Gestational Alloimmune Liver Disease: A Devastating Condition Preventable With Maternal Intravenous Immunoglobulin.

BACKGROUND: Gestational alloimmune liver disease, a form of profound liver failure in the newborn, is the main underlying cause of the entity formerly known as neonatal hemochromatosis. Antepartum maternal intravenous immunoglobulin (IVIG) has been shown to prevent gestational alloimmune liver disease, which otherwise has a recurrence risk above 90% in subsequent pregnancies.

CASE: A 30-year-old woman, gravida 3 para 0120, presented early in gestation. Her previous pregnancy had been complicated by fetal growth restriction, oligohydramnios, and ultimately fatal fulminant neonatal liver failure. With gestational alloimmune liver disease recognized as the primary diagnosis for the liver failure, we began maternal weekly IVIG therapy. She delivered a healthy newborn at term without evidence of hepatic dysfunction.

CONCLUSION: Recognition of gestational alloimmune liver disease enables antepartum treatment that dramatically alters the course of disease.