Clinical analysis of liver transplantation in progressive familial intrahepatic cholestasis

Shen Conghuan, Tao Yifeng, Li Ruidong, Zhang Quanbao, Zhang Xiaofei, Wang Zhengxin.

2018, (6): 424-427. DOI:10.3969/j.issn.2095-5332.2018.06.003

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Objective To explore the therapeutic benefit of liver transplantation （LT）on progressive familial intrahepatic cholestasis （PFIC）. Methods The clinical data of 5 cases with PFIC who underwent liver transplantation at Huashan Hospital affiliated to Fudan University from October 2015 to April 2018，were analyzed retrospectively. There are PFIC Ⅱ patients in 3 cases and 2 PFIC Ⅲ patients in 2 cases ；there were 3 cases male and 2 cases female with a median age of 34 months（9 ～ 102 months）；living donor liver transplantation with left lateral segment patient in 4 cases and classical orthotopic liver transplantation in 1 case. The clinical data， including pre-operative basic features， surgical situation， postoperative management and outcomes were analyzed to evaluate the curative effect of LT. Results All the surgeries achieved success and the liver function recovered smoothly. After the operation，1 case was complicated with lymphatic leakage and 1 case with intrahepatic bile duct dilation. One case was infected by the hepatitis B virus， cytomegalovirus and EB virus. All the children and grafts were alive after median follow up duration of 23 months（4 ～ 34 months）. The clinical manifestations of jaundice and pruritus were gradually vanished. No patient suffered from apparent acute or chronic rejection and vascular complications Conclusions LT is the definitive treatment for PFIC as it could correct the genetic defect.

The data analysis of UNOS pediatric liver transplantation in the United States

Wang Zhen, Zhang Haili, Teng Dahong, Cai Jinzhen, Zheng Hong, Shen Zhongyang.

2018, (6): 428-431. DOI:10.3969/j.issn.2095-5332.2018.06.004

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Objective To analyze the development of liver transplantation for children in the United States in recent ten years through the study of the public database of the United Network for Organ Sharing. Methods The data included the waiting list and the transplantation periods, the change of case number，age composition，gender， blood type，ethnicity，preoperative diagnosis and post-transplant survival rate were analyzed. Results In 2017， 725 new candidates were added to the pediatric liver transplant waiting list，less than in 2005（826 candidates）. The number of living donor pediatric liver transplants decreased from a peak of 79 in 2015 to 67 in 2017，most of the donors were closely related to the recipients. Conclusion Graft survival rate continued to improve over the past decades among recipients receiving deceased and living donor liver transplantations.

Observation of islet transplantation after liver transplantation in children

Wang Shusen , , Pei Guanghui , Wang Jinshan , Li Wei , Liu Yaojuan , Zhang Boya , Wang Zhiping , Zheng Hong , Shen Zhongyang,

2018, (6): 432-434. DOI:10.3969/j.issn.2095-5332.2018.06.005

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Objective To explore the safety and efficacy of islet cell transplantation through percutaneous trans-hepatic puncture of portal vein for treating type 1 diabetes on pediatric liver transplantation recipients. Methods The clinical data of one type 1 diabetic child undergoing islet cell transplantation after liver transplantation in Tianjin First Central Hospital was analyzed retrospectively. The donor pancreas was digested by injecting collagenase solution combined with neutral protease and the islets were purified using continuous density gradients centrifugation. Cultured islets were infused through percutaneous trans-hepatic portal vein puncture to the liver of a pediatric patient with type 1 diabetes after liver transplantation in Tianjin first center hospital. Theblood glucose，insulin dosage，C-peptide，liver and renal function after transplantation were monitored. After transplantation, the patient was treated with anti-rejection and anticoagulation therapy, the insulin was used according to the blood glucose. Results The yield of high purity islet was 212 073 islet equivalents（IEQ）. Islet viability was 95% while the glucose stimulation index（GSI）was 1.77. The transplant tissue volume was 1 ml. After islet transplantation，the fasting blood glucose of the child patient became stable gradually, and the daily insulin dosage gradually decreased. Exogenous insulin therapy was completely stopped 20 d post islet transplantation with normal fasting C-peptide levels. The patient's liver and renal function was stable while no complications after islet transplantation was observed. Conclusion The islet transplantation is effective and safe for treating children with type 1 diabetes after liver transplantation.

