Antenatal immunoglobulin for prevention of neonatal hemochromatosis

Abstract

Neonatal hemochromatosis (NH) is a rare disease with a poor prognosis, particularly prior to 2008. Antenatal maternal high-dose immunoglobulin (Ig) is effective in preventing NH recurrence, but the adverse effects of this treatment have not been documented as yet. Here, we report on a patient who underwent high-dose Ig treatment to prevent NH recurrence. The patient was a 31-year-old pregnant Japanese woman. Her first child died of NH after receiving living donor liver transplantation. The patient received high-dose Ig treatment to prevent recurrence of NH from gestational weeks 16 to 35. During the treatment, platelet count gradually decreased, and cesarean section was required at 35 gestational weeks. The child did not develop liver failure. High-dose Ig prevented the recurrence of NH. Caution should be exercised due to possible adverse effects of this treatment.

Outcomes After Living Donor Liver Transplantation in Pediatric Patients with Inherited Metabolic Diseases

“…Eleven patients with IMDs who had younger siblings after LDLT included patients with neonatal hemochromatosis (n=4), OTCD (n=3), progressive familial intrahepatic cholestasis type 1 (n=1), carbamoyl phosphate synthetase 1 deficiency (n=1), MMA (n=1), and Niemann-Pick disease type C (n=1). Of the younger siblings of 4 patients with neonatal hemochromatosis, 2 underwent antenatal maternal high-dose immunoglobulin treatment to prevent neonatal hemochromatosis [ 22 ]. However, 1 child without immunoglobulin treatment who underwent LDLT from the puerperal mother developed neonatal hemochromatosis and another did not develop neonatal hemochromatosis.…”

“…If a woman has an unplanned pregnancy without having received sufficient information, she may choose to undergo an abortion. In this study, of the younger siblings of 4 patients with neonatal hemochromatosis, 2 were born after the mother received antenatal maternal high-dose immunoglobulin treatment to prevent neonatal hemochromatosis [ 22 ]. One child whose mother did not receive immunoglobulin treatment who underwent LDLT from the puerperal mother developed neonatal hemochromatosis.…”