Optimizing biliary outcomes in living donor liver transplantation: Evolution towards standardization in a high-volume center.

Biliary complications have always been a dreaded cause of morbidity after living donor liver transplantation. While intrinsic variations in both graft and recipient biliary anatomy remain a significant factor to the difficulty of biliary reconstruction, our institution has taken advantage of its high volume of cases to critically review and evaluate modifiable operative risk factors, in particular, our surgical protocols. We present herein, the evolution of our reconstructive biliary technique from conventional methods to our current standard of microsurgical biliary reconstruction for both graft and recipient ducts. Over this period of transition, our center has created a classification system for biliary reconstruction that decreased the biliary complication rates from 40.0% to 10.2%.

Excess Risk of Major Adverse Cardiovascular and Kidney Events after Acute Kidney Injury following Living Donor Liver Transplantation.

Acute kidney injury (AKI) is a well-known risk factor for major adverse kidney events (MAKE) and major adverse cardiovascular events (MACE) in nontransplant settings. However, the association between AKI after liver transplantation (LT) and MACE/MAKE is not established. A retrospective cohort analysis including 512 LT recipients was conducted. The incidence of post-LT AKI was 35.0% (n = 179). In total, 13 patients (2.5%) developed de novo coronary artery disease (CAD), 3 patients (0.6%) diagnosed with heart failure (HF), and 11 patients (2.1%) had stroke. The post-LT AKI group showed a higher incidence of CAD and HF than the no post-LT AKI group (4.5% versus 1.5%, p = 0.042; 1.7% versus 0%, p = 0.018; respectively), while there was no significant difference in the stroke events (2.8% versus 1.8%, p = 0.461). Through Cox regression analysis, history of cardiovascular disease (HR 6.51, 95% CI 2.43-17.46), post-LT AKI (HR 3.06, 95% CI 1.39-6.75), and pre-LT diabetes (HR 2.37, 95% CI 1.09-5.17) were identified as independent predictors of MACE; pre-LT chronic kidney disease (HR 9.54, 95% CI 3.49-26.10), pre-LT diabetes (HR 3.51, 95% CI 1.25-9.86), and post-LT AKI (HR 6.76, 95% CI 2.19-20.91) were risk factors for end-stage renal disease. Post-LT AKI is predictive for the development of MACE and MAKE.

Modifiable factors affecting renal preservation in type I glycogen storage disease after liver transplantation: a single-center propensity-match cohort study.

BACKGROUND AND AIMS: Glycogen storage disease type I (GSD-I) is an autosomal recessive disorder of carbohydrate metabolism, resulting in limited production of glucose and excessive glycogen storage in the liver and kidneys. These patients are characterized by life-threatening hypoglycemia, metabolic derangements, hepatomegaly, chronic kidney disease, and failure to thrive. Liver transplantation (LT) has been performed for poor metabolic control and delayed growth. However, renal outcome was diverse in pediatric GSD patients after LT. The aim of this study was to investigate the long-term outcome of renal function in pediatric GSD-I patients after living donor LT (LDLT), and to identify modifiable variables that potentially permits LT to confer native renal preservation. METHODS: The study included eight GSD-Ia and one GSD-Ib children with a median age of 9.0 (range 4.2-15.7) years at the time of LT. Using propensity score matching, 20 children with biliary atresia (BA) receiving LT were selected as the control group by matching for age, sex, pre-operative serum creatinine (SCr) and pediatric end-stage liver disease (PELD) score. Renal function was evaluated based on the SCr, estimated glomerular filtration rate (eGFR), microalbuminuria, and morphological changes in the kidneys. Comparability in long-term renal outcome in terms of anatomic and functional parameters will help to identify pre-LT factors of GSD-I that affect renal prognosis. RESULTS: The clinical and biochemical characteristics of the GSD and BA groups were similar, including immunosuppressive regimens and duration of follow-up (median 15 years) after LT. Overall, renal function, including eGFR and microalbuminuria was comparable in the GSD-I and BA groups (median eGFR: 111 vs. 123 ml/min/1.73m2, P = 0.268; median urine microalbuminuria to creatinine ratio: 16.0 vs. 7.2 mg/g, P = 0.099, respectively) after LT. However, in the subgroups of the GSD cohort, patients starting cornstarch therapy at an older age (≥ 6-year-old) before transplantation demonstrated a worse renal outcome in terms of eGFR change over years (P < 0.001). In addition, the enlarged kidney in GSD-I returned to within normal range after LT. CONCLUSIONS: Post-LT renal function was well-preserved in most GSD-I patients. Early initiation of cornstarch therapy before preschool age, followed by LT, achieved a good renal prognosis.

Real-World Data on Clinical Outcomes of Patients with Liver Cancer: A Prospective Validation of the National Cancer Centre Singapore Consensus Guidelines for the Management of Hepatocellular Carcinoma.

INTRODUCTION: Real-world management of patients with hepatocellular carcinoma (HCC) is crucially challenging in the current rapidly evolving clinical environment which includes the need for respecting patient preferences and autonomy. In this context, regional/national treatment guidelines nuanced to local demographics have increasing importance in guiding disease management. We report here real-world data on clinical outcomes in HCC from a validation of the Consensus Guidelines for HCC at the National Cancer Centre Singapore (NCCS). METHOD: We evaluated the NCCS guidelines using prospectively collected real-world data, comparing the efficacy of treatment received using overall survival (OS) and progression-free survival (PFS). Treatment outcomes were also independently evaluated against 2 external sets of guidelines, the Barcelona Clinic Liver Cancer (BCLC) and Hong Kong Liver Cancer (HKLC). RESULTS: Overall treatment compliance to the NCCS guidelines was 79.2%. Superior median OS was observed in patients receiving treatment compliant with NCCS guidelines for early (nonestimable vs. 23.5 months p < 0.0001), locally advanced (28.1 vs. 22.2 months p = 0.0216) and locally advanced with macrovascular invasion (10.3 vs. 3.3 months p = 0.0013) but not for metastatic HCC (8.1 vs. 6.8 months p = 0.6300), but PFS was similar. Better clinical outcomes were seen in BCLC C patients who received treatment compliant with NCCS guidelines than in patients with treatment only allowed by BCLC guidelines (median OS 14.2 vs. 7.4 months p = 0.0002; median PFS 6.1 vs. 4.0 months p = 0.0286). Clinical outcomes were, however, similar for patients across all HKLC stages receiving NCCS-recommended treatment regardless of whether their treatment was allowed by HKLC. CONCLUSION: The high overall compliance rate and satisfactory clinical outcomes of patients managed according to the NCCS guidelines confirm its validity. This validation using real-world data considers patient and treating clinician preferences, thus providing a realistic analysis of the usefulness of the NCCS guidelines when applied in the clinics.