Taylor SA, et al. - The intent of the authors was to scrutinize the pregnancy outcomes in pedigrees of neonatal hemochromatosis with the purpose of identifying the spectrum of gestational alloimmune liver disease (GALD) in a large cohort. Findings reported that GALD served as a major cause of fetal loss and neonatal mortality, displaying a high rate of disease recurrence in untreated pregnancies at risk. The occurrence of poor outcomes linked with GALD was commonly noted in the first gestation. This, in turn, warranted a high index of suspicion to diagnose this disorder at first presentation.

Methods

• Data was cumulated from women with a prior offspring with proven neonatal hemochromatosis between 1997 and 2015.
• During this study, pregnancy outcomes were assessed.

Results

• Herein, the pedigrees from 150 women consisted of 350 gestations with outcomes potentially associated with GALD.
• A total of 105 live-born infants were reported without liver disease, 157 live-born infants with liver failure, and 88 fetal losses.
• The occurrence of fetal loss was noted in 25% of total gestations.
• As per the findings, 97 pedigrees contained a single affected offspring.
• On the other hand, 53 contained multiple affected offspring.
• A per-pregnancy repeat occurrence rate of 95% was illustrated through the analysis of these 53 pedigrees.
• In 60% of pedigrees, the occurrence of first poor outcome was found in the first pregnancy .
• Poor outcomes were yielded of the 157 live-born infants with liver failure: 18% survived, 82% died.
• Findings disclosed that among 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.