization demonstrated Epstein-Barr virus-positive lymphoid cells.Finally,we diagnosed the coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder(plasmacytic hyperplasia)in the same lymph nodes.Treatment strategy included anti-CD20 monoclonal antibody(rituximab)and discontinuation of the immunosuppressant therapies.Lymph node biopsies during follow-up examinations revealed lymphoid hyperplasia.CONCLUSION The rare coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes post-liver transplantation possibly associates with immunodeficiency and Epstein-Barr virus and human herpesvirus-8 coinfection.展开更多
BACKGROUND Cytomegalovirus(CMV)infection is common in liver transplant(LT)_recipients,and biliary complications occur in a large number of patients.It has been reported that CMV-DNA is more detectable in bile than in ...BACKGROUND Cytomegalovirus(CMV)infection is common in liver transplant(LT)_recipients,and biliary complications occur in a large number of patients.It has been reported that CMV-DNA is more detectable in bile than in blood.AIM To investigate the effects of CMV infection on biliary complications by comparing the levels of CMV-DNA in the bile and blood of patients after LT.METHODS We conducted a retrospective analysis of 57 patients who underwent LT,10 of these patients had no biliary complications and 47 patients had biliary complications.We also compared the levels of CMV-DNA in patients’bile and blood,which were sampled concurrently.We used RNAscope technology to identify CMV in paraffin-embedded liver sections.RESULTS CMV-DNA was not detected in bile samples and was detected in 2 blood samples from patients without biliary complications.In the 47 patients with biliary complications,CMV-DNA was detected in 22 bile samples and 8 blood samples,both bile and blood samples were positive for CMV-DNA in 6 patients.The identification rate of CMV-DNA in blood was 17.0%,and was 46.8%in bile.Moreover,tissue samples from 4 patients with biliary complications tested positive using RNAscope technology but were negative with hematoxylin and eosin staining.During the follow-up period,graft failure occurred in 13 patients with biliary complications,8 of whom underwent retransplantation,and 3 died.CMV-DNA in bile was detected in 9 of 13 patients with graft failure.CONCLUSION In patients with biliary complications,the identification rate of CMV-DNA in bile was higher than that in blood.Blood CMV-DNA negative patients with biliary complications should still be monitored for CMV-related biliary tract diseases.Potential occult CMV infection may also be a contributing etiological factor in the development of graft failure.展开更多
BACKGROUND Sinusoidal obstructive syndrome(SOS)is a disease that damages hepatic sinusoidal endothelial cells,resulting in progressive occlusion and fibrosis of the lobular central vein and the occurrence of intrahepa...BACKGROUND Sinusoidal obstructive syndrome(SOS)is a disease that damages hepatic sinusoidal endothelial cells,resulting in progressive occlusion and fibrosis of the lobular central vein and the occurrence of intrahepatic sinusoidal portal hypertension.However,SOS after liver transplantation(LT)is uncommon and potentially fatal.Here,we report a rare case of second-time recurrence of SOS after liver retransplantation(rLT).CASE SUMMARY A 22-year-old woman received a living donor LT due to SOS.Four years later,she developed abdominal distention and ascites with no apparent cause.She was diagnosed with recurrence of SOS and underwent rLT.But 2 mo post rLT,the patient suffered from aggravated jaundice and ascites again.She was diagnosed with second-time recurrence of SOS post-rLT according to computed tomography and liver pathology.After treatment with warfarin anticoagulation and immunosuppressant conversion,she gradually recovered with improvement of liver function and liver pathology.During the 17-mo follow-up period,she was in good condition with normal liver function and no ascites.CONCLUSION SOS can be a recurrent disease after LT,and autoimmune antibody and genetic sequencing should be screened before LT.For susceptible patients,anticoagulant drugs should be used for an extended period,and tacrolimus or other pathogenic agents should be avoided.Early diagnosis and treatment can improve the prognosis of patients and avoid graft failure or death.展开更多
