Dr Yen Hun Wee
Eastern Health

 ABSTRACT

Title: A case report of failure of antenatal Intravenous Immunoglobulin to prevent Gestational Alloimmune Liver Disease
Jonathan M. Blackwell 1, Emily K. Hartman 2, Keith G Hartman 1, Guy D. Eslick 3.
1
Mater Hospital, North Sydney,
2 Notre Dame University, Sydney,
3 University of Sydney

Treatment for Gestational Alloimmune Liver Disease (GALD) (also known as Neonatal Haemochromatosis) in mothers with a history of the disease is antenatal intravenous immunoglobulin (IVIG). Reports have demonstrated favourable outcomes. We report a case of failure of antenatal IVIG to prevent GALD.

The mother has a history of one stillbirth at 39 weeks. A second pregnancy was lost at 10 weeks; the foetus had chromosomal abnormalities. A third pregnancy was complicated by moderate thrombocytopenia with an onset at 13 weeks. The child was delivered at 35 weeks. The child developed liver disease rapidly and was diagnosed with GALD. A diagnosis of GALD was made on the first pregnancy retrospectively after histopathology was reexamined.

A fourth pregnancy was treated with IVIG (Intragam P, CSL) according to the protocol current at that time. Mild thrombocytopenia was noted late in this pregnancy. The neonate developed severe liver disease in the third trimester precipitating an emergency delivery. The child went on to have a successful liver transplant.

Results: The literature describes antenatal treatment of GALD in 203 pregnancies. The majority of the neonates were delivered at term. One third of the neonates (31%) presented with abnormal liver function at birth and one quarter (24%) of the neonates required medical treatment. No child required liver transplantation. It is possible that other treatment failures have occurred and have not been reported.

Conclusion: We report a case of failure of antenatal IVIG to prevent GALD. Establishment of a registry for GALD may facilitate a better understanding of this rare disease.