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Objective:To investigate the application value of single-port laparoscopic left lateral donor liver acquisition in pediatric living donor liver transplantation (PLDLT).Methods:The retrospective and descriptive study was conducted. The clinical data of the donor and recipient who were admitted to Beijing Friendship Hospital of Capital Medical University for PLDLT in January 2020 were collected. The donor was a male, aged 28 years with body mass as 62 kg, height as 174 cm and body mass index (BMI) as 20.5 kg/m 2. The recipient was the daughter of the donor, aged 1 year with body mass as 9 kg, height as 75 cm and BMI as 16.0 kg/m 2. The donor underwent single-port laparoscopic left lateral donor liver acquisition. The recipient underwent living donor liver trans-plantation by the same operation team. Observation indicators: (1) intraoperative conditions; (2) postoperative conditions; (3) follow-up. Results:(1) Intraoperative conditions. The donor under-went single-port laparoscopic left lateral donor liver acquisition successfully, with the single-port access system being placed through a transumbilical incision. The operation time, the warm ischemia time of the donor liver and volume of intraoperative blood loss were 240 minutes, 3 minutes and 40 mL, respectively, of the donor. The weight of the donor liver was 233.6 g, and the corrected graft-to-recipient body weight ratio was 2.60%. The recipient underwent living donor liver transplantation successfully. (2) Postoperative conditions. The donor began to take liquid diet at postoperative day 1, and results of laboratory examination showed that the alanine aminotransferase (ALT), aspartate aminotransferase (AST), glutamyltransferase (GGT) and total bilirubin (TBil) of the donor was 239 U/L, 116 U/L, 53 U/L and 22.57 μmol/L. The donor began to take diet with high quality proteins at postoperative day 2, and to get for out-of-bed activities moderately. The donor′s peritoneal drainage fluid was light red at postoperative day 3, and no fluid accumulation was found in the operation area after abdominal B-ultrasound examination, so the peritoneal drainage tube was removed. The donor was discharged at postoperative day 4. The liver function of the recipient recovered to normal level 2 weeks after the operation. (3) Follow-up. The donor was followed up by outpatient examination 2 weeks after discharged, and results of laboratory examination showed that the ALT, AST, GGT and TBil was 44 U/L, 25 U/L, 53 U/L and 9.22 μmol/L, respectively. Neither the donor nor the recipient had complication ≥Ⅱ grade of the Clavien-Dino classification, such as biliary fistula and vascular complication during the 6 months after operation.Conclusion:Single-port laparoscopic left lateral donor liver acquisition can be used into the PLDLT.
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Objective To evaluate the clinical efficacy of liver transplantation in children with Alagille syndrome (ALGS). Methods Clinical data of 12 children with ALGS were collected and retrospectively analyzed. Clinical characteristics of children with ALGS, pathological characteristics of liver tissues, characteristics of liver transplantation, postoperative complications and follow-up of children with ALGS were analyzed. Results JAG1 gene mutation and typical facial features was present in all 12 children. Jaundice was the most common initial symptom, which occurred at 7 (3, 40) d after birth. Upon liver transplantation, the Z scores of height and body weight were calculated as -2.14 (-3.11, -1.83) and -2.32 (-3.12, -1.12). Five children developed severe growth retardation and 4 children with severe malnutrition. Eight of 12 children were diagnosed with cardiovascular abnormalities. Pathological examination showed that the lobular structure of the diseased livers of 4 children was basically maintained, and 8 cases of nodular liver cirrhosis in different sizes including 1 case of single early moderately-differentiated hepatocellular carcinoma. Three children were misdiagnosed with biliary atresia and underwent Kasai portoenterostomy. Eight children underwent living donor liver transplantation, three children underwent cadaveric donor liver transplantation (two cases of split liver transplantation and one case of cadaveric total liver transplantation), and one child underwent domino liver transplantation (donor liver was derived from a patient with maple syrup urine disease). during the follow-up of 30.0(24.5, 41.7) months, the survival rates of the children and liver grafts were both 100%. During postoperative follow-up, the Z scores of height and body weight were calculated as -1.24 (-2.11, 0.60) and -0.83 (-1.65, -0.43), indicating that the growth and development of the children were significantly improved after operation. Conclusions Liver transplantation is an efficacious treatment for children with ALGS complicated with decompensated cirrhosis, severe itching and poor quality of life. For children with ALGS complicated with cardiovascular abnormalities, explicit preoperative evaluation should be delivered, and consultation with pediatric cardiologists should be performed if necessary.
