The Effects of Gestational Alloimmune Liver Disease on Fetal and Infant Morbidity and Mortality.
J Pediatr
; 196: 123-128.e1, 2018 05.
Article
en En
| MEDLINE
| ID: mdl-29499991
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. STUDYDESIGN:
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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Inmunoglobulinas Intravenosas
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Fallo Hepático
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Hemocromatosis
Tipo de estudio:
Etiology_studies
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Observational_studies
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Prevalence_studies
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Risk_factors_studies
Límite:
Female
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Humans
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Infant
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Male
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Newborn
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Pregnancy
Idioma:
En
Revista:
J Pediatr
Año:
2018
Tipo del documento:
Article