BACKGROUND Status epilepticus in patients with hepatic encephalopathy (HE) is a rare butserious condition that is refractory to antiepileptic drugs, and current treatmentplans are vague. Diagnosis may be difficult wit...BACKGROUND Status epilepticus in patients with hepatic encephalopathy (HE) is a rare butserious condition that is refractory to antiepileptic drugs, and current treatmentplans are vague. Diagnosis may be difficult without a clear history of cirrhosis.Liver transplantation (LT) is effective to alleviate symptoms, however, there arefew reports about LT in the treatment of status epilepticus with HE. To ourknowledge, this is the first report of status epilepticus present as initialmanifestation of HE.CASE SUMMARY A 59-year-old woman with a 20-year history of heavy drinking was hospitalizedfor generalized tonic-clonic seizures. She reported no history of episodes of HE,stroke, spontaneous bacterial peritonitis, ascites or gastrointestinal bleeding.Neurological examination revealed a comatose patient, without papilledema.Laboratory examination suggested liver cirrhosis. Plasma ammonia levels uponadmission were five times normal. Brain computed tomography (CT) was normal,while abdominal CT and ultrasound revealed mild ascites, liver cirrhosis andsplenomegaly. Electroencephalography (EEG)showed diffuse slow waves rhythm,consistent with HE, and sharp waves during ictal EEG corresponding to clinicalsemiology of focal tonic seizures. The symptoms were reversed by continuousantiepileptic treatment and lactulose. She was given oral levetiracetam, and focalaware seizures occasionally affected her 10 mo after LT.CONCLUSION Status epilepticus could be an initial manifestation of HE. Antiepileptic drugs combined with lactulose are essential for treatment of status epilepticus with HE,and LT is effective to prevent the relapse.展开更多
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 laparos...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.展开更多
Background and Aims:Patients with biliary atresia(BA)are prone to hepatic decompensation,which might eventually lead to death.This study aimed to identify the possible risk factors affecting in-hospital death in BA pa...Background and Aims:Patients with biliary atresia(BA)are prone to hepatic decompensation,which might eventually lead to death.This study aimed to identify the possible risk factors affecting in-hospital death in BA patients in China.Methods:We collected data from the Hospital Quality Monitoring System,a national inpatient database.All patients aged up to 2 years old with a diagnosis of BA were included.The subjects were divided to three groups,including Kasai portoenterostomy(KP),liver transplantation(LT),and no surgery.Logistic regression with Firth’s method was performed to identify potential influencing variables associated with in-hospital death.Results:During the year 2013 to 2017,there were 14,038 pediatric admissions with a diagnosis of BA.The proportion of in-hospital death in pediatric BA admissions was 1.08%.Compared with patients under six months,there was a higher risk of in-hospital death for children aged six months to 1 year and 1–2 years old.Clinical signs,including cirrhosis,variceal bleeding,and hepatic encephalopathy,were significantly associated with the risk of in-hospital death.In no surgery group,compared to those in Beijing and Shanghai,BA patients admitted in other districts had a lower risk of in-hospital death(OR=0.39,95%CI:0.21,0.70).However,in the LT group,patients admitted in other districts had a higher risk of in-hospital death(OR=9.13,95%CI:3.99,20.87).Conclusions:In-hospital survival remains unsatisfactory for pediatric BA patients with severe complications.Furthermore,more resources and training for BA treatment,especially LT,are essential for districts with poor medical care in the future.展开更多
The fame of regeneration has been attributed to the liver ever since the era of Greek mythology,in which Prometheus’liver could grow back overnight against the daily predation of Zeus’eagle.Hepatectomy is the very b...The fame of regeneration has been attributed to the liver ever since the era of Greek mythology,in which Prometheus’liver could grow back overnight against the daily predation of Zeus’eagle.Hepatectomy is the very basis of liver surgery including liver transplantation,and post-hepatectomy liver regeneration remains the center of interest for both clinicians and scientists.Novel tools for structural and functional assessment of liver regeneration have been constantly developed and validated,laying the groundwork for clinical management and scientific analysis(1).展开更多
