The Struggling Odyssey of Infantile Primary Hyperoxaluria.

Introduction: Oxalate overproduction in Primary Hyperoxaluria type I (PH1) leads to progressive renal failure and systemic oxalate deposition. In severe infantile forms of PH1 (IPH1), end-stage renal disease (ESRD) occurs in the first years of life. Usually, the management of these infantile forms is challenging and consists in an intensive dialysis regimen followed by a liver-kidney transplantation (combined or sequential). Methods: Medical records of all infants with IPH1 reaching ESRD within the first year of life, diagnosed and followed between 2005 and 2018 in two pediatric nephrology departments in Brussels and Paris, have been reviewed. Results: Seven patients were included. They reached ESRD at a median age of 3.5 (2-7) months. Dialysis was started at a median age of 4 (2-10 months). Peritoneal dialysis (PD) was the initial treatment for 6 patients and hemodialysis (HD) for one patient. Liver transplantation (LT) was performed in all patients and kidney transplantation (KT) in six of them. A sequential strategy has been chosen in 5 patients, a combined in one. The kidney transplanted as part of the combined strategy was lost. Median age at LT and KT was 25 (10-41) months and 32.5 (26-75) months, respectively. No death occurred in the series. At the end of a median follow-up of 3 years, mean eGFR was 64 ± 29 ml/min/1.73 m2. All patients presented retinal and bone lesions and five patients presented bones fractures. Conclusion: Despite encouraging survival figures, the morbidity in IPH1 patients remains extremely heavy and its management presents a huge challenge. Thanks to the newly developed RNA-interference drug, the future holds brighter prospects.

Phosphatidylethanol for Monitoring Alcohol Use in Liver Transplant Candidates: An Observational Study.

Liver transplantation remains an essential procedure for many patients suffering from alcoholic liver disease. Alcohol use monitoring remains paramount all through the stages of this complex process. Direct alcohol biomarkers, with improved specificity and sensibility, should replace traditional indirect markers. Phosphatidylethanol (PEth) has been recently tested in alcoholic liver disease patients, but more evidence is needed, especially in comparison with other direct biomarkers. We conducted an observational study among patients awaiting liver transplantation. We analyzed Peth in blood, ethylglucuronide (EtG) in hair and urine and ethylsulphate (EtS) in urine, using mass spectrometry methods. In addition, transaminases, and self-reports were analyzed. A total of 50 patients were included (84% men, mean age 59 years (SD = 6)). 18 patients (36%) screened positive for any marker. Self-reports were positive in 3 patients. EtS was the biomarker with more positive screens. It also was the most frequently exclusive biomarker, screening positive in 7 patients who were negative for all other biomarkers. PEth was positive in 5 patients, being the only positive biomarker in 2 patients. It showed a false negative in a patient admitting alcohol use the previous week and screening positive for EtG and EtS. Hair EtG was positive in 3 patients who had negative Peth, EtG. EtG did not provide any exclusive positive result.A combination of biomarkers seems to be the best option to fully ascertain abstinence in this population. Our study suggest EtS might also play a significant role.

Des-gamma-carboxy prothrombin affects the survival of HCC patients with marginal liver function and curative treatment: ACRoS1402.

PURPOSE: Considering the initial treatment of hepatocellular carcinoma (HCC), the best prognostic index for Child-Pugh classes B and C (CP-BC) patients has not been yet established. This study aimed to elucidate the risk factors for disease-free survival (DFS) and overall survival (OS) in multicenter patients with a poor liver functional reserve after curative treatment. METHODS: Between April 2000 and April 2014, 212 CP-BC patients who received treatment in five high-volume centers in Japan were included in this study. CP-B and C patients were 206 and 6, respectively. Cox proportional hazard regression analyses for DFS and OS were performed to estimate the risk factors. RESULTS: The mean observation time was 1132 days. Mean Child-Pugh score and indocyanine green retention rate at 15 min were 7.5 and 31.5%, respectively. Histological chronic hepatitis and liver cirrhosis were observed in 20% and 74% patients, respectively. In the multivariate analysis, the risk factors for DFS were des-gamma-carboxy prothrombin (DCP) [hazard ratio (HR), 1.6; P = 0.012] and treatment without liver transplantation. Moreover, DCP was identified as an independent risk factor for OS (HR, 1.7; P = 0.01). Tumor size, number, tumor thrombus, Milan criteria, liver cirrhosis, and treatment without liver transplantation were not identified as risk factors for OS. The 5-year OS in patients with high serum DCP levels (< 90 mAU/mL) was significantly better than that in those with low serum DCP levels (P = 0.003). CONCLUSIONS: Serum DCP value before treatment predicted both DFS and OS in CP-BC patients with HCC.

