Background and Aims Biliary atresia is the most common cause of liver disease and liver transplantation in children. The accumulation of bile acids in hepatocytes and the stimulation of the intestinal microbiome can aggravate the disease progression. This study investigated changes in the composition of the gut microbiota and its metabolites in biliary atresia and the possible effects of these changes on disease progression. Methods Stool samples of biliary atresia at different disease stages and matched control individuals were collected (early stage: 16 patients, 16 controls; later stage: 16 patients, 10 controls). Metagenomic sequencing was performed to evaluate the gut microbiota structure. Untargeted metabolomics was performed to detect and analyze the metabolites and bile acid composition. Results A disturbed gut microbiota structure occurred in the early and later stages of biliary atresia. Klebsiella , Streptococcus , Veillonella , and Enterococcus have always been dominant. The abundance of V. atypica displayed significant changes between the early and later stages of biliary atresia. Combined with clinical indicators, Spearman’s analysis showed that Klebsiella and Veillonella atypica strongly correlated with liver enzymes. Enterococcus faecium had an enormously positive relationship with lithocholic acid derivatives. Metabolites involved in tryptophan metabolism were changed in the patients with biliary atresia, which had a significant association with stool V. atypica and blood total bilirubin ( p < 0.05). Conclusions The liver damage of biliary atresia was directly or indirectly exacerbated by the interaction of enriched Klebsiella ( K. pneumoniae ), Veillonella ( V. atypica ), and Enterococcus ( E. faecium ) with dysmetabolism of tryptophan and bile acid.
Objective: Porto-pulmonary hypertension (PoPH) refers to pulmonary hypertension based on severe portal hypertension. Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for liver transplantation (LT). We analyze efficacy and long-term prognosis of liver transplantation for porto-pulmonary hypertension from our center. Methods: A retrospective analysis was performed on 5 patients with PoPH who received liver transplantation in Beijing Friendship Hospital from Jan. 2013 to March 2022, in order to analyze postoperative follow-up results and long-term survival. Results: Five patients with PoPH with a mean age of 35.6 ± 18.6 years (60% females), were included in the analysis. Their primary diseases were hepatic spongiosis, Caroli disease, hepatitis B cirrhosis, drug-induced cirrhosis and primary sclerosing cholangitis. All patients were measured by Swan-Ganz catheterization of right heart, among them, the mean of the highest mean pulmonary artery pressure(mPAP) level before LT were 46.6mmHg(27-58mmHg). The mean of their highest systolic pulmonary artery pressure(SPAP) level measured under echocardiography was 101.36mmHg(51-154mmHg) before LT. The median Model for End-Stage Liver Disease (MELD) score was 16(14-20) at PoPH diagnosis. All patients had been on pulmonary vasodilator therapy(including ambrisentan, remodulin and sildenafil) for more than six months to lower pulmonary artery pressure to mPAP<35mmHg, that can achieve surgical indications before LT. The operations went smoothly, except in one case where pulmonary artery pressure suddenly increased during surgery ended smoothly after the addition of remodulin. Two patients underwent a second liver transplant at 6 months and 65 months postoperatively, and one patient died of acute heart failure after the second transplantation, while the remaining four patients survived. The follow-up time of the survived patients were 29.7, 46.9, 56, 85.2 months. The longest follow-up among them was over seven years. SPAP of them decreased 15.4, 129, 65.3, 20mmHg after LT. Two patient was discontinued pulmonary arterial pressure medications within 1 year after surgery because SPAP were down to normal, and other patients still on medication. Conclusion: Patients with pulmonary vasodilator therapy before LT can have excellent long-term outcomes post-transplant. Oral pulmonary vasodilator therapy can be effective treatment to qualify patients for LT.