A retrospective analysis of 40 cases of pediatric renal transplantation.

Hu Shanbiao , Yu Shaojie , Peng Longkai , Xie Xubiao , Peng Fenghua , Lan Gongbin , Wang Yu , Guo Yong , Fang Chunhua , Nie Manhua , Li Ling

2018, (6): 435-39. DOI:10.3969/j.issn.2095-5332.2018.06.006

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Objective To investigate the clinical features of children's kidney transplantation, surgical method，postoperative immunosuppression and complications. Methods The clinical data of 40 cases of pediatric renal transplant patients in April 2003 to February 2017 were retrospectively analysed. There are 42 case-times kidney transplantations, thirty-one case-times were single kidney transplantations, of which 25 case-times were to anastomosis of the renal artery and the end of the internal iliac artery, and 6 case-times were to anastomosis of the renal artery and the recipient's external/temporal artery end ；In the double kidney transplantation, two case-times were separated double kidney transplantation, and 9 case-times were whole piece transplantation. Results A total of 40 children in the hospital completed 42 case-times kidney transplantations, two of which were re-transplanted. During the follow-up of 1 to 168 months, in these 40 recipients, single renal embolismt with isolated double kidney was in 2 cases ；perioperative pulmonary infection was in 4 cases , of which 2 were died, one was recovered dialysis and 1 were recovered after treatment ；delayed graft function（DGF）was in 4 cases ；acute rejection（AR）was in 4 cases ；urine leak was in 2 cases ；diabetes mellitus was in 1 case ；osteonecrosis of femoral head was in 1 case. The rest of the cases were well restored. The 1-year patient and graft survival rate was respectively 94.2 % and 91.2 %，respectively. Conclusion In spite of its high complications, pediatric renal transplantation is an effective treatment with its satisfactory one-year graft survival rate. Nevertheless, its long-term effect should be further observed.

Diagnosis and treatment of postoperative complications of living donor kidney transplant recipients

Wang Huailin, Liu Bin, Zhu Haidong, Wang Gang, Wang Yuantao, Zhou Honglan, Zhai Xiuyu

2018, (6): 440-443. DOI:10.3969/j.issn.2095-5332.2018.06.007

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Objective To summary the incidence of postoperative complications in 235 living donor kidney transplantation recipients，and improve diagnosis and treatment of complications after renal transplantation. Methods A retrospective analysis of the clinical data of 235 living donor kidney transplantation recipients admitted from January 2012 to June 2017 in the First Hospital of JiLin University，to analyze its postoperative recovery and complications. Results The mean serum creatinine was 100.1 μmol/L when 235 recipients leaving hospital，with 1 case died of respiratory failure and 4 cases of renal allograft failure，the recent reexamination of serum creatinine was 111.8 μmol/L in the remaining 230 patients. Following up for 6 ～ 59 months， complications included acute rejection，pulmonary infection，urinary tract infection，wound infection，renal artery infection，herpes zoster，urinary fistula and ureter obstruction after renal transplantation，delayed graft function， renal artery stenosis，renal allograft failure，immunosuppressant drug poisoning，diarrhea，myelosuppression， hepatic insufficiency，transplantation diabetes. The 1 year survival rates of the recipients and the transplanted kidneys were 99.8% and 98.7%，respectively. Preoperative use of antithymocyte（ATG）or bariximab could significantly reduce the incidence of postoperative rejection（P ＜ 0.05）. Conclusion The survival rates of the transplanted kidney could be improved effectively in some methods，such as preoperative comprehensive assessment，intraoperative detailed operation，regular follow-up and timely intervention of complications after operation.

Correlation between CHD8 protein and tumor recurrence after liver transplantation for hepatitis B virus associated hepatocellular carcinoma

Chen Huijuan , Wang Peixiao , Huang Lihong , Chen Xinguo , Zhang Hongying , Zhang Qing .

