Kasko Oksana, Klose Erica, Rama Ganapathy, Newberry Desi, Jnah Amy
Neonatal Netw. 2018 Sep;37(5):271-280. doi: 10.1891/0730-0832.37.5.271.
Gestational alloimmune liver disease (GALD) is initiated by maternal antibodies that attack fetal hepatocytes. The fetal immune response to the antibodies causes liver damage. The incidence of GALD is four per 100,000 live births in the United States. Frequently, liver injury leads to fetal loss or neonatal demise; nonetheless, the presentation of GALD has a wide range of severity. Survival rates have increased from 20 to 80 percent changes in treatment and understanding of GALD. Current treatment is focused on supportive care with intravenous immunoglobulin (IVIG) and exchange transfusions. Mortality risk is positively associated with the timing of diagnosis. Although there has been an increase in understanding this disease, the discovery of the specific alloantigen is still needed. Relevant embryology, pathophysiology, clinical manifestations, diagnosis, medical treatment, and prognosis are discussed to aid health care professionals in the early identification and treatment for the neonate and family unit.
妊娠同种免疫性肝病(GALD)由攻击胎儿肝细胞的母体抗体引发。胎儿对这些抗体的免疫反应会导致肝脏损伤。在美国,GALD的发病率为每10万例活产中有4例。肝脏损伤常常导致胎儿丢失或新生儿死亡;尽管如此,GALD的表现严重程度范围很广。随着对GALD治疗和认识的改变,存活率已从20%提高到80%。目前的治疗重点是采用静脉注射免疫球蛋白(IVIG)和换血进行支持治疗。死亡风险与诊断时间呈正相关。尽管对这种疾病的认识有所增加,但仍需要发现特定的同种抗原。本文讨论了相关的胚胎学、病理生理学、临床表现、诊断、医学治疗和预后,以帮助医护人员对新生儿及家庭单元进行早期识别和治疗。