Introduction: Liver transplantation (LT) is the therapeutic option for end-stage liver disease associated with alpha1 antitrypsin (A1AT) deficiency. The aim of the present retrospective study was to report on long-term outcomes following LT for A1AT deficiency.
Methods: The medical records of 90 pediatric and adult patients transplanted between 1982 and 2017 in France and Geneva (Switzerland) were reviewed.
Results: The study population consisted of 32 adults and 58 children; median age at transplant was 13.0 years (range: 0.2-65.1), and 65 were male (72.2%). Eighty-two patients (94.8% of children and 84.4% of adults) had the PI*ZZ genotype/phenotype and eight patients (8.9%) had the Pi*SZ genotype/phenotype. Eighty-four patients (93.3%) were transplanted for end-stage liver disease and six (all Pi*ZZ adults) for HCC. Median follow-up after LT was 13.6 years (0.1-31.7). The overall cumulative patient survival rates post-transplant were 97.8% at 1 year, and 95.5%, 95.5%, 92.0%, 89.1% at 5, 10, 15, 20 years respectively. The overall cumulative graft survival rates were 92.2% at 1 year, and 89.9%, 89.9%, 84.4%, 81.5% at 5, 10, 15 and 20 years, respectively.
Conclusions: In a representative cohort of patients having presented with end-stage-liver disease or HCC secondary to A1AT, liver transplantation offered very good patient and graft survival rates.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.dld.2020.10.016 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Duodenal-jejunal bypass ameliorates MASLD in rats by regulating gut microbiota and bile acid metabolism through FXR pathways.
Hepatol Commun
February 2025
Department of Gastroenterology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Background: Although bariatric and metabolic surgical methods, including duodenal-jejunal bypass (DJB), were shown to improve metabolic dysfunction-associated steatotic liver disease (MASLD) in clinical trials and experimental rodent models, their underlying mechanisms remain unclear. The present study therefore evaluated the therapeutic effects and mechanisms of action of DJB in rats with MASLD.
Methods: Rats with MASLD were randomly assigned to undergo DJB or sham surgery.
INTERNATIONAL NORMALIZED RATIO AND ACTIVATED PARTIAL THROMBOPLASTIN TIME DO NOT PREDICT PLASMA TRANSFUSION IN LIVER TRANSPLANTATION.
Arq Bras Cir Dig
January 2025
Department of Gastroenterology, Hospital das Clinicas, Faculty of Medicine, Universidade de São Paulo - São Paulo (SP), Brazil.
Background: Blood loss during liver transplantation (LT) remains a major concern associated with increased morbidity and reduced patient and graft survival. The high complexity of the procedure associated with the multifaceted origin of the bleeding urges early identification of high-risk patients and proper monitoring of hemostasis disorders in order to improve results. The accuracy of international normalized ratio (INR) and activated partial thromboplastin time (aPTT) to evaluate coagulation status in cirrhotic patients has been doubted.
View Article and Find Full Text PDFCHOLANGIOCARCINOMA: EPIDEMIOLOGY, HISTOPATHOLOGY, AND POTENTIAL PROGNOSTIC AND THERAPEUTIC IMPLICATIONS IN A COHORT FROM A REFERENCE CENTER IN SOUTHERN BRAZIL.
Arq Bras Cir Dig
January 2025
Santa Casa de Misericórdia de Porto Alegre - Porto Alegre (RS), Brazil.
Background: Cholangiocarcinoma (CCA) is a rare neoplasm, with high mortality, originating in the bile ducts. Its incidence is higher in Eastern countries due to the endemic prevalence of liver parasites. Factors such as metabolic syndrome, smoking, and pro-inflammatory conditions are also linked to the disease.
View Article and Find Full Text PDFA Supervised Explainable Machine Learning Model for Perioperative Neurocognitive Disorder in Liver-Transplantation Patients and External Validation on the Medical Information Mart for Intensive Care IV Database: Retrospective Study.
J Med Internet Res
January 2025
Department of Anesthesiology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Background: Patients undergoing liver transplantation (LT) are at risk of perioperative neurocognitive dysfunction (PND), which significantly affects the patients' prognosis.
Objective: This study used machine learning (ML) algorithms with an aim to extract critical predictors and develop an ML model to predict PND among LT recipients.
Methods: In this retrospective study, data from 958 patients who underwent LT between January 2015 and January 2020 were extracted from the Third Affiliated Hospital of Sun Yat-sen University.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!