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Objective:To explore the potential immune mechanism of pediatric ABOi-LDLT presenting low humoral immune response to donor specific blood group antigen.Methods:From June 2013 to December 2020, clinical data were retrospectively reviewed for 29 patients of long-term surviving pediatric ABOi-LDLT.There were A to O ABOi-LDLT( n=10)and B to O ABOi-LDLT( n=19). Graft types included left lateral lobe( n=26)and left hemi-liver( n=3). The median age of liver transplantation was 10 months, the median weight 8.0 kg and the median follow-up time 41.9 months.The titers of donor specific blood group antibodies and non-donor specific blood group antibodies(IgG, IgM)were continuously monitored before transplantation and at 1, 3, 6, 12, 24, 36 months post-transplantation.Protocol or event-based liver biopsy was performed to determine whether or not there was antibody-mediated rejection. Results:The titer of donor specific blood group antibody in recipients was significantly lower than that of non-donor specific blood group antibody( P<0.001). Among 18 protocol liver pathological biopsies, two cases were C4d positive for vascular endothelium.Five abnormal event-based liver biopsies were completed and one was C4d positive in bile duct endothelium.No pathological sign of typical blood group antibody mediated antigen-antibody complex mediated cascade immune reaction was detected in liver pathological biopsy.Typical pathological signs of blood group antibody mediated rejection were absent in protocol liver biopsy. Conclusions:Donor specific blood group antibody is expressed at a low level in pediatric ABOi-LDLT recipients.It presents as incomplete immune tolerance to donor specific blood group antigen.
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Objective:To explore the characteristics and significance of Epstein-Barr virus-infected lymphocyte cell types in peripheral blood mononuclear cells(PBMC)in post-transplant lymphoproliferative disorder(PTLD)after pediatric liver transplantation.Methods:From June 2013 to March 2021, retrospective data analysis was performed for 14 pediatric liver transplant recipients with PTLD.The determination of EBV-DNA in PBMC, plasma and TBNK lymphocyte cells was analyzed.Results:EBV-DNA in PBMC showed a high viral load(>10 4 copies/ml)and plasma EBV-DNA was >10 3 copies/ml( n=8). There were dominant B-cell-type infection( n=12)and T/NK-cell-type infection( n=2). After treatment, EBV-DNA in PBMC and plasma turned negative in 7 patients with a decline( n=6)and an increase( n=1). EBV-DNA in B lymphocyte became negative( n=10)with a decline( n=3). In one case, EBV-DNA increased in T, B and NK cells with a high viral load.The remainders recovered after treatment.One case of hemophagocytic syndrome died from a progression of PTLD. Conclusions:A large majority of EBV-related PTLD are dominated by B-cell-type infection and a few belong to T or NK-cell-type infection.Patients with T/NK-cell-type infection have a worse response to therapy and poorer prognosis than those with B-cell-type infection.Determination of EBV-infected lymphocyte cell types is of vital research value for treatment and prognosis.
