Rolfes D B, Ishak K G
Hepatology. 1985 Nov-Dec;5(6):1149-58. doi: 10.1002/hep.1840050615.
Acute fatty liver of pregnancy is a disease of the third trimester which is generally considered to be rare and to have a grave prognosis. This study found an optimistic outlook for patients with acute fatty liver of pregnancy due to early termination of the pregnancy as well as the recognition of milder cases. In prospectively followed women, a maternal mortality of 8% and a fetal mortality of 14% were observed. The disorder also appears to be more common than previously suspected and should be considered in all women with liver dysfunction in late pregnancy, even if they are anicteric. Histologically, the characteristic fine droplet steatosis usually produces distinct vacuolization in sections stained with hematoxylin-eosin. However, early in the course of the illness, liver cells have a ballooned appearance and the presence of lipid is masked. When accompanied by a significant necroinflammatory reaction, this stage may be difficult to distinguish from acute viral hepatitis. Whenever acute fatty liver of pregnancy is suspected, a small piece of the biopsy should be reserved for special stains to confirm the presence of lipid in frozen sections. Significant loss of hepatic parenchyma is a regular accompaniment of acute fatty liver of pregnancy and is due to hepatocytolysis, acidophilic degeneration and liver cell atrophy. Extramedullary hematopoiesis and giant mitochondria are often present; the latter change is probably an adaptive or degenerative response to an altered metabolic environment. Despite the frequent presence of signs and symptoms of toxemia in patients with acute fatty liver of pregnancy, no histologic overlap was observed, suggesting that they represent distinct etiologic entities.
妊娠急性脂肪肝是一种发生于孕晚期的疾病,通常被认为较为罕见且预后严重。本研究发现,由于妊娠早期终止以及对病情较轻病例的识别,妊娠急性脂肪肝患者的预后较为乐观。在对孕妇进行前瞻性随访时,观察到孕产妇死亡率为8%,胎儿死亡率为14%。这种疾病似乎也比之前怀疑的更为常见,对于所有妊娠晚期出现肝功能障碍的女性,即使无黄疸,也应考虑该病。组织学上,特征性的微小滴状脂肪变性通常在苏木精-伊红染色切片中产生明显的空泡化。然而,在疾病早期,肝细胞呈气球样外观,脂质的存在被掩盖。当伴有显著的坏死性炎症反应时,这一阶段可能难以与急性病毒性肝炎相区分。每当怀疑妊娠急性脂肪肝时,应保留一小片活检组织用于特殊染色,以在冰冻切片中确认脂质的存在。肝实质的显著丧失是妊娠急性脂肪肝的常见伴随情况,这是由于肝细胞溶解、嗜酸性变性和肝细胞萎缩所致。常出现髓外造血和巨大线粒体;后者的改变可能是对代谢环境改变的一种适应性或退行性反应。尽管妊娠急性脂肪肝患者常出现毒血症的体征和症状,但未观察到组织学上的重叠,这表明它们代表不同的病因实体。