%0 Journal Article
%T Extrahepatic Nonreticuloendothelial Siderosis Is Not Specific to Gestational Alloimmune Liver Disease
%A Debra Kearney
%A Eumenia Castro
%A Hao Wu
%A Kalyani Patel
%A Milton Finegold
%A William Ferguson
%J Pediatric and Developmental Pathology
%@ 1615-5742
%D 2019
%R 10.1177/1093526619826429
%X Autopsy reports of 78 stillbirths and early infant deaths (up to age 8 weeks) were reviewed to investigate the prevalence of extrahepatic nonreticuloendothelial siderosis (EHNRS) in the context of neonatal liver failure. Of these, 10 liveborns (12.8%), M:F 3:2, with mean gestational age 37.6 weeks (range: 35¨C39) and mean age at the time of demise 19.1 days (range: 7¨C42), showed significant liver injury: infection (n£¿=£¿7, viral£¿>£¿fungal), congenital malformations (n£¿=£¿2), and ischemia (n£¿=£¿1). None had maternal history of gestational alloimmune liver disease (GALD) or previous fetal/neonatal death due to liver failure. Seven of 10 cases (70%) showed EHNRS: pancreas (n£¿=£¿6), kidneys (n£¿=£¿4), thyroid and adrenal glands (n£¿=£¿3), and bronchial glands and heart (n£¿=£¿2). Iron deposition was most frequent in the pancreas (60%), most diffuse in the kidneys, and seen in at least 2 organs, with pancreas and kidney being the most frequent combination. Hepatic C5b-9 expression was variable (1+ to 4+) except 1 case (100% necrosis). The duration of illness and the mean age at the time of demise tended to be higher in those with EHNRS. In summary, hepatic and EHNRS, with or without C5b-9 expression, are not specific for GALD. Other causes of liver failure should be investigated as clinically and pathologically appropriate
%K extrahepatic
%K siderosis
%K GALD
%K C5b-9
%K liver failure
%K neonatal
%U https://journals.sagepub.com/doi/full/10.1177/1093526619826429