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Objective To evaluate the efficacy of liver transplantation for acute liver failure (ALF) in children. Methods Clinical data of 15 children with ALF who underwent liver transplantation were collected and retrospectively analyzed. The proportion of ALF among children undergoing liver transplantation during the same period was calculated. The characteristics, postoperative complications and clinical prognosis of ALF children receiving liver transplantation were analyzed. Results In the same period, the proportion of ALF was 2.0% (15/743) among pediatric recipients undergoing liver transplantation. All 15 children had acute onset of ALF, and most of them were accompanied by fever, diarrhea and progressive yellowing of skin and sclera. Thirteen children were complicated with hepatic encephalopathy before operation (6 cases of stage Ⅳ hepatic encephalopathy), and two children were complicated with myelosuppression and granulocytopenia before liver transplantation. Ten children underwent living donor liver transplantation with relative donor liver, 4 received liver transplantation from donation after cardiac death (DCD), and 1 underwent Domino donor-auxiliary liver transplantation. Of 15 children, 12 recipients had the same blood type with their donors, 1 recipient had compatible blood type with the donor and 2 cases had different blood type with their donors. Among 15 children, 10 cases developed postoperative complications. Postoperative cerebral edema occurred in 5 cases, of whom 4 cases died of diffuse cerebral edema, and the remaining case was in a persistent vegetative state (eyes-open coma). Postoperative cytomegalovirus (CMV) infection was seen in 5 cases. Two children presented with aplastic anemia and survived after bone marrow transplantation, 1 case died of CMV hepatitis and viral encephalitis, and 2 cases died of diffuse brain edema. One child developed graft-versus-host disease (GVHD) after liver transplantation, and died of septic shock after bone marrow transplantation. Nine children survived and obtained favorable liver function during postoperative follow-up. Conclusions Liver transplantation is an efficacious treatment for ALF in children, which may enhance the survival rate. Brain edema is the main cause of death in ALF children following liver transplantation, and treatment such as lowering intracranial pressure, improving brain metabolism and blood purification should be actively performed. Liver transplantation should be promptly performed prior to the incidence of irreversible neurological damage in ALF children, which might prolong the survival and enhance long-term prognosis.
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Objective:To explore the feasibility of using vascular graft interposition for lowering the complications of portal vein during pediatric liver transplantation.Methods:From June 1, 2013 to May 31, 2018, clinical data were collected for 297 children undergoing liver transplantation, including basic demographics, general preoperative status, preoperative tests, imaging findings, graft related profiles, surgical procedures and postoperative follow-ups, etc. Then the authors analyzed the effect of using interposition vessels upon lowering postoperative complications of portal vein reconstruction.Results:With a median age of 12 months, there were 153 boys (51.5%) and 144 girls (48.5%). The primary disease was mostly biliary atresia ( n=222, 74.7%). The median diameter of portal vein was 5 mm. There were 19 cases (6.4%) using vascular interposition. Among 20 cases of portal vein complications, there were portal vein stenosis ( n=17, 5.7%) and portal vein thrombosis ( n=3, 1.0%). After univariate analysis, binary Logistic regression analysis revealed that diameter of recipient's portal vein was an independent risk factor for the occurrence of portal vein complications after liver transplantation. Statistical analysis of children with portal vein diameter <4 mm ( n=90) was carried on and the results showed that there was no inter-group statistical difference ( χ2=3.061, P=0.080)on the occurrence of portal vein complications. Conclusions:Diameter of portal vein is an important factor affecting the strategic choice of portal vein reconstruction during pediatric liver transplantation and an independent risk factor for portal vein complications after liver transplantation. When the diameter of portal vein is ≤4 mm, using interposition vascular anastomosis shows no significant difference with other conventional modes.
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To analyzed a case of pediatric patient with propionic acidemia combined with dilated cardiomyopathy retrospectively, who underwent living donor liver transplantation at the Liver Transplantation Center, Beijing Friendship Hospital, Capital Medical University in March 2019.A 2 years and 6 months female child was admitted to hospital for propionic acidemia.The pretransplant echocardiogram showed left ventricular dilatation and systolic dysfunction, and thus dilated cardiomyopathy was considered.A living donor liver transplant was performed using her mother′s left latera-llobe.On the 14 months postoperatively, the child was on a liberated protein diet, but still required levocarnitine supplementation.Her hepatic and cardiac function returned normal, but growth retardation was still present.During the follow-up period, further propionic acidemia-related complications like metabolic decompensation, or any transplant-related complications were not reported.This case report suggested that liver transplantation is effective on pediatric propionic acidemia combined with cardiomyopathy, which reverses cardiomyopathy, improves cardiac function, relieves strict protein restriction, reduces the risk of metabolic decompensation, and significantly improves quality of life.
