The Effects of Gestational Alloimmune Liver Disease on Fetal and Infant Morbidity and Mortality

Sarah A. Taylor*, Susan Kelly, Estella M. Alonso, Peter F. Whitington

*Corresponding author for this work

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Objectives: To evaluate pregnancy outcomes in pedigrees of neonatal hemochromatosis to determine the spectrum of gestational alloimmune liver disease (GALD) in a large cohort. Study design: We prospectively collected data from women with a prior offspring with proven neonatal hemochromatosis between 1997 and 2015 and analyzed pregnancy outcomes. Results: The pedigrees from 150 women included 350 gestations with outcomes potentially related to GALD. There were 105 live-born infants without liver disease, 157 live-born infants with liver failure, and 88 fetal losses. Fetal loss occurred in 25% of total gestations. Ninety-seven pedigrees contained a single affected offspring, whereas 53 contained multiple affected offspring. Analysis of these 53 pedigrees yielded a per-pregnancy repeat occurrence rate of 95%. Notably, the first poor outcome occurred in the first pregnancy in 60% of pedigrees. Outcomes of the 157 live-born infants with liver failure were poor: 18% survived, 82% died. Of the 134 live-born infants with treatment data, 20 received intravenous immunoglobulin with or without double-volume exchange transfusion of which 9 (45%) survived; 14 infants (10%) received a liver transplant of which 6 (43%) survived. Conclusions: GALD is a significant cause of both fetal loss and neonatal mortality with a high rate of disease recurrence in untreated pregnancies at risk. Poor outcomes related to GALD commonly occur in the first gestation, necessitating a high index of suspicion to diagnose this disorder at first presentation.

Original languageEnglish (US)
Pages (from-to)123-128.e1
JournalJournal of Pediatrics
Volume196
DOIs
StatePublished - May 1 2018

Fingerprint

Infant Mortality
Pedigree
Liver Diseases
Morbidity
Pregnancy
Liver Failure
Pregnancy Outcome
Fetal Mortality
Intravenous Immunoglobulins
Transplants
Recurrence
Liver

Keywords

  • neonatal hemochromatosis
  • neonatal liver failure

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

@article{938b416ac5f443d19991f71e8bb49d44,
title = "The Effects of Gestational Alloimmune Liver Disease on Fetal and Infant Morbidity and Mortality",
abstract = "Objectives: To evaluate pregnancy outcomes in pedigrees of neonatal hemochromatosis to determine the spectrum of gestational alloimmune liver disease (GALD) in a large cohort. Study design: We prospectively collected data from women with a prior offspring with proven neonatal hemochromatosis between 1997 and 2015 and analyzed pregnancy outcomes. Results: The pedigrees from 150 women included 350 gestations with outcomes potentially related to GALD. There were 105 live-born infants without liver disease, 157 live-born infants with liver failure, and 88 fetal losses. Fetal loss occurred in 25{\%} of total gestations. Ninety-seven pedigrees contained a single affected offspring, whereas 53 contained multiple affected offspring. Analysis of these 53 pedigrees yielded a per-pregnancy repeat occurrence rate of 95{\%}. Notably, the first poor outcome occurred in the first pregnancy in 60{\%} of pedigrees. Outcomes of the 157 live-born infants with liver failure were poor: 18{\%} survived, 82{\%} died. Of the 134 live-born infants with treatment data, 20 received intravenous immunoglobulin with or without double-volume exchange transfusion of which 9 (45{\%}) survived; 14 infants (10{\%}) received a liver transplant of which 6 (43{\%}) survived. Conclusions: GALD is a significant cause of both fetal loss and neonatal mortality with a high rate of disease recurrence in untreated pregnancies at risk. Poor outcomes related to GALD commonly occur in the first gestation, necessitating a high index of suspicion to diagnose this disorder at first presentation.",
keywords = "neonatal hemochromatosis, neonatal liver failure",
author = "Taylor, {Sarah A.} and Susan Kelly and Alonso, {Estella M.} and Whitington, {Peter F.}",
year = "2018",
month = "5",
day = "1",
doi = "10.1016/j.jpeds.2017.12.054",
language = "English (US)",
volume = "196",
pages = "123--128.e1",
journal = "Journal of Pediatrics",
issn = "0022-3476",
publisher = "Mosby Inc.",

}

TY - JOUR

T1 - The Effects of Gestational Alloimmune Liver Disease on Fetal and Infant Morbidity and Mortality

AU - Taylor, Sarah A.

