论文部分内容阅读
Background Neonatal haemochromatosis is a rare disease of gestation that results in severe fetal liver injury. We hypothesised an alloimmune aetiology for the disorder on the basis of its high recurrence rate in sibships. In this study, we assessed the effectiveness in preventing or changing the severity of recurrent neonatal haemochromatosis of administering during pregnancy highdose intravenous immunoglobulin (IVIG) derived from pooled serum of multiple donors. Methods Women whose most recent pregnancy ended in documented neonatal haemochromatosis were treated with IVIG, 1 g/kg bodyweight, weekly from the 18th week until the end of gestation in their subsequent pregnancy. The outcomes of treated pregnancies were compared with those of randomly selected previous affected pregnancies for each woman, which were used as historical controls. Findings 15 women were treated through 16 pregnancies. All pregnancies progressed uneventfully and resulted in live babies with normal physical examinations and birthweights that were appropriate for gestational age. 12 babies had evidence of liver involvement with neonatal haemochromatosis: 11 had higher than normal concentrations of serum αfetoprotein and ferritin or serum αfetoprotein alone, including four with coagulopathy (international normalised ratio >1.5), and one had coagulopathy alone. All babies survived with medical or no treatment and were healthy at followup within the past 6 months. In analysis on a permother basis comparing outcomes of treated gestations with those of randomly selected previous affected gestations, gestational IVIG therapy was associated with better infant survival (15 good outcomes vs two in previous pregnancies; P=0.0009). Interpretation Treatment with highdose IVIG during gestation appears to have modified recurrent neonatal haemochromatosis so that it was not lethal to the fetus or neonate. These results further support an alloimmune mechanism for recurrent neonatal haemochromatosis.
Background Neonatal haemochromatosis is a rare disease of gestation that results in severe fetal liver injury. We hypothesised an alloimmune aetiology for the disorder on the basis of its high recurrence rate in sibships. In this study, we assessed the effectiveness in preventing or changing the severity of recurrent neonatal haemochromatosis of pregnancy during high-dose intravenous immunoglobulin (IVIG) derived from pooled serum of multiple donors. Methods Women who most recently pregnancy ended in documented neonatal haemochromatosis were treated with IVIG, 1 g / kg bodyweight, weekly from the 18th week until the end of gestation in their subsequent pregnancy. The outcomes of treated pregnancies were compared with those of randomly elected recently suffered pregnancies for each woman, which were used as historical controls. Findings 15 women were treated through 16 pregnancies. All pregnancies progressed uneventfully and resulted in live babies with normal physical ex aminations and birthweights that were appropriate for gestational age. 12 babies had evidence of liver involvement with neonatal haemochromatosis: 11 had higher than normal concentrations of serum αfetoprotein and ferritin or serum αfetoprotein alone, including four with coagulopathy (international normalized ratio> 1.5), and one had coagulopathy alone. All babies survived with medical or no treatment and were healthy at follow up within the past 6 months. In analysis on a permother basis comparing outcomes of treated gestations with those of randomly selected previous affected gestations, gestational IVIG therapy was associated with better infant survival (15 good outcomes vs two in previous pregnancies; P = 0.0009). Interpretation Treatment with highdose IVIG during gestation appears to have modified recurrent neonatal haemochromatosis so that it was not lethal to the fetus or neonate. These results further support an alloimmune mechanism for recurrent neonatal haemochr omatosis.