Background:Although a number of technical problems and donor safety issues associated with living donor liver transplantation(LDLT)have been resolved,some initial clinical studies showed an increased risk of hepatocel...Background:Although a number of technical problems and donor safety issues associated with living donor liver transplantation(LDLT)have been resolved,some initial clinical studies showed an increased risk of hepatocellular car cinoma(HCC)recurrence in LDLT.This meta-analysis was conducted to assess differences in tumor recurrence between LDLT and deceased donor liver transplantation(DDLT).Methods:After systematic retrievals of studies about LDLT and DDLT for HCC,articles were selected with a rationale of emphasizing inter-group comparability.Results from multivariate analyses were combined and discussed together with univariate analyses.In subgroup analysis,the impact of organ allocation policy was taken into consideration.Results:Seven articles were included in the meta-analysis.Overall,a salient result that emerged from the seven studies was a significant increased risk of HCC recurrence in the LDLT group than in the DDLT group(P=0.01).The most significant increase in hazard ratio was found in studies where organs tended to be allocated to non-tumor patients.Conclusions:An increased risk for HCC recurrence in LDLT as compared with DDLT patients was found.The relatively shorter preoperative observation windows in LDLT may lead to fewer cases of HCC with invasive features being screened out,which may provide a possible explanation for the high rates of HCC recurrence.展开更多
Background: The outcome of pediatric deceased donor liver transplantation (LT) has not been well studied, especially pediatric deceased donor livers used in adult transplantation. This study aimed to evaluate the e...Background: The outcome of pediatric deceased donor liver transplantation (LT) has not been well studied, especially pediatric deceased donor livers used in adult transplantation. This study aimed to evaluate the efficacy of LT using pediatric deceased donor livers and compare the outcomes between pediatric-to-pediatric LT and pediatric-to-adult LT. Methods: A retrospective review of LT using pediatric deceased donor livers from June 2013 to August 2016 was performed. The patients were divided into the pediatric-to-pediatric LT group and pediatric-to-adult LT group based on the ages of the recipients. The survival and incidence of early vascular complications (VCs) were observed between the two groups. We also analyzed the risk factors of early VCs in pediatric LT and the effect of donor hypernatremia on the prognosis of recipients. Results: There were 102 cases of LT using pediatric deceased donor livers in our hospital from June 2013 to August 2016. 83 pediatric-to-pediatric LT (recipients' age ≤13 years) and 19 pediatric-to-adult LT (recipients' age≥19 years). The ratio of early VC was similar in the two groups (19.3% vs. 10.6%, P = 0.514). Low body weight of recipient was an independent risk factor of early VC in pediatric LT (odds ratio: 0.856, 95% confidence interval: 0.752-0.975, P = 0.019). The 1-year cumulative survival rates of grafts and patients were 89.16% and 91.57% in pediatric-to-pediatric LT and 89.47% and 94.74% in pediatric-to-adult LT, respectively (all P 〉 0.05). In all cases. patients using donors with hypernatremia (serum sodium levels ≥ 150mmol/L) had worse graft survival (χ2=4.330, P = 0.037). Conclusions: Pediatric-to-pediatric LT group has similar graft and patient survival rates with those of pediatric-to-adult LT group. Low body weight of recipients is an independent risk factor of early VC in pediatric LT. Patients using donors with hypernatremia have worse graft survival.展开更多
文摘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 3patients 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.
基金Supported by Tianjin Municipal Health Bureau Key Project for Key Laboratory for Critical Care Medicine Development
文摘AIM:To investigate the diagnostic value of glypican-3(GPC3) and its relationship with hepatocellular carcinoma(HCC) recurrence after liver transplantation.METHODS:HCC tissue samples(n = 31) obtained from patients who had undergone liver transplantation were analyzed.GPC3 mRNA and protein expression were analyzed by TaqMan real-time reverse transcription-polymerase chain reaction and immunohistochemistry.Correlation between the GPC3 expression and clinicopathological features was analyzed.The potential prognostic value of GPC3 was investigated by comparing recurrence-free survival between HCC patients with and without GPC3 expression.RESULTS:Using a cutoff value of 3.5 × 10-2,20 of 31 cancerous tissues had expression values of > 3.5 × 10-2,whereas 3 of 31 adjacent non-neoplastic parenchyma and 0 of 20 control liver tissues had expression values of > 3.5 × 10-2(P < 0.001).GPC3 protein was immunoexpressed in 68% of cancerous tissues,but not in adjacent non-neoplastic parenchyma and control liver tissues.Vascular invasion was significantly related to GPC3 expression(P < 0.05).Recurrence-free survival was significantly longer for patients without GPC3 mRNA overexpression(> 3.5 × 10-2) and those without vascular invasion(P < 0.05 for both).CONCLUSION:GPC3 expression may serve as a valuable diagnostic marker for HCC.GPC3 mRNA overexpression may be an adverse indicator for HCC patients after liver transplantation.
