Early Graft Loss With Suspected Seventh-Day Syndrome Following Pediatric Liver Transplantation.

INTRODUCTION: Allograft dysfunction within the first week posttransplant is an uncommon but known complication following liver transplantation. Seventh-Day Syndrome (7DS) is a rare complication of allograft dysfunction following liver transplantation characterized by the rapid clinical deterioration of a formerly well-functioning allograft within the first week posttransplant. The etiology of 7DS is unknown, and treatment options remain limited. While cases of graft survival have been reported, the risk of mortality remains exceedingly high without urgent retransplantation. METHODS: Patient data was retrospectively analyzed and a literature review performed. RESULTS: We present a unique case of split liver transplantation into two pediatric recipients in which one recipient developed rapidly progressive graft failure approximately 1 week postoperatively requiring urgent retransplantation while the other recipient had an unremarkable postoperative course. Upon clinical manifestation of progressive graft failure, the patient was treated with thymoglobulin, rituximab, intravenous immunoglobulin, and plasmapheresis. Despite this, the patient's clinical status continued to decline and she underwent retransplantation 11 days following her initial liver transplant. CONCLUSION: Seventh-Day Syndrome is a rare complication following liver transplantation that is associated with a high risk of morbidity and mortality. Our case adds to the limited literature on 7DS in children and is the first to report a comparative posttransplant clinical course in two recipients who received split grafts from the same donor.

Metastatic pulmonary calcifications after pediatric liver transplantation.

BACKGROUND: Pulmonary calcification (PC) is a rare clinical entity observed following liver transplantation (LT). Most often identified in adults or in patients with concomitant renal failure, PC is rarely reported in children. While the clinical course of PC is largely benign, cases of progressive respiratory failure and death have been reported. Additionally, PC may mimic several other disease processes making diagnosis and management challenging. Currently, little is reported regarding the diagnosis, management, and long-term outcomes of children with PC following LT. METHODS: We performed a retrospective chart review of patients undergoing LT at our institution between 2006 and 2023. We identified two patients who developed PC following LT. Their diagnosis, clinical course, and long-term outcomes are reported. A literature review of the presentation, diagnosis, management, and outcomes of adult and pediatric patients with PC post-LT was also performed. CONCLUSIONS: Pulmonary calcifications are a rare but notable complication after pediatric liver transplantation. Our case series adds to the limited literature on this clinical entity in children but also highlights the fact that effective diagnosis and treatment may be safely accomplished without the use of lung biopsy.

Variation in Pediatric Anesthesiologist Sedation Practices for Pediatric Gastrointestinal Endoscopy.

Background: Despite a worldwide shift toward anesthesiologist-administered sedation for gastrointestinal endoscopy in children, ideal sedation regimens remain unclear and best practices undefined. Aim: The aim of our study was to document variation in anesthesiologist-administered sedation for pediatric endoscopy. Outcomes of interest included coefficients of variation, procedural efficiency, as well as adverse events. Methods: IRB approval was obtained to review electronic health records of children undergoing routine endoscopy at our medical center during a recent calendar year. Descriptive and multivariate analyses were used to examine predictors of sedation practices. Results: 258 healthy children [2-21 years (median 15, (Q1-Q3 = 10-17)] underwent either upper and/or lower endoscopies with sedation administered by anesthesiologists (n = 21), using different sedation regimens (29) that ranged from a single drug administered to 6 sedatives in combination. Most patients did not undergo endotracheal tube intubation for the procedure (208, 81%), and received propofol (255, 89%) either alone or in combination with other sedatives. A total of 10 (3.8%) adverse events (9 sedation related) were documented to occur. The coefficient of variation (CV) for sedation times was high at 64.2%, with regression analysis suggesting 8% was unexplained by procedure time. Multivariable model suggested that longer procedure time (p < 0.0001), younger age (p < 0.0001), and use of endotracheal tube intubation (p = 0.02) were associated with longer sedation time. Discussion: We found great variation in anesthesiologist administered regimens for pediatric endoscopy at our institution that may be unwarranted, presenting may opportunities for minimizing patient risk, as well as for optimizing procedural efficiency.

Non-alcoholic fatty liver disease in children and young adults is associated with increased long-term mortality.

BACKGROUND & AIMS: Longitudinal data are scarce regarding the natural history and long-term risk of mortality in children and young adults with biopsy-confirmed non-alcoholic fatty liver disease (NAFLD). METHODS: This nationwide, matched cohort study included all Swedish children and young adults (≤25 years) with biopsy-confirmed NAFLD (1966-2017; n = 718). NAFLD was confirmed histologically from all liver biopsies submitted to Sweden's 28 pathology departments, and further categorized as simple steatosis or steatohepatitis (NASH). Patients with NAFLD were matched to ≤5 general population controls by age, sex, calendar year and county (n = 3,457). To account for shared genetic and early-life factors, we also matched patients with NAFLD to full-sibling comparators. Using Cox regression, we estimated multivariable-adjusted hazard ratios (aHRs) and 95% CIs. RESULTS: Over a median of 15.8 years, 59 patients with NAFLD died (5.5/1,000 person-years [PY]) compared to 36 population controls (0.7/1,000 PY; difference = 4.8/1,000 PY; multivariable aHR 5.88; 95% CI 3.77-9.17), corresponding to 1 additional death per 15 patients with NAFLD, followed for 20 years. The 20-year absolute risk of overall mortality was 7.7% among patients with NAFLD, and 1.1% among controls (difference = 6.6%; 95% CI 4.0-9.2). Findings persisted after excluding those who died within the first 6 months (aHR 4.65; 95% CI 2.92-7.42), and after using full-sibling comparators (aHR 11.72; 95% CI 3.18-43.23). Simple steatosis was associated with a 5.26-fold higher adjusted rate of mortality compared to controls (95% CI 3.05-9.07), and this was amplified with NASH (aHR 11.51, 95% CI 4.77-27.79). Most of the excess mortality was from cancer (1.67 vs. 0.07/1,000PY; aHR 15.60; 95% CI 4.97-48.93), liver disease (0.93 vs. 0.04/1,000PY; aHR 16.46; 95% CI 2.75-98.43) and cardiometabolic disease (1.12 vs. 0.14/1,000PY; aHR 4.32, 95% CI 1.73-10.79). CONCLUSIONS: Swedish children and young adults with biopsy-confirmed NAFLD have significantly higher rates of overall, cancer-, liver- and cardiometabolic-specific mortality compared to matched general population controls. LAY SUMMARY: Currently, the natural history and long-term risk of mortality in children and young adults with biopsy-confirmed non-alcoholic fatty liver disease (NAFLD) is unknown. This nationwide cohort study compared the risk of all-cause and cause-specific mortality in pediatric and young adult patients in Sweden with biopsy-confirmed NAFLD to matched general population controls. We found that compared to controls, children and young adults with biopsy-confirmed NAFLD and NASH have significantly higher rates of overall, cancer-, liver- and cardiometabolic-specific mortality.