2018, (6): 444-447. DOI:10.3969/j.issn.2095-5332.2018.06.008

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Objective To investigate the correlation between chromodomain helicase DNA-binding protein 8（CHD8）and the clinical pathological features as well as postoperative recurrence of hepatitis B virus associated hepatocellular carcinoma（HCC）. Methods The clinical pathological data and long-term follow-up after surgery data of 251 patients with hepatitis B related HCC who underwent liver transplantation from January 2007 to June 2012 in the Third Medical Center of Chinese PLA General Hospital were analyzed retrospectively. The expression of CHD8 protein in liver specimens was detected by immunohistochemistry. The correlation between CHD8 protein and clinical pathological indexes，such as HBeAg，postoperative recurrence and metastasis were analyzed. Results The expression of CHD8 protein in tumor tissue was lower than that in adjacent tissues（P ＝ 0.00），it was significantly correlated with microsatellite（P ＝ 0.00），vascular invasion（P ＝ 0.00），metastasis of other parts（P ＝ 0.00）. The expression of CHD8 protein was also associated with HBeAg（P ＝ 0.04），recurrence（P ＝ 0.01）and lung metastasis （P ＝ 0.04）. Conclusion The expression of CHD8 protein is associated with metastasis and recurrence after liver transplantation for hepatitis B virus associated HCC，and it maybe a potential marker for tumor recurrence and metastasis.

Application analysis of a new type of double kidney normal temperature mechanical perfusion preservation device

Gao Xinpu, , Zhang Weiye , , Shi Yuan , Fan Pengfei , Wang Qingping , Shen Zhongyang

2018, (6): 448-452. DOI:10.3969/j.issn.2095-5332.2018.06.009

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Objective To discuss the significance and mechanism of normothermic machine perfusion （NMP）for the preservation of marginal donor kidney. Methods A total number 10 small pigs were randomly divided into two groups，experimental group（n ＝ 5）：bilateral donor kidneys were preserved after 30 min of warm ischemia，and autologous kidney transplantation were performed after 7 h preservation with NMP device ；control group（n ＝ 5）：bilateral donor kidneys were preserved after 30 min of warm ischemia，and autologous kidney transplantation were performed after 7 h preservation with UW solution. At each time point，serum creatinine level， renal inflammatory cytokine expression，apoptosis，renal cortical microcirculation changes and survival rate were compared between the two groups. Results The kidneys in both groups were preserved and transplanted，and the success rate of surgery was 100%. During the period of kidney preservation, the pressure and blood flow were stable. At 24 h after transplantation，the serum creatinine level in the experimental group was significantly lower than that in the control group. The levels of ET-1 and caspase-3 in the renal tissue were decreased markedly，and the bloodflow in the renal cortex was increased significantly（P ＜ 0.05）. There was no significant difference in survival rate between two groups（P ＞ 0.05）. Conclusions NMP preservation could significantly reduce preservation damage and ischemia-reperfusion injury. The protective mechanism may result from reducing the expression of ET-1 in renal tissue and improving the microcirculation of the donor kidney，and reducing the level of caspase-3 to reduce ischemia-reperfusion injury.

Investigations on the willingness of organ donation and its impact factors among middle school students

