Liu Joy, Ghaziani Tara T, Wolf Jacqueline L
Department of Internal Medicine, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
Department of Medicine, Division of Gastroenterology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
Am J Gastroenterol. 2017 Jun;112(6):838-846. doi: 10.1038/ajg.2017.54. Epub 2017 Mar 14.
Acute fatty liver of pregnancy (AFLP) is an obstetric emergency characterized by maternal liver failure and may have complications for the mother and fetus, including death. This review examines recent literature on the epidemiology, pathogenesis, diagnosis, and treatment of acute fatty liver of pregnancy. Pathogenesis of this disease has been linked to defects in fatty acid metabolism during pregnancy, especially in the setting of fetal genetic defects in fatty acid oxidation. The value of screening all patients for these genetic defects remains to be determined. Distinguishing AFLP from other high-risk liver diseases of pregnancy that have overlap features, such as HELLP and preeclampsia, can be challenging. Although sensitive diagnostic tools such as the Swansea criteria have been developed, further work is needed to diagnose AFLP more quickly. Although survival rates have improved in the past 30 years, delay in diagnosis and treatment of AFLP has life-threatening consequences; an algorithmic approach to AFLP may be a valuable resource for clinicians. Future epidemiological and long-term studies will improve our prediction of women at risk for developing AFLP and determine the long-term consequences of this condition.
妊娠急性脂肪肝（AFLP）是一种以孕产妇肝功能衰竭为特征的产科急症，可能会给母亲和胎儿带来包括死亡在内的并发症。本综述探讨了近期关于妊娠急性脂肪肝的流行病学、发病机制、诊断和治疗的文献。该疾病的发病机制与孕期脂肪酸代谢缺陷有关，尤其是在胎儿脂肪酸氧化存在遗传缺陷的情况下。对所有患者进行这些遗传缺陷筛查的价值仍有待确定。将AFLP与其他具有重叠特征的妊娠高危肝病（如HELLP和子痫前期）区分开来可能具有挑战性。尽管已经开发了如斯旺西标准等敏感的诊断工具，但仍需要进一步开展工作以更快地诊断AFLP。尽管在过去30年中生存率有所提高，但AFLP诊断和治疗的延迟会带来危及生命的后果；针对AFLP的算法方法可能是临床医生的宝贵资源。未来的流行病学和长期研究将改善我们对有发生AFLP风险女性的预测，并确定这种情况的长期后果。