Effect of protective mechanical ventilation on plasma markers of lung injury and inflammatory mediators during general anesthesia for liver transplantation surgery / 临床麻醉学杂志

Objective To approach the effect of protective mechanical ventilation on acute lung injury after orthotopic liver transplantation, by observing changes of plasma markers of lung injury and inflammatory mediators.Methods Sixty patients scheduled for liver transplantation under general anesthesia, 42 males and 18 females, aged 21-62 years, weighing 43-80 kg, ASA physical status Ⅱ-Ⅳ, were randomly divided into 2 groups: protective mechanical ventilation group (group P) and unprotective mechanical ventilation group (group U).Pulmonary artery blood for plasma markers of lung injury and inflammatory mediators were collected at the following time points: before operation (T1), 3 hours after mechanical ventilation (T2), 2 hours (T3) and 4 hours in neohepatic stage (T4).These mediators included clara cell secretory protein (CC16), surfactant proteins (SP-D), soluble receptor for advanced glycation end-products (sRAGE), TNF-α, IL-6 and IL-8.Moreover, blood gas results were recorded at these 7 time points: T1-T4, 2 hours after operation (T5), before tracheal extubation (T6) and 2 days after operation (T7).The postoperative awakening time, tracheal extubation time, ICU stay time and the incidence of ALI were recorded.Results Compared with T1, plasma level of CC16 in the two groups increased at T2 and T3 (P<0.05 or P<0.01), however, plasma level of SP-D, sRAGE, TNF-α, IL-6 and IL-8 did not increase until T3 (P<0.01).Moreover, plasma level of sRAGE, TNF-α, IL-6 and IL-8 at T4 were higher than those at T1 (P<0.05 or P<0.01).Compared with T1, OIs in the two groups increased at T2, T5 and T6 (P<0.05 or P<0.01), while decreased at T4 in group P (P<0.01) and at T3 and T4 in group U (P<0.01).In group P, patients showed a lower plasma level of CC16 at T2 and T3 (P<0.05 or P<0.01), a higher OI at T3 (P<0.05) and an earlier tracheal extubation after operation [(8.9±3.2) h vs (9.3±2.8) h, P<0.05] compared with group U.There was no significant difference of acute lung injury incidence between the two groups after operation, which was 5(16.6%) and 7 (23.3%), respectively.Conclusion Protective mechanical ventilation may promote oxygenation index, and shorten tracheal extubation time, thus protect lung function of patients in liver transplantation to some extend.

Pediatric liver transplantation from split livers: a single-center experience / 中华器官移植杂志

Objective To analyze and evaluate the efficay of split liver transplantation in children.Method From September 2006 to December 2014,210 children were treated with liver transplantation in Tianjin First Central Hospital.The clinical data were retrospectively analyzed and the difference in postoperative survival was compared between the groups.The 210 childrens were categorized into living donor liver transplantation group (183 cases) and split liver transplantation group (27 cases) based on their operation styles.In living group,all donors to recipients were immediate relatives within three generations.In split group,all donors were men,and livers were obtained from no heartbeat donors.Postoperatively,tacrolimus combined a duplex of prednisolone served as immunosuppression scheme.The survival and incidence of complications were observed.Result There was significant difference in the sex ratio between two groups (P＜0.05).The donor liver cold ischemia time was significantly longer in split group than in living group (P＜0.05).The 1-month,6-month,1-year and 2-year overall survival rate in 210 recipients was 99.5％,98.1％,96.2％ and 94.2％ respectively.The median follow-up time in living group and split group was 15.2months and 26.1 months,respectively.The 1-mont,6-month,1-year and 2-year survival rate was 99.5％,96.7％,92.6％ and 74.1 ％ in living group,and 97.8％,96.2％,77.8％ and 74.0％ in split group,respectively (P＜0.05).During the follow-up period,8 cases died (29.6％) in split group (5deaths due to infection and sepsis,and 3 deaths due to multiple organ failure),and 10 cases died (5.5％) in living group (6 deaths due to infection and sepsis,and 4 deaths due to multiple organ failure).Conclusion In the case of strict selection of donors,split liver transplantation can obtain good effect,but the incidence of complications is higher than living donor liver transplantation.Especially,the biliary complications should be prevented and managed actively.

