Etiology, Characteristics, and Outcomes of Neonatal Liver Failure: Lessons Learned Over the Last 3 Decades

Abstract

To evaluate trends in etiology and outcomes of neonatal liver failure (NLF) over 30years retrospectively at a single institution. Inclusion criteria for this retrospective cohort study were babies presenting at a chronological age of ≤28days between 1991 and 2020 with prothrombin time≥20seconds and biochemical liver injury. Demographics, etiology, laboratory investigations, need for extrahepatic organ support, acute kidney injury, and intervention with liver transplant (LT) were recorded. Survival outcomes were measured as discharge from the hospital alive with native liver or LT. The study period was stratified into 3 10-year blocks. Trends were analyzed for hospital admissions, etiology, and survival outcomes. One hundred twenty-six babies met the NLF criteria. Admissions to the hospital increased from 21 in 1991-2000 to 65 in 2011-2020. An increasing trend in infectious and metabolic causes, while a decreasing trend in indeterminate etiology, was noted. Survival with native liver improved from 23.8% in 1991-2000 to 55.4% in 2011-20 (P=.021), and mortality reduced from 52.4% to 35.4% during the same periods (P=.213). Twenty-three (18.2%) neonates received LT. Post-LT survival outcomes were 100% for gestational alloimmune liver disease, 66.6% in the indeterminate group, and 25% for herpes simplex virus. Specific etiologies (gestational alloimmune liver disease, OR=0.07 [0-0.77, P=.048]), presence of acute kidney injury (OR=6.22 [1.45, 29.38, P=.015]) and need for inotropes (OR=6.22 [1.45, 29.38, P=.028]) influenced mortality in multivariable logistic regression analysis. In the last 30years, advances in diagnosis, treatment, and increasing experience with LT have improved survival in NLF.