了解深圳地区妊娠期急性脂肪肝(AFLP)的发病率、临床特点及母婴结局。

统计深圳地区2003年至2010年8年间分娩孕产妇数，收集符合诊断为妊娠期急性脂肪肝的病例，分析其发病率、临床特点及母婴结局。

8年间深圳地区分娩孕产妇982 761例次，符合妊娠期急性脂肪肝诊断标准的病例44例，发病率为1/22 335。44例妊娠合并急性脂肪肝患者中，合并妊娠期高血压疾病的有23例(52%)；平均年龄(27.7±5.7)岁，发病孕周为30^＋2～40^＋3周，初产妇占33例(75%)，多胎妊娠占9例(20%)。37例(84%)首发症状为不同程度的消化道症状，40例(91%)出现黄疸并进行性加重，39例(89%)病例出现凝血功能障碍，25例(57%)出现肾功能不全，9例(20%)合并肝性脑病，5例(11%)出现多器官功能障碍。孕产妇痊愈39例，死亡5例，孕产妇死亡率为11%；分娩胎儿55例，死胎9例，新生儿死亡1例，围生儿死亡率为18%。

深圳地区妊娠期急性脂肪肝以晚孕期消化道症状为首发症状，同时伴有黄疸、肝酶升高、凝血功能障碍，并出现早发肾功能不全为主要临床特点。早期诊断，及时终止妊娠可改善母婴结局。

To identify the incidence, clinical features and prognosis of acute fatty liver of pregnancy (AFLP).

The clinical data of AFLP from all hospitals in Shenzhen, China in the past 8 years were collected. The incidence, clinical characteristics and outcomes of AFLP were analyzed.

There were 982 761 pregnant women from 2003 to 2010 and 44 of them were diagnosed as AFLP. The incidence of AFLP was 1/22 335. In this cohort of 44 cases with AFLP, 23 patients complicated by gestational hypertension disease, and the incidence was 52%. The mean (SD)maternal age was (27.7±5.7) and the gestational age at onset was 30^{+ 2}-40^{+ 3} weeks. Thirty-three cases (75%) of them were primipara and 9 cases (20%) were multiple pregnancies. The first symptom was gastrointestinal symptoms 37 cases (84%), aggravated jaundice 40 cases (91%), coagulopathy 39 cases (89%), hepatorenal syndrome 25 cases (57%), hepatic encephalopathy 9 cases (20%), and multi-organ dysfunction syndrome 5 cases (11%). Thirty-nine cases were cured while 5 women died. The maternal mortality was 11%. The fetal outcomes showed 45 livinginfants, 9 fetuses death, and 1 neonatal death. The prenatal mortality was 18%.

The first symptom of AFLP was gastrointestinal symptoms in late pregnancy and the AFLP was associated with jaundice, high liver enzymes, coagulopathy, and early-onset acute renal failure. Early diagnosis and timely terminating pregnancy may improve the outcomes.