Intraoperative Iigation of recipient's portasystemic shunt in liver transplantation / 中华普通外科杂志

Objective To investigate the clinical significance of ligating the portasystemic shunt confirmed by preoperative CT evaluation during orthotopic liver transplantation. Methods From January 2007 to August 2008, 35 patients in Tianjin First Central Hospital underwent preoperative three-dimensional CT scan, among them 23 patients had spontaneous major portasystemic shunts, the other 12 patients did not have portasystemic shunts. 16 out of the 23 cases with significant shunts underwent shunt ligation based on portal blood flow volume measured by intraoperative portal vein flowmetry. The shunt of the other 7 patients were left untreated. Results The portal blood flow in the 12 patients without portasystemic shunt as judged by preoperative CT scanning were (1101±70) ml/min. The shunts in 7 patients with portal blood flow greater than 1000 ml/min were not ligated, that of the 16 patients with portal blood flow volume lower than 1000 mL/min were ligated. The portal blood flow volume in those 16 patients before and after ligating the shunt were (657±112) m//min and (1136±161) ml/min, respectively (P<0.05). Postoperatively 2 patients suffered from portal vein thrombosis, among them 1 patient suffered from intermittent disturbance of consciousness, 2 patients died within 3 months, with one dying of respiratory failure from pulmonary aspergillus infection one dying of hepatic failure in 2 months after operation because of graft dysfunction.The other 19 patients with normal blood flow and well-functioning graft were alive. Conclusion The ligation of portasystemic shunt is mandatory in patients when pretransplant CT evaluation showing a major porto-systemic shunts and portal blood flow volume was less than 1000 ml/min.

Changes in dose requirements of rocuronium after right hemihepatectomy in donor for liying donor liver transplantation / 中华麻醉学杂志

Objective To investigate the changes in the dose requirements of rocuronium after right hemihepatectomy in donor of living donor liver transplantation. Methods Sixteen ASA Ⅰ patients of living donor liver transplantation, aged 21-49 yr, weighing 51-86 kg, scheduled for right hemihepatectomy, were enrolled in this study. Anesthesia was induced with iv midazolam and fentanyl, and TCI of propofol and rocuronium. Changes in adductor pollicis muscle were monitored by the train of four (TOF) stimulation. The patients were mechaincally ventilated after tracheal intubation when T1/Tc = 0. The target plasma concentration of rocuroniurn was reduced to 1.0 μg/ml after tracheal intubation and the concentration was regulated to maintain 0 < T1 ≤ 10%. Anesthesia was maintained with sevoflurane inhalation, TCI of propofol, intermittent iv injection of fentanyl. The infusion of rocuronium and inhalation of sevoflurane were stopped before the second time of cholangiography, the total time and amount of rocuronium administered and the recovery of muscle relaxation were recorded at this time (before hemihepatectomy). The infusion of rocuronium and inhalation of sevoflurune were rests-ted after right hemibepatectomy. After the peritoneum was closed, the infusion of rocuroniurn and inhalation of sevoflurane were stopped again, the total time and amount of rocuronium administered and the recovery of muscle relaxation were also recorded at this time (after hemihepatectomy). Results The amount of rocuronium administered was significantly reduced after hemihepatectomy compared with that before hernihepatectomy (P < 0.05). The amount of rocurunium administered after hemihepatectomy was (67 ± 13) % of that before hemihepatectomy. Conclusion Right hemihepatectomy can affect the metabolism of rocuronium in the donor of living donor liver transplantation.

