Background: Emergency living donor liver transplantation (e-LDLT) is crucial for patients experiencing acute liver failure, acute-on-chronic liver failure, or severe, life-threatening cirrhosis. The purpose of this study was to determine the risk factors that affect the death rate of patients who are waiting for e-LDLT by analyzing data on the Korean Network for Organ Sharing (KONOS). Methods: A retrospective examination of KONOS data was performed, encompassing consecutive e-LDLT applications from 2017 to 2021. Exclusions were made for pediatric patients. The data were classified into two distinct groups. Patients who died before getting e-LDLT were classified as Group 1 (n=38), while patients who spontaneously recovered without liver transplantation, non-emergency LDLT, or deceased donor liver transplantation more than 14 days following e-LDLT treatment were classified as Group 2 (n=30). Results: Significantly greater rates of pre-transplant critical care unit stay, pre-transplant ventilator support, or continuous renal replacement treatment were observed in Group 1 compared to Group 2. In comparison to Group 2, Group 1 exhibited notably lower serum albumin levels and higher model for end-stage liver disease (MELD) scores. Significantly, the MELD score increased by more than 10% for 3 days preceding to e-LDLT applications in Group 1 compared to Group 2. The multivariate analysis revealed that the only factor that affected the death of patients waiting for LDLT after e-LDLT applications was pre-transplant ventilator support. Conclusion The present study suggested that patients receiving mechanical ventilator support in the pre-transplant period should be approached cautiously when deciding on e-LDLT.

Background: Deceased donor liver transplantation (DDLT) and living donor liver transplantation (LDLT) are employed to address highly urgent patients, including those with acute liver failure (ALF), acute-on-chronic liver failure (ACLF), or critical cirrhosis. This study compares outcomes between LDLT and DDLT patients with ALF, ACLF, or critical cirrhosis in highly urgent LDLT (HU-LDLT) applications. Methods: This study conducted a retrospective analysis of the Korean Network for Organ Sharing (KONOS) data, which included 391 consecutive HU-LDLT applications from 2017 to 2021. Results: The proportion of DDLT was 15.1% (n=59) within the cohort of HU-LDLT applications. The prevalence of hepatorenal syndrome, duration of pre-transplant intensive care unit (ICU) care, incidence of pre-transplant continuous renal replacement therapy, and median model for end-stage liver disease scores were significantly greater and prolonged in DDLT patients compared to LDLT patients. Statistical analysis revealed no significant differences in postoperative complications or overall survival between the two groups. In the multivariate analysis, only pre-transplant ventilator care emerged as a significant predisposing factor for mortality. Conclusion The present study indicates that LDLT is a viable option, yielding comparable perioperative and long-term outcomes to DDLT for HU patients, which can encourage living liver donation to overcome organ shortages in HU patients.

Although the prevalence of hereditary hemolytic anemia (HHA) is relatively low in Korea, it has been gradually increasing in recent decades due to increment in the proportions of hemoglobinopathies from immigrants of South East Asia, raising awareness of the disease among clinicians, and advances in diagnostic technology. As such, the red blood cell (RBC) Disorder Working Party (WP), previously called HHA WP, of the Korean Society of Hematology (KSH) developed the Korean Standard Operating Procedures (SOPs) for the diagnosis of HHA in 2007. These SOPs have been continuously revised and updated following advances in diagnostic technology [e.g., flow cytometric osmotic fragility test (FOFT) and eosin-5-maleimide (EMA) binding test], current methods for membrane protein or enzyme analysis [e.g., liquid chromatography-tandem mass spectrometry (LC-MS/MS), ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS), high-performance liquid chromatography (HPLC)], and molecular genetic tests using next-generation sequencing (NGS). However, the diagnosis and treatment of HHA remain challenging as they require considerable experience and understanding of the disease. Therefore, in this new Korean Clinical Practice Guidelines for the Diagnosis of HHA, on behalf of the RBC Disorder WP of KSH, updated guidelines to approach patients suspected of HHA are summarized. NGS is proposed to perform prior to membrane protein or enzyme analysis by LC-MS/MS, UPLC-MS/MS or HPLC techniques due to the availability of gene testing in more laboratories in Korea. We hope that this guideline will be helpful for clinicians in making diagnostic decisions for patients with HHA in Korea.