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Objective To summarize the incidence, diagnostic and therapeutic experience of hepatic sinusoidal obstruction syndrome (HSOS) after liver transplantation. Methods Clinical data of 4 patients with HSOS after liver transplantation were retrospectively analyzed. The incidence, clinical manifestations, imaging and pathological characteristics of HSOS after liver transplantation were collected, and the treatment methods and clinical outcomes of patients with HSOS were analyzed. Results The incidence of HSOS after liver transplantation was 0.8%(2/239), and the median time of onset was 4.5(1.7, 9.0) months after liver transplantation. The clinical manifestations of HSOS mainly included abdominal distension, ascites, hepatomegaly, increased bilirubin, and renal insufficiency in partial cases. Enhanced abdominal CT scan of 4 patients with HSOS showed uneven spot-like enhancement and the liver histopathological examination mainly showed the signs of hepatic sinusoidal dilatation complicated with congestion. Four patients were administered with an adjusted regime of immunosuppressant by replacing tacrolimus (Tac) with ciclosporin and adding anticoagulant therapy with warfarin. One patient received transjugular intrahepatic portosystemic shunt (TIPS). After treatment, the symptoms of 3 patients were completely relieved, and 1 patient died. One of the 3 surviving patients died from pulmonary infection and gastrointestinal bleeding. Conclusions HSOS is a rare and fatal complication after liver transplantation. Timely diagnosis and treatment can avoid the incidence of graft failure and improve clinical prognosis of the patients.
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Objective:To review our experience in the use of "Full right-Full left" split liver transplantation in adult-to adult or adult-to adult-size child.Methods:The clinical data of liver donors to 4 recipients of full right-full left split liver transplantation performed at Beijing Friendship Hospital of Capital Medical University from January to December 2019 were reviewed. The surgical methods of split liver transplantation, cold ischemia time, operation time, intraoperative blood transfusion, and postoperative complications and prognosis were analyzed.Results:The 4 recipients of complete right hepatic-left hepatic split liver transplantation included 3 adults and 1 heavy child (45 kg). Their ages ranged from 14 to 48 years, and body weight ranged from 45 to 61 kg. The end-stage liver disease model score were 21, 12, 41, and 30 points. The ratios of graft mass to recipient's body mass ranged from 0.85% to 1.35%. The cold ischemia time was 457-650 min, and the operation time was 460-575 min. Early liver function recovered smoothly in all the 4 patients after transplantation, and no small liver syndrome occurred. Patients were followed up to 6 months after operation. One patient developed anastomotic biliary leak, which was cured by endoscopic retrograde cholangiopancreatographic treatment. Another patient developed biliary stricture presenting with repeated biliary tract infection despite percutaneous transhepatic puncture biliary drainage. A third patient died six months from lung infection.Conclusion:In properly selected patients, using full right-full left hemiliver by split liver transplantation increased organ utilization and provided patients with increased treatment opportunities.