Zeng Hui , Lv Yanyan , Liu Ruihua , Liu Zhuyun , Zhang Zanmei , Gao Lingling

2018, (6): 453-457. DOI:10.3969/j.issn.2095-5332.2018.06.010

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Objective To investigate the impact factors of the willingness of organ donation among middle school students and effective measures to raise public awareness and willingness towards organ donation. Methods Questionnaires including demographic data，donation willingness，knowledge and attitude，death attitude，altruism behavior and family discussion about organ donation were delivered to 228 middle school students in Guangzhou. The correlation between the variables and the willingness of organ donation was analyzed with Spearman rank correlation， and the multivariate analysis was conducted by multiple linear regression model. Results The scores of willingness of self organ donation，willingness of relatives' organ donation，knowledge and attitude toward organ donation among middle school students were 2.29±0.88，3.46±1.11，2.30±1.61，110.11±24.68，respectively. The score on the neutral-acceptance dimension of death attitude was the highest 19.28±4.44. The score of altruism behavior was2.98±0.82. The score of willingness to communication scale was 4.28±1.54. The students with history of blood donation and plan to regularly donate blood had higher willingness to donate organs（P ＝ 0.004）. Willingness of organ donation was positively correlated with attitude towards organ donation，altruism behavior，willingness to communicate and willingness of relatives' organ donation（P ＜ 0.05）. Approach acceptance dimension of death attitude was negatively relevant with willingness of organ donation（P ＜ 0.05）. Conclusion The willingness of organ donation among middle school students were on the average level and its predictors were attitude toward organ donation and altruism behavior. Measurement such as reinforcement of propagenda education，incentive of altruism behavior and improvement of compensation and legislation systems may contribute to the enhancement of the public willingness to donate organ.

De novo autoimmune hepatitisonearly periodinliver transplantation recipients combined with hemochromatosis and hepatitis B infection：a case report and review

Fan Tieyan, Chen Hong, Qiu Shuang.

2018, (6): 458-460. DOI:10.3969/j.issn.2095-5332.2018.06.011

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Objective To retrospectively analyze the clinical and prognostic features，and the diagnosis and treatment strategies of denovo autoimmune hepatitis in patients combined with hepatitis B infection and hemochromatosis after liver transplantation in the early postoperative period． Methods In the past 10 years， 1 patient with hemochromatosis combined with hepatitis B who received liver transplantation in our hospital was diagnosed with autoimmune hepatitis in the early postoperative period. New diagnostic criteria of denovo autoimmune hepatitis ：elevated serum IgG ；positive autoimmune antibody ；histological features of chronic hepatitis ；nonhepatitis. According to the specific criteria，6 ～ 7 points may be suspected with，more than 7 points can be diagnosed with autoimmune hepatitis. Results Only one female patient was dignosed with autoimmune hepatitis in 1 month after liver transplantation. γ globulin was 29.4%. IgG was 27.5 g/L. But autoantibody spectrum was normal. Transaminase was normal under the treatment of 12 mg/d of methylprednisolone after one month. Conclusion De novo autoimmune hepatitis after liver transplantation in the early postoperative period is rare. Early diagnosis and rational treatment are keys for improving its prognosis.

Living donor liver transplantation for an infant with neonatal intrahepatic cholestasis caused by citrin deficiency

Liu Yuan, Xia Qiang, Zhang Jianjun, Xue Feng, Xia Lei, Luo Yi, Qiu Bijun, Feng Mingxuan, Chen Xiaosong, Han Longzhi.

2018, (6): 461-463. DOI:10.3969/j.issn.2095-5332.2018.06.012

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Objective To report the prognosis of living donor liver transplantation for an infant with neonatal intrahepatic cholestasis caused by citrin deficiency（NICCD）. Methods A 8-month-old boy was diagnosed as NICCD and received appropriate treatment after diagnosis. This patient received living donor liver transplantation donated by his heterozygous mother. Results The recipient and donor had an uneventful recovery after operation. Liver function returned to normal one week after transplantation and maintained normal during the 4-month follow-up. Liver biopsy after transplantation indicated biliary atresia with fatty liver. The metabolism of amino acid and ammonia returned to normal after transplantation and dicarboxylic aciduria was not detected. This patient is now receiving normal diet. Conclusion For children diagnosed with NICCD accompanied with end-stage liver disease，liver transplantation is an effective treatment. Meanwhile，for NICCD patients who developed end-stage liver disease after appropriate treatment，other accompanied congenital liver disease should be considered.

Therapeutic effect of parental liver transplantation and domino-assisted liver transplantation on childrenwith metabolic liver disease

Dong Chong, Gao Wei, Ma Nan, Sun Chao, Zhang Wei, Meng Xingchu, Qin Hong, Wu Bing, Shen Zhongyang.