基金Supported by Capital Special Program for Health Research and Development,No.2016-1-2021National Key Technologies R&D Program,No.2015BAI13B09+1 种基金The Training Program of Academic Leaders in Beijing Health System,No.2014-2-002Beijing Municipal Administration of Hospitals Ascent Plan,No.DFL20150101
文摘We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy(FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino graft was implanted during each stage. During the firststage, an auxiliary domino liver transplantation was conducted using a domino graft from a 4-year-old female child with Wilson's disease. After removing the right lobe of the FAP patient's liver, the graft was rotated 90 degrees counterclockwise and placed along the right side of the inferior vena cava(IVC). The orifices of the left, middle, and right hepatic veins were reconstructed using an iliac vein patch and then anastomosed to the right side of the IVC. Thirty days later, a second domino liver graft was implanted. The second domino graft was from a 3-yearold female child with an ornithine carbamyl enzyme defect, and it replaced the residual native liver(left lobe). To balance the function and blood flow between the two grafts, a percutaneous transcatheter selective portal vein embolization was performed, and "the left portal vein" of the first graft was blocked 9 mo after the second transplantation. The liver function indices, blood ammonia, and 24-h urinary copper levels were normal at the end of a 3-year follow-up. These two domino donor grafts from donors with different metabolic disorders restored normal liver function. Our experience demonstrated a new approach for resolving metabolic disorders with domino grafts and utilizing explanted livers from children.
基金supported by a grant from the Tianjin Bureau of Public Health Project (11KG103)
文摘BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.
文摘AIM To assess the efficacy and safety of balloon dilatation for the treatment of hepatic venous outflow obstruction(HVOO) following pediatric liver transplantation.METHODS A total of 246 pediatric patients underwent liver transplantation at our hospital between June 2013 and September 2016. Among these patients, five were ultimately diagnosed with HVOO. Seven procedures(two patients underwent two balloon dilatation procedures) were included in this analysis. The demographic data,types of donor and liver transplant, interventional examination and therapeutic outcomes of these five children were analyzed. The median interval time between pediatric liver transplantation and balloon dilatation procedures was 9.8 mo(range: 1-32).RESULTS Five children with HVOO were successfully treated by balloon angioplasty without stent placement, with seven procedures performed for six stenotic lesions. All children underwent successful percutaneous intervention. Among these five patients, four were treated by single balloon angioplasty, and these patients did not develop recurrent stenosis. In seven episodes of balloon angioplasty across the stenosis, the pressure gradient was 12.0 ± 8.8 mm Hg before balloon dilatation and 1.1 ± 1.5 mm Hg after the procedures, which revealed a statistically significant reduction(P < 0.05). The overall technical success rate among these seven procedures was 100%(7/7), and clinical success was achieved in all five patients(100%). The patients were followed for 4-33 mo(median: 15 mo). No significant procedural complications or procedurerelated deaths occurred.CONCLUSION Balloon dilatation is an effective and safe therapeutic option for HVOO in children undergoing pediatric liver transplantation. Venous angioplasty is also recommended in cases with recurrent HVOO.
基金Supported by Beijing Science and Technology Commission Capital Clinical Application Research,No.Z181100001718220
文摘BACKGROUND Portopulmonary hypertension(PoPH)is not uncommon in patients waiting for liver transplantation(LT).Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for LT.Many patients with liver disease require but cannot receive LT due to severe PoPH and eventually died.We report a patient with severe PoPH who underwent successful LT and had near normal pulmonary pressure without drug treatment.CASE SUMMARY A 39-year-old woman was hospitalized with the chief complaint of jaundice and exertional dyspnea and fatigue.Caroli disease and liver cirrhosis was diagnosed 6 years previously.Her liver disease met the criteria for LT.However,right heart catheterization showed that her mean pulmonary artery pressure was increased at 50 mmHg,pulmonary vascular resistance was 460 dyn?s/cm5 and pulmonary artery wedge pressure was 20 mmHg,which may have been the reasons for her chief complaint.The patient was diagnosed with severe PoPH and was not listed for LT immediately.After 5 mo of pharmacotherapy,her severe PoPH was moderate,and she underwent successful LT.Pulmonary artery pressure steadily decreased according to post-operative echocardiographic monitoring and drugs CONCLUSION The safety of LT can be greatly improved by reducing mean pulmonary artery pressure to a low level,and LT may cure PoPH.