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Objective:To study the hepatic hemodynamics changes and pathophysiological mechanisms of the use of a functional shunt after auxillary liver transplantation to treat portal hypertension associated with a small-for-size graft.Methods:A retrospective analysis of the clinical data of patients with portal hypertension treated with functional shunting of small-volume grafts from a living donor liver at the Beijing Friendship Hospital, Capital Medical University from July 2014 to December 2018, and a total of 6 patients were included as the research objects, including 4 males and 2 females, with a median age of 35.5 (29.0-52.0) years old. Blood flow monitoring data were collected during and after operation, and the characteristics of liver hemodynamics were analyzed.Results:The portal venous blood flow of the remnant native liver gradually decreased to no flow. As a buffer response, the flow velocity of hepatic artery increased. The portal venous blood flow of the graft gradually increased in the early postoperative period and then gradually decreased from post-operation Day 5 to 10 due to gradual increase in portal venous resistance. However, the portal venous perfusion gradually increased from Day 10 after the operation, reached to a level and declined to a stable level about 1 month after the operation. The volume of abdominal drainage slowly decreased after the peak level at Day 5-10 after the operation, and disappeared completely at Day 30 after operation.Conclusions:When using auxiliary liver transplantation for functional shunting to treat portal hypertension, autologous residual liver can act as a guide buffer for the pressure gradient of portal vein hyperperfusion in liver transplantation, and reach a steady state of blood flow distribution about 1 month after surgery, while relying on autologous remnant liver hepatic artery buffer response prevents small liver syndrome.
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Objective To evaluate the effect of liver transplantation on intestinal microflora in children with biliary atresia. Methods The fecal samples and liver function indexes of 16 children with biliary atresia before and 6 months after liver transplantation were collected, and 10 healthy children were selected as the healthy controls. DNA extraction and metagenome sequencing were carried out in the fecal samples. Statistical analysis was performed by software packages, such as R language. The changes of species structure and functional composition of intestinal microflora after liver transplantation were analyzed. The recovery of intestinal microflora in children with biliary atresia after liver transplantation was assessed. The relationship between intestinal microflora and liver function indexes was investigated. Results Following liver transplantation, the number of species of intestinal microflora in children with biliary atresia was increased. The opportunistic pathogens were the dominant species of intestinal microflora in children with biliary atresia before liver transplantation. The abundance of opportunistic pathogens was decreased and the abundance of short-chain fatty acid-producing bacteria was increased after liver transplantation (all P < 0.05). Following liver transplantation, lipid metabolism, amino acid metabolism, carbohydrate metabolism, energy metabolism, metabolism of cofactors and vitamins were enhanced, whereas infectious diseases of bacterial, immune diseases and drug resistance were weakened. Compared with the healthy control group, there were no statistically significant differences in the diversity and structure of intestinal microflora in the post-liver transplant group, but different species were observed between two groups. The liver function indexes of children with biliary atresia after liver transplantation tended to decline (all P < 0.000 1). The abundance of beneficial intestinal microflora was negatively correlated with liver function indexes, whereas the abundance of opportunistic pathogens was positively correlated with liver function indexes (all P < 0.05). Conclusions Liver transplantation may significantly improve the structure and functional composition of intestinal microflora in children with biliary atresia.
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Objective To summarize the incidence, diagnosis and treatment experience of posttransplant lymphoproliferative diseases (PTLD) in the liver transplant recipients. Methods Clinical data of 734 liver transplant recipients were retrospectively analyzed. The incidence, clinical symptoms, laboratory and imaging data of PTLD in liver transplant recipients were collected. The pathological results and treatment methods of PTLD recipients were analyzed. The prognosis of PTLD recipients was evaluated. Results The incidence of PTLD in liver transplant recipients was 2.2% (16/734). The median time of onset after operation was 8(3, 46) months. The main clinical manifestations of PTLD were fever and lymph nodes enlargement. Some patients developed anemia, hepatosplenomegaly, abnormal liver function and digestive system symptoms, etc. Among 16 PTLD recipients, 1 case showed abnormal increase in blood concentration of tacrolimus, 6 cases of elevated transaminase levels, 14 cases of increased Epstein-Barr virus (EBV) DNA load and 5 cases of increased cytomegalovirus (CMV) DNA load. Positron emission tomography and computed tomography (PET/CT) showed hypermetabolism of 18F-flurodeoxyglucose in the enlarged lymph nodes of 13 recipients. CT scan of the neck and abdomen indicated multiple lymph node enlargement in the corresponding area of 2 recipients. Lymph nodes enlargement of 1 recipient showed on ultrasound only. All 16 PTLD recipients received pathological examination. In situ hybridization showed that EBV-encoded small RNA (EBER) was positive in 13 recipients. Reducing the immunosuppressant level was the basal treatment plan for PTLD recipients, and it can be combined with rituximab-targeted therapy and chemotherapy according to different pathological types of PTLD. Surgery and radiotherapy were used for enlarged lymph nodes. One recipient died of transplant liver failure due to PTLD treatment. Conclusions Administration of immunosuppressants after liver transplantation can increase the risk of PTLD. The incidence of PTLD is higher in pediatric liver transplant recipients than in adults. Early diagnosis and reasonable treatment can significantly improve the prognosis of PTLD recipients.
