Whitington Peter F, Kelly Susan
Children's Memorial Hospital, Mail Box 57, 2300 Children's Plaza, Chicago, IL 60614, USA.
Pediatrics. 2008 Jun;121(6):e1615-21. doi: 10.1542/peds.2007-3107. Epub 2008 May 12.
Neonatal hemochromatosis is the result of severe fetal liver injury that seems to result from maternal-fetal alloimmunity. Women who have had an infant affected with neonatal hemochromatosis are at high risk in subsequent pregnancies for having another affected infant. This study was designed to determine whether therapy directed at limiting the severity of gestational alloimmunity can reduce the occurrence of severe neonatal hemochromatosis in infants of women at risk. A secondary objective was to use a prospectively collected data set to examine questions of vital interest about neonatal hemochromatosis.
Women with a history of pregnancy ending in documented neonatal hemochromatosis were treated with intravenous immunoglobulin at 1 g/kg of body weight weekly from week 18 until the end of gestation. Extensive data were prospectively collected regarding the gestational histories of the subjects. The outcomes of treated pregnancies were compared with those of previous affected pregnancies, which were used as historical controls.
Forty-eight women were enrolled to be treated during 53 pregnancies. The gestational histories of these women demonstrated the high risk of occurrence of neonatal hemochromatosis: 92% of pregnancies at risk resulted in intrauterine fetal demise, neonatal death, or liver failure necessitating transplant. In contrast, with gestational therapy, the 53 at-risk gestations resulted in 3 failures and 52 infants who survived intact with medical therapy alone. When compared on a per-woman or per-infant basis, the outcome of gestation at risk for neonatal hemochromatosis was improved by gestational therapy.
Neonatal hemochromatosis seems to be the result of a gestational alloimmune disease, and occurrence of severe neonatal hemochromatosis in at-risk pregnancies can be significantly reduced by treatment with high-dose intravenous immunoglobulin during gestation.
新生儿血色素沉着症是严重胎儿肝损伤的结果，这种损伤似乎源于母胎同种免疫。生育过患新生儿血色素沉着症婴儿的女性，在后续妊娠中有很高风险再次生育患病婴儿。本研究旨在确定针对限制妊娠同种免疫严重程度的治疗能否降低高危女性所生婴儿中严重新生儿血色素沉着症的发生率。次要目的是利用前瞻性收集的数据集来研究有关新生儿血色素沉着症的重要问题。
有记录显示妊娠结局为新生儿血色素沉着症的女性，从妊娠第18周开始至妊娠结束，每周接受1克/千克体重的静脉注射免疫球蛋白治疗。前瞻性收集了关于这些受试者妊娠史的大量数据。将接受治疗的妊娠结局与之前受影响的妊娠结局进行比较，之前的妊娠结局用作历史对照。
48名女性参与了53次妊娠的治疗。这些女性的妊娠史显示出新生儿血色素沉着症的高发病风险：92%的高危妊娠导致宫内胎儿死亡、新生儿死亡或因肝衰竭而需要进行移植。相比之下，通过妊娠治疗，这53次高危妊娠中有3次失败，52名婴儿仅通过药物治疗就完好存活。按每位女性或每名婴儿进行比较时，妊娠治疗改善了新生儿血色素沉着症高危妊娠的结局。
新生儿血色素沉着症似乎是一种妊娠同种免疫疾病的结果，在高危妊娠中，通过妊娠期高剂量静脉注射免疫球蛋白治疗可显著降低严重新生儿血色素沉着症的发生率。