Study on Living Related Liver Transplantation for Treating Wilson′s Disease in Children / 实用儿科临床杂志

Objective To explore the liver pathology from live related liver transplatation(LRLT) of Wilson′s disease(WD) in children,and evaluatethe indication of LRLT.Methods The sample of this study,including the donater and patient,came from the LRLT. It was observed with HE,MASSON,Timm′s and Rubeanic staining.Results With HE and MASSON stainning,hepatocyte showed degeneration,fiber hyperplasic and false lobule formed;Timm′s and Rubeanic stainning showed typical black deposit of granules and conglomerations.Liver pathology revealed that liver cirrhosis appeared,and it was diffrentent from Child Pugh.Conclusions It is of great significance that the liver pathology is useful for the treatment of WD.If liver pathology supportes,the indication of LRLT can be measurably broadened.

Effects of liver transplantation on function and morphological changes of spleen in hepatic cirrhosis rat / 中国普通外科杂志

Objective To investigate the effects of liver transplantation on function and morphological changes of spleen in hepatic cirrhosis rats.Methods Hepatic cirrhosis model was established in rats by subcutaneous injections of carbon tetrachloride.Liver transplantation model was established with two-cuff technique.The portal vein pressure,and morphological changes of spleen were observed before and after liver transplantation in hepatic cirrhosis rats.The serum tuftsin level before and after liver transplantation were detected.Results Before liver transplantation,PVP was increased from 6.28 mmHg to 14.03mmHg(P

A model of orthotopic liver transplantation with grafts harvested from non-heart-beating donors by perfusion through ascending aorta in rats / 中国普通外科杂志

ObjectiveTo investigate the establishment of a rat model of orthotopic liver transplantation with more complete and easier performance of graft perfusion and easier control of warm ischemic time. MethodsA modification of Kamada two-cuff method was used. Cold perfusion of donor livers was done through the (ascending) aorta. Suture anastomosis of suprahepatic vena cava was performed. Infrahepatic vena cava and (portal) vein anastomoses were done by the cuff technique. The bile duct was anastomosed with internal stent. The non-heart-beating donor(NHBD) graft model of orthotopic liver transplantation in rats was thus (established).ResultsThe survival rate of recipients grafted from NHBD with perfusion of ascending aorta was 95% (38/40) for 1 day and 85.5% (35/40) for 7 days. ConclusionsThe livers of NHBD as grafts harvested with perfusion through ascending aorta had more complete and homogeneous perfusion, perfusion was easier to perform and warm ischemia time was more accurately controlled. The one-week posttransplantion (survival) rate was longer than that reported in the literature.

An Experience of Ballon Angioplasty in Hepatic Artery Stenosis After Living Related Living Transplantation

Liver transplantation is an accepted and successful mode of treatment for pediatric end-stage liver disease. Living related liver transplatation(LRLT) in child has certain potential advantages, such as short cold ischemic time, accurate graft size and vessel diameter match based on elective preoperative preparations. Recently, introduction of microvasular surgery technique to hepatic artery reconstruction has been used, but still remained the possibility of hepatic artery thrombosis. Herein, We report a LRLT case, which showed hepatic artery stenosis postoperatively, successfully dilated by ballon angioplasty technique.

Postoperatiive renal hemodynamic in orthotopic liver transplant recipents / 中国普通外科杂志

Objective To investigate the renal hemodynamic changes after orthotopic liver transplantation(OLT)and the correlative parameters. Methods In 20 patients undergoing OLT for cirrhosis,the following renal arterial resistance index(RI) was measured before surgery and 7days,30days, 6 months and 1 year after operation by using color Doppler flow imaging(CDFI) and serum creatinine detection.Meanwhile the same parameters were measured in 10 healthy as controls. Results Both RI and serum Cr rised after OLT ( P 0.05). Conclusions Most alteration of renal hemodynamic parameters in cirrhosis are restored to normal after OLT in 1 year. Preoperative renal abnormalities and intraopterative alteration of hemodynamic may contribute to postoperative renal dysfunction. Cyclosporine (CsA) is the most likely etiologic agent of postoperative renal dysfunction.