Bacq Y
Service d'hepatogastroentérologie, Hôpital Trousseau, Tours, France.
Semin Perinatol. 1998 Apr;22(2):134-40. doi: 10.1016/s0146-0005(98)80045-1.
Acute fatty liver of pregnancy is a rare clinical entity unique to pregnancy that occurs during the third trimester. The obstetric team must be familiar with this disease because early diagnosis and prompt delivery have dramatically improved prognosis, which was often fatal for both mother and child. Clinicians must have a high index of suspicion for this condition when a woman has nausea or vomiting, abdominal pain (particularly epigastric), jaundice, polyuria-polydipsia (without diabetes), increased serum transaminase activity or thrombocytopenia in late pregnancy. The disease rarely recurs during a subsequent pregnancy. The cause is unknown, but some cases of acute fatty liver of pregnancy have been associated with a genetic deficiency of fatty acid beta-oxidation. Because of the possibility of this congenital deficiency, infants of affected mothers should undergo close follow-up from birth.
妊娠急性脂肪肝是一种妊娠期间特有的罕见临床病症,发生于孕晚期。产科团队必须熟悉这种疾病,因为早期诊断和及时分娩已显著改善了预后,而该病过去常常对母婴均致命。当孕妇在妊娠晚期出现恶心或呕吐、腹痛(尤其是上腹部)、黄疸、多尿多饮(无糖尿病)、血清转氨酶活性升高或血小板减少时,临床医生必须对这种情况高度怀疑。该病在随后的妊娠中很少复发。病因不明,但一些妊娠急性脂肪肝病例与脂肪酸β氧化的遗传缺陷有关。由于存在这种先天性缺陷的可能性,患病母亲的婴儿自出生起就应接受密切随访。