大剂量免疫球蛋白对妊娠期复发性新生儿血色素沉着病的治疗

作     者：Whitington P.F. Hibbard J.U. 高雪莲 

作者机构：Department of Pediatrics Feinberg Sch. of Med. of NW Univ. Children’s Memorial Hospital 2300 Children’s Plaza Box 57 Chicago IL 60614 United States 

出 版 物：《世界核心医学期刊文摘（妇产科学分册）》 (Core Journal in Obstetrics/Gynecology)

年 卷 期：2005年第1卷第4期

页      面：5-6页

学科分类：1002[医学-临床医学] 100202[医学-儿科学] 10[医学] 

主　　题：妊娠期妇女 免疫球蛋白 妊娠疾病 同胞群 高复发率 血清甲胎蛋白 终止妊娠 血色素沉着病 免疫性疾病 同种免疫 

摘      要：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 highdose 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 αfetoprotein and ferritin or serum αfetoprotein 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 followup within the past 6 months. In analysis on a permother 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 highdose 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.