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Objective:To explore the clinical feasibility and efficacy of using donated liver procured from donors complicated with intra-peritoneal widespread dissecting aneurysm.Methods:One case of liver donation was assigned to our center from COTRS. Intra-peritoneal widespread dissecting aneurysm was detected intraoperatively with an involvement of coeliac trunk until artery superior to bifercation of HA (hepatic artery). HA reconstruction was extremely challenging. With the final attempt of using donors artery next to hilus lienis as a bridge vessel, success of reconstruction was achieved.Results:During an early postoperative period, satisfactory graft blood flow was established without surgical complications, the patient was discharged smoothly. At Month 13, blood flow of graft remained decent.Conclusions:Through a review of the relevant articles, a few cases have been successful using of donated liver from donors with intra-peritoneal dissecting aneurysm as long as proper hepatic artery is not involved and the difficulty of HA reconstruction remains relatively low. As for widespread intra-peritoneal dissecting aneurysm, donor liver should be employed cautiously.
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Objective To analyze the clinical characteristics, pathogenic causes and therapeutic experience of right diaphragmatic hernia after pediatric living donor liver transplantation. Methods Clinical data of 3 recipients with right diaphragmatic hernia after pediatric living donor liver transplantation were retrospectively analyzed. The clinical characteristics, diagnosis and treatment process and therapeutic experience were analyzed and summarized. Results The primary diseases of 3 children with diaphragmatic hernia after living donor liver transplantation were biliary atresia. The diaphragmatic hernia occurred at 4-6 months after liver transplantation. The contents of diaphragmatic hernia included the intraperitoneal and interperitoneal tissues and organs. Diaphragmatic defects were all located in the posterior medial area of the right diaphragm. The primary stage intermittently suturing repair was performed during intraoperative period. No diaphragmatic hernia recurred during long-term follow-up. Conclusions The clinical manifestations of right diaphragmatic hernia after pediatric living donor liver transplantation are diverse. The risk factors include malnutrition, low body weight, surgical trauma, chemical erosion caused by bile leakage, focal infection and pleural-peritoneal pressure gradient, etc. Surgical intervention is the preferred treatment strategy for diaphragmatic hernia after liver transplantation.
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Objective To evaluate the effect of donor-derived infection on the clinical prognosis of the recipients undergoing liver transplantation. Methods Clinical data of 75 donors and recipients undergoing liver transplantation were retrospectively analyzed. According to the culture results of donor organ lavage fluid, all recipients were divided into the positive group (n=26) and negative group (n=49). Clinical parameters of the recipients during perioperative period were observed in the positive and negative groups. The sputum and peritoneal drainage fluid of the recipients undergoing liver transplantation were cultured. The incidence of postoperative infection of the recipients was observed. The 1.5-year survival curve of the recipients was analyzed by Kaplan-Meier method. Results In the positive group, the incidence of portal vein stenosis and thrombosis was significantly higher than that in the negative group (P < 0.05). Among 75 recipients undergoing liver transplantation, 33 cases (44%) developed postoperative infection mainly in the lung and abdominal cavity. The infection rate significantly differed between the positive group (77%) and negative group (27%, P < 0.05). In the positive group, sputum culture was positive in 10 recipients and peritoneal drainage culture was positive in 11 recipients. The sputum culture outcomes of 4 recipients were consistent with those of the organ lavage fluid culture of their donors. The peritoneal drainage culture results of 6 recipients were consistent with those of the organ lavage fluid culture of their donors. After anti-infection treatment, 2 recipients in the positive group died at postoperative 5 and 12 d, and the culture results of the remaining recipients were negative. In the negative group, 7 recipients were positive for sputum culture and 6 recipients were positive for peritoneal drainage culture. The culture results of all recipients were negative following anti-infection therapy. Two recipients died from graft failure at postoperative 1 month and 1 year. The 1.5-year survival rate did not significantly differ between the positive and negative groups (P > 0.05). Conclusions The effect of donor-derived infection on the early prognosis of liver transplant recipients cannot be neglected, whereas it exerts mild impact on the intermediate- and long-term clinical prognosis of the recipients.