基金Supported by the Beijing Municipal Science and Technology Commission,No.Z181100001718220.
文摘BACKGROUND Cytomegalovirus(CMV)infection is a common infection in liver transplant recipients,which is related to chronic rejection and biliary complications.It is often diagnosed based on serum CMV-DNA or CMV pp65.To our knowledge,this is the first report of the successful treatment of occult CMV cholangitis in a pediatric liver transplantation(LT)recipient.CASE SUMMARY A 7-mo-old baby girl received LT due to biliary atresia and cholestasis cirrhosis.At 1 mo following LT,the patient suffered from aggravated jaundice with no apparent cause.As imaging results showed intrahepatic and extrahepatic bile duct dilatation,the patient was diagnosed with biliary complications and percutaneous cholangiography and biliary drainage was performed.However,there was little biliary drainage and her liver function deteriorated.CMV-DNA was isolated from the bile with the surprising outcome that 3×106 copies/mL were present,whereas the CMV-DNA in serum was negative.Following antiviral therapy with ganciclovir,she gradually recovered and bilirubin decreased to normal levels.During the 4-year follow-up period,her liver function remained normal.CONCLUSION Bile CMV sampling can be used for the diagnosis of occult CMV infection,especially in patients with negative serum CMV-DNA and CMV pp65.Testing for CMV in the biliary tract may serve as a novel approach for the diagnosis of cholestasis post-LT.Timely diagnosis and treatment will decrease the risk of graft loss.
基金Capital's Funds for Health Improvement and Research,No.2020-1-2024.
文摘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.
基金Supported by Capital Research Project for Specialty Clinical Application,No.Z181100001718220.
文摘BACKGROUND Single-port laparoscopy has been used in a variety of abdominal operations.We report the first case of single-port laparoscopic left lateral sectionectomy in pediatric laparoscopic living donor liver transplantation.CASE SUMMARY A 28-year-old man volunteered for living liver donation to his daughter who was diagnosed with liver cirrhosis and portal hypertension after the Kasai procedure for biliary atresia.His body mass index was 20.5 kg/m2.Liver dynamic computed tomography showed:(1)Left lateral graft volume of 232.76 cm3 with a graft-torecipient weight ratio of 2.59%;and(2)Right hepatic artery derived from the superior mesenteric artery.A single-port access system was placed through a transumbilical incision,including four trocars:two 12-mm ports for a camera and endoscopic stapler and two 5-mm working ports.Liver parenchyma was dissected by a Harmonic and Cavitron Ultrasonic Surgical Aspirator,while bipolar was used for coagulation.The bile duct was transected above the bifurcation by indocyanine green fluorescence cholangiography.The specimen was retrieved from the umbilical incision.The total operation time was 4 h without blood transfusion.The final graft weight was 233.6 g with graft-torecipient weight ratio of 2.60%.The donor was discharged uneventfully on postoperative day 4.CONCLUSION Single-port laparoscopic left lateral sectionectomy is feasible in pediatric laparoscopic living donor liver transplantation in an experienced transplant center.
