https://pubmed.ncbi.nlm.nih.gov/33633086/ Acute Liver Failure

J Pediatr Gastroenterol Nutr. 2021 Feb 24.
doi: 10.1097/MPG.0000000000003103.Online ahead of print.
Applying an Age-Specific Definition to Better Characterize Etiologies and Outcomes in Neonatal Acute Liver Failure

Kristin Borovsky 1, Anna M Banc-Husu, Samantha A Saul, Katie Neighbors, Susan Kelly, Estella M Alonso, Sarah A Taylor
Abstract

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. 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%), cardiac-associated ischemia (16%), other ischemia (14%), genetic etiologies (9%), Trisomy 21 associated myelodysplasia (7%), hemophagocytic lymphohistiocytosis (HLH) (2%), and not identified (7%). Infants with viral etiologies had the highest alanine aminotransferase at presentation (1179 IU/L, 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 was significantly higher in patients that survived with their native liver (p = 0.04).

Conclusions: Overall, outcome for neonatal ALF is poor. While initial laboratory values can differentiate viral infection or GALD-NH, further studies are needed to identify laboratory parameters that predict survival with native liver by etiology to ultimately improve patient outcomes.