Liver allograft fibrosis (LAF) is prevalent among children with long-term survival after liver transplantation (LT). The authors aimed to identify clinical risk factors, with a focus on the impact of immunosuppression (IS) level in the early post-transplant period on LAF.A retrospective study was conducted on pediatric LT recipients with at least 1-year of follow-up. Cox regression models were used to analyze risk factors associated with LAF, and landmark analysis was used to evaluate the impact of IS level on LAF. Longitudinal analysis was also conducted in patients with paired biopsies.A total of 139 patients involving 174 liver biopsies were included. With 2.3 to 5.9 years of follow-up, LAF was detected in 91.4% of patients (7.9% were significant), up to 88.2% of whom showed normal liver function. Episodes of acute rejection, biliary complications, cytomegalovirus infection, and prolonged cold ischemia time were independent risk factors. Besides, the risk of LAF in patients with relatively low IS levels at postoperative 1-3, 3-6, 6-12, and 12-36 months was higher than the counterparts. Especially, in patients with relatively high IS levels (mean tacrolimus trough concentration ≥5.1 ng/ml) during postoperative 12-36 months, the risk of LAF was 67% lower in the short future ( P =0.006). In paired analysis, patients with increased IS levels were more likely to achieve fibrosis-reduction (HR=7.53, P =0.025).Mild to moderate LAF is common among pediatric LT recipients and can appear early and silently. Maintaining adequate levels of IS during 1-3 years after LT seems crucial to ensure protection against LAF.
Domino cross-auxiliary liver transplantation represents an innovative procedure for the treatment of selective noncirrhotic metabolic liver diseases.The treatment strategies and experiences in domino cross-auxiliary liver transplantations, including the world's first case in 2013, are reviewed.Six patients with noncirrhotic metabolic liver diseases receiving domino cross-auxiliary liver transplantation included the following: familial amyloidosis with multiple neuropathy (case 1), ornithine transcarbamylase deficiency (cases 3, 5, and 6), and Wilson's disease (cases 2 and 4). Five patients achieved a favorable postoperative survival outcome and quality of life, whereas case 2 died of multiple organ failure at 3 months post liver transplant (LT). Case 1 experienced an imbalance in portal vein blood perfusion between the 2 domino livers at 6 months after LT but improved after interventional radiology treatment. Cases 3 and 4 showed domino grafts associated with hypercholesterolemia after LT, but total cholesterol levels decreased to normal ranges after dietary adjustment. Case 5 showed an effortless recovery after surgery with no complications during the follow-up period. Case 6 experienced an occult domino liver graft rejection, which resulted in graft dysfunction and eventual recurrence of the primary metabolic liver disease (ornithine transcarbamylase deficiency). A liver retransplantation may be required for this patient.Domino cross-auxiliary liver transplantation is an innovative and effective treatment for metabolic liver diseases in the patients who are strictly selected on the basis of pathophysiological and genetic criteria. Special attention to rejection monitoring and imbalance regeneration are required with this procedure.
Objective
To Establish an evaluation system of the quality of life for Chinese liver and kidney transplant recipients, and develop a specialized scale to measure the quality of life (QOL) for such recipients preliminarily.
Methods
By combining subjective and objective methods, this study determined the evaluation system and created items of QOL scale based on literature analysis, Delphi method (expert consultation), interviews, and experience of researchers. Then, a nationwide clinical survey was conducted on 5 Level III Class A hospitals. 454 valid questionnaires were collected, including 153 from liver recipients, and 301 from kidney recipients. Subjective statistical methods such as critical Ratio method, correlation coefficient method, dispersion tendency method, and exploratory factor analysis were used for selection of questionnaire items.
Results
The study established an evaluation system of QOL for liver and kidney transplant recipients, including 4 primary indexes and 12 secondary indexes, and developed QOL scales that were appropriate for liver and kidney transplant recipients respectively.
Conclusions
The structure of QOL scale for liver and kidney transplant recipients was basically consistent with theoretical assumption. The QOL scale for patients showed good reliability and validity, therefore, it can fully reflect the quality of their life.
Key words:
Liver transplantation; Kidney transplantation; Quality of life; Evaluation studies; Scale; Delphi technique; Item selection
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 ⅠDSA (HLA-A mismatch), and 1 case of ⅡDSA (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.
Key words:
donor specific antibody; liver transplantation; immunoglobulin; complement C1q; complement C4d
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.
Key words:
Liver transplantation; Child; Metabolic disease
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.
Key words:
Liver transplantation; Kidney transplantation; Quality of life; Influencing factor
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