Treem William R
Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Duke Children's Hospital, Durham, NC, USA.
Semin Gastrointest Dis. 2002 Jan;13(1):55-66.
Accumulation of micro- and macrovesicular fat in hepatocytes is the hallmark of several liver diseases of pregnancy, including acute fatty liver of pregnancy (AFLP) and severe pre-eclampsia. While the exact origin of AFLP is not known, this pathologic finding and its similarity to the hepatopathology found in infants and children with inborn errors of mitochondrial fatty acid-oxidation suggests a common pathogenesis. Over the last few years, the incidence of AFLP and other severe complications of pregnancy have been found to be increased in women who carry a fetus with a defect in fatty acid oxidation, and who are themselves carriers of a genetic mutation that partially compromises their own intramitochondrial fatty acid oxidation pathway. This article reviews evidence that links AFLP with both genetic and acquired factors that compromise intramitochondrial fatty acid oxidation. Based on this review, recommendations are offered for obstetric, perinatal, and neonatal management of affected mothers and infants.
肝细胞中微泡和大泡脂肪的蓄积是几种妊娠肝病的标志，包括妊娠急性脂肪肝（AFLP）和重度子痫前期。虽然AFLP的确切病因尚不清楚，但这一病理发现及其与线粒体脂肪酸氧化先天性缺陷的婴幼儿肝脏病理的相似性提示了共同的发病机制。在过去几年中，已发现携带脂肪酸氧化缺陷胎儿且自身携带部分损害其线粒体内脂肪酸氧化途径的基因突变的女性中，AFLP和其他严重妊娠并发症的发生率有所增加。本文综述了将AFLP与损害线粒体内脂肪酸氧化的遗传和后天因素联系起来的证据。基于这一综述，针对受影响的母亲和婴儿的产科、围产期和新生儿管理提出了建议。