基金Supported by Beijing Municipal Science&Technology Commission,No. Z181100001718220Capital's Funds for Health Improvement and Research,No. 2020-1-2024。
文摘BACKGROUND Ethylmalonic encephalopathy(EE) is a rare autosomal recessive metabolic disorder caused by impaired mitochondrial sulfur dioxygenase. Due to poor therapeutic effect of current conventional treatments, progressive psychomotor regression and neurological impairment usually contribute to early death in the first decade of life. Liver transplantation(LT) is emerging as a novel therapeutic option for EE;however, worldwide experience is still limited.CASE SUMMARY An 18-mo-old patient with the diagnosis of EE received a living donor liver transplant in our institution, which, to our knowledge, is the first case in AsianPacific countries. During 20 mo of follow-up, the longitudinal metabolic evaluations revealed a wild fluctuation in urinary EMA levels, still far beyond the normal range. Urinary 2-methylsuccinic acid levels gradually restored to normal, whereas the concentrations of urinary isobutyrylglycine and plasma C4-and C5-acylcarnitines fluctuated markedly and still remained above the reference limits. Only mild amelioration of petechiae and ecchymosis was observed, and no dramatic reversion of chronic mucoid diarrhea and orthostatic acrocyanosis occurred. Although brain magnetic resonance imaging suggested a certain improvement in basal ganglia lesions, the patient still presented developmental delay and neurologic disability.CONCLUSION LT may bring about a partial but not complete cure to EE. Given its definite role in defending against the devastating natural progression of EE, LT should still be considered for patients with EE in the absence of other superior therapeutic options. Early establishment of diagnosis and initiation of conventional treatment pre-transplant, timely LT, and continuous administration of metabolismcorrecting medications post-transplant may be helpful in minimizing the neurologic impairment and maximizing the therapeutic value of LT in EE.
基金Supported by the Digestive Medical Coordinated Development Center of Beijing Hospitals Authority,No.XXX0102。
文摘BACKGROUND Kaposi sarcoma and post-transplant lymphoproliferative disorder have been occasionally reported in post-liver transplant patients.However,the simultaneous occurrence of these two diseases in the same lymph nodes is very rare.CASE SUMMARY We report the case of a 19-mo-old boy,who presented with intermittent fever and enlarged cervical lymph nodes after liver transplantation.Six cervical lymph nodes were biopsied,and the histopathological examinations revealed multifocal hyperplasia of spindle cells around small blood vessels,extravasated erythrocytes,and heavy infiltration of plasma cells in the cortex and medulla of the lymph nodes.The immunohistochemical analyses of spindle cells revealed positive expression of CD34,CD31,erythroblast transformation-specific-related gene,friend leukemia integration 1,and human herpesvirus-8.The lymphoproliferative lesions expressed CD38,CD138,and multiple myeloma 1.Epstein-Barr encoded RNA in situ hybridization demonstrated Epstein-Barr virus-positive lymphoid cells.Finally,we diagnosed the coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder(plasmacytic hyperplasia)in the same lymph nodes.Treatment strategy included anti-CD20 monoclonal antibody(rituximab)and discontinuation of the immunosuppressant therapies.Lymph node biopsies during follow-up examinations revealed lymphoid hyperplasia.CONCLUSION The rare coexistence of Kaposi sarcoma and post-transplant lymphoproliferative disorder in the same lymph nodes post-liver transplantation possibly associates with immunodeficiency and Epstein-Barr virus and human herpesvirus-8 coinfection.
基金The National Natural Science Foundation of China,No.81570586.
文摘BACKGROUND Cytomegalovirus(CMV)infection is common in liver transplant(LT)_recipients,and biliary complications occur in a large number of patients.It has been reported that CMV-DNA is more detectable in bile than in blood.AIM To investigate the effects of CMV infection on biliary complications by comparing the levels of CMV-DNA in the bile and blood of patients after LT.METHODS We conducted a retrospective analysis of 57 patients who underwent LT,10 of these patients had no biliary complications and 47 patients had biliary complications.We also compared the levels of CMV-DNA in patients’bile and blood,which were sampled concurrently.We used RNAscope technology to identify CMV in paraffin-embedded liver sections.RESULTS CMV-DNA was not detected in bile samples and was detected in 2 blood samples from patients without biliary complications.In the 47 patients with biliary complications,CMV-DNA was detected in 22 bile samples and 8 blood samples,both bile and blood samples were positive for CMV-DNA in 6 patients.The identification rate of CMV-DNA in blood was 17.0%,and was 46.8%in bile.Moreover,tissue samples from 4 patients with biliary complications tested positive using RNAscope technology but were negative with hematoxylin and eosin staining.During the follow-up period,graft failure occurred in 13 patients with biliary complications,8 of whom underwent retransplantation,and 3 died.CMV-DNA in bile was detected in 9 of 13 patients with graft failure.CONCLUSION In patients with biliary complications,the identification rate of CMV-DNA in bile was higher than that in blood.Blood CMV-DNA negative patients with biliary complications should still be monitored for CMV-related biliary tract diseases.Potential occult CMV infection may also be a contributing etiological factor in the development of graft failure.
