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Objectives: Acute-on-chronic liver failure (ACLF) is well-studied in adults and characterized by decompensated cirrhosis, multi-organ failure, and early mortality. Studies of ACLF in children are limited. We sought to characterize the prevalence and clinical factors associated with pediatric ACLF (PACLF). Methods: A retrospective review of children 3 months to 18 years listed for liver transplantation and hospitalized for decompensated cirrhosis between January 2007 and December 2017 at a single pediatric hospital. Primary outcome was the development of PACLF, characterized as failure of at least 1 extrahepatic organ (mechanical ventilation, renal replacement therapy, vasoactive medications, grade III/IV hepatic encephalopathy). Characteristics were recorded for each hospitalization. Results: Sixty-six patients had 186 hospitalizations with mean age at admission 4.0 ± 5.6 years and diagnosis of biliary atresia (BA) in 65%. PACLF developed in 20 patients during 23 hospitalizations (12%) and respiratory failure was most common (17/23, 74%). Duration of intensive care unit stay, 13.1 ± 1.2 days versus 0.6 ± 0.6 days (P < 0.001) and length of stay, 24.3 ± 5.0 days versus 7.9 ± 1.9 days (P = 0.003) were longer in PACLF compared with non-PACLF. Mortality during PACLF hospitalizations was 22%. Clinical factors associated with PACLF were reported from a generalized linear mixed model and included increased admission creatinine (P < 0.0001), increased aspartate aminotransferase (AST) (P = 0.014), increased international normalized ration (INR) (P = 0.0015), and a positive blood culture (P = 0.007). Conclusion: In this pediatric series, PACLF developed in 12% of hospitalizations and mortality was high. Admission creatinine, AST, INR, and presence of a positive blood culture were associated with PACLF development.

Objective: Neonatal acute liver failure (ALF) is a rare disease with high mortality for which no standard age-specific definition exists. To advance the understanding of neonatal ALF, we characterize the etiology, presenting features, treatment, and outcomes in infants within 1 month of life. Methods: We performed a single-center 11-year retrospective chart review of neonates 30 days of life with ALF as defined by an INR of !2.0. Comparisons were made by etiology and survival with native liver (SNL). Estimated survival was performed using the Kaplan-Meier method. Results: Forty-three patients met inclusion criteria for neonatal ALF. Etiologies included viral infection (23%), gestational alloimmune liver disease with neonatal hemochromatosis (GALD-NH) (21%), cardiacassociated ischemia (16%), other ischemia (14%), genetic etiologies (9%), Trisomy 21-associated myelodysplasia (TAM) (7%), hemophagocytic lymphohistiocytosis (HLH) (2%), and not identified (7%). Infants with viral etiologies had the highest alanine aminotransferase (ALT) at presentation (1179 IU/L, interquartile range [IQR] 683-1585 IU/L) in contrast to low levels in GALD-NH (23 IU/L, IQR 18-64 IU/L). Across all etiologies, only 33% were alive at 1 year. Overall median survival was 74 days; 17 days for viral infection and 74 days for GALD-NH. Among laboratory values at presentation, alpha-fetoprotein (AFP) was significantly higher in patients that survived with their native liver (P ¼ 0.04). Conclusions: Overall, outcome for neonatal ALF is poor. Although initial laboratory values can differentiate viral infection or GALD-NH, further studies are needed to identify laboratory parameters that predict SNL by etiology to ultimately improve patient outcomes.

NVUGI bleeding requiring therapeutic endoscopic intervention is rare in pediatrics. A high rate (40%) of rebleeding was noted with a large proportion (66%) of patients receiving single modality therapy. Two patients required surgical intervention to control bleeding and both presented with bleeding duodenal ulcers. An insufficient number of therapeutic procedures is available for adequate fellow training requiring supplemental simulator and hands-on animal model, or adult endoscopy unit training.

Background:In pediatric liver transplant recipients, hepatic artery thrombosis and portal vein thrombosis are major causes of acute graft failure and mortality within 30 days of transplantation. There is, however, a strong possibility of graft salvage if flow can be re-established to reduce ischemic injury. The current standard treatment is surgical revascularization, and if unsuccessful, retransplantation.Due to our success in treating these complications with catheter-directed therapies, we sought to summarize and publish the outcomes of all patients who experienced hepatic artery thrombosis or portal vein thrombosis within 30 days of liver transplantation. Methods:We conducted a retrospective cohort analysis of 27 pediatric liver transplant recipients who experienced hepatic artery thrombosis (n = 13), portal vein thrombosis (n = 9), or both (n = 5) between September 2012 and March 2021. We collected and tabulated data on the patients and therapies performed to treat them, including success rates, primary and secondary patency, and clinical outcomes.Results: Among these patients, 6 were managed with anticoagulation and relisting for transplant and 21 had a primary revascularization attempt. Surgical recanalization was attempted in 7 patients of which 3 had successful recanalization (43%) and catheter-directed recanalization was attempted in 14 patients with 100% success in re-establishing blood flow to the graft. Additionally, patency was increased, and mortality was decreased in patients treated with catheter-directed recanalization compared to surgical revascularization or anticoagulation alone. Conclusion:This data illustrates the need to further investigate catheter-directed thrombolysis as a potential first-line treatment for postoperative HAT and PVT in pediatric liver transplant recipients. How to cite this article: Moreno NF, Hernandez JA, Huang C-S, et al. Our evolution in the treatment of hepatic artery and portal vein thrombosis in pediatric liver transplantation: Success with catheter-directed therapies.

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