Gestational autoimmune disease in newborns with an indeterminate cause of death following a complete autopsy

Journal of Neonatal-perinatal Medicine
Hector Melin-AldanaPeter F Whitington

Abstract

Gestational alloimmune liver disease (GALD) is the result of neonatal complement-mediated severe liver injury mediated 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 cause of death classified as indeterminate. We reviewed the pathologic diagnoses from autopsy material 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 cause of death were identified and divided in 3 groups: (1) No clinical or autopsy-derived diagnoses (n = 7), (2) Defined clinical diagnoses but no cause of death determined at autopsy (n = 2), and (3) Liver disease, but no clinical or autopsy diagnoses 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...Continue Reading

References

Oct 18, 2003·Gastroenterology Clinics of North America·A S KniselyPeter F Whitington
Mar 25, 2006·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Peter F Whitington
Dec 4, 2008·Advances in Pediatrics·Alice Chang HoftmanE Richard Stiehm
Jun 1, 2010·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Xiaomin PanPeter F Whitington
May 20, 2011·The Journal of Pediatrics·Peter F WhitingtonPadmini Malladi
Feb 11, 2012·Journal of Hepatology·Silvana BonillaPeter F Whitington
Nov 20, 2012·Journal of Zoo and Wildlife Medicine : Official Publication of the American Association of Zoo Veterinarians·Stephanie Venn-WatsonJudy St Leger

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Citations

Mar 8, 2016·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Sarah A Taylor, Peter F Whitington
Dec 30, 2017·Journal of Pediatric Gastroenterology and Nutrition·Khaled AleneziGovind B Chavhan

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Methods Mentioned

BETA
flow cytometry

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