基金Beijing Municipal Science&Technology Commission,No.Z181100001718220.
文摘BACKGROUND Sinusoidal obstructive syndrome(SOS)is a disease that damages hepatic sinusoidal endothelial cells,resulting in progressive occlusion and fibrosis of the lobular central vein and the occurrence of intrahepatic sinusoidal portal hypertension.However,SOS after liver transplantation(LT)is uncommon and potentially fatal.Here,we report a rare case of second-time recurrence of SOS after liver retransplantation(rLT).CASE SUMMARY A 22-year-old woman received a living donor LT due to SOS.Four years later,she developed abdominal distention and ascites with no apparent cause.She was diagnosed with recurrence of SOS and underwent rLT.But 2 mo post rLT,the patient suffered from aggravated jaundice and ascites again.She was diagnosed with second-time recurrence of SOS post-rLT according to computed tomography and liver pathology.After treatment with warfarin anticoagulation and immunosuppressant conversion,she gradually recovered with improvement of liver function and liver pathology.During the 17-mo follow-up period,she was in good condition with normal liver function and no ascites.CONCLUSION SOS can be a recurrent disease after LT,and autoimmune antibody and genetic sequencing should be screened before LT.For susceptible patients,anticoagulant drugs should be used for an extended period,and tacrolimus or other pathogenic agents should be avoided.Early diagnosis and treatment can improve the prognosis of patients and avoid graft failure or death.
基金Supported by Beijing MunicipalScience & TechnologyCommission, No.Z181100001718220.
文摘BACKGROUND Status epilepticus in patients with hepatic encephalopathy (HE) is a rare butserious condition that is refractory to antiepileptic drugs, and current treatmentplans are vague. Diagnosis may be difficult without a clear history of cirrhosis.Liver transplantation (LT) is effective to alleviate symptoms, however, there arefew reports about LT in the treatment of status epilepticus with HE. To ourknowledge, this is the first report of status epilepticus present as initialmanifestation of HE.CASE SUMMARY A 59-year-old woman with a 20-year history of heavy drinking was hospitalizedfor generalized tonic-clonic seizures. She reported no history of episodes of HE,stroke, spontaneous bacterial peritonitis, ascites or gastrointestinal bleeding.Neurological examination revealed a comatose patient, without papilledema.Laboratory examination suggested liver cirrhosis. Plasma ammonia levels uponadmission were five times normal. Brain computed tomography (CT) was normal,while abdominal CT and ultrasound revealed mild ascites, liver cirrhosis andsplenomegaly. Electroencephalography (EEG)showed diffuse slow waves rhythm,consistent with HE, and sharp waves during ictal EEG corresponding to clinicalsemiology of focal tonic seizures. The symptoms were reversed by continuousantiepileptic treatment and lactulose. She was given oral levetiracetam, and focalaware seizures occasionally affected her 10 mo after LT.CONCLUSION Status epilepticus could be an initial manifestation of HE. Antiepileptic drugs combined with lactulose are essential for treatment of status epilepticus with HE,and LT is effective to prevent the relapse.
基金supported by grants from Capital’s Funds for Health Improvement and Research(No.2020-1-2024)Beijing Municipal Science&Technology Commission(No.Z211100002921026).
文摘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.
基金supported by grants from Capital’s Funds for Health Improvement and Research (Grant Number:2020-1-2024)Research Foundation of Beijing Friendship Hospital,Capital Medical University (No.yyqdkt2017-27).
文摘Background and Aims:Patients with biliary atresia(BA)are prone to hepatic decompensation,which might eventually lead to death.This study aimed to identify the possible risk factors affecting in-hospital death in BA patients in China.Methods:We collected data from the Hospital Quality Monitoring System,a national inpatient database.All patients aged up to 2 years old with a diagnosis of BA were included.The subjects were divided to three groups,including Kasai portoenterostomy(KP),liver transplantation(LT),and no surgery.Logistic regression with Firth’s method was performed to identify potential influencing variables associated with in-hospital death.Results:During the year 2013 to 2017,there were 14,038 pediatric admissions with a diagnosis of BA.The proportion of in-hospital death in pediatric BA admissions was 1.08%.Compared with patients under six months,there was a higher risk of in-hospital death for children aged six months to 1 year and 1–2 years old.Clinical signs,including cirrhosis,variceal bleeding,and hepatic encephalopathy,were significantly associated with the risk of in-hospital death.In no surgery group,compared to those in Beijing and Shanghai,BA patients admitted in other districts had a lower risk of in-hospital death(OR=0.39,95%CI:0.21,0.70).However,in the LT group,patients admitted in other districts had a higher risk of in-hospital death(OR=9.13,95%CI:3.99,20.87).Conclusions:In-hospital survival remains unsatisfactory for pediatric BA patients with severe complications.Furthermore,more resources and training for BA treatment,especially LT,are essential for districts with poor medical care in the future.