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Objective To investigate the safety and effectiveness of anatomical partial splenectomy during liver transplantation in pediatric patients to prevent postoperative refractory hypersplenism.Methods From January 2015 to August 2018,7 pediatric patients with preoperative severe hypersplenism underwent anatomical subtotal splenectomy together with liver transplantation at our institution.Clinical informations,including operative time,intraoperative bleedinh,postoperative hospital stay,postoperative complications,platelet counts,leukocyte counts and the length and thickness of spleen determined by abdominal ultrasound,were collected retrospectively and statistically analyzed.Results The median total operation time was 495 min (320-768 min),the median intraoperative blood loss was 350 mL (300-1300 mL) and the median hospital stay was 19 days (14-55 days).Patients were followed up for 7.0-36.6 months (median 20.1 months).The length and thickness of spleen were reduced immediately from (18.89 ± 1.77) to (11.13 ± 2.28) cm (P＜0.001)and from (6.31 ± 0.53) to (4.97 ± 1.29) cm (P＜0.05),respectively.During the follow-up period of the first week,the mean platelet counts and leukocyte counts increased from (46.71 ± 18.91) × 109/L to (173.71 ± 73.15) × 109/L (P＜0.001) and from (1.59 ± 0.42) × 109/L to (11.12 ± 4.17) × 109/L (P＜0.001),respectively.During the one-year follow-up period,there was no residual splenic regrowth,and the peripheral blood cell counts remained normal.All patients survived to date with no procedure-related complications.Conclusions The anatomical subtotal splenectomy during liver transplantation in pediatric transplant recipients with preoperative severe splenomegaly and hypersplenism is a feasible option for the prevention of posttransplant refractory hypersplenism.
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Objective To retrospectively explore the clinical symptoms ,diagnosis ,treatment and prognosis of posttransplant lymphoproliferative disorder (PTLD) after pediatric liver transplantation .Methods The diagnosis and treatment of PTLD were reviewed for 3 children recipient with living donor liver transplantation .Their primary diseases were biliary atresia ,glycogen storage disease type III and ornithine-transcarbamylase deficiency . All of them received FK506 for immunosuppression therapy . They were diagnosed as PTLD at 7 ,8 ,6 months post-operation respectively .Their major clinical manifestations were non-specific ,including fever ,diarrhea and anemia .Positron emission tomography/computed tomography (PET/CT) and ultrasound revealed enlarged mesenteric lymph nodes with neck lymphoadenopathy (n=2) . Pathological examinations of resected enlarged lymph nodes indicated post-transplantation lymphoproliferative disorder .One case was diffuse large B cell lymphoma and two of them belonged to preliminary EBER + . Results After a definite diagnosis ,there was one cycle of R-CHOP regimen (rituximab ,cyclophosphamide , pirarubicin ,vincristine ,dexamethasone) or 2 cycles of rituximab along with a .reduction of anti-rejection drug and they stayed in remission .Three were followed up for 37 ,39 and 20 months respectively from May 31 , 2019 . Currently transplanted liver function was stable and EBV viral load remained negative persistently .Conclusions This case highlights the complexity of clinical presentations and co-morbidities of PTLD . Reducing immunosuppressive agents and using rituximab plus chemotherapy can achieve a satisfactory efficacy for Epstein-Barr virus-related PTLD patients after pediatric liver transplantation .
