Feldman Amy G, Whitington Peter F
Department of Pediatrics, Feinberg Medical School of Northwestern University, Ann and Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Box 57, Chicago, IL 60611-2605, United States.
J Clin Exp Hepatol. 2013 Dec;3(4):313-20. doi: 10.1016/j.jceh.2013.10.004. Epub 2013 Nov 27.
Neonatal hemochromatosis is a clinical condition in which severe liver disease in the newborn is accompanied by extrahepatic siderosis. Gestational alloimmune liver disease (GALD) has been established as the cause of fetal liver injury resulting in nearly all cases of NH. In GALD, a women is exposed to a fetal antigen that she does not recognize as "self" and subsequently begins to produce IgG antibodies that are directed against fetal hepatocytes. These antibodies bind to fetal liver antigen and activate the terminal complement cascade resulting in hepatocyte injury and death. GALD can cause congenital cirrhosis or acute liver failure with and without iron overload and siderosis. Practitioners should consider GALD in cases of fetal demise, stillbirth, and neonatal acute liver failure. Identification of infants with GALD is important as treatment is available and effective for subsequent pregnancies.
新生儿血色沉着症是一种临床病症，表现为新生儿严重肝病伴有肝外铁沉积。妊娠性同种免疫性肝病（GALD）已被确认为几乎所有新生儿血色沉着症病例中胎儿肝损伤的病因。在妊娠性同种免疫性肝病中，女性接触到一种她不识别为“自身”的胎儿抗原，随后开始产生针对胎儿肝细胞的IgG抗体。这些抗体与胎儿肝脏抗原结合并激活终末补体级联反应，导致肝细胞损伤和死亡。妊娠性同种免疫性肝病可导致先天性肝硬化或伴有或不伴有铁过载和铁沉积的急性肝衰竭。从业者在胎儿死亡、死产和新生儿急性肝衰竭的病例中应考虑妊娠性同种免疫性肝病。识别患有妊娠性同种免疫性肝病的婴儿很重要，因为有针对后续妊娠的可用且有效的治疗方法。
