Maternal Immunoglobulin Treatment of Gestational Alloimmune Liver Disease to Prevent Neonatal Liver Failure: A Single Center Case Series.

BACKGROUND

Gestational alloimmune liver disease (GALD), previously known as neonatal hemochromatosis, is a rare maternal-fetal alloimmune disease causing severe fetal-neonatal liver failure. Understanding the immunological origin resulted in the concept of treatment with high-dose intravenous immunoglobins (IVIG) during gestation.

METHODS

This retrospective single-center cohort study consists of seven women with index cases of GALD, followed by a total of ten IVIG-treated singleton pregnancies.

RESULTS

Out of seven index pregnancies, five resulted in neonatal deaths. In a total of ten subsequent pregnancies all seven women at risk received antenatal IVIG (1g/kg body weight weekly) starting at median 14 weeks of gestation. Pregnancy courses were uneventful, except for one case of fetal growth restriction and preterm delivery due to severe preeclampsia. No severe maternal adverse effects of repetitive IVIG (median 23 applications) were observed. None of the infants from IVIG-treated women developed severe GALD. Two out of ten neonates displayed slightly abnormal laboratory parameters but did not require exchange transfusion or any other specific treatment. Based on standard laboratory data, we developed a scoring method to identify neonates at risk for GALD-associated liver failure.

CONCLUSION

This case series indicates that weekly antenatal IVIG treatment is highly efficient to prevent GALD-associated neonatal liver failure. In contrast to other cases series, there were no major maternal side effects. An introduced novel laboratory scoring system to decide on early postnatal intervention requires validation in a larger number of neonates.