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Objective To analyze the donor specific antibody (DSA) in liver transplantation,and discuss the therapeutic schemes.Methods We retrospectively analyzed prospectively collected samples from 139 cases of liver transplantation from September 1,2013 to July 1,2015.Luminex assays were applied to determine human leukocyte antigen,panel reactive antibody (PRA).For PRA positive cases,DSA,C1q and C4d were detected,and liver biopsy was done.Results Of 139 cases enrolled,there were 12 cases positive for DSAs,including 2 cases of PreDSA:1 case of Ⅰ DSA (HLA-A mismatch),and 1 case of Ⅱ DSA (HLA-DQ mismatch).Ten cases of de novo DSA (including 1 case of PreDSA) all were HLA-DQ mismatch.The liver biopsy on 5 cases showed hepatic fibrosis,early rejection and intrahepatic cholestasis,and only 2 cases showed positive C4d.Of 6 cases of DSA,5 cases showed positive C1q.In the patients positive for DSA,tacrolimus dose was adjusted postoperatively,adding mycophenolatemofetil or increasing its dose,or methylprednisolone and immunoglobulin given.Conclusion DSAs are important indicators of sensitized recipients in liver transplantation,associated with trends toward worse outcomes in patients or allografts.The monitoring of DSA is requisite in order to adjust the immunosuppressant.
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Objective To Analyze the clinical outcomes of pediatric liver transplantation (LT) for liver-based metabolic disorders.Methods We conducted a retrospective analysis on 42 pediatric patients with liver-based metabolic disorders from June 2013 to March 2017,and analyzed the pediatric end stage liver disease model (PELD),growth and development,type of transplant,postoperative complications and prognosis of patients.Results There were 42 children with liver-based metabolic disorders (15.56%) out of all the 270 children who underwent LT.The median age was 51.0 months (range,3.4-160.9 months).Of the 42 children,19 received living donor liver transplantation (LDLT),18 cases received deceased donor liver transplantation (DDLT) and 5 cases received domino liver transplantation.1-,2-and 3-year cumulative survival rate of 42 recipients was 97.7%,93.6% and 93.6%,and that of the grafts was 95.3%,91.4% and 91.4%,respectively.As compared with the 194 children with biliary atresia who underwent LT,significant difference was found in PELD and weight Z-score between the two groups.Conclusion Liver transplantation is a valuable option for children with metabolic disorders,and it has gained a better prognosis.
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Objective To explore the factors associated with the quality of life (QOL) in patients after liver and kidney transplantation.Methods A multicenter cross-sectional survey was carried out in 5 Level Ⅲ Class A hospitals.153 liver transplant recipients and 301 kidney transplant recipients of outpatient clinic follow-up from March to December in 2015 were selected and investigated with a self-designed general state questionnaire,and Quality of Life Scale for Liver and Kidney Transplant Recipients which developed by our research group.Results There were significant differences in QOL total score in liver and kidney transplant recipients among groups of marital status and occupation.Divorced,unemployed and low-educated patients showed lower QOL total score than married,employed and high-educated ones (P ＜ 0.05).As compared with non-living-related transplantation group,patients undergoing living-related transplantation presented a better status in QOL total scores (P＜0.05).The QOL total score was obviously lower in patients suffering from complications and rejection than in those without occurrence of complications and rejection (P＜ 0.01).Postoperative time was correlated positively and significantly with QOL scores,and variances existed among different stages postoperation (P ＜ 0.01).Multivariable regression analysis demonstrated that education,marital status,postoperative time,type of donor and chemotherapy were the factors influencing liver transplant recipients' QOL,while marital status,postoperative time,type of donor,medical care assurance,complications and rejection after operation had effect on kidney transplant recipients.Conclusion Attaching importance to QOF among liver and kidney transplant recipients,and implementing scientific and effective nursing intervention based on the characteristics of them are necessary.