2018, (6): 464-466. DOI:10.3969/j.issn.2095-5332.2018.06.013

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Objective To investigate the effect of living donor liver transplantation plus domino auxiliary liver transplantation in the treatment of metabolic liver disease in children. Methods The first patient with ornithine aminotransferase deficiency（OTCD）received living donor liver transplantation（left lateral liver）and the patient's right lobe was procured for domino auxiliary donor liver transplantation. At the same time，the recipient of domino auxiliary liver transplantation was type Ⅰ crigler-najjar syndrome. The right half of the liver with the middle hepatic vein was resected，The domino liver was retained for vascular and biliary reconstruction. Results Recipie nt who received living donor liver transplantation had normal and sustained liver function and normal blood ammonia， the patient who received domino auxiliary liver transplantation had normal bilirubin and blood ammonia，Abdominal CT examination followed up one year after the transplantation was normal. Conclusion Non-sclerosing metabolic liver diseases can be treated by liver transplantation, and their livers can be used as domino donor livers，this type of liver graft can be successfully applied to auxiliary liver transplantation of different metabolic liver diseases，thus it provids new ideas for patients with metabolic liver diseases to expand the source of donor livers.

The experience on organ procurement after brain death

Ma Ming, Li Junjie, Tian Dazhi, Jiang Wentao.

2018, (6): 467-469. DOI:10.3969/j.issn.2095-5332.2018.06.014

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Objective To discuss the clinical experience of liver and kidney procurement from donation after brain death（DBD）donors. Methods The clinical data of 12 DBD donors undergoing liver and kidney procurement in Tianjin First Central Hospital from May 2016 to March 2018 were analyzed retrospectively. The clinical experience was summarized. Results According to the diagnostic criteria for brain death established by Brain Injury Evaluation Quality Control Center of National Health and Family Planning Commission，the livers and kidneys of 12 DBD donors were procured after brain death．Modified liver and kidney perfusion was performed by arterial and portal vein intubation using the catheter made in Tianjin First Central Hospital．The operation time was 40 ～ 60 min．A total of 12 liver grafts and 24 renal grafts were harvested，those organs were successfully applied in clinical liver and renal transplantation．No primary nonfunction was observed in the liver or renal grafts. Conclusion Timely assessment of brain death，rigorous and cautious measurement for organ maintenance and modified techniques for combined liver and kidney procurement play a key roles in the success of liver and kidney procurement from DBD organ donation donors．

The prevention management of deep vein thrombosis after renal transplantation by air pressure pump limb circulation driver

Li Yue , He Chongxiang , Yang Zhijian , Cheng Chuhong , Ye Qifa , .

2018, (6): 473-476. DOI:10.3969/j.issn.2095-5332.2018.06.017

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Objective To investigate the effect of early use of pneumatic circulator on airway pressure in renal transplant recipients to prevent and reduce the formation of deep venous thrombosis in the lower extremity（LDVT） after renal transplantation. Methods A total number of 80 cases of renal transplantation patients were randomly divided into treatment group and control group，40 cases in each group. In the control group，the patient received only routine hospitalization and nursing，symptomatic treatment; in the treatment group，the patient received based on the control group plus air pressure pump limb circulation driven compression therapy，according to the patient's condition and tolerance，as directed by the doctor twice daily，each 30 min，7 d for a course of treatment. Two groups of patients underwent color Doppler ultrasound examination of the grafts and deep veins of both lower extremities at3 d，7 d and 14 d after operation to observe the incidence of LDVT and to measure the velocity of femoral vein blood flow，and the data were statistically analyzed analysis. Results The observation group of 40 cases，no case of LDVT formation. The control group of 40 cases，LDVT occurred in 4 cases，all occurred in the renal transplantation side，3 cases of renal vein thrombosis，saphenous vein thrombosis in 1 case. The incidence of LDVT between the two groups was statistically significant（P ＜ 0.05） Conclusion It is safe and effective to use air circulator to prevent and reduce the incidence of LDVT and improve the nursing quality of patients.

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