Gestational autoimmune disease in newborns with an indeterminate cause of death following a complete autopsy
2015
Gestational
alloimmuneliver disease (GALD) is the result of neonatal complement-mediated severe
liver injurymediated by maternal alloantibodies, which is detected by immunohistochemistry staining for the complement C5b-9 complex. GALD leads to the
neonatal hemochromatosis(NH) phenotype, which also shows extrahepatic
siderosis, and can result in neonatal death. At
autopsy, the histologic damage of the liver in GALD may be subtle and misinterpreted as non-specific post- mortem changes, resulting in the
causeof
deathclassified as
indeterminate. We reviewed the pathologic diagnoses from
autopsymaterial from 1996 to 2011 of infants 0-90 days of age from our institution. Liver samples were stained with H&E, trichrome and for C5b-9. 13 cases originally diagnosed as
indeterminate
causeof
deathwere identified and divided in 3 groups: (1) No clinical or
autopsy-derived diagnoses (n = 7), (2) Defined clinical diagnoses but no
causeof
deathdetermined at
autopsy(n = 2), and (3) Liver disease, but no clinical or
autopsydiagnoses to establish the cause of the
liver injury(n = 4). On reexamination, all group 1 and 3 cases were reclassified as GALD, based on a positive C5b-9 stain. Group 2 cases were not GALD, retaining the original, clinically-based
causeof
death. We conclude that, in cases of
indeterminatecause of neonatal death, very careful examination for hepatocyte injury/necrosis, extrahepatic
siderosis, liver fibrosis and/or C5b-9 stain should be considered.
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