AU - Kelly, Susan

AU - Alonso, Estella M.

AU - Whitington, Peter F.

PY - 2018/5/1

Y1 - 2018/5/1

N2 - Objectives: To evaluate pregnancy outcomes in pedigrees of neonatal hemochromatosis to determine the spectrum of gestational alloimmune liver disease (GALD) in a large cohort. Study design: We prospectively collected data from women with a prior offspring with proven neonatal hemochromatosis between 1997 and 2015 and analyzed pregnancy outcomes. Results: The pedigrees from 150 women included 350 gestations with outcomes potentially related to GALD. There were 105 live-born infants without liver disease, 157 live-born infants with liver failure, and 88 fetal losses. Fetal loss occurred in 25% of total gestations. Ninety-seven pedigrees contained a single affected offspring, whereas 53 contained multiple affected offspring. Analysis of these 53 pedigrees yielded a per-pregnancy repeat occurrence rate of 95%. Notably, the first poor outcome occurred in the first pregnancy in 60% of pedigrees. Outcomes of the 157 live-born infants with liver failure were poor: 18% survived, 82% died. Of the 134 live-born infants with treatment data, 20 received intravenous immunoglobulin with or without double-volume exchange transfusion of which 9 (45%) survived; 14 infants (10%) received a liver transplant of which 6 (43%) survived. Conclusions: GALD is a significant cause of both fetal loss and neonatal mortality with a high rate of disease recurrence in untreated pregnancies at risk. Poor outcomes related to GALD commonly occur in the first gestation, necessitating a high index of suspicion to diagnose this disorder at first presentation.

AB - Objectives: To evaluate pregnancy outcomes in pedigrees of neonatal hemochromatosis to determine the spectrum of gestational alloimmune liver disease (GALD) in a large cohort. Study design: We prospectively collected data from women with a prior offspring with proven neonatal hemochromatosis between 1997 and 2015 and analyzed pregnancy outcomes. Results: The pedigrees from 150 women included 350 gestations with outcomes potentially related to GALD. There were 105 live-born infants without liver disease, 157 live-born infants with liver failure, and 88 fetal losses. Fetal loss occurred in 25% of total gestations. Ninety-seven pedigrees contained a single affected offspring, whereas 53 contained multiple affected offspring. Analysis of these 53 pedigrees yielded a per-pregnancy repeat occurrence rate of 95%. Notably, the first poor outcome occurred in the first pregnancy in 60% of pedigrees. Outcomes of the 157 live-born infants with liver failure were poor: 18% survived, 82% died. Of the 134 live-born infants with treatment data, 20 received intravenous immunoglobulin with or without double-volume exchange transfusion of which 9 (45%) survived; 14 infants (10%) received a liver transplant of which 6 (43%) survived. Conclusions: GALD is a significant cause of both fetal loss and neonatal mortality with a high rate of disease recurrence in untreated pregnancies at risk. Poor outcomes related to GALD commonly occur in the first gestation, necessitating a high index of suspicion to diagnose this disorder at first presentation.

KW - neonatal hemochromatosis

KW - neonatal liver failure

UR - http://www.scopus.com/inward/record.url?scp=85042596270&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85042596270&partnerID=8YFLogxK

U2 - 10.1016/j.jpeds.2017.12.054

DO - 10.1016/j.jpeds.2017.12.054

M3 - Article

VL - 196

SP - 123-128.e1

JO - Journal of Pediatrics

JF - Journal of Pediatrics

SN - 0022-3476

ER -