文摘The fame of regeneration has been attributed to the liver ever since the era of Greek mythology,in which Prometheus’liver could grow back overnight against the daily predation of Zeus’eagle.Hepatectomy is the very basis of liver surgery including liver transplantation,and post-hepatectomy liver regeneration remains the center of interest for both clinicians and scientists.Novel tools for structural and functional assessment of liver regeneration have been constantly developed and validated,laying the groundwork for clinical management and scientific analysis(1).
基金grants from the National Natural Science Foundation of China(No.71603272)National Science and Technology Major Project(No.2017ZX10203205-001-005).
文摘Background:Although a number of technical problems and donor safety issues associated with living donor liver transplantation(LDLT)have been resolved,some initial clinical studies showed an increased risk of hepatocellular car cinoma(HCC)recurrence in LDLT.This meta-analysis was conducted to assess differences in tumor recurrence between LDLT and deceased donor liver transplantation(DDLT).Methods:After systematic retrievals of studies about LDLT and DDLT for HCC,articles were selected with a rationale of emphasizing inter-group comparability.Results from multivariate analyses were combined and discussed together with univariate analyses.In subgroup analysis,the impact of organ allocation policy was taken into consideration.Results:Seven articles were included in the meta-analysis.Overall,a salient result that emerged from the seven studies was a significant increased risk of HCC recurrence in the LDLT group than in the DDLT group(P=0.01).The most significant increase in hazard ratio was found in studies where organs tended to be allocated to non-tumor patients.Conclusions:An increased risk for HCC recurrence in LDLT as compared with DDLT patients was found.The relatively shorter preoperative observation windows in LDLT may lead to fewer cases of HCC with invasive features being screened out,which may provide a possible explanation for the high rates of HCC recurrence.
文摘Background: The outcome of pediatric deceased donor liver transplantation (LT) has not been well studied, especially pediatric deceased donor livers used in adult transplantation. This study aimed to evaluate the efficacy of LT using pediatric deceased donor livers and compare the outcomes between pediatric-to-pediatric LT and pediatric-to-adult LT. Methods: A retrospective review of LT using pediatric deceased donor livers from June 2013 to August 2016 was performed. The patients were divided into the pediatric-to-pediatric LT group and pediatric-to-adult LT group based on the ages of the recipients. The survival and incidence of early vascular complications (VCs) were observed between the two groups. We also analyzed the risk factors of early VCs in pediatric LT and the effect of donor hypernatremia on the prognosis of recipients. Results: There were 102 cases of LT using pediatric deceased donor livers in our hospital from June 2013 to August 2016. 83 pediatric-to-pediatric LT (recipients' age ≤13 years) and 19 pediatric-to-adult LT (recipients' age≥19 years). The ratio of early VC was similar in the two groups (19.3% vs. 10.6%, P = 0.514). Low body weight of recipient was an independent risk factor of early VC in pediatric LT (odds ratio: 0.856, 95% confidence interval: 0.752-0.975, P = 0.019). The 1-year cumulative survival rates of grafts and patients were 89.16% and 91.57% in pediatric-to-pediatric LT and 89.47% and 94.74% in pediatric-to-adult LT, respectively (all P 〉 0.05). In all cases. patients using donors with hypernatremia (serum sodium levels ≥ 150mmol/L) had worse graft survival (χ2=4.330, P = 0.037). Conclusions: Pediatric-to-pediatric LT group has similar graft and patient survival rates with those of pediatric-to-adult LT group. Low body weight of recipients is an independent risk factor of early VC in pediatric LT. Patients using donors with hypernatremia have worse graft survival.